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Sample records for anderson-fabry disease long-term

  1. Time of Anderson-Fabry Disease Detection and Cardiovascular Presentation

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    K. Selthofer-Relatic

    2018-01-01

    Full Text Available Background. Anderson-Fabry disease is an X-linked inherited disease, which manifests in a different manner depending on gender and genotype. Making a working diagnosis of Anderson-Fabry disease is difficult because of several reasons: (a that it is a multiorgan disease with wide variety of phenotypes, (b different timelines of presentation, (c gender differences, and (d possible coexistence with other comorbidities. Late-onset/cardiac type of presentation with minimal involvement of other organs can additionally make diagnosis difficult. Aim. To describe different cardiac manifestations at different time points in the course of the disease: (1 72-year-old female (echocardiography detection, heterozygote, significant left and mild right ventricular hypertrophy; (2 62-year-old male (echocardiography detection, hemizygote, left ventricular hypertrophy, implanted cardiac pacemaker, a performed percutaneous coronary intervention after myocardial infarction, degenerative medium degree aortic valve stenosis; (3 45-year-old female (asymptomatic/family screening, heterozygote, thickened mitral papillary muscle, mild left ventricular hypertrophy, first degree diastolic dysfunction; and (4 75-year-old female (symptomatic/family screening, heterozygote, cardiomyopathy with reduced left ventricular ejection fraction after heart surgery (mitral valve annuloplasty and plastic repair of the tricuspid valve. Conclusion. All patients have Anderson-Fabry disease but with different clinical presentations depending on the gender, the type of mutation, and the time of detection. All these features can make the patients’ profiles unique and delay the time of detection.

  2. Anderson-Fabry, the histrionic disease: from genetics to clinical management

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    Franco Cecchi

    2013-02-01

    Full Text Available Anderson-Fabry disease (AFD is an Xlinked lysosomal storage disorder of glycosphingolipid catabolism, due to deficiency or absence of a galactosidase A (α-gal A enzyme. The disease may affect males and females, the latter with an average 10 years delay. Metabolites storage (mostly Gb3 and lyso-Gb3 leads to progressive cellular and multiorgan dysfunction, with either early and late onset variable clinical manifestations that usually reduce quality of life and life expectancy. Heart and kidney failure, stroke and sudden death are the most devastating complications. AFD is always been considered a very rare disease, although new epidemiologic data, based on newborn screening, showed that AFD prevalence is probably underestimated and much higher than previously reported, especially for late-onset atypical phenotypes. Currently, the diagnosis may be easier and simpler by evaluating α-gal A enzyme activity and genetic analysis for GLA gene mutations on dried blood spot. While a marked α-gal A deficiency leads to diagnosis of AFD in hemizygous males, the molecular analysis is mandatory in heterozygous females. However, referral to a center with an expert multidisciplinary team is highly advisable, in order to ensure careful management and treatment of patients, based also on accurate molecular and biochemical data interpretation. While long-term efficacy of enzyme replacement therapy (ERT in advanced stage is still debated, increasing evidence shows greater efficacy of early treatment initiation. Concomitant, organ-specific therapy is also needed. New treatment approaches, such as chemical chaperone therapy, alone or in combination with ERT, are currently under investigation. The present review illustrates the major features of the disease, focusing also on biochemical and genetic aspects.

  3. Focal Reduction in Cardiac 123I-Metaiodobenzylguanidine Uptake in Patients With Anderson-Fabry Disease.

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    Yamamoto, Saori; Suzuki, Hideaki; Sugimura, Koichiro; Tatebe, Shunsuke; Aoki, Tatsuo; Miura, Masanobu; Yaoita, Nobuhiro; Sato, Haruka; Kozu, Katuya; Ota, Hideki; Takanami, Kentaro; Takase, Kei; Shimokawa, Hiroaki

    2016-11-25

    It remains to be elucidated whether cardiac sympathetic nervous activity is impaired in patients with Anderson-Fabry disease (AFD).Methods and Results:We performed 123 I-meta-iodobenzylguanidine (MIBG) scintigraphy and gadolinium-enhanced cardiovascular magnetic resonance (CMR) in 5 AFD patients. MIBG uptake in the inferolateral wall, where wall thinning and delayed enhancement were noted on CMR, was significantly lower compared with the anteroseptal wall. The localized reduction in MIBG uptake was also noted in 2 patients with no obvious abnormal findings on CMR. Cardiac sympathetic nervous activity is impaired in AFD before development of structural myocardial abnormalities. (Circ J 2016; 80: 2550-2551).

  4. The histological basis of late gadolinium enhancement cardiovascular magnetic resonance in a patient with Anderson-Fabry disease

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    Moon, J. C.; Sheppard, M.; Reed, E.; Lee, P.; Elliott, P. M.; Pennell, D. J.

    2006-01-01

    Anderson-Fabry Disease (AFD) is a storage disease that mimics hypertrophic cardiomyopathy. Late gadolinium enhancement (LGE) by cardiovascular magnetic resonance occurs in approximately 50% of patients in the basal inferolateral LV wall, but how an intracellular storage disease causes focal LGE is unknown. We present a whole-heart histological validation that LGE is caused by focal myocardial collagen scarring. This scarring may be the substrate for electrical re-entry and sudden arrhythmic d...

  5. Atypical Distribution of Late Gadolinium Enhancement of the Left Ventricle on Cardiac Magnetic Resonance in Classical Anderson-Fabry Disease

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    Kasuya, Shusuke; Suzuki, Masayo; Inaoka, Tsutomu; Odashima, Masayuki; Nakatsuka, Tomoya; Ishikawa, Rumiko; Tokuyama, Wataru; Terada, Hitoshi

    2016-01-01

    Anderson-Fabry disease (AFD) is an X-linked lysosomal storage disorder caused by a deficiency of alpha-galactosidase A. Approximately 50% of patients with AFD may have cardiac involvement. Gadolinium-enhanced cardiac magnetic resonance (CMR) is useful for the diagnosis of cardiac involvement of AFD by recognizing typical late gadolinium enhancement (LGE) patterns. We report a 48-year-old man with cardiac involvement in classical AFD, showing atypical distribution of the LGE at the mid-lateral...

  6. Clinical and genetic predictors of major cardiac events in patients with Anderson-Fabry Disease.

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    Patel, Vimal; O'Mahony, Constantinos; Hughes, Derralynn; Rahman, Mohammad Shafiqur; Coats, Caroline; Murphy, Elaine; Lachmann, Robin; Mehta, Atul; Elliott, Perry M

    2015-06-01

    Anderson-Fabry Disease (AFD) is an X linked lysosomal storage disorder caused by mutations in the α-galactosidase A gene. Some mutations are associated with prominent and, in many cases, exclusive cardiac involvement. The primary aims of this study were to determine the incidence of major cardiac events in AFD and to identify clinical and genetic predictors of adverse outcomes. We studied 207 patients with AFD (47% male, mean age 44 years, mean follow-up 7.1 years). Fifty-eight (28%) individuals carried mutations that have been previously associated with a cardiac predominant phenotype. Twenty-one (10%) developed severe heart failure (New York Heart Association functional class (NYHA) ≥3), 13 (6%) developed atrial fibrillation (AF), 13 (6%) received devices for the treatment of bradycardia; there were a total of 7 (3%) cardiac deaths. The incidence of the primary endpoint (a composite of new onset AF, NYHA ≥ 3 symptoms, device insertion for bradycardia and cardiac death) was 2.64 per 100 person-years (CI 1.78 to 3.77). Age (HR 1.04, CI 1.01 to 1.08, p=0.004), Mainz Severity Score Index score (HR 1.05, CI 1.01 to 1.09, p=0.012) and QRS duration (HR 1.03, CI 1.00 to 1.05, p=0.020) were significant independent predictors of the primary endpoint. The presence of a cardiac genetic variant did not predict the primary end point. AFD is associated with a high burden of cardiac morbidity and mortality. Adverse cardiac outcomes are associated with age, global disease severity and advanced cardiac disease but not the presence of cardiac genetic variants. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

  7. Cardiac sympathetic neuronal damage precedes myocardial fibrosis in patients with Anderson-Fabry disease

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    Imbriaco, Massimo; Piscopo, Valentina; Ponsiglione, Andrea; Nappi, Carmela; Puglia, Marta; Dell'Aversana, Serena; Spinelli, Letizia; Cuocolo, Alberto; Pellegrino, Teresa; Petretta, Mario; Riccio, Eleonora; Pisani, Antonio

    2017-01-01

    Cardiac sympathetic denervation may be detectable in patients with Anderson-Fabry disease (AFD), suggesting its usefulness for early detection of the disease. However, the relationship between sympathetic neuronal damage measured by 123 I-metaiodobenzylguanidine (MIBG) imaging with myocardial fibrosis on cardiac magnetic resonance (CMR) is still unclear. Cardiac sympathetic innervation was assessed by 123 I-MIBG single-photon emission computed tomography (SPECT) in 25 patients with genetically proved AFD. Within one month from MIBG imaging, all patients underwent contrast-enhanced CMR. MIBG defect size and fibrosis size on CMR were measured for the left ventricle (LV) and expressed as %LV. Patients were divided into three groups according to MIBG and CMR findings: (1) matched normal, without MIBG defects and without fibrosis on CMR (n = 10); (2) unmatched, with MIBG defect but without fibrosis (n = 5); and (3) matched abnormal, with MIBG defect and fibrosis (n = 10). The three groups did not differ with respect to age, gender, α-galactosidase, proteinuria, glomerular filtration rate, and troponin I, while New York Heart Association class (p = 0.008), LV hypertrophy (p = 0.05), and enzyme replacement therapy (p = 0.02) were different among groups. Although in patients with matched abnormal findings, there was a significant correlation between MIBG defect size and area of fibrosis at CMR (r 2 = 0.98, p < 0.001), MIBG defect size was larger than fibrosis size (26 ± 23 vs. 18 ± 13%LV, p = 0.02). Sympathetic neuronal damage is frequent in AFD patients, and it may precede myocardial damage, such as fibrosis. Thus, 123 I-MIBG imaging can be considered a challenging technique for early detection of cardiac involvement in AFD. (orig.)

  8. Cardiac sympathetic neuronal damage precedes myocardial fibrosis in patients with Anderson-Fabry disease

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    Imbriaco, Massimo; Piscopo, Valentina; Ponsiglione, Andrea; Nappi, Carmela; Puglia, Marta; Dell' Aversana, Serena; Spinelli, Letizia; Cuocolo, Alberto [University Federico II, Department of Advanced Biomedical Sciences, Naples (Italy); Pellegrino, Teresa [National Council of Research, Institute of Biostructure and Bioimaging, Naples (Italy); Petretta, Mario [University Federico II, Department of Translational Medical Sciences, Naples (Italy); Riccio, Eleonora; Pisani, Antonio [University of Naples Federico II, Department of Public Health, Naples (Italy)

    2017-12-15

    Cardiac sympathetic denervation may be detectable in patients with Anderson-Fabry disease (AFD), suggesting its usefulness for early detection of the disease. However, the relationship between sympathetic neuronal damage measured by {sup 123}I-metaiodobenzylguanidine (MIBG) imaging with myocardial fibrosis on cardiac magnetic resonance (CMR) is still unclear. Cardiac sympathetic innervation was assessed by {sup 123}I-MIBG single-photon emission computed tomography (SPECT) in 25 patients with genetically proved AFD. Within one month from MIBG imaging, all patients underwent contrast-enhanced CMR. MIBG defect size and fibrosis size on CMR were measured for the left ventricle (LV) and expressed as %LV. Patients were divided into three groups according to MIBG and CMR findings: (1) matched normal, without MIBG defects and without fibrosis on CMR (n = 10); (2) unmatched, with MIBG defect but without fibrosis (n = 5); and (3) matched abnormal, with MIBG defect and fibrosis (n = 10). The three groups did not differ with respect to age, gender, α-galactosidase, proteinuria, glomerular filtration rate, and troponin I, while New York Heart Association class (p = 0.008), LV hypertrophy (p = 0.05), and enzyme replacement therapy (p = 0.02) were different among groups. Although in patients with matched abnormal findings, there was a significant correlation between MIBG defect size and area of fibrosis at CMR (r{sup 2} = 0.98, p < 0.001), MIBG defect size was larger than fibrosis size (26 ± 23 vs. 18 ± 13%LV, p = 0.02). Sympathetic neuronal damage is frequent in AFD patients, and it may precede myocardial damage, such as fibrosis. Thus, {sup 123}I-MIBG imaging can be considered a challenging technique for early detection of cardiac involvement in AFD. (orig.)

  9. The histological basis of late gadolinium enhancement cardiovascular magnetic resonance in a patient with Anderson-Fabry disease.

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    Moon, James C; Sheppard, Mary; Reed, Emma; Lee, Phillip; Elliott, Perry M; Pennell, Dudley J

    2006-01-01

    Anderson-Fabry Disease (AFD) is a storage disease that mimics hypertrophic cardiomyopathy. Late gadolinium enhancement (LGE) by cardiovascular magnetic resonance occurs in approximately 50% of patients in the basal inferolateral LV wall, but how an intracellular storage disease causes focal LGE is unknown. We present a whole-heart histological validation that LGE is caused by focal myocardial collagen scarring. This scarring may be the substrate for electrical re-entry and sudden arrhythmic death. The reasons for this distribution of fibrosis are unclear, but may reflect inhomogeneous left ventricular wall stress.

  10. Intraoperative Diagnosis of Anderson-Fabry Disease in Patients With Obstructive Hypertrophic Cardiomyopathy Undergoing Surgical Myectomy.

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    Cecchi, Franco; Iascone, Maria; Maurizi, Niccolò; Pezzoli, Laura; Binaco, Irene; Biagini, Elena; Fibbi, Maria Laura; Olivotto, Iacopo; Pieruzzi, Federico; Fruntelata, Ana; Dorobantu, Lucian; Rapezzi, Claudio; Ferrazzi, Paolo

    2017-10-01

    Diagnostic screening for Anderson-Fabry cardiomyopathy (AFC) is performed in the presence of specific clinical red flags in patients with hypertrophic cardiomyopathy (HCM) older than 25 years. However, left ventricular outflow tract obstruction (LVOTO) has been traditionally considered an exclusion criteria for AFC. To examine a series of patients diagnosed with HCM and severe basal LVOTO undergoing myectomy in whom the diagnosis of AFC was suspected by the cardiac surgeon intraoperatively and confirmed by histological and genetic examinations. This retrospective analysis of patients undergoing surgical septal reduction strategies was conducted in 3 European tertiary referral centers for HCM from July 2013 to December 2016. Patients with a clinical diagnosis of obstructive HCM referred for surgical management of LVOTO were observed for at least 18 months after the procedure (mean [SD] follow-up, 33 [14] months). Etiology of patients with HCM who underwent surgical myectomy. From 2013, 235 consecutive patients with a clinical diagnosis of HCM underwent septal myectomy. The cardiac surgeon suspected a storage disease in 3 patients (1.3%) while inspecting their heart samples extracted from myectomy. The mean (SD) age at diagnosis for these 3 patients was 42 (4) years; all were male. None of the 3 patients presented with extracardiac features suggestive of AFC. All patients showed asymmetrical left ventricular hypertrophy, with maximal left ventricular thickness in the basal septum (19-31 mm), severe basal LVOTO (70-120 mm Hg), and left atrial dilatation (44-57 mm). Only 1 patient presented with late gadolinium enhancement on cardiovascular magnetic resonance at the right ventricle insertion site. The mean (SD) age at surgical procedure was 63 (5) years. On tactile sensation, the surgeon felt a spongy consistency of the surgical samples, different from the usual stony-elastic consistency typical of classic HCM, and this prompted histological examinations. Histology

  11. Gadolinium enhanced cardiovascular magnetic resonance in Anderson-Fabry disease. Evidence for a disease specific abnormality of the myocardial interstitium.

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    Moon, James C C; Sachdev, Bhavesh; Elkington, Andrew G; McKenna, William J; Mehta, Atul; Pennell, Dudley J; Leed, Philip J; Elliott, Perry M

    2003-12-01

    Anderson-Fabry Disease (AFD), an X-linked disorder of sphingolipid metabolism, is a cause of idiopathic left ventricular hypertrophy but the mechanism of hypertrophy is poorly understood. Gadolinium enhanced cardiovascular magnetic resonance can detect focal myocardial fibrosis. We hypothesised that hyperenhancement would be present in AFD. Eighteen males (mean 43+/-14 years) and eight female heterozygotes (mean 48+/-12 years) with AFD underwent cine and late gadolinium cardiovascular magnetic resonance. Nine male (50%) had myocardial hyperenhancement ranging from 3.4% to 20.6% (mean 7.7+/-5.7%) of total myocardium; in males, percentage hyperenhancement related to LV mass index (r=0.78, P=0.0002) but not to ejection fraction or left ventricular volumes. Lesser hyperenhancement was also found in four (50%) heterozygous females (mean 4.6%). In 12 (92%) patients with abnormal gadolinium uptake, hyperenhancement occurred in the basal infero-lateral wall where, unlike myocardial infarction, it was not sub-endocardial. In two male patients with severe LVH (left ventricular hypertrophy) and systolic impairment there was additional hyperenhancement in other myocardial segments. These observations suggests that myocardial fibrosis occurs in AFD and may contribute to the hypertrophy and the natural history of the disease.

  12. Identification and assessment of Anderson-Fabry disease by cardiovascular magnetic resonance noncontrast myocardial T1 mapping.

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    Sado, Daniel M; White, Steven K; Piechnik, Stefan K; Banypersad, Sanjay M; Treibel, Thomas; Captur, Gabriella; Fontana, Marianna; Maestrini, Viviana; Flett, Andrew S; Robson, Matthew D; Lachmann, Robin H; Murphy, Elaine; Mehta, Atul; Hughes, Derralynn; Neubauer, Stefan; Elliott, Perry M; Moon, James C

    2013-05-01

    Anderson-Fabry disease (AFD) is a rare but underdiagnosed intracellular lipid disorder that can cause left ventricular hypertrophy (LVH). Lipid is known to shorten the magnetic resonance imaging parameter T1. We hypothesized that noncontrast T1 mapping by cardiovascular magnetic resonance would provide a novel and useful measure in this disease with potential to detect early cardiac involvement and distinguish AFD LVH from other causes. Two hundred twenty-seven subjects were studied: patients with AFD (n=44; 55% with LVH), healthy volunteers (n=67; 0% with LVH), patients with hypertension (n=41; 24% with LVH), patients with hypertrophic cardiomyopathy (n=34; 100% with LVH), those with severe aortic stenosis (n=21; 81% with LVH), and patients with definite amyloid light-chain (AL) cardiac amyloidosis (n=20; 100% with LVH). T1 mapping was performed using the shortened modified Look-Locker inversion sequence on a 1.5-T magnet before gadolinium administration with primary results derived from the basal and midseptum. Compared with health volunteers, septal T1 was lower in AFD and higher in other diseases (AFD versus healthy volunteers versus other patients, 882±47, 968±32, 1018±74 milliseconds; Pgadolinium enhancement (1001±82 versus 891±38 milliseconds; P<0.0001). Noncontrast T1 mapping shows potential as a unique and powerful measurement in the imaging assessment of LVH and AFD.

  13. Use of Myocardial T1 Mapping at 3.0 T to Differentiate Anderson-Fabry Disease from Hypertrophic Cardiomyopathy.

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    Karur, Gauri R; Robison, Sean; Iwanochko, Robert M; Morel, Chantal F; Crean, Andrew M; Thavendiranathan, Paaladinesh; Nguyen, Elsie T; Mathur, Shobhit; Wasim, Syed; Hanneman, Kate

    2018-04-24

    Purpose To compare left ventricular (LV) and right ventricular (RV) 3.0-T cardiac magnetic resonance (MR) imaging T1 values in Anderson-Fabry disease (AFD) and hypertrophic cardiomyopathy (HCM) and evaluate the diagnostic value of native T1 values beyond age, sex, and conventional imaging features. Materials and Methods For this prospective study, 30 patients with gene-positive AFD (37% male; mean age ± standard deviation, 45.0 years ± 14.1) and 30 patients with HCM (57% male; mean age, 49.3 years ± 13.5) were prospectively recruited between June 2016 and September 2017 to undergo cardiac MR imaging T1 mapping with a modified Look-Locker inversion recovery (MOLLI) acquisition scheme at 3.0 T (repetition time msec/echo time msec, 280/1.12; section thickness, 8 mm). LV and RV T1 values were evaluated. Statistical analysis included independent samples t test, receiver operating characteristic curve analysis, multivariable logistic regression, and likelihood ratio test. Results Septal LV, global LV, and RV native T1 values were significantly lower in AFD compared with those in HCM (1161 msec ± 47 vs 1296 msec ± 55, respectively [P 3.0 T are significantly lower in patients with AFD compared with those with HCM and provide independent and incremental diagnostic value beyond age, sex, and conventional imaging features. © RSNA, 2018.

  14. Cardiovascular magnetic resonance demonstration of the spectrum of morphological phenotypes and patterns of myocardial scarring in Anderson-Fabry disease.

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    Deva, Djeven Parameshvara; Hanneman, Kate; Li, Qin; Ng, Ming Yen; Wasim, Syed; Morel, Chantal; Iwanochko, Robert M; Thavendiranathan, Paaladinesh; Crean, Andrew Michael

    2016-03-31

    Although it is known that Anderson-Fabry Disease (AFD) can mimic the morphologic manifestations of hypertrophic cardiomyopathy (HCM) on echocardiography, there is a lack of cardiovascular magnetic resonance (CMR) literature on this. There is limited information in the published literature on the distribution of myocardial fibrosis in patients with AFD, with scar reported principally in the basal inferolateral midwall. All patients with confirmed AFD undergoing CMR at our center were included. Left ventricular (LV) volumes, wall thicknesses and scar were analyzed offline. Patients were categorized into 4 groups: (1) no wall thickening; (2) concentric hypertrophy; (3) asymmetric septal hypertrophy (ASH); and (4) apical hypertrophy. Charts were reviewed for clinical information. Thirty-nine patients were included (20 males [51%], median age 45.2 years [range 22.3-64.4]). Almost half (17/39) had concentric wall thickening. Almost half (17/39) had pathologic LV scar; three quarters of these (13/17) had typical inferolateral midwall scar. A quarter (9/39) had both concentric wall thickening and typical inferolateral scar. A subgroup with ASH and apical hypertrophy (n = 5) had greater maximum wall thickness, total LV scar, apical scar and mid-ventricular scar than those with concentric hypertrophy (n = 17, p < 0.05). Patients with elevated LVMI had more overall arrhythmia (p = 0.007) more ventricular arrhythmia (p = 0.007) and sustained ventricular tachycardia (p = 0.008). Concentric thickening and inferolateral mid-myocardial scar are the most common manifestations of AFD, but the spectrum includes cases morphologically identical to apical and ASH subtypes of HCM and these have more apical and mid-ventricular LV scar. Significant LVH is associated with ventricular arrhythmia.

  15. First experience of simultaneous PET/MRI for the early detection of cardiac involvement in patients with Anderson-Fabry disease

    International Nuclear Information System (INIS)

    Nappi, Carmela; Altiero, Michele; Imbriaco, Massimo; Giudice, Caterina Anna; Spinelli, Letizia; Cuocolo, Alberto; Nicolai, Emanuele; Aiello, Marco; Diomiaiuti, Claudio Tommaso; Pisani, Antonio

    2015-01-01

    Anderson-Fabry disease (AFD) is an X-linked lysosomal storage disorder associated with severe multiorgan dysfunction and premature death. Early diagnosis and treatment strategies play a key role in patient outcome. We investigated the potential role of hybrid PET/MR imaging in the assessment of early cardiac involvement in AFD patients. Thirteen AFD patients without cardiac symptoms and with normal left ventricular function underwent simultaneous cardiac PET/MR imaging after administration of 18 F-FDG. Cardiac FDG uptake was quantified by measuring the standardized uptake value in 17 myocardial segments in each subject. The coefficient of variation (COV, i.e. the standard deviation divided by the average) of the uptake of the 17 segments was calculated as an index of heterogeneity in the heart. Six patients exhibited focal late gadolinium enhancement (LGE) indicating intramyocardial fibrosis, and four of these also had positive short inversion time inversion recovery (STIR) sequences. All patients with LGE and positive STIR MR images showed focal FDG uptake in the corresponding myocardial segments indicating inflammation. Of the seven patients with negative LGE and STIR images, five showed homogeneous FDG cardiac uptake and two showed heterogeneous FDG uptake. The COV was significantly greater in patients with focal FDG uptake (0.25 ± 0.02) than in those without (0.14 ± 0.07, p < 0.01). PET/MR imaging is clinically feasible for the early detection of cardiac involvement in patients with AFD. Further studies evaluating the role of hybrid PET/MR imaging in management of the disease in larger patient populations are warranted. (orig.)

  16. First experience of simultaneous PET/MRI for the early detection of cardiac involvement in patients with Anderson-Fabry disease

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    Nappi, Carmela; Altiero, Michele; Imbriaco, Massimo; Giudice, Caterina Anna; Spinelli, Letizia; Cuocolo, Alberto [University Federico II, Department of Advanced Biomedical Sciences, Naples (Italy); Nicolai, Emanuele; Aiello, Marco; Diomiaiuti, Claudio Tommaso [IRCCS SDN, Naples (Italy); Pisani, Antonio [University Federico II, Department of Public Health, Naples (Italy)

    2015-03-26

    Anderson-Fabry disease (AFD) is an X-linked lysosomal storage disorder associated with severe multiorgan dysfunction and premature death. Early diagnosis and treatment strategies play a key role in patient outcome. We investigated the potential role of hybrid PET/MR imaging in the assessment of early cardiac involvement in AFD patients. Thirteen AFD patients without cardiac symptoms and with normal left ventricular function underwent simultaneous cardiac PET/MR imaging after administration of {sup 18}F-FDG. Cardiac FDG uptake was quantified by measuring the standardized uptake value in 17 myocardial segments in each subject. The coefficient of variation (COV, i.e. the standard deviation divided by the average) of the uptake of the 17 segments was calculated as an index of heterogeneity in the heart. Six patients exhibited focal late gadolinium enhancement (LGE) indicating intramyocardial fibrosis, and four of these also had positive short inversion time inversion recovery (STIR) sequences. All patients with LGE and positive STIR MR images showed focal FDG uptake in the corresponding myocardial segments indicating inflammation. Of the seven patients with negative LGE and STIR images, five showed homogeneous FDG cardiac uptake and two showed heterogeneous FDG uptake. The COV was significantly greater in patients with focal FDG uptake (0.25 ± 0.02) than in those without (0.14 ± 0.07, p < 0.01). PET/MR imaging is clinically feasible for the early detection of cardiac involvement in patients with AFD. Further studies evaluating the role of hybrid PET/MR imaging in management of the disease in larger patient populations are warranted. (orig.)

  17. Functional analysis of variant lysosomal acid glycosidases of Anderson-Fabry and Pompe disease in a human embryonic kidney epithelial cell line (HEK 293 T).

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    Ebrahim, Hatim Y; Baker, Robert J; Mehta, Atul B; Hughes, Derralynn A

    2012-03-01

    The functional significance of missense mutations in genes encoding acid glycosidases of lysosomal storage disorders (LSDs) is not always clear. Here we describe a method of investigating functional properties of variant enzymes in vitro using a human embryonic kidney epithelial cell line. Site-directed mutagenesis was performed on the parental plasmids containing cDNA encoding for alpha-galactosidase A (α-Gal A) and acid maltase (α-Glu) to prepare plasmids encoding relevant point mutations. Mutant plasmids were transfected into HEK 293 T cells, and transient over-expression of variant enzymes was measured after 3 days. We have illustrated the method by examining enzymatic activities of four unknown α-Gal A and one α-Glu variants identified in our patients with Anderson-Fabry disease and Pompe diseases respectively. Comparison with control variants known to be either pathogenic or non-pathogenic together with over-expression of wild-type enzyme allowed determination of the pathogenicity of the mutation. One leader sequence novel variant of α-Gal A (p.A15T) was shown not to significantly reduce enzyme activity, whereas three other novel α-Gal A variants (p.D93Y, p.L372P and p.T410I) were shown to be pathogenic as they resulted in significant reduction of enzyme activity. A novel α-Glu variant (p.L72R) was shown to be pathogenic as this significantly reduced enzyme activity. Certain acid glycosidase variants that have been described in association with late-onset LSDs and which are known to have variable residual plasma and leukocyte enzyme activity in patients appear to show intermediate to low enzyme activity (p.N215S and p.Q279E α-Gal A respectively) in the over-expression system.

  18. Relationship between left ventricular diastolic function and myocardial sympathetic denervation measured by {sup 123}I-meta-iodobenzylguanidine imaging in Anderson-Fabry disease

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    Spinelli, Letizia; Giudice, Caterina Anna; Imbriaco, Massimo; Trimarco, Bruno; Cuocolo, Alberto [University Federico II, Department of Advanced Biomedical Sciences, Naples (Italy); Pellegrino, Teresa [Institute of Biostructure and Bioimaging, National Council of Research, Naples (Italy); Pisani, Antonio; Riccio, Eleonora [University Federico II, Department of Public Health, Naples (Italy); Salvatore, Marco [IRCCS SDN, Naples (Italy)

    2016-04-15

    Whether cardiac sympathetic nervous function abnormalities may be present in patients with Anderson-Fabry disease (AFD) remains unexplored. We investigated the relationship between left ventricular (LV) function and cardiac sympathetic nervous function in patients with AFD. Twenty-five patients (12 men, mean age 43 ± 13 years) with genetically proved AFD and preserved LV ejection fraction and ten age and gender-matched control subjects underwent speckle tracking echocardiography and {sup 123}I-meta-iodobenzylguanidine (MIBG) imaging from which early and late heart to mediastinum (H/M) ratios and myocardial washout rate values were calculated. In AFD patients, a significant correlation between late H/M ratio and LV mass index (r = -61, p = 0.001), left atrial volume (r = -0.72, p < 0.001), systolic pulmonary artery pressure (r = -0.75, p < 0.001), and early diastolic untwisting rate (r = -0.66, p < 0.001) was found. Ten AFD patients exhibited a late H/M ratio below two fold standard deviation of control subjects (≤1.75). Patients showing late H/M ratio ≤ 1.75 had significantly higher LV mass index, relative wall thickness, left atrial volume and systolic pulmonary artery pressure, lower systolic longitudinal strain and an early diastolic untwisting rate compared to patients with late H/M ratio > 1.75. At multivariable linear regression analysis, early diastolic untwisting rate was the only independent predictor of late H/M ratio ≤ 1.75 (odds ratio 1.15, 95 % confidence interval 1.07-1.31, p < 0.05). The present findings provide the first demonstration of a cardiac sympathetic derangement in AFD patients with preserved LV ejection fraction, which is mostly related to LV diastolic dysfunction. (orig.)

  19. Enzyme replacement therapy for Anderson-Fabry disease: A complementary overview of a Cochrane publication through a linear regression and a pooled analysis of proportions from cohort studies.

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    El Dib, Regina; Gomaa, Huda; Ortiz, Alberto; Politei, Juan; Kapoor, Anil; Barreto, Fellype

    2017-01-01

    Anderson-Fabry disease (AFD) is an X-linked recessive inborn error of glycosphingolipid metabolism caused by a deficiency of alpha-galactosidase A. Renal failure, heart and cerebrovascular involvement reduce survival. A Cochrane review provided little evidence on the use of enzyme replacement therapy (ERT). We now complement this review through a linear regression and a pooled analysis of proportions from cohort studies. To evaluate the efficacy and safety of ERT for AFD. For the systematic review, a literature search was performed, from inception to March 2016, using Medline, EMBASE and LILACS. Inclusion criteria were cohort studies, patients with AFD on ERT or natural history, and at least one patient-important outcome (all-cause mortality, renal, cardiovascular or cerebrovascular events, and adverse events) reported. The pooled proportion and the confidence interval (CI) are shown for each outcome. Simple linear regressions for composite endpoints were performed. 77 cohort studies involving 15,305 participants proved eligible. The pooled proportions were as follows: a) for renal complications, agalsidase alfa 15.3% [95% CI 0.048, 0.303; I2 = 77.2%, p = 0.0005]; agalsidase beta 6% [95% CI 0.04, 0.07; I2 = not applicable]; and untreated patients 21.4% [95% CI 0.1522, 0.2835; I2 = 89.6%, plinear regression showed that Fabry patients receiving agalsidase alfa are more likely to have higher rates of composite endpoints compared to those receiving agalsidase beta. Agalsidase beta is associated to a significantly lower incidence of renal, cardiovascular and cerebrovascular events than no ERT, and to a significantly lower incidence of cerebrovascular events than agalsidase alfa. In view of these results, the use of agalsidase beta for preventing major organ complications related to AFD can be recommended.

  20. Comprehensive clinical evaluation of a large Spanish family with Anderson-Fabry disease, novel GLA mutation and severe cardiac phenotype.

    Science.gov (United States)

    San Román-Monserrat, Irene; Moreno-Flores, Victoria; López-Cuenca, David; Rodríguez-González-Herrero, Elena; Guillén-Navarro, Encarna; Rodríguez-González-Herrero, Beatriz; Alegría-Fernández, Marisol; Poza-Cisneros, Gabriela; Piñero-Fernández, Juan A; Sornichero-Martínez, Javier; Gimeno-Blanes, Juan R

    2014-06-06

    Fabry disease is an X-linked multisystemic lysosomal-storage condition. We describe a large family with a novel GLA mutation: p.M187R/g7219 T>G. Anamnesis/physical-exam, blood/urine analysis, α-Gal-A activity and/or genetic study of at-risk individuals and multidisciplinary evaluation in confirmed cases. 4 males and 13 heterozygous-females displayed the mutation. Cardiac/renal/neurological disease was diagnosed at a mean age of 41/29/39 years in males and 51/56/46 years in females. Onset mean age was 20 years versus 42 years. 9/15 had cardiomyopathy. Delta wave suggestive of accessory pathway was identified in 1 male and 2 females. 1 female had cardiac arrest (ventricular fibrillation, 61 years). 2 females and 1 male died suddenly (63, 64 and 57 years). Cardiac-subscore of Mainz Severity-Score-Index was severe for males and females over 40 years. 4/15(26%) developed early renal disease. 2 males needed dialysis. 1 male died at 69 years in spite of kidney-heart transplant. We describe the largest genetically confirmed Spanish family using multidisciplinary evaluation and MSSI calculation. The novel mutation p.M187R/g7219 T>G is associated with a particularly malignant cardiac phenotype in males and females over 40 years. Severity was higher than that of the largest Spanish FOS-cohort. Short-PR with delta is being reported for the first time. Copyright © 2013 Elsevier España, S.L. All rights reserved.

  1. Pituitary diseases : long-term psychological consequences

    NARCIS (Netherlands)

    Tiemensma, Jitske

    2012-01-01

    Nowadays, pituitary adenomas can be appropriately treated, but patients continue to report impaired quality of life (QoL) despite long-term remission or cure. In patients with Cushing’s disease, Cushing’s syndrome or acromegaly, doctors should be aware of subtle cognitive impairments and the

  2. Long-term skeletal findings in Menkes disease

    International Nuclear Information System (INIS)

    Amador, Eva; Domene, Ruth; Fuentes, Cristian; Carreno, Juan-Carlos; Enriquez, Goya

    2010-01-01

    Skeletal findings in infants with Menkes disease, the most characteristic of which are metaphyseal spurs, long-bone fractures and wormian bones, have been widely reported. However, the changes in skeletal features over time are not well known. The long-term findings differ completely from those initially observed and consist of undertubulation and metaphyseal flaring, similar to the findings seen in some types of bone dysplasia. The initial and long-term radiological features in an 8-year-old boy with Menkes disease are illustrated. (orig.)

  3. Long-term results of peripheral arterial disease rehabilitation

    NARCIS (Netherlands)

    Menard, J.R.; Smith, H.E.; Riebe, D.; Braun, C.M.; Blissmer, B.; Patterson, R.B.

    2004-01-01

    Purpose Although the Peripheral Arterial Disease Rehabilitation Program (PADRx) improves walking ability and quality of life over brief periods of follow-up, the long-term durability of results has not been established. This study examined functional status, walking ability, and quality of life in

  4. Long-term efficacy of rasagiline in early Parkinson's disease.

    Science.gov (United States)

    Lew, Mark F; Hauser, Robert A; Hurtig, Howard I; Ondo, William G; Wojcieszek, Joanne; Goren, Tamar; Fitzer-Attas, Cheryl J

    2010-06-01

    This study was designed to follow the long-term efficacy, safety, and tolerability of rasagiline for Parkinson's disease (PD) with data collected from all patients who had ever taken rasagiline during the 12-month TEMPO monotherapy trial (N = 398) and subsequent open-label extension. Patients were followed for up to 6.5 years with a mean of 3.5 +/- 2.1 years. After 12 months, additional PD medications were added as required. Of patients remaining in the trial at 2 years, 46% were maintained on rasagiline monotherapy. The majority of patients received a dopamine agonist prior to levodopa as the first additional dopaminergic agent. Analysis using a Kaplan-Meier method indicated that by 5.4 years only 25% of patients progressed to Hoehn & Yahr stage III. Rasagiline was well tolerated, with 11.3% of patients (45/398) withdrawing because of an adverse event. Rasagiline therapy for PD was effective, well tolerated, and safe in this long-term trial.

  5. Long term cardiovascular consequences of chronic lung disease of prematurity.

    Science.gov (United States)

    Poon, Chuen Yeow; Edwards, Martin Oliver; Kotecha, Sailesh

    2013-12-01

    Pulmonary arterial (PA) hypertension in preterm infant is an important consequence of chronic lung disease of prematurity (CLD) arising mainly due to impaired alveolar development and dysregulated angiogenesis of the pulmonary circulation. Although PA pressure and resistance in these children normalise by school age, their pulmonary vasculature remains hyper-reactive to hypoxia until early childhood. Furthermore, there is evidence that systemic blood pressure in preterm born children with or without CLD is mildly increased at school age and in young adulthood when compared to term-born children. Arterial stiffness may be increased in CLD survivors due to increased smooth muscle tone of the pre-resistance and resistance vessels rather than the loss of elasticity in the large arteries. This review explores the long term effects of CLD on the pulmonary and systemic circulations along with their clinical correlates and therapeutic approaches. Copyright © 2012 Elsevier Ltd. All rights reserved.

  6. Long term results of radiotherapy of degenerative joint diseases

    Energy Technology Data Exchange (ETDEWEB)

    Lindner, H; Freislederer, R

    1982-04-01

    At the Radiologic Department of the Staedt. Krankenhaus Passau, 473 patients with degenerative diseases in the big joints and the spine were irradiated with the caesium unit between 1971 and 1979. Among these patients, 249 could be followed up during a prolonged period (1/2 to 9 years, i.e. 4.2 years on an average). According to the categories of v. Pannewitz, 11% were pain-free at this moment, 21% showed an essential improvement, 29% showed an improvement, and 39% were not influenced by the treatment. 13.5% showed recurrent pains; these were mentioned as 'not influenced' in the statistical analysis. It is proved that the relief of pain does not depend on the age of the patients, but on the anamnesis period, the results of the X-ray examiantion, and the degree of the restriction of mobility. Due to the delay of irradiation, a preliminary treatment mostly produces a less favorable radiotherapeutic result. Compared with other therapeutic methods, the long term results of radiotherapy of degenerative joint diseases are generally favorable. This conclusion is also confirmed by the results of patients checked up more than five years after the treatment.

  7. Role of long term antibiotics in chronic respiratory diseases.

    Science.gov (United States)

    Suresh Babu, K; Kastelik, J; Morjaria, J B

    2013-06-01

    Antibiotics are commonly used in the management of respiratory disorders such as cystic fibrosis (CF), non-CF bronchiectasis, asthma and COPD. In those conditions long-term antibiotics can be delivered as nebulised aerosols or administered orally. In CF, nebulised colomycin or tobramycin improve lung function, reduce number of exacerbations and improve quality of life (QoL). Oral antibiotics, such as macrolides, have acquired wide use not only as anti-microbial agents but also due to their anti-inflammatory and pro-kinetic properties. In CF, macrolides such as azithromycin have been shown to improve the lung function and reduce frequency of infective exacerbations. Similarly macrolides have been shown to have some benefits in COPD including reduction in a number of exacerbations. In asthma, macrolides have been reported to improve some subjective parameters, bronchial hyperresponsiveness and airway inflammation; however have no benefits on lung function or overall asthma control. Macrolides have also been used with beneficial effects in less common disorders such as diffuse panbronchiolitis or post-transplant bronchiolitis obliterans syndrome. In this review we describe our current knowledge the use of long-term antibiotics in conditions such as CF, non-CF bronchiectasis, asthma and COPD together with up-to-date clinical and scientific evidence to support our understanding of the use of antibiotics in those conditions. Copyright © 2013 Elsevier Ltd. All rights reserved.

  8. Subtle cognitive impairments in patients with long-term cure of Cushing's disease

    NARCIS (Netherlands)

    Tiemensma, Jitske; Kokshoorn, Nieke E.; Biermasz, Nienke R.; Keijser, Bart-Jan S. A.; Wassenaar, Moniek J. E.; Middelkoop, Huub A. M.; Pereira, Alberto M.; Romijn, Johannes A.

    2010-01-01

    Active Cushing's disease is associated with cognitive impairments. We hypothesized that previous hypercortisolism in patients with Cushing's disease results in irreversible impairments in cognitive functioning. Therefore, our aim was to assess cognitive functioning after long-term cure of Cushing's

  9. [Chronic obstructive pulmonary disease: I. Long-term prognostic scores].

    Science.gov (United States)

    Junod, Alain F

    2013-10-16

    The chronic obstructive pulmonary disease or COPD will probably be in the year 2020 the third cause of death in the world. It appears therefore appropriate to try to make available tools capable of assessing the prognosis of patients with this disease. In the first part of this series of two papers, the question of the prognosis of stable COPD over several years is addressed. Eight prognostic scores are discussed, all of them published between 2004 and 2012. Their components and characteristics are analysed and commented upon, with, in particular, emphasis on their discriminating power. An Internet program (www.medhyg. ch/scoredoc) supplements this review.

  10. Long-term survival in patients hospitalized for chronic obstructive pulmonary disease

    DEFF Research Database (Denmark)

    Gudmundsson, Gunnar; Ulrik, Charlotte Suppli; Gislason, Thorarinn

    2012-01-01

    Mortality rate is high in patients with chronic obstructive pulmonary disease (COPD). Our aim was to investigate long-term mortality and associated risk factors in COPD patients previously hospitalized for a COPD exacerbation.......Mortality rate is high in patients with chronic obstructive pulmonary disease (COPD). Our aim was to investigate long-term mortality and associated risk factors in COPD patients previously hospitalized for a COPD exacerbation....

  11. Long Term Follow-up of Ventilated Patients with Thoracic Restriction and Neuromuscular Disease

    Directory of Open Access Journals (Sweden)

    Dina Brooks

    2002-01-01

    Full Text Available OBJECTIVE: To evaluate the long term effects of home mechanical ventilation (HMV on pulmonary function, nighttime gas exchange, daytime arterial blood gases, sleep architecture and functional exercise capacity (6 min walk. Patients with respiratory failure attributable to thoracic restrictive disease (TRD (kyphoscoliosis or neuromuscular disease (NMD were assessed, ventilated, trained and followed in a dedicated unit for the care of patients requiring long term ventilation.

  12. [Long-term disease in Danish children reported by the parents].

    Science.gov (United States)

    Nielsen, Anne M; Koefoed, Birgitte Gade; Møller, Ralf; Laursen, Bjarne

    2006-01-23

    The aim of this study was to report the prevalence and nature of long-term diseases and their consequences in children under the age of 16 in Denmark, and to identify the socio-demographic determinants of disease. Parents and stepparents participating in the Danish Health and Morbidity Survey, 2000, were interviewed at home about long-term diseases, including impairments and sequelae after injury and disease, in children under the age of 16 living at home. Answers were given for 7,670 children, and diseases were coded according to ICD-10 by two doctors. Logistic regression analysis was used to identify the determinants and consequences of disease. A total of 16.2% of children had one or more long-term diseases, boys (17.5%) more frequently than girls (14.8%). The prevalence increased through the first six years of life. A social gradient was seen: children of parents with low socioeconomic status or with little education had a higher prevalence. The most frequent disease was asthma (4.9%). Also frequent were congenital disorders (1.6%), otitis media (1.4%) and hearing impairment (0.6%). Children with long-term disease suffered more frequently than others from poor health in general, recent sick leave and poor thriving. The figures for long-term disease reported by the parents participating in the study were in accordance with what was found in earlier studies, but stigmatising and less severe diseases, as well as periodically recurring diseases, were probably underreported. Attention should be paid to the high prevalence of asthma, to the poorer thriving and to the general health status of children with long-term disease, and to the social inequality in children's health.

  13. Long term enzyme replacement therapy for Fabry disease: effectiveness on kidney, heart and brain

    NARCIS (Netherlands)

    Rombach, Saskia M.; Smid, Bouwien E.; Bouwman, Machtelt G.; Linthorst, Gabor E.; Dijkgraaf, Marcel G. W.; Hollak, Carla E. M.

    2013-01-01

    Fabry disease is an X-linked lysosomal storage disorder caused by α-galactosidase A deficiency leading to renal, cardiac, cerebrovascular disease and premature death. Treatment with α-galactosidase A (enzyme replacement therapy, ERT) stabilises disease in some patients, but long term effectiveness

  14. Relationship between chronic obstructive pulmonary disease and subclinical coronary artery disease in long-term smokers

    DEFF Research Database (Denmark)

    Rasmussen, Thomas; Køber, Lars; Pedersen, Jesper Holst

    2013-01-01

    Cardiovascular conditions are reported to be the most frequent cause of death in patients with chronic obstructive pulmonary disease (COPD). However, it remains unsettled whether severity of COPD per se is associated with coronary artery disease (CAD) independent of traditional cardiovascular risk...... factors. The aim of this study was to examine the relationship between the presence and severity of COPD and the amount of coronary artery calcium deposit, an indicator of CAD and cardiac risk, in a large population of current and former long-term smokers....

  15. Sickle Cell Disease with Cyanotic Congenital Heart Disease: Long-Term Outcomes in 5 Children.

    Science.gov (United States)

    Iannucci, Glen J; Adisa, Olufolake A; Oster, Matthew E; McConnell, Michael; Mahle, William T

    2016-12-01

    Sickle cell disease is a risk factor for cerebrovascular accidents in the pediatric population. This risk is compounded by hypoxemia. Cyanotic congenital heart disease can expose patients to prolonged hypoxemia. To our knowledge, the long-term outcome of patients who have combined sickle cell and cyanotic congenital heart disease has not been reported. We retrospectively reviewed patient records at our institution and identified 5 patients (3 girls and 2 boys) who had both conditions. Their outcomes were uniformly poor: 4 died (age range, 12 mo-17 yr); 3 had documented cerebrovascular accidents; and 3 developed ventricular dysfunction. The surviving patient had developmental delays. On the basis of this series, we suggest mitigating hypoxemia, and thus the risk of stroke, in patients who have sickle cell disease and cyanotic congenital heart disease. Potential therapies include chronic blood transfusions, hydroxyurea, earlier surgical correction to reduce the duration of hypoxemia, and heart or bone marrow transplantation.

  16. Long-term Outcomes of Elective Surgery for Diverticular Disease: A Call for Standardization.

    Science.gov (United States)

    Biondi, Alberto; Santullo, Francesco; Fico, Valeria; Persiani, Roberto

    2016-10-01

    To date, the appropriate management of diverticular disease is still controversial. The American Society of Colon and Rectal Surgeons declared that the decision between conservative or surgical approach should be taken by a case-by-case evaluation. There is still lack of evidence in literature about long-term outcomes after elective sigmoid resection for diverticular disease. Considering the potentially key role of the surgical technique in long-term outcomes, there is the need for surgeons to define strict rules to standardize the surgical technique. Currently there are 5 areas of debate in elective surgery for diverticular disease: laparoscopic versus open approach, the site of the proximal and distal colonic division, the vascular approach and the mobilization of the splenic flexure. The purpose of this paper is to review existing knowledge about technical aspects, which represent how the surgeon is able to affect the long-term results.

  17. Quality of life in patients after long-term biochemical cure of cushing's disease

    NARCIS (Netherlands)

    M.O. van Aken (Maarten); A.M. Pereira (Alberto); N.R. Biermasz; S.W. van Thiel (Sjoerd); H. Hoftijzer (Hendrieke); J.W.A. Smit (Jan); F. Roelfsema (Ferdinand); S.W.J. Lamberts (Steven); J.A. Romijn (Johannes)

    2005-01-01

    textabstractTo evaluate the long-term impact of cured Cushing's disease on subjective well-being, we assessed quality of life by validated health-related questionnaires in 58 patients cured from Cushing's disease by transsphenoidal surgery (n = 58), some of whom received additional radiotherapy (n =

  18. Quality of life in patients after long-term biochemical cure of Cushing's disease

    NARCIS (Netherlands)

    van Aken, M. O.; Pereira, A. M.; Biermasz, N. R.; van Thiel, S. W.; Hoftijzer, H. C.; Smit, J. W. A.; Roelfsema, F.; Lamberts, S. W. J.; Romijn, J. A.

    2005-01-01

    To evaluate the long-term impact of cured Cushing's disease on subjective well-being, we assessed quality of life by validated health-related questionnaires in 58 patients cured from Cushing's disease by transsphenoidal surgery (n = 58), some of whom received additional radiotherapy (n = 11) and/or

  19. Gastrointestinal Symptoms in Celiac Disease Patients on a Long-Term Gluten-Free Diet.

    Science.gov (United States)

    Laurikka, Pilvi; Salmi, Teea; Collin, Pekka; Huhtala, Heini; Mäki, Markku; Kaukinen, Katri; Kurppa, Kalle

    2016-07-14

    Experience suggests that many celiac patients suffer from persistent symptoms despite a long-term gluten-free diet (GFD). We investigated the prevalence and severity of these symptoms in patients with variable duration of GFD. Altogether, 856 patients were classified into untreated (n = 128), short-term GFD (1-2 years, n = 93) and long-term GFD (≥3 years, n = 635) groups. Analyses were made of clinical and histological data and dietary adherence. Symptoms were evaluated by the validated GSRS questionnaire. One-hundred-sixty healthy subjects comprised the control group. Further, the severity of symptoms was compared with that in peptic ulcer, reflux disease, inflammatory bowel disease (IBD) and irritable bowel syndrome (IBS). Altogether, 93% of the short-term and 94% of the long-term treated patients had a strict GFD and recovered mucosa. Untreated patients had more diarrhea, indigestion and abdominal pain than those on GFD and controls. There were no differences in symptoms between the short- and long-term GFD groups, but both yielded poorer GSRS total score than controls (p = 0.03 and p = 0.05, respectively). Furthermore, patients treated 1-2 years had more diarrhea (p = 0.03) and those treated >10 years more reflux (p = 0.04) than controls. Long-term treated celiac patients showed relatively mild symptoms compared with other gastrointestinal diseases. Based on our results, good response to GFD sustained in long-term follow-up, but not all patients reach the level of healthy individuals.

  20. Long-Term Follow-Up of Impulse Control Disorders in Parkinson’s Disease

    OpenAIRE

    Mamikonyan, Eugenia; Siderowf, Andrew D.; Duda, John E.; Potenza, Marc N.; Horn, Stacy; Stern, Matthew B.; Weintraub, Daniel

    2008-01-01

    Recent studies have linked dopamine agonist (DA) usage with the development of impulse control disorders (ICDs) in Parkinson’s disease (PD). Little is known about optimal management strategies or the long-term outcomes of affected patients. To report on the clinical interventions and long-term outcomes of PD patients who developed an ICD after DA initiation. Subjects contacted by telephone for a follow-up interview after a mean time period of 29.2 months. They were administered a modified Min...

  1. Bilateral high frequency subthalamic stimulation in Parkinson's disease: long-term neurological follow-up

    NARCIS (Netherlands)

    Romito, L. M.; Scerrati, M.; Contarino, M. F.; Iacoangeli, M.; Bentivoglio, A. R.; Albanese, A.

    2003-01-01

    AIM: High frequency stimulation of the subthalamic nucleus (STN) is gaining recognition as a new symptomatic treatment for Parkinson's disease (PD). The first available long-term observations show the stability of the efficacy of this procedure in time. METHODS: Quadripolar leads were implanted

  2. Long-term clinical outcomes in type 1 Gaucher disease following 10 years of imiglucerase treatment

    NARCIS (Netherlands)

    Weinreb, Neal J.; Goldblatt, Jack; Villalobos, Jacobo; Charrow, Joel; Cole, J. Alexander; Kerstenetzky, Marcelo; vom Dahl, Stephan; Hollak, Carla

    2013-01-01

    We studied the effect of long-term alglucerase/imiglucerase (Ceredase®/Cerezyme®, Genzyme, a Sanofi company, Cambridge, MA, USA) treatment on hematological, visceral, and bone manifestations of Gaucher disease type 1 (GD1). The International Collaborative Gaucher Group (ICGG) Gaucher Registry

  3. Short and long term radiation induced cardiovascular disease in patients with cancer

    DEFF Research Database (Denmark)

    Nielsen, Kirsten Melgaard; Offersen, Birgitte Vrou; Nielsen, Hanne Melgaard

    2017-01-01

    Radiation-induced cardiovascular disease is well described as a late effect in cancer patients treated with radiation therapy. Advancements in surgery, radiotherapy, and chemotherapy have led to an increasing number of cancer survivors with resultant long-term side effects related to their cancer...

  4. Nutritional status and long-term mortality in hospitalised patients with chronic obstructive pulmonary disease (COPD)

    DEFF Research Database (Denmark)

    Hallin, Runa; Gudmundsson, Gunnar; Suppli Ulrik, Charlotte

    2007-01-01

    Patients with chronic obstructive pulmonary disease (COPD) often have difficulties with keeping their weight. The aim of this investigation was to study nutritional status in hospitalised Nordic COPD patients and to investigate the association between nutritional status and long-term mortality in...

  5. Indoor air in long term care facilities and spread of infectious diseases

    NARCIS (Netherlands)

    te Kulve, M.; Loomans, M.G.L.C.; Huisman, E.; Kort, H.S.M.

    2014-01-01

    Not much is known about the favourable indoor air conditions in long term care facilities (ltcf’s), where older adults suffering from dementia live. Due to the decrease in cognition function, it is hard to evaluate comfort and health in this group. Nevertheless, infectious diseases are a persistent

  6. Serum YKL-40 predicts long-term mortality in patients with stable coronary disease

    DEFF Research Database (Denmark)

    Harutyunyan, Marina; Gøtze, Jens P; Winkel, Per

    2013-01-01

    We investigated whether the inflammatory biomarker YKL-40 could improve the long-term prediction of death made by common risk factors plus high-sensitivity C-reactive protein (hs-CRP) and N-terminal-pro-B natriuretic peptide (NT-proBNP) in patients with stable coronary artery disease (CAD)....

  7. Long-term pruritus as the initial and sole clinical manifestation of occult Hodgkin's disease.

    Science.gov (United States)

    Omidvari, Shapour H; Khojasteh, Habib Noorani; Mohammadianpanah, Mohammad; Monabati, Ahmad; Mosalaei, Ahmad; Ahmadloo, Niloofar

    2004-06-01

    Pruritus or itch is a frequent symptom of patients with Hodgkin's disease. It often occurs during the clinical course of the disease and rarely may precede the diagnosis of underlying disease. In this report, we present a 16-year-old patient who had history of generalized pruritus without any skin rash for 4 years before the diagnosis of Hodgkin's disease. Within that period, she had received symptom-oriented medications, with no significant effect. After the first cycle of chemotherapy, her pruritus resolved completely. This case suggests that long-term generalized pruritus may be indicative of a significant underlying problem like Hodgkin's disease.

  8. The mid-to-long term therapeutic efficacy of Graves' disease after interventional embolization

    International Nuclear Information System (INIS)

    Li Weiduo; Yang Jianyong; Zhuang Wenquan; Chen Wei; Li Heping

    2002-01-01

    Objective: To explore the mid-to-long term therapeutic efficacy of Graves' disease after interventional embolization. Methods: Twenty-five patients of Graves' disease treated with interventional embolization were followed up for 24-57 months. T 3 and T 4 were monitored at pre-operation, six months, 12 months, 2, 3 and 4 years after operation, respectively. Other references included pulse, thyroid size, and vessel's murmur. Results: Twenty-two patients completely relieved from the hyperthyroidism during the follow-up. Only one patient suffered from recurrence. Other two patients were still on maintaining dosage of antithyroid drug therapy. No hypothyroidism or hypoparathyroidism was found during this term. Conclusion: Mid-to-long term follow-up showed satisfactory efficacy of interventional therapy, offering another alternative for treatment of refractory Graves' disease

  9. [Predictors of long-term remission after transsphenoidal surgery in Cushing's disease].

    Science.gov (United States)

    Abellán Galiana, Pablo; Fajardo Montañana, Carmen; Riesgo Suárez, Pedro Antonio; Gómez Vela, José; Escrivá, Carlos Meseguer; Lillo, Vicente Rovira

    2013-10-01

    There is no consensus on the remission criteria for Cushing's disease or on the definition of disease recurrence after transsphenoidal surgery, and comparison of the different published series is therefore difficult. A long-term recurrence rate of Cushing's disease ranging from 2%-25% has been reported. Predictors of long-term remission reported include: 1) adenoma-related factors (aggressiveness, size, preoperative identification in MRI), 2) surgery-related factors, mainly neurosurgeon experience, 3) clinical factors, of which dependence on and duration of glucocorticoid treatment are most important, and 4) biochemical factors. Among the latter, low postoperative cortisol levels, less than 2 mcg/dL predict for disease remission. However, even when undetectable plasma cortisol levels are present, long-term recurrence may still occur and lifetime follow-up is required. We report the preliminary results of the first 20 patients with Cushing's disease operated on at our hospital using nadir cortisol levels less than 2 mcg/dl as remission criterion. Copyright © 2012 SEEN. Published by Elsevier Espana. All rights reserved.

  10. Regional cerebral blood flow in Alzheimer's disease. Comparison between short and long-term donepezil therapy

    International Nuclear Information System (INIS)

    Ushijima, Yo; Okuyama, Chio; Kubota, Takao; Nakai, Takako; Nishimura, Tsunehiko; Mori, Satoru

    2006-01-01

    Treatment with donepezil improves cognitive function of patients with Alzheimer's disease (AD) when compared to a placebo-controlled group. The purpose of this study was to investigate changes in regional cerebral blood flow (rCBF) of AD patients in short-term and long-term treatment with donepezil. rCBF was measured by N-isopropyl-p- 123 I-iodoamphetamine (IMP) autoradiography method. CBF measurements were performed in 17 AD patients before treatment and after 3 months (short-term therapy) and 1 year (long-term therapy). Regions of interest were set at cerebral cortex and cerebellar hemisphere. We used absolute CBF and relative CBF expressed as ratio to cerebellar CBF. Significant increases in relative rCBF were noted in the frontal, parietal and temporal lobes at the end of short-term therapy. rCBF was decreased after the long-term therapy, whereas rCBF was still increased to a slight extent, as compared with the pre-treatment levels. Absolute rCBF showed minimal change and a tendency to decline. Relative rCBF significantly increased in the short-term donepezil therapy, while following the long-term therapy, rCBF decreased to the pre-treatment level. (author)

  11. Bronchopulmonary Dysplasia: Chronic Lung Disease of Infancy and Long-Term Pulmonary Outcomes

    Directory of Open Access Journals (Sweden)

    Lauren M. Davidson

    2017-01-01

    Full Text Available Bronchopulmonary dysplasia (BPD is a chronic lung disease most commonly seen in premature infants who required mechanical ventilation and oxygen therapy for acute respiratory distress. While advances in neonatal care have resulted in improved survival rates of premature infants, limited progress has been made in reducing rates of BPD. Lack of progress may in part be attributed to the limited therapeutic options available for prevention and treatment of BPD. Several lung-protective strategies have been shown to reduce risks, including use of non-invasive support, as well as early extubation and volume ventilation when intubation is required. These approaches, along with optimal nutrition and medical therapy, decrease risk of BPD; however, impacts on long-term outcomes are poorly defined. Characterization of late outcomes remain a challenge as rapid advances in medical management result in current adult BPD survivors representing outdated neonatal care. While pulmonary disease improves with growth, long-term follow-up studies raise concerns for persistent pulmonary dysfunction; asthma-like symptoms and exercise intolerance in young adults after BPD. Abnormal ventilatory responses and pulmonary hypertension can further complicate disease. These pulmonary morbidities, combined with environmental and infectious exposures, may result in significant long-term pulmonary sequalae and represent a growing burden on health systems. Additional longitudinal studies are needed to determine outcomes beyond the second decade, and define risk factors and optimal treatment for late sequalae of disease.

  12. Control of neglected tropical diseases needs a long-term commitment

    Directory of Open Access Journals (Sweden)

    Mubila Likezo

    2010-10-01

    Full Text Available Abstract Background Neglected tropical diseases are widespread, particularly in sub-Saharan Africa, affecting over 2 billion individuals. Control of these diseases has gathered pace in recent years, with increased levels of funding from a number of governmental or non-governmental donors. Focus has currently been on five major 'tool-ready' neglected tropical diseases (lymphatic filariasis, onchocerciasis, schistosomiasis, soil-transmitted helminthiasis and trachoma, using a package of integrated drug delivery according to the World Health Organization guidelines for preventive chemotherapy. Discussion Success in controlling these neglected tropical diseases has been achieved in a number of countries in recent history. Experience from these successes suggests that long-term sustainable control of these diseases requires: (1 a long-term commitment from a wider range of donors and from governments of endemic countries; (2 close partnerships of donors, World Health Organization, pharmaceutical industries, governments of endemic countries, communities, and non-governmental developmental organisations; (3 concerted action from more donor countries to provide the necessary funds, and from the endemic countries to work together to prevent cross-border disease transmission; (4 comprehensive control measures for certain diseases; and (5 strengthened primary healthcare systems as platforms for the national control programmes and capacity building through implementation of the programmes. Conclusions The current level of funding for the control of neglected tropical diseases has never been seen before, but it is still not enough to scale up to the 2 billion people in all endemic countries. While more donors are sought, the stakeholders must work in a coordinated and harmonised way to identify the priority areas and the best delivery approaches to use the current funds to the maximum effect. Case management and other necessary control measures should be

  13. Nutritional status and long-term mortality in hospitalised patients with chronic obstructive pulmonary disease (COPD)

    DEFF Research Database (Denmark)

    Hallin, Runa; Gudmundsson, Gunnar; Suppli Ulrik, Charlotte

    2007-01-01

    Patients with chronic obstructive pulmonary disease (COPD) often have difficulties with keeping their weight. The aim of this investigation was to study nutritional status in hospitalised Nordic COPD patients and to investigate the association between nutritional status and long-term mortality in...... years. Further studies are needed in order to show whether identifying and treating weight loss and depletion of fat-free mass (FFM) is a way forward in improving the prognosis for hospitalised COPD patients. Udgivelsesdato: 2007-Sep...

  14. Long-term assessment of fatigue in patients with culture-confirmed Lyme disease.

    Science.gov (United States)

    Wormser, Gary P; Weitzner, Erica; McKenna, Donna; Nadelman, Robert B; Scavarda, Carol; Nowakowski, John

    2015-02-01

    Fatigue is a common symptom with numerous causes. Severe fatigue is thought to be an important manifestation of post-treatment Lyme disease syndrome. The frequency with which severe fatigue occurs as a long-term sequela in prospectively followed patients with Lyme disease is unknown. Patients with culture-confirmed Lyme disease who originally presented with erythema migrans have been evaluated annually in a prospective study to determine their long-term outcome. In 2011-2013, subjects were evaluated for fatigue using an 11-item Fatigue Severity Scale (FSS-11) that has been used in studies of post-treatment Lyme disease syndrome. An FSS-11 score of ≥4.0 is indicative of severe fatigue. A total of 100 subjects were assessed, 52% of whom were male; the mean age was 64.9 years (range, 42-86 years). The mean duration of follow-up was 15.4 years (range, 11-20 years). Nine subjects had severe fatigue but in none as a consequence of Lyme disease. Only 3 subjects were thought to possibly have persistent fatigue from Lyme disease. The FSS-11 value for these 3 individuals was less than 4, averaging 2.27, and none had functional impairment. Severe fatigue was found in 9 patients (9%) with culture-confirmed early Lyme disease at 11 to 20 years after presentation, but was due to causes other than Lyme disease. Fatigue of lesser severity was possibly due to Lyme disease, but was found in only 3% of 100 patients, and therefore is rarely a long-term complication of this infection. Copyright © 2015 Elsevier Inc. All rights reserved.

  15. Ischemic heart disease after mantlefield irradiation for Hodgkin's disease in long-term follow-up

    International Nuclear Information System (INIS)

    Reinders, J.G.; Heijmen, B.J.M.; Olofsen-van Acht, M.J.J.; Putten, W.L.J. van; Levendag, P.C.

    1999-01-01

    Background and purpose: In patients with Hodgkin's disease treated by radiotherapy with a moderate total dose and a low (mean) fraction dose to the heart, the risk of ischemic heart disease was investigated during long-term follow-up.Materials and methods: The medical records of 258 patients treated in the period 1965-1980 with radiotherapy alone as the primary treatment were reviewed. The median follow-up was 14.2 years (range 0.7-26.2). The mean total dose and fraction dose to the heart were 37.2 Gy (SD 2.9) and 1.64 Gy (SD 0.09), respectively. The impact on the development of ischemic heart disease of treatment-related parameters, such as the applied (fraction) dose, irradiation technique (one or two fields per day), and chemotherapy in case of a relapse, was investigated. The incidence of ischemic heart disease in this patient population was compared with the expected incidence based on gender, age and calendar period-specific data for the Dutch population.Results: Thirty-one patients (12%) experienced ischemic heart disease (actuarial risk at 20-25 years: 21.2% (95% C.I. 15-30). Twenty-five of them were hospitalized. When compared with the expected incidence, the relative risk (RR) of hospital admission for ischemic heart disease was 2.7 (95% C.I. 1.7-4.0). There were 12 deaths (4.7%) due to ischemic myocardial or sudden death (actuarial risk at 25 years: 10.2% (95% C.I. 5.3-19), compared to 2.3 cases that were expected to have died from these causes, yielding a standardized mortality ratio (SMR) of 5.3 (95% C.I. 2.7-9.3). Gender (male), pretreatment cardiac medical history and increasing age appeared to be the only significant factors for the development of ischemic heart disease.Conclusions: Despite the moderate total dose and the low (mean) fraction dose to the heart, the observed incidence of ischemic heart disease is high, especially after long follow-up periods. Treatment related cardiac disease in patients treated for Hodgkin's disease has only been

  16. Long-term cost-effectiveness of disease management in systolic heart failure.

    Science.gov (United States)

    Miller, George; Randolph, Stephen; Forkner, Emma; Smith, Brad; Galbreath, Autumn Dawn

    2009-01-01

    Although congestive heart failure (CHF) is a primary target for disease management programs, previous studies have generated mixed results regarding the effectiveness and cost savings of disease management when applied to CHF. We estimated the long-term impact of systolic heart failure disease management from the results of an 18-month clinical trial. We used data generated from the trial (starting population distributions, resource utilization, mortality rates, and transition probabilities) in a Markov model to project results of continuing the disease management program for the patients' lifetimes. Outputs included distribution of illness severity, mortality, resource consumption, and the cost of resources consumed. Both cost and effectiveness were discounted at a rate of 3% per year. Cost-effectiveness was computed as cost per quality-adjusted life year (QALY) gained. Model results were validated against trial data and indicated that, over their lifetimes, patients experienced a lifespan extension of 51 days. Combined discounted lifetime program and medical costs were $4850 higher in the disease management group than the control group, but the program had a favorable long-term discounted cost-effectiveness of $43,650/QALY. These results are robust to assumptions regarding mortality rates, the impact of aging on the cost of care, the discount rate, utility values, and the targeted population. Estimation of the clinical benefits and financial burden of disease management can be enhanced by model-based analyses to project costs and effectiveness. Our results suggest that disease management of heart failure patients can be cost-effective over the long term.

  17. Persistent and progressive long-term lung disease in survivors of preterm birth.

    Science.gov (United States)

    Urs, Rhea; Kotecha, Sailesh; Hall, Graham L; Simpson, Shannon J

    2018-04-13

    Preterm birth accounts for approximately 11% of births globally, with rates increasing across many countries. Concurrent advances in neonatal care have led to increased survival of infants of lower gestational age (GA). However, infants born poor respiratory outcomes throughout childhood, into adolescence and adulthood. Indeed, survivors of preterm birth have shown increased respiratory symptoms, altered lung structure, persistent and even declining lung function throughout childhood. The mechanisms behind this persistent and sometimes progressive lung disease are unclear, and the implications place those born preterm at increased risk of respiratory morbidity into adulthood. This review aims to summarise what is known about the long-term pulmonary outcomes of contemporary preterm birth, examine the possible mechanisms of long-term respiratory morbidity in those born preterm and discuss addressing the unknowns and potentials for targeted treatments. Copyright © 2018 Elsevier Ltd. All rights reserved.

  18. Lenalidomide in relapsed and refractory multiple myeloma disease: feasibility and benefits of long-term treatment.

    Science.gov (United States)

    Zago, Manola; Oehrlein, Katharina; Rendl, Corinna; Hahn-Ast, Corinna; Kanz, Lothar; Weisel, Katja

    2014-12-01

    Lenalidomide in combination with dexamethasone is an effective and well-established treatment of relapsed or refractory multiple myeloma (rrMM) disease. Due to the scarcity of reports assessing benefit and risk of long-term lenalidomide treatment in non-selected rrMM patients, we retrospectively analysed the long-term outcome in patients with rrMM treated with lenalidomide and dexamethasone. Sixty-seven patients (pts) who were treated with lenalidomide/dexamethasone for rrMM in the approved indication from 2007 to 2011 were included in this retrospective, single-centre analysis. Kaplan-Meier survival estimates were compared between total population, patients on lenalidomide for more than 12 months (mo) and patients discontinuing therapy earlier than 12 months. Median overall survival (OS) of the total patient population was 33.2 mo. OS of pts treated beyond 12 mo was 42.9 mo compared to 20.2 mo (p = 0.027) for pts stopping lenalidomide earlier than 12 mo for other reasons than progression disease (PD). OS of pts >12 mo on lenalidomide treatment did not significantly differ between pts who had received previous autologous transplantation, allogeneic transplantation or conventional therapy. Main non-hematologic toxicities were infections of grade 3/4 in 25 % and thrombembolic events of all grades in 18 % of patients. To the best of our knowledge, this is the first report on feasibility and efficacy of long-term lenalidomide treatment in a non-selected patient cohort. OS of pts >12 mo on lenalidomide is superior when compared to pts discontinued earlier for reasons other than PD. Our data confirm the current use of lenalidomide as a continuous long-term treatment strategy.

  19. Modelling Gaucher disease progression: long-term enzyme replacement therapy reduces the incidence of splenectomy and bone complications

    NARCIS (Netherlands)

    van Dussen, Laura; Biegstraaten, Marieke; Dijkgraaf, Marcel Gw; Hollak, Carla Em

    2014-01-01

    Long-term complications and associated conditions of type 1 Gaucher Disease (GD) can include splenectomy, bone complications, pulmonary hypertension, Parkinson disease and malignancies. Enzyme replacement therapy (ERT) reverses cytopenia and reduces organomegaly. To study the effects of ERT on

  20. Multidimensional Analyses of Long-Term Clinical Courses of Asthma and Chronic Obstructive Pulmonary Disease

    Directory of Open Access Journals (Sweden)

    Toru Oga

    Full Text Available ABSTRACT: Asthma and chronic obstructive pulmonary disease (COPD are chronic respiratory disorders involving obstructive airway defects. There have been many discussions on their similarities and differences. Although airflow limitation expressed as forced expiratory volume in one second (FEV1 has been considered to be the main diagnostic assessment in both diseases, it does not reflect the functional impairment imparted to the patients by these diseases. Therefore, multidimensional approaches using multiple measurements in assessing disease control or severity have been recommended, and multiple endpoints in addition to FEV1 have been set recently in clinical trials so as not to miss the overall effects. In particular, as improving symptoms and health status as well as pulmonary function are important goals in the management of asthma and COPD, some patient-reported measurements such as health-related quality of life or dyspnea should be included. Nonetheless, there have been few reviews on the long-term clinical course comparing asthma and COPD as predicted by measurements other than airflow limitation. Here, we therefore analyzed and compared longitudinal changes in both physiological measurements and patient-reported measurements in asthma and COPD. Although both diseases showed similar long-term progressive airflow limitation similarly despite guideline-based therapies, disease progression was different in asthma and COPD. In asthma, patient-reported assessments of health status, disability and psychological status remained clinically stable over time, in contrast to the significant deterioration of these parameters in COPD. Thus, because a single measurement of airflow limitation is insufficient to monitor these diseases, multidimensional analyses are important not only for disease control but also for understanding disease progression in asthma and COPD. KEY WORDS: asthma, COPD, longitudinal survey, multidimensional analysis, patient

  1. Mandibular third molar development after mantle radiation in long-term survivors of childhood Hodgkin's disease

    International Nuclear Information System (INIS)

    McGinnis, J.P. Jr.; Hopkins, K.P.; Thompson, E.I.; Hustu, H.O.

    1987-01-01

    Sequential panoramic radiographs were assessed for mandibular third molar development in 47 long-term survivors of childhood Hodgkin's disease after treatment with 37 Gy mantle field radiation. To make a comparison, panoramic radiographs of 149 healthy, nonirradiated children were reviewed for the presence of mandibular third molars. In children between the ages of 7 and 12 years, bilateral agenesis of mandibular third molars was more frequent in patients who had been treated with mantle radiation than in nonirradiated patients. Unilateral agenesis, crown hypoplasia, and root growth impairment of mandibular third molars were also found. Similar, apparent, radiation-induced developmental anomalies were noted in maxillary third molars of the irradiated patients

  2. In utero and early life arsenic exposure in relation to long-term health and disease

    Energy Technology Data Exchange (ETDEWEB)

    Farzan, Shohreh F.; Karagas, Margaret R. [Children' s Environmental Health and Disease Prevention Research Center at Dartmouth, Hanover, NH 03755 (United States); Section of Biostatistics and Epidemiology, Department of Community and Family Medicine and Norris Cotton Cancer Center, Geisel School of Medicine at Dartmouth, Lebanon, NH 03756 (United States); Chen, Yu, E-mail: yu.chen@nyumc.org [Department of Population Health, New York University School of Medicine, New York, NY 10016 (United States)

    2013-10-15

    Background: There is a growing body of evidence that prenatal and early childhood exposure to arsenic from drinking water can have serious long-term health implications. Objectives: Our goal was to understand the potential long-term health and disease risks associated with in utero and early life exposure to arsenic, as well as to examine parallels between findings from epidemiological studies with those from experimental animal models. Methods: We examined the current literature and identified relevant studies through PubMed by using combinations of the search terms “arsenic”, “in utero”, “transplacental”, “prenatal” and “fetal”. Discussion: Ecological studies have indicated associations between in utero and/or early life exposure to arsenic at high levels and increases in mortality from cancer, cardiovascular disease and respiratory disease. Additional data from epidemiologic studies suggest intermediate effects in early life that are related to risk of these and other outcomes in adulthood. Experimental animal studies largely support studies in humans, with strong evidence of transplacental carcinogenesis, atherosclerosis and respiratory disease, as well as insight into potential underlying mechanisms of arsenic's health effects. Conclusions: As millions worldwide are exposed to arsenic and evidence continues to support a role for in utero arsenic exposure in the development of a range of later life diseases, there is a need for more prospective studies examining arsenic's relation to early indicators of disease and at lower exposure levels. - Highlights: • We review in utero and early-life As exposure impacts on lifelong disease risks. • Evidence indicates that early-life As increases risks of lung disease, cancer and CVD. • Animal work largely parallels human studies and may lead to new research directions. • Prospective studies and individual exposure assessments with biomarkers are needed. • Assessing intermediary

  3. In utero and early life arsenic exposure in relation to long-term health and disease

    International Nuclear Information System (INIS)

    Farzan, Shohreh F.; Karagas, Margaret R.; Chen, Yu

    2013-01-01

    Background: There is a growing body of evidence that prenatal and early childhood exposure to arsenic from drinking water can have serious long-term health implications. Objectives: Our goal was to understand the potential long-term health and disease risks associated with in utero and early life exposure to arsenic, as well as to examine parallels between findings from epidemiological studies with those from experimental animal models. Methods: We examined the current literature and identified relevant studies through PubMed by using combinations of the search terms “arsenic”, “in utero”, “transplacental”, “prenatal” and “fetal”. Discussion: Ecological studies have indicated associations between in utero and/or early life exposure to arsenic at high levels and increases in mortality from cancer, cardiovascular disease and respiratory disease. Additional data from epidemiologic studies suggest intermediate effects in early life that are related to risk of these and other outcomes in adulthood. Experimental animal studies largely support studies in humans, with strong evidence of transplacental carcinogenesis, atherosclerosis and respiratory disease, as well as insight into potential underlying mechanisms of arsenic's health effects. Conclusions: As millions worldwide are exposed to arsenic and evidence continues to support a role for in utero arsenic exposure in the development of a range of later life diseases, there is a need for more prospective studies examining arsenic's relation to early indicators of disease and at lower exposure levels. - Highlights: • We review in utero and early-life As exposure impacts on lifelong disease risks. • Evidence indicates that early-life As increases risks of lung disease, cancer and CVD. • Animal work largely parallels human studies and may lead to new research directions. • Prospective studies and individual exposure assessments with biomarkers are needed. • Assessing intermediary endpoints may

  4. Long-term outcomes of internal carotid artery disease treated using radial artery graft

    International Nuclear Information System (INIS)

    Murai, Yasuo; Teramoto, Akira; Mizunari, Takayuki; Kobayashi, Shiro; Umeoka, Katsuya; Tateyama, Kojiro

    2009-01-01

    Complex internal carotid artery disease presents a surgical challenge because limitations and difficulty are encountered with either clipping or endovascular treatment. Our review of previous reports suggests that no current vascular assessment can accurately predict occurrence of ischemic complications after internal carotid artery ligation. The present study concerns long-term clinical outcome of radial artery grafting followed by parent artery trapping or proximal occlusion for management of these difficult lesions. Between September 1997 and October 2007, we performed radial artery grafting followed immediately by parent artery occlusion in 20 sides of 19 patients with complex internal carotid arteries disease with follow-up for more than 36 months (5 men, 14 women; mean follow-up duration, 62 months). All patients underwent postoperative MRI and MR angiography (MRA) every year to assess graft patency, ischemic complications, and de novo aneurysm. Another 20 carotid aneurysms with visual disturbance were assessed concerning outcome. Among 13 patients with cranial nerve (III and VI) disturbances, all dysfunctions were improved in cases treated within 8 months of onset to operation. On the other hand, patients with second cranial nerve disturbances were not improved in cases treated after 4 months of onset. No long-term complications were discovered with MRI and MRA. With appropriate attention to surgical technique, radial artery grafting followed by acute parent artery occlusion is a safe treatment for complex internal carotid artery aneurysms. Long-term safety is satisfactory, with no delayed complications such as graft stenosis, ischemic complications or de novo aneurysm formations in follow-up periods of more than 3 years. Good clinical outcome of cranial nerve palsy was achieved in patients treated within 8 months of onset for cranial nerve (CN) III and VI, and 4 of CN II palsy. (author)

  5. Cyclosporine therapy in inflammatory bowel disease: short-term and long-term results.

    Science.gov (United States)

    Gurudu, S R; Griffel, L H; Gialanella, R J; Das, K M

    1999-09-01

    Intravenous cyclosporine therapy followed by oral cyclosporine therapy reduce the need for urgent surgery in steroid-refractory inflammatory bowel disease (IBD). Our objective is to report short- and long-term results of cyclosporine therapy in IBD patients. Thirteen patients with steroid-refractory IBD, seven patients with ulcerative colitis (UC), and six patients with Crohn's disease (CD) were treated with intravenous cyclosporine (4 mg/kg/day) for a mean period of 11.4+/-2.8 days (range, 4-15 days). Subsequently the patients were started on oral cyclosporine (8 mg/kg/day) and followed for a mean of 10.3+/-10 months (range, 1-30 months). Twelve patients responded to intravenous cyclosporine therapy. One patient with UC developed sepsis on the fourth day of intravenous cyclosporine therapy and needed urgent colectomy. Nine of 12 initial responders (6 patients with UC and 3 patients with CD) relapsed during follow-up despite oral cyclosporine and underwent elective surgery. One patient with CD relapsed 3 months after discontinuation of oral cyclosporine. Only two patients with CD are in long-term remission. There were no long-term side effects in any of the 13 treated patients. In conclusion, intravenous cyclosporine was effective in inducing remission or significant improvement in 12 of 13 patients with steroid-refractory IBD. However, with subsequent oral cyclosporine the remission could be maintained only for a short while. Each of the six patients with UC needed colectomy and three of the five patients with CD had intestinal resection within 12 months despite oral cyclosporine therapy.

  6. Long-term effects of earthquake experience of young persons on cardiovascular disease risk factors

    Science.gov (United States)

    Li, Na; Wang, Yumei; Yu, Lulu; Song, Mei; Wang, Lan; Ji, Chunpeng

    2016-01-01

    Introduction The aim of the study was to study the long-term effect on cardiovascular disease risk factors of stress from direct experience of an earthquake as a young person. Material and methods We selected workers born between July 1, 1958 and July 1, 1976 who were examined at Kailuan General Hospital between May and October of 2013. Data on cardiovascular events were taken during the workers’ annual health examination conducted between 2006 and 2007. All subjects were divided into three groups according to their experience of the Tangshan earthquake of July 28, 1976, as follows: control group; exposed group 1 and exposed group 2. We compared cardiovascular disease risk factors between the three groups as well as by gender and age. Results One thousand one hundred and ninety-six workers were included in the final statistical analysis. Among all subjects, resting heart rate (p = 0.003), total cholesterol (p earthquake compared with unexposed controls, but were unrelated to loss of relatives. No significant difference in triglyceride levels was observed between the three groups (p = 0.900). Further refinement showed that the effects were restricted to males 40 years of age or older at the time of analysis, but were due primarily to age at the time of earthquake exposure (p = 0.002, p Earthquake experience in the early years of life has long-term effects on adult resting heart rate, total cholesterol, and fasting plasma glucose, especially among men. PMID:28144258

  7. Long-term outcome of anti-glomerular basement membrane antibody disease treated with immunoadsorption.

    Science.gov (United States)

    Biesenbach, Peter; Kain, Renate; Derfler, Kurt; Perkmann, Thomas; Soleiman, Afschin; Benharkou, Alexandra; Druml, Wilfred; Rees, Andrew; Säemann, Marcus D

    2014-01-01

    Anti-glomerular basement membrane (GBM) antibody disease may lead to acute crescentic glomerulonephritis with poor renal prognosis. Current therapy favours plasma exchange (PE) for removal of pathogenic antibodies. Immunoadsorption (IAS) is superior to PE regarding efficiency of antibody-removal and safety. Apart from anecdotal data, there is no systemic analysis of the long-term effects of IAS on anti-GBM-disease and antibody kinetics. To examine the long-term effect of high-frequency IAS combined with standard immunosuppression on patient and renal survival in patients with anti-GBM-disease and to quantify antibody removal and kinetics through IAS. Retrospective review of patients treated with IAS for anti-GBM-antibody disease confirmed by biopsy and/or anti-GBM-antibodies. University Hospital of Vienna, Austria. 10 patients with anti-GBM-disease treated with IAS. Patient and renal survival, renal histology, anti-GBM-antibodies. Anti-GBM-antibodies were reduced by the first 9 IAS treatments (mean number of 23) to negative levels in all patients. Renal survival was 40% at diagnosis, 70% after the end of IAS, 63% after one year and 50% at the end of observation (mean 84 months, range 9 to 186). Dialysis dependency was successfully reversed in three of six patients. Patient survival was 90% at the end of observation. IAS efficiently eliminates anti-GBM-antibodies suggesting non-inferiority to PE with regard to renal and patient survival. Hence IAS should be considered as a valuable treatment option for anti-GBM-disease, especially in patients presenting with a high percentage of crescents and dialysis dependency due to an unusual high proportion of responders.

  8. Long-term outcome of anti-glomerular basement membrane antibody disease treated with immunoadsorption.

    Directory of Open Access Journals (Sweden)

    Peter Biesenbach

    Full Text Available Anti-glomerular basement membrane (GBM antibody disease may lead to acute crescentic glomerulonephritis with poor renal prognosis. Current therapy favours plasma exchange (PE for removal of pathogenic antibodies. Immunoadsorption (IAS is superior to PE regarding efficiency of antibody-removal and safety. Apart from anecdotal data, there is no systemic analysis of the long-term effects of IAS on anti-GBM-disease and antibody kinetics.To examine the long-term effect of high-frequency IAS combined with standard immunosuppression on patient and renal survival in patients with anti-GBM-disease and to quantify antibody removal and kinetics through IAS.Retrospective review of patients treated with IAS for anti-GBM-antibody disease confirmed by biopsy and/or anti-GBM-antibodies.University Hospital of Vienna, Austria.10 patients with anti-GBM-disease treated with IAS.Patient and renal survival, renal histology, anti-GBM-antibodies.Anti-GBM-antibodies were reduced by the first 9 IAS treatments (mean number of 23 to negative levels in all patients. Renal survival was 40% at diagnosis, 70% after the end of IAS, 63% after one year and 50% at the end of observation (mean 84 months, range 9 to 186. Dialysis dependency was successfully reversed in three of six patients. Patient survival was 90% at the end of observation.IAS efficiently eliminates anti-GBM-antibodies suggesting non-inferiority to PE with regard to renal and patient survival. Hence IAS should be considered as a valuable treatment option for anti-GBM-disease, especially in patients presenting with a high percentage of crescents and dialysis dependency due to an unusual high proportion of responders.

  9. Long-term, regular remote ischemic preconditioning improves endothelial function in patients with coronary heart disease

    Energy Technology Data Exchange (ETDEWEB)

    Liang, Y.; Li, Y.P.; He, F.; Liu, X.Q.; Zhang, J.Y. [Department of Cardiology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou (China)

    2015-04-28

    Remote ischemic preconditioning (RIPre) can prevent myocardial injury. The purpose of this study was to assess the beneficial effects of long-term regular RIPre on human arteries. Forty patients scheduled for coronary artery bypass graft (CABG) surgery were assigned randomly to a RIPre group (n=20) or coronary heart disease (CHD) group (n=20). Twenty patients scheduled for mastectomy were enrolled as a control group. RIPre was achieved by occluding arterial blood flow 5 min with a mercury sphygmomanometer followed by a 5-min reperfusion period, and this was repeated 4 times. The RIPre procedure was repeated 3 times a day for 20 days. In all patients, arterial fragments discarded during surgery were collected to evaluate endothelial function by flow-mediated dilation (FMD), CD34{sup +} monocyte count, and endothelial nitric oxide synthase (eNOS expression). Phosphorylation levels of STAT-3 and Akt were also assayed to explore the underlying mechanisms. Compared with the CHD group, long-term regular RIPre significantly improved FMD after 20 days (8.5±2.4 vs 4.9±4.2%, P<0.05) and significantly reduced troponin after CABG surgery (0.72±0.31 and 1.64±0.19, P<0.05). RIPre activated STAT-3 and increased CD34{sup +} endothelial progenitor cell counts found in arteries. Long-term, regular RIPre improved endothelial function in patients with CHD, possibly due to STAT-3 activation, and this may have led to an increase in endothelial progenitor cells.

  10. Long-term health-related quality of life for disease-free esophageal cancer patients.

    LENUS (Irish Health Repository)

    Donohoe, Claire L

    2012-02-01

    BACKGROUND: Health-related quality of life (HRQL) has been studied extensively during the first year following esophagectomy, but little is known about HRQL in long-term survivors. The aim of this study was to investigate HRQL in patients alive at least 1 year after surgical resection for esophageal cancer using validated European Organisation for Research and Treatment of Cancer (EORTC) quality of life (QOL) questionnaires (QLQ). METHODS: Eligible patients, without known disease recurrence and at least 1 year after esophagectomy, were identified from a prospectively maintained database. Patients completed general (QLQ-C30) and esophageal cancer-specific (QLQ-OES18, OG25) questionnaires. A numeric score (0-100) was computed in each conceptual area and compared with validated cancer (n = 1031) and age-matched (n = 7802) healthy populations using two-tailed unpaired t-tests. A cohort of 80 patients had pretreatment scores recorded. RESULTS: Altogether, 132 of 156 eligible patients (84%) completed the self-rated questionnaire, 105 (67.3%) were men, and the mean age was 62 years (range 29-84 years). The mean time since esophagectomy was 70.3 months (12-299 months). Global health status was significantly reduced at least 1 year after esophagectomy (mean +\\/- SD score 48.4 +\\/- 18.6) when compared with patients with esophageal cancer prior to treatment (55.6 +\\/- 24.1) and the general population (71.2 +\\/- 22.4) (p < 0.0001). In a prospective cohort of eighty patients, symptoms related to swallowing difficulty, reflux, pain, and coughing significantly decreased in the long term (p < 0.0001). The degree of subjective swallowing dysfunction was highly correlated with a poor QOL (Spearman\\'s rho = 0.508, p < 0.01). CONCLUSIONS: Global health status remains significantly reduced in long-term survivors after esophagectomy compared with population controls, and swallowing dysfunction is highly associated with this compromised QOL.

  11. Long-term follow-up on Cushing disease patient after transsphenoidal surgery

    Directory of Open Access Journals (Sweden)

    Insook Jeong

    2014-09-01

    Full Text Available Cushing disease is caused by excessive adrenocorticotropic hormone (ACTH production by the pituitary adenoma. Transsphenoidal surgery is its first-line treatment. The incidence of Cushing disease in children and adolescents is so rare that long-term prognoses have yet to be made in most cases. We followed-up on a 16-year-old male Cushing disease patient who presented with rapid weight gain and growth retardation. The laboratory findings showed increased 24-hour urine free cortisol and lack of overnight cortisol suppression by low-dose dexamethasone test. The serum cortisol and 24-hour urine free cortisol, by high-dose dexamethasone test, also showed a lack of suppression, and a bilateral inferior petrosal sinus sampling suggested lateralization of ACTH secretion from the right-side pituitary gland. However, after a right hemihypophysectomy by the transsphenoidal approach, the 24-hour urine free cortisol levels were persistently high. Thus the patient underwent a total hypophysectomy, since which time he has been treated with hydrocortisone, levothyroxine, recombinant human growth hormone, and testosterone enanthate. Intravenous bisphosphonate for osteoporosis had been administered for three years. At his current age of 26 years, his final height had attained the target level range; his bone mineral density was normal, and his pubic hair was Tanner stage 4. This report describes the long-term treatment course of a Cushing disease patient according to growth profile, pubertal status, and responses to hormone replacement therapy. The clinical results serve to emphasize the importance of growth optimization, puberty, and bone health in the treatment management of Cushing disease patients who have undergone transsphenoidal surgery.

  12. Long-term follow-up on Cushing disease patient after transsphenoidal surgery.

    Science.gov (United States)

    Jeong, Insook; Oh, Moonyeon; Kim, Ja Hye; Cho, Ja Hyang; Choi, Jin-Ho; Yoo, Han-Wook

    2014-09-01

    Cushing disease is caused by excessive adrenocorticotropic hormone (ACTH) production by the pituitary adenoma. Transsphenoidal surgery is its first-line treatment. The incidence of Cushing disease in children and adolescents is so rare that long-term prognoses have yet to be made in most cases. We followed-up on a 16-year-old male Cushing disease patient who presented with rapid weight gain and growth retardation. The laboratory findings showed increased 24-hour urine free cortisol and lack of overnight cortisol suppression by low-dose dexamethasone test. The serum cortisol and 24-hour urine free cortisol, by high-dose dexamethasone test, also showed a lack of suppression, and a bilateral inferior petrosal sinus sampling suggested lateralization of ACTH secretion from the right-side pituitary gland. However, after a right hemihypophysectomy by the transsphenoidal approach, the 24-hour urine free cortisol levels were persistently high. Thus the patient underwent a total hypophysectomy, since which time he has been treated with hydrocortisone, levothyroxine, recombinant human growth hormone, and testosterone enanthate. Intravenous bisphosphonate for osteoporosis had been administered for three years. At his current age of 26 years, his final height had attained the target level range; his bone mineral density was normal, and his pubic hair was Tanner stage 4. This report describes the long-term treatment course of a Cushing disease patient according to growth profile, pubertal status, and responses to hormone replacement therapy. The clinical results serve to emphasize the importance of growth optimization, puberty, and bone health in the treatment management of Cushing disease patients who have undergone transsphenoidal surgery.

  13. Pituitary function following megavoltage therapy for Cushings' disease; long term follow up

    International Nuclear Information System (INIS)

    Sharpe, G.F.; Kendall-Taylor, P.; Prescott, R.W.G.; Ross, W.M.; Davison, C.; Watson, M.J.; Cook, D.B.

    1985-01-01

    Eight patients who had received megavoltage therapy for Cushings' disease 5-12 years previously have been reviewed. The long term response to this therapy was assessed with respect to efficacy of treatment in inducing continued remission and disturbance of hypothalamic-pituitary function. One patient showed clear evidence of relapse of Cushings' disease. One patient had unequivocal hypopituitarism. Basal levels of growth hormone (GH), TSH, LH, and FSH were not statistically different from controls, but provocative testing revealed significant abnormalities of response of cortisol/ACTH, GH, prolactin and LH. Six out of eight patients had absent diurnal cortisol variation and five patients had elevated serum prolactin levels. Thus, in this group of patients normal pituitary-adrenal function has not been satisfactorily restored. It is clear that significant disturbances of hypothalamic-pituitary function follow megavoltage therapy and these may progress to overt hypopituitarism. (author)

  14. Subdiaphragmatic stage I and II Hodgkin's disease - long-term follow-up and prognostic factors

    International Nuclear Information System (INIS)

    Liao Zhongxing; Ha, Chul S.; Fuller, Lillian M.; Hagemeister, Fredrick B.; Cabanillas, Fernando; Tucker, Susan L.; Hess, Mark A.; Cox, James D.

    1997-01-01

    Purpose: To report long term follow-up results and analyze prognostic factors for overall and disease-free survival in patients with subdiaphragmatic stage I and II Hodgkin's disease. Methods and Materials: From September 1962 to April 1995, 109 patients presented to the M. D. Anderson Cancer Center with subdiaphragmatic Hodgkin's disease. The medical records of these patients were retrospectively reviewed. Twenty-two patients who received no treatment at M. D. Anderson Cancer Center or who had radiation therapy at other institutions were excluded. The remaining 87 patients formed the basis of this study. The median age of our group was 33 years with a male:female ratio of 3.3:1. The histological subtypes were nodular sclerosis in 21 (24.1%) patients, mixed cellularity in 31 (35.6%), lymphocyte predominence in 33 (37.9%), lymphocyte depletion in 1 (1.1%) and unclassified histology in 1 (1.1%). Thirty three (37%) patients underwent laparotomy, 74 (85.1%) had lymphangiography, and 35 (40.2%) had computerized tomography of the abdomen. Twenty two (25%) patients had more than three sites of nodal involvement at presentation, 56 (64.4%) had pelvic or abdominal disease, and 14 (18.4%) had bulky disease which was defined as disease with largest dimension ≥ 7 cm. Stage distribution was IA in 33.3%, IIA in 39.1%, and IIB in 27.6%. Sixty (69%) patients were treated with radiotherapy alone, 23 (26.4%) with chemotherapy and radiation, and 4 (4.6%) with chemotherapy alone. Results: The 10 and 20 year actuarial overall survival rates for all the patients were 74.6% and 55.3%, and the corresponding disease free survival rates were 72.4% and 67.5%, respectively. On univariate analysis, age, B symptoms, nodular sclerosis or mixed cellularity histology, and decreased albumin and hemoglobin level were statistically significant adverse pretreatment factors for overall survival. B symptoms, decreased albumin level, more than 3 sites of disease at presentation, and stage were

  15. Preeclampsia and long-term risk of cardiovascular disease: what do obstetrician-gynecologists know?

    Science.gov (United States)

    2013-01-01

    Background Preeclampsia (PE), a hypertensive disorder of pregnancy affects 2-8% of women and is associated with increased cardiovascular disease (CVD) risk later in life. There is little information about the knowledge of obstetrician-gynecologists in German outpatient care setting regarding the future health risk of PE and knowledge of the current guidelines on treatment and counseling patients post PE. This study aimed to assess whether obstetrician-gynecologists are aware of PE’s association with maternal long-term adverse outcomes and providing appropriate counseling. Methods A random sample of 500 obstetrician-gynecologists in the federal state of Lower Saxony was mailed a survey and a reminder with a second copy of the survey. The questionnaire elicited both personal information, and knowledge on future disease risks, e.g. cardiovascular disease (CVD) and current guidelines as well as on counseling practice. Descriptive analysis was used to analyze the responses. Results A total of 212 obstetrician-gynecologists (42.4%) responded to the questionnaire. A large proportion of physicians stated that PE was associated with a higher risk for the development for hypertension (86.6%), stroke (78.5%) and kidney disease (78.0%). Of the participants 75.8% reported that women after PE have a shorter life expectancy. Respondents with knowledge of the current guidelines of the German Association of Obstetrics and Gynecology concerning follow up and risk management of PE (45.2%) were more often aware of the development of CVD and stroke and counseled patients on self -blood-pressure measurement, meaning and long-term-risks of PE and attached importance to family history of PE compared to physicians with no knowledge of the guidelines. Conclusion Although the majority of obstetrician-gynecologists were aware of higher CVD risk after PE, weaknesses exist in the follow up care and counseling of these patients. These deficiencies would be amendable to directed educational

  16. Long-Term Health of Dopaminergic Neuron Transplants in Parkinson's Disease Patients

    Directory of Open Access Journals (Sweden)

    Penelope J. Hallett

    2014-06-01

    Full Text Available To determine the long-term health and function of transplanted dopamine neurons in Parkinson’s disease (PD patients, the expression of dopamine transporters (DATs and mitochondrial morphology were examined in human fetal midbrain cellular transplants. DAT was robustly expressed in transplanted dopamine neuron terminals in the reinnervated host putamen and caudate for at least 14 years after transplantation. The transplanted dopamine neurons showed a healthy and nonatrophied morphology at all time points. Labeling of the mitochondrial outer membrane protein Tom20 and α-synuclein showed a typical cellular pathology in the patients’ own substantia nigra, which was not observed in transplanted dopamine neurons. These results show that the vast majority of transplanted neurons remain healthy for the long term in PD patients, consistent with clinical findings that fetal dopamine neuron transplants maintain function for up to 15–18 years in patients. These findings are critically important for the rational development of stem-cell-based dopamine neuronal replacement therapies for PD.

  17. Physician personal characteristics influencing long-term treatment of patients with cardiovascular diseases

    Directory of Open Access Journals (Sweden)

    Strokova E.V.

    2013-06-01

    Full Text Available The main purpose of the article is to identify the peculiarities of a doctor personality, affecting long-term therapy in patients with cardiovascular diseases. Materials and methods: To determine the type of temperament, the presence and intensity of the syndrome of emotional burnout and capacity for empathy therapists and cardiologists were asked to fill in a number of questionnaires. Each doctor had a group of patients contacting by telephone for a year after the discharge from the hospital. During the telephone contact, the patients were asked about the continuation of their therapy recommended in the hospital, the regularity of therapy, the frequency of absence, and the assessment of a physician by the patients. Results: 35 questionnaires were suitable for interpretation. Through one year after the discharge from the hospital it was able to contact with 147 patients, 18.4% (27 of patients completely stopped the treatment by recommended drugs. Positive assessment of physicians was associated with the continuation of the therapy by recommended drugs and regularity of drug taking (p=0,03. Patients assessed physicians positively more often in cases of low level of emotional state, high level of depersonalization (cynicism and the reduction of personal accomplishment (feeling of professional inefficiency in a doctor. Conclusion: Assessment of physicians by patients is reliably and significantly influenced by continuation of long-term therapy and regularity of drug taking.

  18. Long-term safety of rituximab induced peripheral B-cell depletion in autoimmune neurological diseases.

    Directory of Open Access Journals (Sweden)

    Anza B Memon

    Full Text Available B-cells play a pivotal role in several autoimmune diseases, including patients with immune-mediated neurological disorders (PIMND, such as neuromyelitis optica (NMO, multiple sclerosis (MS, and myasthenia gravis (MG. Targeting B-cells has been an effective approach in ameliorating both central and peripheral autoimmune diseases. However, there is a paucity of literature on the safety of continuous B-cell depletion over a long period of time.The aim of this study was to examine the long-term safety, incidence of infections, and malignancies in subjects receiving continuous therapy with a B-cell depleting agent rituximab over at least 3 years or longer.This was a retrospective study involving PIMND who received continuous cycles of rituximab infusions every 6 to 9 months for up to 7 years. The incidence of infection related adverse events (AE, serious adverse events (SAE, and malignancies were observed.There were a total of 32 AE and 4 SAE with rituximab treatment. The 3 SAE were noted after 9 cycles (48 months and 1 SAE was observed after 11 cycles (60 months of rituximab. There were no cases of Progressive multifocal leukoencephalopathy (PML and malignancies observed throughout the treatment period. Rituximab was well tolerated without any serious infusion reactions. Also, rituximab was found to be beneficial in treating PIMND over a 7-year period.This study demonstrates that long-term depletion of peripheral B-cells appears safe and efficacious in treating PIMND. Longer and larger prospective studies with rituximab are needed to carefully ascertain risks associated with chronic B-cell depletion, including malignancies. Recognizing that this is a small, retrospective study, such data nonetheless complement the growing literature documenting the safety and tolerability of B-cell depleting agents in neurological diseases.

  19. Chronic obstructive pulmonary disease and long-term exposure to traffic-related air pollution: a cohort study

    DEFF Research Database (Denmark)

    Andersen, Zorana J; Hvidberg, Martin; Jensen, Steen S

    2011-01-01

    Short-term exposure to air pollution has been associated with exacerbation of chronic obstructive pulmonary disease (COPD), whereas the role of long-term exposures on the development of COPD is not yet fully understood.......Short-term exposure to air pollution has been associated with exacerbation of chronic obstructive pulmonary disease (COPD), whereas the role of long-term exposures on the development of COPD is not yet fully understood....

  20. Estimation of somatic development and mental state of children with neoplasmatic disease after long-term chemotherapy

    International Nuclear Information System (INIS)

    Korzon, M.; Kaminska, B.; Bohdan, Z.; Liberek, A.; Rokosz, K.

    1993-01-01

    33 children with neoplasmatic disease after long-term complex treatment were subjected to a single estimation of somatic development and to psychological examinations. From examinations it appears that long-term chemo- and radiotherapy or surgical treatment in these patients had no negative influence on their physical development. Psychological analysis had shown mind disturbances in children with is connected with this special kind of the disease and stress that children and their parents had undergone. (author)

  1. The Need for Total Hip Arthroplasty in Perthes Disease: A Long-term Study

    DEFF Research Database (Denmark)

    Froberg, Lonnie; Christensen, Finn; Pedersen, Niels Wisbech

    2010-01-01

    BACKGROUND: Legg-Calvé-Perthes disease (LCPD) was described a century ago. In previous long-term reports of patients with LCPD, nonoperative treatment varied considerably. The likelihood of hip osteoarthritis (OA) developing in patients with LCPD and possible need for THA are not well defined...... with hips with Classes I/II femoral heads. PATIENTS AND METHODS: The study population consisted of 167 patients with LCPD treated with a Thomas splint. The control population consisted of gender- and age-matched control subjects who were participants in the Copenhagen City Heart Study: the Osteoarthritis...... OF EVIDENCE: Level III, prognostic study. See Guidelines for Authors for a complete description of levels of evidence....

  2. Long-term follow-up of low-dose external pituitary irradiation for Cushing's disease

    International Nuclear Information System (INIS)

    Littley, M.D.; Shalet, S.M.; Beardwell, C.G.; Ahmed, S.R.; Sutton, M.L.

    1990-01-01

    Twenty-four patients (three male) with Cushing's disease, aged between 11 and 67 years, were treated with low-dose external pituitary irradiation (20 Gy in eight fractions over 10-12 days) and followed for between 13 and 171 months (median 93 months). Eleven patients (46%) went into remission 4-36 months after irradiation, but five subsequently relapsed. In this series, the low incidence of radiation-induced hypopituitarism and absence of other complications attributable to radiotherapy suggest that low-dose pituitary irradiation may be a useful treatment option in selected patients. However, long-term follow-up has demonstrated a high relapse rate and failure to prevent Nelson's syndrome in adrenalectomized patients, indicating that it should not be used as primary treatment in preference to selective adenomectomy. (author)

  3. Using music therapy to help a client with Alzheimer's disease adapt to long-term care.

    Science.gov (United States)

    Kydd, P

    2001-01-01

    The purpose of this case study is to illustrate how music therapy can be used to help the elderly successfully adjust to living in a long-term care (LTC) facility. LTC residents, particularly those with Alzheimer's disease or related dementia, may exhibit behaviors such as depression, withdrawal, anxiety, emotional liability, confusion, and memory difficulties, frequently related to the disorder, but often exacerbated by difficulty in adjustment to the change in lifestyle. The subject of this case study demonstrated these symptoms. Music therapy helped him adjust to life in a LTC setting by improving his quality of life and enhancing his relationships with those around him. As chronicled in this study, music therapy may facilitate a resident's adjustment to life in a LTC facility. N.B. Names and identifying information have been changed to protect privacy.

  4. Long-term neurocognitive outcomes of patients with end-stage renal disease during infancy.

    Science.gov (United States)

    Johnson, Rebecca J; Warady, Bradley A

    2013-08-01

    End-stage renal disease (ESRD) during infancy has been associated with poor short-term neurocognitive outcomes. Limited information exists regarding long-term outcomes. Neurocognitive outcomes for 12 patients diagnosed with ESRD during the first 16 months of life were assessed. Nine patients (mean age: 11 years) were compared to their healthy siblings (mean age: 10 years) on measures of intellectual and executive functioning, memory, and academic achievement using paired-samples t tests. Patients' Full Scale IQ (FSIQ) scores (M = 78, SD = 16.1) were significantly lower than sibling controls (M = 94, SD = 18.9; p executive functioning, memory, and academic achievement. In summary, patients diagnosed with ESRD as infants had intellectual and metacognitive functioning significantly lower than sibling controls. Fewer months on dialysis and younger age at transplant were associated with better outcomes.

  5. Successful long-term treatment of Cushing disease with mifepristone (RU486).

    Science.gov (United States)

    Basina, Marina; Liu, Hau; Hoffman, Andrew R; Feldman, David

    2012-01-01

    We describe a girl with Cushing disease for whom surgery and radiation treatments failed and the subsequent clinical course with mifepristone therapy. We present the patient's clinical, biochemical, and imaging findings. A 16-year-old girl presented with classic Cushing disease. After transsphenoidal surgery, Cyberknife radiosurgery, ketoconazole, and metyrapone did not control her disease, and she was prescribed mifepristone, which was titrated to a maximal dosage of 1200 mg daily with subsequent symptom improvement. Mifepristone (RU486) is a high-affinity, nonselective antagonist of the glucocorticoid receptor. There is limited literature on its use as an off-label medication to treat refractory Cushing disease. Over her 8-year treatment with mifepristone, her therapy was complicated by hypertension and hypokalemia requiring spironolactone and potassium chloride. She received a 2-month drug holiday every 4 to 6 months to allow for withdrawal menstrual bleeding with medroxyprogesterone acetate. Urinary cortisol, serum cortisol, and corticotropin levels remained elevated during mifepristone drug holidays. While on mifepristone, her signs and symptoms of Cushing disease resolved. Repeated magnetic resonance imaging demonstrated stable appearance of the residual pituitary mass. Bilateral adrenalectomy was performed, and mifepristone was discontinued after 95 months of medical therapy. We describe the longest duration of mifepristone therapy thus reported for the treatment of refractory Cushing disease. Mifepristone effectively controlled all signs and symptoms of hypercortisolism. Menstruating women who take the drug on a long-term basis should receive periodic drug holidays to allow for menses. The lack of reliable serum biomarkers to monitor the success of mifepristone therapy requires careful clinical judgment and may make its use difficult in Cushing disease.

  6. Long-term odor recognition memory in unipolar major depression and Alzheimer׳s disease.

    Science.gov (United States)

    Naudin, Marine; Mondon, Karl; El-Hage, Wissam; Desmidt, Thomas; Jaafari, Nematollah; Belzung, Catherine; Gaillard, Philippe; Hommet, Caroline; Atanasova, Boriana

    2014-12-30

    Major depression and Alzheimer׳s disease (AD) are often observed in the elderly. The identification of specific markers for these diseases could improve their screening. The aim of this study was to investigate long-term odor recognition memory in depressed and AD patients, with a view to identifying olfactory markers of these diseases. We included 20 patients with unipolar major depressive episodes (MDE), 20 patients with mild to moderate AD and 24 healthy subjects. We investigated the cognitive profile and olfactory memory capacities (ability to recognize familiar and unfamiliar odors) of these subjects. Olfactory memory test results showed that AD and depressed patients were characterized by significantly less correct responses and more wrong responses than healthy controls. Detection index did not differ significantly between patients with major depression and those with AD when the results were analyzed for all odors. However, MDE patients displayed an impairment of olfactory memory for both familiar and unfamiliar odors, whereas AD subjects were impaired only in the recognition of unfamiliar odors, with respect to healthy subjects. If preservation of olfactory memory for familiar stimuli in patients with mild to moderate AD is confirmed, this test could be used in clinical practice as a complementary tool for diagnosis. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  7. Long-Term Adult Feline Liver Organoid Cultures for Disease Modeling of Hepatic Steatosis

    Directory of Open Access Journals (Sweden)

    Hedwig S. Kruitwagen

    2017-04-01

    Full Text Available Summary: Hepatic steatosis is a highly prevalent liver disease, yet research is hampered by the lack of tractable cellular and animal models. Steatosis also occurs in cats, where it can cause severe hepatic failure. Previous studies demonstrate the potential of liver organoids for modeling genetic diseases. To examine the possibility of using organoids to model steatosis, we established a long-term feline liver organoid culture with adult liver stem cell characteristics and differentiation potential toward hepatocyte-like cells. Next, organoids from mouse, human, dog, and cat liver were provided with fatty acids. Lipid accumulation was observed in all organoids and interestingly, feline liver organoids accumulated more lipid droplets than human organoids. Finally, we demonstrate effects of interference with β-oxidation on lipid accumulation in feline liver organoids. In conclusion, feline liver organoids can be successfully cultured and display a predisposition for lipid accumulation, making them an interesting model in hepatic steatosis research. : In this study Kruitwagen and colleagues establish and characterize a feline liver organoid culture, which has adult stem cell properties and can be differentiated toward hepatocyte-like cells. They propose liver organoids as a tool to model hepatic steatosis and show that feline liver organoids accumulate more lipids than human organoids when provided with excess fatty acids. Keywords: feline liver organoids, adult liver stem cells, hepatic steatosis, disease modeling, feline hepatic lipidosis, species differences

  8. Long-Term Cognitive Improvement After Benfotiamine Administration in Patients with Alzheimer's Disease.

    Science.gov (United States)

    Pan, Xiaoli; Chen, Zhichun; Fei, Guoqiang; Pan, Shumei; Bao, Weiqi; Ren, Shuhua; Guan, Yihui; Zhong, Chunjiu

    2016-12-01

    To date, we still lack disease-modifying therapies for Alzheimer's disease (AD). Here, we report that long-term administration of benfotiamine improved the cognitive ability of patients with AD. Five patients with mild to moderate AD received oral benfotiamine (300 mg daily) over 18 months. All patients were examined by positron emission tomography with Pittsburgh compound B (PiB-PET) and exhibited positive imaging with β-amyloid deposition, and three received PiB-PET imaging at follow-up. The five patients exhibited cognitive improvement as assayed by the Mini-Mental Status Examination (MMSE) with an average increase of 3.2 points at month 18 of benfotiamine administration. The three patients who received follow-up PiB-PET had a 36.7% increase in the average standardized uptake value ratio in the brain compared with that in the first scan. Importantly, the MMSE scores of these three had an average increase of 3 points during the same period. Benfotiamine significantly improved the cognitive abilities of mild to moderate AD patients independently of brain amyloid accumulation. Our study provides new insight to the development of disease-modifying therapy.

  9. Analysis of the immune system of multiple myeloma patients achieving long-term disease control by multidimensional flow cytometry

    Science.gov (United States)

    Pessoa de Magalhães, Roberto J.; Vidriales, María-Belén; Paiva, Bruno; Fernandez-Gimenez, Carlos; García-Sanz, Ramón; Mateos, Maria-Victoria; Gutierrez, Norma C.; Lecrevisse, Quentin; Blanco, Juan F; Hernández, Jose; de las Heras, Natalia; Martinez-Lopez, Joaquin; Roig, Monica; Costa, Elaine Sobral; Ocio, Enrique M.; Perez-Andres, Martin; Maiolino, Angelo; Nucci, Marcio; De La Rubia, Javier; Lahuerta, Juan-Jose; San-Miguel, Jesús F.; Orfao, Alberto

    2013-01-01

    Multiple myeloma remains largely incurable. However, a few patients experience more than 10 years of relapse-free survival and can be considered as operationally cured. Interestingly, long-term disease control in multiple myeloma is not restricted to patients with a complete response, since some patients revert to having a profile of monoclonal gammopathy of undetermined significance. We compared the distribution of multiple compartments of lymphocytes and dendritic cells in the bone marrow and peripheral blood of multiple myeloma patients with long-term disease control (n=28), patients with newly diagnosed monoclonal gammopathy of undetermined significance (n=23), patients with symptomatic multiple myeloma (n=23), and age-matched healthy adults (n=10). Similarly to the patients with monoclonal gammopathy of undetermined significance and symptomatic multiple myeloma, patients with long-term disease control showed an expansion of cytotoxic CD8+ T cells and natural killer cells. However, the numbers of bone marrow T-regulatory cells were lower in patients with long-term disease control than in those with symptomatic multiple myeloma. It is noteworthy that B cells were depleted in patients with monoclonal gammopathy of undetermined significance and in those with symptomatic multiple myeloma, but recovered in both the bone marrow and peripheral blood of patients with long-term disease control, due to an increase in normal bone marrow B-cell precursors and plasma cells, as well as pre-germinal center peripheral blood B cells. The number of bone marrow dendritic cells and tissue macrophages differed significantly between patients with long-term disease control and those with symptomatic multiple myeloma, with a trend to cell count recovering in the former group of patients towards levels similar to those found in healthy adults. In summary, our results indicate that multiple myeloma patients with long-term disease control have a constellation of unique immune changes

  10. The needs of the caregiver in the long-term treatment of Alzheimer disease.

    Science.gov (United States)

    Bullock, Roger

    2004-01-01

    The long-term well-being of caregivers should be included as part of the treatment of patients with Alzheimer disease (AD). Throughout the process of caring for patients with AD, caregivers frequently experience social, emotional, physical, and financial losses, which become more significant as the disease progresses. Minimizing these losses is a goal in the overall management of AD. Successful treatment of the patient has been shown to positively impact quality of life for the caregiver. Randomized, controlled studies of acetylcholinesterase inhibitors (AChEIs) have demonstrated the effectiveness of these agents in stabilizing cognitive function and delaying behavioral symptoms. Moreover, a decrease in the incidence of nursing home placement has been associated with this therapy. The growing burden of AD on families and society as a whole warrants the investigation of ways to minimize the impact of AD. AChEIs play an important role in this effort. Further studies are needed to more closely examine the impact of specific AChEIs on caregiver burden.

  11. Long-Term Adult Feline Liver Organoid Cultures for Disease Modeling of Hepatic Steatosis.

    Science.gov (United States)

    Kruitwagen, Hedwig S; Oosterhoff, Loes A; Vernooij, Ingrid G W H; Schrall, Ingrid M; van Wolferen, Monique E; Bannink, Farah; Roesch, Camille; van Uden, Lisa; Molenaar, Martijn R; Helms, J Bernd; Grinwis, Guy C M; Verstegen, Monique M A; van der Laan, Luc J W; Huch, Meritxell; Geijsen, Niels; Vries, Robert G; Clevers, Hans; Rothuizen, Jan; Schotanus, Baukje A; Penning, Louis C; Spee, Bart

    2017-04-11

    Hepatic steatosis is a highly prevalent liver disease, yet research is hampered by the lack of tractable cellular and animal models. Steatosis also occurs in cats, where it can cause severe hepatic failure. Previous studies demonstrate the potential of liver organoids for modeling genetic diseases. To examine the possibility of using organoids to model steatosis, we established a long-term feline liver organoid culture with adult liver stem cell characteristics and differentiation potential toward hepatocyte-like cells. Next, organoids from mouse, human, dog, and cat liver were provided with fatty acids. Lipid accumulation was observed in all organoids and interestingly, feline liver organoids accumulated more lipid droplets than human organoids. Finally, we demonstrate effects of interference with β-oxidation on lipid accumulation in feline liver organoids. In conclusion, feline liver organoids can be successfully cultured and display a predisposition for lipid accumulation, making them an interesting model in hepatic steatosis research. Copyright © 2017 The Author(s). Published by Elsevier Inc. All rights reserved.

  12. Chronic disease associated with long-term concentrations of nitrogen dioxide

    Energy Technology Data Exchange (ETDEWEB)

    Abbey, D.E.; Colome, S.D.; Mills, P.K.; Burchette, R.; Beeson, W.L.; Tian, Y. (Loma Linda Univ., CA (United States))

    1993-04-01

    A prospective epidemiologic cohort study of 6,000 residentially stable and non-smoking Seventh-day Adventists (SDA) in California was conducted to evaluate long-term cumulative levels of ambient nitrogen dioxide (NO2) in association with several chronic diseases. These diseases included respiratory symptoms, cancer, myocardial infarction (MI), and all natural causes mortality. Cumulative ambient concentrations of NO2 were estimated for each study subject using monthly interpolations from fixed site monitoring stations and applying these estimates to the monthly residence and work place zip code histories of study participants. In addition, a personal NO2 exposure study on a randomly selected sample of 650 people in southern California was conducted to predict total personal NO2 exposure using household and lifestyle characteristics and ambient NO2 concentrations. It was found that good predictability could be obtained (correlation coefficient between predicted and observed values = 0.79) from a model predicting personal NO2. The resulting regression equations from the personal NO2 exposure study were applied to the epidemiologic study cohort to adjust ambient concentrations of NO2.

  13. Homeopathy in paediatric atopic diseases: long-term results in children with atopic dermatitis.

    Science.gov (United States)

    Rossi, Elio; Bartoli, Paola; Bianchi, Alba; Da Frè, Monica

    2012-01-01

    To study the socio-demographic features, the prescribed remedies and the outcome of atopic diseases in children treated with homeopathy at the Homeopathic Clinic of Lucca (Italy), and the long-term outcome of children suffering from atopic dermatitis (AD) after an approximate 8-year period (range 5-10 years). Our data derive from an observational longitudinal study carried out on 213 children (38.6%) with atopic diseases out of 551 children consecutively examined from September 1998 to December 2008. We used the Glasgow Homeopathic Hospital Outcome Score to evaluate the results that were classified on the basis of a Likert scale. Eighty-three (39%) children were affected by asthma, 51 (24%) by allergic rhinoconjunctivitis, 76 (36%) by AD and 3 (1%) by food intolerance. Follow-up patients were 104 (48.8%), and 65 (62.5%) of them reported a major improvement or resolution. The parents of paediatric patients suffering from AD, who had started homeopathic treatment at homeopathy in atopic children. Furthermore, according to the data from the literature paediatric patients treated with homeopathy seem to show a reduced tendency to maintain AD and develop asthma (and allergic rhinitis) in adult age. Copyright © 2011 The Faculty of Homeopathy. Published by Elsevier Ltd. All rights reserved.

  14. Short- and long-term response to corticosteroid therapy in chronic beryllium disease.

    Science.gov (United States)

    Marchand-Adam, S; El Khatib, A; Guillon, F; Brauner, M W; Lamberto, C; Lepage, V; Naccache, J-M; Valeyre, D

    2008-09-01

    Chronic beryllium disease (CBD) is a granulomatous disorder that affects the lung after exposure to beryllium. The present study reports short- and long-term evolution of granulomatous and fibrotic components in eight patients with severe CBD receiving corticosteroid therapy. Eight patients with confirmed CBD were studied at baseline, after initial corticosteroid treatment (4-12 months), at relapse and at the final visit. Beryllium exposure, Glu(69) (HLA-DPB1 genes coding for glutamate at position beta69) polymorphism, symptoms, pulmonary function tests (PFT), serum angiotensin-converting enzyme (SACE) and high-resolution computed tomography (HRCT) quantification of pulmonary lesions were analysed. The CBD patients were observed for a median (range) of 69 (20-180) months. After stopping beryllium exposure, corticosteroids improved symptoms and PFT (vital capacity +26%, diffusing capacity of the lung for carbon monoxide +15%), and decreased SACE level and active lesion HRCT score. In total, 18 clinical relapses occurred after the treatment was tapered and these were associated with SACE and active lesion HRCT score impairment. At the final visit, corticosteroids had completely stabilised all parameters including both HRCT scores of active lesions and fibrotic lesions in six out of eight patients. Corticosteroids were beneficial in chronic beryllium disease. They were effective in suppressing granulomatosis lesions in all cases and in stopping the evolution to pulmonary fibrosis in six out of eight patients.

  15. Long-term disease-free survival in advanced melanomas treated with nitrosoureas: mechanisms and new perspectives.

    Science.gov (United States)

    Durando, Xavier; Thivat, Emilie; D'Incan, Michel; Sinsard, Anne; Madelmont, Jean-Claude; Chollet, Philippe

    2005-11-15

    Median survival of metastatic malignant melanoma is 6.0 to 7.5 months, with a 5-year survival of approximately 6.0%. Although long-term complete remissions are rare, few reports describe cases after chemotherapy. Fifty-three patients with metastatic melanoma were treated with Cystemustine, a chloroethyl nitrosourea (CENU) (60 or 90 mg/m2). We describe 5 cases, presenting with complete response with long-term disease-free survival of long-term remission of 14, 12, 9, 7 and 6 years after Cystemustine therapy alone. Long-term survival has already been described in literature, but in all cases they have been obtained after chemotherapy associated with or followed by surgery. But despite these noteworthy and encouraging but also rare results, it appears essential to increase Cystemustine efficiency.

  16. Long-term disease-free survival in advanced melanomas treated with nitrosoureas: mechanisms and new perspectives

    Directory of Open Access Journals (Sweden)

    Sinsard Anne

    2005-11-01

    Full Text Available Abstract Background Median survival of metastatic malignant melanoma is 6.0 to 7.5 months, with a 5-year survival of ~6.0%. Although long-term complete remissions are rare, few reports describe cases after chemotherapy. Fifty-three patients with metastatic melanoma were treated with Cystemustine, a chloroethyl nitrosourea (CENU (60 or 90 mg/m2. Case presentation We describe 5 cases, presenting with complete response with long-term disease-free survival of long-term remission of 14, 12, 9, 7 and 6 years after Cystemustine therapy alone. Conclusion Long-term survival has already been described in literature, but in all cases they have been obtained after chemotherapy associated with or followed by surgery. But despite these noteworthy and encouraging but also rare results, it appears essential to increase cystemustine efficiency.

  17. Effect of Baseline Nutritional Status on Long-term Multivitamin Use and Cardiovascular Disease Risk

    Science.gov (United States)

    Rautiainen, Susanne; Gaziano, J. Michael; Christen, William G.; Bubes, Vadim; Kotler, Gregory; Glynn, Robert J.; Manson, JoAnn E.; Buring, Julie E.

    2017-01-01

    Importance Long-term multivitamin use had no effect on risk of cardiovascular disease (CVD) in the Physicians’ Health Study II. Baseline nutritional status may have modified the lack of effect. Objective To investigate effect modification by various baseline dietary factors on CVD risk in the Physicians’ Health Study II. Design, Setting, and Participants The Physicians’ Health Study II was a randomized, double-blind, placebo-controlled trial testing multivitamin use (multivitamin [Centrum Silver] or placebo daily) among US male physicians. The Physicians’ Health Study II included 14 641 male physicians 50 years or older, 13 316 of whom (91.0%) completed a baseline 116-item semiquantitative food frequency questionnaire and were included in the analyses. This study examined effect modification by baseline intake of key foods, individual nutrients, dietary patterns (Alternate Healthy Eating Index and Alternate Mediterranean Diet Score), and dietary supplement use. The study began in 1997, with continued treatment and follow-up through June 1, 2011. Interventions Multivitamin or placebo daily. Main Outcomes and Measures Major cardiovascular events, including nonfatal myocardial infarction, nonfatal stroke, and CVD mortality. Secondary outcomes included myocardial infarction, total stroke, CVD mortality, and total mortality individually. Results In total, 13 316 male physicians (mean [SD] age at randomization, 64.0 [9.0] years in those receiving the active multivitamin and 64.0 [9.1] years in those receiving the placebo) were observed for a mean (SD) follow-up of 11.4 (2.3) years. There was no consistent evidence of effect modification by various foods, nutrients, dietary patterns, or baseline supplement use on the effect of multivitamin use on CVD end points. Statistically significant interaction effects were observed between multivitamin use and vitamin B6 intake on myocardial infarction, between multivitamin use and vitamin D intake on CVD mortality

  18. Results of repeated transsphenoidal surgery in Cushing's disease. Long-term follow-up.

    Science.gov (United States)

    Valderrábano, Pablo; Aller, Javier; García-Valdecasas, Leopoldo; García-Uría, José; Martín, Laura; Palacios, Nuria; Estrada, Javier

    2014-04-01

    Transsphenoidal surgery (TSS) is the treatment of choice for Cushing's disease (CD). However, the best treatment option when hypercortisolism persists or recurs remains unknown. The aim of this study was to analyze the short and long-term outcome of repeat TSS in this situation and to search for response predictors. Data from 26 patients with persistent (n=11) or recurrent (n=15) hypercortisolism who underwent repeat surgery by a single neurosurgeon between 1982 and 2009 were retrospectively analyzed. Remission was defined as normalization of urinary free cortisol (UFC) levels, and recurrence as presence of elevated UFC levels after having achieved remission. The following potential outcome predictors were analyzed: adrenal status (persistence or recurrence) after initial TSS, tumor identification in imaging tests, degree of hypercortisolism before repeat TSS, same/different surgeon in both TSS, and time to repeat surgery. Immediate postoperative remission was achieved in 12 patients (46.2%). Five of the 10 patients with available follow-up data relapsed after surgery (median time to recurrence, 13 months). New hormone deficiencies were seen in seven patients (37%), and two patients had cerebrospinal fluid leakage. No other major complications occurred. None of the preoperative factors analyzed was predictive of surgical outcome. When compared to initial surgery, repeat TSS for CD is associated to a lower remission rate and a higher risk of recurrence and complications. Further studies are needed to define outcome predictors. Copyright © 2013 SEEN. Published by Elsevier Espana. All rights reserved.

  19. Models of Disease Vector Control: When Can Aggressive Initial Intervention Lower Long-Term Cost?

    Science.gov (United States)

    Oduro, Bismark; Grijalva, Mario J; Just, Winfried

    2018-04-01

    Insecticide spraying of housing units is an important control measure for vector-borne infections such as Chagas disease. As vectors may invade both from other infested houses and sylvatic areas and as the effectiveness of insecticide wears off over time, the dynamics of (re)infestations can be approximated by [Formula: see text]-type models with a reservoir, where housing units are treated as hosts, and insecticide spraying corresponds to removal of hosts. Here, we investigate three ODE-based models of this type. We describe a dual-rate effect where an initially very high spraying rate can push the system into a region of the state space with low endemic levels of infestation that can be maintained in the long run at relatively moderate cost, while in the absence of an aggressive initial intervention the same average cost would only allow a much less significant reduction in long-term infestation levels. We determine some sufficient and some necessary conditions under which this effect occurs and show that it is robust in models that incorporate some heterogeneity in the relevant properties of housing units.

  20. Telemedicine support to patients with chronic diseases for better long-term control at home

    Directory of Open Access Journals (Sweden)

    Drago Rudel

    2017-02-01

    Full Text Available Authors in many scientific publications suggest that the telemonitoring of health parameters is a useful tool for supporting patients with long-term conditions staying at home and their self-management of the disease. Those patients are likely to benefit from timely and adequate response to deteriorated conditions detected by the telemedicine system. Almost all of the studies state that telemedicine provided as telemonitoring can be an effective add-on tool in the hands of patients and medical experts for the self-management of patients with, for example, heart failure or diabetes. In this paper the principles of patient telemonitoring are presented as applied within a telemedicine service provided by the Centre for Telehealth (CEZAR at the General Hospital Slovenj Gradec (Slovenia. The centre supports patients with diabetes mellitus type 2 and/or with chronic congestive heart failure. The service was set-up in 2014 as part of a European project called UNITED4HEALTH. Since then over 550 patients from the Carinthia and Saleška regions (Slovenia have been receiving telemedicine support for more than two years. The clinical outcomes of the telemedicine service published elsewhere prove that the selected telemedicine service model is adequate and the implemented technological solution is acceptable for all service users: the patients and the clinicians.

  1. Long-term use of adalimumab in the treatment of rheumatic diseases

    Directory of Open Access Journals (Sweden)

    Charalampos Papagoras

    2009-05-01

    Full Text Available Charalampos Papagoras, Paraskevi V Voulgari, Alexandros A DrososRheumatology Clinic, Department of Internal Medicine, Medical School, University of Ioannina, Ioannina, GreeceAbstract: Adalimumab, a fully humanized monoclonal antibody against tumor necrosis factor-alpha (TNFα, has been evaluated in various randomized placebo-controlled trials in rheumatoid arthritis, ankylosing spondylitis, psoriatic arthritis and juvenile idiopathic arthritis. In the short time frame of these trials adalimumab has been shown to be effective in reducing disease activity, slowing radiographic disease progression and improving patients’ quality of life, while at the same time demonstrating an acceptable safety profile. Furthermore, release of adalimumab on the market, prospective observational studies, as well as open-label extensions of the original double-blind trials have provided experience and data about the long-term efficacy and safety of the drug. Initial effectiveness, in terms of reducing disease activity, is sustained, while in most cases patients treated with adalimumab experienced a slower radiographic progression and consequently less disability and improved health-related quality-of-life outcomes. Moreover, long-standing treatment of thousands of patients with adalimumab outside the controlled context of clinical trials was not related to new safety signals, with the most common adverse events being respiratory infections. The most common serious adverse events seem to be tuberculosis reactivation, while a putative association with malignant lymphoma development is not yet proven. Besides, both of these adverse reactions pertain to the whole TNFα blocker group. In conclusion, adalimumab is a safe and effective option for the treatment of patients with rheumatoid arthritis, ankylosing spondylitis, psoriatic arthritis and juvenile idiopathic arthritis. Keywords: adalimumab, tumor necrosis factor-alpha, rheumatoid arthritis, ankylosing spondylitis

  2. LONG-TERM OUTCOME OF THE DIFFERENT TREATMENT ALTERNATIVES FOR RECURRENT AND PERSISTENT CUSHING DISEASE.

    Science.gov (United States)

    Espinosa-de-Los-Monteros, Ana Laura; Sosa-Eroza, Ernesto; Espinosa, Etual; Mendoza, Victoria; Arreola, Rocio; Mercado, Moises

    2017-07-01

    Treatment alternatives for persistent and recurrent Cushing disease (CD) include pituitary surgical re-intervention, radiation therapy (RT), pharmacotherapy, and bilateral adrenalectomy (BA). The decision of which of these alternatives is better suited for the individual patient rests on clinical judgment and the availability of resources. This retrospective cohort study was performed at a referral center to evaluate the long-term efficacy of different secondary interventions for persistent and recurrent CD. We evaluated the hospital charts of 84 patients (77 female, median age 34 years, median follow up 6.3 years) with CD diagnosed, treated, and followed at our multidisciplinary clinic according to a pre-established protocol. Of the 81 patients who were initially treated with transsphenoidal surgery (TSS), 61.7% had a long-lasting remission, 16% had persistent disease, and 22% achieved remission but relapsed during follow-up. The most frequently used secondary treatment was pituitary re-intervention, followed by ketoconazole, RT, and BA. Early remissions were observed in 66.6% of the re-operated and in 58.3% of the radiated patients; long-lasting remission was achieved in 33.3% and 41.6% of these patients, respectively. Nelson syndrome developed in 41.6% of the patients who underwent BA. Upon last follow-up, 88% of all the patients are in remission, and 9.5% are biochemically controlled with ketoconazole. The efficacy of treatment alternatives for recurrent or persistent CD varies considerably among patients and multiple interventions are often required to achieve long-lasting remission. ACTH = adrenocorticotrophic hormone; BA = bilateral adrenalectomy; CBG = cabergoline; CD = Cushing disease; CV = coefficient of variation; DXM = dexamethasone; IQR = interquartile range; RT = radiation therapy; SRS = stereotactic radiosurgery; TSS = transsphenoidal surgery; UFC = urinary free cortisol; ULN = upper limit of normal.

  3. Uptake of genetic testing and long-term tumor surveillance in von Hippel-Lindau disease

    Directory of Open Access Journals (Sweden)

    Chávez Mireya

    2010-01-01

    procedures in non mutation-carriers. However, mutation-carriers showed poor adherence to long-term tumor surveillance. Therefore, many of them did not obtain the full benefit of early detection and treatment, which is central to the reduction of morbidity and mortality in VHL disease. Studies designed to improve adherence to vigilance protocols will be necessary to improve treatment and quality of life in patients with hereditary cancer syndromes.

  4. Long-term outcome of laparoscopic and open surgery in patients with Crohn’s disease

    Directory of Open Access Journals (Sweden)

    Hoffmann M

    2017-11-01

    Full Text Available Martin Hoffmann,1 Dina Siebrasse,1 Erik Schlöricke,2 Ralf Bouchard,1 Tobias Keck,1 Claudia Benecke1 1Department of Surgery, University Medical Center Schleswig-Holstein, Campus Lübeck, Lübeck, 2Department of Surgery, Westküstenklinikum Heide, Heide, Germany Aim: To investigate, via data analysis, the long-term outcome of patients who underwent either laparoscopic or open surgery for Crohn’s disease.Methods: A total of 113 patients who had undergone first abdominal surgery due to Crohn’s disease between January 2000 and December 2010 in a maximum care provider facility, were included in the statistical analysis. All patients provided their informed consent prior to inclusion. Data were collected from a database. Follow-up data included data from central mortality registries, general practitioners and a specialized clinic. Statistical analysis of the general patient data and the different operations and approaches were performed with the use of chi-square test, Fisher’s exact test, and the Mann–Whitney U test.Results: The median follow-up period in case of open and laparoscopic surgery was found to be 9 and 6 years, respectively. Statistically significant differences were observed for the following parameters in case of open and laparoscopic surgery, respectively: age (median=44 vs. 36 years, range=15–76 vs. 15–72 years; p=0.007, urgency of operation (23 out of 34 planned vs. 6 out of 70 planned; p<0.001, year of resection (median=2003 vs. 2006, range=2000–2010 vs. 2000–2010; p=0.001. The length of stay in hospital was significantly shorter in the laparoscopic group than that of open surgery group (8 vs. 11 days, respectively; p<0.0001. We did not control for factors such as age and comorbidities in our statistical analysis. We also did not find any differences with regard to perioperative and 90-day mortality, surgical complications, length of specimen, stoma, surgical recurrence rate (10% in both groups and number of re

  5. Mitral valve reconstruction in Barlow disease: long-term echographic results and implications for surgical management.

    Science.gov (United States)

    Jouan, Jérôme; Berrebi, Alain; Chauvaud, Sylvain; Menasché, Philippe; Carpentier, Alain; Fabiani, Jean-Noël

    2012-04-01

    Owing to the complexity of the underlying lesions, Barlow disease remains a challenge for surgeons performing mitral valve repair. We aimed to assess whether our most recent results involving several surgeons were comparable with those of a previous experience in which mitral valve repair was performed by a more limited group of surgeons. From September 2000 to January 2007, 200 patients with Barlow disease (135 men and 65 women; mean age, 56 ± 13 years) were referred to our institution for surgical treatment of their mitral regurgitation. We retrospectively analysed the mitral lesions characteristics, the surgical techniques used, and clinical outcomes. Follow-up echocardiograms were biannually reviewed. Lesions comprised annular dilatation, excess tissue, and leaflet prolapse in all cases. The most frequent prolapsed segments were P2 (88.5%; n = 177) and A2 (55.5%; n = 111). Annular calcifications and restrictive valvular motion were associated in 20% (n = 40). Repair was feasible in 94.7% (n = 179/189) of non-redo interventions. Immediate postoperative echocardiography showed residual mitral regurgitation greater than 1+ in 6 cases; these patients were all reoperated on within the next months. Operative mortality was 1.5% (n = 3). Mean follow-up was 77.5 ± 25.6 months. At 8 years postoperatively, overall survival was 88.6% ± 3.1%, freedom from reintervention was 95.3% ± 1.7%, and freedom from late recurrent moderate mitral regurgitation (>2+) was 90.2% ± 3.1% Provided that the fundamental principles of mitral valve reconstruction are respected, the surgical techniques are highly reproducible with good long-term results, similar to those published during the pioneering phase of this surgery. Copyright © 2012 The American Association for Thoracic Surgery. Published by Mosby, Inc. All rights reserved.

  6. Peyronie's disease - a perspective on the disease and the long-term ...

    African Journals Online (AJOL)

    From 1966 to 1988, 98 of 108 patients with symptomatic Peyronie's disease received radiotherapy at our institution. In 11 of 61 patients (18%) who attended the clinic regularly for follow-up for longer than a year, new lesions distinct from the original lesions developed. This confirms that there is progression of the disease in ...

  7. Long-Term Outcomes of Total Exudative Retinal Detachments in Stage 3B Coats Disease.

    Science.gov (United States)

    Li, Albert S; Capone, Antonio; Trese, Michael T; Sears, Jonathan E; Kychenthal, Andres; De la Huerta, Irina; Ferrone, Philip J

    2018-06-01

    To evaluate the long-term outcomes of treatment of total exudative retinal detachments (ERDs) secondary to Coats disease (stage 3B) and the role of vitrectomy. Retrospective, observational case series. A total of 16 eyes in 16 patients undergoing treatment for total ERDs secondary to Coats disease with at least 5 years of follow-up. We reviewed the records of patients with stage 3B Coats disease. The interventions, including the timing of vitrectomy if used, and clinical course were recorded. The primary outcome measures were visual acuity at the most recent appointment, whether there was progression to neovascular glaucoma (NVG) or phthisis bulbi, and need for enucleation. All patients received ablative treatment (photocoagulation or cryotherapy), with 8 having scleral buckling (SB) and 6 having external drainage of subretinal fluid (XD). Of the 12 patients who had pars plana vitrectomy (PPV), 8 had early PPV (EV) in the first year after presenting, and 4 of 8 in the expectant management group had late PPV (late vitrectomy) at a mean of 4.3 years post-presentation for treatment of significant traction retinal detachment (TRD). The other 4 patients of 8 in the expectant management group did not require vitrectomy. Mean follow-up overall was 9 1/2 years. At the date of last follow-up, 50% had no light perception or light perception vision, which was consistent across the subgroups that underwent EV (4/8), late vitrectomy (2/4), or no PPV (2/4). A total of 4 of 16 patients had progression to NVG or phthisis, 1 of whom required enucleation. In this retrospective series of patients with Stage 3B Coats disease, ablative therapy with a combination of PPV, XD, or SB was effective in preventing progression to NVG or phthisis in the majority of patients, thus preserving the globe. Half of the patients (4/8) in this series who did not undergo PPV in the early vitrectomy group developed late-onset TRD, suggesting a possible role for early prophylactic vitrectomy with possible

  8. Management of Long-Term Complications of HIV Disease: Focus on Cardiovascular Disease.

    Science.gov (United States)

    Currier, Judith S

    2018-04-01

    HIV-infected individuals on effective antiretroviral therapy experience a number of non-AIDS noncommunicable diseases, such as cardiovascular disease, more frequently than uninfected individuals. Common pathways for such diseases are chronic immune activation and inflammation, including the prolonged inflammation associated with lower nadir CD4+ cell count. Prevention and treatment of non-AIDS conditions include treatment of traditional risk factors, lifestyle interventions, earlier initiation of antiretroviral therapy, and potentially therapies specifically targeting inflammation and immune activation (eg, statins). This article summarizes a presentation by Judith S. Currier, MD, at the IAS-USA continuing education program, Improving the Management of HIV Disease, held in New York, New York, in February 2017.

  9. Long-term results of radioiodine (131I) therapy in 331 patients with Graves' disease

    International Nuclear Information System (INIS)

    Saito, Shintaro; Sakurada, Toshiro; Yamamoto, Makiko; Yoshida, Katsumi; Kaise, Kazuo

    1980-01-01

    To evaluate the long-term results of 131 I treatment for Graves' disease, the thyroid function was studied in 331 patients 5 - 17 years after this therapy. Twenty-five patients were hypothyroid and had already been on thyroid medication. Among the remaining 306 patients without any treatment for thyroid disorder, 188 patients (61.4%) had a normal thyroid-stimulating hormone (TSH) level (less than 10 μU/ml) of whom 151 were euthyroid with normal thyroxine (T 4 ) and triiodothyronine (T 3 ) level. 118 patients (38.6%) had a high TSH level, of whom 22 were hypothyroid with low T 4 and T 3 levels and 38 were with normal T 3 and low T 4 levels, and 14 patients out of this 38 were clinically hypothyroid. Although all of the patients with high TSH levels do not require the replacement therapy for hypothyroidism, the overt hypothyroidism seems to occur sooner or later in patients with a lower T 4 level. Among 331 patients studied, 61 (18.4%) were clinically hypothyroid. The incidence of hypothyroidism was 20.7% after 10 years of 131 I treatment and 33% after 15 - 17 years. The incidence of patients with high TSH levels was 50% after 10 years of 131 I treatment and 60% after 15 - 17 years. Both of cumulative incidence of hypothyroidism and patients with high TSH levels increased linearly with years after the treatment, which coincided with the linear decrease of mean T 4 and T 3 levels and linear increase of mean TSH level with years after the treatment. The difference of positive incidence of antithyroid antibodies between cases of normal TSH level and high TSH level was not significant. (author)

  10. Long-term clinical outcomes in type 1 Gaucher disease following 10 years of imiglucerase treatment.

    Science.gov (United States)

    Weinreb, Neal J; Goldblatt, Jack; Villalobos, Jacobo; Charrow, Joel; Cole, J Alexander; Kerstenetzky, Marcelo; vom Dahl, Stephan; Hollak, Carla

    2013-05-01

    We studied the effect of long-term alglucerase/imiglucerase (Ceredase®/Cerezyme®, Genzyme, a Sanofi company, Cambridge, MA, USA) treatment on hematological, visceral, and bone manifestations of Gaucher disease type 1 (GD1). The International Collaborative Gaucher Group (ICGG) Gaucher Registry identified GD1 patients treated with alglucerase/imiglucerase who had dose and clinical data at first infusion and after 10 years of follow-up. Data for hemoglobin, platelet count, organ volumes, bone pain, and bone crisis were analyzed. Tests of the null hypothesis (no change from first infusion to 10 years) were performed using t tests for within-patient absolute change in continuous measurements and McNemar/chi-square tests for change in distributions using categorical values. An alpha level of 0.05 designated statistical significance. As of October 2011, 557 nonsplenectomized and 200 splenectomized patients met the inclusion criteria. The majority of GD1 patients had at least one N370S allele. Compared with nonsplenectomized patients at first infusion, splenectomized patients had lower percentages of anemia (26.0 % vs. 42.8 %) and thrombocytopenia (14.2 % vs. 76.3 %), similar percentages of moderate or severe hepatomegaly (81.2 % vs. 80.0 %), and higher percentages of bone pain (88.9 % vs. 52.4 %) and bone crises (38.3 % vs. 16.0 %). After 10 years, both groups showed significant (p < 0.05) improvements in mean hemoglobin levels, platelet count, liver, and spleen (nonsplenectomized) volumes, and bone crises. Initial dosing in both groups ranged from <15 U/kg to ≤90 U/kg every 2 weeks. After 10 years, the majority was receiving 15 to ≤45 U/kg every 2 weeks. Ten years of imiglucerase treatment results in sustainable improvements in all GD1 parameters.

  11. Long-term sinonasal outcomes of aspirin desensitization in aspirin exacerbated respiratory disease.

    Science.gov (United States)

    Cho, Kyu-Sup; Soudry, Ethan; Psaltis, Alkis J; Nadeau, Kari C; McGhee, Sean A; Nayak, Jayakar V; Hwang, Peter H

    2014-10-01

    This study aimed to assess sinonasal outcomes in patients with aspirin exacerbated respiratory disease (AERD) undergoing aspirin desensitization following endoscopic sinus surgery (ESS). Case series with chart review. University hospital. A retrospective review of sinonasal outcomes was conducted for 30 AERD patients undergoing aspirin desensitization and maintenance therapy following ESS. Sinonasal outcomes were prospectively assessed by the Sinonasal Outcomes Test-22 (SNOT-22) and endoscopic polyp grading system. Data were collected preoperatively, 1 and 4 weeks postsurgery (before desensitization), and 1, 6, 12, 18, 24, and 30 months after aspirin desensitization. Twenty-eight of 30 patients (93.3%) successfully completed aspirin desensitization, whereas 2 of 30 (6.7%) were unable to complete desensitization due to respiratory intolerance. Of the 21 patients who successfully completed a minimum of 24 weeks of follow-up, 20 (95.2%) patients demonstrated sustained endoscopic and symptomatic improvement for a median follow-up period of 33 months. After surgical treatment but before desensitization, patients experienced significant reductions in SNOT-22 and polyp grade scores. In the first 6 months after aspirin desensitization, patients experienced further significant reductions in SNOT-22 scores, whereas polyp grade remained stable. The improvements in symptom endoscopic scores were preserved throughout the follow-up period after desensitization. No patients required additional sinus surgery. One patient had to discontinue aspirin therapy due to gastrointestinal side effects. No other adverse reactions to aspirin were noted. Aspirin desensitization following ESS appears to be a well-tolerated and effective adjunctive therapy for long-term control of nasal polyposis in patients with AERD. © American Academy of Otolaryngology—Head and Neck Surgery Foundation 2014.

  12. Long-term functional outcome after surgical repair of cranial cruciate ligament disease in dogs

    OpenAIRE

    Mölsä, Sari H; Hyytiäinen, Heli K; Hielm-Björkman, Anna K; Laitinen-Vapaavuori, Outi M

    2014-01-01

    Background Cranial cruciate ligament (CCL) rupture is a very common cause of pelvic limb lameness in dogs. Few studies, using objective and validated outcome evaluation methods, have been published to evaluate long-term (>1 year) outcome after CCL repair. A group of 47 dogs with CCL rupture treated with intracapsular, extracapsular, and osteotomy techniques, and 21 healthy control dogs were enrolled in this study. To evaluate long-term surgical outcome, at a minimum of 1.5 years after unilate...

  13. Peyronie's disease - a perspective on the disease and the long-term ...

    African Journals Online (AJOL)

    Abstract FrOIIl 1966 to 1988, 98 of 108 patients with sYIIlP- to=atic Peyronie's disease received radiotherapy at our institution. In 11 of 61 patients (18%) who attended the clinic regularly for follow-up for longer than a year, new lesions distinct frOIIl the original lesions developed. This confirms that there is progression.

  14. Renal function predicts long-term outcome on enzyme replacement therapy in patients with Fabry disease.

    Science.gov (United States)

    Lenders, Malte; Schmitz, Boris; Stypmann, Jörg; Duning, Thomas; Brand, Stefan-Martin; Kurschat, Christine; Brand, Eva

    2017-12-01

    Renal and cardiac involvement is responsible for substantial morbidity and mortality in Fabry disease (FD). We analysed the incidence of FD-related renal, cardiac and neurologic end points in patients with FD on long-term enzyme replacement therapy (ERT). A retrospective analysis of prospectively collected data from two German FD centres was performed. The impact of renal and cardiac function at ERT-naïve baseline on end point development despite ERT was analysed. Fifty-four patients (28 females) receiving ERT (mean 81 ± 21 months) were investigated. Forty per cent of patients were diagnosed with clinical end points before ERT initiation and 50% of patients on ERT developed new clinical end points. In patients initially diagnosed with an end point before ERT initiation, the risk for an additional end point on ERT was increased {hazard ratio [HR] 3.83 [95% confidence interval (CI) 1.61-9.08]; P = 0.0023}. A decreased glomerular filtration rate (eGFR) ≤75 mL/min/1.73 m2 in ERT-naïve patients at baseline was associated with an increased risk for cardiovascular end points [HR 3.59 (95% CI 1.15-11.18); P = 0.0273] as well as for combined renal, cardiac and neurologic end points on ERT [HR 4.77 (95% CI 1.93-11.81); P = 0.0007]. In patients with normal kidney function, left ventricular hypertrophy at baseline predicted a decreased end point-free survival [HR 6.90 (95% CI 2.04-23.27); P = 0.0018]. The risk to develop an end point was independent of sex. In addition to age, even moderately impaired renal function determines FD progression on ERT. In patients with FD, renal and cardiac protection is warranted to prevent patients from deleterious manifestations of the disease. © The Author 2016. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved.

  15. Testicular function in young men in long-term remission after treatment for the early stages of Hodgkin's disease

    DEFF Research Database (Denmark)

    Specht, L; Geisler, C; Hansen, M M

    1984-01-01

    16 young men in long-term remission after standard treatment for the early stages of Hodgkin's disease were examined for testicular function 48 to 125 months after termination of therapy. The patients had received mantle field irradiation, plus either irradiation of infradiaphragmatic lymph nodes...... to chemotherapy, especially including alkylating agents....

  16. Long-term quasi-continuous oxygen saturation levels obtained from sternal photoplethysmography on patients with obstructive lung diseases

    DEFF Research Database (Denmark)

    Chreiteh, Shadi; Saadi, Dorthe Bodholt; Belhage, Bo

    2016-01-01

    the sternum of patients admitted to the hospital with obstructive lung diseases. Due to the lack of a gold standard reference that is suitable for long-term monitoring without interfering with the patient's activity level, we extracted reliable segments based on knowledge from the basic pulse oximeter theory...

  17. Chronic orbital inflammatory disease and optic neuropathy associated with long-term intranasal cocaine abuse: 2 cases and literature review

    NARCIS (Netherlands)

    Siemerink, Martin J.; Freling, Nicole J. M.; Saeed, Peerooz

    2017-01-01

    Orbital inflammatory disease and secondary optic neuropathy is a rare but devastating complication of long-term intranasal cocaine abuse. We describe 2 patients with a history of intranasal cocaine consumption who presented with subacute onset of unilateral vision loss from optic neuropathy and

  18. Short- and long-term black tea consumption reverses endothelial dysfunction in patients with coronary artery disease.

    Science.gov (United States)

    Duffy, S J; Keaney , J F; Holbrook, M; Gokce, N; Swerdloff, P L; Frei, B; Vita, J A

    2001-07-10

    Epidemiological studies suggest that tea consumption decreases cardiovascular risk, but the mechanisms of benefit remain undefined. Endothelial dysfunction has been associated with coronary artery disease and increased oxidative stress. Some antioxidants have been shown to reverse endothelial dysfunction, and tea contains antioxidant flavonoids. Methods and Results-- To test the hypothesis that tea consumption will reverse endothelial dysfunction, we randomized 66 patients with proven coronary artery disease to consume black tea and water in a crossover design. Short-term effects were examined 2 hours after consumption of 450 mL tea or water. Long-term effects were examined after consumption of 900 mL tea or water daily for 4 weeks. Vasomotor function of the brachial artery was examined at baseline and after each intervention with vascular ultrasound. Fifty patients completed the protocol and had technically suitable ultrasound measurements. Both short- and long-term tea consumption improved endothelium- dependent flow-mediated dilation of the brachial artery, whereas consumption of water had no effect (Peffect on endothelium-independent nitroglycerin-induced dilation. An equivalent oral dose of caffeine (200 mg) had no short-term effect on flow-mediated dilation. Plasma flavonoids increased after short- and long-term tea consumption. Short- and long-term black tea consumption reverses endothelial vasomotor dysfunction in patients with coronary artery disease. This finding may partly explain the association between tea intake and decreased cardiovascular disease events.

  19. Long-term Follow-Up of Individuals with Celiac Disease: An Evaluation of Current Practice Guidelines

    Directory of Open Access Journals (Sweden)

    Jocelyn A Silvester

    2007-01-01

    Full Text Available INTRODUCTION: Celiac disease can be treated by following a strict gluten-free diet for life. If properly followed, the diet resolves symptoms and nutritional deficiencies. It is generally recommended that individuals with celiac disease have careful long-term follow-up. However, it is not clear which elements of disease status evaluation, laboratory investigations and self-management support should be included in follow-up.

  20. Short- and Long-term Outcomes in Patients with Connective Tissue Diseases Undergoing Percutaneous Coronary Intervention

    Directory of Open Access Journals (Sweden)

    Li Zhou

    2016-01-01

    Conclusions: Patients with CTD and CAD may have severe coronary lesions. PCI in these patients tends to result in an increased rate of stent thrombosis and TVR during long-term follow-up, which may be influenced by traditional and nontraditional risk factors.

  1. Impact of the components of Mediterranean nutrition regimen on long-term prognosis of diabetic patients with coronary artery disease

    Directory of Open Access Journals (Sweden)

    Soheila Mosharraf

    2013-11-01

    Full Text Available BACKGROUND: The impact of different nutritional regimens on long-term prognosis and outcome in diabetic patients with coronary artery disease (CAD has been questioned. Therefore, the objective of the present study was to determine the effects of different nutritional components of Mediterranean regimen on long-term cardiovascular events in diabetic patients with CAD in the Iranian population. METHODS: In a prospective cohort study, we recruited 233 consecutive patients with the diagnosis of type 2 diabetes mellitus and with at least 6 months of documented CAD. Nutritional assessment was obtained by a validated semi-quantitative food frequency questionnaire (FFQ and the diet score was calculated on the basis of the Mediterranean diet quality index (Med-DQI. For Assessing long-term CAD prognosis, the patients were followed by telephone for one year. The study endpoint was long-term major adverse cardiac and cerebrovascular event (MACCE. RESULTS: Death was observed in 19 patients (8.2% during the one-year follow-up. Two patients (0.9% suffered non-fatal myocardial infarction and 14 (6.0% needed revascularization within 1 year after discharge from hospital. Overall MACCE within one year in the study population was 12.4%. There were significant differences between number of deaths and dietary scores of saturated fatty acid, cholesterol, meats, fish, and fruit and vegetables (P < 0.05. Moreover, significant differences were found between MACCE rate and dietary scores of saturated fatty acid, cholesterol, and fruit and vegetables (P < 0.05. Using multivariate logistic regression models, Mediterranean dietary regimen could effectively predict long-term death as well as MACCE adjusted for gender and age variables. CONCLUSION: Mediterranean dietary regimens, including low level of cholesterol and saturated fatty acid, can effectively improve long-term outcome including death and MACCE in diabetic patients with CAD.   Keywords: Diabetes Mellitus, Coronary

  2. Symptomatic radiation-induced cardiac disease in long-term survivors of esophageal cancer

    Energy Technology Data Exchange (ETDEWEB)

    Iwahashi, Noriaki; Kosuge, Masami; Kimura, Kazuo [Division of Cardiology, Yokohama City University Medical Center, Yokohama (Japan); Sakamaki, Kentaro [Department of Biostatistics, Yokohama City University Medical Center, Yokohama (Japan); Kunisaki, Chikara [Department of Surgery, Gastroenterological Center, Yokohama City University Medical Center, Yokohama (Japan); Ogino, Ichiro; Watanabe, Shigenobu

    2016-06-15

    To evaluate clinical and dosimetric factors retrospectively affecting the risk of symptomatic cardiac disease (SCD) in esophageal cancer patients treated with radiotherapy. A total of 343 patients with newly diagnosed esophageal cancer were managed with concurrent chemoradiotherapy or radiotherapy alone. Of these, 58 patients were followed at our hospital for at least 4 years. Median clinical follow-up was 79 months. Cardiac toxicity was determined by Common Terminology Criteria for Adverse Events (CTCAE) v. 4.0. The maximum and mean doses to the heart and percentage of the volume were calculated from the dose-volume histograms. SCD manifested in 11 patients. The heart diseases included three pericardial effusions, one pericardial effusion with valvular disease and paroxysmal atrial tachycardia, three atrial fibrillations, one sinus tachycardia, one coronary artery disease, one chest pain with strongly suspected coronary artery disease, and one congestive heart failure. The actual incidence of SCD was 13.8 % at 5 years. Univariate and multivariate analyses of continuous variables revealed that the risk of developing an SCD depended on the volume of the heart receiving a dose greater than 45 Gy (V45), 50 Gy (V50), and 55 Gy (V55). No other clinical factors were found to influence the risk of SCD. For V45, V50, and V55, the lowest significant cutoff values were 15, 10, and 5 %, respectively. High-dose and large-volume irradiation of the heart increased the risk of SCD in long-term survivors. Using modern radiotherapy techniques, it is important to minimize the heart dose-volume parameters without reducing the tumor dose. (orig.) [German] Beurteilung von klinischen und dosimetrischen Faktoren, die mit Risiken eines retrospektiven Auftretens von symptomatischen Herzerkrankungen (SCD) bei Patienten zusammenhaengen, die aufgrund eines Oesophaguskarzinoms strahlentherapeutisch behandelt wurden. Insgesamt 343 Patienten mit neu diagnostiziertem Oesophaguskarzinom wurden mit

  3. Long-Term Nationwide Follow-Up Study of Simple Congenital Heart Disease Diagnosed in Otherwise Healthy Children

    DEFF Research Database (Denmark)

    Videbæk, Jørgen; Laursen, Henning Bækgaard; Olsen, Morten

    2016-01-01

    BACKGROUND: Systematic follow-up is currently not recommended for patients with simple congenital heart disease; however, only a few data exist on the long-term prognosis of simple congenital heart disease. METHODS AND RESULTS: We undertook a nationwide follow-up study of a cohort of 1241 simple...... congenital heart disease patients, diagnosed from 1963 through 1973, in otherwise healthy children and alive at 15 years of age. We identified 10 age- and sex-matched general population controls per patient. We followed the study population through Danish public registries from the age of 15 years up...... with simple congenital heart disease in the 1960s have substantially increased long-term mortality and cardiac morbidity compared with the general population. Further studies on the effectiveness of systematic medical follow-up programs appear warranted....

  4. Long term follow-up of Cushing's disease treated with reserpine and pituitary irradiation followed by subtotal adrenalectomy

    International Nuclear Information System (INIS)

    Murayama, Masanori; Yasuda, Keigo; Minamori, Yoshiaki; Mercado-Asis, L.B.; Morita, Hiroyuki; Miura, Kiyoshi; Yamakita, Noriyoshi.

    1994-01-01

    Subtotal adrenalectomy was given to 10 adult patients with Cushing's disease, concurrently with or following therapeutic regimen by long term reserpine administration and pituitary irradiation. In the present study, we describe long term follow-up results. Two patients died after the operation due to acute adrenal crisis and pneumonia, respectively. The other 8 patients achieved clinical and biochemical remissions and were followed for long term. Three patients relapsed 9, 14 or 17 years after achieving remission, two patients developed hypopituitarism 12 or 20 years after and one died of cerebral vascular accident at 64 years, 5 years after the remission. The remaining 2 patients maintained remission for 10 or 18 years, respectively. During the remission periods of 0.5 to 20 years with a mean of 10.1±6.7 years, 6 of 7 patients examined by 1 mg overnight dexamethasone test showed normal suppressibility of plasma cortisol. Provocative tests of plasma GH by 1-arginine infusion and/or insulin-induced hypoglycemia were performed in 6 patients in the early remission period. All of 5 patients in the arginine infusion test and 3 of 5 in the insulin-induced hypoglycemia test showed normal responses. Furthermore, to facilitate prediction of long term response or failure to our therapeutic regimen, long term reserpine administration and pituitary irradiation, pretreatment clinical and biochemical characteristics were analyzed retrospectively in 3 divided groups; the present 10 patients treated with reserpine and pituitary irradiation followed by subtotal adrenalectomy, 11 patients achieving long term remission treated by our regimen alone, and 7 patients failed with our regimen alone. There were no significant factors predictive of response to our regimen. (author)

  5. The clinical utility of long-term humidification therapy in chronic airway disease.

    Science.gov (United States)

    Rea, Harold; McAuley, Sue; Jayaram, Lata; Garrett, Jeffrey; Hockey, Hans; Storey, Louanne; O'Donnell, Glenis; Haru, Lynne; Payton, Matthew; O'Donnell, Kevin

    2010-04-01

    Persistent airway inflammation with mucus retention in patients with chronic airway disorders such as COPD and bronchiectasis may lead to frequent exacerbations, reduced lung function and poor quality of life. This study investigates if long-term humidification therapy with high flow fully humidified air at 37 degrees C through nasal cannulae can improve these clinical outcomes in this group of patients. 108 patients diagnosed with COPD or bronchiectasis were randomised to daily humidification therapy or usual care for 12 months over which exacerbations were recorded. Lung function, quality of life, exercise capacity, and measures of airway inflammation were also recorded at baseline, 3 and 12 months. Patients on long-term humidification therapy had significantly fewer exacerbation days (18.2 versus 33.5 days; p = 0.045), increased time to first exacerbation (median 52 versus 27 days; p = 0.0495) and reduced exacerbation frequency (2.97/patient/year versus 3.63/patient/year; p = 0.067) compared with usual care. Quality of life scores and lung function improved significantly with humidification therapy compared with usual care at 3 and 12 months. Long-term humidification therapy significantly reduced exacerbation days, increased time to first exacerbation, improved lung function and quality of life in patients with COPD and bronchiectasis. Clinical trial registered with www.actr.org.au; Number ACTRN2605000623695. Copyright 2010 Elsevier Ltd. All rights reserved.

  6. Applicability of long-term electroencephalography in pre-mortem diagnosis of Creutzfeldt-Jakob disease: A case report.

    Science.gov (United States)

    Attaripour Isfahani, Sanaz; Dougherty, Michelle; Gliebus, Gediminas Peter

    2017-01-01

    Creutzfeldt-Jakob disease accounts for more than 90% of all sporadic prion disease cases. The molecular MM2 genotype has been divided into cortical and thalamic subtypes based on structures involved and is characterized clinically by progressive dementia without ataxia or typical electroencephalography changes. Proposed diagnostic criteria for MM2 cortical type sporadic Creutzfeldt-Jakob disease include progressive dementia, cortical hyper-intensity on diffusion-weighted magnetic resonance imaging, increased cerebrospinal fluid 14-3-3 protein level, and the exclusion of other types of dementia. The presence of periodic discharges on electroencephalography in MM2 cortical type were reported in 42% of the cases. We are reporting a case of sporadic Creutzfeldt-Jakob disease cortical MM2-type presenting with rapid cognitive decline, who survived 8 months since symptom onset. Brain imaging, cerebrospinal fluid analysis, and long-term electroencephalography monitoring were obtained and diagnosis was confirmed by autopsy. Short-term electroencephalography recording, performed 5 months after symptom onset, demonstrated diffuse background slowing without epileptiform activity. Long-term video electroencephalography monitoring demonstrated generalized slowing, maximum in bilateral frontal areas, which intermittently would become rhythmic (1-2 Hz) without hemispheric predominance. If the findings do not clearly meet the proposed clinical criteria for sporadic Creutzfeldt-Jakob disease, the use of long-term electroencephalography could increase the sensitivity. We question whether the lack of the characteristic findings on electroencephalography in some cases could be due to insufficient time of recording. Application of long-term electroencephalography monitoring increases the sensitivity of electroencephalography and the certainty of pre-mortem diagnosis of sporadic Creutzfeldt-Jakob disease.

  7. Long-term intravenous treatment of Pompe disease with recombinant human alpha-glucosidase from milk.

    Science.gov (United States)

    Van den Hout, Johanna M P; Kamphoven, Joep H J; Winkel, Léon P F; Arts, Willem F M; De Klerk, Johannes B C; Loonen, M Christa B; Vulto, Arnold G; Cromme-Dijkhuis, Adri; Weisglas-Kuperus, Nynke; Hop, Wim; Van Hirtum, Hans; Van Diggelen, Otto P; Boer, Marijke; Kroos, Marian A; Van Doorn, Pieter A; Van der Voort, Edwin; Sibbles, Barbara; Van Corven, Emiel J J M; Brakenhoff, Just P J; Van Hove, Johan; Smeitink, Jan A M; de Jong, Gerard; Reuser, Arnold J J; Van der Ploeg, Ans T

    2004-05-01

    Recent reports warn that the worldwide cell culture capacity is insufficient to fulfill the increasing demand for human protein drugs. Production in milk of transgenic animals is an attractive alternative. Kilogram quantities of product per year can be obtained at relatively low costs, even in small animals such as rabbits. We tested the long-term safety and efficacy of recombinant human -glucosidase (rhAGLU) from rabbit milk for the treatment of the lysosomal storage disorder Pompe disease. The disease occurs with an estimated frequency of 1 in 40,000 and is designated as orphan disease. The classic infantile form leads to death at a median age of 6 to 8 months and is diagnosed by absence of alpha-glucosidase activity and presence of fully deleterious mutations in the alpha-glucosidase gene. Cardiac hypertrophy is characteristically present. Loss of muscle strength prevents infants from achieving developmental milestones such as sitting, standing, and walking. Milder forms of the disease are associated with less severe mutations and partial deficiency of alpha-glucosidase. In the beginning of 1999, 4 critically ill patients with infantile Pompe disease (2.5-8 months of age) were enrolled in a single-center open-label study and treated intravenously with rhAGLU in a dose of 15 to 40 mg/kg/week. Genotypes of patients were consistent with the most severe form of Pompe disease. Additional molecular analysis failed to detect processed forms of alpha-glucosidase (95, 76, and 70 kDa) in 3 of the 4 patients and revealed only a trace amount of the 95-kDa biosynthetic intermediate form in the fourth (patient 1). With the more sensitive detection method, 35S-methionine incorporation, we could detect low-level synthesis of -glucosidase in 3 of the 4 patients (patients 1, 2, and 4) with some posttranslation modification from 110 kDa to 95 kDa in 1 of them (patient 1). One patient (patient 3) remained totally deficient with both detection methods (negative for cross

  8. Improved maternal nutrition decreases children’s long-term risk of non-communicable diseases (NCDs) and obesity

    DEFF Research Database (Denmark)

    Robertson, Aileen

    Improved maternal nutrition to decrease children’s long-term risk of non-communicable diseases (NCDs) and obesity The nutritional well-being of pregnant women affects not only their health and their fetuses' development but also children's long-term risk of developing NCDs or obesity, according...... to a new report from WHO/Europe. "Good maternal nutrition. The best start in life" was launched under the auspices of the Minister of Health of Latvia during a consultation on maternal nutrition, in Riga on 27–28 June 2016. While the importance of good nutrition in the early development of children has...... – affects not only her child's health as an infant but also the child's risk of obesity and related chronic diseases as an adult. In short, maternal nutrition can truly have an intergenerational impact. Fighting NCDs and obesity through measures to improve maternal nutrition: NCDs are the leading cause...

  9. Megavoltage pituitary irradiation in the management of Cushing's disease and Nelson's syndrome: long-term follow-up

    International Nuclear Information System (INIS)

    Howlett, T.A.; Plowman, P.N.; Wass, J.A.H.; Rees, L.H.; Jones, A.E.; Besser, G.M.

    1989-01-01

    The authors report the long-term follow-up (up to 17.3 years) of the clinical and biochemical effects of megavoltage pituitary irradiation (radiotherapy;RT), administered as primary or secondary therapy, for pituitary Cushing's disease and Nelson's syndrome in 52 patients. Irradiation was administered, from a 4-15 MeV linear accelerator, via a three-field technique, to a total dose of 4500 cGy (rad) in 25 fractions over 35 days. (author)

  10. Comparison of Efficacy of Long?term Oral Treatment with Telmisartan and Benazepril in Cats with Chronic Kidney Disease

    OpenAIRE

    Sent, U.; G?ssl, R.; Elliott, J.; Syme, H. M.; Zimmering, T.

    2015-01-01

    Background The efficacy and benefits of telmisartan in cats with chronic kidney disease (CKD) have not previously been reported. Hypothesis Long?term treatment of cats with CKD using telmisartan decreases urine protein?to?creatinine ratio (UP/C) similar to benazepril. Animals Two?hundred and twenty?four client?owned adult cats with CKD. Methods Prospective, multicenter, controlled, randomized, parallel group, blinded clinical trial with noninferiority design. Cats were allocated in a 1 : 1 ra...

  11. Heart diseases and long-term risk of dementia and Alzheimer's disease: a population-based CAIDE study.

    Science.gov (United States)

    Rusanen, Minna; Kivipelto, Miia; Levälahti, Esko; Laatikainen, Tiina; Tuomilehto, Jaakko; Soininen, Hilkka; Ngandu, Tiia

    2014-01-01

    Many cardiovascular risk factors are shown to increase the risk of dementia and Alzheimer's disease (AD), but the impact of heart disease on later development of dementia is still unclear. The aim of the study was to investigate the long-term risk of dementia and Alzheimer's disease (AD) related to midlife and late-life atrial fibrillation (AF), heart failure (HF), and coronary artery disease (CAD) in a population-based study with a follow-up of over 25 years. Cardiovascular Risk Factors, Aging and Dementia (CAIDE) study includes 2000 participants who were randomly selected from four separate, population-based samples originally studied in midlife (1972, 1977, 1982, or 1987). Re-examinations were carried out in 1998 and 2005-2008. Altogether 1,510 (75.5%) persons participated in at least one re-examination, and 127 (8.4%) persons were diagnosed with dementia (of which 102 had AD). AF in late-life was an independent risk factor for dementia (HR 2.61, 95% CI 1.05-6.47; p = 0.039) and AD (HR 2.54, 95% CI 1.04-6.16; p = 0.040) in the fully adjusted analyses. The association was even stronger among the apolipoprotein E (APOE) ε4 non-carriers. Late-life HF, but not CAD, tended to increase the risks as well. Heart diseases diagnosed at midlife did not increase the risk of later dementia and AD. Late-life heart diseases increase the subsequent risk of dementia and AD. Prevention and effective treatment of heart diseases may be important also from the perspective of brain health and cognitive functioning.

  12. Long-term functional outcome after surgical repair of cranial cruciate ligament disease in dogs.

    Science.gov (United States)

    Mölsä, Sari H; Hyytiäinen, Heli K; Hielm-Björkman, Anna K; Laitinen-Vapaavuori, Outi M

    2014-11-19

    Cranial cruciate ligament (CCL) rupture is a very common cause of pelvic limb lameness in dogs. Few studies, using objective and validated outcome evaluation methods, have been published to evaluate long-term (>1 year) outcome after CCL repair. A group of 47 dogs with CCL rupture treated with intracapsular, extracapsular, and osteotomy techniques, and 21 healthy control dogs were enrolled in this study. To evaluate long-term surgical outcome, at a minimum of 1.5 years after unilateral CCL surgery, force plate, orthopedic, radiographic, and physiotherapeutic examinations, including evaluation of active range of motion (AROM), symmetry of thrust from the ground, symmetry of muscle mass, and static weight bearing (SWB) of pelvic limbs, and goniometry of the stifle and tarsal joints, were done. At a mean of 2.8 ± 0.9 years after surgery, no significant differences were found in average ground reaction forces or SWB between the surgically treated and control dog limbs, when dogs with no other orthopedic findings were included (n = 21). However, in surgically treated limbs, approximately 30% of the dogs had decreased static or dynamic weight bearing when symmetry of weight bearing was evaluated, 40-50% of dogs showed limitations of AROM in sitting position, and two-thirds of dogs had weakness in thrust from the ground. The stifle joint extension angles were lower (P <0.001) and flexion angles higher (P <0.001) in surgically treated than in contralateral joints, when dogs with no contralateral stifle problems were included (n = 33). In dogs treated using the intracapsular technique, the distribution percentage per limb of peak vertical force (DPVF) in surgically treated limbs was significantly lower than in dogs treated with osteotomy techniques (P =0.044). The average long-term dynamic and static weight bearing of the surgically treated limbs returned to the level of healthy limbs. However, extension and flexion angles of the surgically treated stifles

  13. Short and long-term labour market consequences of coronary heart disease

    DEFF Research Database (Denmark)

    Kruse, Marie; Sørensen, Jan; Davidsen, Michael

    2009-01-01

    National Cohort study and were followed from the year of their first hospital admission for CHD and onwards for up to 23 years. Individuals with CHD were individually matched with individuals without CHD. We analysed their short-term labour market participation and compared the long-term withdrawal risk...... for the two groups through Cox regression. RESULTS: In the year after the first CHD-related admission, 79% of individuals with CHD maintained their labour force participation compared with 93% of individuals without CHD. Individuals with CHD had a hazard ratio of 1.32 for withdrawal compared...... cardiac rehabilitation, which aims to maintain labour market participation. CONCLUSION: Individuals with CHD have a significantly increased risk of withdrawing from the labour market. Especially younger individuals and those employed as manual labour seem to have greater problems in maintaining labour...

  14. [Epidemiologic findings on the spontaneous long-term course of psychogenic disease over 10 years].

    Science.gov (United States)

    Franz, M; Schepank, H; Reister, G; Schellberg, D

    1994-01-01

    207 individuals were selected from a random sample of the adult urban population of Mannheim according to the criterion of medium psychogenic impairment (high-risk population) and investigated three times between 1979 and 1991 with regard to prevalence and severity of psychogenic disorders. In contrast to clinical investigations, the present data render statements on the spontaneous course of psychogenic disorders in the general population. The existing psychogenic impairment was determined by means of various operationalizations (symptomatology, ICD-diagnoses, severity of impairment). The available data indicate a high stability of psychogenic impairment in the spontaneous course. Group statistically the severity of impairment even increases in the long term course. However, different subtypes of course in the investigated high-risk population can be identified by a cluster analysis.

  15. Long-term exposure to ambient air pollution and mortality due to cardiovascular disease and cerebrovascular disease in Shenyang, China.

    Directory of Open Access Journals (Sweden)

    Pengfei Zhang

    Full Text Available BACKGROUND: The relationship between ambient air pollution exposure and mortality of cardiovascular and cerebrovascular diseases in human is controversial, and there is little information about how exposures to ambient air pollution contribution to the mortality of cardiovascular and cerebrovascular diseases among Chinese. The aim of the present study was to examine whether exposure to ambient-air pollution increases the risk for cardiovascular and cerebrovascular disease. METHODOLOGY/PRINCIPAL FINDINGS: We conducted a retrospective cohort study among humans to examine the association between compound-air pollutants [particulate matter <10 µm in aerodynamic diameter (PM(10, sulfur dioxide (SO(2 and nitrogen dioxide (NO(2] and mortality in Shenyang, China, using 12 years of data (1998-2009. Also, stratified analysis by sex, age, education, and income was conducted for cardiovascular and cerebrovascular mortality. The results showed that an increase of 10 µg/m(3 in a year average concentration of PM(10 corresponds to 55% increase in the risk of a death cardiovascular disease (hazard ratio [HR], 1.55; 95% confidence interval [CI], 1.51 to 1.60 and 49% increase in cerebrovascular disease (HR, 1.49; 95% CI, 1.45 to 1.53, respectively. The corresponding figures of adjusted HR (95%CI for a 10 µg/m(3 increase in NO(2 was 2.46 (2.31 to 2.63 for cardiovascular mortality and 2.44 (2.27 to 2.62 for cerebrovascular mortality, respectively. The effects of air pollution were more evident in female that in male, and nonsmokers and residents with BMI<18.5 were more vulnerable to outdoor air pollution. CONCLUSION/SIGNIFICANCE: Long-term exposure to ambient air pollution is associated with the death of cardiovascular and cerebrovascular diseases among Chinese populations.

  16. Long-term exposure to ambient ultrafine particles and respiratory disease incidence in in Toronto, Canada: a cohort study.

    Science.gov (United States)

    Weichenthal, Scott; Bai, Li; Hatzopoulou, Marianne; Van Ryswyk, Keith; Kwong, Jeffrey C; Jerrett, Michael; van Donkelaar, Aaron; Martin, Randall V; Burnett, Richard T; Lu, Hong; Chen, Hong

    2017-06-19

    Little is known about the long-term health effects of ambient ultrafine particles (respiratory disease incidence. In this study, we examined the relationship between long-term exposure to ambient UFPs and the incidence of lung cancer, adult-onset asthma, and chronic obstructive pulmonary disease (COPD). Our study cohort included approximately 1.1 million adults who resided in Toronto, Canada and who were followed for disease incidence between 1996 and 2012. UFP exposures were assigned to residential locations using a land use regression model. Random-effect Cox proportional hazard models were used to estimate hazard ratios (HRs) describing the association between ambient UFPs and respiratory disease incidence adjusting for ambient fine particulate air pollution (PM 2.5 ), NO 2 , and other individual/neighbourhood-level covariates. In total, 74,543 incident cases of COPD, 87,141 cases of asthma, and 12,908 cases of lung cancer were observed during follow-up period. In single pollutant models, each interquartile increase in ambient UFPs was associated with incident COPD (HR = 1.06, 95% CI: 1.05, 1.09) but not asthma (HR = 1.00, 95% CI: 1.00, 1.01) or lung cancer (HR = 1.00, 95% CI: 0.97, 1.03). Additional adjustment for NO 2 attenuated the association between UFPs and COPD and the HR was no longer elevated (HR = 1.01, 95% CI: 0.98, 1.03). PM 2.5 and NO 2 were each associated with increased incidence of all three outcomes but risk estimates for lung cancer were sensitive to indirect adjustment for smoking and body mass index. In general, we did not observe clear evidence of positive associations between long-term exposure to ambient UFPs and respiratory disease incidence independent of other air pollutants. Further replication is required as few studies have evaluated these relationships.

  17. The Prune Belly syndrome: urological aspects and long-term outcomes of a rare disease.

    Science.gov (United States)

    Zugor, Vahudin; Schott, Günter E; Labanaris, Apostolos P

    2012-04-02

    Prune-Belly syndrome is a disorder characterized by the following triad of symptoms: deficiency of the abdominal muscles, malformations of the urinary tract and bilateral cryptorchidism. This study included a total of 16 patients. The findings included clinical characteristics, diagnostics, therapy and long-term clinical outcomes. All patients were asked to complete a questionnaire and, in some cases, were given further examination. All patients were diagnosed with congenital aplasia of the abdominal wall and a variety of urogenital malformations. Cryptorchidism was present in 11 patients (68.8%), malformations of the prostate in 3 (18.8%), urethral malformations in 8 (50%) and mega-ureter in 14 patients (87.5%). A mega-bladder was observed in 13 patients (81.3%). Distinctive renal malformations, such as renal dysplasia, in 3 patients (18.8%) and hydronephrosis in 9 patients (56.3%), respectively. Abdominoplasty was performed on 4 patients (25%). Urethral surgery was performed in 10 patients (62.5%). Seven patients (43.8%) required ureter surgery, most of which involved re-implantation of the ureter and, in some cases, additional ureter modeling. Renal surgery was performed on 5 patients. Four patients with non-functioning kidneys with hydronephrosis underwent a nephrectomy and one patient pyeloplasty. We demonstrate that successful treatment is possible even in cases of serious and complex malformations, such as those of the Prune-Belly syndrome. Treatment must be tailored to the individual patient. The severity of the renal dysplasia is the main prognostic factor.

  18. Bone marrow involvement in Gaucher disease at MRI: what long-term evolution can we expect under enzyme replacement therapy?

    International Nuclear Information System (INIS)

    Fedida, Benjamin; Touraine, Sebastien; Laredo, Jean-Denis; Stirnemann, Jerome; Belmatoug, Nadia; Petrover, David

    2015-01-01

    To study the long-term evolution of the bone marrow burden (BMB) score at MRI in patients with Gaucher disease (GD) under enzyme replacement therapy (ERT). Forty patients treated for GD were retrospectively studied in a referral centre. BMB scores were assessed on spine and femur MR examinations performed between January 2003 and June 2014. The long-term evolution of the BMB scores was analyzed using a linear mixed model. A total of 121 MRI examinations were performed during the study period with a mean follow-up of 7.1 years ± 5.6, an average rate of 3.1 MR examinations ± 1.7 per patient and an interval of 2.3 years ± 1.1 between examinations. Patients had received ERT during 12 years on average ± 6.7. The trend of BMB scores with time decreased significantly by 15 % (P = 0.008) during the total study period and 39 % (P = 0.01) during the first 5 years of treatment. No changes in BMB scores were observed after five years of treatment. In Gaucher patients, the trend of MRI BMB scores with time decreased significantly under ERT the first 5 years of treatment before a long-term stabilization. (orig.)

  19. Effective Treatment of Intestinal Behçet's Disease with Long-Term, Low-Dose Clarithromycin

    Directory of Open Access Journals (Sweden)

    Yukiya Hakozaki

    2013-03-01

    Full Text Available A 51-year-old man was referred for body weight loss and lower right abdominal pain. Total colonoscopy revealed discrete and round ulceration at the ileocecal valve, and he was diagnosed with intestinal Behçet's disease (BD. By treatment with glucocorticoid, colchicine and salazosulfapyridine, the symptoms and ulceration were improved, but cessation of glucocorticoid resulted in relapse of ulceration at the terminal ileum. Long-term, low-dose treatment with clarithromycin (CAM was implemented for chronic respiratory infections. Furthermore, we expected that this CAM treatment would also be effective in BD. During this long-term, low-dose treatment with CAM, discrete ulceration at the terminal ileum was never revealed by follow-up total colonoscopy once or twice per year for 7 years. No reports have described the effectiveness of this treatment in patients with intestinal BD; however, we confirm that long-term treatment with low-dose CAM might have clinical benefits for patients with intestinal BD.

  20. Bone marrow involvement in Gaucher disease at MRI: what long-term evolution can we expect under enzyme replacement therapy?

    Energy Technology Data Exchange (ETDEWEB)

    Fedida, Benjamin; Touraine, Sebastien; Laredo, Jean-Denis [Hopital Lariboisiere, AP-HP, Department of Musculoskeletal Imaging, Paris (France); Stirnemann, Jerome [Universite Paris-Diderot Hopital Bichat, AP-HP, Department of Biostatistics and Medical Data Processing, INSERM UMR 738, Paris (France); Geneva University Hospital, Division of General Internal Medicine, Faculty of Medicine, Geneva (Switzerland); Belmatoug, Nadia [Hopital Beaujon, AP-HP, Referral Center for Lysosomal Diseases (RCLD), Clichy (France); Hopital Beaujon, AP-HP, Department of Internal Medicine, Clichy (France); Petrover, David [Hopital Lariboisiere, AP-HP, Department of Musculoskeletal Imaging, Paris (France); Hopital Beaujon, AP-HP, Referral Center for Lysosomal Diseases (RCLD), Clichy (France)

    2015-10-15

    To study the long-term evolution of the bone marrow burden (BMB) score at MRI in patients with Gaucher disease (GD) under enzyme replacement therapy (ERT). Forty patients treated for GD were retrospectively studied in a referral centre. BMB scores were assessed on spine and femur MR examinations performed between January 2003 and June 2014. The long-term evolution of the BMB scores was analyzed using a linear mixed model. A total of 121 MRI examinations were performed during the study period with a mean follow-up of 7.1 years ± 5.6, an average rate of 3.1 MR examinations ± 1.7 per patient and an interval of 2.3 years ± 1.1 between examinations. Patients had received ERT during 12 years on average ± 6.7. The trend of BMB scores with time decreased significantly by 15 % (P = 0.008) during the total study period and 39 % (P = 0.01) during the first 5 years of treatment. No changes in BMB scores were observed after five years of treatment. In Gaucher patients, the trend of MRI BMB scores with time decreased significantly under ERT the first 5 years of treatment before a long-term stabilization. (orig.)

  1. Immunoglobulins in Neonates with Rhesus Hemolytic Disease of the Fetus and Newborn: Long-Term Outcome in a Randomized Trial.

    Science.gov (United States)

    van Klink, Jeanine M M; van Veen, Suzanne J; Smits-Wintjens, Vivianne E H J; Lindenburg, Irene T M; Rijken, Monique; Oepkes, Dick; Lopriore, Enrico

    2016-01-01

    Prophylactic intravenous immunoglobulin (IVIg) does neither reduce the need for exchange transfusion nor the rates of other adverse neonatal outcomes in neonates with rhesus hemolytic disease of the fetus and newborn (rhesus HDFN) according to our randomized controlled trial analysis. Our objective was to assess the long-term neurodevelopmental outcome in the children included in the trial and treated with either IVIg or placebo. All families of the children included in the trial were asked to participate in this follow-up study. The long-term neurodevelopmental outcome in children at least 2 years of age was assessed using standardized tests. The primary outcome was the incidence of neurodevelopmental impairment defined as at least one of the following: cerebral palsy, severe cognitive and/or motor developmental delay (with a test score of less than -2 SD), bilateral deafness or blindness. Sixty-six of the 80 children (82.5%) who had been recruited to the initial randomized controlled trial participated in the follow-up study. The children were assessed at a median age of 4 years (range 2-7). The median cognitive score was 96 (range 68-118) in the IVIg group and 97 (range 66-118) in the placebo group (p = 0.79). There was no difference in the rate of neurodevelopmental impairment between the IVIg and the placebo group [3% (1/34) vs. 3% (1/32); p = 1.00]. The long-term neurodevelopmental outcome in children treated with IVIg was not different from that in children treated with placebo. Standardized long-term follow-up studies with large enough case series and sufficient power are needed to replicate these findings. © 2015 S. Karger AG, Basel.

  2. Factors governing long-term adherence to a gluten-free diet in adult patients with coeliac disease.

    Science.gov (United States)

    Villafuerte-Galvez, J; Vanga, R R; Dennis, M; Hansen, J; Leffler, D A; Kelly, C P; Mukherjee, R

    2015-09-01

    A strict gluten-free diet is the cornerstone of treatment for coeliac disease. Studies of gluten-free diet adherence have rarely used validated instruments. There is a paucity of data on long-term adherence to the gluten-free diet in the adult population. To determine the long-term adherence to the gluten-free diet and potential associated factors in a large coeliac disease referral centre population. We performed a mailed survey of adults with clinically, serologically and histologically confirmed coeliac disease diagnosed ≥5 years prior to survey. The previously validated Celiac Disease Adherence Test was used to determine adherence. Demographic, socio-economic and potentially associated factors were analysed with adherence as the outcome. The response rate was 50.1% of 709 surveyed, the mean time on a gluten-free diet 9.9 ± 6.4 years. Adequate adherence (celiac disease adherence test score 75% of respondents. Perceived cost remains a barrier to adherence. Perceptions of effectiveness of gluten-free diet as well as its knowledge, are potential areas for intervention. © 2015 John Wiley & Sons Ltd.

  3. The Prune Belly syndrome: urological aspects and long-term outcomes of a rare disease

    Directory of Open Access Journals (Sweden)

    Vahudin Zugor

    2012-06-01

    Full Text Available Prune-Belly syndrome is a disorder characterized by the following triad of symptoms: deficiency of the abdominal muscles, malformations of the urinary tract and bilateral cryptorchidism. This study included a total of 16 patients. The findings included clinical characteristics, diagnostics, therapy and long-term clinical outcomes. All patients were asked to complete a questionnaire and, in some cases, were given further examination. All patients were diagnosed with congenital aplasia of the abdominal wall and a variety of urogenital malformations. Cryptorchidism was present in 11 patients (68.8%, malformations of the prostate in 3 (18.8%, urethral malformations in 8 (50% and mega-ureter in 14 patients (87.5%. A mega-bladder was observed in 13 patients (81.3%. Distinctive renal malformations, such as renal dysplasia, in 3 patients (18.8% and hydronephrosis in 9 patients (56.3%, respectively. Abdominoplasty was performed on 4 patients (25%. Urethral surgery was performed in 10 patients (62.5%. Seven patients (43.8% required ureter surgery, most of which involved re-implantation of the ureter and, in some cases, additional ureter modeling. Renal surgery was performed on 5 patients. Four patients with non-functioning kidneys with hydronephrosis underwent a nephrectomy and one patient pyeloplasty. We demonstrate that successful treatment is possible even in cases of serious and complex malformations, such as those of the Prune-Belly syndrome. Treatment must be tailored to the individual patient. The severity of the renal dysplasia is the main prognostic factor.

  4. Soil microbiome characteristics and soilborne disease development associated with long-term potato cropping system practices

    Science.gov (United States)

    Potato cropping system practices substantially affect soil microbial communities and the development of soilborne diseases. Cropping systems incorporating soil health management practices, such as longer rotations, disease-suppressive crops, reduced tillage, and/or organic amendments can potentially...

  5. Long term outcomes following achievement of clinically inactive disease in juvenile idiopathic arthritis: the importance of definition.

    Science.gov (United States)

    Shoop-Worrall, Stephanie Jw; Verstappen, Suzanne Mm; McDonagh, Janet E; Baildam, Eileen; Chieng, Alice; Davidson, Joyce; Foster, Helen; Ioannou, Yiannis; McErlane, Flora; Wedderburn, Lucy R; Thomson, W; Hyrich, Kimme L

    2018-04-12

    Potential targets for treat-to-target strategies in JIA are minimal disease activity (MDA) and clinically inactive disease (CID). Short and long-term outcomes following achievement of MDA and CID on the cJADAS10 and CID on Wallace's preliminary criteria were compared. Children recruited to the Childhood Arthritis Prospective Study, a UK multicentre inception cohort, were selected if recruited prior to January 2011 and diagnosed with oligoarthritis or rheumatoid factor negative or positive polyarthritis. At one year following diagnosis, children were assessed for MDA on the cJADAS10 and CID on both Wallace's preliminary criteria and the cJADAS10. Associations were tested between these disease states and i) functional ability, ii) absence of limited joints, iii) psychosocial health and v) pain at one year and annually to five years. Of 832 children, 70% were female and the majority had oligoarthritis (68%). At one year, 21% had achieved CID according to both definitions, 7% on Wallace's preliminary criteria only, 16% on cJADAS10 only and 56% on neither. Only 10% of children in the entire cohort achieved MDA without also having CID. Achieving either early CID state was associated with greater absence of limited joints. However, only CID on cJADAS10 was associated with improved functional ability and psychosocial health. Achieving CID was superior to MDA in terms of short and long-term pain and the absence of limited joints. CID on the cJADAS10 may be a preferable treatment target to CID on Wallace's preliminary criteria in terms of both feasibility of application and long-term outcomes. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.

  6. Ongoing disease activity and changing categories in a long-term nordic cohort study of juvenile idiopathic arthritis

    DEFF Research Database (Denmark)

    Nordal, Ellen; Zak, Marek; Aalto, Kristiina

    2011-01-01

    ), including biologic medications, were used in 58.0% of the children during the observation period. Ongoing disease activity was mostly mild, but 22.9% developed some JIA-related damage. At the last follow-up, remission off medication was found in 42.4% of the children, 8.9% were in remission on medication...... results underline the need to identify early predictors of outcome, to further improve therapy and to continue long-term follow-up of children with JIA....

  7. Risk factors for mortality and ischemic heart disease in patients with long-term type 1 diabetes

    DEFF Research Database (Denmark)

    Grauslund, Jakob; Mejnert Jørgensen, Trine; Nybo, Mads

    2010-01-01

    AIMS: The purpose of this study is to evaluate the effect of glycemic regulation, dyslipidemia, and renal dysfunction on mortality (all-cause and cardiovascular) and ischemic heart disease (IHD) in a long-term follow-up of a population-based cohort of Danish type 1 diabetic patients with at least......-density lipoprotein cholesterol (inversely), total cholesterol, creatinine, and macroalbuminuria. Furthermore, all markers except macroalbuminuria were associated with IHD. Microalbuminuria at baseline was not related to any of the endpoints. CONCLUSIONS: Glycemic regulation, dyslipidemia, and renal dysfunction were...

  8. Current Standards of Care and Long Term Outcomes for Thalassemia and Sickle Cell Disease.

    Science.gov (United States)

    Chonat, Satheesh; Quinn, Charles T

    2017-01-01

    Thalassemia and sickle cell disease (SCD) are disorders of hemoglobin that affect millions of people worldwide. The carrier states for these diseases arose as common, balanced polymorphisms during human history because they afforded protection against severe forms of malaria. These complex, multisystem diseases are reviewed here with a focus on current standards of clinical management and recent research findings. The importance of a comprehensive, multidisciplinary and lifelong system of care is also emphasized.

  9. Impact of acute kidney injury on long-term mortality and progression to chronic kidney disease among critically ill children

    Directory of Open Access Journals (Sweden)

    Najlaa G. Al-Otaibi

    2017-02-01

    Full Text Available Objectives: To determine the 2-year outcome of acute kidney injury (AKI following admission to pediatric critical care units (PICU. Methods: A retrospective cohort study was conducted between January 2012 and December 2013. We followed 131 children admitted to PICU, King Abdulaziz University Hospital, Jeddah, Kingdom of Saudi Arabia with a diagnosis of AKI, based on pRIFLE (pediatric risk, injury, failure, loss, and end-stage renal disease, for 2 years. During the study period, 46 children died and 38 of survivors completed the follow-up. Factors affecting long-term progression to chronic kidney disease were also evaluated. Results: The 2-year mortality was more than 40%. The main determinant of the 2-year mortality was the pediatric risk of mortality (PRISM score, which increased the risk of mortality by 6% per each one score (adjusted odds ratio, 1.06: 95% confidence interval: 1.00-1.11. By the end of the 2 years, 33% of survivors had reduction in the glomerular filtration rate and proteinuria, and 73% were hypertensive. Patients with more severe renal impairment at admission, based on the pRIFLE criteria, had higher mortality rate. This association, however, was not independent since it was influenced by baseline disease severity (PRISM score. Conclusion: Large proportion of patients admitted to PICU with AKI either died during the first 2 months of follow-up or developed long-term complications. The severity of AKI, however, was not an independent risk factor for mortality.

  10. Long-Term Adult Feline Liver Organoid Cultures for Disease Modeling of Hepatic Steatosis

    NARCIS (Netherlands)

    Kruitwagen, Hedwig S.; Oosterhoff, Loes A.; Vernooij, Ingrid G.W.H.; Schrall, Ingrid M.; van Wolferen, Monique E.; Bannink, Farah; Roesch, Camille; van Uden, Lisa; Molenaar, Martijn R.; Helms, J. Bernd; Grinwis, Guy C.M.; Verstegen, Monique M.A.; van der Laan, Luc J.W.; Huch, Meritxell; Geijsen, Niels; Vries, Robert G.; Clevers, Hans; Rothuizen, Jan; Schotanus, Baukje A.; Penning, Louis C.; Spee, Bart

    2017-01-01

    Hepatic steatosis is a highly prevalent liver disease, yet research is hampered by the lack of tractable cellular and animal models. Steatosis also occurs in cats, where it can cause severe hepatic failure. Previous studies demonstrate the potential of liver organoids for modeling genetic diseases.

  11. Long-term effects of potato cropping system strategies on soilborne diseases and soil microbial communities

    Science.gov (United States)

    Cropping systems incorporating soil health management practices, such as longer rotations, disease-suppressive crops, reduced tillage, and/or organic amendments can substantially affect soil microbial communities, and potentially reduce soilborne potato diseases and increase productivity, but long-t...

  12. Long-term tricuspid valve prosthesis-related complications in patients with congenital heart disease

    NARCIS (Netherlands)

    van Slooten, Ymkje J.; Freling, Hendrik G.; van Melle, Joost P.; Mulder, Barbara J. M.; Jongbloed, Monique R. M.; Ebels, Tjark; Voors, Adriaan A.; Pieper, Petronella G.

    2014-01-01

    In patients with acquired valvar disease, morbidity and mortality rates after tricuspid valve replacement (TVR) are high. However, in adult patients with congenital heart disease, though data concerning outcome after TVR are scarce, even poorer results are suggested in patients with Ebstein anomaly.

  13. Long-term tricuspid valve prosthesis-related complications in patients with congenital heart disease

    NARCIS (Netherlands)

    van Slooten, Ymkje J.; Freling, Hendrik G.; van Melle, Joost P.; Mulder, Barbara J. M.; Jongbloed, Monique R. M.; Ebels, Tjark; Voors, Adriaan A.; Pieper, Petronella G.

    OBJECTIVES: In patients with acquired valvar disease, morbidity and mortality rates after tricuspid valve replacement (TVR) are high. However, in adult patients with congenital heart disease, though data concerning outcome after TVR are scarce, even poorer results are suggested in patients with

  14. Long-term trihexyphenidyl exposure alters neuroimmune response and inflammation in aging rat: relevance to age and Alzheimer's disease.

    Science.gov (United States)

    Huang, Yuqi; Zhao, Zhe; Wei, Xiaoli; Zheng, Yong; Yu, Jianqiang; Zheng, Jianquan; Wang, Liyun

    2016-07-01

    Clinical studies have shown an association between long-term anticholinergic (AC) drug exposure and Alzheimer's disease (AD) pathogenesis, which has been primarily investigated in Parkinson's disease (PD). However, long-term AC exposure as a risk factor for developing neurodegenerative disorders and the exact mechanisms and potential for disease progression remain unclear. Here, we have addressed the issue using trihexyphenidyl (THP), a commonly used AC drug in PD patients, to determine if THP can accelerate AD-like neurodegenerative progression and study potential mechanisms involved. Male Sprague-Dawley rats (SD) were intraperitoneally injected with THP (0.3 and 1.0 mg/kg) or normal saline (NS) for 7 months. Alterations in cognitive and behavioral performance were assessed using the Morris water maze (MWM) and open field tests. After behavior tests, whole genome oligo microarrays, quantitative real-time reverse transcription-polymerase chain reaction (qRT-PCR), immunohistochemistry, and immunofluorescence-confocal were used to investigate the global mechanisms underlying THP-induced neuropathology with aging. Compared with NS controls, the MWM test results showed that THP-treated rats exhibited significantly extended mean latencies during the initial 3 months of testing; however, this behavioral deficit was restored between the fourth and sixth month of MWM testing. The same tendencies were confirmed by MWM probe and open field tests. Gene microarray analysis identified 68 (47 %) upregulated and 176 (53 %) downregulated genes in the "THP-aging" vs. "NS-aging" group. The most significant populations of genes downregulated by THP were the immune response-, antigen processing and presentation-, and major histocompatibility complex (MHC)-related genes, as validated by qRT-PCR. The decreased expression of MHC class I in THP-treated aging brains was confirmed by confocal analysis. Notably, long-term THP treatment primed hippocampal and cortical microglia to

  15. Health effects of long-term exposure to air pollution: An overview of major respiratory and cardiovascular diseases and diabetes

    Directory of Open Access Journals (Sweden)

    Jovanovic-Andersen Zorana

    2012-01-01

    Full Text Available Large number of studies provided convincing evidence for adverse effects of exposure to outdoor air pollution on human health, and served as basis for current USA and EU Air Quality Standards and limit values. Still, new knowledge is emerging, expanding our understanding of vast effects of exposure to air pollution on human health of this ubiquitous exposure affecting millions of people in urban setting. This paper focuses on the studies of health effects of long-term (chronic exposures to air pollution, and includes major chronic and acute diseases in adults and especially elderly, which will present increasing public health burden, due to improving longevity and projected increasing numbers of elderly. The paper gives overview over the most relevant and latest literature presented by different health outcomes: chronic obstructive pulmonary disease, asthma, pneumonia, cardiovascular disease, and diabetes.

  16. Efficacy of long-term intralesional triamcinolone in Morbihan's disease and its possible association with mast cell infiltration.

    Science.gov (United States)

    Tsiogka, Aikaterini; Koller, Josef

    2018-04-23

    Morbihan's disease is characterized by chronic persistent facial edema of the upper half of the face, absence of typical diagnostic findings, and refractoriness to treatment. A 44-year-old man was diagnosed with Morbihan's disease based on clinical signs and histopathology, which showed dermal edema in upper dermis, discrete lymphocytic infiltrate without granulomatous reaction, and mast cell infiltration. After long-term therapy with intralesional triamcinolone a remarkable objective and subjective clinical response was observed. Reported cases of Morbihan's disease are reviewed, with respect to their treatment and histopathological findings. Mast cell infiltration has been observed on histopathology in most patients who responded to intralesional triamcinolone, suggesting a possible marker of response. The long-lasting response seen in our case indicates the efficacy of intralesional triamcinolone in this rare condition. © 2018 Wiley Periodicals, Inc.

  17. The long-term results of resection and multiple resections in Crohn's disease.

    Science.gov (United States)

    Krupnick, A S; Morris, J B

    2000-01-01

    Crohn's disease is a panenteric, transmural inflammatory disease of unknown origin. Although primarily managed medically, 70% to 90% of patients will require surgical intervention. Surgery for small bowel Crohn's is usually necessary for unrelenting stenotic complications of the disease. Fistula, abscess, and perforation can also necessitate surgical intervention. Most patients benefit from resection or strictureplasty with an improved quality of life and remission of disease, but recurrence is common and 33% to 82% of patients will need a second operation, and 22% to 33% will require more than two resections. Short-bowel syndrome is unavoidable in a small percentage of Crohn's patients because of recurrent resection of affected small bowel and inflammatory destruction of the remaining mucosa. Although previously a lethal and unrelenting disease with death caused by malnutrition, patients with short-bowel syndrome today can lead productive lives with maintenance on total parenteral nutrition (TPN). This lifestyle, however, does not come without a price. Severe TPN-related complications, such as sepsis of indwelling central venous catheters and liver failure, do occur. Future developments will focus on more powerful and effective anti-inflammatory medication specifically targeting the immune mechanisms responsible for Crohn's disease. Successful medical management of the disease will alleviate the need for surgical resection and reduce the frequency of short-bowel syndrome. Improving the efficacy of immunosuppression and the understanding of tolerance induction should increase the safety and applicability of small-bowel transplant for those with short gut. Tissue engineering offers the potential to avoid immunosuppression altogether and supplement intestinal length using the patient's own tissues.

  18. Importance of Collateralization in Patients With Large Artery Intracranial Occlusive Disease: Long-Term Longitudinal Assessment of Cerebral Hemodynamic Function

    Directory of Open Access Journals (Sweden)

    Larissa McKetton

    2018-04-01

    Full Text Available Patients with large artery intracranial occlusive disease (LAICOD are at risk for both acute ischemia and chronic hypoperfusion. Collateral circulation plays an important role in prognosis, and imaging plays an essential role in diagnosis, treatment planning, and prognosis of patients with LAICOD. In addition to standard structural imaging, assessment of cerebral hemodynamic function is important to determine the adequacy of collateral supply. Among the currently available methods of assessment of cerebral hemodynamic function, measurement of cerebrovascular reactivity (CVR using blood oxygen level-dependent (BOLD MRI and precisely controlled CO2 has shown to be a safe, reliable, reproducible, and clinically useful method for long-term assessment of patients. Here, we report a case of long-term follow-up in a 28-year-old Caucasian female presented to the neurology clinic with a history of TIAs and LAICOD of the right middle cerebral artery (MCA. Initial structural MRI showed a right MCA stenosis and a small right coronal radiate lacunar infarct. Her CVR study showed a large area of impaired CVR with a paradoxical decrease in BOLD signal with hypercapnia involving the right MCA territory indicating intracerebral steal. The patient was managed medically with anticoagulant and antiplatelet therapy and was followed-up for over 9 years with both structural and functional imaging. Cortical thickness (CT measures were longitudinally assessed from a region of interest that was applied to subsequent time points in the cortical region exhibiting steal physiology and in the same region of the contralateral healthy hemisphere. In the long-term follow-up, the patient exhibited improvement in her CVR as demonstrated by the development of collaterals with negligible changes to CT. Management of patients with LAICOD remains challenging since no revascularization strategies have shown efficacy except in patients with moyamoya disease. Management is well

  19. Importance of Collateralization in Patients With Large Artery Intracranial Occlusive Disease: Long-Term Longitudinal Assessment of Cerebral Hemodynamic Function.

    Science.gov (United States)

    McKetton, Larissa; Venkatraghavan, Lakshmikumar; Poublanc, Julien; Sobczyk, Olivia; Crawley, Adrian P; Rosen, Casey; Silver, Frank L; Duffin, James; Fisher, Joseph A; Mikulis, David J

    2018-01-01

    Patients with large artery intracranial occlusive disease (LAICOD) are at risk for both acute ischemia and chronic hypoperfusion. Collateral circulation plays an important role in prognosis, and imaging plays an essential role in diagnosis, treatment planning, and prognosis of patients with LAICOD. In addition to standard structural imaging, assessment of cerebral hemodynamic function is important to determine the adequacy of collateral supply. Among the currently available methods of assessment of cerebral hemodynamic function, measurement of cerebrovascular reactivity (CVR) using blood oxygen level-dependent (BOLD) MRI and precisely controlled CO 2 has shown to be a safe, reliable, reproducible, and clinically useful method for long-term assessment of patients. Here, we report a case of long-term follow-up in a 28-year-old Caucasian female presented to the neurology clinic with a history of TIAs and LAICOD of the right middle cerebral artery (MCA). Initial structural MRI showed a right MCA stenosis and a small right coronal radiate lacunar infarct. Her CVR study showed a large area of impaired CVR with a paradoxical decrease in BOLD signal with hypercapnia involving the right MCA territory indicating intracerebral steal. The patient was managed medically with anticoagulant and antiplatelet therapy and was followed-up for over 9 years with both structural and functional imaging. Cortical thickness (CT) measures were longitudinally assessed from a region of interest that was applied to subsequent time points in the cortical region exhibiting steal physiology and in the same region of the contralateral healthy hemisphere. In the long-term follow-up, the patient exhibited improvement in her CVR as demonstrated by the development of collaterals with negligible changes to CT. Management of patients with LAICOD remains challenging since no revascularization strategies have shown efficacy except in patients with moyamoya disease. Management is well defined for acute

  20. Interstitial lung disease caused by TS-1: a case of long-term drug retention as a fatal adverse reaction.

    Science.gov (United States)

    Park, Joong-Min; Hwang, In Gyu; Suh, Suk-Won; Chi, Kyong-Choun

    2011-12-01

    TS-1 is an oral anti-cancer agent for gastric cancer with a high response rate and low toxicity. We report a case of long-term drug retention of TS-1 causing interstitial lung disease (ILD) as a fatal adverse reaction. A 65-year-old woman underwent a total gastrectomy with pathologic confirmation of gastric adenocarcinoma. She received 6 cycles of TS-1 and low-dose cisplatin for post-operative adjuvant chemotherapy followed by single-agent maintenance therapy with TS-1. After 8 months, the patient complained of a productive cough with sputum and mild dyspnea. A pulmonary evaluation revealed diffuse ILD in the lung fields, bilaterally. In spite of discontinuing chemotherapy and the administration of corticosteroids, the pulmonary symptoms did not improve, and the patient died of pulmonary failure. TS-1-induced ILD can be caused by long-term drug retention that alters the lung parenchyma irreversibly, the outcome of which can be life-threatening. Pulmonary evaluation for early detection of disease is recommended.

  1. Long-term Compliance with Oral 5-aminosalicylic Acid Therapy and Risk of Disease Recurrence in Patients with Ulcerative Colitis

    DEFF Research Database (Denmark)

    Prosberg, Michelle V; Vester-Andersen, Marianne K; Andersson, Mikael

    2016-01-01

    BACKGROUND: Noncompliance to long-term medical therapy is a well-known problem among patients treated for ulcerative colitis, but studies of long-term consequences in unselected patients are lacking. The authors aimed to determine the risk of recurrence according to long-term compliance with oral 5...

  2. The Long-Term Consumption of Oats in Celiac Disease Patients Is Safe: A Large Cross-Sectional Study

    Science.gov (United States)

    Aaltonen, Katri; Laurikka, Pilvi; Huhtala, Heini; Mäki, Markku; Kaukinen, Katri; Kurppa, Kalle

    2017-01-01

    A strict gluten-free diet (GFD) can be diversified by non-contaminated oats, but there is a shortage of long-term studies concerning its safety. We compared long-term treatment outcomes and factors associated with the introduction of oats between celiac patients on a GFD with or without oats. Eight hundred sixty-nine previously diagnosed celiac patients were interviewed. The validated Gastrointestinal Symptom Rating Scale (GSRS), Psychological General Well-Being (PGWB), and Short-Form 36 Health Survey (SF-36) questionnaires were used to assess symptoms and quality of life, serological tests were performed, and results of histology were confirmed from patient records. We found the median duration of GFD to be 10 years and 82% using oats. Factors predicting the consumption of oats were diagnosis after the year 2000, advice from a dietitian, detection by screening, and mild clinical presentation. Oat consumers and non-consumers did not differ in dietary adherence (96.5% vs. 97.4%, p = 0.746), the prevalence of symptoms (22.9% vs. 22.5%, p = 0.931), positivity for endomysial antibodies (8.8% vs. 6.0%, p = 0.237), histological recovery after one year (63.1% vs. 60.0%, p = 0.773), malignancy (4.8% vs. 3.3%, p = 0.420), osteoporosis/osteopenia (9.2% vs. 11.0%, p = 0.489), or fractures (26.9% vs. 27.9%, p = 0.791). The oat consumers had better SF-36 physical role limitations and general health scores. Based on our results, the long-term consumption of oats in celiac disease patients is safe and may improve quality of life. PMID:28617328

  3. Neuropsychological long-term sequelae of Ebola virus disease survivors - A systematic review

    NARCIS (Netherlands)

    Lötsch, Felix; Schnyder, Jenny; Goorhuis, Abraham; Grobusch, Martin P.

    2017-01-01

    The recent West African Ebola virus disease (EVD) outbreak had catastrophic impact on populations, health care systems and economies of the affected countries. Somatic symptoms have been reported to persist long beyond the acute infection. This review was conducted to provide an overview on neuro-

  4. LONG-TERM SUCCESS OF AORTOILIAC OPERATION FOR ARTERIOSCLEROTIC OBSTRUCTIVE DISEASE

    NARCIS (Netherlands)

    VANDENAKKER, PJ; VANSCHILFGAARDE, R; BRAND, R; VANBOCKEL, JH; TERPSTRA, JL

    The current retrospective study was performed on 747 patients with aortoiliac obstructive disease who under-went reconstructive operation. Unlike many other centers, the University Hospital Leiden has, throughout the years, maintained the strategy of avoiding the implantation of a prosthesis in

  5. Congenital Diaphragmatic Hernia: Long-term Risk of Gastroesophageal Reflux Disease

    NARCIS (Netherlands)

    Peetsold, Marieke G.; Kneepkens, C. M. F. Frank; Heij, Hugo A.; Ijsselstijn, Hanneke; Tibboel, Dick; Gemke, Reinoud J. B. J.

    2010-01-01

    Objectives: Gastroesophageal reflux disease (GERD) is a well-recognized consequence of congenital diaphragmatic hernia (CDH). Results of studies examining predictive factors for early and late GERD are inconclusive. The aim of this study was to assess the incidence of early ( <2 years) and late GERD

  6. Congenital Diaphragmatic Hernia: Long-term Risk of Gastroesophageal Reflux Disease

    NARCIS (Netherlands)

    Peetsold, M.G.; Kneepkens, C.M.F.; Heij, H.A.; IJsselstijn, H.; Tibboel, D.; Gemke, R.J.B.J.

    2010-01-01

    Objectives: Gastroesophageal reflux disease (GERD) is a well-recognized consequence of congenital diaphragmatic hernia (CDH). Results of studies examining predictive factors for early and lateGERDare inconclusive. The aim of this study was to assess the incidence of early (<2 years) and late GERD

  7. Similar long-term overall and disease-free survival after conventional and extralevator abdominoperineal excision

    DEFF Research Database (Denmark)

    Klein, Mads; Colov, Emilie Palmgren; Gögenur, Ismail

    2016-01-01

    , respectively, (log-rank p = 0.59)). In Cox regression, the type of procedure did not affect DFS or OS. Factors of importance for DFS included increasing age, ypN-positive disease and neoadjuvant chemoradiation therapy. Factors of importance for OS included increasing age, circumferential resection margin (CRM...

  8. Long-term outcome in levothyroxine treated patients with subclinical hypothyroidism and concomitant heart disease

    DEFF Research Database (Denmark)

    Andersen, Mette Nygaard; Olsen, Anne-Marie Schjerning; Madsen, Jesper Clausager

    2016-01-01

    Context: Subclinical hypothyroidism is a common condition that may lead to impaired cardiac function. Objective: This study sought to examine the effects of levothyroxine treatment in patients with subclinical hypothyroidism and heart disease. Design: This was a register-based historical cohort s...

  9. Nonalcoholic fatty liver disease and fatigue in long-term survivors of childhood-onset craniopharyngioma

    NARCIS (Netherlands)

    Hoffmann, Anika; Bootsveld, Klaus; Gebhardt, Ursel; Daubenbuchel, Anna M. M.; Sterkenburg, Anthe S.; Muller, Hermann L.

    Objective: Hypothalamic obesity in childhood craniopharyngioma (CP) patients carries a high risk for development of metabolic syndrome. In metabolic syndrome, the development of nonalcoholic fatty liver disease (NAFLD) is known. The aim of this study is to detect the risk for NAFLD in

  10. Long-term follow-up of children thought to have temporary brittle bone disease

    Directory of Open Access Journals (Sweden)

    Paterson CR

    2011-06-01

    Full Text Available Colin R Paterson1, Elizabeth A Monk21Department of Medicine (retired, 2School of Accounting and Finance, University of Dundee, Dundee, ScotlandBackground: In addition to nonaccidental injury, a variety of bone disorders may underlie the finding of unexplained fractures in young children. One controversial postulated cause is temporary brittle bone disease, first described in 1990.Methods: Eighty-five patients with fractures showing clinical and radiological features of temporary brittle bone disease were the subject of judicial hearings to determine whether it was appropriate for them to return home. Sixty-three patients did, and follow-up information was available for 61 of these. The mean follow-up period was 6.9 years (range 1–17, median 6.Results: We found that none of the children had sustained any further injuries that were thought to represent nonaccidental injury; no child was re-removed from home. Three children had fractures. In each case there was general agreement that the fractures were accidental. Had the original fractures in these children been the result of nonaccidental injury, it would have been severe and repeated; the average number of fractures was 9.1.Conclusion: The fact that no subsequent suspicious injuries took place after return home is consistent with the view that the fractures were unlikely to have been caused by nonaccidental injury, and that temporary brittle bone disease is a distinctive and identifiable disorder.Keywords: fractures, osteogenesis imperfecta, temporary brittle bone disease, nonaccidental injury

  11. Long-Term Adult Feline Liver Organoid Cultures for Disease Modeling of Hepatic Steatosis

    NARCIS (Netherlands)

    Kruitwagen, H.S. (Hedwig S.); Oosterhoff, L.A. (Loes A.); Vernooij, I.G.W.H. (Ingrid G.W.H.); Schrall, I.M. (Ingrid M.); M.E. van Wolferen (Monique); Bannink, F. (Farah); Roesch, C. (Camille); van Uden, L. (Lisa); Molenaar, M.R. (Martijn R.); J.B. Helms (J. Bernd); G.C.M. Grinwis (Guy C.); M.M.A. Verstegen (Monique); L.J.W. van der Laan (Luc); M. Huch (Meritxell); N. Geijsen (Niels); R.G.J. Vries (Robert); H.C. Clevers (Hans); J. Rothuizen (J.); B.A. Schotanus (Baukje A.); C. Penning (Corine); B. Spee (B.)

    2017-01-01

    textabstractHepatic steatosis is a highly prevalent liver disease, yet research is hampered by the lack of tractable cellular and animal models. Steatosis also occurs in cats, where it can cause severe hepatic failure. Previous studies demonstrate the potential of liver organoids for modeling

  12. Quantifying the benefits of achieving or maintaining long-term low risk profile for cardiovascular disease: The Doetinchem Cohort Study.

    Science.gov (United States)

    Hulsegge, Gerben; Smit, Henriëtte A; van der Schouw, Yvonne T; Daviglus, Martha L; Verschuren, W M Monique

    2015-10-01

    Studies investigating the relation between risk profiles and cardiovascular disease have measured risk at baseline only. We investigated maintenance and changes of risk profiles over time and their potential impact on incident cardiovascular disease. Population-based cohort study. Risk factors were measured at baseline (1987-1991) among 5574 cardiovascular disease-free adults aged 20-59 years. They were classified into four risk categories according to smoking status, presence of diabetes and widely accepted cut-off values for blood pressure, total cholesterol/HDL-ratio and body mass index. Categories were subdivided (maintenance, deterioration, improvement) based on risk factor levels at six and 11 years of follow-up. Multivariable-adjusted hazard ratios (HRs) and 95% confidence intervals (95% CIs) for cardiovascular disease incidence 5-10 years following the risk-change period were fitted using Cox proportional hazards models. Only 12% of participants were low risk at baseline, and only 7% maintained it. Participants who maintained a low risk profile over 11 years had seven times lower risk of cardiovascular disease (HR: 0.14, 95% CI: 0.05-0.41) than participants with long-term high risk profile, whereas those low risk at baseline whose profile deteriorated had three times lower risk (HR: 0.36, 95% CI: 0.18-0.71). Our results suggest that, within each baseline risk profile group, compared with a stable profile, improving profiles may be associated with up to two-fold lower HRs, and deteriorating profiles with about two-fold higher HRs. Our study, using long-term risk profiles, demonstrates the full benefits of low risk profile. These findings underscore the importance of achieving and maintaining low risk from young adulthood onwards. © The European Society of Cardiology 2014.

  13. Graves' disease in children: long-term outcomes of medical therapy.

    Science.gov (United States)

    Rabon, Shona; Burton, Amy M; White, Perrin C

    2016-10-01

    Management options are limited for the treatment of Graves' disease, and there is controversy regarding optimal treatment. We describe the demographic and biochemical characteristics of children with Graves' disease and the outcomes of its management. This is a retrospective study reviewing medical records from 2001 to 2011 at a tertiary-care paediatric hospital. Diagnostic criteria included elevated free T4 and total T3, suppressed TSH, and either positive thyroid-stimulating immunoglobulin or thyroid receptor antibodies or clinical signs suggestive of Graves' disease, for example exophthalmos. Patients were treated with antithyroid drugs (ATD), radioactive iodine, or thyroidectomy. The main outcome measures were remission after medical therapy for at least 6 months and subsequent relapse. A total of 291 children met diagnostic criteria. A total of 62 were male (21%); 117 (40%) were Hispanic, 90 (31%) Caucasian, and 59 (20%) African American. Mean age (±standard deviation) at diagnosis was 12·3 ± 3·8 (range 3-18·5) years. At diagnosis, 268 patients were started on an antithyroid drug and 23 underwent thyroid ablation or thyroidectomy. Fifty-seven (21%) children achieved remission and 16 (28%) of these patients relapsed, almost all within 16 months. Gender and ethnicity did not affect rates of remission or relapse. Of 251 patients treated with methimazole, 53 (21%) had an adverse reaction, including rash, arthralgias, elevated transaminases, or neutropenia. Most children with Graves' disease treated with ATD do not experience remission, but most remissions do not end in relapse. Adverse reactions to methimazole are common but generally mild. © 2016 John Wiley & Sons Ltd.

  14. Long-Term Outcome in Levothyroxine Treated Patients With Subclinical Hypothyroidism and Concomitant Heart Disease.

    Science.gov (United States)

    Andersen, Mette Nygaard; Olsen, Anne-Marie Schjerning; Madsen, Jesper Clausager; Kristensen, Søren Lund; Faber, Jens; Torp-Pedersen, Christian; Gislason, Gunnar H; Selmer, Christian

    2016-11-01

    Subclinical hypothyroidism is a common condition that may lead to impaired cardiac function. This study sought to examine the effects of levothyroxine treatment in patients with subclinical hypothyroidism and heart disease. This was a register-based historical cohort study. The study was composed of Danish primary care patients and hospital outpatients age 18 years and older with established heart disease who were diagnosed with subclinical hypothyroidism in 1997-2011. Patients were stratified according to whether they claimed a subsequent prescription of levothyroxine. Event rates and incidence rate ratios (IRR) were calculated by use of time-dependent multivariable Poisson regression models. Measures included all-cause mortality and major adverse cardiac events (MACEs), defined as cardiovascular death, fatal or nonfatal myocardial infaction and stroke, and all-cause hospital admissions. Of 61 611 patients with a diagnosis of cardiac disease having their first time thyroid function testing, 1192 patients with subclinical hypothyroidism (mean age 73.6 [SD ± 13.3] y, 63.8% female) were included, of whom 136 (11.4%) were treated with levothyroxine. During a median follow-up time of 5.6 y (interquartile range, 6.5 y), 694 (58.2%) patients died. Patients treated with levothyroxine displayed no significantly increased risk of all-cause mortality (adjusted IRR, 1.17; 95% confidence interval [CI], 0.90-1.52), MACE (adjusted IRR, 1.08; 95% CI, 0.80-1.45), or hospital admission (adjusted IRR, 0.94; 95% CI, 0.71-1.24), when compared with patients not treated with levothyroxine. Levothyroxine treatment in patients with subclinical hypothyroidism and heart disease was not associated with a significant benefit nor risk of all-cause mortality, MACE, or hospital admission in this large real-world cohort study.

  15. DISEASE MODIFYING THERAPY AND OUTCOME OF RHEUMATOID ARTHRITIS: RETROSPECTIVE ASSESSMENT OF LONG-TERM RESULTS

    Directory of Open Access Journals (Sweden)

    D. E. Karateyev

    2000-01-01

    Full Text Available Aim of study: To investigate the relation between patterns of basic treatment and the outcome of disease in patients with rheumatoid arthritis (RA. Material and methods: We studied retrospectively two groups of patients with RA (1987 ACR criteria: 1 main group - died (72 patients, mean duration of disease from the onset to death 12.8±0.9 years; 2 control group - alive to 1999 with duration of disease at least 15 years (90 patients, mean follow-up period 19.4±0.47 years. Results: Some patterns of basic treatment were observed: 1 "passive ” strategy - only hydroxychloroquine or sulphasalazine during many years; 2 "inteirupted " treatment - early treatment gold or cytotoxics, but when improvement occurred, therapy with DMARD was interrupted for 1-2 years or more, after that re-started etc.; 3 “emergency” strategy’ - 7-10years of "passive" treatment, after that se\\>ere destruction and/or amyloidosis appeared, and cytotoxics started; 4 typical "pyramid " strategy; 5 "active " strategy - early start of treatment with active DMARD (melotrexate, gold with consecutive change of DMARDs without interruption of basic treatment (very close to "sawtooth ” strategy. The most of died patients were treated with "passive ” and "interrupted" strategies, but the most of patients in control group were treated with "pyramid” and "active" strategies. Conclusion: active strategy of basic treatment has a positive influence on the survival ofpatients with R.A.

  16. Osteomalacia in a patient with Paget's bone disease treated with long-term etidronate.

    Science.gov (United States)

    Hoppé, E; Masson, C; Laffitte, A; Chappard, D; Audran, M

    2012-08-01

    A 93 year-old woman with Paget's disease of bone had been treated with etidronate without interruption during 20 years. The daily dose was usual (5mg/kg/day) but this prescription had never been stopped by her physicians. Two fractures had already occurred in pagetic (right tibia) and non pagetic bones (right fibula) within the last 2 years, and she presented rib fractures, another right tibia fracture and right femur fracture during hospitalization time. X-rays films showed major osteolysis of left ulna and right tibia. Blood samples and technetium bone scan brought no evidence for sarcoma or lytic evolution of the disease. A transiliac bone biopsy on non pagetic bone site confirmed the diagnosis of osteomalacia (increased osteoid parameters), with secondary hyperparathyroidism (hook resorption). In Paget's disease of bone, continuous treatment by etidronate may induce generalized osteomalacia, and increase the risk of fracture in both pagetic and non-pagetic bones. Whereas physicians and pharmaceutical industry try to improve the observance of those drugs, this striking observation also points out that a prescription always needs to be updated. Copyright © 2012 Elsevier Masson SAS. All rights reserved.

  17. Effects of vaccination against viral haemorrhagic disease and myxomatosis on long-term mortality rates of European wild rabbits.

    Science.gov (United States)

    Calvete, C; Estrada, R; Lucientes, J; Osacar, J J; Villafuerte, R

    2004-09-25

    The effects of vaccination against myxomatosis and viral haemorrhagic disease (VHD) on long-term mortality rates in European rabbits (Oryctolagus cuniculus) were studied from 1993 to 1996 by radiotracking a free-living population of wild rabbits. During the three months after immunisation, unvaccinated young rabbits weighing between 180 and 600 g were 13.6 times more likely to die than vaccinated young rabbits. In adult rabbits, vaccination did not significantly decrease mortality, mainly owing to the high proportion of rabbits which had previously been exposed to the antigens of both diseases. Compared with adult rabbits with natural antibodies to VHD, rabbits without these antibodies were 5.2 times more likely to die of VHD during annual outbreaks.

  18. Chronic orbital inflammatory disease and optic neuropathy associated with long-term intranasal cocaine abuse: 2 cases and literature review.

    Science.gov (United States)

    Siemerink, Martin J; Freling, Nicole J M; Saeed, Peerooz

    2017-10-01

    Orbital inflammatory disease and secondary optic neuropathy is a rare but devastating complication of long-term intranasal cocaine abuse. We describe 2 patients with a history of intranasal cocaine consumption who presented with subacute onset of unilateral vision loss from optic neuropathy and limitation of abduction in the affected eye. Magnetic resonance imaging findings included an orbital mass in combination with absent nasal septum and partial destruction of the paranasal sinuses. Biopsies and histopathologic examination of the nasal cavity and the orbital mass revealed chronic inflammation. Both patients were treated with oral corticosteroids, ocular movements completely normalized but no improvement of visual acuity was noted. Intranasal cocaine abuse can cause orbital complications from chronic sinonasal inflammatory disease and these patients are at risk to develop optic neuropathy. Optic neuropathy may be caused by compression, infiltration, or ischaemia.

  19. Infectious Diseases in Older Adults of Long-Term Care Facilities: Update on Approach to Diagnosis and Management.

    Science.gov (United States)

    Jump, Robin L P; Crnich, Christopher J; Mody, Lona; Bradley, Suzanne F; Nicolle, Lindsay E; Yoshikawa, Thomas T

    2018-04-01

    The diagnosis, treatment, and prevention of infectious diseases in older adults in long-term care facilities (LTCFs), particularly nursing facilities, remains a challenge for all health providers who care for this population. This review provides updated information on the currently most important challenges of infectious diseases in LTCFs. With the increasing prescribing of antibiotics in older adults, particularly in LTCFs, the topic of antibiotic stewardship is presented in this review. Following this discussion, salient points on clinical relevance, clinical presentation, diagnostic approach, therapy, and prevention are discussed for skin and soft tissue infections, infectious diarrhea (Clostridium difficile and norovirus infections), bacterial pneumonia, and urinary tract infection, as well as some of the newer approaches to preventive interventions in the LTCF setting. © 2018, Copyright the Authors Journal compilation © 2018, The American Geriatrics Society.

  20. Long term clinical history of an Italian cohort of infantile onset Pompe disease treated with enzyme replacement therapy.

    Science.gov (United States)

    Parini, Rossella; De Lorenzo, Paola; Dardis, Andrea; Burlina, Alberto; Cassio, Alessandra; Cavarzere, Paolo; Concolino, Daniela; Della Casa, Roberto; Deodato, Federica; Donati, Maria Alice; Fiumara, Agata; Gasperini, Serena; Menni, Francesca; Pagliardini, Veronica; Sacchini, Michele; Spada, Marco; Taurisano, Roberta; Valsecchi, Maria Grazia; Di Rocco, Maja; Bembi, Bruno

    2018-02-08

    Enzyme replacement therapy (ERT) has deeply modified the clinical history of Infantile Onset Pompe Disease (IOPD). However, its long-term effectiveness is still not completely defined. Available data shows a close relationship between clinical outcome and patients' cross-reactive immunological status (CRIM), being CRIM-negative status a negative prognostic factor. At the same time limited data are available on the long-term treatment in CRIM-positive infants. A retrospective multicentre observational study was designed to analyse the long-term effectiveness of ERT in IOPD. Thirteen Italian centres spread throughout the country were involved and a cohort of 28 patients (15 females, 13 males, born in the period: February 2002-January 2013) was enrolled. IOPD diagnosis was based on clinical symptoms, enzymatic and molecular analysis. All patients received ERT within the first year of life. Clinical, laboratory, and functional data (motor, cardiac and respiratory) were collected and followed for a median period of 71 months (5 years 11 months). Median age at onset, diagnosis and start of ERT were 2, 3 and 4 months, respectively. CRIM status was available for 24/28 patients: 17/24 (71%) were CRIM-positive. Nineteen patients (67%) survived > 2 years: 4 were CRIM-negative, 14 CRIM-positive and one unknown. Six patients (5 CRIM-positive and one unknown) never needed ventilation support (21,4%) and seven (6 CRIM-positive and one unknown: 25%) developed independent ambulation although one subsequently lost this function. Brain imaging study was performed in 6 patients and showed peri-ventricular white matter abnormalities in all of them. Clinical follow-up confirmed the better prognosis for CRIM-positive patients, though a slow, progressive worsening of motor and/or respiratory functions was detected in 8 patients. These data are the result of the longest independent retrospective study on ERT in IOPD reported so far outside clinical trials. The data obtained

  1. CNS germinoma: disease control and long-term functional outcome for 12 children treated with craniospinal irradiation

    International Nuclear Information System (INIS)

    Merchant, Thomas E.; Sherwood, Scot H.; Mulhern, Raymond K.; Rose, Susan R.; Thompson, Stephen J.; Sanford, Robert A.; Kun, Larry E.

    2000-01-01

    Purpose: To provide evidence that radiation therapy alone in the form of craniospinal irradiation (CSI) and a boost to the primary site of disease provides effective disease control and limited additional morbidity for patients with CNS germinoma. Methods and Materials: Twelve patients with a median age of 12 years (range 9-16 years) with CNS germinoma were treated with CSI (median 25.6 Gy, range 23.4-32 Gy) and a boost to the primary site of disease (50.4 Gy, range 45-54 Gy) between January 1987 and June 1998. All patients were biopsied prior to radiation therapy and none received chemotherapy. No patients were lost to follow-up and the majority had long-term (> 45 month) pre- and postirradiation endocrine and psychology assessment. Results: All 12 patients are alive and no failures have occurred with a median follow-up of 69 months (range 14-143 months). Preirradiation endocrine deficiencies were present in 6 of 6 suprasellar tumors and 1 of 6 pineal tumors; with follow-up there was no substantial difference between age and gender adjusted pre- and postirradiation stature and weight. With long-term follow-up, there were no significant differences between pre- and postirradiation full-scale, verbal, and performance IQ scores. Conclusions: This study confirms the ability of radiation therapy alone to achieve disease control with a high rate of success in pediatric patients and demonstrates that the treatment toxicity faced by these patients may be less than anticipated. Because these patients present with substantial preexisting morbidity at diagnosis and may be of an age where the potential for radiation-related side effects is relatively small, the superiority of treatment alternatives may be difficult to prove

  2. A comparative study of the long-term effect of thyroid arterial embolization with surgical thyroidectomy in treating Graves' disease

    International Nuclear Information System (INIS)

    Tian Jinlin; Chen Shuofei; Du Yahui; Li Chunlei; Wang Wei; Li Yunsong; Guo Yuehui

    2012-01-01

    Objective: To compare long-term (5 years) therapeutic effect of thyroid arterial embolization with surgical thyroidectomy in treating Graves' disease. Methods: A total of 54 patients with Graves' disease, who were encountered during the period from Jan. 2003 to Dec. 2010 and received thyroid arterial embolization treatment, were collected and were used as interventional group. While 40 patients with Graves' disease, who were encountered during the same period and received surgical thyroidectomy, were used as surgical group. The 1-year, 3-year and 5-year cure rates and efficiency rates (improved and cure) were calculated and the results were compared between the two groups. Results: The 1-year, 3-year and 5-year cure rates of the interventional group were 68.52%, 61.18% and 47.88%, respectively. The 1-year, 3-year and 5-year cure rates of the surgical group were 97.50%, 78.54% and 69.48%, respectively. The 1-year, 3-year and 5-year efficiency rates in the interventional group and in the surgical group were 98.15%, 80.48%, 60.90% and 97.50%, 76.42%, 67.60%, respectively. By using Ridit analysis, statistically significant difference in the 1-year, 3-year and 5-year cure rates and efficiency rates existed between the interventional group and the surgical group (P 0.05%). Conclusion: For the treatment of Graves' disease, thyroid arterial embolization has satisfactory short-term and mid-term result, although its long-term result is less effective than that of thyroidectomy. (authors)

  3. Long-term efficacy and tolerability of azilsartan medoxomil/chlorthalidone vs olmesartan medoxomil/hydrochlorothiazide in chronic kidney disease.

    Science.gov (United States)

    Bakris, George L; Zhao, Lin; Kupfer, Stuart; Juhasz, Attila; Hisada, Michie; Lloyd, Eric; Oparil, Suzanne

    2018-04-01

    An open-label, long-term study evaluated safety and tolerability of azilsartan medoxomil/chlorthalidone (AZL-M/CLD) vs olmesartan/hydrochlorothiazide (OLM/HCTZ) in hypertensive participants with stage 3 chronic kidney disease. Initial therapy was AZL-M/CLD 20/12.5 mg (n = 77) or OLM/HCTZ 20/12.5 mg (n = 76), but could be up-titrated (AZL-M/CLD to 40/25 mg; OLM/HCTZ to 40/25 mg [US] or 20/25 mg [Europe]) with other agents added during weeks 4-52. Primary endpoint was proportion of participants with ≥ 1 adverse event (AE) through week 52. Baseline demographics were similar. AEs did not differ between groups (88.3%, AZL-M/CLD vs 76.3%, OLM/HCTZ; P = .058). AZL-M/CLD showed greater systolic BP reductions after initial dosing (P = .037) but not during long-term follow-up (P = .588). A greater proportion of participants up-titrated to the highest dose with OLM/HCTZ (48.7%) vs AZL-M/CLD (29.9%) (P = .021) and were taking additional antihypertensive medications (26.3% vs 16.9%). Both AZL-M/CLD and OLM/HCTZ showed similar efficacy and tolerability. ©2018 Wiley Periodicals, Inc.

  4. Long-term changes in ADAS-cog: what is clinically relevant for disease modifying trials in Alzheimer?

    Science.gov (United States)

    Vellas, B; Andrieu, S; Cantet, C; Dartigues, J F; Gauthier, S

    2007-01-01

    With the development of long-term disease modifying trials, changes in ADAS-Cog at 18 months will rise certainly many questions. We decided to look in the Real.fr study at the links between changes in cognition, ADAS-Cog and function. A total of 346 Alzheimer's patients with ADAS-cog at entry and at 18 months. were eligible for this analysis. These patients were on average 77.44 years old and 254 (72.36%) were women. The great majority lived at home and about 93% were treated with a cholinesterase inhibitor at baseline. Thirty three patients (9%) had a gain of more than 2 points at the ADAS-cog at 18 months (Group I, improvement); 130 (38%) were considered as stable, the reference group (Group II ) characterized by a stability at the ADAS-cog: decline of 2 points to gain of 2 points, 112 subjects (32%) had a moderate decline between 2 and 7 at the ADAScog (Group III) and finally 71 subjects (21%) had a severe impairment more than seven points at the ADAS-cog. A loss of one Basic ADL is certainly highly relevant, and such a change was found at 18 months in more than half of the subjects, which is not surprising for a long-term evolution in mild to moderate AD. An impairment of more than 7 points at the ADAS-cog was found in 21% of the subjects at 18 months and was associated with loss.

  5. What to Do with a Second Chance in Life? Long-Term Experiences of Non-carriers of Huntington's Disease.

    Science.gov (United States)

    Winnberg, Elisabeth; Winnberg, Ulrika; Pohlkamp, Lilian; Hagberg, Anette

    2018-04-07

    Little is known about how people's lives are influenced when going from a 50% risk status of Huntington's disease (HD) to no risk after performing predictive testing. In this study, 20 interviews were conducted to explore the long-term (> 5 years) experiences after receiving predictive test results as a non-carrier of HD. The results showed a broad variety of both positive and negative reactions. The most prominent positive reaction reported was feelings of relief and gratitude, of not carrying the HD mutation for themselves and for their children. Also, the non-carrier status promoted in some individuals' significant life changes such as a wishing to have (more) children, pursuing a career or breaking up from an unhappy relationship. However, negative reactions on their psychological well-being were also described. Some had experienced psychological pressure of needing to do something extraordinary in their lives; others expressed feelings of guilt towards affected or untested siblings, resulting in sadness or clinical depression. The new genetic risk status could generate a need of re-orientation, a process that for some persons took several years to accomplish. The results of the present study show the importance of offering long-term post-result counselling for non-carriers in order to deal with the psychological consequences that may follow predictive testing.

  6. [Musical long-term memory throughout the progression of Alzheimer disease].

    Science.gov (United States)

    Groussard, Mathilde; Mauger, Caroline; Platel, Hervé

    2013-03-01

    In Alzheimer patients with a solid musical background, isolated case-reports have reported the maintenance of remarkable musical abilities despite clear difficulties in their verbal memory and linguistic functions. These reports have encouraged a number of scientists to undertake more systematic studies which would allow a rigorous approach to the analysis of musical memory in Alzheimer patients with no formal musical background. Although restricted in number, the latest data are controversial regarding preserved musical capacities in Alzheimer patients. Our current review of the literature addresses this topic and advances the hypothesis that the processes of musical memory are function of illness progression. In the earlier stages, the majority of evaluations concerned musical episodic memory and suggested a dysfunction of this memory whereas in the moderate and severe stages, musical semantic memory and implicit learning are the majority of investigations and seemed more resistant to Alzheimer disease. In summary, our current review bring to understand the memory circuits involved and highlight the necessity to adapted the investigational tools employed to conform with the severity of the signs and symptoms of progressive Alzheimer disease in order to demonstrate the preserved musical capacities.

  7. Long-term results of stereotactic radiosurgery to the pituitary gland in Cushing's disease

    International Nuclear Information System (INIS)

    Degerblad, M.; Raehn, T.; Bergstrand, G.; Thoren, M.

    1986-01-01

    Gamma radiation from 60 Co delivered with stereotactic technique was given to the pituitary gland in 35 patients, aged 18-65 years, with Cushing's disease. The doses were 70-100 Gy in each single irradiation. The size of the sella turcica was normal in the majority of the patients. The observation time was 3-9 years in 29 patiens. Out of them, 14 (48%) obtained clinical remission and normal urinary cortisol after one irradiation. Eight achieved remission after two to four irradiations. In total, 22 out of 29 patients (76%) obtained remission. In 12 of them remission was obtained in 1 year and in another 10 within 3 years. No recurrences were observed. Improvement was seen in 2 patients after one and three irradiations. Bilateral adrenalectomy was performed in 5 patients owing to unsatisfactory effect of irradiation. Pituitary insufficiency with gonadotropin, thyrotropin or cortocotropin failure was demonstrated in 12 of 22 patients in remission. This occurred 4 months to 7 years after the first irradiation. Another 6 patients were followed less than 3 years after the first irradiation. Two obtained remission after the first treatment, whereas the other 4 improved. Stereotactic pituitary irradiation is suggested as a non-invasive therapeutic alternative in Cushing's disease for example in patients with considerable surgical risk or as a supplement to pituitary microsurgery. (author)

  8. Long-term prognosis of fatty liver: risk of chronic liver disease and death

    DEFF Research Database (Denmark)

    Dam-Larsen, S.; Franzmann, M.; Andersen, I.B.

    2004-01-01

    BACKGROUND AND AIMS: Fatty liver is a common histological finding in human liver biopsy specimens. It affects 10-24% of the general population and is believed to be a marker of risk of later chronic liver disease. The present study examined the risk of development of cirrhotic liver disease...... and the risk of death in a cohort diagnosed with pure fatty liver without inflammation. METHODS: A total of 215 patients who had a liver biopsy performed during the period 1976-1987 were included in the study. The population consisted of 109 non-alcoholic and 106 alcoholic fatty liver patients. Median follow...... up time was 16.7 (0.2-21.9) years in the non-alcoholic and 9.2 (0.6-23.1) years in the alcoholic group. Systematic data collection was carried out by review of all medical records. All members of the study cohort were linked through their unique personal identification number to the National Registry...

  9. Echocardiographic and clinical findings in patients with Fabry disease during long-term enzyme replacement therapy

    DEFF Research Database (Denmark)

    Madsen, Christoffer Valdorff; Bundgaard, Henning; Rasmussen, Åse Krogh

    2017-01-01

    from baseline to follow-up; 30 mm [15-53] vs. 25 mm [3-44], p vs. 1520 mm·ms [550-5740], p within the non-ERT group. During follow-up, cardiac symptoms and use of cardiovascular procedures and -medication increased...... significantly in the ERT group, whereas no differences were observed within the non-ERT group. DISCUSSION: We raise concerns regarding the efficacy and benefit of ERT on cardiac involvement in Fabry disease and stress the need for further research....... and Holter-monitoring. RESULTS: We included 66 patients; 47 patients (27 women) received ERT (ERT group) and 19 patients (15 women) did not (non-ERT group). The groups were followed for a median of 8 [0-12] years and 6 [0-13] years, respectively. Comparison between ERT and non-ERT receiving patients by left...

  10. Long-Term Perseveration in Alzheimer’s Disease: A Case Report

    Directory of Open Access Journals (Sweden)

    Edith V. Sullivan

    1991-01-01

    Full Text Available The most common clinical sign of Alzheimer's disease (AD is progressive memory loss. Presented here is a case of AD who, despite ultimate profound dementia with severe amnesia, showed retention of a perseverative response she developed during 26 encounters, over 4.5 years, with the Brown–Peterson distractor test. From Test 9 onwards, she responded from the first distractor-filled trial with one consonant trigram, appearing in none of the seven test forms given her. At Test 26, she could not repeat heard trigrams yet faithfully responded with her perseverative trigram. The trigram, ostensibly declarative information, apparently became part and parcel of the task's procedure. Although perseveration is a form of impairment probably resulting from Alzheimer pathology involving frontal and parietal cortex, it may also reflect a form of preserved memory, albeit distorted, supported by posterior cortical regions spared in AD.

  11. Long-term preclinical magnetic resonance imaging alterations in sporadic Creutzfeldt-Jakob disease.

    Science.gov (United States)

    Zanusso, Gianluigi; Camporese, Giulia; Ferrari, Sergio; Santelli, Luca; Bongianni, Matilde; Fiorini, Michele; Monaco, Salvatore; Manara, Renzo; Cagnin, Annachiara

    2016-10-01

    An asymptomatic 74-year-old woman, on follow-up for a carotid body tumor, showed magnetic resonance imaging (MRI) focal restricted diffusion confined to the left temporal and occipital cortices. Thirteen months later, diffusion-weighted images revealed a bilateral cortical ribbon sign involving all lobes. After 1 month, the patient developed gait instability and cognitive decline rapidly evolving to severe dementia and death within 3 months. Prion protein gene sequence, molecular, and neuropathological studies confirmed the diagnosis of sporadic Creutzfeldt-Jakob disease (sCJD) MM1 subtype. Here we show the kinetics of MRI changes and prion spreading in preclinical sCJD MM1. Ann Neurol 2016;80:629-632. © 2016 American Neurological Association.

  12. Severe Periodontal Disease Associated with Long-Term Treatment with Intravenous Immunoglobulin

    Directory of Open Access Journals (Sweden)

    Jôice Dias Corrêa

    2014-01-01

    Full Text Available Intravenous immunoglobulin (IVIG is used in the treatment of neuropathy. This case report presents, for the first time, a patient with severe periodontal destruction after chronic therapy with IVIG. The patient reported having extracted his maxillary anterior teeth himself due to high mobility. Clinical examination and radiographic images show a generalized and severe periodontitis. No significant alterations in genetic or microbiological features were observed. The present case suggests that periodontal disease aggravation could be considered a new adverse effect of IVIG therapy. Postulated mechanisms are immune complexes formation, complement activation, and a direct effect in osteoclasts. In conclusion, it is important that patients that will receive IVIG treatment underwent dental evaluation.

  13. Long-term effects of beta-blocker use on lung function in Japanese patients with chronic obstructive pulmonary disease

    Directory of Open Access Journals (Sweden)

    Oda N

    2017-04-01

    Full Text Available Naohiro Oda,1 Nobuaki Miyahara,1,2 Hirohisa Ichikawa,3 Yasushi Tanimoto,4 Kazuhiro Kajimoto,5 Makoto Sakugawa,6 Haruyuki Kawai,7 Akihiko Taniguchi,1 Daisuke Morichika,1 Mitsune Tanimoto,1 Arihiko Kanehiro,1 Katsuyuki Kiura1 1Department of Allergy and Respiratory Medicine, Okayama University Hospital, 2Department of Medical Technology, Okayama University Graduate School of Health Sciences, Okayama, 3Department of Respiratory Medicine, KKR Takamatsu Hospital, Takamatsu, 4Department of Respiratory Medicine, National Hospital Organization Minami-Okayama Medical Center, Okayama, 5Department of Respiratory Medicine, Kobe Red Cross Hospital, Kobe, 6Department of Respiratory Medicine, Okayama Red Cross Hospital, 7Department of Respiratory Medicine, Okayama Saiseikai Hospital, Okayama, Japan Background: Some recent studies have suggested that beta-blocker use in patients with chronic obstructive pulmonary disease (COPD is associated with a reduction in the frequency of acute exacerbations. However, the long-term effects of beta-blocker use on lung function of COPD patients have hardly been evaluated. Patients and methods: We retrospectively reviewed 31 Japanese COPD patients taking beta-blockers for >1 year and 72 patients not taking them. The association between beta-blocker use and the annual change in forced expiratory volume in 1 second (FEV1 was assessed. Results: At baseline, patient demographic characteristics were as follows: 97 males (mean age 67.0±8.2 years; 32 current smokers; and Global Initiative for Chronic Obstructive Lung disease (GOLD stages I: n=26, II: n=52, III: n=19, and IV: n=6. Patients taking beta-blockers exhibited a significantly lower forced vital capacity (FVC, FEV1, and %FVC, and a more advanced GOLD stage. The mean duration of beta-blocker administration was 2.8±1.7 years. There were no differences in the annual change in FEV1 between patients who did and did not use beta-blockers (-7.6±93.5 mL/year vs -4.7±118.9 m

  14. Contemporary Reflections on the Safety of Long-Term Aspirin Treatment for the Secondary Prevention of Cardiovascular Disease

    Science.gov (United States)

    Fanaroff, Alexander C.; Roe, Matthew T.

    2018-01-01

    Aspirin has been the cornerstone of therapy for the secondary prevention treatment of patients with cardiovascular disease since landmark trials were completed in the late 1970s and early 1980s that demonstrated the efficacy of aspirin for reducing the risk of ischemic events. Notwithstanding the consistent benefits demonstrated with apirin for both acute and chronic cardiovascular disease, there are a number of toxicities associated with aspirin that have been showcased by recent long-term clinical trials that have included an aspirin monotherapy arm. As an inhibitor of cyclooxygenase, aspirin impairs gastric mucosal protective mechanisms. Prior trials have shown that up to 15–20% of patients developed gastrointestinal symptoms with aspirin monotherapy and roughly 1% of patients per year had a clinically significant bleeding event, including 1 in 1000 patients who suffered an intracranial or fatal bleed. These risks have been shown to be compounded for patients with acute coronary syndromes (ACS) and those undergoing percutaneous coronary intervention (PCI), who are also treated with other anti-thrombotic agents during the acute care/procedural period, as well as for an extended time period afterwards. Given observations of substantial increases in bleeding rates from many prior long-term clinical trials that have evaluated aspirin together with other oral platelet inhibitors or oral anti-coagulants, the focus of contemporary research has pivoted towards tailored anti-thrombotic regimens that attempt to either shorten the duration of exposure to aspirin or replace aspirin with an alternative anti-thrombotic agent. While these shifts are occurring, the safety profile of aspirin when used for the secondary prevention treatment of patients with established cardiovascular disease deserves further consideration. PMID:27028617

  15. Evolutionary decay and the prospects for long-term disease intervention using engineered insect vectors

    Science.gov (United States)

    2015-01-01

    After a long history of applying the sterile insect technique to suppress populations of disease vectors and agricultural pests, there is growing interest in using genetic engineering both to improve old methods and to enable new methods. The two goals of interventions are to suppress populations, possibly eradicating a species altogether, or to abolish the vector’s competence to transmit a parasite. New methods enabled by genetic engineering include the use of selfish genes toward either goal as well as a variety of killer-rescue systems that could be used for vector competence reduction. This article reviews old and new methods with an emphasis on the potential for evolution of resistance to these strategies. Established methods of population suppression did not obviously face a problem from resistance evolution, but newer technologies might. Resistance to these newer interventions will often be mechanism-specific, and while it is too early to know where resistance evolution will become a problem, it is at least possible to propose properties of interventions that will be more or less effective in blocking resistance evolution. PMID:26160736

  16. A long-term study on the therapeutic effects of radium-224 in Bechterew's disease

    International Nuclear Information System (INIS)

    Schmitt, E.

    1978-01-01

    The article deals with the question of the real extents of the positive effects of Thorium x in Bechterew's disease and the risks which are taken by applying this treatment. 129 out of 200 patients treated in the Frankfurt clinic in the time 1952-1970 were explored with the help of a detailed list of questions. 80 of these patients could make a direct comparison with other treatment methods and stated the Thorium X-treatment to be substantially more efficient. A long-lasting improvement was seen in 52% of the patients. Out of 80 patients who underwent a post-examination, 79.5% felt an improvement directly after the treatment, 75.6% even still 2 years after the treatment. The clinical values and the laboratory examinations, however, did not reveal such a favourable picture. In a comparison group of patients who had not been treated with Thorium X, only 19.9% showed an improvement. Using the usual methods of clinic, lab., and radiology, no radiation damage could be observed. The author is of the opinion that treatment with Thorium X does not bear an increased risk, if compared with medicamentous treatment methods. However, strict indication and exact dosage should be adhered to. (MG) [de

  17. Long term follow-up of patients with Cushing's disease treated by interstitial irradiation

    International Nuclear Information System (INIS)

    Sandler, L.M.; Richards, N.T.; Carr, D.H.; Mashiter, K.; Joplin, G.F.

    1987-01-01

    The first 86 patients with Cushing's disease treated with interstitial irradiation (by needle implantation) as the sole therapy were reviewed. In the 82 patients who were reassessed 1 yr after treatment 63 (77%) achieved remission. This study comprises the outcome and complications in the 54 patients who had a remission and whom we were able to follow. The follow-up period ranged from 3-26 yr (mean, 10.5) from the time of remission. No instance of clinical or radiological relapse has occurred. Of these 54 patients, yttrium-90 alone was used in 32, of whom 12 (37%) required corticosteroid or T4 replacement therapy in a mean time of 3.5 months; in 7 of these 12 we elected to give an ablative dose. Gold-198 alone was used in 15 patients, of whom 7 (47%) developed hypopituitarism in a mean time of 76 months. Both isotopes were used in 7 patients. A diurnal serum cortisol rhythm was found in 28 of the 31 patients who were not receiving corticosteroid therapy. In 5 of the 7 patients with an initially abnormal pituitary fossa, serial radiological studies revealed remodelling in 3. There have been no complications in the last 17 years. Pituitary implantation with yttrium-90 is an effective alternative to transsphenoidal hypophysectomy, with a high remission rate, no recurrence (as yet), no operative complications, and avoidance of hormone replacement in the majority

  18. Primary hydatid disease of the femur: unsuspected and incidental MRI findings with long-term curative results on medical treatment alone

    International Nuclear Information System (INIS)

    Poyanli, A.; Sencer, S.; Akan, K.; Poyanli, O.; Sayrak, H.

    2001-01-01

    This report describes the early magnetic resonance imaging (MRI) findings and long-term follow-up results of albendazole treatment in a 16-year-old girl with primary hydatid disease of the femur diagnosed incidentally during the course of a post-traumatic knee infection. As far as we know, this is the first report of the early MRI findings and long-term outcome of medical treatment in primary hydatid disease of the femur in this age group. (orig.)

  19. Primary hydatid disease of the femur: unsuspected and incidental MRI findings with long-term curative results on medical treatment alone

    Energy Technology Data Exchange (ETDEWEB)

    Poyanli, A.; Sencer, S. [Dept. of Radiology, Istanbul Faculty of Medicine (Turkey); Akan, K.; Poyanli, O. [Dept. of Orthopaedics and Traumatology, Goeztepe SSK Educational Hospital, Istanbul (Turkey); Sayrak, H. [Dept. of Pathology, Goeztepe SSK Educational Hospital, Istanbul (Turkey)

    2001-11-01

    This report describes the early magnetic resonance imaging (MRI) findings and long-term follow-up results of albendazole treatment in a 16-year-old girl with primary hydatid disease of the femur diagnosed incidentally during the course of a post-traumatic knee infection. As far as we know, this is the first report of the early MRI findings and long-term outcome of medical treatment in primary hydatid disease of the femur in this age group. (orig.)

  20. Nonalcoholic fatty liver disease in long-term survivors of childhood-onset craniopharyngioma

    Directory of Open Access Journals (Sweden)

    So Yoon Jung

    2017-09-01

    Full Text Available Purpose Hypothalamic obesity in childhood-onset (CO- craniopharyngioma patients may predispose to nonalcoholic fatty liver disease (NAFLD. This study reviewed the characteristics of NAFLD associated with CO-craniopharyngioma. Methods This study retrospectively reviewed 75 patients who underwent surgery for craniopharyngioma while younger than 15 years of age between 2000 and 2016. Results Elevated aspartate aminotransferase (AST or alanine aminotransferase (ALT above 40 IU/L was observed in 51 of the 75 (68% CO-craniopharyngioma patients. Imaging studies were performed in 32 patients with elevated liver enzymes. The estimated prevalence of NAFLD in CO-craniopharyngioma was 47%. NAFLD was detected in 22 patients (male 59%, 4.3±4.0 years after first surgery. The mean age at the time of the initial operation was 9.1±2.9 years. Six patients (27.3% were diagnosed within 1 year. Among the 19 patients with initial height and weight data, the body mass index (BMI z-score (BMI_Z at the time of diagnosis with NAFLD was 1.37±1.01 (range, -0.75 to 3.18, with 4 patients (18.2% being overweight and 9 (40.9% being obese. BMI_Z increased above BMI_Z at the time of the operation in 13 patients (68.4%. The increment in BMI_Z was 1.13 (range, 0.10–2.84. Seventeen patients did not receive growth hormone. An insulin-like growth factor-I level <3rd percentile was observed in 19 patients. Conclusions NAFLD is common in survivors of CO-craniopharyngioma and may develop earlier. If the ALT or AST is above 40 IU/L, a diagnostic work-up should be started.

  1. Nonalcoholic fatty liver disease in long-term survivors of childhood-onset craniopharyngioma.

    Science.gov (United States)

    Jung, So Yoon; Lee, Yun Jeong; Lee, Hye Jin; Lee, Young Ah; Moon, Jin Soo; Ko, Jae Sung; Yang, Sei Won; Shin, Choong Ho

    2017-09-01

    Hypothalamic obesity in childhood-onset (CO-) craniopharyngioma patients may predispose to nonalcoholic fatty liver disease (NAFLD). This study reviewed the characteristics of NAFLD associated with CO-craniopharyngioma. This study retrospectively reviewed 75 patients who underwent surgery for craniopharyngioma while younger than 15 years of age between 2000 and 2016. Elevated aspartate aminotransferase (AST) or alanine aminotransferase (ALT) above 40 IU/L was observed in 51 of the 75 (68%) CO-craniopharyngioma patients. Imaging studies were performed in 32 patients with elevated liver enzymes. The estimated prevalence of NAFLD in CO-craniopharyngioma was 47%. NAFLD was detected in 22 patients (male 59%, 4.3±4.0 years after first surgery). The mean age at the time of the initial operation was 9.1±2.9 years. Six patients (27.3%) were diagnosed within 1 year. Among the 19 patients with initial height and weight data, the body mass index (BMI) z-score (BMI_Z) at the time of diagnosis with NAFLD was 1.37±1.01 (range, -0.75 to 3.18), with 4 patients (18.2%) being overweight and 9 (40.9%) being obese. BMI_Z increased above BMI_Z at the time of the operation in 13 patients (68.4%). The increment in BMI_Z was 1.13 (range, 0.10-2.84). Seventeen patients did not receive growth hormone. An insulin-like growth factor-I level <3rd percentile was observed in 19 patients. NAFLD is common in survivors of CO-craniopharyngioma and may develop earlier. If the ALT or AST is above 40 IU/L, a diagnostic work-up should be started.

  2. Ginsenoside Rg1 ameliorates hippocampal long-term potentiation and memory in an Alzheimer's disease model.

    Science.gov (United States)

    Li, Fengling; Wu, Xiqing; Li, Jing; Niu, Qingliang

    2016-06-01

    The complex etiopathogenesis of Alzheimer's disease (AD) has limited progression in the identification of effective therapeutic agents. Amyloid precursor protein (APP) and presenilin‑1 (PS1) are always overexpressed in AD, and are considered to be the initiators of the formation of β‑amyloid plaques and the symptoms of AD. In the present study, a transgenic AD model, constructed via the overexpression of APP and PS1, was used to verify the protective effects of ginsenoside Rg1 on memory performance and synaptic plasticity. AD mice (6‑month‑old) were treated via intraperitoneal injection of 0.1‑10 mg/kg ginsenoside Rg1. Long‑term memory, synaptic plasticity, and the levels of AD‑associated and synaptic plasticity‑associated proteins were measured following treatment. Memory was measured using a fear conditioning task and protein expression levels were investigated using western blotting. All the data was analyzed by one-way analysis of variance or t‑test. Following 30 days of consecutive treatment, memory in the AD mouse model was ameliorated in the 10 mg/kg ginsenoside Rg1 treatment group. As demonstrated by biochemical experiments, ginsenoside Rg1 treatment reduced the accumulations of β‑amyloid 1‑42 and phosphorylated (p)‑Tau in the AD model. Additionally, brain-derived neurotrophic factor (BDNF) and p‑TrkB synaptic plasticity‑associated proteins were upregulated following ginsenoside Rg1 application. Correspondingly, long‑term potentiation (LTP) was restored following ginsenoside Rg1 application in the AD mice model. Taken together, ginsenoside Rg1 repaired hippocampal LTP and memory, likely through facilitating the clearance of AD‑associated proteins and through activation of the BDNF‑TrkB pathway. Therefore, ginsenoside Rg1 may be a candidate drug for the treatment of AD.

  3. Long-term impact of subthalamic stimulation on cognitive function in patients with advanced Parkinson's disease.

    Science.gov (United States)

    Acera, M; Molano, A; Tijero, B; Bilbao, G; Lambarri, I; Villoria, R; Somme, J; Ruiz de Gopegui, E; Gabilondo, I; Gomez-Esteban, J C

    2017-07-13

    The aim of this study was to evaluate the effects of deep brain stimulation of the subthalamic nucleus (DBS-SN) on cognitive function in patients with Parkinson's disease (PD) 5 years after surgery. We conducted a prospective study including 50 patients with PD who underwent DBS-SN (62.5% were men; mean age of 62.2±8.2 years; mean progression time of 14.1±6.3 years). All patients were assessed before the procedure and at one year after surgery; 40 patients were further followed up until the 5-year mark. Follow-up assessments included the following neuropsychological tests: Mini-Mental State Examination (MMSE), Mattis Dementia Rating Scale (MDRS), letter-number sequencing of the WAIS-III (WAIS-III-LN), clock-drawing test, Rey auditory verbal learning test (RAVLT), Benton Visual Retention Test (BVRT), Judgment of Line Orientation (JLO) test, FAS Phonemic Verbal Fluency Test, Stroop test, and the Montgomery-Asberg Depression Rating Scale (MADRS). Patients were found to score lower on the MMSE (-0.89%), clock-drawing test (-2.61%), MDRS (-1.72%), and especially phonemic (-13.28%) and sematic verbal fluency tests (-12.40%) at one year after surgery. Delayed recall on the RAVLT worsened one year after the procedure (-10.12%). At 5 years, impairment affected mainly verbal fluency; scores decreased an additional 16.10% and 16.60% in semantic and phonemic verbal fluency, respectively. Moderate decreases were observed in immediate recall (-16.87%), WAIS-III-LN (-16.67%), and JLO test (-11.56%). In our sample, DBS-SN did not result in global cognitive impairment 5 years after surgery. Verbal function was found to be significantly impaired one year after the procedure. Impaired learning and visuospatial function may be attributed to degeneration associated with PD. Copyright © 2017 Sociedad Española de Neurología. Publicado por Elsevier España, S.L.U. All rights reserved.

  4. PROTHROMBOTIC POLYMORPHISMS AND LONG-TERM PROGNOSIS OF PATIENTS WITH STABLE ISCHEMIC HEART DISEASE

    Directory of Open Access Journals (Sweden)

    A. L. Komarov

    2011-01-01

    Full Text Available Aim. To estimate influence of thrombosis associated genetic factors on cardiovascular complications (CVC occurrence in patients with stable ischemic heart disease (IHD on the base of 5-year prospective survey. Material and methods. A total of 503 patients with the mean age of 59.4 years were enrolled into the study. The follow-up period was 5.4 years. Composite endpoint included the following cases of fatal and nonfatal CVC: death, acute coronary syndrome, ischemic stroke/transient ischemic attack, peripheral arterial thrombosis and revascularization of affected vascular system. We determined prevalence and prognostic value of mutations and polymorphisms in genes that encode blood clotting factors (factor V Leiden G1691A, prothrombin G20210A, ß-fibrinogen 455G> A, platelet GPIIIa receptor (C1565T and enzymes involved in homocysteine metabolism (methylentetrahydrofolate reductase  (C667 T MTHFR and A1298C, methionine synthase (MTR A2756G, methionine synthase-reductase (MTRR A66G and transcobalamin (TCN C776G. Results. Overall incidence rate of vascular events made up 31.0%. MTHFR and TCN polymorphisms proved to be significant in regard to cardiovascular risk among all studied genetic indices. Carriage of at least C667 T one MTHFR polymorphic allele increased risk of CVC 1.64 times (95% confidence interval (CI 1.2-2.3, p=0.003. Homozygous carriage of MTHFR 1298 AА and TCN 776 СС “wild” genotypes increased risk of CVC 1.63 times (95% CI 1.2-2.3, р=0.006 and 1.37 times (95% CI 1.001-1.89, р=0.04, respectively. Such genetic variants as MTHFR C667 T/СТ and 1298 AА impacted prognosis only given concomitant decrease in plasma folate level, which was observed in 56.1% of the patients. Conclusion. It can be recommended to test the presence of MTHFR C667 T, MTHFR 1298 AА and TCN 776 СС, and to simultaneously assess folate level in IHD patients in order to clarify risk of unfavorable cardiovascular events.

  5. Long-term survival in patients hospitalized for chronic obstructive pulmonary disease: a prospective observational study in the Nordic countries

    Directory of Open Access Journals (Sweden)

    Gudmundsson G

    2012-09-01

    Full Text Available Gunnar Gudmundsson,1,2 Charlotte Suppli Ulrik,3 Thorarinn Gislason,1,2 Eva Lindberg,4 Eva Brøndum,3 Per Bakke,5 Christer Janson31Department of Respiratory Medicine, Allergy and Sleep, National University Hospital, Reykjavik, Iceland; 2Faculty of Medicine, University of Iceland, Reykjavik, Iceland; 3Department of Respiratory Diseases, Hvidovre Hospital, University of Copenhagen, Copenhagen, Denmark; 4Department of Medical Sciences: Respiratory Medicine and Allergology, Uppsala University, Uppsala, Sweden; 5Haukeland University Hospital, Bergen, NorwayBackground and aim: Mortality rate is high in patients with chronic obstructive pulmonary disease (COPD. Our aim was to investigate long-term mortality and associated risk factors in COPD patients previously hospitalized for a COPD exacerbation.Methods: A total of 256 patients from the Nordic countries were followed for 8.7 ± 0.4 years after the index hospitalization in 2000–2001. Prior to discharge, the St George’s Respiratory Questionnaire was administered and data on therapy and comorbidities were obtained. Information on long-term mortality was obtained from national registries in each of the Nordic countries.Results: In total, 202 patients (79% died during the follow up period, whereas 54 (21% were still alive. Primary cause of death was respiratory (n = 116, cardiovascular (n = 43, malignancy (n = 28, other (n = 10, or unknown (n = 5. Mortality was related to older age, with a hazard risk ratio (HRR of 1.75 per 10 years, lower forced expiratory volume in 1 second (FEV1 (HRR 0.80, body mass index (BMI <20 kg/m2 (HRR 3.21, and diabetes (HRR 3.02. Older age, lower BMI, and diabetes were related to both respiratory and cardiovascular mortality. An association was also found between lower FEV1 and respiratory mortality, whereas mortality was not significantly associated with therapy, anxiety, or depression.Conclusion: Almost four out of five patients died within 9 years following an admission

  6. Long-term effect of inhaled budesonide in mild and moderate chronic obstructive pulmonary disease: a randomised controlled trial

    DEFF Research Database (Denmark)

    Vestbo, Jørgen; Sørensen, T; Lange, Peter

    1999-01-01

    BACKGROUND: Little is known about the long-term efficacy of inhaled corticosteroids in chronic obstructive pulmonary disease (COPD). We investigated the efficacy of inhaled budesonide on decline in lung function and respiratory symptoms in a 3-year placebo-controlled study of patients with COPD....... METHODS: We used a parallel-group, randomised, double-blind, placebo-controlled design in a singlecentre study, nested in a continuing epidemiological survey (the Copenhagen City Heart Study). Inclusion criteria were as follows: no asthma; a ratio of forced expiratory volume in 1 s (FEV1) and vital...... capacity of 0.7 or less; FEV1 which showed no response (budesonide, 800 microg plus 400 microg daily for 6 months followed by 400 microg twice daily for 30 months, or placebo...

  7. Social-occupational rehabilitation of patients with Hodgkin's disease in a long-term period after irradiation

    International Nuclear Information System (INIS)

    Bajsogolov, G.D.; Shishkin, I.P.

    1983-01-01

    Some data on 239 patients with Hodkin disease in a long-term (5-12 yrs.) remission achieved with radiotherapy, have been analysed. The number of married persons and the number of children in a family were less, and the number of divorces equal to indices determined during mass screening of population. The rates of morbidity with a less of capacity to work were consistent with control values. Most of the patients (90.3%) after therapy resumed their work the nature of which was mainly the same (88.6%). In expert medical evaluation of the working capacity there are no reliable criteria. It should proceed from the completeness of remission and the nature of the pathological process

  8. Long-Term Safety of In Utero Exposure to Anti-TNFα Drugs for the Treatment of Inflammatory Bowel Disease

    DEFF Research Database (Denmark)

    Chaparro, M; Verreth, A; Lobaton, T

    2018-01-01

    OBJECTIVES: The long-term safety of exposure to anti-tumor necrosis factor (anti-TNFα) drugs during pregnancy has received little attention. We aimed to compare the relative risk of severe infections in children of mothers with inflammatory bowel disease (IBD) who were exposed to anti-TNFα drugs...... in utero with that of children who were not exposed to the drugs. METHODS: Retrospective multicenter cohort study. Exposed cohort: children from mothers with IBD receiving anti-TNFα medication (with or without thiopurines) at any time during pregnancy or during the 3 months before conception. Non......-exposed cohort: children from mothers with IBD not treated with anti-TNFα agents or thiopurines at any time during pregnancy or the 3 months before conception. The cumulative incidence of severe infections after birth was estimated using Kaplan-Meier curves, which were compared using the log-rank test. Cox...

  9. Determinants and Long-Term Outcomes of Percutaneous Coronary Interventions vs. Surgery for Multivessel Disease According to Clinical Presentation.

    Science.gov (United States)

    Hong, Sung-Jin; Kim, Byeong-Keuk; Shin, Sanghoon; Suh, Yongsung; Kim, Seunghwan; Ahn, Chul-Min; Kim, Jung-Sun; Ko, Young-Guk; Choi, Donghoon; Hong, Myeong-Ki; Jang, Yangsoo

    2018-03-23

    The long-term outcome of percutaneous coronary intervention (PCI) vs. coronary artery bypass graft (CABG), particularly for patients with non-ST-elevation acute coronary syndrome (NSTE-ACS), remains controversial.Methods and Results:We retrospectively analyzed 2,827 patients (stable coronary artery disease [SCAD], n=1,601; NSTE-ACS, n=1,226) who underwent either PCI (n=1,732) or CABG (n=1,095). The 8-year composite of cardiac death and myocardial infarction (MI) was compared between PCI and CABG before and after propensity matching. For patients with NSTE-ACS, PCI was performed more frequently for those with higher Thrombolysis in Myocardial Infarction risk score and 3-vessel disease, and PCI led to significantly higher 8-year composite of cardiac death and MI than CABG (14.1% vs. 5.9%, hazard ratio [HR]=2.22, 95% confidence interval [CI]=1.37-3.58, P=0.001). There was a significant interaction between clinical presentation and revascularization strategy (P-interaction=0.001). However, after matching, the benefit of CABG vs. PCI was attenuated in patients with NSTE-ACS, whereas it was pronounced in those with SCAD. Interactions between clinical presentation and revascularization strategy were not observed (P-interaction=0.574). Although the determinants of PCI vs. CABG in real-world clinical practice differ according to the clinical presentation, a significant interaction between clinical presentation and revascularization strategy was not noted for long-term outcomes. The revascularization strategy for patients with NSTE-ACS can be based on the criteria applied to patients with SCAD.

  10. Critical appraisal of the long-term impact of memantine in treatment of moderate to severe Alzheimer’s disease

    Directory of Open Access Journals (Sweden)

    Umamon Puangthong

    2009-10-01

    Full Text Available Umamon Puangthong, Ging-Yuek Robin HsiungDivision of Neurology, Department of Medicine, University of British Columbia, Vancouver, BC, CanadaAbstract: Alzheimer’s disease is the most common cause of dementia in older adults. The clinical features include progressive memory decline as well as cognitive deficits with executive dysfunction, language, visual perceptual difficulties, apraxia and agnosia. During the moderate to severe stage of the disease, there is a major decline in memory and function, while neuropsychiatric disturbances often emerge and patients become difficult to manage. These distressing symptoms increase caregiver burden and add to the direct costs of care of the patients. Any improvements in patient function and behavioral symptoms can reduce caregiver burden. Memantine has been available for a number of years in Europe and in North America. In this article, we examine the pharmacological rationale for its use, and the current clinical evidence for its efficacy and long-term effectiveness in the management of cognitive and behavioral symptoms in moderate to severe stages of Alzheimer’s disease.Keywords: memantine, Alzheimer’s disease, dementia

  11. Bone marrow mononuclear cell implantation in myocardial laser channels in the ischemic heart disease surgery. Long-term results

    Science.gov (United States)

    Chernyavskiy, Alexander; Fomichev, Alexey; Minin, Stanislav; Nikitin, Nikita

    2017-10-01

    Background: The problem of incomplete myocardial revascularization for diffuse and distal lesions of the myocardium is still relevant. We assessed the clinical and instrumental long-term results of autologous bone marrow cell (BMC) implantation in laser channels in ischemic heart disease with diffuse and distal coronary disease. 35 coronary heart disease (CHD) patients with diffuse and distal coronary disease during coronary artery bypass grafting (CABG) underwent BMC implantation in laser channels. The control group consisted of 29 patients. All patients in this group underwent only CABG. Clinical and instrumental assessment of the method's effect was carried out at two weeks, six months, and six years after surgery. Indirect revascularization showed more significant decreasing of the functional class (FC) New York Heart Association (NYHA), myocardial perfusion and contractility improvement. Autologous BMC implantation in laser channels is an effective method of CHD surgical treatment if it is impossible to perform direct myocardial revascularization. The indirect revascularization effect is formed in the first six months after surgery and remains at the same level for six years.

  12. X11beta rescues memory and long-term potentiation deficits in Alzheimer's disease APPswe Tg2576 mice.

    LENUS (Irish Health Repository)

    Mitchell, Jacqueline C

    2009-12-01

    Increased production and deposition of amyloid beta-protein (Abeta) are believed to be key pathogenic events in Alzheimer\\'s disease. As such, routes for lowering cerebral Abeta levels represent potential therapeutic targets for Alzheimer\\'s disease. X11beta is a neuronal adaptor protein that binds to the intracellular domain of the amyloid precursor protein (APP). Overexpression of X11beta inhibits Abeta production in a number of experimental systems. However, whether these changes to APP processing and Abeta production induced by X11beta overexpression also induce beneficial effects to memory and synaptic plasticity are not known. We report here that X11beta-mediated reduction in cerebral Abeta is associated with normalization of both cognition and in vivo long-term potentiation in aged APPswe Tg2576 transgenic mice that model the amyloid pathology of Alzheimer\\'s disease. Overexpression of X11beta itself has no detectable adverse effects upon mouse behaviour. These findings support the notion that modulation of X11beta function represents a therapeutic target for Abeta-mediated neuronal dysfunction in Alzheimer\\'s disease.

  13. Reduced bone mass and preserved marrow adipose tissue in patients with inflammatory bowel diseases in long-term remission.

    Science.gov (United States)

    Bastos, C M; Araújo, I M; Nogueira-Barbosa, M H; Salmon, C E G; de Paula, F J A; Troncon, L E A

    2017-07-01

    Bone marrow adipose tissue has not been studied in patients with inactive inflammatory bowel disease. We found that these patients have preserved marrow adiposity even with low bone mass. Factors involved in bone loss in active disease may have long-lasting effects but do not seem to affect bone marrow adiposity. Reduced bone mass is known to occur at varying prevalence in patients with inflammatory bowel diseases (IBD) because of inflammation, malnutrition, and steroid therapy. Osteoporosis may develop in these patients as the result of an imbalanced relationship between osteoblasts and adipocytes in bone marrow. This study aimed to evaluate for the first time bone mass and bone marrow adipose tissue (BMAT) in a particular subgroup of IBD patients characterized by long-term, steroid-free remission. Patients with Crohn's disease (CD; N = 21) and ulcerative colitis (UC; N = 15) and controls (C; N = 65) underwent dual X-ray energy absorptiometry and nuclear magnetic resonance spectroscopy of the L3 lumbar vertebra for BMAT assessment. Both the CD and UC subgroups showed significantly higher proportions of patients than controls with Z-score ≤-2.0 at L1-L4 (C 1.54%; CD 19.05%; UC 20%; p = 0.02), but not at other sites. The proportions of CD patients with a T-score ˂-1.0 at the femoral neck (C 18.46%; CD 47.62%; p = 0.02) and total hip (C 16.92%; CD 42.86%; p = 0.03) were significantly higher than among controls. There were no statistically significant differences between IBD patients and controls regarding BMAT at L3 (C 28.62 ± 8.15%; CD 29.81 ± 6.90%; UC 27.35 ± 9.80%; p = 0.67). IBD patients in long-term, steroid-free remission may have a low bone mass in spite of preserved BMAT. These findings confirm the heterogeneity of bone disorders in IBD and may indicate that factors involved in bone loss in active disease may have long-lasting effects on these patients.

  14. Gamma knife radiosurgery in patients with persistent acromegaly or Cushing's disease: long-term risk of hypopituitarism.

    Science.gov (United States)

    Cohen-Inbar, Or; Ramesh, Arjun; Xu, Zhiyuan; Vance, Mary Lee; Schlesinger, David; Sheehan, Jason P

    2016-04-01

    For patient with a recurrent or residual acromegaly or Cushing's disease (CD) after resection, gamma knife radiosurgery (GKRS) is often used. Hypopituitarism is the most common adverse effect after GKRS treatment. The paucity of studies with long-term follow-up has hampered understanding of the latent risks of hypopituitarism in patients with acromegaly or CD. We report the long-term risks of hypopituitarism for patients treated with GKRS for acromegaly or CD. From a prospectively created, IRB-approved database, we identified all patients with acromegaly or CD treated with GKRS at the University of Virginia from 1989 to 2008. Only patients with a minimum endocrine follow-up of 60 months were included. The median follow-up is 159·5 months (60·1-278). Thorough radiological and endocrine assessments were performed immediately before GKRS and at regular follow-up intervals. New onset of hypopituitarism was defined as pituitary hormone deficits after GKRS requiring corresponding hormone replacement. Sixty patients with either acromegaly or CD were included. Median tumour volume at time of GKRS was 1·3 cm(3) (0·3-13·4), and median margin dose was 25 Gy (6-30). GKRS-induced new pituitary deficiency occurred in 58·3% (n = 35) of patients. Growth hormone deficiency was most common (28·3%, n = 17). The actuarial overall rates of hypopituitarism at 3, 5 and 10 years were 10%, 21·7% and 53·3%, respectively. The median time to hypopituitarism was 61 months after GKRS (range, 12-160). Cavernous sinus invasion of the tumour was found to correlate with the occurrence of a new or progressive hypopituitarism after GKRS (P = 0·018). Delayed hypopituitarism increases as a function of time after radiosurgery. Hormone axes appear to vary in terms of radiosensitivity. Patients with adenoma in the cavernous sinus are more prone to develop loss of pituitary function after GKRS. © 2015 John Wiley & Sons Ltd.

  15. Long-term monitoring of an amphibian community after a climate change- and infectious disease-driven species extirpation.

    Science.gov (United States)

    Bosch, Jaime; Fernández-Beaskoetxea, Saioa; Garner, Trenton W J; Carrascal, Luis María

    2018-02-15

    Infectious disease and climate change are considered major threats to biodiversity and act as drivers behind the global amphibian decline. This is, to a large extent, based on short-term studies that are designed to detect the immediate and strongest biodiversity responses to a threatening process. What few long-term studies are available, although typically focused on single species, report outcomes that often diverge significantly from the short-term species responses. Here, we report the results of an 18-year survey of an amphibian community exposed to both climate warming and the emergence of lethal chytridiomycosis. Our study shows that the impacts of infectious disease are ongoing but restricted to two out of nine species that form the community, despite the fact all species can become infected with the fungus. Climate warming appears to be affecting four out of the nine species, but the response of three of these is an increase in abundance. Our study supports a decreasing role of infectious disease on the community, and an increasing and currently positive effect of climate warming. We caution that if the warming trends continue, the net positive effect will turn negative as amphibian breeding habitat becomes unavailable as water bodies dry, a pattern that already may be underway. © 2018 John Wiley & Sons Ltd.

  16. Musical and Verbal Memory in Alzheimer's Disease: A Study of Long-Term and Short-Term Memory

    Science.gov (United States)

    Menard, Marie-Claude; Belleville, Sylvie

    2009-01-01

    Musical memory was tested in Alzheimer patients and in healthy older adults using long-term and short-term memory tasks. Long-term memory (LTM) was tested with a recognition procedure using unfamiliar melodies. Short-term memory (STM) was evaluated with same/different judgment tasks on short series of notes. Musical memory was compared to verbal…

  17. Cardiovascular disease in women with polycystic ovary syndrome at long-term follow-up: a retrospective cohort study.

    Science.gov (United States)

    Wild, S; Pierpoint, T; McKeigue, P; Jacobs, H

    2000-05-01

    Polycystic ovary syndrome (PCOS) is associated with higher prevalence of cardiovascular risk factors but the relative prevalence of cardiovascular disease in women with PCOS has not previously been reported. We have compared cardiovascular mortality and morbidity in middle-aged women previously diagnosed with PCOS and age-matched control women. A retrospective cohort study of women diagnosed with PCOS in the United Kingdom before 1979. Seventy cohort members died before 31 March 1999. Morbidity data were collected from 319 women with PCOS and 1060 age-matched control women. Sixty-one women with PCOS and 63 control women attended a clinical examination. Data were collected from death certificates, general practitioners' records and questionnaires with measurement of cardiovascular risk factors in a subsample of questionnaire respondents. All-cause and cardiovascular mortality in the cohort were similar to women in the general population (standardized mortality ratios (95% CI): 93 (72-117) and 78 (45-124), respectively). Women with PCOS had higher levels of several cardiovascular risk factors: diabetes (P = 0.002) hypertension (P = 0.04), hypercholesterolaemia (P heart disease (CHD) was not significantly more common in women with PCOS (crude OR (95%CI) 1.5 (0.7-2.9)) but the crude OR for cerebrovascular disease was 2.8 (1.1-7.1). At long-term follow-up, a history of nonfatal cerebrovascular disease and cardiovascular risk factors including diabetes are more prevalent among women with polycystic ovary syndrome. Morbidity and mortality from of coronary heart disease among women with polycystic ovary syndrome is not as high as previously predicted. This finding challenges our understanding of the aetiology of coronary heart disease in women.

  18. Long-term Assessment of Post-Treatment Symptoms in Patients With Culture-Confirmed Early Lyme Disease.

    Science.gov (United States)

    Weitzner, Erica; McKenna, Donna; Nowakowski, John; Scavarda, Carol; Dornbush, Rhea; Bittker, Susan; Cooper, Denise; Nadelman, Robert B; Visintainer, Paul; Schwartz, Ira; Wormser, Gary P

    2015-12-15

    Lyme disease patients with erythema migrans are said to have post-treatment Lyme disease symptoms (PTLDS) if there is persistence of subjective symptoms for at least 6 months following antibiotic treatment and resolution of the skin lesion. The purpose of this study was to characterize PTLDS in patients with culture-confirmed early Lyme disease followed for >10 years. Adult patients with erythema migrans with a positive skin or blood culture for Borrelia burgdorferi were enrolled in a prospective study beginning in 1991 and followed up at 6 months and annually thereafter to determine the long-term outcome of this infection. The genotype of the infecting strain of B. burgdorferi was evaluated in subjects with PTLDS. One hundred twenty-eight subjects with culture-confirmed early Lyme disease, of whom 55% were male, were followed for a mean ± SD of 14.98 ± 2.71 years (median = 15 years; range = 11-20 years). Fourteen (10.9%) were regarded as having possible PTLDS, but only 6 (4.7%) had PTLDS documented at their last study visit. Nine (64.3%) had only a single symptom. None of the 6 with PTLDS at their last visit was considered to be functionally impaired by the symptom(s). PTLDS was not associated with a particular genotype of B. burgdorferi. PTLDS may persist for >10 years in some patients with culture-confirmed early Lyme disease. Such long-standing symptoms were not associated with functional impairment or a particular strain of B. burgdorferi. © The Author 2015. Published by Oxford University Press on behalf of the Infectious Diseases Society of America. All rights reserved. For Permissions, please e-mail: journals.permissions@oup.com.

  19. Long-term Levodopa Treatment Accelerates the Circadian Rhythm Dysfunction in a 6-hydroxydopamine Rat Model of Parkinson's Disease

    Institute of Scientific and Technical Information of China (English)

    Si-Yue Li; Ya-Li Wang; Wen-Wen Liu; Dong-Jun Lyu; Fen Wang; Cheng-Jie Mao; Ya-Ping Yang; Li-Fang Hu; Chun-Feng Liu

    2017-01-01

    Background:Parkinson's disease (PD) patients with long-term levodopa (L-DOPA) treatment are suffering from severe circadian dysfunction.However,it is hard to distinguish that the circadian disturbance in patients is due to the disease progression itself,or is affected by L-DOPA replacement therapy.This study was to investigate the role of L-DOPA on the circadian dysfunction in a rat model of PD.Methods:The rat model of PD was constructed by a bilateral striatal injection with 6-hydroxydopamine (6-OHDA),followed by administration of saline or 25 mg/kg L-DOPA for 21 consecutive days.Rotarod test,footprint test,and open-field test were carried out to evaluate the motor function.Striatum,suprachiasmatic nucleus (SCN),liver,and plasma were collected at 6:00,12:00,18:00,and 24:00.Quantitative real-time polymerase chain reaction was used to examine the expression of clock genes.Enzyme-linked immunosorbent assay was used to determine the secretion level of cortisol and melatonin.High-performance liquid chromatography was used to measure the neurotransmitters.Analysis of variance was used for data analysis.Results:L-DOPA alleviated the motor deficits induced by 6-OHDA lesions in the footprint and open-field test (P < 0.01,P < 0.001,respectively).After L-DOPA treatment,Bmal1 decreased in the SCN compared with 6-OHDA group at 12:00 (P < 0.01) and 24:00 (P < 0.001).In the striatum,the expression ofBmal1,Rorα was lower than that in the 6-OHDA group at 18:00 (P < 0.05) and L-DOPA seemed to delay the peak of Per2 to 24:00.In liver,L-DOPA did not affect the rhythmicity and expression of these clock genes (P > 0.05).In addition,the cortisol secretion was increased (P > 0.05),but melatonin was further inhibited after L-DOPA treatment at 6:00 (P < 0.01).Conclusions:In the circadian system of advanced PD rat models,circadian dysfunction is not only contributed by the degeneration of the disease itself but also long-term L-DOPA therapy may further aggravate it.

  20. SPECT myocardial perfusion imaging. Long-term prognostic value in diabetic patients with and without coronary artery disease

    International Nuclear Information System (INIS)

    Koehli, M.; Monbaron, D.; Gaillard, R.C.; Ruiz, J.; Prior, J.O.; Bischof Delaloye, A.; Calcagni, M.L.; Fivaz-Arbane, M.; Stauffer, J.C.

    2006-01-01

    Aim: To determine the long-term prognostic value of SPECT myocardial perfusion imaging (MPI) for the occurrence of cardiovascular events in diabetic patients. Patients, methods: SPECT MPI of 210 consecutive Caucasian diabetic patients were analysed using Kaplan-Meier event-free survival curve and independent predictors were determined by Cox multivariate analyses. Results: Follow-up was complete in 200 (95%) patients with a median period of 3.0 years (0.8-5.0). The population was composed of 114 (57%) men, age 65 ± 10 years, 181 (90.5%) type 2 diabetes mellitus, 50 (25%) with a history of coronary artery disease (CAD) and 98 (49%) presenting chest pain prior to MPI. The prevalence of abnormal MPI was 58%. Patients with a normal MPI had neither cardiac death, nor myocardial infarction, independently of a history of coronary artery disease or chest pain. Among the independent predictors of cardiac death and myocardial infarction, the strongest was abnormal MPI (p 5-fold increase in cardiovascular events. This emphasizes the value of SPECT MPI in predicting and risk-stratifying cardiovascular events in diabetic patients. (orig.)

  1. A Biobank for Long-term and Sustainable Research in the Field of Congenital Heart Disease in Germany

    Directory of Open Access Journals (Sweden)

    Thomas Pickardt

    2016-08-01

    Full Text Available Congenital heart disease (CHD is the most frequent birth defect (0.8%–1% of all live births. Due to the advance in prenatal and postnatal early diagnosis and treatment, more than 90% of these patients survive into adulthood today. However, several mid- and long-term morbidities are dominating the follow-up of these patients. Due to the rarity and heterogeneity of the phenotypes of CHD, multicenter registry-based studies are required. The CHD-Biobank was established in 2009 with the aim to collect DNA from patients and their parents (trios or from affected families, as well as cardiovascular tissues from patients undergoing corrective heart surgery for cardiovascular malformations. Clinical/phenotype data are matched to the International Paediatric and Congenital Cardiac Code (IPCCC and the International Statistical Classification of Diseases and Related Health Problems 10th Revision (ICD-10. The DNA collection currently comprises samples from approximately 4200 participants with a wide range of CHD phenotypes. The collection covers about 430 trios and 120 families with more than one affected member. The cardiac tissue collection comprises 1143 tissue samples from 556 patients after open heart surgery. The CHD-Biobank provides a comprehensive basis for research in the field of CHD with high standards of data privacy, IT management, and sample logistics.

  2. Impact of Early-Life Exposures to Infections, Antibiotics, and Vaccines on Perinatal and Long-term Health and Disease

    Directory of Open Access Journals (Sweden)

    Steven L. Raymond

    2017-06-01

    Full Text Available Essentially, all neonates are exposed to infections, antibiotics, or vaccines early in their lives. This is especially true for those neonates born underweight or premature. In contrast to septic adults and children who are at an increased risk for subsequent infections, exposure to infection during the neonatal period is not associated with an increased risk of subsequent infection and may be paradoxically associated with reductions in late-onset sepsis (LOS in the most premature infants. Perinatal inflammation is also associated with a decreased incidence of asthma and atopy later in life. Conversely, septic neonates are at increased risk of impaired long-term neurodevelopment. While the positive effects of antibiotics in the setting of infection are irrefutable, prolonged administration of broad-spectrum, empiric antibiotics in neonates without documented infection is associated with increased risk of LOS, necrotizing enterocolitis, or death. Vaccines provide a unique opportunity to prevent infection-associated disease; unfortunately, vaccinations have been largely unsuccessful when administered in the first month of life with the exception of vaccines against hepatitis B and tuberculosis. Future vaccines will require the use of novel adjuvants to overcome this challenge. This review describes the influence of infections, antibiotics, and vaccines during the first days of life, as well as the influence on future health and disease. We will also discuss potential immunomodulating therapies, which may serve to train the preterm immune system and reduce subsequent infectious burden without subjecting neonates to the risks accompanied by virulent pathogens.

  3. Modelling the tumour microenvironment in long-term microencapsulated 3D co-cultures recapitulates phenotypic features of disease progression.

    Science.gov (United States)

    Estrada, Marta F; Rebelo, Sofia P; Davies, Emma J; Pinto, Marta T; Pereira, Hugo; Santo, Vítor E; Smalley, Matthew J; Barry, Simon T; Gualda, Emilio J; Alves, Paula M; Anderson, Elizabeth; Brito, Catarina

    2016-02-01

    3D cell tumour models are generated mainly in non-scalable culture systems, using bioactive scaffolds. Many of these models fail to reflect the complex tumour microenvironment and do not allow long-term monitoring of tumour progression. To overcome these limitations, we have combined alginate microencapsulation with agitation-based culture systems, to recapitulate and monitor key aspects of the tumour microenvironment and disease progression. Aggregates of MCF-7 breast cancer cells were microencapsulated in alginate, either alone or in combination with human fibroblasts, then cultured for 15 days. In co-cultures, the fibroblasts arranged themselves around the tumour aggregates creating distinct epithelial and stromal compartments. The presence of fibroblasts resulted in secretion of pro-inflammatory cytokines and deposition of collagen in the stromal compartment. Tumour cells established cell-cell contacts and polarised around small lumina in the interior of the aggregates. Over the culture period, there was a reduction in oestrogen receptor and membranous E-cadherin alongside loss of cell polarity, increased collective cell migration and enhanced angiogenic potential in co-cultures. These phenotypic alterations, typical of advanced stages of cancer, were not observed in the mono-cultures of MCF-7 cells. The proposed model system constitutes a new tool to study tumour-stroma crosstalk, disease progression and drug resistance mechanisms. Copyright © 2015 The Authors. Published by Elsevier Ltd.. All rights reserved.

  4. Fully Implantable Deep Brain Stimulation System with Wireless Power Transmission for Long-term Use in Rodent Models of Parkinson's Disease.

    Science.gov (United States)

    Heo, Man Seung; Moon, Hyun Seok; Kim, Hee Chan; Park, Hyung Woo; Lim, Young Hoon; Paek, Sun Ha

    2015-03-01

    The purpose of this study to develop new deep-brain stimulation system for long-term use in animals, in order to develop a variety of neural prostheses. Our system has two distinguished features, which are the fully implanted system having wearable wireless power transfer and ability to change the parameter of stimulus parameter. It is useful for obtaining a variety of data from a long-term experiment. To validate our system, we performed pre-clinical test in Parkinson's disease-rat models for 4 weeks. Through the in vivo test, we observed the possibility of not only long-term implantation and stability, but also free movement of animals. We confirmed that the electrical stimulation neither caused any side effect nor damaged the electrodes. We proved possibility of our system to conduct the long-term pre-clinical test in variety of parameter, which is available for development of neural prostheses.

  5. Long-term outcome of arterial lesions in Behçet disease: a series of 101 patients.

    Science.gov (United States)

    Saadoun, David; Asli, Bouchra; Wechsler, Bertrand; Houman, Habib; Geri, Guillaume; Desseaux, Kristel; Piette, Jean-Charles; Huong, Du Le Thi; Amoura, Zahir; Salem, Tara Ben; Cluzel, Philippe; Koskas, Fabien; Resche-Rigon, Mathieu; Cacoub, Patrice

    2012-01-01

    The vasculitis of Behçet disease (BD) is distinctive because of involvement of both arteries and veins of all sizes. The concept of vasculo-Behçet disease has been adopted for cases in which vascular manifestations are present and often dominate the clinical features. While venous manifestations are frequent and have been reported in many publications, data regarding arterial lesions in patients with BD are rare and often isolated. In this study, we report the main characteristics, treatment, and long-term outcome of 101 patients with arterial lesions among a cohort of 820 (12.3%) BD patients. Factors that affect prognosis were assessed by multivariate analysis. There were 93 (91.2%) male patients; the median (Q1-Q3) age at diagnosis of BD was 33 (27-41) years. Arterial lesions included aneurysms (47.3%), occlusions (36.5%), stenosis (13.5%), and aortitis (2.7%). Lesions mainly involved the aorta (n = 25) and femoral (n = 23) and pulmonary (n = 21) arteries. Patients with arterial lesions were more frequently male (91.2% vs. 62.4%, respectively; p = 0.017) and had higher rates of venous involvement (80.4% vs. 29.8%, respectively; p < 0.001) compared to patients without arterial manifestations. Thirty-nine (38.6%) patients achieved complete remission. In multivariate analysis, the presence of venous involvement (odds ratio [OR], 0.29; 95% confidence interval [CI], 0.08-1.11) and arterial occlusive lesions (OR, 0.13; 95% CI, 0.01-1.25) were negatively associated with complete remission. The use of immunosuppressants (OR, 3.38; 95% CI, 0.87-13.23) was associated with the occurrence of complete remission. The 20-year survival rate was significantly lower in BD patients with arterial involvement than in those without arterial lesions (73% vs. 89%, respectively; p < 0.0001). In conclusion, the long-term outcome of arterial lesions in BD is poor, especially in the case of occlusive lesions and associated venous involvement. The use of immunosuppressants improved the

  6. Long-Term Health Benefit of Transcatheter Aortic Valve Replacement in Patients With Chronic Lung Disease.

    Science.gov (United States)

    Crestanello, Juan A; Popma, Jeffrey J; Adams, David H; Deeb, G Michael; Mumtaz, Mubashir; George, Barry; Huang, Jian; Reardon, Michael J

    2017-11-27

    This study sought to characterize the long-term effect of chronic lung disease (CLD) on mortality, clinical outcomes, quality of life, and health benefits after transcatheter aortic valve replacement (TAVR) with a self-expanding bioprosthesis. The long-term effect of CLD after TAVR is unknown. Prevalence and severity of CLD was determined at baseline in high- and extreme-risk patients with aortic stenosis from the CoreValve US Pivotal Trial. Clinical outcomes and health status were assessed using the Kansas City Cardiomyopathy Questionnaire overall summary score (KCCQ-OS). A favorable health benefit was defined as alive with a KCCQ-OS ≥60 and stability (CLD was present in 55% (20% mild, 13% moderate, 22% severe) of the 1,030 patients studied. All-cause mortality was higher in patients with moderate and severe CLD at 1 year (19.6% mild, 28.1% moderate, 26.9% severe CLD vs. 19.2% non-CLD; p = 0.030) and 3 years (44.8% mild, 53.0% moderate, 51.9% severe vs. 37.7% non-CLD; p CLD at 1 and 3 years. All patients had a nearly 20-point improvement in KCCQ-OS at 1 and 3 years. However, only 43.3% of patients with CLD had a favorable health benefit at 1 year and 22.5% at 3 years. Moderate and severe CLD increases 1- and 3-year mortality after TAVR. Although functional status and quality of life were improved in CLD at 1 and 3 years after TAVR, a favorable health benefit was only achieved in selected patients. (Safety and Efficacy Study of the Medtronic CoreValve System in the Treatment of Symptomatic Severe Aortic Stenosis in High Risk and Very High Risk Subjects Who Need Aortic Valve Replacement; NCT01240902). Copyright © 2017 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.

  7. Adjuvant IMRT/XELOX radiochemotherapy improves long-term overall- and disease-free survival in advanced gastric cancer

    International Nuclear Information System (INIS)

    Boda-Heggemann, J.; Schneider, V.; Weiss, C.

    2013-01-01

    Purpose: In a retrospective analysis, adjuvant intensity-modulated radiation therapy (IMRT) combined with modern chemotherapy improved advanced gastric cancer survival rates compared to a combination of three-dimensional conformal radiation therapy (3D-CRT) and conventional chemotherapy. We report on the long-term outcomes of two consecutive patient cohorts that were treated with either IMRT and intensive chemotherapy, or 3D-CRT and conventional chemotherapy. Patients and methods: Between 2001 and 2008, 65 consecutive gastric cancer patients received either 3D-CRT (n = 27) or IMRT (n = 38) following tumor resection. Chemotherapy comprised predominantly 5-fluorouracil/folinic acid (5-FU/FA) in the earlier cohort and capecitabine plus oxaliplatin (XELOX) in the latter. The primary endpoints were overall survival (OS) and disease-free survival (DFS). Results: Median OS times were 18 and 43 months in the 3D-CRT and IMRT groups, respectively (p = 0.0602). Actuarial 5-year OS rates were 26 and 47 %, respectively. Within the IMRT group, XELOX gave better results than 5-FU/FA in terms of OS, but this difference was not statistically significant. The primary cause of death in both groups was distant metastasis. Median DFS times were 14 and 35 months in the 3D-CRT and IMRT groups, respectively (p = 0.0693). Actuarial 5-year DFS rates were 22 and 44 %, respectively. Among patients receiving 5-FU/FA, DFS tended to be better in the IMRT group, but this was not statistically significant. A similar analysis for the XELOX group was not possible as 3D-CRT was almost never used to treat these patients. No late toxicity exceeding grade 3 or secondary tumors were observed. Conclusion: After a median follow-up period of over 5 years, OS and DFS were improved in the IMRT/XELOX treated patients compared to the 3D-CRT/5-FU/FA group. Long-term observation revealed no clinical indications of therapy-induced secondary tumors or renal toxicity. (orig.)

  8. Adjuvant IMRT/XELOX radiochemotherapy improves long-term overall- and disease-free survival in advanced gastric cancer

    Energy Technology Data Exchange (ETDEWEB)

    Boda-Heggemann, J.; Schneider, V. [Heidelberg Univ., Universitaetsmedizin Mannheim (Germany). Dept. of Radiation Oncology; Weiss, C. [Heidelberg Univ., Universitaetsmedizin Mannheim (Germany). Dept. of Biomathematics and Medical Statistics] [and others

    2013-05-15

    Purpose: In a retrospective analysis, adjuvant intensity-modulated radiation therapy (IMRT) combined with modern chemotherapy improved advanced gastric cancer survival rates compared to a combination of three-dimensional conformal radiation therapy (3D-CRT) and conventional chemotherapy. We report on the long-term outcomes of two consecutive patient cohorts that were treated with either IMRT and intensive chemotherapy, or 3D-CRT and conventional chemotherapy. Patients and methods: Between 2001 and 2008, 65 consecutive gastric cancer patients received either 3D-CRT (n = 27) or IMRT (n = 38) following tumor resection. Chemotherapy comprised predominantly 5-fluorouracil/folinic acid (5-FU/FA) in the earlier cohort and capecitabine plus oxaliplatin (XELOX) in the latter. The primary endpoints were overall survival (OS) and disease-free survival (DFS). Results: Median OS times were 18 and 43 months in the 3D-CRT and IMRT groups, respectively (p = 0.0602). Actuarial 5-year OS rates were 26 and 47 %, respectively. Within the IMRT group, XELOX gave better results than 5-FU/FA in terms of OS, but this difference was not statistically significant. The primary cause of death in both groups was distant metastasis. Median DFS times were 14 and 35 months in the 3D-CRT and IMRT groups, respectively (p = 0.0693). Actuarial 5-year DFS rates were 22 and 44 %, respectively. Among patients receiving 5-FU/FA, DFS tended to be better in the IMRT group, but this was not statistically significant. A similar analysis for the XELOX group was not possible as 3D-CRT was almost never used to treat these patients. No late toxicity exceeding grade 3 or secondary tumors were observed. Conclusion: After a median follow-up period of over 5 years, OS and DFS were improved in the IMRT/XELOX treated patients compared to the 3D-CRT/5-FU/FA group. Long-term observation revealed no clinical indications of therapy-induced secondary tumors or renal toxicity. (orig.)

  9. Successful long-term control of Cushing’s disease after partial resection of gigantic ACTH-secreting pituitary adenoma

    Directory of Open Access Journals (Sweden)

    Vatroslav Čerina

    2016-03-01

    Full Text Available Only 4-9% of patients with Cushing’s disease (CD harbor pituitary macroadenomas. Clinical and biochemical features of macrocorticotropinomas are poorly understood. Some evidence exist that these tumors presents clinical features more similar to a non-functioning adenomas, being though defined silent corticotropinomas, rather than to ACTH-secreting adenomas. In this paper, we report a case of a 60-year old woman with a history of obesity, arterial hypertension and diabetes mellitus who presented with overt central hypothyroidism. Magnetic resonance imaging disclosed giant pituitary adenoma measuring 50 mm. Endocrinological evaluation confirmed CD: ACTH 50.3 pmol/L, urinary free-cortisol of 739 nmol/24h and cortisol of 639 nmol/L after 1 mg dexamethasone suppression test. Tumor mass was reduced by 50% using purely endoscopic transsphenoidal approach. Thirty-eight months after the partial resection, the patient had well controlled CD: ACTH 20.2 pmol/L, urinary free-cortisol of 238 nmol/24h, cortisol of 105 nmol/L after 1 mg dexamethasone suppression test. To the best of our knowledge, this is the largest ACTH-secreting adenoma ever reported. Our case suggests that tumor size does not necessarily correlate with aggressiveness of CD in patients with macrocorticotropinomas and that long-term control of CD may be achieved albeit incomplete surgical removal. Further studies are needed in order to determine the best treatment option for patients with macrocorticotropinomas.

  10. Embolization of renal arteries before transplantation in patients with polycystic kidney disease: a single institution long-term experience

    Energy Technology Data Exchange (ETDEWEB)

    Petitpierre, F.; Cornelis, F.; Lasserre, A.S.; Tricaud, E.; Le Bras, Y.; Grenier, N. [Pellegrin Hospital, Department of Radiology, Bordeaux (France); Couzi, L.; Merville, P. [Pellegrin Hospital, Department of Nephrology, Bordeaux (France); Combe, C.; Ferriere, J.M. [Pellegrin Hospital, Department of Urology, Bordeaux (France)

    2015-11-15

    We aimed to retrospectively assess the long-term safety and efficacy of embolization of renal arteries (ERA) in patients with polycystic kidney disease (PKD) before renal transplantation. Between January 2008 and November 2013, 82 ERA procedures were performed on 76 kidneys in 73 patients (mean age 53 years, range: 34-72). All patients had terminal-stage PKD and were under dialysis and on the renal transplant waiting list with a temporary contraindication due to excessive renal volume. ERA was considered successful in 89.5 % (68/76) of embolized kidneys, meaning that the temporary contraindication for transplantation could be withdrawn for 65 patients (on average 5.6 months, range: 2.8-24.3, after ERA). Mean volume reduction was 40 (range: 2-69) at 3 months and 59 % (35-86) thereafter (both p < 0.001). Post-embolization syndrome occurred after 15 of 82 procedures (18.3 %). The severe complication rate was 4.9 %. Forty-three (67.7 %) transplantations were successfully conducted after ERA, with a mean follow-up of 26.2 months (range: 1.8-59.5), and the estimated 5-year graft survival rate was 95.3 % [95 % CI: 82.7-98.8]. ERA is a safe and effective alternative to nephrectomy before renal transplantation in patients with PKD. (orig.)

  11. Long-term follow-up study of compensated low-dose 131I therapy for Graves' disease

    International Nuclear Information System (INIS)

    Sridama, V.; McCormick, M.; Kaplan, E.L.; Fauchet, R.; DeGroot, L.J.

    1984-01-01

    We treated 187 patients who had Graves' disease with low-dose radioactive iodide ( 131 I), using a protocol that included a compensation for thyroid size. The incidence of early hypothyroidism (12 per cent) was acceptably low in the first year after 131 I treatment, but we found a cumulative high incidence (up to 76 per cent) at the end of the 11th year. In contrast, the incidence of permanent hypothyroidism was relatively stable in 166 surgically treated patients, increasing from 19 to 27 per cent at the end of 11 years. Among 122 medically treated patients, only 40 per cent entered remission, and hypothyroidism developed in 2 per cent during the same period of follow-up. The long-term incidence of hypothyroidism in our patients treated with low-dose 131 I therapy was much higher than that found in earlier studies using a comparable dose. Our study suggests that it will be difficult to modify therapy with 131 I alone to produce both early control of thyrotoxicosis and a low incidence of hypothyroidism

  12. Long-term neurodevelopmental outcome after intrauterine transfusion for hemolytic disease of the fetus/newborn: the LOTUS study.

    Science.gov (United States)

    Lindenburg, Irene T; Smits-Wintjens, Vivianne E; van Klink, Jeanine M; Verduin, Esther; van Kamp, Inge L; Walther, Frans J; Schonewille, Henk; Doxiadis, Ilias I; Kanhai, Humphrey H; van Lith, Jan M; van Zwet, Erik W; Oepkes, Dick; Brand, Anneke; Lopriore, Enrico

    2012-02-01

    To determine the incidence and risk factors for neurodevelopmental impairment (NDI) in children with hemolytic disease of the fetus/newborn treated with intrauterine transfusion (IUT). Neurodevelopmental outcome in children at least 2 years of age was assessed using standardized tests, including the Bayley Scales of Infant Development, the Wechsler Preschool and Primary Scale of Intelligence, and the Wechsler Intelligence Scale for Children, according to the children's age. Primary outcome was the incidence of neurodevelopmental impairment defined as at least one of the following: cerebral palsy, severe developmental delay, bilateral deafness, and/or blindness. A total of 291 children were evaluated at a median age of 8.2 years (range, 2-17 years). Cerebral palsy was detected in 6 (2.1%) children, severe developmental delay in 9 (3.1%) children, and bilateral deafness in 3 (1.0%) children. The overall incidence of neurodevelopmental impairment was 4.8% (14/291). In a multivariate regression analysis including only preoperative risk factors, severe hydrops was independently associated with neurodevelopmental impairment (odds ratio, 11.2; 95% confidence interval, 1.7-92.7). Incidence of neurodevelopmental impairment in children treated with intrauterine transfusion for fetal alloimmune anemia is low (4.8%). Prevention of fetal hydrops, the strongest preoperative predictor for impaired neurodevelopment, by timely detection, referral and treatment may improve long-term outcome. Copyright © 2012 Mosby, Inc. All rights reserved.

  13. Long-Term Benefits of Smoking Cessation on Gastroesophageal Reflux Disease and Health-Related Quality of Life.

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    Yukie Kohata

    Full Text Available Smoking is associated with gastroesophageal reflux disease (GERD. Varenicline, a nicotinic receptor partial agonist, is used to aid smoking cessation. The purpose of this study was to prospectively examine the long-term benefits of smoking cessation on GERD and health-related quality of life (HR-QOL.Patients treated with varenicline were asked to fill out a self-report questionnaire about their smoking habits, gastrointestinal symptoms, and HR-QOL before and 1 year after smoking cessation. The prevalence of GERD, frequency of symptoms, and HR-QOL scores were compared. We also investigated associations between clinical factors and newly-developed GERD.A total of 141 patients achieved smoking cessation (success group and 50 did not (failure group at 1 year after the treatment. The GERD improvement in the success group (43.9% was significantly higher than that in the failure group (18.2%. The frequency of reflux symptoms significantly decreased only in the success group. There were no significant associations between newly developed GERD and clinical factors including increased body mass index and successful smoking cessation. HR-QOL significantly improved only in the success group.Smoking cessation improved both GERD and HR-QOL. Smoking cessation should be recommended for GERD patients.

  14. Embolization of renal arteries before transplantation in patients with polycystic kidney disease: a single institution long-term experience

    International Nuclear Information System (INIS)

    Petitpierre, F.; Cornelis, F.; Lasserre, A.S.; Tricaud, E.; Le Bras, Y.; Grenier, N.; Couzi, L.; Merville, P.; Combe, C.; Ferriere, J.M.

    2015-01-01

    We aimed to retrospectively assess the long-term safety and efficacy of embolization of renal arteries (ERA) in patients with polycystic kidney disease (PKD) before renal transplantation. Between January 2008 and November 2013, 82 ERA procedures were performed on 76 kidneys in 73 patients (mean age 53 years, range: 34-72). All patients had terminal-stage PKD and were under dialysis and on the renal transplant waiting list with a temporary contraindication due to excessive renal volume. ERA was considered successful in 89.5 % (68/76) of embolized kidneys, meaning that the temporary contraindication for transplantation could be withdrawn for 65 patients (on average 5.6 months, range: 2.8-24.3, after ERA). Mean volume reduction was 40 (range: 2-69) at 3 months and 59 % (35-86) thereafter (both p < 0.001). Post-embolization syndrome occurred after 15 of 82 procedures (18.3 %). The severe complication rate was 4.9 %. Forty-three (67.7 %) transplantations were successfully conducted after ERA, with a mean follow-up of 26.2 months (range: 1.8-59.5), and the estimated 5-year graft survival rate was 95.3 % [95 % CI: 82.7-98.8]. ERA is a safe and effective alternative to nephrectomy before renal transplantation in patients with PKD. (orig.)

  15. Long-term Hyperglycemia Naturally Induces Dental Caries but Not Periodontal Disease in Type 1 and Type 2 Diabetic Rodents.

    Science.gov (United States)

    Nakahara, Yutaka; Ozaki, Kiyokazu; Matsuura, Tetsuro

    2017-11-01

    Periodontal disease (PD) in patients with diabetes is described as the sixth complication of diabetes. We have previously shown that diabetes increases dental caries, and carious inflammation might have a strong effect on the adjacent periodontal tissue in diabetic rodent models. However, the possibility that hyperglycemia may induce PD in diabetic animals could not be completely eliminated. The goal of this study was to confirm the presence of PD in diabetic animal models by preventing carious inflammation with fluoride administration. F344 rats injected with alloxan (type 1 diabetic model) and db/db mice (type 2 diabetic model) were given either tap water alone or tap water containing fluoride. A cariostatic effect of fluoride was evident in the diabetic animals. Meanwhile, fluoride treatment drastically attenuated periodontal inflammation in addition to preventing dental caries. Furthermore, with fluoride treatment, periodontitis was notably nonexistent in the periodontal tissue surrounding the normal molars, whereas the caries-forming process was clearly observed in the teeth that were enveloped with persistent periodontitis, suggesting that enhanced periodontal inflammation might have been derived from the dental caries in the diabetic rodents rather than from the PD. In conclusion, long-term hyperglycemia naturally induces dental caries but not PD in type 1 and type 2 diabetic rodents. © 2017 by the American Diabetes Association.

  16. Long-term disease and economic outcomes of prior authorization criteria for Hepatitis C treatment in Pennsylvania Medicaid.

    Science.gov (United States)

    Kabiri, Mina; Chhatwal, Jagpreet; Donohue, Julie M; Roberts, Mark S; James, A Everette; Dunn, Michael A; Gellad, Walid F

    2017-09-01

    Several highly effective but costly therapies for hepatitis C virus (HCV) are available. As a consequence of their high price, 36 state Medicaid programs limited treatment coverage to patients with more advanced HCV stages. States have only limited information available to predict the long-term impact of these decisions. We adapted a validated hepatitis C microsimulation model to the Pennsylvania Medicaid population to estimate the existing HCV prevalence in Pennsylvania Medicaid and estimate the impact of various HCV drug coverage policies on disease outcomes and costs. Outcome measures included rates of advanced-stage HCV outcomes and treatment and disease costs in both Medicaid and Medicare. We estimated that 46,700 individuals in Pennsylvania Medicaid were infected with HCV in 2015, 33% of whom were still undiagnosed. By expanding treatment to include mild fibrosis stage (Metavir F2), Pennsylvania Medicaid will spend an additional $273 million on medications in the next decade with no substantial reduction in the incidence of liver cancer or liver-related death. Medicaid patients who are not eligible for treatment under restricted policies would get treatment once they transition to the Medicare program, which would incur 10% reduction in HCV-related costs due to early treatment in Medicaid. Further expanding treatment to patients with early fibrosis stages (F0 or F1) would cost Medicaid an additional $693 million during the next decade but would reduce the number of individuals in need of treatment in Medicare by 46% and decrease Medicare treatment costs by 23%. In some scenarios, outcomes could worsen with eligibility expansion if there is inadequate capacity to treat all patients. Expansion of HCV treatment coverage to less severe stages of liver disease may not substantially improve liver related outcomes for patients in Pennsylvania Medicaid in scenarios in which coverage through Medicare is widely available. Published by Elsevier Inc.

  17. Genetic stability of foot-and-mouth disease virus during long-term infections in natural hosts.

    Science.gov (United States)

    Ramirez-Carvajal, Lisbeth; Pauszek, Steven J; Ahmed, Zaheer; Farooq, Umer; Naeem, Khalid; Shabman, Reed S; Stockwell, Timothy B; Rodriguez, Luis L

    2018-01-01

    Foot-and-mouth disease (FMD) is a severe infection caused by a picornavirus that affects livestock and wildlife. Persistence in ruminants is a well-documented feature of Foot-and-mouth disease virus (FMDV) pathogenesis and a major concern for disease control. Persistently infected animals harbor virus for extended periods, providing a unique opportunity to study within-host virus evolution. This study investigated the genetic dynamics of FMDV during persistent infections of naturally infected Asian buffalo. Using next-generation sequencing (NGS) we obtained 21 near complete FMDV genome sequences from 12 sub-clinically infected buffalo over a period of one year. Four animals yielded only one virus isolate and one yielded two isolates of different serotype suggesting a serial infection. Seven persistently infected animals yielded more than one virus of the same serotype showing a long-term intra-host viral genetic divergence at the consensus level of less than 2.5%. Quasi-species analysis showed few nucleotide variants and non-synonymous substitutions of progeny virus despite intra-host persistence of up to 152 days. Phylogenetic analyses of serotype Asia-1 VP1 sequences clustered all viruses from persistent animals with Group VII viruses circulating in Pakistan in 2011, but distinct from those circulating on 2008-2009. Furthermore, signature amino acid (aa) substitutions were found in the antigenically relevant VP1 of persistent viruses compared with viruses from 2008-2009. Intra-host purifying selective pressure was observed, with few codons in structural proteins undergoing positive selection. However, FMD persistent viruses did not show a clear pattern of antigenic selection. Our findings provide insight into the evolutionary dynamics of FMDV populations within naturally occurring subclinical and persistent infections that may have implications to vaccination strategies in the region.

  18. Genetic stability of foot-and-mouth disease virus during long-term infections in natural hosts.

    Directory of Open Access Journals (Sweden)

    Lisbeth Ramirez-Carvajal

    Full Text Available Foot-and-mouth disease (FMD is a severe infection caused by a picornavirus that affects livestock and wildlife. Persistence in ruminants is a well-documented feature of Foot-and-mouth disease virus (FMDV pathogenesis and a major concern for disease control. Persistently infected animals harbor virus for extended periods, providing a unique opportunity to study within-host virus evolution. This study investigated the genetic dynamics of FMDV during persistent infections of naturally infected Asian buffalo. Using next-generation sequencing (NGS we obtained 21 near complete FMDV genome sequences from 12 sub-clinically infected buffalo over a period of one year. Four animals yielded only one virus isolate and one yielded two isolates of different serotype suggesting a serial infection. Seven persistently infected animals yielded more than one virus of the same serotype showing a long-term intra-host viral genetic divergence at the consensus level of less than 2.5%. Quasi-species analysis showed few nucleotide variants and non-synonymous substitutions of progeny virus despite intra-host persistence of up to 152 days. Phylogenetic analyses of serotype Asia-1 VP1 sequences clustered all viruses from persistent animals with Group VII viruses circulating in Pakistan in 2011, but distinct from those circulating on 2008-2009. Furthermore, signature amino acid (aa substitutions were found in the antigenically relevant VP1 of persistent viruses compared with viruses from 2008-2009. Intra-host purifying selective pressure was observed, with few codons in structural proteins undergoing positive selection. However, FMD persistent viruses did not show a clear pattern of antigenic selection. Our findings provide insight into the evolutionary dynamics of FMDV populations within naturally occurring subclinical and persistent infections that may have implications to vaccination strategies in the region.

  19. Long-term health and quality-of-life consequences of mass screening for childhood celiac disease: A 10-year follow-up study

    NARCIS (Netherlands)

    Koppen, E.J. van; Schweizer, J.J.; Csizmadia, C.G.D.S.; Krom, Y.; Hylkema, H.B.; Geel, A.M. van; Koopman, H.M.; Verloove-Vanhorick, S.P.; Mearin, M.L.

    2009-01-01

    OBJECTIVE. Mass screening for celiac disease is controversial. The objective of this study was to determine whether detection of childhood celiac disease by mass screening improves long-term health status and health-related quality of life. METHODS.We conducted a prospective 10-year follow-up study

  20. Anorectal complications and function in patients suffering from inflammatory bowel disease: a series of patients with long-term follow-up

    NARCIS (Netherlands)

    Lam, T.J.; van Bodegraven, A.A.; Felt-Bersma, R.J.F.

    2014-01-01

    Aim: The aim of this study is to describe the long-term course of anorectal complains and function in a single centre cohort patients suffering from inflammatory bowel disease (IBD) with perianal lesions. Methods: Between 1993 and 2000, 56 IBD patients (43 Crohn's disease and 13 ulcerative colitis)

  1. Long-term Course of Alzheimer Disease in Patients Treated According to the Dutch Dementia Guideline at a Memory Clinic A "Real-Life" Study

    NARCIS (Netherlands)

    Droogsma, Erika; van Asselt, Dieneke; van Steijn, Jolanda; Diekhuis, Marjolein; Veeger, Nic; De Deyn, Peter P.

    2016-01-01

    Introduction:There is little knowledge of the long-term course of Alzheimer disease (AD) in light of current pharmacological and nonpharmacological interventions provided in a real-life setting.Methods:The Frisian Alzheimer's Disease Cohort study is a real-life study of the course of AD in patients

  2. Long-term prognosis of chronic kidney disease in non-ST elevation acute coronary syndrome treated with invasive strategy.

    Science.gov (United States)

    Roldán Torres, Ildefonso; Salvador Mercader, Inmaculada; Cabadés Rumbeu, Claudia; Díez Gil, José Luis; Ferrando Cervelló, José; Monteagudo Viana, Marta; Fernández Galera, Rubén; Mora Llabata, Vicente

    Patients with chronic kidney disease (CKD) have an increased risk of adverse cardiovascular outcomes after non-ST elevation acute coronary syndrome (NSTEACS). However, the information available on this specific population, is scarce. We evaluate the impact of CKD on long-term prognosis in patients with NSTEACS managed with invasive strategy. We conduct a prospective registry of patients with NSTEACS and coronary angiography. CKD was defined as a glomerular filtration rate < 60ml/min/1,73m 2 . The composite primary end-point was cardiac death and non fatal cardiovascular readmission. We estimated the cumulative probability and hazard rate (HR) of combined primary end-point at 3-years according to the presence or absence of CKD. We included 248 p with mean age of 66.9 years, 25% women. CKD was present at baseline in 67 patients (27%). Patients with CKD were older (74.9 vs. 63.9 years; P<.0001) with more prevalence of hypertension (89.6 vs. 66.3%; P<.0001), diabetes (53.7 vs. 35.9%; P=.011), history of heart failure (13.4 vs. 3.9%; P=.006) and anemia (47.8 vs. 16%; P<.0001). No differences in the extent of coronary artery disease. CKD was associated with higher cumulative probability (49.3 vs. 28.2%; log-rank P=.001) and HR of the primary combined end-point (HR: 1.94; CI95%: 1.12-3.27; P=.012). CKD was an independent predictor of adverse cardiovascular outcomes at 3-years (HR: 1.66; CI95%: 1.05-2.61; P=.03). In NSTEACS patients treated with invasive strategie CKD is associated independently with an increased risk of adverse cardiovascular outcomes at 3years. Copyright © 2016 Sociedad Española de Nefrología. Published by Elsevier España, S.L.U. All rights reserved.

  3. SPECT myocardial perfusion imaging. Long-term prognostic value in diabetic patients with and without coronary artery disease

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    Koehli, M. [Dept. of Endocrinology, Diabetology and Metabolism, Univ. Hospital Lausanne (Switzerland); Dept. of Nuclear Medicine, Univ. Hospital Lausanne (Switzerland); Monbaron, D.; Gaillard, R.C.; Ruiz, J. [Dept. of Endocrinology, Diabetology and Metabolism, Univ. Hospital Lausanne (Switzerland); Prior, J.O.; Bischof Delaloye, A. [Dept. of Nuclear Medicine, Univ. Hospital Lausanne (Switzerland); Calcagni, M.L. [Dept. of Nuclear Medicine, Univ. Hospital Lausanne (Switzerland); Dept. of Cardiology, Univ. Hospital Lausanne (Switzerland); Fivaz-Arbane, M.; Stauffer, J.C. [Inst. of Nuclear Medicine, Univ. Cattolica del Sacro Cuore, Roma (Italy)

    2006-07-01

    Aim: To determine the long-term prognostic value of SPECT myocardial perfusion imaging (MPI) for the occurrence of cardiovascular events in diabetic patients. Patients, methods: SPECT MPI of 210 consecutive Caucasian diabetic patients were analysed using Kaplan-Meier event-free survival curve and independent predictors were determined by Cox multivariate analyses. Results: Follow-up was complete in 200 (95%) patients with a median period of 3.0 years (0.8-5.0). The population was composed of 114 (57%) men, age 65 {+-} 10 years, 181 (90.5%) type 2 diabetes mellitus, 50 (25%) with a history of coronary artery disease (CAD) and 98 (49%) presenting chest pain prior to MPI. The prevalence of abnormal MPI was 58%. Patients with a normal MPI had neither cardiac death, nor myocardial infarction, independently of a history of coronary artery disease or chest pain. Among the independent predictors of cardiac death and myocardial infarction, the strongest was abnormal MPI (p < 0.0001), followed by history of CAD (Hazard Ratio (HR) = 15.9; p = 0.0001), diabetic retinopathy (HR = 10.0; p = 0.001) and inability to exercise (HR = 7.7; p = 0.02). Patients with normal MPI had a low revascularisation rate of 2.4% during the follow-up period. Compared to normal MPI, cardiovascular events increased 5.2 fold for reversible defects, 8.5 fold for fixed defects and 20.1 fold for the association of both defects. Conclusion: Diabetic patients with normal MPI had on excellent prognosis independently of history of CAD. On the opposite, an abnormal MPI led to a > 5-fold increase in cardiovascular events. This emphasizes the value of SPECT MPI in predicting and risk-stratifying cardiovascular events in diabetic patients. (orig.)

  4. BIOCHEMICAL CONTROL DURING LONG-TERM FOLLOW-UP OF 230 ADULT PATIENTS WITH CUSHING DISEASE: A MULTICENTER RETROSPECTIVE STUDY.

    Science.gov (United States)

    Geer, Eliza B; Shafiq, Ismat; Gordon, Murray B; Bonert, Vivien; Ayala, Alejandro; Swerdloff, Ronald S; Katznelson, Laurence; Lalazar, Yelena; Manuylova, Ekaterina; Pulaski-Liebert, Karen J; Carmichael, John D; Hannoush, Zeina; Surampudi, Vijaya; Broder, Michael S; Cherepanov, Dasha; Eagan, Marianne; Lee, Jackie; Said, Qayyim; Neary, Maureen P; Biller, Beverly M K

    2017-08-01

    Cushing disease (CD) results from excessive exposure to glucocorticoids caused by an adrenocorticotropic hormone-secreting pituitary tumor. Inadequately treated CD is associated with significant morbidity and elevated mortality. Multicenter data on CD patients treated in routine clinical practice are needed to assess treatment outcomes in this rare disorder. The study purpose was to describe the burden of illness and treatment outcomes for CD patients. Eight pituitary centers in four U.S. regions participated in this multicenter retrospective chart review study. Subjects were CD patients diagnosed at ≥18 years of age within the past 20 years. Descriptive statistical analyses were conducted to examine presenting signs, symptoms, comorbidities, and treatment outcomes. Of 230 patients, 79% were female (median age at diagnosis, 39 years; range, 18 to 78 years). Length of follow-up was 0 to 27.5 years (median, 1.9 years). Pituitary adenomas were 0 to 51 mm. The most common presenting comorbidities included hypertension (67.3%), polycystic ovary syndrome (43.5%), and hyperlipidemia (41.5%). Biochemical control was achieved with initial pituitary surgery in 41.4% patients (91 of 220), not achieved in 50.0% of patients (110 of 220), and undetermined in 8.6% of patients (19 of 220). At the end of follow-up, control had been achieved with a variety of treatment methods in 49.1% of patients (110 of 224), not achieved in 29.9% of patients (67 of 224), and undetermined in 21.0% of patients (47 of 224). Despite multiple treatments, at the end of follow-up, biochemical control was still not achieved in up to 30% of patients. These multicenter data demonstrate that in routine clinical practice, initial and long-term control is not achieved in a substantial number of patients with CD. BLA = bilateral adrenalectomy CD = Cushing disease CS = Cushing syndrome eCRF = electronic case report form MRI = magnetic resonance imaging PCOS = polycystic ovary syndrome.

  5. SPARC, FOXP3, CD8 and CD45 correlation with disease recurrence and long-term disease-free survival in colorectal cancer.

    Directory of Open Access Journals (Sweden)

    Angela Chew

    Full Text Available BACKGROUND: SPARC is a matricellular protein involved in tissue remodelling, cell migration and angiogenesis, while forkhead box P3 (FOXP3 protein functions as a transcription factor involved in immune cell regulation. Both SPARC and FOXP3 can play an anti-tumorigenic role in cancer progression. The aim was to determine if SPARC, FOXP3, CD8 and CD45RO expression levels are associated with colorectal cancer (CRC stage, disease outcome and long-term cancer-specific survival (CSS in stage II and III CRC. METHODS AND FINDINGS: SPARC expression was initially assessed in 120 paired normal and stage I-IV CRCs. Subsequently, approximately 1000 paired patient samples of stage II or III CRCs in tissue microarrays were stained for SPARC, FOXP3, CD8 or CD45RO. Proportional hazards modelling assessed correlations between these markers and clinicopathological data, including disease outcome and cancer specific survival (CSS. Both SPARC and FOXP3 expression were significantly greater in CRC than normal colon (p<0.0001. High SPARC expression correlated with good disease outcome (≥60 mths without disease recurrence, p = 0.0039 and better long-term CSS in stage II CRC (<0.0001. In stage III CRC, high SPARC expression correlated with better long-term CSS (p<0.0001 and less adjuvant chemotherapy use (p = 0.01. High FOXP3 correlated with a good disease outcome, better long-term CSS and less adjuvant chemotherapy use in stage II (p<0.0037, <0.0001 and p = 0.04 respectively, but not in stage III CRC. High CD8 and CD45RO expression correlated with better disease outcome in stage II CRC, and better CSS, but the differences were not as marked as for SPARC and FOXP3. CONCLUSIONS: These data suggest that high SPARC and FOXP3 are associated with better disease outcome in stage II CRC and may be prognostic indicators of CSS. Further assessment of whether these markers predict patients at high risk of recurrence with stage II CRC and functional studies of these

  6. Long-term cannabidiol treatment prevents the development of social recognition memory deficits in Alzheimer's disease transgenic mice.

    Science.gov (United States)

    Cheng, David; Spiro, Adena S; Jenner, Andrew M; Garner, Brett; Karl, Tim

    2014-01-01

    Impairments in cognitive ability and widespread pathophysiological changes caused by neurotoxicity, neuroinflammation, oxidative damage, and altered cholesterol homeostasis are associated with Alzheimer's disease (AD). Cannabidiol (CBD) has been shown to reverse cognitive deficits of AD transgenic mice and to exert neuroprotective, anti-oxidative, and anti-inflammatory properties in vitro and in vivo. Here we evaluate the preventative properties of long-term CBD treatment in male AβPPSwe/PS1ΔE9 (AβPP × PS1) mice, a transgenic model of AD. Control and AD transgenic mice were treated orally from 2.5 months of age with CBD (20 mg/kg) daily for 8 months. Mice were then assessed in the social preference test, elevated plus maze, and fear conditioning paradigms, before cortical and hippocampal tissues were analyzed for amyloid load, oxidative damage, cholesterol, phytosterols, and inflammation. We found that AβPP × PS1 mice developed a social recognition deficit, which was prevented by CBD treatment. CBD had no impact on anxiety or associative learning. The prevention of the social recognition deficit was not associated with any changes in amyloid load or oxidative damage. However, the study revealed a subtle impact of CBD on neuroinflammation, cholesterol, and dietary phytosterol retention, which deserves further investigation. This study is the first to demonstrate CBD's ability to prevent the development of a social recognition deficit in AD transgenic mice. Our findings provide the first evidence that CBD may have potential as a preventative treatment for AD with a particular relevance for symptoms of social withdrawal and facial recognition.

  7. The impact of combined pulmonary fibrosis and chronic obstructive pulmonary disease on long-term survival after lung cancer surgery.

    Science.gov (United States)

    Sekine, Yasuo; Sakairi, Yuichi; Yoshino, Mitsuru; Koh, Eitetsu; Hata, Atsushi; Suzuki, Hidemi; Yoshino, Ichiro

    2014-06-01

    The purpose of this study was to determine the impact of pulmonary fibrosis (PF) on postoperative complications and on long-term survival after surgical resection in lung cancer patients with chronic obstructive pulmonary disease (COPD). A retrospective chart review was conducted of 380 patients with COPD who had undergone pulmonary resection for lung cancer at the University Hospital between 1990 and 2005. The definition of COPD was a preoperative forced expiratory volume in 1 second/forced vital capacity (FEV1/FVC) ratio of less than 70%; PF was defined as obvious bilateral fibrous change in the lower lung fields, confirmed by computed tomography. PF was present in 41 patients (10.8%) with COPD; the remaining 339 patients (89.2%) did not have PF. The preoperative FVC/FEV1 was significantly lower in the group of patients with PF than in the group without (p < 0.05). Acute lung injury and home oxygen therapy were significantly more common in the PF group; however, the 30-day mortality was similar between the groups. The cumulative survival at 3 and 5 years was 53.6 and 36.9%, respectively, in the PF group and 71.4 and 66.1%, respectively, in the non-PF group (p = 0.0009). Increased age, decreased body mass index, advanced pathologic stage, and the existence of PF were identified as independent risk factors for decreased survival. PF is a risk factor for decreased survival after surgical treatment in lung cancer patients with COPD. Georg Thieme Verlag KG Stuttgart · New York.

  8. Long-term outcomes from Healthy Eating and Exercise Lifestyle Program for overweight people with heart disease and diabetes.

    Science.gov (United States)

    Alharbi, Muaddi; Gallagher, Robyn; Kirkness, Ann; Sibbritt, David; Tofler, Geoffrey

    2016-02-01

    The benefits of exercise and weight reduction for overweight or obese people with coronary heart disease and/or diabetes mellitus are well recognised. The Healthy Eating and Exercise Lifestyle Program demonstrated these outcomes at 4 months, but longer-term outcomes are not yet reported. To determine whether positive weight, body mass index, waist and exercise duration outcomes were sustained in the long term (12 months) and to identify the independent predictors of these outcomes at 4 and 12 months. Longitudinal design, combining data of all Healthy Eating and Exercise Lifestyle Program participants (intervention and wait-list control, n = 134). Participants had a body mass index between 27 and 39 kg/m(2) and had completed cardiac rehabilitation and/or diabetes education programmes. Healthy Eating and Exercise Lifestyle Program intervention included an active phase of two 1-hour group-based supervised structured exercise sessions every week for 4 months and four 90-minute group information and support sessions. The maintenance phase included one 90-minute group-based booster information session and three 15-minute goal-focused telephone follow-up calls over 8 months. Participants had statistically significant reductions from baseline in weight, body mass index and waist circumference and improvements in exercise duration and capacity at 4 and 12 months. Time, self-efficacy, depressive symptoms and male gender were independent predictors for body mass index, waist and/or exercise duration (p Healthy Eating and Exercise Lifestyle Program was an effective programme to achieve and sustain weight loss and increase exercise participation over 1 year. © The European Society of Cardiology 2014.

  9. Long-term safety and efficacy of AAV gene therapy in the canine model of glycogen storage disease type Ia.

    Science.gov (United States)

    Lee, Young Mok; Conlon, Thomas J; Specht, Andrew; Coleman, Kirsten E; Brown, Laurie M; Estrella, Ana M; Dambska, Monika; Dahlberg, Kathryn R; Weinstein, David A

    2018-05-25

    Viral mediated gene therapy has progressed after overcoming early failures, and gene therapy has now been approved for several conditions in Europe and the USA. Glycogen storage disease (GSD) type Ia, caused by a deficiency of glucose-6-phosphatase-α, has been viewed as an outstanding candidate for gene therapy. This follow-up report describes the long-term outcome for the naturally occurring GSD-Ia dogs treated with rAAV-GPE-hG6PC-mediated gene therapy. A total of seven dogs were treated with rAAV-GPE-hG6PC-mediated gene therapy. The first four dogs were treated at birth, and three dogs were treated between 2 and 6 months of age to assess the efficacy and safety in animals with mature livers. Blood and urine samples, radiographic studies, histological evaluation, and biodistribution were assessed. Gene therapy improved survival in the GSD-Ia dogs. With treatment, the biochemical studies normalized for the duration of the study (up to 7 years). None of the rAAV-GPE-hG6PC-treated dogs had focal hepatic lesions or renal abnormalities. Dogs treated at birth required a second dose of rAAV after 2-4 months; gene therapy after hepatic maturation resulted in improved efficacy after a single dose. rAAV-GPE-hG6PC treatment in GSD-Ia dogs was found to be safe and efficacious. GSD-Ia is an attractive target for human gene therapy since it is a monogenic disorder with limited tissue involvement. Blood glucose and lactate monitoring can be used to assess effectiveness and as a biomarker of success. GSD-Ia can also serve as a model for other hepatic monogenic disorders.

  10. Long-term follow-up study on the safety of deep brain stimulation for treating Parkinson's disease

    Directory of Open Access Journals (Sweden)

    Xi WU

    2015-10-01

    Full Text Available Objective To evaluate the safety of deep brain stimulation (DBS for treating Parkinson's disease (PD under long-term follow-up and modify the surgical procedure. Methods A total of 362 PD patients underwent DBS, and almost 613 electrodes were implanted into these patients. Both surgical and hardware-related complications of DBS were retrospectively analyzed. Results Perioperative complications included postoperative confusion or delirium in 21 cases (5.80%, intracranial hemorrhage in 4 cases (1.10%; 2 asymptomatic cortical microhemorrhage and 2 basal ganglia trajectory microhemorrhage, generalized tonic-clonic seizures (GTCS in 2 cases (0.55% , urinary tract infection in 4 cases (1.10% , pulmonary infection in 7 cases (1.93%, implantable pulse generator (IPG hematoma in 11 cases (3.04%, IPG seroma in 3 cases (0.83%. All these patients were cured. They were followed-up for 12-146 months (median 34 months. Hardware-related complications included infection of incisional wound and/or skin erosion (9 cases, 2.49% , extension wire fracture caused by IPG displacement (one case, 0.28% , IPG shifting to abdomen due to fixation wire fracture (one case, 0.28%, slightly migrated electrode due to fall (one case, 0.28%, and discomfort about occipital incision (one case, 0.28%. Conclusions The overall risk of both surgical and hardware-related adverse events of DBS for treating PD is acceptably low. DOI: 10.3969/j.issn.1672-6731.2015.10.005

  11. Bentall and De Bono surgery for correction of valve and ascending aortic disease: long-term results.

    Science.gov (United States)

    Silva, Virgílio Figueiredo; Real, Daniel Sundfeld Spiga; Branco, João Nelson Rodrigues; Catani, Roberto; Kim, Hyong Chun; Buffolo, Enio; Fonseca, José Honório de Almeida Palma da

    2008-01-01

    A retrospective study was performed in a series of consecutive patients who underwent a Bentall and De Bono procedure. Data were removed of medical records and follow-up data were obtained from clinical records and direct contact with patients. A total of 39 patients were studied between January 1996 and December 2005. The median age was 47 years (range 14-70). There were 33 males and six females. Eleven (25.5%) patients presented Marfan syndrome and one (2.5%) Turner syndrome. Nineteen (48.5%) patients had hypertension, eight (20.5%) had history of smoking, six (15.5%) had history of alcoholism, eight (20.5%) had dyslipidemia, two (5.0%) had diabetes and one (2.56%) had myocardial infarct previously. Twenty-eight (72%) patients were in II-III NYHA class in the moment of the surgery. Annulo-aortic ecstasy was present in 14 (35.9%) patients and aortic aneurysms in 16 (41%). The median time in intensive care unit was 8.79 days with range 2-23 days. Four (10.0%) patients underwent an emergency operation and 35 (90%) elective. The overall hospital mortality was 5% (2/39). The event-free survival is 94.87% at 1 year and 84.61% at in 5 and 10. The median time of follow-up was 46.5 months (range 14-120 months). The Bentall and De Bono technique obtained excellent results in the short-term and long-term, which support the continued use of the compositive graft technique as the preferred method of treatment for patients with aortic root disease. Our findings confirm the current literature data.

  12. Duration of high-dose aspirin therapy does not affect long-term coronary artery outcomes in Kawasaki disease.

    Science.gov (United States)

    Migally, Karl; Braunlin, Elizabeth A; Zhang, Lei; Binstadt, Bryce A

    2018-05-02

    BackgroundHigh-dose aspirin (HDA) is used with intravenous immunoglobulin (IVIg) in Kawasaki disease (KD). Practice regarding HDA varies, and it is unclear whether HDA duration affects the long-term course.MethodsWe retrospectively studied KD patients at our hospital for over 10 years. Patients were categorized as having received HDA for 0, 1-7, or >7 days. Primary outcome was the maximum coronary Z-score at diagnosis and follow-up; secondary outcomes included inflammatory markers.ResultsOne hundred and three patients had HDA duration documented, of which 35 patients had coronary artery abnormalities (CAAs) at diagnosis. There was no difference in demographics or inflammatory markers between the HDA groups, and no difference in HDA duration between patients with or without CAAs. Seventeen patients received no HDA; they had longer illness and defervescence duration before diagnosis, and were less likely to receive IVIg. For CAAs, multivariate regression revealed that HDA duration did not predict the coronary Z-score at 9-15 months. Higher Z-score at diagnosis was associated with higher Z-score at 9-15 months.ConclusionThe only factor associated with coronary Z-score at 9-15 months was the Z-score at diagnosis. At our institution, longer illness and defervescence duration and the lack of IVIg administration were associated with not administering HDA. HDA duration did not affect the clinically relevant outcomes, particularly CAA persistence.Pediatric Research advance online publication, 2 May 2018; doi:10.1038/pr.2018.44.

  13. Comparison of Efficacy of Long-term Oral Treatment with Telmisartan and Benazepril in Cats with Chronic Kidney Disease.

    Science.gov (United States)

    Sent, U; Gössl, R; Elliott, J; Syme, H M; Zimmering, T

    2015-01-01

    The efficacy and benefits of telmisartan in cats with chronic kidney disease (CKD) have not previously been reported. Long-term treatment of cats with CKD using telmisartan decreases urine protein-to-creatinine ratio (UP/C) similar to benazepril. Two-hundred and twenty-four client-owned adult cats with CKD. Prospective, multicenter, controlled, randomized, parallel group, blinded clinical trial with noninferiority design. Cats were allocated in a 1:1 ratio to either telmisartan (1 mg/kg; n = 112) or benazepril (0.5-1.0 mg/kg; n = 112) PO q24 h. The primary endpoint was prospectively defined as the change in proteinuria (benazepril:telmisartan) based on a log transformed weighted average of UP/C change from baseline (AUC 0→t/t) as a percentage compared using a confidence interval (CI) approach. Changes of UP/C from baseline were assessed on all study days and corrected for multiple comparisons. Telmisartan proved noninferior to benazepril in controlling proteinuria (CI, -0.035 to 0.268). At Day 180, UP/C compared to baseline in the telmisartan group was significantly lower (-0.05 ± 0.31; P = .016), whereas in the benazepril group the change (-0.02 ± 0.48) was not statistically significant (P = .136). Similar results were obtained at all assessment points with significant decrease in UP/C occurring with telmisartan but not benazepril. Both telmisartan and benazepril were well tolerated and safe. Telmisartan proved to be noninferior to benazepril and significantly decreased proteinuria relative to baseline at all assessment points whereas benazepril did not. Copyright © 2015 The Authors. Journal of Veterinary Internal Medicine published by Wiley Periodicals, Inc. on behalf of the American College of Veterinary Internal Medicine.

  14. Providing physicians with feedback on medication adherence for people with chronic diseases taking long-term medication.

    Science.gov (United States)

    Zaugg, Vincent; Korb-Savoldelli, Virginie; Durieux, Pierre; Sabatier, Brigitte

    2018-01-10

    Poor medication adherence decreases treatment efficacy and worsens clinical outcomes, but average rates of adherence to long-term pharmacological treatments for chronic illnesses are only about 50%. Interventions for improving medication adherence largely focus on patients rather than on physicians; however, the strategies shown to be effective are complex and difficult to implement in clinical practice. There is a need for new care models addressing the problem of medication adherence, integrating this problem into the patient care process. Physicians tend to overestimate how well patients take their medication as prescribed. This can lead to missed opportunities to change medications, solve adverse effects, or propose the use of reminders in order to improve patients' adherence. Thus, providing physicians with feedback on medication adherence has the potential to prompt changes that improve their patients' adherence to prescribed medications. To assess the effects of providing physicians with feedback about their patients' medication adherence for improving adherence. We also assessed the effects of the intervention on patient outcomes, health resource use, and processes of care. We conducted a systematic search of the Cochrane Central Register of Controlled Trials (CENTRAL), MEDLINE, and Embase, all from database inception to December 2016 and without any language restriction. We also searched ISI Web of Science, two trials registers, and grey literature. We included randomised trials, controlled before-after studies, and interrupted time series studies that compared the effects of providing feedback to physicians about their patients' adherence to prescribed long-term medications for chronic diseases versus usual care. We included published or unpublished studies in any language. Participants included any physician and any patient prescribed with long-term medication for chronic disease. We included interventions providing the prescribing physician with

  15. Long-term outcome in rheumatoid arthritis: a simple algorithm of baseline parameters can predict radiographic damage, disability, and disease course at 12-year followup

    NARCIS (Netherlands)

    Drossaers-Bakker, K. W.; Zwinderman, A. H.; Vliet Vlieland, T. P. M.; van Zeben, D.; Vos, K.; Breedveld, F. C.; Hazes, J. M. W.

    2002-01-01

    OBJECTIVES: To predict the long-term outcome of rheumatoid arthritis (RA) with respect to radiographic damage, disability, and disease course using baseline variables, and to construct decision trees identifying patients on an individual level at the extremes of the outcome spectrum of these 3

  16. Reversibility of cortical hyperostosis following long-term prostaglandin E1 therapy in infants with ductus-dependent congenital heart disease

    DEFF Research Database (Denmark)

    Høst, A; Halken, S; Andersen, P E

    1988-01-01

    Two neonates with complex cyanotic congenital heart disease, receiving long-term prostaglandin E1 infusion, for 59 and 78 days respectively, demonstrated significant radiographic changes of symmetric cortical hyperostosis of the long bones. Bone biopsies from one of the patients elucidated...

  17. Subtrochanteric Fracture In A Chinese Woman With Paget’s Disease Of Bone And On Long Term Bisphosphonate Therapy: Could It Be An Insufficiency Fracture?

    Directory of Open Access Journals (Sweden)

    CK Lee

    2011-03-01

    Full Text Available Paget’s disease is common in Western countries but is very rare in Chinese populations. Although bisphosphonate has been widely used to treat symptomatic Paget’s disease, prolonged use may be associated with insufficiency fracture. We highlight this rare case of Paget’s disease in a Chinese lady who presented with an insufficiency fracture following long-term use of bisphosphonate.

  18. Does the method of weight loss effect long-term changes in weight, body composition or chronic disease risk factors in overweight or obese adults? A systematic review.

    Directory of Open Access Journals (Sweden)

    Richard A Washburn

    Full Text Available Differences in biological changes from weight loss by energy restriction and/or exercise may be associated with differences in long-term weight loss/regain.To assess the effect of weight loss method on long-term changes in weight, body composition and chronic disease risk factors.PubMed and Embase were searched (January 1990-October 2013 for studies with data on the effect of energy restriction, exercise (aerobic and resistance on long-term weight loss. Twenty articles were included in this review.Primary source, peer reviewed randomized trials published in English with an active weight loss period of >6 months, or active weight loss with a follow-up period of any duration, conducted in overweight or obese adults were included.Considerable heterogeneity across trials existed for important study parameters, therefore a meta-analysis was considered inappropriate. Results were synthesized and grouped by comparisons (e.g. diet vs. aerobic exercise, diet vs. diet + aerobic exercise etc. and study design (long-term or weight loss/follow-up.Forty percent of trials reported significantly greater long-term weight loss with diet compared with aerobic exercise, while results for differences in weight regain were inconclusive. Diet+aerobic exercise resulted in significantly greater weight loss than diet alone in 50% of trials. However, weight regain (∼ 55% of loss was similar in diet and diet+aerobic exercise groups. Fat-free mass tended to be preserved when interventions included exercise.

  19. Cardiovascular disease is the main cause of long-term excess mortality after ischemic stroke in young adults

    NARCIS (Netherlands)

    Rutten-Jacobs, L.C.A.; Arntz, R.M.; Maaijwee, N.A.M.M.; Schoonderwaldt, H.C.; Dorresteijn, L.D.; Dijk, E.J. van; Leeuw, F.E. de

    2015-01-01

    Adults with stroke at a young age (18-50 years) remain at an increased risk of death for decades. It is unclear what cause underlies this long-term excess mortality and whether this is sex and time specific. Therefore, we investigated sex-specific temporal changes in cause of death after transient

  20. Association Between Satellite-based Estimates of Long-term PM2.5 Exposure and Coronary Artery Disease

    Science.gov (United States)

    Background: Epidemiological studies have identified associations between long-term PM2.5 exposure and cardiovascular events, though most have relied on concentrations from central-site air quality monitors. Methods: We utilized a cohort of 5679 patients who had undergone cardiac ...

  1. Long-term effects of total and source-specific particulate air pollution on incident cardiovascular disease in Gothenburg, Sweden.

    Science.gov (United States)

    Stockfelt, Leo; Andersson, Eva M; Molnár, Peter; Gidhagen, Lars; Segersson, David; Rosengren, Annika; Barregard, Lars; Sallsten, Gerd

    2017-10-01

    Long-term exposure to air pollution increases cardiopulmonary morbidity and mortality, but it is not clear which components of air pollution are the most harmful, nor which time window of exposure is most relevant. Further studies at low exposure levels have also been called for. We analyzed two Swedish cohorts to investigate the effects of total and source-specific particulate matter (PM) on incident cardiovascular disease for different time windows of exposure. Two cohorts initially recruited to study predictors of cardiovascular disease (the PPS cohort and the GOT-MONICA cohort) were followed from 1990 to 2011. We collected data on residential addresses and assigned each individual yearly total and source-specific PM and Nitrogen Oxides (NO x ) exposures based on dispersion models. Using multivariable Cox regression models with time-dependent exposure, we studied the association between three different time windows (lag 0, lag 1-5, and exposure at study start) of residential PM and NO x exposure, and incidence of ischemic heart disease, stroke, heart failure and atrial fibrillation. During the study period, there were 2266 new-onset cases of ischemic heart disease, 1391 of stroke, 925 of heart failure and 1712 of atrial fibrillation. The majority of cases were in the PPS cohort, where participants were older. Exposure levels during the study period were moderate (median: 13µg/m 3 for PM 10 and 9µg/m 3 for PM 2.5 ), and similar in both cohorts. Road traffic and residential heating were the largest local sources of PM air pollution, and long distance transportation the largest PM source in total. In the PPS cohort, there were positive associations between PM in the last five years and both ischemic heart disease (HR: 1.24 [95% CI: 0.98-1.59] per 10µg/m 3 of PM 10 , and HR: 1.38 [95% CI: 1.08-1.77] per 5µg/m 3 of PM 2.5 ) and heart failure. In the GOT-MONICA cohort, there were positive but generally non-significant associations between PM and stroke (HR: 1

  2. Long-term effect of galantamine on cognitive function in patients with Alzheimer’s disease versus a simulated disease trajectory: an observational study in the clinical setting

    Directory of Open Access Journals (Sweden)

    Nakagawa R

    2017-04-01

    Full Text Available Ryoko Nakagawa,1 Takashi Ohnishi,1 Hisanori Kobayashi,1 Toshio Yamaoka,2 Tsutomu Yajima,3 Ai Tanimura,4 Toshiya Kato,4 Kazutake Yoshizawa1 1Evidence Generation Department, Medical Affairs Division, 2Clinical Data Management Department, R&D Division, 3Biostatistics Department, Quantitative Science Division, 4Drug Surveillance Department, R&D Division, Janssen Pharmaceutical K.K., Tokyo, Japan Background: Long-term maintenance of cognitive function is an important goal of treatment for Alzheimer’s disease (AD, but evidence about the long-term efficacy of cholinesterase inhibitors is sparse. To evaluate the long-term efficacy and safety of galantamine for AD in routine clinical practice, we conducted a 72-week post-marketing surveillance study. The effect of galantamine on cognitive function was estimated in comparison with a simulated disease trajectory. Patients and methods: Patients with mild-to-moderate AD received flexible dosing of galantamine (16–24 mg/day during this study. Cognitive function was assessed by the mini mental state examination (MMSE and the clinical status was determined by the Clinical Global Impression-Improvement (CGI-I. Changes of the MMSE score without treatment were estimated in each patient using Mendiondo’s model. Generalized linear mixed model analysis was performed to compare the simulated MMSE scores with the actual scores. Results: Of the 661 patients who were enrolled, 642 were evaluable for safety and 554 were assessed for efficacy. The discontinuation rate was 46.73%. Cognitive decline indicated by the mean change of actual MMSE scores was significantly smaller than the simulated decline. Individual analysis demonstrated that >70% of patients had better actual MMSE scores than their simulated scores. Significant improvement of CGI-I was also observed during the observation period. Adverse events occurred in 28.5% of patients and were serious in 8.41%. The reported events generally corresponded with the

  3. Persistence of normal cardiac function and myocardial perfusion in irradiated long-term survivors of Hodgkin's disease

    International Nuclear Information System (INIS)

    Constine, L.S.; Schwartz, R.G.; Savage, D.E.; King, V.; Muhs, A.; Rubin, P.

    1996-01-01

    Purpose: The risk of myocardial infarction and cardiac dysfunction following mantle irradiation (RT) for Hodgkin's disease is controversial. The relative risk of fatal myocardial infarction is 2.8 in our Hodgkin's patients, similar to other reports. Sensitive evaluations of cardiac function and myocardial perfusion might be expected to reveal pre-clinical abnormalities of potential significance. We hypothesized the presence of pre-clinical cardiac toxicity and progressive deterioration of left ventricular performance and myocardial ischemia over time in long-term survivors of Hodgkin's disease. The data reported herein extend our previous study in patient number (n=50) and follow-up duration (mean 16.5 years). Materials and Methods: Equilibrium radionuclide angiocardiography (ERNA) was used to quantify left ventricular (LV) systolic and diastolic function with LV ejection fraction (LVEF) and peak filling rate (PFR), respectively. Quantitative myocardial perfusion scintigraphy (MPS) and ECG stress testing with exercise or dipyridamole were used to assess myocardial perfusion and electrical function. Patients at least 1.0 year after RT were eligible if ≤ 50 years old at RT and without known Hodgkin's or cardiac disease. Fifty patients, ages 10-46 years (mean 26.0) at RT, were tested 1.1 to 29.1 years (mean 9.1) after RT. Seventeen patients were tested 2 - 3 times separated by 0.5 - 6.5 years (mean 3.3). The mean central cardiac RT dose was 35.1 Gy (range 18.5 - 47.5) in daily 1.5-2.0 Gy fractions. Twelve patients were additionally irradiated to the left ventricle (LVRT), usually through partial transmission left lung shields (range 14.3-21.3 Gy). Results: No patient had symptomatic cardiac disease at the time of evaluation. The mean LVEF (first test, n = 50) was 60 ± 6% (range 42-73%) [normal ≥ 50%], and PFR (first test, n=44) was 3.43 ± 0.83 end diastolic volume per second (range 1.5-5.2 EDV/sec) [normal ≥ 2.54 EDV/sec] with 2 and 7 patients below normal

  4. Long-term prognosis of asthma, chronic obstructive pulmonary disease, and asthma-chronic obstructive pulmonary disease overlap in the Copenhagen City Heart study

    DEFF Research Database (Denmark)

    Lange, Peter; Çolak, Yunus; Ingebrigtsen, Truls Sylvan

    2016-01-01

    BACKGROUND: Long-term prognosis of patients with characteristics of both chronic obstructive pulmonary disease (COPD) and asthma, named asthma-COPD overlap, is poorly described. We investigated the long-term prognosis of individuals with different types of chronic airway disease, with a special...... were 39·48 (95% CI 25·93-60·11) in asthma-COPD overlap with early-onset asthma, 83·47 (61·67-112·98) in asthma-COPD overlap with late-onset asthma, 23·80 (17·43-33·50) in COPD, and 14·74 (10·06-21·59) in asthma compared with never-smokers without lung disease (all p... focus on individuals with asthma-COPD overlap. METHODS: We assigned participants from the Copenhagen City Heart Study into six subgroups: healthy never-smokers, ever-smokers without asthma and COPD, those with asthma with low cumulated smoking exposure and no airflow limitation, those with COPD, those...

  5. Long-term enzyme replacement therapy is associated with reduced proteinuria and preserved proximal tubular function in women with Fabry disease

    DEFF Research Database (Denmark)

    Prabakaran, Thaneas; Birn, Henrik; Bibby, Bo M

    2014-01-01

    dysfunction in women with Fabry disease treated with ERT. METHODS: A retrospective, single centre, cohort study evaluated the long-term association between ERT, albuminuria and eGFR in 13 women with Fabry disease and mild renal involvement. In particular, we analysed the changes in the proteinuric profile...... to end-stage renal failure. In women with Fabry disease, accumulation of GL-3 in the glomerular podocytes and other renal cells induces progressive, proteinuric nephropathy, but not as severe as in men. Enzyme replacement therapy (ERT) with recombinant α-Gal A reduces cellular GL-3 deposits in podocytes...... in albuminuria was paralleled by a decrease in both glomerular and tubular urine protein markers. CONCLUSIONS: The data indicate that long-term ERT is associated with a reduction in albuminuria and glomerular and tubular urinary protein markers in women with Fabry disease and mild renal manifestations....

  6. Long-term exposure to air pollution and the incidence of Parkinson's disease: A nested case-control study.

    Directory of Open Access Journals (Sweden)

    Chiu-Ying Chen

    , comorbid conditions such as dementia (ORs = 3.53-3.93, Ps < 0.001, stroke (ORs = 2.99-3.01, Ps < 0.001, depression (ORs = 2.51-2.64, Ps < 0.001, head injury (ORs = 1.24-1.29, 0.001 ≤ Ps < 0.01 or 0.01 ≤ Ps < 0.05, sleep disorder (OR = 1.23-1.26, 0.001 ≤ Ps < 0.01, and hypertension (ORs = 1.18-1.19, 0.01 ≤ Ps < 0.05 also significantly increased the risk for PD development.Although PM10 plays a significant role in PD development, the associated chemical/metal compounds that are capable of inducing adverse biological mechanisms still warrant further exploration. Because of a link between comorbid conditions and PM exposure, research on the causal relationship between long-term exposure to PM and the development of PD should be considered with caution because other possible modifiers or mediators, comorbid diseases in particular, may be involved.

  7. Long-term collections

    CERN Multimedia

    Collectes à long terme

    2007-01-01

    The Committee of the Long Term Collections (CLT) asks for your attention for the following message from a young Peruvian scientist, following the earthquake which devastated part of her country a month ago.

  8. Long-term disease control and toxicity outcomes following surgery and intensity modulated radiation therapy (IMRT) in pediatric craniopharyngioma.

    Science.gov (United States)

    Greenfield, Brad J; Okcu, Mehmet F; Baxter, Patricia A; Chintagumpala, Murali; Teh, Bin S; Dauser, Robert C; Su, Jack; Desai, Snehal S; Paulino, Arnold C

    2015-02-01

    To report long-term progression-free survival (PFS) and late-toxicity outcomes in pediatric craniopharyngioma patients treated with IMRT. Twenty-four children were treated with IMRT to a median dose of 50.4Gy (range, 49.8-54Gy). The clinical target volume (CTV) was the gross tumor volume (GTV) with a 1cm margin. The planning target volume (PTV) was the CTV with a 3-5mm margin. Median follow-up was 107.3months. The 5- and 10-year PFS rates were 65.8% and 60.7%. The 5- and 10-year cystic PFS rates were 70.2% and 65.2% while the 5- and 10-year solid PFS were the same at 90.7%. Endocrinopathy was seen in 42% at initial diagnosis and in 74% after surgical intervention, prior to IMRT. Hypothalamic dysfunction and visual deficits were associated with increasing PTV and number of surgical interventions. IMRT is a viable treatment option for pediatric craniopharyngioma. Despite the use of IMRT, majority of the craniopharyngioma patients experienced long-term toxicity, many of which present prior to radiotherapy. Limitations of retrospective analyses on small patient cohort elicit the need for a prospective multi-institutional study to determine the absolute benefit of IMRT in pediatric craniopharyngioma. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  9. Life-long diseases need life-long treatment: long-term safety of ciclosporin in canine atopic dermatitis

    Science.gov (United States)

    Nuttall, Tim; Reece, Douglas; Roberts, Elizabeth

    2014-01-01

    Ciclosporin (Atopica; Novartis Animal Health) has been licensed for canine atopic dermatitis (AD) since 2002. Adverse events (AEs) have been reported in 55 per cent of 759 dogs in 15 clinical trials, but are rare in pharmacovigilance data (71.81 AEs/million capsules sold). Gastrointestinal reactions were most common, but were mild and rarely required intervention. Other AEs were rare (≤1 per cent in clinical trials; metabolism is not clinically significant for normal dogs. Concomitant treatment with most drugs is safe. Effects on cytochrome P450 and MDR1 P-glycoprotein activity may elevate plasma ciclosporin concentrations, but short-term changes are not clinically significant. Monitoring of complete blood counts, urinalysis or ciclosporin levels is not justified except with higher than recommended doses and/or long-term concurrent immunosuppressive drugs. Ciclosporin is not a contraindication for killed (including rabies) vaccines, but the licensed recommendation is that live vaccination is avoided during treatment. In conclusion, ciclosporin has a positive risk-benefit profile for the long-term management of canine AD. PMID:24682696

  10. Long-term disease control and toxicity outcomes following surgery and intensity modulated radiation therapy (IMRT) in pediatric craniopharyngioma

    International Nuclear Information System (INIS)

    Greenfield, Brad J.; Okcu, Mehmet F.; Baxter, Patricia A.; Chintagumpala, Murali; Teh, Bin S.; Dauser, Robert C.; Su, Jack; Desai, Snehal S.; Paulino, Arnold C.

    2015-01-01

    Purpose: To report long-term progression-free survival (PFS) and late-toxicity outcomes in pediatric craniopharyngioma patients treated with IMRT. Patients and methods: Twenty-four children were treated with IMRT to a median dose of 50.4 Gy (range, 49.8–54 Gy). The clinical target volume (CTV) was the gross tumor volume (GTV) with a 1 cm margin. The planning target volume (PTV) was the CTV with a 3–5 mm margin. Median follow-up was 107.3 months. Results: The 5- and 10-year PFS rates were 65.8% and 60.7%. The 5- and 10-year cystic PFS rates were 70.2% and 65.2% while the 5- and 10-year solid PFS were the same at 90.7%. Endocrinopathy was seen in 42% at initial diagnosis and in 74% after surgical intervention, prior to IMRT. Hypothalamic dysfunction and visual deficits were associated with increasing PTV and number of surgical interventions. Conclusions: IMRT is a viable treatment option for pediatric craniopharyngioma. Despite the use of IMRT, majority of the craniopharyngioma patients experienced long-term toxicity, many of which present prior to radiotherapy. Limitations of retrospective analyses on small patient cohort elicit the need for a prospective multi-institutional study to determine the absolute benefit of IMRT in pediatric craniopharyngioma

  11. Does the Method of Weight Loss Effect Long-Term Changes in Weight, Body Composition or Chronic Disease Risk Factors in Overweight or Obese Adults? A Systematic Review

    Science.gov (United States)

    Washburn, Richard A.; Szabo, Amanda N.; Lambourne, Kate; Willis, Erik A.; Ptomey, Lauren T.; Honas, Jeffery J.; Herrmann, Stephen D.; Donnelly, Joseph E.

    2014-01-01

    Background Differences in biological changes from weight loss by energy restriction and/or exercise may be associated with differences in long-term weight loss/regain. Objective To assess the effect of weight loss method on long-term changes in weight, body composition and chronic disease risk factors. Data Sources PubMed and Embase were searched (January 1990-October 2013) for studies with data on the effect of energy restriction, exercise (aerobic and resistance) on long-term weight loss. Twenty articles were included in this review. Study Eligibility Criteria Primary source, peer reviewed randomized trials published in English with an active weight loss period of >6 months, or active weight loss with a follow-up period of any duration, conducted in overweight or obese adults were included. Study Appraisal and Synthesis Methods Considerable heterogeneity across trials existed for important study parameters, therefore a meta-analysis was considered inappropriate. Results were synthesized and grouped by comparisons (e.g. diet vs. aerobic exercise, diet vs. diet + aerobic exercise etc.) and study design (long-term or weight loss/follow-up). Results Forty percent of trials reported significantly greater long-term weight loss with diet compared with aerobic exercise, while results for differences in weight regain were inconclusive. Diet+aerobic exercise resulted in significantly greater weight loss than diet alone in 50% of trials. However, weight regain (∼55% of loss) was similar in diet and diet+aerobic exercise groups. Fat-free mass tended to be preserved when interventions included exercise. PMID:25333384

  12. Long-Term Collections

    CERN Multimedia

    Comité des collectes à long terme

    2011-01-01

    It is the time of the year when our fireman colleagues go around the laboratory for their traditional calendars sale. A part of the money of the sales will be donated in favour of the long-term collections. We hope that you will welcome them warmly.

  13. Risk for development of severe liver disease in lean patients with nonalcoholic fatty liver disease: A long-term follow-up study.

    Science.gov (United States)

    Hagström, Hannes; Nasr, Patrik; Ekstedt, Mattias; Hammar, Ulf; Stål, Per; Hultcrantz, Rolf; Kechagias, Stergios

    2018-01-01

    Most patients with nonalcoholic fatty liver disease (NAFLD) are overweight or obese. However, a significant proportion of patients have a normal body mass index (BMI), denoted as lean NAFLD. The long-term prognosis of lean NAFLD is unclear. We conducted a cohort study of 646 patients with biopsy-proven NAFLD. Patients were defined as lean (BMI lean and nonlean NAFLD. Lean NAFLD was seen in 19% of patients, while 52% were overweight and 29% were obese. Patients with lean NAFLD were older, had lower transaminases, lower stages of fibrosis, and lower prevalence of nonalcoholic steatohepatitis at baseline compared to patients with a higher BMI. During a mean follow-up of 19.9 years (range 0.4-40 years) representing 12,631 person years and compared to patients who were overweight, patients with lean NAFLD had no increased risk for overall mortality (hazard ratio 1.06; P =  0.73) while an increased risk for development of severe liver disease was found (hazard ratio 2.69; P =  0.007). Conclusion : Although patients with lean NAFLD have lower stages of fibrosis, they are at higher risk for development of severe liver disease compared to patients with NAFLD and a higher BMI, independent of available confounders. ( Hepatology Communications 2018;2:48-57).

  14. Definitive Radiotherapy for Ewing Tumors of Extremities and Pelvis: Long-Term Disease Control, Limb Function, and Treatment Toxicity

    International Nuclear Information System (INIS)

    Indelicato, Daniel J.; Keole, Sameer R.; Shahlaee, Amir H.; Shi Wenyin; Morris, Christopher G.; Marcus, Robert B.

    2008-01-01

    Purpose: More than 70% of Ewing tumors occur in the extremities and pelvis. This study identified factors influencing local control and functional outcomes after management with definitive radiotherapy (RT). Patients and Methods: A total of 75 patients with a localized Ewing tumor of the extremity or pelvis were treated with definitive RT at the University of Florida between 1970 and 2006 (lower extremity tumors in 30, pelvic tumors in 26, and upper extremity tumors in 19). RT was performed on a once-daily (40%) or twice-daily (60%) basis. The median dose was 55.2 Gy in 1.8-Gy daily fractions or 55.0 Gy in 1.2-Gy twice-daily fractions. The median observed follow-up was 4.7 years. Functional outcome was assessed using the Toronto Extremity Salvage Score. Results: The 10-year actuarial overall survival, cause-specific survival, freedom from relapse, and local control rate was 48%, 48%, 42%, and 71%, respectively. Of the 72 patients, 3 required salvage amputation. Inferior cause-specific survival was associated with larger tumors (81% for tumors 3 . Conclusions: Limb preservation was effectively achieved through definitive RT. Treating limited field sizes with hyperfractionated high-energy RT could minimize long-term complications and provides superior functional outcomes

  15. Primary tacrolimus (FK506) therapy and the long-term risk of post-transplant lymphoproliferative disease in pediatric liver transplant recipients.

    Science.gov (United States)

    Cacciarelli, T V; Reyes, J; Jaffe, R; Mazariegos, G V; Jain, A; Fung, J J; Green, M

    2001-10-01

    While the overall incidence of post-transplant lymphoproliferative disease (PTLD) in pediatric liver transplant recipients has been reported to be 4-11%, the long-term risk of PTLD associated with primary tacrolimus therapy is unknown. Therefore, in order to determine the incidence and long-term risk of PTLD, the present study examined 131 pediatric recipients who underwent liver transplantation (LTx) between October 1989 and December 1991 and received primary tacrolimus therapy. This cohort of children was evaluated over an extended time-period (until December 31 1996) with a mean follow-up of 6.3 yr. Actuarial Kaplan-Meier analysis was utilized to determine the risk of PTLD over time. The overall incidence of PTLD was 13% (17/131) with an average age of 4.3 +/- 0.75 yr at diagnosis. Pretransplant Epstein-Barr virus (EBV) serologies were negative in 82%, positive in 12%, and not available in 6% of the patients. The median time to diagnosis of PTLD post-Tx was 11.9 months (mean 16.4 +/- 3.9, range 1.7-63.0 months). Mean tacrolimus dose and plasma trough level (as evaluated by enzyme-linked immunosorbent assay [ELISA]) at the time of diagnosis was 0.32 +/- 0.06 mg/kg/day and 1.3 +/- 0.3 ng/mL, respectively. The cumulative long-term risk of PTLD was found to increase over time: 3% at 6 months, 8% at 1 yr, 12% at 2 yr, 14% at 3 yr, and 15% at 4 and 5 yr. Mortality from PTLD was 12% (two of 17 patients). Primary tacrolimus use in pediatric LTx has a long-term risk of PTLD approaching 15%, with the majority of episodes (78%) occurring in the first 2 yr, suggesting that intense EBV surveillance should occur early post-transplantation.

  16. Relationships between duplex findings and quality of life in long-term follow-up of patients treated for chronic venous disease.

    Science.gov (United States)

    Huang, Ying; Gloviczki, Peter

    2016-03-01

    Relationships between duplex findings and data on health-related quality of life (QoL) to assess long-term results of treatment of varicose veins and chronic venous insufficiency (CVI) are not well known. The goal of this review was to correlate duplex findings and QoL assessments in clinical studies with long-term follow-up. A review of the English language literature on PUBMED revealed 17 clinical studies, including 9 randomized controlled trials (RCTs), 6 prospective, and 2 retrospective studies that included patients with at least 5-year follow-up after endovenous laser ablation (EVLA), radiofrequency ablation (RFA), ultrasound-guided foam sclerotherapy (UGFS), and traditional superficial venous surgery. At 5 years, great saphenous vein (GSV) occlusion rate on duplex ultrasound ranged from 66% to 82% for EVLA, from 62% to 92% for RFA, from 41% to 58% for UGFS and from 54% to 85% for surgery. Freedom from GSV reflux rates were 82% and 84%, respectively for EVLA and surgery, and ranged between 84% and 95% for RFA. Significant improvements were observed in several domains of generic QoL and in most domains of venous disease-specific QoL, irrespective of the treatment. In at least one RCT, CIVIQ scores correlated well with abnormal duplex findings in patients who underwent treatment with UGFS. In another RCT, long-term AVVQ was significantly better after surgery as compared with UGFS similar to results of duplex findings. Analysis of the available literature confirmed that all four techniques were effective in the abolishment of reflux or obliteration of the GSV. Moreover, well-designed RCTs with large sample size are needed to produce robust long-term data on clinical outcome after treatment of varicose veins and CVI and to better understand the relationships between duplex-derived data and QoL assessments. © The Author(s) 2016.

  17. Usefulness of desmopressin testing to predict relapse during long-term follow-up in patients in remission from Cushing’s disease

    Directory of Open Access Journals (Sweden)

    Alberto Giacinto Ambrogio

    2017-11-01

    Full Text Available Recurrence of Cushing’s disease after successful transsphenoidal surgery occurs in some 30% of the patients and the response to desmopressin shortly after surgery has been proposed as a marker for disease recurrence. The aim of the present study was to evaluate the response to desmopressin over time after surgery. We tested 56 patients with Cushing’s disease in remission after transsphenoidal surgery with desmopressin for up to 20 years after surgery. The ACTH and cortisol response to desmopressin over time was evaluated in patients on long-term remission or undergoing relapse; an increase by at least 27 pg/mL in ACTH levels identified responders. The vast majority of patients who underwent successful adenomectomy failed to respond to desmopressin after surgery and this response pattern was maintained over time in patients on long-term remission. Conversely, a response to desmopressin reappeared in patients who subsequently developed a recurrence of Cushing’s disease, even years prior to frank hypercortisolism. It appears therefore that a change in the response pattern to desmopressin proves predictive of recurrence of Cushing’s disease and may indicate which patients require close monitoring.

  18. Quantifying the benefits of achieving or maintaining long-term low risk profile for cardiovascular disease : The doetinchem cohort study

    NARCIS (Netherlands)

    Hulsegge, Gerben; Smit, Henriëtte A.; Van Der Schouw, Yvonne T.; Daviglus, Martha L.; Verschuren, W. M Monique

    2015-01-01

    Background: Studies investigating the relation between risk profiles and cardiovascular disease have measured risk at baseline only. We investigated maintenance and changes of risk profiles over time and their potential impact on incident cardiovascular disease. Design: Population-based cohort

  19. Quantifying the benefits of achieving or maintaining long-term low risk profile for cardiovascular disease: The Doetinchem Cohort Study

    NARCIS (Netherlands)

    Hulsegge, G.; Smit, H.A.; van der Schouw, Y.T.; Daviglus, M.L.; Verschuren, W.M.

    2015-01-01

    Background: Studies investigating the relation between risk profiles and cardiovascular disease have measured risk at baseline only. We investigated maintenance and changes of risk profiles over time and their potential impact on incident cardiovascular disease. Design: Population-based cohort

  20. Rate of primary refractory disease in B and T-cell non-Hodgkin's lymphoma: correlation with long-term survival.

    Directory of Open Access Journals (Sweden)

    Corrado Tarella

    Full Text Available BACKGROUND: Primary refractory disease is a main challenge in the management of non-Hodgkin's Lymphoma (NHL. This survey was performed to define the rate of refractory disease to first-line therapy in B and T-cell NHL subtypes and the long-term survival of primary refractory compared to primary responsive patients. METHODS: Medical records were reviewed of 3,106 patients who had undergone primary treatment for NHL between 1982 and 2012, at the Hematology Centers of Torino and Bergamo, Italy. Primary treatment included CHOP or CHOP-like regimens (63.2%, intensive therapy with autograft (16.9%, or other therapies (19.9%. Among B-cell NHL, 1,356 (47.8% received first-line chemotherapy with rituximab. Refractory disease was defined as stable/progressive disease, or transient response with disease progression within six months. RESULTS: Overall, 690 (22.2% patients showed primary refractory disease, with a higher incidence amongst T-cell compared to B-cell NHL (41.9% vs. 20.5%, respectively, p<0.001. Several other clinico-pathological factors at presentation were variably associated with refractory disease, including histological aggressive disease, unfavorable clinical presentation, Bone Marrow involvement, low lymphocyte/monocyte ration and male gender. Amongst B-cell NHL, the addition of rituximab was associated with a marked reduction of refractory disease (13.6% vs. 26.7% for non-supplemented chemotherapy, p<0.001. Overall, primary responsive patients had a median survival of 19.8 years, compared to 1.3 yr. for refractory patients. A prolonged survival was consistently observed in all primary responsive patients regardless of the histology. The long life expectancy of primary responsive patients was documented in both series managed before and after 2.000. Response to first line therapy resulted by far the most predictive factor for long-term outcome (HR for primary refractory disease: 16.52, p<0.001. CONCLUSION: Chemosensitivity to primary

  1. Effect of a very low-protein diet on outcomes: long-term follow-up of the Modification of Diet in Renal Disease (MDRD) Study.

    Science.gov (United States)

    Menon, Vandana; Kopple, Joel D; Wang, Xuelei; Beck, Gerald J; Collins, Allan J; Kusek, John W; Greene, Tom; Levey, Andrew S; Sarnak, Mark J

    2009-02-01

    The long-term effect of a very low-protein diet on the progression of kidney disease is unknown. We examined the effect of a very low-protein diet on the development of kidney failure and death during long-term follow-up of the Modification of Diet in Renal Disease (MDRD) Study. Long-term follow-up of study B of the MDRD Study (1989-1993). The MDRD Study examined the effects of dietary protein restriction and blood pressure control on progression of kidney disease. This analysis includes 255 trial participants with predominantly stage 4 nondiabetic chronic kidney disease. A low-protein diet (0.58 g/kg/d) versus a very low-protein diet (0.28 g/kg/d) supplemented with a mixture of essential keto acids and amino acids (0.28 g/kg/d). Kidney failure (initiation of dialysis therapy or transplantation) and all-cause mortality until December 31, 2000. Kidney failure developed in 227 (89%) participants, 79 (30.9%) died, and 244 (95.7%) reached the composite outcome of either kidney failure or death. Median duration of follow-up until kidney failure, death, or administrative censoring was 3.2 years, and median time to death was 10.6 years. In the low-protein group, 117 (90.7%) participants developed kidney failure, 30 (23.3%) died, and 124 (96.1%) reached the composite outcome. In the very low-protein group, 110 (87.3%) participants developed kidney failure, 49 (38.9%) died, and 120 (95.2%) reached the composite outcome. After adjustment for a priori-specified covariates, hazard ratios were 0.83 (95% confidence interval, 0.62 to 1.12) for kidney failure, 1.92 (95% confidence interval, 1.15 to 3.20) for death, and 0.89 (95% confidence interval, 0.67 to 1.18) for the composite outcome in the very low-protein diet group compared with the low-protein diet group. Lack of dietary protein measurements during follow-up. In long-term follow-up of the MDRD Study, assignment to a very low-protein diet did not delay progression to kidney failure, but appeared to increase the risk of

  2. Effective antimicrobial stewardship in a long-term care facility through an infectious disease consultation service: keeping a LID on antibiotic use.

    Science.gov (United States)

    Jump, Robin L P; Olds, Danielle M; Seifi, Nasim; Kypriotakis, Georgios; Jury, Lucy A; Peron, Emily P; Hirsch, Amy A; Drawz, Paul E; Watts, Brook; Bonomo, Robert A; Donskey, Curtis J

    2012-12-01

    We introduced a long-term care facility (LTCF) infectious disease (ID) consultation service (LID service) that provides on-site consultations to residents of a Veterans Affairs (VA) LTCF. We determined the impact of the LID service on antimicrobial use and Clostridium difficile infections at the LTCF. A 160-bed VA LTCF. Systemic antimicrobial use and positive C. difficile tests at the LTCF were compared for the 36 months before and the 18 months after the initiation of the ID consultation service through segmented regression analysis of an interrupted time series. Relative to that in the preintervention period, total systemic antibiotic administration decreased by 30% (Peffective means to achieve antimicrobial stewardship.

  3. Long-term outcomes of tissue-based ACTH-antibody assay-guided transsphenoidal resection of pituitary adenomas in Cushing disease.

    Science.gov (United States)

    Erfe, J Mark; Perry, Avital; McClaskey, John; Inzucchi, Silvio E; James, Whitney Sheen; Eid, Tore; Bronen, Richard A; Mahajan, Amit; Huttner, Anita; Santos, Florecita; Spencer, Dennis

    2017-10-13

    OBJECTIVE Cushing disease is caused by a pituitary micro- or macroadenoma that hypersecretes adrenocorticotropic hormone (ACTH), resulting in hypercortisolemia. For decades, transsphenoidal resection (TSR) has been an efficacious treatment but with certain limitations, namely precise tumor localization and complete excision. The authors evaluated the novel use of a double-antibody sandwich assay for the real-time quantitation of ACTH in resected pituitary specimens with the goals of augmenting pathological diagnosis and ultimately improving long-term patient outcome. METHODS This study involved a retrospective review of records and an analysis of assay values, pathology slides, and MRI studies of patients with Cushing disease who had undergone TSR in the period from 2009 to 2014 and had at least 1 year of follow-up in coordination with an endocrinologist. In the operating room, biopsy specimens from the patients had been analyzed for tissue ACTH concentration. Additional samples were simultaneously sent for frozen-section pathological analysis. The ACTH assay performance was compared against pathology assessments of surgical tumor samples using receiver operating characteristic (ROC) analysis and against pre- and postoperative MRI studies. RESULTS Fourteen patients underwent TSR with guidance by ACTH-antibody assay and pathological assessment of 127 biopsy samples and were followed up for an average of 3 years. The ACTH threshold for discriminating adenomatous from normal tissue was 290,000 pg/mg of tissue, based on jointly maximized sensitivity (95.0%) and specificity (71.3%). Lateralization discordance between preoperative MRI studies and surgical visualization was noted in 3 patients, confirming the impression that MRI alone may not achieve optimal localization. A majority of the patients (85.7%) attained long-term disease remission based on urinary free cortisol levels, plasma cortisol levels, and long-term corticosteroid therapy. Comparisons of patient

  4. Risk of death from cardiovascular disease associated with low-level arsenic exposure among long-term smokers in a US population-based study

    International Nuclear Information System (INIS)

    Farzan, Shohreh F.; Chen, Yu; Rees, Judy R.; Zens, M. Scot; Karagas, Margaret R.

    2015-01-01

    High levels of arsenic exposure have been associated with increases in cardiovascular disease risk. However, studies of arsenic's effects at lower exposure levels are limited and few prospective studies exist in the United States using long-term arsenic exposure biomarkers. We conducted a prospective analysis of the association between toenail arsenic and cardiovascular disease mortality using longitudinal data collected on 3939 participants in the New Hampshire Skin Cancer Study. Using Cox proportional hazard models adjusted for potential confounders, we estimated hazard ratios and 95% confidence intervals associated with the risk of death from any cardiovascular disease, ischemic heart disease, and stroke, in relation to natural-log transformed toenail arsenic concentrations. In this US population, although we observed no overall association, arsenic exposure measured from toenail clipping samples was related to an increased risk of ischemic heart disease mortality among long-term smokers (as reported at baseline), with increased hazard ratios among individuals with ≥ 31 total smoking years (HR: 1.52, 95% CI: 1.02, 2.27), ≥ 30 pack-years (HR: 1.66, 95% CI: 1.12, 2.45), and among current smokers (HR: 1.69, 95% CI: 1.04, 2.75). These results are consistent with evidence from more highly exposed populations suggesting a synergistic relationship between arsenic exposure and smoking on health outcomes and support a role for lower-level arsenic exposure in ischemic heart disease mortality. - Highlights: • Arsenic (As) has been associated with increased cardiovascular disease (CVD) risk. • Little is known about CVD effects at lower levels of As exposure common in the US. • Few have investigated the joint effects of As and smoking on CVD in US adults. • We examine chronic low-level As exposure and smoking in relation to CVD mortality. • Arsenic exposure may increase ischemic heart disease mortality among smokers in US

  5. Risk of death from cardiovascular disease associated with low-level arsenic exposure among long-term smokers in a US population-based study

    Energy Technology Data Exchange (ETDEWEB)

    Farzan, Shohreh F. [Department of Epidemiology, Geisel School of Medicine at Dartmouth, Lebanon, NH (United States); Departments of Population Health and Environmental Medicine, New York University School of Medicine, New York, NY (United States); Chen, Yu [Departments of Population Health and Environmental Medicine, New York University School of Medicine, New York, NY (United States); Rees, Judy R.; Zens, M. Scot [Department of Epidemiology, Geisel School of Medicine at Dartmouth, Lebanon, NH (United States); Karagas, Margaret R., E-mail: margaret.r.karagas@dartmouth.edu [Department of Epidemiology, Geisel School of Medicine at Dartmouth, Lebanon, NH (United States)

    2015-09-01

    High levels of arsenic exposure have been associated with increases in cardiovascular disease risk. However, studies of arsenic's effects at lower exposure levels are limited and few prospective studies exist in the United States using long-term arsenic exposure biomarkers. We conducted a prospective analysis of the association between toenail arsenic and cardiovascular disease mortality using longitudinal data collected on 3939 participants in the New Hampshire Skin Cancer Study. Using Cox proportional hazard models adjusted for potential confounders, we estimated hazard ratios and 95% confidence intervals associated with the risk of death from any cardiovascular disease, ischemic heart disease, and stroke, in relation to natural-log transformed toenail arsenic concentrations. In this US population, although we observed no overall association, arsenic exposure measured from toenail clipping samples was related to an increased risk of ischemic heart disease mortality among long-term smokers (as reported at baseline), with increased hazard ratios among individuals with ≥ 31 total smoking years (HR: 1.52, 95% CI: 1.02, 2.27), ≥ 30 pack-years (HR: 1.66, 95% CI: 1.12, 2.45), and among current smokers (HR: 1.69, 95% CI: 1.04, 2.75). These results are consistent with evidence from more highly exposed populations suggesting a synergistic relationship between arsenic exposure and smoking on health outcomes and support a role for lower-level arsenic exposure in ischemic heart disease mortality. - Highlights: • Arsenic (As) has been associated with increased cardiovascular disease (CVD) risk. • Little is known about CVD effects at lower levels of As exposure common in the US. • Few have investigated the joint effects of As and smoking on CVD in US adults. • We examine chronic low-level As exposure and smoking in relation to CVD mortality. • Arsenic exposure may increase ischemic heart disease mortality among smokers in US.

  6. No increased risk of hepatocellular carcinoma in cirrhosis due to Wilson disease during long-term follow-up

    NARCIS (Netherlands)

    van Meer, Suzanne; de Man, Robert A.; van den Berg, Aad P.; Houwen, Roderick H. J.; Linn, Francisca H. H.; van Oijen, Martijn G. H.; Siersema, Peter D.; van Erpecum, Karel J.

    Background and AimsData on risk of hepatocellular carcinoma (HCC) in patients with Wilson disease are scarce. We determine HCC risk in a well-defined cohort of Wilson patients. MethodsAll patients with a confirmed diagnosis of Wilson disease (Leipzig score4) in three Dutch university referral

  7. N-terminal pro-B-type natriuretic peptide and long-term mortality in stable coronary heart disease

    DEFF Research Database (Denmark)

    Kragelund, Charlotte; Grønning, Bjørn; Køber, Lars

    2005-01-01

    quartile was 2.4 (95 percent confidence interval, 1.5 to 4.0; Pfamily history with respect to ischemic heart disease; the presence or absence of a history......-term mortality in patients with stable coronary disease and provides prognostic information above and beyond that provided by conventional cardiovascular risk factors and the degree of left ventricular systolic dysfunction....

  8. Long-term predictive value of postsurgical cortisol concentrations for cure and risk of recurrence in Cushing's disease

    NARCIS (Netherlands)

    Pereira, Alberto M.; van Aken, Maarten O.; van Dulken, Hans; Schutte, Pieter J.; Biermasz, Nienke R.; Smit, Jan W. A.; Roelfsema, Ferdinand; Romijn, Johannes A.

    2003-01-01

    We assessed the value of postoperative plasma cortisol concentrations to predict cure and recurrence of Cushing's disease after transsphenoidal surgery (TS). Seventy-eight of 80 consecutive patients treated by TS for Cushing's disease were evaluated. TS cured 72% (n = 56) of the patients. Two weeks

  9. The roles of self-efficacy and motivation in the prediction of short- and long-term adherence to exercise among patients with coronary heart disease.

    Science.gov (United States)

    Slovinec D'Angelo, Monika E; Pelletier, Luc G; Reid, Robert D; Huta, Veronika

    2014-11-01

    Poor adherence to regular exercise is a documented challenge among people with heart disease. Identifying key determinants of exercise adherence and distinguishing between the processes driving short- and long-term adherence to regular exercise is a valuable endeavor. The purpose of the present study was to test a model of exercise behavior change, which incorporates motivational orientations and self-efficacy for exercise behavior, in the prediction of short- and long-term exercise adherence. Male and female patients (N = 801) hospitalized for coronary heart disease were recruited from 3 tertiary care cardiac centers and followed for a period of 1 year after hospital discharge. A prospective, longitudinal design was used to examine the roles of motivation and self-efficacy (measured at recruitment and at 2 and 6 months after discharge) in the prediction of exercise behavior at 6 and 12 months. Baseline measures of exercise and clinical and demographic covariates were included in the analyses. Structural equation modeling showed that both autonomous motivation and self-efficacy were important determinants of short-term (6-month) exercise behavior regulation, but that only autonomous motivation remained a significant predictor of long-term (12-month) exercise behavior. Self-efficacy partially mediated the relationship between motivation for exercise and 6-month exercise behavior. This research confirmed the roles of autonomous motivation and self-efficacy in the health behavior change process and emphasized the key function of autonomous motivation in exercise maintenance. Theoretical and cardiac rehabilitation program applications of this research are discussed. PsycINFO Database Record (c) 2014 APA, all rights reserved.

  10. Repeat interventions as a long-term treatment strategy in the management of progressive coronary artery disease.

    NARCIS (Netherlands)

    K.G. Lehmann (Kenneth); P.W.J.C. Serruys (Patrick); M.J.B.M. van den Brand (Marcel); P.J. de Feyter (Pim); A.C.P. Maas (Arthur); R.T. van Domburg (Ron)

    1996-01-01

    textabstractObjectives. This study investigates whether repeat coronary interventions, applied over an extended time period, can successfully curtail the progression of ischemic symptoms and angiographic lumen narrowing. Background. Coronary artery disease is a chronic and generally progressive

  11. Impact of chronic kidney disease on long-term ischemic and bleeding outcomes in medically managed patients with acute coronary syndromes

    DEFF Research Database (Denmark)

    Melloni, Chiara; Cornel, Jan H; Hafley, Gail

    2016-01-01

    AIMS: We aimed to study the relationship of chronic kidney disease stages with long-term ischemic and bleeding outcomes in medically managed acute coronary syndrome patients and the influence of more potent antiplatelet therapies on platelet reactivity by chronic kidney disease stage. METHODS...... AND RESULTS: We estimated creatinine clearance for 8953 medically managed acute coronary syndrome patients enrolled in the Targeted Platelet Inhibition to Clarify the Optimal Strategy to Medically Manage Acute Coronary Syndromes trial. Patients were classified by chronic kidney disease stage: normal renal...... function/mild (creatinine clearance >60 mL/min); moderate (creatinine clearance 30-60 mL/min); severe (creatinine clearance event rates through 30 months were evaluated for ischemic (cardiovascular death, myocardial infarction or stroke; primary end point) and bleeding (Global Use...

  12. EXAMINATION OF ELECTROPHYSIOLOGICAL PARAMETERS OF THE ATRIUMS IN PATIENTS WITH LONG-TERM PERSISTENT FORM OF ATRIAL FIBRILLATION AND VALVULAR HEART DISEASE

    Directory of Open Access Journals (Sweden)

    A. A. Kulikov

    2017-01-01

    Full Text Available The study objective is to examine electrophysiological parameters of atrial myocardium, characteristics of atrioventricular conduction, and potential factors affecting recurrent atrial fibrillation (AF in patients with persistent and long-term persistent forms of AF prior to the Labirynth IIIB surgery with single-step correction of valvular heart disease.Materials and methods. The study included 100 adults (48 men, 52 women with persistent and long-term persistent forms of AF and different valvular heart diseases. Mean patient age was 59 years. Mean AF duration was 4 years. All patients were prescribed antiarrhythmic therapy but it proved ineffective. In 15 % of patients, restoration of the sinus rhythm was attempted through electrical cardioversion but long-term control of the sinus rhythm wasn’t achieved. All patients were diagnosed with organic pathology of the mitral valve. Also, in 80 % of patients, relative insufficiency of the tricuspid valve was detected. Chronic heart failure functional class per NYHA was III. Size of the left atrium was 5 cm, mean left ventricular ejection fraction was 61 %. All patients underwent electrical cardioversion. After successful restoration of the sinus rhythm, endocardial electrophysiology study (EES of the heart was performed. Then, correction of valvular pathologies and the Labyrinth IIIB surgery were performed. Results. Examination of refractoriness of different parts of the atriums has shown that effective refractory period (ERP of the atrioventricular node was minimal compared to other parts of the atriums. Maximal ERP duration was observed in the upper part of the right atrium. Therefore, in patients with long history of AF, heterogeneity of atrial myocardium ERP duration is observed. In 17 % of patients, atrial vulnerability was detected. The area of atrial vulnerability was always associated with ERP. Its duration in patients with atrial vulnerability was significantly higher.Conclusion. Long-term

  13. Prediction of remission in Graves` disease treated with long-term carbimazole therapy: evaluation of technetium-99m thyroid uptake and TSH concentrations as prognostic indicators

    Energy Technology Data Exchange (ETDEWEB)

    Prakash, R. [Dept. of Nuclear Medicine, Batra Hospital, New Delhi (India)

    1996-02-01

    Computerized technetium-99m thyroid uptake and thyrotropin (TSH) estimation using a sensitive immunoradiometric assay were performed at presentation and following completion of an 18-month course of antithyroid drug therapy in 45 patients with Graves` disease. All patients had increased {sup 99m}Tc thyroid uptake and subnormal TSH levels before the start of treatment. Twentytwo patients developed recurrent hyperthyroidism in a 3-year follow-up period. Of the 22 patients with relapse, 20 had had a persistently increased {sup 99m}Tc thyroid uptake at the end of the course of carbimazole treatment, whereas TSH had remained subnormal in 18 of the 22. All 23 patients who remained in remission until the end of the 3-year follow-up had had normal {sup 99m}Tc thyroid uptake following completion of antihyroid drug treatment. TSH levels had reverted to normal in 19 cases, but remained subnormal in four cases in this group at the end of treatment. The results suggest a high likelihood of relapse in patients who have persistently increased {sup 99m}Tc thyroid uptake and subnormal TSH after a full course of carbimazole treatment. Patients whose {sup 99m}Tc thyroid uptake and TSH levels have reverted to normal are likely to stay in long-term remission. Assessment of {sup 99m}Tc thyroid uptake and TSH levels following completion of carbimazole therapy for Graves` disease offers useful information regarding long-term prognosis. (orig.)

  14. Long-Term Collections

    CERN Multimedia

    Staff Association

    2016-01-01

    45 years helping in developing countries! CERN personnel have been helping the least fortunate people on the planet since 1971. How? With the Long-Term Collections! Dear Colleagues, The Staff Association’s Long-Term Collections (LTC) Committee is delighted to share this important milestone in the life of our Laboratory with you. Indeed, whilst the name of CERN is known worldwide for scientific discoveries, it also shines in the many humanitarian projects which have been supported by the LTC since 1971. Several schools and clinics, far and wide, carry its logo... Over the past 45 years, 74 projects have been supported (9 of which are still ongoing). This all came from a group of colleagues who wanted to share a little of what life offered them here at CERN, in this haven of mutual understanding, peace and security, with those who were less fortunate elsewhere. Thus, the LTC were born... Since then, we have worked as a team to maintain the dream of these visionaries, with the help of regular donat...

  15. Long-Term Collection

    CERN Multimedia

    Staff Association

    2016-01-01

    Dear Colleagues, As previously announced in Echo (No. 254), your delegates took action to draw attention to the projects of the Long-Term Collections (LTC), the humanitarian body of the CERN Staff Association. On Tuesday, 11 October, at noon, small Z-Cards were widely distributed at the entrances of CERN restaurants and we thank you all for your interest. We hope to have achieved an important part of our goal, which was to inform you, convince you and find new supporters among you. We will find out in the next few days! An exhibition of the LTC was also set up in the Main Building for the entire week. The Staff Association wants to celebrate the occasion of the Long-Term Collection’s 45th anniversary at CERN because, ever since 1971, CERN personnel have showed great support in helping the least fortunate people on the planet in a variety of ways according to their needs. On a regular basis, joint fundraising appeals are made with the Directorate to help the victims of natural disasters around th...

  16. Collectes à long terme

    CERN Multimedia

    Collectes à long terme

    2014-01-01

    En cette fin d’année 2014 qui approche à grands pas, le Comité des Collectes à Long Terme remercie chaleureusement ses fidèles donatrices et donateurs réguliers pour leurs contributions à nos actions en faveur des plus démunis de notre planète. C’est très important, pour notre Comité, de pouvoir compter sur l’appui assidu que vous nous apportez. Depuis plus de 40 ans maintenant, le modèle des CLT est basé principalement sur des actions à long terme (soit une aide pendant 4-5 ans par projet, mais plus parfois selon les circonstances), et sa planification demande une grande régularité de ses soutiens financiers. Grand MERCI à vous ! D’autres dons nous parviennent au cours de l’année, et ils sont aussi les bienvenus. En particulier, nous tenons à remercier...

  17. Secondary hip dysplasia in Legg-Calvé-Perthes disease - a long-term case-control study

    DEFF Research Database (Denmark)

    Froberg, Lonnie; Christensen, Finn; Pedersen, Niels Wisbech

    Poor long-time results in Legg-Calvé-Perthes disease (LCP) have in previous reports been attributed to the presence of degenerative joint disease due to deformities of the femoral head. Reports have stated that hip dysplasia (HD) pre-dispose to hip osteoarthritis (OA). The increased risk of OA...... excluded, however we do not have any reason to believe that they were excluded systematically. The reviewed Stulberg classification was used. The advantages of the classification included the simplicity of application and reliability with good inter- and intraobserver agreement. Previous report has also...

  18. Long-term follow-up of a patient cohort with Legg-Calvé-Perthes disease

    DEFF Research Database (Denmark)

    Froberg, Lonnie; Christensen, Finn; Pedersen, Niels W

    2011-01-01

    system were applied. A significant correlation between the Herring group and Stulberg was found (P=0.03). There was a significant correlation between age at onset of the disease and Stulberg outcome (P=0.05). The Herring classification correlated to the Stulberg outcome. An age of 7 years or more......The purpose of this study was to evaluate prognostic risk factors related to a poor radiological outcome. The study consisted of 74 patients with Legg-Calvé-Perthes disease treated using a Thomas splint. The modified Herring lateral pillar classification and the reviewed Stulberg classification...

  19. Surgical correction of Peyronie's disease via tunica albuginea plication or partial plaque excision with pericardial graft: long-term follow up.

    Science.gov (United States)

    Taylor, Frederick L; Levine, Laurence A

    2008-09-01

    Limited publications exist regarding long-term outcomes of surgical correction for Peyronie's Disease (PD). To report on long-term postoperative parameters including rigidity, curvature, length, sensation, function, and patient satisfaction in men with PD treated surgically via Tunica Albuginea Plication (TAP) or Partial Plaque Excision with Tutoplast Human Pericardial Grafting (PEG). Objective and subjective data regarding patients who underwent either TAP or PEG. We report on 142 patients (61 TAP and 81 PEG) with both objective data and subjective patient reports on their postoperative experience. Patients underwent either TAP or PEG following our previously published algorithm. Data was collected via chart review and an internally generated survey, in which patients were asked about their rigidity, straightness, penile length, sensation, sexual function and satisfaction. Average follow up for TAP patients was 72 months (range 8-147) and 58 months (range 6-185) for PEG patients. At survey time, 93% of TAP and 91% of PEG patients reported curvatures of less than 30o. Rigidity was reportedly as good as or better than preoperative in 81% of TAP and 68% of PEG patients, and was adequate for coitus in 90% of TAP and 79% of PEG patients with or without the use of PDE5i. Objective flaccid stretched penile length measurements obtained pre and postoperatively show an average overall length gain of 0.6 cm (range -3.5-3.5) for TAP and 0.2 cm (range -1.5-2.0) for PEG patients. Sensation was reportedly as good as or better than preoperative in 69% of both TAP and PEG patients; 98% of TAP patients and 90% of PEG are able to achieve orgasm. 82% of TAP patients and 75% of PEG patients were either very satisfied or satisfied. Our long-term results support both TAP and PEG as durable surgical therapy for men with clinically significant PD.

  20. Roles of Vascular and Metabolic Components in Cognitive Dysfunction of Alzheimer disease: Short- and Long-term Modification by Non-genetic Risk Factors

    Directory of Open Access Journals (Sweden)

    Naoyuki eSato

    2013-11-01

    Full Text Available It is well known that a specific set of genetic and non-genetic risk factors contributes to the onset of Alzheimer disease (AD. Non-genetic risk factors include diabetes, hypertension in mid-life, and probably dyslipidemia in mid-life. This review focuses on the vascular and metabolic components of non-genetic risk factors. The mechanisms whereby non-genetic risk factors modify cognitive dysfunction are divided into four components, short- and long-term effects of vascular and metabolic factors. These consist of 1 compromised vascular reactivity, 2 vascular lesions, 3 hypo/hyperglycemia, and 4 exacerbated AD histopathological features, respectively. Vascular factors compromise cerebrovascular reactivity in response to neuronal activity and also cause irreversible vascular lesions. On the other hand, representative short-term effects of metabolic factors on cognitive dysfunction occur due to hypoglycemia or hyperglycemia. Non-genetic risk factors also modify the pathological manifestations of AD in the long-term. Therefore, vascular and metabolic factors contribute to aggravation of cognitive dysfunction in AD through short-term and long-term effects. Beta-amyloid could be involved in both vascular and metabolic components. It might be beneficial to support treatment in AD patients by appropriate therapeutic management of non-genetic risk factors, considering the contributions of these four elements to the manifestation of cognitive dysfunction in individual patients, though all components are not always present. It should be clarified how these four components interact with each other. To answer this question, a clinical prospective study that follows up clinical features with respect to these four components: 1 functional MRI or SPECT for cerebrovascular reactivity, 2 MRI for ischemic lesions and atrophy, 3 clinical episodes of hypoglycemia and hyperglycemia, 4 amyloid-PET and tau-PET for pathological features of AD, would be required.

  1. Effect of Warfarin Treatment on Survival of Patients With Pulmonary Arterial Hypertension (PAH) in the Registry to Evaluate Early and Long-Term PAH Disease Management (REVEAL).

    Science.gov (United States)

    Preston, Ioana R; Roberts, Kari E; Miller, Dave P; Sen, Ginny P; Selej, Mona; Benton, Wade W; Hill, Nicholas S; Farber, Harrison W

    2015-12-22

    Long-term anticoagulation is recommended in idiopathic pulmonary arterial hypertension (IPAH). In contrast, limited data support anticoagulation in pulmonary arterial hypertension (PAH) associated with systemic sclerosis (SSc-PAH). We assessed the effect of warfarin anticoagulation on survival in IPAH and SSc-PAH patients enrolled in Registry to Evaluate Early and Long-term PAH Disease Management (REVEAL), a longitudinal registry of group I PAH. Patients who initiated warfarin on study (n=187) were matched 1:1 with patients never on warfarin, by enrollment site, etiology, and diagnosis status. Descriptive analyses were conducted to compare warfarin users and nonusers by etiology. Survival analyses with and without risk adjustment were performed from the time of warfarin initiation or a corresponding quarterly update in matched pairs to avoid immortal time bias. Time-varying covariate models were used as sensitivity analyses. Mean warfarin treatment was 1 year; mean international normalized ratios were 1.9 (IPAH) and 2.0 (SSc-PAH). Two-thirds of patients initiating warfarin discontinued treatment before the last study assessment. There was no survival difference with warfarin in IPAH patients (adjusted hazard ratio, 1.37; P=0.21) or in SSc-PAH patients (adjusted hazard ratio, 1.60; P=0.15) in comparison with matched controls. However, SSc-PAH patients receiving warfarin within the previous year (hazard ratio, 1.57; P=0.031) or any time postbaseline (hazard ratio, 1.49; P=0.046) had increased mortality in comparison with warfarin-naïve patients. No significant survival advantage was observed in IPAH patients who started warfarin. In SSc-PAH patients, long-term warfarin was associated with poorer survival than in patients not receiving warfarin, even after adjusting for confounders. URL: http://www.clinicaltrials.gov. Unique identifier: NCT00370214. © 2015 The Authors.

  2. Roles of vascular and metabolic components in cognitive dysfunction of Alzheimer disease: short- and long-term modification by non-genetic risk factors.

    Science.gov (United States)

    Sato, Naoyuki; Morishita, Ryuichi

    2013-11-05

    It is well known that a specific set of genetic and non-genetic risk factors contributes to the onset of Alzheimer disease (AD). Non-genetic risk factors include diabetes, hypertension in mid-life, and probably dyslipidemia in mid-life. This review focuses on the vascular and metabolic components of non-genetic risk factors. The mechanisms whereby non-genetic risk factors modify cognitive dysfunction are divided into four components, short- and long-term effects of vascular and metabolic factors. These consist of (1) compromised vascular reactivity, (2) vascular lesions, (3) hypo/hyperglycemia, and (4) exacerbated AD histopathological features, respectively. Vascular factors compromise cerebrovascular reactivity in response to neuronal activity and also cause irreversible vascular lesions. On the other hand, representative short-term effects of metabolic factors on cognitive dysfunction occur due to hypoglycemia or hyperglycemia. Non-genetic risk factors also modify the pathological manifestations of AD in the long-term. Therefore, vascular and metabolic factors contribute to aggravation of cognitive dysfunction in AD through short-term and long-term effects. β-amyloid could be involved in both vascular and metabolic components. It might be beneficial to support treatment in AD patients by appropriate therapeutic management of non-genetic risk factors, considering the contributions of these four elements to the manifestation of cognitive dysfunction in individual patients, though all components are not always present. It should be clarified how these four components interact with each other. To answer this question, a clinical prospective study that follows up clinical features with respect to these four components: (1) functional MRI or SPECT for cerebrovascular reactivity, (2) MRI for ischemic lesions and atrophy, (3) clinical episodes of hypoglycemia and hyperglycemia, (4) amyloid-PET and tau-PET for pathological features of AD, would be required.

  3. Long-term safety and efficacy of deferasirox (Exjade) for up to 5 years in transfusional iron-overloaded patients with sickle cell disease.

    Science.gov (United States)

    Vichinsky, Elliott; Bernaudin, Françoise; Forni, Gian Luca; Gardner, Renee; Hassell, Kathryn; Heeney, Matthew M; Inusa, Baba; Kutlar, Abdullah; Lane, Peter; Mathias, Liesl; Porter, John; Tebbi, Cameron; Wilson, Felicia; Griffel, Louis; Deng, Wei; Giannone, Vanessa; Coates, Thomas

    2011-08-01

    To date, there is a lack of long-term safety and efficacy data for iron chelation therapy in transfusion-dependent patients with sickle cell disease (SCD). To evaluate the long-term safety and efficacy of deferasirox (a once-daily oral iron chelator), patients with SCD completing a 1-year, Phase II, randomized, deferoxamine (DFO)-controlled study entered a 4-year extension, continuing to receive deferasirox, or switching from DFO to deferasirox. Average actual deferasirox dose was 19·4 ± 6·3 mg/kg per d. Of 185 patients who received at least one deferasirox dose, 33·5% completed the 5-year study. The most common reasons for discontinuation were withdrawal of consent (23·8%), lost to follow-up (9·2%) and adverse events (AEs) (7·6%). Investigator-assessed drug-related AEs were predominantly gastrointestinal [including nausea (14·6%), diarrhoea (10·8%)], mild-to-moderate and transient in nature. Creatinine clearance remained within the normal range throughout the study. Despite conservative initial dosing, serum ferritin levels in patients with ≥ 4 years deferasirox exposure significantly decreased by -591 μg/l (95% confidence intervals, -1411, -280 μg/l; P = 0·027; n = 67). Long-term deferasirox treatment for up to 5 years had a clinically acceptable safety profile, including maintenance of normal renal function, in patients with SCD. Iron burden was substantially reduced with appropriate dosing in patients treated for at least 4 years. © 2011 Blackwell Publishing Ltd.

  4. Long-term safety and efficacy of deferasirox (Exjade®) for up to 5 years in transfusional iron-overloaded patients with sickle cell disease

    Science.gov (United States)

    Vichinsky, Elliott; Bernaudin, Françoise; Forni, Gian Luca; Gardner, Renee; Hassell, Kathryn; Heeney, Matthew M; Inusa, Baba; Kutlar, Abdullah; Lane, Peter; Mathias, Liesl; Porter, John; Tebbi, Cameron; Wilson, Felicia; Griffel, Louis; Deng, Wei; Giannone, Vanessa; Coates, Thomas

    2011-01-01

    To date, there is a lack of long-term safety and efficacy data for iron chelation therapy in transfusion-dependent patients with sickle cell disease (SCD). To evaluate the long-term safety and efficacy of deferasirox (a once-daily oral iron chelator), patients with SCD completing a 1-year, Phase II, randomized, deferoxamine (DFO)-controlled study entered a 4-year extension, continuing to receive deferasirox, or switching from DFO to deferasirox. Average actual deferasirox dose was 19·4 ± 6·3 mg/kg per d. Of 185 patients who received at least one deferasirox dose, 33·5% completed the 5-year study. The most common reasons for discontinuation were withdrawal of consent (23·8%), lost to follow-up (9·2%) and adverse events (AEs) (7·6%). Investigator-assessed drug-related AEs were predominantly gastrointestinal [including nausea (14·6%), diarrhoea (10·8%)], mild-to-moderate and transient in nature. Creatinine clearance remained within the normal range throughout the study. Despite conservative initial dosing, serum ferritin levels in patients with ≥4 years deferasirox exposure significantly decreased by −591 μg/l (95% confidence intervals, −1411, −280 μg/l; P=0·027; n=67). Long-term deferasirox treatment for up to 5 years had a clinically acceptable safety profile, including maintenance of normal renal function, in patients with SCD. Iron burden was substantially reduced with appropriate dosing in patients treated for at least 4 years. PMID:21592110

  5. Long-term efficacy of aspirin desensitization in aspirin-exacerbated respiratory disease. Review of two clinical cases

    Directory of Open Access Journals (Sweden)

    Julio César Cambray-Gutiérrez

    2016-05-01

    Clinical cases: two patients diagnosed with respiratory disease exacerbated by aspirin, with poor asthma control and need for multiple polypectomies, despite optimal pharmacological management, carrying out protocol desensitization to aspirin (AAS successful, now after 4 years of having carried out, they have adequate asthma control without need for polypectomies with a maintenance dose of aspirin 150 mg/day.

  6. Initial cognitive response to cholinesterase inhibitors and subsequent long-term course in patients with mild Alzheimer's disease

    NARCIS (Netherlands)

    Droogsma, Erika; van Asselt, Dieneke; Diekhuis, Marjolein; Veeger, Nic; van der Hooft, Cornelis; De Deyn, Peter Paul

    Background: Some guidelines recommend to discontinue treatment with cholinesterase inhibitors (ChEIs) in patients with Alzheimer's disease (AD) without an initial response to ChEI treatment. Evidence supporting this recommendation, however, is limited. This study aimed to investigate the relation

  7. Long-term neurodevelopmental outcome after intrauterine transfusion for hemolytic disease of the fetus/newborn: the LOTUS study

    NARCIS (Netherlands)

    Lindenburg, Irene T.; Smits-Wintjens, Vivianne E.; van Klink, Jeanine M.; Verduin, Esther; van Kamp, Inge L.; Walther, Frans J.; Schonewille, Henk; Doxiadis, Ilias I.; Kanhai, Humphrey H.; van Lith, Jan M.; van Zwet, Erik W.; Oepkes, Dick; Brand, Anneke; Lopriore, Enrico

    2012-01-01

    To determine the incidence and risk factors for neurodevelopmental impairment (NDI) in children with hemolytic disease of the fetus/newborn treated with intrauterine transfusion (IUT). Neurodevelopmental outcome in children at least 2 years of age was assessed using standardized tests, including the

  8. Long-term effects of pallidal or subthalamic deep brain stimulation on quality of life in Parkinson's disease

    NARCIS (Netherlands)

    Volkmann, Jens; Albanese, Alberto; Kulisevsky, Jaime; Tornqvist, Aana-Lena; Houeto, Jean-Luc; Pidoux, Bernard; Bonnet, Anne-Marie; Mendes, Alexandre; Benabid, Alim-Louis; Fraix, Valerie; van Blercom, Nadege; Xie, Jing; Obeso, José; Rodriguez-Oroz, Maria Cruz; Guridi, Jurge; Schnitzler, Alfons; Timmermann, Lars; Gironell, Alexandre A.; Molet, Juan; Pascual-Sedano, Benta; Rehncrona, Stig; Moro, Elena; Lang, Anthony C.; Lozano, Andres M.; Bentivoglio, Anna Rita; Scerrati, Massimo; Contarino, Maria Fiorella; Romito, Luigi; Janssens, Marc; Agid, Yves

    2009-01-01

    We assessed the effects of deep brain stimulation of the subthalamic nucleus (STN-DBS) or internal pallidum (GPi-DBS) on health-related quality of life (HrQoL) in patients with advanced Parkinson's disease participating in a previously reported multicenter trial. Sickness Impact Profile (SIP)

  9. Booster Sessions Enhance the Long-Term Effectiveness of Spaced Retrieval in Older Adults with Probable Alzheimer's Disease

    Science.gov (United States)

    Cherry, Katie E.; Hawley, Karri S.; Jackson, Erin M.; Boudreaux, Emily O.

    2009-01-01

    Six older adults with probable Alzheimer's disease (AD) were trained to recall a name-face association using the spaced retrieval technique. In this study, we retested these persons in a 6-month follow-up program. For half of the participants, three booster sessions were administered at 6, 12, and 18 weeks after original training to promote…

  10. Acute terminal ileitis, yersiniosis, and Crohn's disease: a long-term follow-up study of the relationships

    DEFF Research Database (Denmark)

    Jess, T; Jess, Per

    2001-01-01

    University Hospital during the period 1976-1998 were diagnosed as having ATI while undergoing surgery for suspected acute appendicitis. The patients were followed up, and both Yersinia titers and the development of Crohn's disease were registered. Results: Forty-four patients (83%) were tested for Yersinia...

  11. Long-term follow-up reveals high incidence of colorectal cancer in Indian patients with inflammatory bowel disease.

    Science.gov (United States)

    Bopanna, Sawan; Kedia, Saurabh; Das, Prasenjit; Dattagupta, S; Sreenivas, V; Mouli, V Pratap; Dhingra, Rajan; Pradhan, Rajesh; Kumar, N Suraj; Yadav, Dawesh P; Makharia, Govind; Ahuja, Vineet

    2017-08-01

    As the magnitude of sporadic colorectal cancer (CRC) in India is low, magnitude of CRC in ulcerative colitis (UC) is also considered low. As a result, screening for CRC in UC although advocated may not be followed everywhere. We report our data of UC-related CRC from a low-incidence area of sporadic CRC. A total of 1012 patients with left-sided colitis/pancolitis having more than one full-length colonoscopy performed at least a year after the onset of symptoms were included in retrospective analysis of prospectively maintained case records. In addition, 136 patients with duration of disease >10 years underwent surveillance white-light colonoscopy prospectively during the study period. A total of 1012 individuals were finally included (6542 person-years of follow-up, 68.5% males, disease duration: 6.4 ± 6.8 years). Twenty (1.97%) patients developed CRC. Two (10%) patients developed CRC during the first decade, 10/20 (50%) during the second and 8/20 (40%) after the second decade of disease. The cumulative risk of developing CRC was 1.5%, 7.2% and 23.6% in the first, second and third decade, respectively. Of 136 high-risk UC cases, five (3.6%) had CRC on screening colonoscopy. Disease duration and increasing age of onset were associated with higher risk of CRC. Cumulative risk of CRC in Indian UC patients is as high as 23.6% at 30 years. The risk of CRC increases with increasing age of onset and increasing duration of disease. A low risk of sporadic CRC does not confer a low risk of UC-related CRC, and regular screening is warranted.

  12. [Long-term outcome with end-stage renal disease - survival is not enough: does dialysis or kidney transplantation matter?].

    Science.gov (United States)

    Schulz, K-H; Thaiss, F

    2012-04-01

    Patients with end-stage renal disease require renal replacement therapy with either dialysis or kidney transplantation. Survival and quality of life (QoL) after transplantation are superior to chronic dialysis. Early living donor kidney transplantation is best for patient and graft survival. Preemptive living-related kidney transplantation therefore is the best medical treatment option for these patients. Patients with end-stage renal disease suffer from multiple physical and psychological complaints. The prevalence of depressive disorders is 20-25% in this population. Studies on QoL in children after kidney transplantation show a reduced physical QoL, but an overall good psychological QoL. Alarming results of numerous studies are the high non-adherence rates in adolescents. Especially exercise interventions during dialysis and after kidney transplantation show promising results. Whether QoL of patients will improve with new approaches to immunosuppressive therapy remains to be evaluated in future studies.

  13. Simulating infectious disease risk based on climatic drivers: from numerical weather prediction to long term climate change scenario

    Science.gov (United States)

    Caminade, C.; Ndione, J. A.; Diallo, M.; MacLeod, D.; Faye, O.; Ba, Y.; Dia, I.; Medlock, J. M.; Leach, S.; McIntyre, K. M.; Baylis, M.; Morse, A. P.

    2012-04-01

    Climate variability is an important component in determining the incidence of a number of diseases with significant health and socioeconomic impacts. In particular, vector born diseases are the most likely to be affected by climate; directly via the development rates and survival of both the pathogen and the vector, and indirectly through changes in the surrounding environmental conditions. Disease risk models of various complexities using different streams of climate forecasts as inputs have been developed within the QWeCI EU and ENHanCE ERA-NET project frameworks. This work will present two application examples, one for Africa and one for Europe. First, we focus on Rift Valley fever over sub-Saharan Africa, a zoonosis that affects domestic animals and humans by causing an acute fever. We show that the Rift Valley fever outbreak that occurred in late 2010 in the northern Sahelian region of Mauritania might have been anticipated ten days in advance using the GFS numerical weather prediction system. Then, an ensemble of regional climate projections is employed to model the climatic suitability of the Asian tiger mosquito for the future over Europe. The Asian tiger mosquito is an invasive species originally from Asia which is able to transmit West Nile and Chikungunya Fever among others. This species has spread worldwide during the last decades, mainly through the shipments of goods from Asia. Different disease models are employed and inter-compared to achieve such a task. Results show that the climatic conditions over southern England, central Western Europe and the Balkans might become more suitable for the mosquito (including the proviso that the mosquito has already been introduced) to establish itself in the future.

  14. Long-term dietary supplementation of pomegranates, figs and dates alleviate neuroinflammation in a transgenic mouse model of Alzheimer's disease.

    Directory of Open Access Journals (Sweden)

    Musthafa Mohamed Essa

    Full Text Available Alzheimer's disease (AD is a devastating age-related neurodegenerative disease with no specific treatment at present. The APPsw/Tg2576 mice exhibit age-related deterioration in memory and learning as well as amyloid-beta (Aβ accumulation, and this mouse strain is considered an effective model for studying the mechanism of accelerated brain aging and senescence. The present study was aimed to investigate the beneficial effects of dietary supplements pomegranate, figs, or the dates on suppressing inflammatory cytokines in APPsw/Tg2576 mice. Changes in the plasma cytokines and Aβ, ATP, and inflammatory cytokines were investigated in the brain of transgenic mice. Significantly enhanced levels of inflammatory cytokines IL-1β, IL-2, IL-3, IL-4, IL-5, IL-6, IL-9, IL-10, TNF-α and Eotaxin activity were decreased by administration of the diet supplements containing pomegranates, figs, or dates. In addition, putative delays in the formation of senile plaques, as indicated by a decreasing tendency of brain Aβ1-40 and Aβ1-42 contents, were observed. Thus, novel results mediated by reducing inflammatory cytokines during aging may represent one mechanism by which these supplements exert their beneficial effects against neurodegenerative diseases such as AD.

  15. Long-term interleukin-6 levels and subsequent risk of coronary heart disease: two new prospective studies and a systematic review.

    Directory of Open Access Journals (Sweden)

    John Danesh

    2008-04-01

    Full Text Available The relevance to coronary heart disease (CHD of cytokines that govern inflammatory cascades, such as interleukin-6 (IL-6, may be underestimated because such mediators are short acting and prone to fluctuations. We evaluated associations of long-term circulating IL-6 levels with CHD risk (defined as nonfatal myocardial infarction [MI] or fatal CHD in two population-based cohorts, involving serial measurements to enable correction for within-person variability. We updated a systematic review to put the new findings in context.Measurements were made in samples obtained at baseline from 2,138 patients who had a first-ever nonfatal MI or died of CHD during follow-up, and from 4,267 controls in two cohorts comprising 24,230 participants. Correction for within-person variability was made using data from repeat measurements taken several years apart in several hundred participants. The year-to-year variability of IL-6 values within individuals was relatively high (regression dilution ratios of 0.41, 95% confidence interval [CI] 0.28-0.53, over 4 y, and 0.35, 95% CI 0.23-0.48, over 12 y. Ignoring this variability, we found an odds ratio for CHD, adjusted for several established risk factors, of 1.46 (95% CI 1.29-1.65 per 2 standard deviation (SD increase of baseline IL-6 values, similar to that for baseline C-reactive protein. After correction for within-person variability, the odds ratio for CHD was 2.14 (95% CI 1.45-3.15 with long-term average ("usual" IL-6, similar to those for some established risk factors. Increasing IL-6 levels were associated with progressively increasing CHD risk. An updated systematic review of electronic databases and other sources identified 15 relevant previous population-based prospective studies of IL-6 and clinical coronary outcomes (i.e., MI or coronary death. Including the two current studies, the 17 available prospective studies gave a combined odds ratio of 1.61 (95% CI 1.42-1.83 per 2 SD increase in baseline IL-6

  16. Impaired quality of life in patients with treated acromegaly despite long-term biochemically stable disease: Results from a 5-years prospective study.

    Science.gov (United States)

    Kyriakakis, Nikolaos; Lynch, Julie; Gilbey, Stephen G; Webb, Susan M; Murray, Robert D

    2017-06-01

    Patients with acromegaly demonstrate impaired quality of life (QoL), but data on long-term QoL changes in treated acromegaly are limited. This study evaluates and identifies factors that influence QoL in patients with long-term biochemical remission. The study consists of a cross-sectional arm comparing QoL between patients with treated and controlled acromegaly and healthy controls; and a longitudinal arm assessing QoL changes in patients with biochemically stable disease during 5.7±0.6 years of follow-up. A total of 58 patients and 116 matched controls were recruited for the cross-sectional arm; 28 patients completed the longitudinal arm. Three generic questionnaires (Psychological General Well-Being Schedule [PGWBS], 36-item Short-Form [SF-36], EuroQoL [EQ-5D]) and the disease-specific acromegaly QoL questionnaire (AcroQoL) were applied. Quality of life assessment was performed 11.6±8.2 years following diagnosis and treatment of acromegaly. Patients with treated acromegaly had lower QoL scores compared with controls in all questionnaires with the exception of the PGWBS "Anxiety" subscale. The AcroQoL "Appearance" subscale and the "Physical Function" subscales of the remaining questionnaires were the most underscored domains. No difference in the total and subscale scores of all questionnaires was observed between baseline and follow-up, with the exception of the SF-36 "Physical Function," where a decline was found (58.5±24.7% vs 43.1±31.1%; P=.002). However, after adjusting for covariates, no significant change in any of the QoL scores was seen. Duration of IGF-1/GH control was positively correlated with QoL scores in most questionnaires at baseline, whereas use of GH lowering therapy at the time of QoL assessment was a negative predictive factor of QoL. Patients with biochemically controlled acromegaly demonstrate impaired QoL, which persists despite long-term disease control. This primarily consists of impaired physical function and secondly of impaired

  17. Magnetic resonance imaging of bone marrow changes in Gaucher disease during enzyme replacement therapy: first German long-term results

    International Nuclear Information System (INIS)

    Poll, L.W.; Koch, J.A.; Scherer, A.; Boerner, D.; Moedder, U.; Dahl, S. vom; Niederau, C.; Haeussinger, D.; Willers, R.

    2001-01-01

    Objective:. Since 1991, enzyme replacement therapy (ERT) has been available for patients with Gaucher disease in Germany. The aim of this study was to analyse the MR pattern of bone marrow involvement and response to ERT in Gaucher disease type I. Patients and design:. Thirty patients with Gaucher disease type I had MRI examinations prior to initiation of ERT with alglucerase/imiglucerase and during follow-up. Median MR follow-up and duration of ERT were 36 months. Coronal T1- and T2-weighted spin-echo images of the lower extremities were obtained to evaluate changes in the appearance of yellow marrow. MR images were categorized as having either a homogeneous (type A) or non-homogeneous patchy (type B) appearance of bone involvement and response to ERT was assessed by two radiologists. Results:. Overall, 19 of 30 patients (63%) showed an increased signal intensity on T1- and T2-weighted images after 36 months of ERT, consistent with partial reconversion of fatty marrow during treatment. Focal bone lesions surrounded by a low signal intensity (SI) rim did not respond to ERT, suggesting bone infarcts. Of the 11 patients with bone infarcts (low SI rim lesion), 82% had the non-homogeneous type B pattern (P=0.0021). In 86% of patients with splenectomy, bone infarcts were seen (P<0.05). Conclusions:. MRI using T1- and T2-weighted spin-echo sequences is a valuable, non-invasive method for monitoring bone marrow response in patients receiving ERT. A non- homogeneous patchy signal intensity of bone marrow involvement correlates with the presence of bone infarcts (P=0.0021). (orig.)

  18. Long-term follow-up of testicular function following radiation therapy for early-stage Hodgkin's disease

    International Nuclear Information System (INIS)

    Kinsella, T.J.; Trivette, G.; Rowland, J.; Sorace, R.; Miller, R.; Fraass, B.; Steinberg, S.M.; Glatstein, E.; Sherins, R.J.

    1989-01-01

    Seventeen male patients with pathological staged I-IIIA1 Hodgkin's disease were followed prospectively for radiation damage to the testes from low-dose scattered irradiation. During conventionally fractionated radiation therapy, the testicular dose ranged from 6 to 70 cGy. Testicular function was measured in a prospective fashion by repeated analyses (every 6 to 12 months) of serum follicle-stimulating hormone (FSH), luteinizing hormone (LH), and testosterone. Patients were also followed by serial semen analyses and by a questionnaire on fertility. The follow-up period ranged from 3 to 7 years after completion of radiation therapy. In patients receiving greater than or equal to 20 cGy, there was a dose-dependent increase in serum FSH values following irradiation, with the maximum difference at 6 months compared with pretreatment levels. All patients showed a return to normal FSH values within 12 to 24 months following irradiation. No significant changes in LH and testosterone were observed in this patient group. Eight patients with a normal pretreatment semen analysis provided serial semen samples and two patients showed transient oligospermia with complete recovery by 18 months following treatment. Four patients have fathered normal offspring following radiation therapy. We conclude that low doses (greater than 20 cGy) of scatter irradiation during treatment for Hodgkin's disease can result in transient injury to the seminiferous tubule as manifested by elevations of FSH for 6 to 24 months following treatment. Below 20 cGy, FSH values remained in the normal range. No evidence of Leydig cell injury (using LH and testosterone) was seen in this dose range (up to 70 cGy). Thus, patients with early-stage Hodgkin's disease can be treated with radiation therapy with little to no risk of irreversible testicular injury. Radiation treatment techniques to shield the testes are discussed

  19. Magnetic resonance imaging of bone marrow changes in Gaucher disease during enzyme replacement therapy: first German long-term results

    Energy Technology Data Exchange (ETDEWEB)

    Poll, L.W.; Koch, J.A.; Scherer, A.; Boerner, D.; Moedder, U. [Duesseldorf Univ. (Germany). Inst. fuer Diagnostische Radiologie; Dahl, S. vom; Niederau, C.; Haeussinger, D. [Duesseldorf Univ. (Germany). Medizinische Fakultaet; Willers, R. [Duesseldorf Univ. (Germany). Rechenzentrum

    2001-09-01

    Objective:. Since 1991, enzyme replacement therapy (ERT) has been available for patients with Gaucher disease in Germany. The aim of this study was to analyse the MR pattern of bone marrow involvement and response to ERT in Gaucher disease type I. Patients and design:. Thirty patients with Gaucher disease type I had MRI examinations prior to initiation of ERT with alglucerase/imiglucerase and during follow-up. Median MR follow-up and duration of ERT were 36 months. Coronal T1- and T2-weighted spin-echo images of the lower extremities were obtained to evaluate changes in the appearance of yellow marrow. MR images were categorized as having either a homogeneous (type A) or non-homogeneous patchy (type B) appearance of bone involvement and response to ERT was assessed by two radiologists. Results:. Overall, 19 of 30 patients (63%) showed an increased signal intensity on T1- and T2-weighted images after 36 months of ERT, consistent with partial reconversion of fatty marrow during treatment. Focal bone lesions surrounded by a low signal intensity (SI) rim did not respond to ERT, suggesting bone infarcts. Of the 11 patients with bone infarcts (low SI rim lesion), 82% had the non-homogeneous type B pattern (P=0.0021). In 86% of patients with splenectomy, bone infarcts were seen (P<0.05). Conclusions:. MRI using T1- and T2-weighted spin-echo sequences is a valuable, non-invasive method for monitoring bone marrow response in patients receiving ERT. A non- homogeneous patchy signal intensity of bone marrow involvement correlates with the presence of bone infarcts (P=0.0021). (orig.)

  20. ranibizumab in the management of advanced Coats disease Stages 3B and 4: long-term outcomes.

    Science.gov (United States)

    Gaillard, Marie-Claire; Mataftsi, Assimina; Balmer, Aubin; Houghton, Susan; Munier, Francis L

    2014-11-01

    Laser photocoagulation and cryotherapy to completely destroy telangiectatic vessels and ischemic retina in Coats disease is barely applicable in advanced cases with total retinal detachment, and globe survival is notoriously poor in Stages 3B and 4. Anti-vascular endothelial growth factor intravitreal injections may offer new prospects for these patients. This study is a retrospective review of all consecutive patients with Coats disease treated with neoadjuvant or adjuvant intravitreal ranibizumab plus conventional and amblyopia treatment as appropriate. Nine patients (median age, 13 months) presenting Coats Stages 3B and 4 (5 and 4 eyes, respectively) were included. Iris neovascularization resolved within 2 weeks and retinal reapplication within 4 months in all patients. At last follow-up, globe survival was 100% with anatomical success in 8 of the 9 eyes. With a median follow-up of 50 months, fibrotic vitreoretinopathy was developed in 5 of the 9 cases, one leading to tractional retinal detachment and ultimately phthisis bulbi. The remaining 4 of the 9 eyes achieved some vision (range, 0.02-0.063). To the best of the authors' knowledge, this is the largest reported series of late-stage Coats undergoing anti-vascular endothelial growth factor therapy, a homogenous cohort of patients treated with a single agent and with the longest follow-up. This study supports the role of ranibizumab in advanced disease by transient restoration of the hemato-retinal barrier and suppression of neovascularization to facilitate classic treatment. At the last follow-up, the authors report unprecedented anatomical success and functional outcome.

  1. Comparison of Different Disease-Specific Health-Related Quality of Life Measurements in Patients with Long-Term Noninvasive Ventilation

    Directory of Open Access Journals (Sweden)

    Toru Oga

    2017-01-01

    Full Text Available Background. Two disease-specific questionnaires have been developed to assess health-related quality of life (HRQL in patients with chronic respiratory failure: the Severe Respiratory Insufficiency (SRI Questionnaire and the Maugeri Respiratory Failure (MRF Questionnaire. We aimed to compare the characteristics of the SRI, MRF-26, and St. George’s Respiratory Questionnaire (SGRQ for use in patients with home noninvasive ventilation (NIV. Methods. Fifty-six outpatients receiving long-term NIV were recruited and underwent assessments of pulmonary function, arterial blood gas, HRQL, dyspnea, and psychological status. Results. Correlations of the SRI and MRF-26 with the SGRQ were modest. While pulmonary function was weakly related to only some domains of the SRI and MRF-26, the modified Medical Research Council (mMRC dyspnea scale and Hospital Anxiety and Depression Scale (HADS were significantly related to all domains of the SRI and MRF-26. Multiple regression analyses showed that HADS depression and mMRC accounted for 34% and 27% of the variance in the SRI, 24% and 37% in the MRF-26, and 17% and 46% in the SGRQ, respectively. Conclusions. The SRI and MRF-26 were reliable questionnaires for patients receiving long-term NIV. Dyspnea and psychological status were their main common determinants. The SRI covers more psychological health impairments than the MRF. This trial is registered with ClinicalTrials.gov Identifier: NCT00905476.

  2. GMDPtoolbox: A Matlab library for designing spatial management policies. Application to the long-term collective management of an airborne disease.

    Science.gov (United States)

    Cros, Marie-Josée; Aubertot, Jean-Noël; Peyrard, Nathalie; Sabbadin, Régis

    2017-01-01

    Designing management policies in ecology and agroecology is complex. Several components must be managed together while they strongly interact spatially. Decision choices must be made under uncertainty on the results of the actions and on the system dynamics. Furthermore, the objectives pursued when managing ecological systems or agroecosystems are usually long term objectives, such as biodiversity conservation or sustainable crop production. The framework of Graph-Based Markov Decision Processes (GMDP) is well adapted to the qualitative modeling of such problems of sequential decision under uncertainty. Spatial interactions are easily modeled and integrated control policies (combining several action levers) can be designed through optimization. The provided policies are adaptive, meaning that management actions are decided at each time step (for instance yearly) and the chosen actions depend on the current system state. This framework has already been successfully applied to forest management and invasive species management. However, up to now, no "easy-to-use" implementation of this framework was available. We present GMDPtoolbox, a Matlab toolbox which can be used both for the design of new management policies and for comparing policies by simulation. We provide an illustration of the use of the toolbox on a realistic crop disease management problem: the design of long term management policy of blackleg of canola using an optimal combination of three possible cultural levers. This example shows how GMDPtoolbox can be used as a tool to support expert thinking.

  3. Frequency and Cause of Persistent Symptoms in Celiac Disease Patients on a Long-term Gluten-free Diet.

    Science.gov (United States)

    Stasi, Elisa; Marafini, Irene; Caruso, Roberta; Soderino, Federica; Angelucci, Erika; Del Vecchio Blanco, Giovanna; Paoluzi, Omero A; Calabrese, Emma; Sedda, Silvia; Zorzi, Francesca; Pallone, Francesco; Monteleone, Giovanni

    2016-03-01

    To estimate the frequency and cause of nonresponsive celiac disease (CD). Treatment of CD is based on life-long adherence to a gluten-free diet (GFD). Some celiac patients experience persistence of symptoms despite a GFD. This condition is defined as nonresponsive CD. Celiac patients on a GFD for at least 12 months underwent diet compliance assessment, laboratory tests, breath tests, endoscopic, and histologic evaluations according to the symptoms/signs reported. Seventy of 321 (21.8%) patients had persistent or recurrent symptoms/signs. The cause of symptom persistence was evaluated in 56 of 70 patients. Thirteen of 56 (23%) patients were antiendomysial antibody positive. Among the patients with negative serology, 1 had fibromyalgia, and 3 had evidence that disproved the diagnosis of CD. The remaining 39 patients with negative serology underwent duodenal biopsy sampling, which evidenced histologic alterations in 24 patients. Among the 15 patients with normal histology 3 were lactose intolerant, 9 had irritable bowel syndrome, 2 had gastroesophageal reflux disease, and in 1 patient a cause for the persistent symptom was not identified. In patients with confirmed diagnosis of CD, exposure to dietary gluten was the main cause of persistence of symptoms/signs, and consistently after dietary modification, symptoms resolved in 63% of the patients at later time points during follow-up. Nonresponsive CD occurs in nearly one fifth of celiac patients on GFD and its occurrence suggests further investigations to optimize the management of celiac patients.

  4. Reassessment of Ambulatory Blood Pressure Improves Renal Risk Stratification in Nondialysis Chronic Kidney Disease: Long-Term Cohort Study.

    Science.gov (United States)

    Minutolo, Roberto; Gabbai, Francis B; Chiodini, Paolo; Garofalo, Carlo; Stanzione, Giovanna; Liberti, Maria Elena; Pacilio, Mario; Borrelli, Silvio; Provenzano, Michele; Conte, Giuseppe; De Nicola, Luca

    2015-09-01

    In nondialysis chronic kidney disease, ambulatory blood pressure (ABP) performs better than clinic BP in predicting outcome, but whether repeated assessment of ABP further refines prognosis remains ill-defined. We recruited 182 consecutive hypertensive patients with nondialysis chronic kidney disease who underwent 2 ABPs 12 months apart to evaluate the enhancement in risk stratification provided by a second ABP obtained 1 year after baseline on the risk (hazard ratio and 95% confidence interval) of composite renal end point (death, chronic dialysis, and estimated glomerular filtration rate decline ≥40%). The difference in daytime and nighttime systolic BP between the 2 ABPs (daytime and nighttime bias) was added to a survival model including baseline ABP. Net reclassification improvement was also calculated. Age was 65.6±13.4 years; 36% had diabetes mellitus and 36% had previous cardiovascular event; estimated glomerular filtration rate was 42.2±19.6 mL/min per 1.73 m(2), and clinic BP was 145±18/80±11 mm Hg. Baseline ABP (daytime, 131±16/75±10 and nighttime, 122±18/66±10 mm Hg) and daytime/nighttime BP goals (58.2% and 43.4%) did not change at month 12. Besides baseline ABP values, bias for daytime and nighttime systolic BP linearly associated with renal outcome (1.12, 1.04-1.21 and 1.18, 1.08-1.29 for every 5-mm Hg increase, respectively). Classification of patients at risk improved when considering nighttime systolic level at second ABP (net reclassification improvement, 0.224; 95% confidence interval, 0.005-0.435). Patients with first and second ABPs above target showed greater renal risk (2.15, 1.29-3.59 and 1.71, 1.07-2.72, for daytime and nighttime, respectively). In nondialysis chronic kidney disease, reassessment of ABP at 1 year further refines renal prognosis; such reassessment should specifically be considered in patients with uncontrolled BP at baseline. © 2015 American Heart Association, Inc.

  5. Long-term outcome of FFR-guided PCI for stable coronary artery disease in daily clinical practice

    DEFF Research Database (Denmark)

    De Backer, Ole; Biasco, Luigi; Lønborg, Jacob

    2016-01-01

    AIMS: Our aim was to investigate the strength of fractional flow reserve (FFR)-guided percutaneous coronary intervention (PCI) for stable coronary artery disease (CAD) in daily practice. METHODS AND RESULTS: For this study, 3,512 patients with stable CAD and at least one 50-89% coronary stenosis...... were identified; those patients thought to require PCI (n=1,716) were selected. Of these, 962 (56%) were treated based on angiography (XA) alone, whereas 754 patients (44%) had an FFR-guided treatment. In the latter group, 321 patients (43%) were reallocated to another treatment, predominantly medical...... treatment. After propensity score matching, the number of indicated lesions was 957 in the XA-guided group and 947 in the FFR-guided group. FFR guidance resulted in PCI deferral in 462 lesions (48.8%). In a seven-day landmark analysis, the rate of periprocedural myocardial infarction (MI) was less than half...

  6. The nutritional index 'CONUT' is useful for predicting long-term prognosis of patients with end-stage liver diseases.

    Science.gov (United States)

    Fukushima, Koji; Ueno, Yoshiyuki; Kawagishi, Naoki; Kondo, Yasuteru; Inoue, Jun; Kakazu, Eiji; Ninomiya, Masashi; Wakui, Yuta; Saito, Naoko; Satomi, Susumu; Shimosegawa, Tooru

    2011-07-01

    Organ allocation in Japan remains difficult due to the shortage of deceased-donor livers. The screening tool for controlling nutritional status (CONUT) has been considered to be an established assessment model for evaluating nutritional aspects in surgical patients. However, the application of this CONUT for evaluating the prognosis of patients with end-stage liver diseases has not been evaluated. We assessed the predictability of the prognoses of 58 patients with end-stage liver disease using various prognostic models. The patients registered at the transplantation center of Tohoku University Hospital for the waiting list of Japan Organ Transplant Network for liver transplantation were retrospectively analyzed. The prognoses of the patients were evaluated using the following 5 models: CONUT, the model for ELD with incorporation of sodium (MELD-Na), Child-Turcotte-Pugh score (CTP), prognostic nutritional indices (Onodera: PNI-O), and the Japan Medical Urgency criteria of the liver (JMU). Cox's proportional hazard model, log-rank test and concordance(c)-static were used for the statistics. The indices were 17.74 ± 5.80 for MELD-Na, 9.21 ± 2.19 for CTP, 33.92 ± 11.16 for PNI-O, and 7.57 ± 3.09 for CONUT. Univariate analysis revealed the significance of CONUT (p = 0.017, Odds: 1.325) but not MELD-Na, CTP, JMU or PNI-O for prediction. The cumulative survival rate was clearly discriminated at CONUT point 7. The c-static was 0.081 for the 6-month (M) survival rate, 0.172 for 12M, 0.517 for 36M, 0.821 for 48M, and 0.938 for 60M for CONUT. In conclusion, CONUT shows best predictability for the distant prognoses of patients with ELD.

  7. Clinical, demographic characteristics and results of the long term follow-up in adolescents and adults with congenital heart disease

    Directory of Open Access Journals (Sweden)

    I.G. Lebid

    2016-05-01

    Full Text Available The aim – to analyze clinical and demographic indicators in adolescents and adults with congenital heart disease (CHD to provide strategy of cardiac care for these patients, to assess risk of cardiological and cardiac surgery interventions in patients with congenital heart malformations. Materials and methods. 2569 consecutive patients, aged 16–88 years, mean age 24.14 ± 0.20 years, were selected in electronic database from April 01, 2011 to December 31, 2015. The majority (92.57 % of the included patients (n = 2378 were younger than 40 years. Results. Among all CHD patients, a significant majority had septal defects (39 % and left heart lesions (24 %, followed by congenital lesions of thoracic arteries and veins (16 % and right heart lesions (10 %. The annual number of the examined patients with CHD progressively increased (from 210 in 2011 to 656 in 2015. The number of patients aged 18 years or older mostly increased. The number of patients older than 40 years increased from nine patients in 2011 to 75 adults in 2015. Patients after cardiac surgery and percutaneous transcatheter interventions dominated (n=1553, 60.45 %, compared to the patients without any interventions for CHD (n = 1016, 39.55 %. Only one intervention for CHD was performed in the majority of these patients (n = 1255, 80.81 %, 12.94 % needed two interventions, 3.99 % – three, 2.26 % – three or more interventions. Conclusions. Among patients with congenital heart disease, patients younger than 40 years old were prevalent (92.57 %, with no significant gender differences. Septal defects (ASD, VSD, left heart lesions (congenital aortic valve stenosis and insufficiency, congenital lesions of thoracic arteries and veins (patent ductus arteriosus and aorta coarctation were registered most often in adolescents and adults.

  8. Mantle irradiation alone for pathologic stage I and II Hodgkin's disease: long-term follow-up and patterns of failure

    International Nuclear Information System (INIS)

    Liao Zhongxing; Ha, Chul S.; Vlachaki, Maria T.; Hagemeister, Frederick; Cabanillas, Fernando; Hess, Mark; Tucker, Susan; Cox, James D.

    2001-01-01

    Purpose: We performed a retrospective study to determine the long-term outcome, patterns of failure, and prognostic factors for patients with pathologic Stage I or II Hodgkin's disease (HD) who were treated with mantle irradiation alone. Methods and Materials: The medical records of 145 patients with pathologic Stage I or II supradiaphragmatic Hodgkin's disease treated with mantle irradiation alone between June 1967 and June 1991 were reviewed. Patterns of failure, overall survival (OS) rate, and progression-free survival (PFS) rate were determined. Univariate and multivariate analyses were performed to identify adverse prognostic factors for OS and PFS. The number of adverse prognostic factors per patient was counted, and a prognostic score was assigned to each patient. The log-rank test was used to compare the OS or PFS rates among patients with prognostic scores 0, 1, and 2. Results: The median patient age was 27 years (range 10-66), with almost even male to female distribution. Every patient had splenectomy and negative laparotomy (LAP). Fifty-one patients had Stage I disease (IA-49, IB-2) and 94 Stage II (IIA-89, IIB-5). The histologic subtypes were nodular sclerosing in 110, mixed cellularity in 28, lymphocyte predominance in 5, lymphocyte depleted in 1, and unclassified in 1. Twelve patients with Stage II disease had ≥ 3 sites of nodal involvement. Fifty-four patients had a prognostic score of 0, 70 of 1, and 21 of 2. The median follow-up time for the 109 surviving patients was 146 months (range 25-381). The 10- and 20-year actuarial OS rates for the whole group were 87.6% and 65.3%, respectively. The corresponding actuarial PFS rates were 75.3% and 74.2%, respectively. Thirty-six patients (9 Stage I, 27 Stage II) had relapses in a total of 41 sites. Failures by histology were 29 patients with nodular sclerosing, 6 with mixed cellularity, and 1 with lymphocyte predominance. Failures by sites were: trans-diaphragmatic, 22 (para-aortic nodes, 15; as the only

  9. Cardiac function and central haemodynamics in severe chronic obstructive lung disease. Acute and long-term effects of felodipine

    Energy Technology Data Exchange (ETDEWEB)

    Bratel, T; Hedenstierna, G; Lundquist, H; Nyquist, O; Ripe, E

    1988-01-01

    Eleven patients, with advanced chronic obstructive lung disease (COLD), received an infusion of the calcium antagonist felodipine at a rate of 0.9 mg/h. Pulmonary and systemic vascular resistances (PVR and SVR) at rest were reduced by 18% and 33%, respectively. Cardiac output increased by 33%. The right venticular and left venticular ejection fractions (RVEF and LVEF), measured by equilibrium gated radionuclide ventriculography, Increased by 32% and 25%, respectively. During exercise both PVR and SVR fell by a mean of 30%. RVEF and LVEF both increased by about 14%. After three months of oral felopdipine treatment, a dose-related decrease in PVR was noted at rest (r=-0.83) compared with pretreatment values. There was an increase in RVEF which correlated to a reduction in PVR. Three patiens discontinued the trial due to side effects. It is concluded that the reduction of PVR induced by felodipine is accompanied by an improvement in right heart function as measured by ejection fraction measurements.

  10. Experience of long-term use of non-invasive ventilation in motor neuron disease: an interpretative phenomenological analysis.

    Science.gov (United States)

    Ando, Hikari; Chakrabarti, Biswajit; Angus, Robert M; Cousins, Rosanna; Thornton, Everard W; Young, Carolyn A

    2014-03-01

    Although non-invasive ventilation (NIV) can promote quality of life in motor neuron disease (MND), previous studies have disregarded the impact of progression of illness. This study explored how patients' perceptions of NIV treatment evolve over time and how this was reflected in their adherence to NIV. Five patients with MND (male=4, mean age=59 years), from a bigger cohort who were prospectively followed, had multiple post-NIV semistructured interviews, covering more than 12 months, along with ventilator interaction data. The transcribed phenomenological data were analysed using qualitative methodology. Three themes emerged: experience of NIV, influence on attitudes and perceived impact of NIV on prognosis. The ventilator interaction data identified regular use of NIV by four participants who each gave positive account of their experience of NIV treatment, and irregular use by one participant who at interview revealed a negative attitude to NIV treatment and in whom MND induced feelings of hopelessness. This exploratory study suggests that a positive coping style, adaptation and hope are key factors for psychological well-being and better adherence to NIV. More studies are needed to determine these relationships.

  11. Competing pressures on populations: long-term dynamics of food availability, food quality, disease, stress and animal abundance.

    Science.gov (United States)

    Chapman, Colin A; Schoof, Valérie A M; Bonnell, Tyler R; Gogarten, Jan F; Calmé, Sophie

    2015-05-26

    Despite strong links between sociality and fitness that ultimately affect the size of animal populations, the particular social and ecological factors that lead to endangerment are not well understood. Here, we synthesize approximately 25 years of data and present new analyses that highlight dynamics in forest composition, food availability, the nutritional quality of food, disease, physiological stress and population size of endangered folivorous red colobus monkeys (Procolobus rufomitratus). There is a decline in the quality of leaves 15 and 30 years following two previous studies in an undisturbed area of forest. The consumption of a low-quality diet in one month was associated with higher glucocorticoid levels in the subsequent month and stress levels in groups living in degraded forest fragments where diet was poor was more than twice those in forest groups. In contrast, forest composition has changed and when red colobus food availability was weighted by the protein-to-fibre ratio, which we have shown positively predicts folivore biomass, there was an increase in the availability of high-quality trees. Despite these changing social and ecological factors, the abundance of red colobus has remained stable, possibly through a combination of increasing group size and behavioural flexibility. © 2015 The Author(s) Published by the Royal Society. All rights reserved.

  12. Long-term consequences of developmental vascular defects on retinal vessel homeostasis and function in a mouse model of Norrie disease.

    Directory of Open Access Journals (Sweden)

    Susanne C Beck

    Full Text Available Loss of Norrin signalling due to mutations in the Norrie disease pseudoglioma gene causes severe vascular defects in the retina, leading to visual impairment and ultimately blindness. While the emphasis of experimental work so far was on the developmental period, we focus here on disease mechanisms that induce progression into severe adult disease. The goal of this study was the comprehensive analysis of the long-term effects of the absence of Norrin on vascular homeostasis and retinal function. In a mouse model of Norrie disease retinal vascular morphology and integrity were studied by means of in vivo angiography; the vascular constituents were assessed in detailed histological analyses using quantitative retinal morphometry. Finally, electroretinographic analyses were performed to assess the retinal function in adult Norrin deficient animals. We could show that the primary developmental defects not only persisted but developed into further vascular abnormalities and microangiopathies. In particular, the overall vessel homeostasis, the vascular integrity, and also the cellular constituents of the vascular wall were affected in the adult Norrin deficient retina. Moreover, functional analyses indicated to persistent hypoxia in the neural retina which was suggested as one of the major driving forces of disease progression. In summary, our data provide evidence that the key to adult Norrie disease are ongoing vascular modifications, driven by the persistent hypoxic conditions, which are ineffective to compensate for the primary Norrin-dependent defects.

  13. Long-term consequences of developmental vascular defects on retinal vessel homeostasis and function in a mouse model of Norrie disease.

    Science.gov (United States)

    Beck, Susanne C; Feng, Yuxi; Sothilingam, Vithiyanjali; Garcia Garrido, Marina; Tanimoto, Naoyuki; Acar, Niyazi; Shan, Shenliang; Seebauer, Britta; Berger, Wolfgang; Hammes, Hans-Peter; Seeliger, Mathias W

    2017-01-01

    Loss of Norrin signalling due to mutations in the Norrie disease pseudoglioma gene causes severe vascular defects in the retina, leading to visual impairment and ultimately blindness. While the emphasis of experimental work so far was on the developmental period, we focus here on disease mechanisms that induce progression into severe adult disease. The goal of this study was the comprehensive analysis of the long-term effects of the absence of Norrin on vascular homeostasis and retinal function. In a mouse model of Norrie disease retinal vascular morphology and integrity were studied by means of in vivo angiography; the vascular constituents were assessed in detailed histological analyses using quantitative retinal morphometry. Finally, electroretinographic analyses were performed to assess the retinal function in adult Norrin deficient animals. We could show that the primary developmental defects not only persisted but developed into further vascular abnormalities and microangiopathies. In particular, the overall vessel homeostasis, the vascular integrity, and also the cellular constituents of the vascular wall were affected in the adult Norrin deficient retina. Moreover, functional analyses indicated to persistent hypoxia in the neural retina which was suggested as one of the major driving forces of disease progression. In summary, our data provide evidence that the key to adult Norrie disease are ongoing vascular modifications, driven by the persistent hypoxic conditions, which are ineffective to compensate for the primary Norrin-dependent defects.

  14. Gastroesophageal reflux disease and Barrett's esophagus after laparoscopic sleeve gastrectomy: a possible, underestimated long-term complication.

    Science.gov (United States)

    Genco, Alfredo; Soricelli, Emanuele; Casella, Giovanni; Maselli, Roberta; Castagneto-Gissey, Lidia; Di Lorenzo, Nicola; Basso, Nicola

    2017-04-01

    Morbidly obese patients are affected by gastroesophageal reflux disease (GERD) and hiatal hernia (HH) more frequently than lean patients. Because of conflicting results, the indication to sleeve gastrectomy (SG) in patients with GERD is still debated. To evaluate the incidence of GERD on the basis of clinical, endoscopic, and histologic data in patients undergoing SG. University hospital, Rome, Italy. From July 2007 to January 2010, 162 patients underwent primary SG. Preoperatively all patients underwent visual analogue scale (VAS) evaluation of GERD symptoms, proton pump inhibitors (PPIs) consumption recording, and esophagogastroduodenoscopy (EGD). Stomach resection started 6 cm from pylorus on a 48Fr bougie. Staple line was reinforced by an oversewing suture. A postoperative clinical control with VAS evaluation, PPI consumption, and EGD was proposed to all patients. Three patients were excluded because of the occurrence of major postoperative complications. A total of 110 patients accepted to take part in the study (follow-up rate: 69.1%). At a mean 58 months of follow-up, incidence of GERD symptoms, VAS mean score, and PPI intake significantly increased compared with preoperative values (68.1% versus 33.6%: Preflux was found in 73.6% and 74.5% of cases, respectively. A significant increase in the incidence and in the severity of erosive esophagitis (EE) was evidenced, whereas nondysplastic Barrett's esophagus (BE) was newly diagnosed in 19 patients (17.2%). No significant correlations were found between GERD symptoms and endoscopic findings. In the present series the incidence of EE and of BE in SG patients was considerably higher than that reported in the current literature, and it was not related to GERD symptoms. Endoscopic surveillance after SG should be advocated irrespective of the presence of GERD symptoms. Copyright © 2017 American Society for Bariatric Surgery. Published by Elsevier Inc. All rights reserved.

  15. Long-Term Disease Control of a Pancreatic Neuroendocrine Tumor with Lanreotide Autogel®: A Case Report

    Directory of Open Access Journals (Sweden)

    Willem Lybaert

    2014-09-01

    Full Text Available The CLARINET study (ClinicalTrials.gov: NCT00353496 showed that somatostatin analogs are able to stabilize tumor growth in patients with intestinal and pancreatic neuroendocrine tumors (NETs. Here, we present a case of NET originating from the pancreatic tail that was treated with lanreotide Autogel®. A 60-year-old patient underwent resection of a pancreatic NET with splenectomy and distal pancreatectomy. Four months after surgery, there was an increase in chromogranin A levels, along with a hypercaptating lesion of approximately 3.5 cm at the residual part of the pancreatic corpus. Treatment with 30 mg monthly-administered octreotide long-acting release (LAR was initiated. After 3 months of treatment, a control CT scan revealed diffuse metastases in the liver, although the patient presented no symptoms and liver tests were normal. Due to difficulties with the administration of octreotide LAR, treatment was switched to lanreotide Autogel® 120 mg, administered as monthly deep-subcutaneous injections. Progression-free survival, as shown by 3-monthly CT scans, was obtained for 2 years without the need to increase the lanreotide Autogel® dose, and the patient reported no side effects. After these 2 years, deterioration of the patient's clinical status and weight loss were observed, along with increased size of the liver lesions and appearance of peritoneal metastases. Chemotherapy treatment with cisplatinum-etoposide was initiated, while the lanreotide Autogel® injections were continued. After three chemotherapy cycles, a rapid decline in the patient's quality of life was noted, and she requested discontinuation of the chemotherapy and lanreotide injections. One month later, the patient died due to clinical progressive disease.

  16. Association of long-term exposure to community noise and traffic-related air pollution with coronary heart disease mortality.

    Science.gov (United States)

    Gan, Wen Qi; Davies, Hugh W; Koehoorn, Mieke; Brauer, Michael

    2012-05-01

    In metropolitan areas, road traffic is a major contributor to ambient air pollution and the dominant source of community noise. The authors investigated the independent and joint influences of community noise and traffic-related air pollution on risk of coronary heart disease (CHD) mortality in a population-based cohort study with a 5-year exposure period (January 1994-December 1998) and a 4-year follow-up period (January 1999-December 2002). Individuals who were 45-85 years of age and resided in metropolitan Vancouver, Canada, during the exposure period and did not have known CHD at baseline were included (n = 445,868). Individual exposures to community noise and traffic-related air pollutants, including black carbon, particulate matter less than or equal to 2.5 μm in aerodynamic diameter, nitrogen dioxide, and nitric oxide, were estimated at each person's residence using a noise prediction model and land-use regression models, respectively. CHD deaths were identified from the provincial death registration database. After adjustment for potential confounders, including traffic-related air pollutants or noise, elevations in noise and black carbon equal to the interquartile ranges were associated with 6% (95% confidence interval: 1, 11) and 4% (95% confidence interval: 1, 8) increases, respectively, in CHD mortality. Subjects in the highest noise decile had a 22% (95% confidence interval: 4, 43) increase in CHD mortality compared with persons in the lowest decile. These findings suggest that there are independent effects of traffic-related noise and air pollution on CHD mortality.

  17. Introduction: Long term prediction

    International Nuclear Information System (INIS)

    Beranger, G.

    2003-01-01

    Making a decision upon the right choice of a material appropriate to a given application should be based on taking into account several parameters as follows: cost, standards, regulations, safety, recycling, chemical properties, supplying, transformation, forming, assembly, mechanical and physical properties as well as the behaviour in practical conditions. Data taken from a private communication (J.H.Davidson) are reproduced presenting the life time range of materials from a couple of minutes to half a million hours corresponding to applications from missile technology up to high-temperature nuclear reactors or steam turbines. In the case of deep storage of nuclear waste the time required is completely different from these values since we have to ensure the integrity of the storage system for several thousand years. The vitrified nuclear wastes should be stored in metallic canisters made of iron and carbon steels, stainless steels, copper and copper alloys, nickel alloys or titanium alloys. Some of these materials are passivating metals, i.e. they develop a thin protective film, 2 or 3 nm thick - the so-called passive films. These films prevent general corrosion of the metal in a large range of chemical condition of the environment. In some specific condition, localized corrosion such as the phenomenon of pitting, occurs. Consequently, it is absolutely necessary to determine these chemical condition and their stability in time to understand the behavior of a given material. In other words the corrosion system is constituted by the complex material/surface/medium. For high level nuclear wastes the main features for resolving problem are concerned with: geological disposal; deep storage in clay; waste metallic canister; backfill mixture (clay-gypsum) or concrete; long term behavior; data needed for modelling and for predicting; choice of appropriate solution among several metallic candidates. The analysis of the complex material/surface/medium is of great importance

  18. Long-Term Symbolic Learning

    National Research Council Canada - National Science Library

    Kennedy, William G; Trafton, J. G

    2007-01-01

    What are the characteristics of long-term learning? We investigated the characteristics of long-term, symbolic learning using the Soar and ACT-R cognitive architectures running cognitive models of two simple tasks...

  19. Long-term disease-specific and cognitive quality of life after intensity-modulated radiation therapy: a cross-sectional survey of nasopharyngeal carcinoma survivors

    International Nuclear Information System (INIS)

    Kiang, Alan; Weinberg, Vivian K.; Cheung, Ka Ho Nicholas; Shugard, Erin; Chen, Josephine; Quivey, Jeanne M.; Yom, Sue S.

    2016-01-01

    There is a lack of data on quality of life in long-term survivors of nasopharyngeal carcinoma (NPC) who have been treated with intensity-modulated radiation therapy (IMRT). We characterized long-term disease-specific and cognitive QoL in NPC survivors after IMRT. We conducted a cross-sectional study of surviving patients diagnosed and treated for NPC at our center with curative-intent IMRT, with or without chemotherapy. Patients who were deceased, still undergoing treatment, with known recurrent disease, or treated with RT modality other than IMRT were excluded. QoL was measured by FACT-NP and FACT-Cog. Between May and November 2013, 44 patients completed cognitive (FACT-Cog), general (FACT-G), and NPC-specific (NPCS) QoL assessments. Patients were categorized into 4 cohorts based on duration since IMRT (≤2.5, >2.5–6, >6–10, and >10–16 years). There was no significant difference in age (p = 0.20) or stage ((I/II vs III/IV: p = 0.78) among the cohorts. The 4 cohorts differed overall for all QoL measures (ANOVA: p < 0.02 for each), due to improved scores >2.5–6 years post-IMRT compared with ≤2.5 years post-IMRT (post hoc tests: p ≤ 0.04 for each). No differences were observed between >2.5–6 and >6–10 years post-IMRT, but lower mean FACT-Cog and NPCS scores were observed for >10 years compared to >2.5–6 years post-IMRT (post hoc: p < 0.05 for each). All QoL measures were low during the initial recovery period (≤2.5 years) and were higher by 6 years post-IMRT. At >10 years post-IMRT, lower scores were observed in the domains of NPC-specific and cognitive QoL. Survivors of NPC, even if treated with IMRT, are at risk for detriment in domain-specific QoL measures at very long-term follow-up

  20. Quality of life in women undergoing urinary diversion for bladder cancer: results of a multicenter study among long-term disease-free survivors.

    Science.gov (United States)

    Gacci, Mauro; Saleh, Omar; Cai, Tommaso; Gore, John L; D'Elia, Carolina; Minervini, Andrea; Masieri, Lorenzo; Giannessi, Claudia; Lanciotti, Michele; Varca, Virginia; Simonato, Alchiede; Serni, Sergio; Carmignani, Giorgio; Carini, Marco

    2013-03-12

    Women undergoing radical cystectomy (RC) and urinary diversion for bladder cancer experience substantial limitations in health-related quality of life (HRQOL). However, the level of discomfort caused by different urinary diversion has been never evaluated in long term survivors. The aim of this multicenter study is to evaluate differences in HRQOL among recurrence-free women undergoing cutaneous ureterostomy (CUS), Bricker's ileal conduit (BK-IC) and Orthotopic neobladder VIP (ONB-VIP) in disease-free females treated with radical cystectomy (RC), with long-term follow up (mean 60.1 months; range 36-122 months). All consecutively treated female patients from two urological institutions who underwent RC and urinary diversion from January 2000 to December 2008, with no evidence of tumor recurrence at a minimum follow up of 36 months, were included. Patients received the European Organisation for Research and Treatment of Cancer (EORTC) generic (QLQ-C30) and bladder cancer-specific instruments (QLQ-BLM30) and the Functional Assessment of Cancer Therapy for Bladder Cancer (FACT-BL). Clinical data and questionnaire results were analyzed in order to evaluate the HRQOL differences among diversion groups. We identified 37 females (median age: 68, range 45-82 years), including 12 status-post CUS, 16 who underwent BK-IC, and 9 who underwent ONB-VIP. Most were healthy (24/37 with no comorbidities, 4/37 Charlson 1-2, 9/37 Charlson 3 or greater - we didn't considered bladder cancer in Charlson evaluation because bladder cancer was the main inclusion criteria). Women undergoing CUS endorsed worse FACT-BL scores compared with BK-IC and ONB-VIP patients, worse HRQOL regarding physical and emotional well-being (p=0.008 and p=0.02, respectively), and a trend toward worse EORTC QLQ-C30 scores for appetite loss and fatigue (p=0.05 for both). In our study long-term disease-free females treated with CUS endorsed worse HRQOL compared with women who underwent BK-IC or ONB-VIP, mostly due

  1. Lung function and long-term safety of tiotropium/olodaterol in East Asian patients with chronic obstructive pulmonary disease

    Directory of Open Access Journals (Sweden)

    Bai C

    2017-11-01

    Full Text Available Chunxue Bai,1 Masakazu Ichinose,2 Sang Haak Lee,3 Kwan Ho Lee,4 Olaf Jöns,5 Ulrich Bothner,6 Yihua Zhao,7 Roland Buhl8 1Department of Pulmonary Medicine, Shanghai Respiratory Research Institute, Zhongshan Hospital, Fudan University, Shanghai, China; 2Department of Respiratory Medicine, Tohoku University Graduate School of Medicine, Sendai, Japan; 3Department of Internal Medicine, St Paul’s Hospital, College of Medicine, The Catholic University of Korea, Seoul, South Korea; 4Department of Internal Medicine, Yeungnam University Medical Center, Daegu, South Korea; 5Department of Medicine TA Respiratory Diseases, 6Department of Pharmacovigilance, Boehringer Ingelheim Pharma GmbH & Co. KG, Ingelheim, Germany; 7Department of Biostatistics and Data Sciences, Boehringer Ingelheim Pharmaceuticals, Inc., Ridgefield, CT, USA; 8Pulmonary Department, Mainz University Hospital, Mainz, Germany Background and purpose: While the efficacy and safety of combined tiotropium and olodaterol in patients with COPD was established in a large clinical trial program, it is important to assess whether clinical data can be applied to geographic patient groups, particularly for East Asian patients who may have a different phenotypic profile to the global trial population. This study aimed to compare the lung function and safety profiles of tiotropium/olodaterol and monocomponents in East Asian and global populations from the TONADO® trials.Materials and methods: In the replicate, double-blind, parallel-group, active-controlled, randomized, 52-week, Phase III TONADO studies, patients received tiotropium/olodaterol, tiotropium, or olodaterol. We assessed the forced expiratory volume in 1 second (FEV1 area under the curve from 0 to 3 hours (AUC0–3 response and trough FEV1 response at 24 weeks for the approved doses, tiotropium/olodaterol 5/5 µg, tiotropium 5 µg, and olodaterol 5 µg. Treatment-emergent adverse events were recorded throughout treatment and ≤21 days

  2. Liver Fibrosis, but No Other Histologic Features, Is Associated With Long-term Outcomes of Patients With Nonalcoholic Fatty Liver Disease

    DEFF Research Database (Denmark)

    Angulo, Paul; Kleiner, David E; Dam-Larsen, Sanne

    2015-01-01

    BACKGROUND & AIMS: Histologic analysis of liver biopsy specimens allows for grading and staging of nonalcoholic fatty liver disease (NAFLD). We performed a longitudinal study to investigate the long-term prognostic relevance of histologic features for patients with NAFLD. METHODS: We performed...... a retrospective analysis of 619 patients diagnosed with NAFLD from 1975 through 2005 at medical centers in the United States, Europe, and Thailand. Patients underwent laboratory and biopsy analyses, and were examined every 3-12 months after their diagnosis. Outcomes analyzed were overall mortality, liver...... transplantation, and liver-related events. Cumulative outcomes were compared by log-rank analysis. Cox proportional-hazards regression was used to estimate adjusted hazard ratios (HRs). Time at risk was determined from the date of liver biopsy to the date of outcome or last follow-up examination. RESULTS: Over...

  3. Individualized Therapy Is a Long-Term Cost-Effective Method Compared to Dose Intensification in Crohn’s Disease Patients Failing Infliximab

    DEFF Research Database (Denmark)

    Steenholdt, Casper; Brynskov, Jørn; Thomsen, Ole

    2015-01-01

    .002. Figures were similar for patients having completed the 12-week trial as per protocol (50 % reduction in costs) (n = 45). Among patients continuing the allocated study intervention throughout the entire 20-week follow-up period (n = 29), costs were reduced by 60 % in algorithm-treated patients: $7......,056 versus $17,776; p year. Conclusion: Economic benefit of algorithm-based interventions at infliximab failure is maintained throughout 1 year. Background: In Crohn’s disease patients failing infliximab therapy, interventions defined...... by an algorithm based on infliximab and anti-infliximab antibody measurements have proven more cost-effective than intensifying the infliximab regimen. Aim: This study investigated long-term economic outcomes at the week 20 follow-up study visit and after 1 year. Clinical outcomes were assessed at week 20...

  4. Legionnaires' Disease Outbreak at a Long-Term Care Facility Caused by a Cooling Tower Using an Automated Disinfection System--Ohio, 2013.

    Science.gov (United States)

    Quinn, Celia; Demirjian, Alicia; Watkins, Louise Francois; Tomczyk, Sara; Lucas, Claressa; Brown, Ellen; Kozak-Muiznieks, Natalia; Benitez, Alvaro; Garrison, Laurel E; Kunz, Jasen; Brewer, Scott; Eitniear, Samantha; DiOrio, Mary

    2015-12-01

    On July 9, 2013, an outbreak of Legionnaires' disease (LD) was identified at Long-Term Care Facility A in central Ohio. This article describes the investigation of the outbreak and identification of the outbreak source, a cooling tower using an automated biocide delivery system. In total, 39 outbreak LD cases were identified; among these, six patients died. Water samples from a cooling tower were positive for Legionella pneumophila serogroup 1, reactive to monoclonal antibody 2, with matching sequence type to a patient isolate. An electronic control system turned off cooling tower pumps during low-demand periods, preventing delivery of disinfectant by a timed-release system, and leading to amplification of Legionella in the cooling tower. Guidelines for tower maintenance should address optimal disinfection when using automated systems.

  5. Living and managing with the long-term implications of neonatal chronic lung disease: The experiences and perspectives of children and their parents.

    Science.gov (United States)

    Bray, Lucy; Shaw, Nigel J; Snodin, Jill

    2015-01-01

    The purpose of the study was to investigate children's perspectives of living with chronic lung disease (CLD) and their parents' long-term experiences of caring for them. CLD caused by prematurity of birth is associated with continuing respiratory, neuro-developmental and psychosocial issues. 10 children (6-15 years old) with CLD and 12 parents were involved in semi-structured qualitative interviews. Data were analyzed using thematic analysis. CLD was described as 'getting easier over time' and that you 'learnt to live with it.' Expertise was acquired in controlling symptoms and recognizing the onset of illness, despite expressed uncertainty of the nature of the condition. Children experienced difficulties engaging in peer activities and encountered cases of teasing and isolation at school. CLD was reported as becoming easier over time to live with. Despite this, children experienced challenges engaging in peer activities and families described difficulties interacting with health and education services. Copyright © 2015 Elsevier Inc. All rights reserved.

  6. LONG TERM COLLECTIONS

    CERN Multimedia

    STAFF ASSOCIATION

    2010-01-01

    ACKNOWLEDGMENTS The Long-Term Collections (CLT) committee would like to warmly thank its faithful donors who, year after year, support our actions all over the world. Without you, all this would not be possible. We would like to thank, in particular, the CERN Firemen’s Association who donated 5000 CHF in the spring thanks to the sale of their traditional calendar, and the generosity of the CERN community. A huge thank you to the firemen for their devotion to our cause. And thank you to all those who have opened their door, their heart, and their purses! Similarly, we warmly thank the CERN Yoga Club once again for its wonderful donation of 2000 CHF we recently received. We would also like to tell you that all our projects are running well. Just to remind you, we are currently supporting the activities of the «Réflexe-Partage» Association in Mali; the training centre of «Education et Développement» in Abomey, Benin; and the orphanage and ...

  7. Long-term follow-up studies on iodine-131 treatment of hyperthyroid Graves' disease based on the measurement of thyroid volume by ultrasonography

    International Nuclear Information System (INIS)

    Tsuruta, Masako; Nagayama, Yuji; Yokoyama, Naokata; Izumi, Motomori; Nagataki, Shigenobu

    1993-01-01

    In the present series of studies, the long-term (four year) effect of 80 Gy of 131 I treatment was evaluated in patients with hyperthyroid Graves' disease whose thyroid volumes have been accurately estimated with a high resolution ultrasound scanner. One year after 131 I treatment, 23.1% (3 out of 13 patients) remained hyperthyroid, 69.2% (9 out of 13) became euthyroid, and 7.7% (1 out of 13) were in a hypothyroid state. Since three patients in a hyperthyroid state one year after treatment were subsequently treated with either antithyroid drugs or additional 131 I treatment, the remaining ten patients (9 euthyroid and 1 hypothyroid patients) have been followed up for three more years. Two patients developed a hypothyroid state three years after treatment and one patient four years after treatment. Overall, 60% (6 out of 10 patients) were in a euthyroid state and 40% (4 out of 10) in a hypothyroid state, four years after 80 Gy 131 I treatment. There was no significant difference between eu- and hypo-thyroid groups in the sex ratio, age, radiation doses, therapeutic dose, thyroid gland volume, 24-hr 131 I uptake, the effective half-life of 131 I in the thyroid or the duration of hyperthyroidism. In our preliminary studies, the incidence of late hypothyroidism in our 131 I treatment is similar to those previously reported. These suggest that uncertain factor(s), such as inhomogeneity of iodine distribution in the thyroid, unequal sensitivity of the thyroid cells to the radiation, and/or persistent destructive effects of the autoimmune process may influence the long-term effect of 131 I treatment of Graves' disease. (author)

  8. Cyst Ablation Using a Mixture of N-Butyl Cyanoacrylate and Iodized Oil in Patients with Autosomal Dominant Polycystic Kidney Disease: the Long-Term Results

    Energy Technology Data Exchange (ETDEWEB)

    Kim, See Hyung; Kim, Seung Hyup; Cho, Jeong Yeon [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    2009-08-15

    We wanted to assess the long-term results of cyst ablation with using N-butyl cyanoacrylate (NBCA) and iodized oil in patients with autosomal dominant polycystic kidney disease (ADPKD) and symptomatic cysts. Cyst ablation using a mixture of NBCA and iodized oil was performed in 99 cysts from 21 patients who had such symptoms as abdominal distension and pain. The collapse or reaccumulation of the ablated cysts after the procedure was assessed during the follow-up period of 36 to 90 months. The treatment effects, including symptom relief, and the clinical data such as the blood pressure and serum creatinine levels were also assessed, together with the complications. The procedure was technically successful in all 99 cysts from the 21 patients. Any procedure-related significant complications were not detected. Seventy-seven of 99 cysts (78%) were successfully collapsed on the follow-up CT. Twenty-two cysts showed reaccumulation during long-term follow-up period. The clinical symptoms were relieved in 17 of the 21 patients (76%). Four of 12 patients (33%) with hypertension and two of six patients (33%) with azotemia were improved. End stage renal disease (ESRD) occurred in six of the 21 patients (28%) during the follow-up period. The mean age of ESRD in our patients was 57 years. The mean time interval for the development of ESRD was 19 months. Ablation using a mixture of NBCA and iodized oil may be an effective, safe method for obtaining symptom relief in patients with ADPKD.

  9. Long-term follow-up studies on iodine-131 treatment of hyperthyroid Graves' disease based on the measurement of thyroid volume by ultrasonography

    Energy Technology Data Exchange (ETDEWEB)

    Tsuruta, Masako; Nagayama, Yuji; Yokoyama, Naokata; Izumi, Motomori; Nagataki, Shigenobu (Nagasaki Univ. (Japan). School of Medicine)

    1993-08-01

    In the present series of studies, the long-term (four year) effect of 80 Gy of [sup 131]I treatment was evaluated in patients with hyperthyroid Graves' disease whose thyroid volumes have been accurately estimated with a high resolution ultrasound scanner. One year after [sup 131]I treatment, 23.1% (3 out of 13 patients) remained hyperthyroid, 69.2% (9 out of 13) became euthyroid, and 7.7% (1 out of 13) were in a hypothyroid state. Since three patients in a hyperthyroid state one year after treatment were subsequently treated with either antithyroid drugs or additional [sup 131]I treatment, the remaining ten patients (9 euthyroid and 1 hypothyroid patients) have been followed up for three more years. Two patients developed a hypothyroid state three years after treatment and one patient four years after treatment. Overall, 60% (6 out of 10 patients) were in a euthyroid state and 40% (4 out of 10) in a hypothyroid state, four years after 80 Gy [sup 131]I treatment. There was no significant difference between eu- and hypo-thyroid groups in the sex ratio, age, radiation doses, therapeutic dose, thyroid gland volume, 24-hr [sup 131]I uptake, the effective half-life of [sup 131]I in the thyroid or the duration of hyperthyroidism. In our preliminary studies, the incidence of late hypothyroidism in our [sup 131]I treatment is similar to those previously reported. These suggest that uncertain factor(s), such as inhomogeneity of iodine distribution in the thyroid, unequal sensitivity of the thyroid cells to the radiation, and/or persistent destructive effects of the autoimmune process may influence the long-term effect of [sup 131]I treatment of Graves' disease. (author).

  10. Do mental health consumers want to improve their long-term disease risk behaviours? A survey of over 2000 psychiatric inpatients.

    Science.gov (United States)

    Bartlem, Kate; Bailey, Jacqueline; Metse, Alexandra; Asara, Ashley; Wye, Paula; Clancy, Richard; Wiggers, John; Bowman, Jenny

    2017-12-02

    Policies and clinical guidelines acknowledge the role mental health services have in addressing the physical health of individuals with a mental illness; however, little research has explored interest in reducing health risk behaviours or the acceptability of receiving support to reduce such risks among psychiatric inpatients. This study estimated the prevalence of four long-term disease risk behaviours (tobacco smoking, hazardous alcohol consumption, inadequate fruit and/or vegetable consumption, and inadequate physical activity); patient interest in reducing these risks; and acceptability of being provided care to do so during a psychiatric inpatient stay. A cross-sectional survey was undertaken with 2075 inpatients from four inpatient psychiatric facilities in one health district in Australia (October 2012-April 2014). Prevalence of risk behaviours ranged from 50.2% (inadequate physical activity) to 94.8% (inadequate fruit and/or vegetable consumption). The majority of respondents (88.4%) had more than one risk behaviour, and most were seriously considering improving their risk behaviours (47.6% to 65.3%). The majority (80.4%) agreed that it would be acceptable to be provided support and advice to change such behaviours during their psychiatric inpatient stay. Some diagnoses were associated with smoking and hazardous alcohol consumption, interest in reducing alcohol consumption and increasing fruit and/or vegetable consumption, and acceptability of receiving advice and support. The findings reinforce the need and opportunity for psychiatric inpatient facilities to address the long-term disease risk behaviours of their patients. © 2017 The Authors International Journal of Mental Health Nursing published by John Wiley & Sons Australia, Ltd on behalf of Australian College of Mental Health Nurses Inc.

  11. An association between long-term exposure to ambient air pollution and mortality from lung cancer and respiratory diseases in Japan.

    Science.gov (United States)

    Katanoda, Kota; Sobue, Tomotaka; Satoh, Hiroshi; Tajima, Kazuo; Suzuki, Takaichiro; Nakatsuka, Haruo; Takezaki, Toshiro; Nakayama, Tomio; Nitta, Hiroshi; Tanabe, Kiyoshi; Tominaga, Suketami

    2011-01-01

    Evidence for a link between long-term exposure to air pollution and lung cancer is limited to Western populations. In this prospective cohort study, we examined this association in a Japanese population. The study comprised 63 520 participants living in 6 areas in 3 Japanese prefectures who were enrolled between 1983 and 1985. Exposure to particulate matter less than 2.5 µm in aerodynamic diameter (PM(2.5)), sulfur dioxide (SO(2)), and nitrogen dioxide (NO(2)) was assessed using data from monitoring stations located in or nearby each area. The Cox proportional hazards model was used to calculate the hazard ratios associated with the average concentrations of these air pollutants. The 10-year average concentrations of PM(2.5), SO(2), and NO(2) before recruitment (1974-1983) were 16.8 to 41.9 µg/m(3), 2.4 to 19.0 ppb, and 1.2 to 33.7 ppb, respectively (inter-area range). During an average follow-up of 8.7 years, there were 6687 deaths, including 518 deaths from lung cancer. The hazard ratios for lung cancer mortality associated with a 10-unit increase in PM(2.5) (µg/m(3)), SO(2) (ppb), and NO(2) (ppb) were 1.24 (95% confidence interval: 1.12-1.37), 1.26 (1.07-1.48), and 1.17 (1.10-1.26), respectively, after adjustment for tobacco smoking and other confounding factors. In addition, a significant increase in risk was observed for male smokers and female never smokers. Respiratory diseases, particularly pneumonia, were also significantly associated with all the air pollutants. Long-term exposure to air pollution is associated with lung cancer and respiratory diseases in Japan.

  12. Cardiovascular diseases mortality following cancer during childhood: long term risk, role of chemotherapy and of radiation dose to heart and brain

    International Nuclear Information System (INIS)

    Tukenova, Markhaba; Guibout, Catherine; Oberlin, Odile; Doyon, Francoise; Moussannif, Abdedaid; Haddy, Nadia; Diallo, Ibrahima; Vathaire, Florent de; Pacquement, Helene; Hawkins, Mike; Winter, Dave

    2008-01-01

    Full text: Background: A multi-centric French-UK cohort study was performed to evaluate the role of treatment in the long-term overall and cause-specific mortality among childhood cancer survivors. Methods: This study cohort included 4,120 patients treated for a solid tumours before the age of 17 between 1942-1986, in 8 centres in France and UK and who survived at least 5 years from diagnosis. Detailed clinical and therapeutic data were extracted for each patients from medical records. For 2868 of the 2868 patients who received radiotherapy, radiation doses were estimated using DOS E G software at 188 anatomical sites, including heart (7 sites) and lungs (10 sites). We obtained the death causes of 95 % of dead patients. Overall and cause-specific mortality standardized ratios (SMR), absolute excess risk (AER) of death were studied using Poisson regression. Results: 603 patients died during the follow-up, i.e. 8.5-fold (95 % CI: 7.7-9.1) more than that expected in the general population. A total of 32 patients died of cardiovascular diseases, i.e. 4.8-fold (95 % CI, 3.3 to 6.7) more than expected, 21 of which were cardiac diseases, i.e. 6.0-fold more (95% CI, 3.8 to 9.0). Overall, patients who had received radiotherapy had a 5.4-fold (95% CI, 1.5 to 32.1) higher risk of mortality due to cardiovascular disease than those who had not. Mortality due to cardiac disease was related to the administration of alkylating agents and / or vinca alkaloids, and to that of anthracyclines. Each additional 100 mg of anthracyclines per m 2 of body surface area increased the mortality rate due to heart diseases by 92% (95% CI, 16% to 318%). Patients who had received between 5 to 14.9 Gy to the heart during radiotherapy had a 14.5-fold (95% CI, 2.0 to 291) higher risk of mortality from cardiac diseases than patients who had not received radiotherapy, this ratio being 32.6 (95% CI, 5.6 to 622) in those who had received more than 15 Gy. Conclusion: Childhood cancer survivors are at high

  13. Postoperative plasma cortisol levels predict long-term outcome in patients with Cushing's disease and determine which patients should be treated with pituitary irradiation after surgery

    International Nuclear Information System (INIS)

    Imaki, Toshihiro; Tsushima, Toshio; Hizuka, Naomi; Odagiri, Emi; Murata, Yoji; Takano, Kazue; Suda, Toshihiro

    2001-01-01

    Transsphenoidal surgery is the treatment of choice for ACTH-producing pituitary adenoma (Cushing's disease) and pituitary irradiation is widely considered the most appropriate treatment for patients with Cushing's disease for whom transsphenoidal surgery has been unsuccessful. We studied 49 consecutive patients who underwent transsphenoidal surgery for the treatment of Cushing's disease at Tokyo Women's Medical University from 1977-1997 with a mean follow-up duration of 87.6 months (range, 24-253 months). We examined the relationship between postoperative endocrinological data, assessed between 3 and 8 weeks after surgery, and long-term outcome and efficacy of pituitary irradiation after surgery. Long-term remission was defined as the regression of the symptom and signs of Cushing's syndrome, and restoration of normal levels of plasma ACTH, cortisol and urinary free cortisol, together with adequate suppression of morning plasma cortisol levels following the administration of low dose (1 mg) of dexamethasone. Thirty patients had no additional treatment after pituitary surgery. Only 1 of 25 patients (4%) whose postoperative plasma cortisol level was less than 2 μg/dl developed recurrent disease whereas 3 out of 5 patients with postoperative plasma cortisol levels higher than 2 μg/dl relapsed. Postoperative external pituitary radiation was used to treat the remaining 19 patients. Four patients who received radiation therapy had a low or undetectable postoperative plasma cortisol level (<2 μg/dl, 56 nmol/L) and all of these patients developed hypopituitarism whereas 5 patients with subnormal plasma cortisol levels (2.0-10.0 μg/dl) remained in remission. Among 10 patients with persistent disease after surgery, 6 entered remission 6-47 months after irradiation but one of them subsequently relapsed after 108 months. These results suggest that additional therapy should be avoided in patients with a postoperative plasma cortisol less than 2 μg/dl because relapse is

  14. Evaluating long term forecasts

    Energy Technology Data Exchange (ETDEWEB)

    Lady, George M. [Department of Economics, College of Liberal Arts, Temple University, Philadelphia, PA 19122 (United States)

    2010-03-15

    The U.S. Department of Energy's Energy Information Administration (EIA), and its predecessor organizations, has published projections of U.S. energy production, consumption, distribution and prices annually for over 30 years. A natural issue to raise in evaluating the projections is an assessment of their accuracy compared to eventual outcomes. A related issue is the determination of the sources of 'error' in the projections that are due to differences between the actual versus realized values of the associated assumptions. One way to do this would be to run the computer-based model from which the projections are derived at the time the projected values are realized, using actual rather than assumed values for model assumptions; and, compare these results to the original projections. For long term forecasts, this approach would require that the model's software and hardware configuration be archived and available for many years, possibly decades, into the future. Such archival creates many practical problems; and, in general, it is not being done. This paper reports on an alternative approach for evaluating the projections. In the alternative approach, the model is run many times for cases in which important assumptions are changed individually and in combinations. A database is assembled from the solutions and a regression analysis is conducted for each important projected variable with the associated assumptions chosen as exogenous variables. When actual data are eventually available, the regression results are then used to estimate the sources of the differences in the projections of the endogenous variables compared to their eventual outcomes. The results presented here are for residential and commercial sector natural gas and electricity consumption. (author)

  15. Long-term physical activity in leisure time and mortality from coronary heart disease, stroke, respiratory diseases, and cancer. The Copenhagen City Heart Study

    DEFF Research Database (Denmark)

    Schnohr, Peter; Lange, Peter; Scharling, Henrik

    2006-01-01

    BACKGROUND: The purpose of this study was to describe the associations between different levels of long-term physical activity in leisure time and subsequent causes of deaths. DESIGN: The Copenhagen City Heart Study is a prospective cardiovascular population study of 19 329 men and women aged 20......-93 in 1976. Physical activity in leisure time was estimated at the examinations in 1976-78 and 1981-83. This analysis consists of 2136 healthy men and 2758 women aged 20-79 years, with unchanged physical activity at the two examinations, and with all covariates included in the multivariate analyses: smoking...... gained years of expected lifetime from age 50. Men with high physical activity survived 6.8 years longer, and men with moderate physical activity 4.9 years longer than sedentary men. For women the figures were 6.4 and 5.5 years, respectively. CONCLUSION: Long-term moderate or high physical activity...

  16. Long-term outcomes of coronary artery bypass grafting versus stent-PCI for unprotected left main disease: a meta-analysis.

    Science.gov (United States)

    De Rosa, Salvatore; Polimeni, Alberto; Sabatino, Jolanda; Indolfi, Ciro

    2017-09-06

    Coronary artery bypass graft (CABG) surgery has traditionally represented the standard of care for left main coronary artery (LMCA) disease. However, percutaneous coronary intervention with stent implantation (PCI) has more recently emerged as a valuable alternative. The long-time awaited results of the largest randomized trials on the long-term impact of PCI versus CABG in LMCA disease, the newly published NOBLE and EXCEL studies, revealed contrasting results. Thus, aim of the present meta-analysis was to review the most robust evidence from randomized comparisons of CABG versus PCI for revascularization of LMCA. Randomized studies comparing long-term clinical outcomes of CABG or Stent-PCI for the treatment of LMCA disease were searched for in PubMed, the Chochrane Library and Scopus electronic databases. A total of 5 randomized studies were selected, including 4499 patients. No significant difference between CABG and PCI was found in the primary analysis on the composite endpoint of death, stroke and myocardial infarction (OR = 1·06 95% CI 0·80-1·40; p = 0·70). Similarly, no differences were observed between CABG and PCI for all-cause death (OR = 1·03 95% CI 0·81-1·32; p = 0·81). Although not statistically significant, a lower rate of stroke was registered in the PCI arm (OR = 0·86; p = 0·67), while a lower rate of myocardial infarction was found in the CABG arm (OR = 1·43; p = 0·17). On the contrary, a significantly higher rate of repeat revascularization was registered in the PCI arm (OR = 1·76 95% CI 1·45-2·13; p PCI and CABG for the treatment of LMCA disease in the composite endpoint of death, stroke and myocardial infarction. Hence, a large part of patients with unprotected left main coronary artery disease can be managed equally well by means of both these revascularization strategies.

  17. Long-term engraftment, graft-vs.-host disease, and immunologic reconstitution after experimental transplantation of allogeneic peripheral blood cells from G-CSF-treated donors.

    Science.gov (United States)

    Pan, L; Bressler, S; Cooke, K R; Krenger, W; Karandikar, M; Ferrara, J L

    1996-10-01

    Peripheral blood cells (PBPC) are an alternative source of bone marrow for allogeneic transplantation. Reports from recent clinical trials granulocyte colony-stimulating factor (G-CSF)-mobilized PBPC for allogeneic transplantation show incidence and severity of graft-vs.-host disease (GVHD) similar to those observed in conventional bone marrow transplantation (BMT), despite the presence of 10- to 20-fold more T cell in the PBPC inoculum. In the present study, we examined the effects of pretreatment of donors with G-CSF on GVHD, long-term engraftment, and lymphocyte reconstitution in a murine parent-->F1 model (B6.Ly-5a-->B6d2F1) using splenocytes as a source of peripheral progenitor cells. Recipients of splenocytes from G-CSF-treated donors experienced less mortality from acute GVHD and showed sustained weight gain by day 100 after transplantation. At that time, there was no histological evidence od GVHD in either liver or gut. Recipients of splenocytes from G-CSF-treated donors showed complete donor engraftment within 1 month, which was sustained until the end of the observation period. In contrast, recipients of T cell-depleted splenocytes showed slower donor engraftment and persistent donor/host chimerism. In addition, lymphocyte phenotype and function in mice receiving splenocytes from G-CSF-treated donors was significantly restored by day 100 after transplantation. Thus, the use of G-CSF-mobilized PBPC may provide significant advantages to conventional BMT by reducing GVHD without impairing long-term engraftment and immunologic reconstruction.

  18. Long-term symptom control of gastro-oesophageal reflux disease 12 years after laparoscopic Nissen or 180° anterior partial fundoplication in a randomized clinical trial.

    Science.gov (United States)

    Roks, D J; Broeders, J A; Baigrie, R J

    2017-06-01

    Laparoscopic 180° anterior fundoplication has been shown to achieve similar reflux control to Nissen fundoplication, with fewer side-effects, up to 5 years. However, there is a paucity of long-term follow-up data on this technique and antireflux surgery in general. This study reports 12-year outcomes of a double-blind RCT comparing laparoscopic Nissen versus 180° laparoscopic anterior fundoplication for gastro-oesophageal reflux disease (GORD). Patients with proven GORD were randomized to laparoscopic Nissen or 180° anterior fundoplication. The 12-year outcome measures included reflux control, dysphagia, gas-related symptoms and patient satisfaction. Measures included scores on a visual analogue scale, a validated Dakkak score for dysphagia and Visick scores. Of the initial 163 patients randomized (Nissen 84, anterior 79), 90 (55·2 per cent) completed 12-year follow-up (Nissen 52, anterior 38). There were no differences in heartburn, dysphagia, gas-related symptoms, patient satisfaction or surgical reintervention rate. Use of acid-suppressing drugs was less common after Nissen than after 180° anterior fundoplication: four of 52 (8 per cent) and 11 of 38 (29 per cent) respectively (P = 0·008). The proportion of patients with absent or only mild symptoms was slightly higher after Nissen fundoplication: 45 of 50 (90 per cent) versus 28 of 38 (74 per cent) (P = 0·044). The two surgical procedures provided similar control of heartburn and post-fundoplication symptoms, with similar patient satisfaction and reoperation rates on long-term follow-up. © 2017 BJS Society Ltd Published by John Wiley & Sons Ltd.

  19. Intermittent versus every-day mesalazine therapy in preventing complications of diverticular disease: a long-term follow-up study.

    Science.gov (United States)

    Tursi, A; Di Mario, F; Brandimarte, G; Elisei, W; Picchio, M; Loperfido, S; Dal Bo', N; Ferrara, F; Marcello, R; Heras Salvat, H; Scarpignato, C

    2013-12-01

    Mesalazine seems to be effective in preventing recurrence of acute uncomplicated diverticulitis (AUD), but the optimal mesalazine scheme to achieve these results is still debated. To assess the effectiveness of two different mesalazine-based treatments in preventing recurrence of AUD and the occurrence of other complications of diverticular disease (DD) during a long-term follow-up. We reviewed 311 patients suffer from recent episode of AUD and undergoing to mesalazine treatment: 207 (group A, 105 males, median age 63 years, range 47-74 years) were treated with mesalazine 1.6 g for 10 days each month, whilst 104 (group B, 55 males, median age 65 years, range 50-72 years) were treated with mesalazine 1.6 g every day. Patients were followed-up every 6 months (median 7.5 months, range 5-13 months). Patients were followed-up for a mean time of 3 years (range 12-72 months). Overall, occurrence of complication recurred more frequently in group A than in group B (p = 0.030, log-rank test). Acute diverticulitis recurred in 17 (8.2%) patients in group A and in 3 (2.9%) in group B; diverticular bleeding occurred in 4 (1.9%) patients in group A and in 1 (0.96%) patient in group B; surgery was required in 3 (1.4%) patients in group A and in no (0%) patient in group B. This is the first study showing that long-term mesalazine treatment is significantly better that intermittent mesalazine treatment in preventing occurrence of DD complications after an attack of acute diverticulitis.

  20. Chronic graft-versus-host disease in the rat radiation chimera: I. clinical features, hematology, histology, and immunopathology in long-term chimeras

    International Nuclear Information System (INIS)

    Beschorner, W.E.; Tutschka, P.J.; Santos, G.W.

    1982-01-01

    The clinical features, pathology, and immunopathology of chronic graft-versus-host disease (GVHD) developing in the long-term rat radiation chimera are described. At 6 to 12 months post-transplant, the previously stable ACI/LEW chimeras developed patchy to diffuse severe hair loss and thickened skin folds, and had microscopic features resembling scleroderma, Sjogren's syndrome, and chronic hepatitis. Skin histology showed dermal inflammation and acanthosis with atrophy of the appendages, with progression to dermal sclerosis. The liver revealed chronic hepatitis with bile duct injury and proliferation and periportal piecemeal necrosis. The tongue had considerable submucosal inflammation, muscular necrosis, and atrophy and arteritis. The serous salivary glands, lacrimal glands, and bronchi had lymphocytic inflammation and injury to duct, acinar, and mucosal columnar epithelium. The thymus had lymphocyte depletion of the medulla with prominent epithelium. The spleen and lymph nodes had poorly developed germinal centers but increased numbers of plasma cells. IgM was observed along the basement membrane and around the basal cells of the skin and tongue and along the basement membrane of the bile ducts. IgM was present also in the arteries of the tongue. Immunoglobulins eluted from the skin, cross-reacted with the bile duct epithelium and usually with both ACI and Lewis skin. Increased titers of speckled antinuclear antibodies were present in the serum of rats with chronic (GVHD). Chronic GVHD in the long-term rat radiation chimera is very similar to human chronic GVHD and is a potentially excellent model for autoimmune disorders including scleroderma, Sjorgren's syndrome, and chronic hepatitis

  1. Chronic graft-versus-host disease in the rat radiation chimera: I. clinical features, hematology, histology, and immunopathology in long-term chimeras

    Energy Technology Data Exchange (ETDEWEB)

    Beschorner, W.E.; Tutschka, P.J.; Santos, G.W.

    1982-04-01

    The clinical features, pathology, and immunopathology of chronic graft-versus-host disease (GVHD) developing in the long-term rat radiation chimera are described. At 6 to 12 months post-transplant, the previously stable ACI/LEW chimeras developed patchy to diffuse severe hair loss and thickened skin folds, and had microscopic features resembling scleroderma, Sjogren's syndrome, and chronic hepatitis. Skin histology showed dermal inflammation and acanthosis with atrophy of the appendages, with progression to dermal sclerosis. The liver revealed chronic hepatitis with bile duct injury and proliferation and periportal piecemeal necrosis. The tongue had considerable submucosal inflammation, muscular necrosis, and atrophy and arteritis. The serous salivary glands, lacrimal glands, and bronchi had lymphocytic inflammation and injury to duct, acinar, and mucosal columnar epithelium. The thymus had lymphocyte depletion of the medulla with prominent epithelium. The spleen and lymph nodes had poorly developed germinal centers but increased numbers of plasma cells. IgM was observed along the basement membrane and around the basal cells of the skin and tongue and along the basement membrane of the bile ducts. IgM was present also in the arteries of the tongue. Immunoglobulins eluted from the skin, cross-reacted with the bile duct epithelium and usually with both ACI and Lewis skin. Increased titers of speckled antinuclear antibodies were present in the serum of rats with chronic (GVHD). Chronic GVHD in the long-term rat radiation chimera is very similar to human chronic GVHD and is a potentially excellent model for autoimmune disorders including scleroderma, Sjorgren's syndrome, and chronic hepatitis.

  2. Altered duodenal microbiota composition in celiac disease patients suffering from persistent symptoms on a long-term gluten-free diet.

    Science.gov (United States)

    Wacklin, Pirjo; Laurikka, Pilvi; Lindfors, Katri; Collin, Pekka; Salmi, Teea; Lähdeaho, Marja-Leena; Saavalainen, Päivi; Mäki, Markku; Mättö, Jaana; Kurppa, Kalle; Kaukinen, Katri

    2014-12-01

    A significant fraction of celiac disease patients suffer from persistent symptoms despite a long-term gluten-free diet (GFD) and normalized small bowel mucosa. The commonly suggested reasons, such as inadvertent gluten-intake or presence of other gastrointestinal disease, do not explain the symptoms in all these patients. Recently, alterations in intestinal microbiota have been associated with autoimmune disorders, including celiac disease. This led us to test a hypothesis that abnormal intestinal microbiota may be associated with persisting gastrointestinal symptoms in treated celiac disease patients. Duodenal microbiota was analyzed in 18 GFD-treated patients suffering from persistent symptoms and 18 treated patients without symptoms by 16S rRNA gene pyrosequencing. The celiac disease patients had been following a strict GFD for several years and had restored small bowel mucosa and negative celiac autoantibodies. Their symptoms on GFD were assessed with Gastrointestinal Symptom Rating Scale. The results of several clustering methods showed that the treated celiac disease patients with persistent symptoms were colonized by different duodenal microbiota in comparison with patients without symptoms. The treated patients with persistent symptoms had a higher relative abundance of Proteobacteria (P=0.04) and a lower abundance of Bacteroidetes (P=0.01) and Firmicutes (P=0.05). Moreover, their microbial richness was reduced. The results indicated intestinal dysbiosis in patients with persistent symptoms even while adhering to a strict GFD. Our findings indicate that dysbiosis of microbiota is associated with persistent gastrointestinal symptoms in treated celiac disease patients and open new possibilities to treat this subgroup of patients.

  3. Selective impact of disease on short-term and long-term components of self-reported memory: a population-based HUNT study.

    Science.gov (United States)

    Almkvist, Ove; Bosnes, Ole; Bosnes, Ingunn; Stordal, Eystein

    2017-05-09

    Subjective memory is commonly considered to be a unidimensional measure. However, theories of performance-based memory suggest that subjective memory could be divided into more than one dimension. To divide subjective memory into theoretically related components of memory and explore the relationship to disease. In this study, various aspects of self-reported memory were studied with respect to demographics and diseases in the third wave of the HUNT epidemiological study in middle Norway. The study included all individuals 55 years of age or older, who responded to a nine-item questionnaire on subjective memory and questionnaires on health (n=18 633). A principle component analysis of the memory items resulted in two memory components; the criterion used was an eigenvalue above 1, which accounted for 54% of the total variance. The components were interpreted as long-term memory (LTM; the first component; 43% of the total variance) and short-term memory (STM; the second component; 11% of the total variance). Memory impairment was significantly related to all diseases (except Bechterew's disease), most strongly to brain infarction, heart failure, diabetes, cancer, chronic obstructive pulmonary disease and whiplash. For most diseases, the STM component was more affected than the LTM component; however, in cancer, the opposite pattern was seen. Subjective memory impairment as measured in HUNT contained two components, which were differentially associated with diseases. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  4. Long-term outcomes of liver transplant patients with human immunodeficiency virus infection and end-stage-liver-disease: single center experience

    Directory of Open Access Journals (Sweden)

    Vernadakis S

    2011-08-01

    Full Text Available Abstract Objective Orthotopic-liver-transplantation (OLT in patients with Human-Immunodeficiency-Virus infection (HIV and end-stage-liver-disease (ESDL is rarely reported. The purpose of this study is to describe our institutional experience on OLT for HIV positive patients. Material and methods This is a retrospective study of all HIV-infected patients who underwent OLT at the University Hospital of Essen, from January 1996 to December 2009. Age, sex, HIV transmission-way, CDC-stage, etiology of ESDL, concomitant liver disease, last CD4cell count and HIV-viral load prior to OLT were collected and analysed. Standard calcineurin-inhibitors-based immunosuppression was applied. All patients received anti-fungal and anti-pneumocystis carinii pneumonia prophylaxis post-OLT. Results Eight transplanted HIV-infected patients with a median age of 46 years (range 35-61 years were included. OLT indications were HCV (n = 5, HBV (n = 2, HCV/HBV/HDV-related cirrhosis (n = 1 and acute liver-failure (n = 1. At OLT, CD4 cell-counts ranged from 113-621 cells/μl, and HIV viral-loads from Conclusions OLT in HIV-infected patients and ESLD is an acceptable therapeutic option in selected patients. Long-term survival can be achieved without HIV disease-progression under antiretroviral therapy and management of the viral hepatitis co-infection.

  5. Does early verbal fluency decline after STN implantation predict long-term cognitive outcome after STN-DBS in Parkinson's disease?

    Science.gov (United States)

    Borden, Alaina; Wallon, David; Lefaucheur, Romain; Derrey, Stéphane; Fetter, Damien; Verin, Marc; Maltête, David

    2014-11-15

    An early and transient verbal fluency (VF) decline and impairment in frontal executive function, suggesting a cognitive microlesion effect may influence the cognitive repercussions related to subthalamic nucleus deep brain stimulation (STN-DBS). Neuropsychological tests including semantic and phonemic verbal fluency were administered both before surgery (baseline), the third day after surgery (T3), at six months (T180), and at an endpoint multiple years after surgery (Tyears). Twenty-four patients (mean age, 63.5 ± 9.5 years; mean disease duration, 12 ± 5.8 years) were included. Both semantic and phonemic VF decreased significantly in the acute post-operative period (44.4 ± 28.2% and 34.3 ± 33.4%, respectively) and remained low at 6 months compared to pre-operative levels (decrease of 3.4 ± 47.8% and 10.8 ± 32.1%) (P < 0.05). Regression analysis showed phonemic VF to be an independent factor of decreased phonemic VF at six months. Age was the only independent predictive factor for incident Parkinson's disease dementia (PDD) (F (4,19)=3.4, P<0.03). An acute post-operative decline in phonemic VF can be predictive of a long-term phonemic VF deficit. The severity of this cognitive lesion effect does not predict the development of dementia which appears to be disease-related. Copyright © 2014 Elsevier B.V. All rights reserved.

  6. Effect of Nonalcoholic Fatty Liver Disease on In-Hospital and Long-Term Outcomes in Patients With ST-Segment Elevation Myocardial Infarction.

    Science.gov (United States)

    Keskin, Muhammed; Hayıroğlu, Mert İlker; Uzun, Ahmet Okan; Güvenç, Tolga Sinan; Şahin, Sinan; Kozan, Ömer

    2017-11-15

    Nonalcoholic fatty liver disease (NAFLD) is a risk factor for coronary artery disease. We investigated the effect of NAFLD grade on in-hospital and long-term outcomes in patients with ST-segment elevation myocardial infarction (STEMI). The study group consisted of 360 patients with STEMI. The patients were classified according to the grade of the NAFLD using ultrasonography. Based on this classification, all patients were divided into 4 subgroups as grade 0 (no fatty liver disease), grade 1, grade 2, and grade 3. Hierarchical logistic regression and Cox proportional regression analysis were used to establish the relation between NAFLD grade and outcomes. In-hospital mortality for grade 0, 1, 2, and 3 NAFLDs were 4.7%, 8.3%, 11.3%, and 33.9%, respectively. Three-year mortality for grade 0, 1, 2, and 3 NAFLDs were 5.6%, 7.8%, 9.5%, and 33.3%, respectively. In the multivariable hierarchical logistic regression analysis, in-hospital mortality risks were higher for patients with grade 3 NAFLD (odds ratio 4.2). In a multivariable Cox proportional regression analysis, the mortality risk was higher for patients with grade 3 NAFLD (hazard ratio 4.0). In conclusion, in patients with STEMI, the presence of NAFLD is associated with unfavorable clinical outcomes. Among these patients, grade 3 NAFLD had the highest mortality rates. The present study supports NAFLD screening in patients with STEMI. Copyright © 2017 Elsevier Inc. All rights reserved.

  7. Long-term Course of Alzheimer Disease in Patients Treated According to the Dutch Dementia Guideline at a Memory Clinic: A "Real-Life" Study.

    Science.gov (United States)

    Droogsma, Erika; van Asselt, Dieneke; van Steijn, Jolanda; Diekhuis, Marjolein; Veeger, Nic; De Deyn, Peter P

    2016-01-01

    There is little knowledge of the long-term course of Alzheimer disease (AD) in light of current pharmacological and nonpharmacological interventions provided in a "real-life" setting. The Frisian Alzheimer's Disease Cohort study is a "real-life" study of the course of AD in patients (n=576) treated with pharmacological (ie, cholinesterase inhibitors) and nonpharmacological (ie, case management, respite care) interventions. Disease course was described by changes in cognition (Mini Mental State Examination, clock-drawing test) and number of types of professional care applying a repeated-measures analysis using a marginal model (population-based average model). In addition, behavioral and psychological symptoms, and proportions of nursing home admissions and deaths were investigated. During 3.5 years, the average Mini Mental State Examination decreased from 22.24 to 18.91, the clock-drawing test score increased from 3.38 to 4.05, the number of types of professional care increased from 0.85 to 2.64, and the patients with behavioral and psychological symptoms increased from 29.0% to 70.2%. The proportion of patients admitted to a nursing home was 40.8% and 41.0% died. Cognition and behaviour of AD patients deteriorated accompanied with an increase in care-dependency during 3.5 years. Nevertheless, compared with the precholinesterase inhibitor era, current pharmacological and nonpharmacological interventions appear to slow cognitive decline, which emphasizes that they seem to have a favorable effect.

  8. Long-term results of 2-mm punch grafting in patients with vitiligo vulgaris and segmental vitiligo: effect of disease activity

    NARCIS (Netherlands)

    Fongers, A.; Wolkerstorfer, A.; Nieuweboer-Krobotova, L.; Krawczyk, P.; Tóth, G. G.; van der Veen, J. P. W.

    2009-01-01

    Background Punch grafting is a simple and frequently used technique for the treatment of stable vitiligo, resistant to medical therapy. However, studies reporting long-term results are exceptional. Objectives To evaluate the long-term results of 2-mm punch grafting in patients with vitiligo vulgaris

  9. Long-term results of 2-mm punch grafting in patients with vitiligo vulgaris and segmental vitiligo : effect of disease activity

    NARCIS (Netherlands)

    Fongers, A.; Wolkerstorfer, A.; Nieuweboer-Krobotova, L.; Krawczyk, P.; Toth, G. G.; van der Veen, J. P. W.

    Background Punch grafting is a simple and frequently used technique for the treatment of stable vitiligo, resistant to medical therapy. However, studies reporting long-term results are exceptional. Objectives To evaluate the long-term results of 2-mm punch grafting in patients with vitiligo vulgaris

  10. Long-term results of 2-mm punch grafting in patients with vitiligo vulgaris and segmental vitiligo : effect of disease activity

    NARCIS (Netherlands)

    Fongers, A.; Wolkerstorfer, A.; Nieuweboer-Krobotova, L.; Krawczyk, P.; Toth, G. G.; van der Veen, J. P. W.

    2009-01-01

    Background Punch grafting is a simple and frequently used technique for the treatment of stable vitiligo, resistant to medical therapy. However, studies reporting long-term results are exceptional. Objectives To evaluate the long-term results of 2-mm punch grafting in patients with vitiligo vulgaris

  11. Long-term follow-up of patients treated with primary radiotherapy for supradiaphragmatic Hodgkin's disease at St. Jude Children's Research Hospital

    International Nuclear Information System (INIS)

    Shah, Amit B.; Hudson, Melissa M.; Poquette, Catherine A.; Luo Xiaolong; Wilimas, Judith A.; Kun, Larry E.

    1999-01-01

    Objective: To assess disease control, patterns of relapse, factors predictive of relapse, and late effects of treatment, we reviewed all cases of supradiaphragmatic (SD) Hodgkin's disease (HD) treated with primary radiation therapy (RT) at our institution. Methods: We retrospectively reviewed the disease characteristics, treatment history, and long-term outcome of the 106 patients with Stage I and II supradiaphragmatic HD who received definitive irradiation at St. Jude Children's Research Hospital between 1970 and 1995. As of the date of analysis, 95 patients are alive, with a median follow-up of 13.3 years (range, 1.9-24.2 years). Results: The median age at diagnosis was 14.7 years (range, 3.7-22.7). Involved-field RT was given to 13 patients (12%), whereas 37 (35%) had mantle RT, 51 patients (48%) had subtotal nodal irradiation, and 5 (5%) had total nodal irradiation. Relapsed disease developed in 26 patients at a median of 1.8 years (range, 0.2-9.3 years). The 5- and 10-year estimated cumulative incidences of relapse were 20.9% ± 4.0% and 25.1% ± 4.3%, respectively. With a median dose of 36 Gy (range, 32-40), in-field failure rate was 6.2%, whereas subdiaphragmatic relapse in sites irradiated prophylactically was 1.5%. There was a trend toward an increased incidence of relapse with higher ESR (p 0.088) and greater number of sites of disease (p = 0.087). Age, stage, histology, nodal disease ≥6 cm, the presence of bulky mediastinal disease, and the method of staging did not affect the incidence of relapse. The pattern of failure could not be predicted based on the stage of disease, the extent of subdiaphragmatic staging, the extent of radiation therapy, or the sequence of RT fields - 'ping pong' vs. sequential. Subset analysis of Stage II patients revealed significantly more relapses in clinically staged patients. Excluding Stage IA patients with high cervical disease or peripheral nodal disease, nodal extension failures were more common for patients

  12. Disease course and long-term outcome of juvenile localized scleroderma: Experience from a single pediatric rheumatology Centre and literature review.

    Science.gov (United States)

    Martini, Giorgia; Fadanelli, Gloria; Agazzi, Anna; Vittadello, Fabio; Meneghel, Alessandra; Zulian, Francesco

    2018-05-03

    Juvenile Localized Scleroderma (JLS) is a rare disorder that may cause severe aesthetic sequelae and functional disability. To date, data on natural history and long-term outcome are discordant and difficult to compare due to the heterogeneity of clinical subtypes, treatments and methods to evaluate activity and outcome in previous studies. A retrospective and cross-sectional study including 133 patients followed between January 1991 and December 2016 was conducted at our Pediatric Rheumatology Centre. Disease course was drawn by retrospective analysis of patients' clinical features, treatment, disease course and outcome at the last evaluation. Disease activity and severity of tissue damage were assessed by using parameters derived from the Localized Scleroderma Cutaneous Assessment Tool (LoSCAT) and thermography. Most patients achieved complete remission, as only 12.5%, all with the linear subtype, had still active disease after over 10 years of follow-up. At least one disease relapse occurred in 22.2% of patients and first flare was observed 20 months after first treatment discontinuation. Mild tissue damage was observed in more than half of patients, in 25.4% was moderate and in 23.0% severe; 19.8% presented a functional limitation. The entity of skin and subcutaneous fat loss established at the early stages of the disease as 27.8% of patients with shorter disease duration had severe damage and the rates remained constant in patients with longer follow-up. The delay in start of systemic treatment was associated with longer disease activity and higher relapse rate. Patients with linear scleroderma (LS), pansclerotic morphea (PM) and mixed subtype (MS) presented more severe aesthetic and functional damage but did not differ from other subtypes as for rate of complete remission. JLS in some patients can be a very aggressive disease with persistent activity after >10 years and/or several disease relapses. As tissue damage establishes early in disease course a

  13. Ovarian Suppression With Triptorelin During Adjuvant Breast Cancer Chemotherapy and Long-term Ovarian Function, Pregnancies, and Disease-Free Survival: A Randomized Clinical Trial.

    Science.gov (United States)

    Lambertini, Matteo; Boni, Luca; Michelotti, Andrea; Gamucci, Teresa; Scotto, Tiziana; Gori, Stefania; Giordano, Monica; Garrone, Ornella; Levaggi, Alessia; Poggio, Francesca; Giraudi, Sara; Bighin, Claudia; Vecchio, Carlo; Sertoli, Mario Roberto; Pronzato, Paolo; Del Mastro, Lucia

    Whether the administration of luteinizing hormone-releasing hormone analogues (LHRHa) during chemotherapy is a reliable strategy to preserve ovarian function is controversial owing to both the lack of data on long-term ovarian function and pregnancies and the safety concerns about the potential negative interactions between endocrine therapy and chemotherapy. To evaluate long-term results of LHRHa-induced ovarian suppression during breast cancer chemotherapy. Parallel, randomized, open-label, phase 3 superiority trial conducted at 16 Italian sites. Between October 2003 and January 2008, 281 premenopausal women with stage I to III hormone receptor-positive or hormone receptor-negative breast cancer were enrolled. Last annual follow-up was June 3, 2014. Patients were randomized to receive adjuvant or neoadjuvant chemotherapy alone (control group) or chemotherapy plus triptorelin (LHRHa group). The primary planned end point was incidence of chemotherapy-induced early menopause. Post hoc end points were long-term ovarian function (evaluated by yearly assessment of menstrual activity and defined as resumed by the occurrence of at least 1 menstrual cycle), pregnancies, and disease-free survival (DFS). A total of 281 women (median age, 39 [range, 24-45] years) were randomized. Median follow-up was 7.3 years (interquartile range, 6.3-8.2 years). The 5-year cumulative incidence estimate of menstrual resumption was 72.6% (95% CI, 65.7%-80.3%) among the 148 patients in the LHRHa group and 64.0% (95% CI, 56.2%-72.8%) among the 133 patients in the control group (hazard ratio [HR], 1.28 [95% CI, 0.98-1.68]; P = .07; age-adjusted HR, 1.48 [95% CI, 1.12-1.95]; P = .006). Eight pregnancies (5-year cumulative incidence estimate of pregnancy, 2.1% [95% CI, 0.7%-6.3%]) occurred in the LHRHa group and 3 (5-year cumulative incidence estimate of pregnancy, 1.6% [95% CI, 0.4%-6.2%]) in the control group (HR, 2.56 [95% CI, 0.68-9.60]; P = .14; age-adjusted HR, 2.40 [95% CI, 0

  14. Comparative long-term results of mitral valve repair in adults with chronic rheumatic disease and degenerative disease: is repair for "burnt-out" rheumatic disease still inferior to repair for degenerative disease in the current era?

    Science.gov (United States)

    Dillon, Jeswant; Yakub, Mohd Azhari; Kong, Pau Kiew; Ramli, Mohd Faizal; Jaffar, Norfazlina; Gaffar, Intan Fariza

    2015-03-01

    Mitral valve repair is perceived to be of limited durability for advanced rheumatic disease in adults. We aim to examine the long-term outcomes of repair for rheumatic disease, identify predictors of durability, and compare with repair for degenerative disease. Rheumatic and degenerative mitral valve repairs in patients aged 40 years or more were prospectively analyzed. The primary outcomes investigated were mortality, freedom from reoperation, and valve failure. Logistic regression analysis was performed to define predictors of poor outcome. Between 1997 and 2011, 253 rheumatic and 148 degenerative mitral valves were repaired. The age of patients in both groups was similar, with a mean of 54.1 ± 8.4 years versus 55.6 ± 7.3 years (P = .49). Freedom from reoperation for rheumatic valves at 5 and 10 years was 98.4%, comparable to 95.3% (P = .12) for degenerative valves. Freedom from valve failure at 5 and 10 years was 91.4% and 81.5% for rheumatic repairs and 82.5% and 75.4% for degenerative repairs, respectively (P = .15). The presence of residual mitral regurgitation greater than 2+ before discharge was the only significant independent predictor of reoperation, whereas residual mitral regurgitation greater than 2+ and leaflet procedures were significant risk factors for valve failure. The durability of rheumatic mitral valve repair in the current era has improved and is comparable to the outstanding durability of repairs for degenerative disease, even in the adult rheumatic population. Modifications of standard repair techniques, adherence to the importance of good leaflet coaptation, and strict quality control with stringent use of intraoperative transesophageal echocardiography have all contributed to the improved long-term results. Copyright © 2015 The American Association for Thoracic Surgery. Published by Elsevier Inc. All rights reserved.

  15. Sustained Improvement of Arterial Stiffness and Blood Pressure after Long-Term Rosuvastatin Treatment in Patients with Inflammatory Joint Diseases: Results from the RORA-AS Study.

    Directory of Open Access Journals (Sweden)

    Eirik Ikdahl

    Full Text Available Patients with inflammatory joint diseases (IJD have a high prevalence of hypertension and increased arterial stiffness. The aim of the present study was to evaluate the effect of long-term rosuvastatin treatment on arterial stiffness, measured by augmentation index (AIx and aortic pulse wave velocity (aPWV, and blood pressure (BP in IJD patients with established atherosclerosis.Eighty-nine statin naïve IJD patients with carotid atherosclerotic plaque(s (rheumatoid arthritis n = 55, ankylosing spondylitis n = 23, psoriatic arthritis n = 11 received rosuvastatin for 18 months to achieve low-density lipoprotein cholesterol goal ≤1.8 mmol/L. Change in AIx (ΔAIx, aPWV (ΔaPWV, systolic BP (ΔsBP and diastolic BP (ΔdBP from baseline to study end was assessed by paired samples t-tests. Linear regression was applied to evaluate associations between cardiovascular disease (CVD risk factors, rheumatic disease specific variables and medication, and ΔAIx, ΔaPWV, ΔsBP and ΔdBP.AIx, aPWV, sBP and dBP were significantly reduced from baseline to study end. The mean (95%CI changes were: ΔAIx: -0.34 (-0.03, -0.65% (p = 0.03, ΔaPWV: -1.69 (-0.21, -3.17 m/s2 (p = 0.03, ΔsBP: -5.27 (-1.61, -8.93 mmHg (p = 0.004 and ΔdBP -2.93 (-0.86, -5.00 mmHg (p = 0.01. In linear regression models, ∆aPWV was significantly correlated with ΔsBP and ΔdBP (for all: p<0.001.There is an unmet need of studies evaluating CVD prevention in IJD patients. We have shown for the first time that long-term intensive lipid lowering with rosuvastatin improved arterial stiffness and induced a clinically significant BP reduction in patients with IJD. These improvements were linearly correlated and may represent novel insight into the pleiotropic effects by statins.ClinicalTrials.gov NCT01389388.

  16. Navigating Long-Term Care

    Directory of Open Access Journals (Sweden)

    James D. Holt MD

    2017-03-01

    Full Text Available Americans over age 65 constitute a larger percentage of the population each year: from 14% in 2010 (40 million elderly to possibly 20% in 2030 (70 million elderly. In 2015, an estimated 66 million people provided care to the ill, disabled, and elderly in the United States. In 2000, according to the Centers for Disease Control and Prevention (CDC, 15 million Americans used some form of long-term care: adult day care, home health, nursing home, or hospice. In all, 13% of people over 85 years old, compared with 1% of those ages 65 to 74, live in nursing homes in the United States. Transitions of care, among these various levels of care, are common: Nursing home to hospital transfer, one of the best-studied transitions, occurs in more than 25% of nursing home residents per year. This article follows one patient through several levels of care.

  17. Hyperthyroidism in patients with ischaemic heart disease after iodine load induced by coronary angiography: Long-term follow-up and influence of baseline thyroid functional status.

    Science.gov (United States)

    Bonelli, Nadia; Rossetto, Ruth; Castagno, Davide; Anselmino, Matteo; Vignolo, Francesca; Parasiliti Caprino, Mirko; Gaita, Fiorenzo; Ghigo, Ezio; Garberoglio, Roberto; Grimaldi, Roberto; Maccario, Mauro

    2018-02-01

    To study the effect of a iodine load on thyroid function of patients with ischaemic heart disease (IHD) and the long-term influence of unknown subclinical hyperthyroidism. Subclinical hyperthyroidism is considered an independent risk factors for cardiovascular morbidity of patients with IHD. They routinely undergo coronary angiography with iodine contrast media (ICM) which may induce or even worsen hyperthyroidism. A cross-sectional study followed by a longitudinal study on patients with subclinical hyperthyroidism. 810 consecutive IHD outpatients without known thyroid diseases or treatment with drugs influencing thyroid activity undergoing elective coronary angiography. We evaluated thyroid function either before and 1 month after ICM; patients with thyrotoxicosis at baseline or after ICM were then followed up for 1 year. 58 patients had hyperthyroidism at baseline (HB, 7.2%), independently associated to FT4 levels, thyroid nodules and family history of thyroid diseases. After ICM, the prevalence of hyperthyroidism was 81 (10%). Hyperthyroidism after ICM was positively predicted by baseline fT4 levels, thyroid nodules, age over 60, male gender, family history of thyroid diseases. Three months after ICM, 34 patients (4.2%) still showed hyperthyroidism (22 from HB, 13 treated with methimazole). One year after ICM, hyperthyroidism was still present in 20 patients (2.5%, all from HB, 13 treated). The prevalence of spontaneous subclinical hyperthyroidism in IHD is surprisingly elevated and is further increased by iodine load, particularly in patients with thyroid nodules and familial history of thyroid diseases, persisting in a not negligible number of them even after one year. © 2017 John Wiley & Sons Ltd.

  18. Long Term Care

    Science.gov (United States)

    ... can help! CLICK HERE TO FIND YOUR LOCAL SOCIAL WORKER. Futher Reading ARTICLE TITLE AUTHOR HDSA PUBLICATION DATE Choosing Home Healthcare Assistance The Marker Fall 2001 Palliative Care in Huntington’s Disease Richard ...

  19. Long term health-related quality of life after burns is strongly dependent on pre-existing disease and psychosocial issues and less due to the burn itself.

    Science.gov (United States)

    Orwelius, L; Willebrand, M; Gerdin, B; Ekselius, L; Fredrikson, M; Sjöberg, F

    2013-03-01

    Health-related quality of life (HRQoL) is reduced after a burn, and is affected by coexisting conditions. The aims of the investigation were to examine and describe effects of coexisting disease on HRQoL, and to quantify the proportion of burned people whose HRQoL was below that of a reference group matched for age, gender, and coexisting conditions. A nationwide study covering 9 years and examined HRQoL 12 and 24 months after the burn with the SF-36 questionnaire. The reference group was from the referral area of one of the hospitals. The HRQoL of the burned patients was below that of the reference group mainly in the mental dimensions, and only single patients were affected in the physical dimensions. The factor that significantly affected most HRQoL dimensions (n=6) after the burn was unemployment, whereas only smaller effects could be attributed directly to the burn. Poor HRQoL was recorded for only a small number of patients, and the decline were mostly in the mental dimensions when compared with a group adjusted for age, gender, and coexisting conditions. Factors other than the burn itself, such as mainly unemployment and pre-existing disease, were most important for the long term HRQoL experience in these patients. Copyright © 2012 Elsevier Ltd and ISBI. All rights reserved.

  20. Predicting short and long-term exercise intentions and behaviour in patients with coronary artery disease: A test of protection motivation theory.

    Science.gov (United States)

    Tulloch, Heather; Reida, Robert; D'Angeloa, Monika Slovinec; Plotnikoff, Ronald C; Morrina, Louise; Beatona, Louise; Papadakisa, Sophia; Pipe, Andrew

    2009-03-01

    The purpose of this study was to examine the utility of protection motivation theory (PMT) in the prediction of exercise intentions and behaviour in the year following hospitalisation for coronary artery disease (CAD). Patients with documented CAD (n = 787), recruited at hospital discharge, completed questionnaires measuring PMT's threat (i.e. perceived severity and vulnerability) and coping (i.e. self-efficacy, response efficacy) appraisal constructs at baseline, 2 and 6 months, and exercise behaviour at baseline, 6 and 12 months post-hospitalisation. Structural equation modelling showed that the PMT model of exercise at 6 months had a good fit with the empirical data. Self-efficacy, response efficacy, and perceived severity predicted exercise intentions, which, in turn predicted exercise behaviour. Overall, the PMT variables accounted for a moderate amount of variance in exercise intentions (23%) and behaviour (20%). In contrast, the PMT model was not reliable for predicting exercise behaviour at 12 months post-hospitalisation. The data provided support for PMT applied to short-term, but not long-term, exercise behaviour among patients with CAD. Health education should concentrate on providing positive coping messages to enhance patients' confidence regarding exercise and their belief that exercise provides health benefits, as well as realistic information about disease severity.

  1. [Primary prevention of cardiovascular diseases: long term results of five year long preventive intervention in 12-year old boys (ten year prospective study)].

    Science.gov (United States)

    Rozanov, V B; Aleksandov, A A; Shugaeva, E N; Perova, N V; Maslennikova, G Ia; Smirnova, S G; Olfer'ev, A M

    2007-01-01

    In a longitudinal cohort (prevention group, n=213, comparison group, n=163) of 10-year prospective follow-up we addressed efficacy of 5-year-long multifactor preventive intervention, conducted in a sample of population of 12 year old boys. Preventive intervention was carried out both at populational level and among persons with risk factors of development of cardiovascular diseases with the use of group, individual, and partly family approaches, and was directed at rationalization of nutrition, elevation of physical activity and prevention of harmful habits. During first 3 years of prevention we succeeded to achieve stable statistically significant lowering of mean levels of total cholesterol, low density lipoprotein cholesterol, triglycerides, and atherogeneity index, as well as to affect fatty component of body mass (skinfold thickness). Long term effect of 5-year long preventive intervention manifested as significantly lower level of systolic blood pressure, lower prevalence of low levels of high density lipoprotein cholesterol, smaller increment of low density lipoprotein cholesterol and index of atherogeneity in the prevention group. These results evidence that prevention of main factors of risk of development of cardiovascular diseases (obesity, arterial hypertension, disorders of lipid composition of the blood, and low physical activity) in child and adolescent age in the period of active growth and development is feasible, effective, safe and is able to lead to decrease of levels of these factors in adults, but should last uninterruptedly until formation of stable habits of healthy life style.

  2. Comparison of Long terms Follow up Results in Patients with Cervical Disk DiseaseTreated With Anterior PEEK CageImplantation and Without it in Rasoul Akram Hospital

    Directory of Open Access Journals (Sweden)

    Mir Abolfazl Motiei

    2012-08-01

    Full Text Available Abstract Background: Anterior interbody fusion of the cervical spine have become the gold standard for treating spinal diseases, hence the aim of this study was to compare long term follow up results in patients with cervical disk disease treated with anterior PEEK cage implantation and without it in anterior approach. Methods: Retrospectively 63 patients with known cervical discogenic disorders who went under surgery with and without cage implantation were enrolled. The neurological examination and neurologic function were assessed by using the Japanese Orthopedic Association (JOA scoring system and neurological cervical spine scale (NCSS before and 8 years after surgery in each patient and at the end all complications were recorded. Results: In the first group, there were 15 males and 14 females (mean age: 49±10 years and in the second group there were 27 male and 7 female (mean age: 47±9 years. The NCSS score was significantly different between two groups after surgery (p=0.035 but there was no significant difference before surgery (p=0.163. No statistical significance difference was also observed in JOA score and complications before and after procedure, but JOA post surgery score between two groups had significant difference (p=0.047 . Conclusion: In conclusion, present study showed that PEEK cage implantation is a highly useful alternative to the conventional treatment methods.

  3. Long-term ambient concentrations of total suspended particulates and oxidants as related to incidence of chronic disease in California Seventh-Day Adventists

    Energy Technology Data Exchange (ETDEWEB)

    Abbey, D.E.; Mills, P.K.; Petersen, F.F.; Beeson, W.L. (Loma Linda Univ. School of Medicine, CA (United States))

    1991-08-01

    Cancer incidence and mortality in a cohort of 6000 nonsmoking California Seventh-Day Adventists were monitored for a 6-year period, and relationships with long-term cumulative air pollution were observed. Total suspended particulates (TSP) and ozone were measured in terms of numbers of hours in excess of several threshold levels corresponding to national standards as well as mean concentration. For all malignant neoplasms among females, risk increased with increasing exceedance frequencies of all thresholds of TSP except the lowest one, and those increased risks were highly statistically significant. For respiratory cancers, increased risk was associated with only one threshold of ozone, and this result was of borderline significance. Respiratory disease symptoms were assessed in 1977 and again in 1987 using the National Heart, Lung and Blood Institute respiratory symptoms questionnaire on a subcohort of 3914 individuals. Multivariate analyses which adjusted for past and passive smoking and occupational exposures indicated statistically significantly elevated relative risks ranging up to 1.7 for incidence of asthma, definite symptoms of airway obstructive disease, and chronic bronchitis with TSP in excess of all thresholds except the lowest one but not for any thresholds of ozone. A trend association was noted between the threshold of 10 pphm ozone and incidence of asthma. These results are presented within the context of standards setting for these constituents of air pollution.

  4. Long-term ambient concentrations of total suspended particulates and oxidants as related to incidence of chronic disease in California Seventh-Day Adventists.

    Science.gov (United States)

    Abbey, D E; Mills, P K; Petersen, F F; Beeson, W L

    1991-08-01

    Cancer incidence and mortality in a cohort of 6000 nonsmoking California Seventh-Day Adventists were monitored for a 6-year period, and relationships with long-term cumulative ambient air pollution were observed. Total suspended particulates (TSP) and ozone were measured in terms of numbers of hours in excess of several threshold levels corresponding to national standards as well as mean concentration. For all malignant neoplasms among females, risk increased with increasing exceedance frequencies of all thresholds of TSP except the lowest one, and those increased risks were highly statistically significant. For respiratory cancers, increased risk was associated with only one threshold of ozone, and this result was of borderline significance. Respiratory disease symptoms were assessed in 1977 and again in 1987 using the National Heart, Lung and Blood Institute respiratory symptoms questionnaire on a subcohort of 3914 individuals. Multivariate analyses which adjusted for past and passive smoking and occupational exposures indicated statistically significantly (p less than 0.05) elevated relative risks ranging up to 1.7 for incidence of asthma, definite symptoms of airway obstructive disease, and chronic bronchitis with TSP in excess of all thresholds except the lowest one but not for any thresholds of ozone. A trend association (p = 0.056) was noted between the threshold of 10 pphm ozone and incidence of asthma. These results are presented within the context of standards setting for these constituents of air pollution.

  5. Long Term Financing of Infrastructure

    OpenAIRE

    Sinha, Sidharth

    2014-01-01

    Infrastructure projects, given their long life, require long term financing. The main sources of long term financings are insurance and pension funds who seek long term investments with low credit risk. However, in India household financial savings are mainly invested in bank deposits. Insurance and pension funds account for only a small percentage of household financial savings. In addition most infrastructure projects do not qualify for investment by insurance and pension funds because of t...

  6. Timing of renal replacement therapy and long-term risk of chronic kidney disease and death in intensive care patients with acute kidney injury.

    Science.gov (United States)

    Christiansen, Søren; Christensen, Steffen; Pedersen, Lars; Gammelager, Henrik; Layton, J Bradley; Brookhart, M Alan; Christiansen, Christian Fynbo

    2017-12-28

    The optimal time to initiate renal replacement therapy (RRT) in intensive care unit (ICU) patients with acute kidney injury (AKI) is unclear. We examined the impact of early RRT on long-term mortality, risk of chronic kidney disease (CKD), and end-stage renal disease (ESRD). This cohort study included all adult patients treated with continuous RRT in the ICU at Aarhus University Hospital, Skejby, Denmark (2005-2015). Data were obtained from a clinical information system and population-based registries. Early treatment was defined as RRT initiation at AKI stage 2 or below, and late treatment was defined as RRT initiation at AKI stage 3. Inverse probability of treatment (IPT) weights were computed from propensity scores. The IPT-weighted cumulative risk of CKD (estimated glomerular filtration rate regression. The mortality, CKD, and ESRD analyses included 1213, 303, and 617 patients, respectively. The 90-day mortality in the early RRT group was 53.6% compared with 46.0% in the late RRT group (HR 1.24, 95% CI 1.03-1.48). The 90-day to 5-year mortality was 37.7% and 41.5% in the early and late RRT groups, respectively (HR 0.95, 95% CI 0.70-1.29). The 5-year risk of CKD was 35.9% in the early RRT group and 44.9% in the late RRT group (HR 0.74, 95% CI 0.46-1.18). The 5-year risk of ESRD was 13.3% in the early RRT group and 16.7% in the late RRT group (HR 0.79, 95% CI 0.47-1.32). Early initiation was associated with increased 90-day mortality. In patients surviving to day 90, early initiation was not associated with a major impact on long-term mortality or risk of CKD and ESRD. Despite potential residual confounding due to the observational design, our findings do not support that early RRT initiation is superior to late initiation.

  7. Long-Term Results After High-Dose Radiotherapy and Adjuvant Hormones in Prostate Cancer: How Curable Is High-Risk Disease?

    Energy Technology Data Exchange (ETDEWEB)

    Zapatero, Almudena, E-mail: azapatero.hlpr@salud.madrid.org [Department of Radiation Oncology, Hospital Universitario de la Princesa, Madrid (Spain); Garcia-Vicente, Feliciano [Department of Medical Physics, Hospital Universitario de la Princesa, Madrid (Spain); Martin de Vidales, Carmen; Cruz Conde, Alfonso; Ibanez, Yamile [Department of Radiation Oncology, Hospital Universitario de la Princesa, Madrid (Spain); Fernandez, Inmaculada; Rabadan, Mariano [Department of Urology, Hospital Universitario de la Princesa, Madrid (Spain)

    2011-12-01

    Purpose: To analyze long-term outcome and prognostic factors for high-risk prostate cancer defined by National Comprehensive Cancer Network criteria treated with high-dose radiotherapy and androgen deprivation in a single institution. Methods and Materials: A total of 306 patients treated between 1995 and 2007 in a radiation dose-escalation program fulfilled the National Comprehensive Cancer Network high-risk criteria. Median International Commission on Radiation Units and Measurements radiation dose was 78 Gy (range, 66.0-84.1 Gy). Long-term androgen deprivation (LTAD) was administered in 231 patients, short-term androgen deprivation (STAD) in 59 patients, and no hormones in 16 patients. The Phoenix (nadir plus 2 ng/mL) consensus definition was used for biochemical control. Multivariate analysis was performed to determine the independent prognostic impact of clinical and treatment factors. Median follow-up time was 64 months (range, 24-171 months). Results: The actuarial overall survival at 5 and 10 years was 95.7% and 89.8%, respectively, and the corresponding biochemical disease-free survival (bDFS) was 89.5% and 67.2%, respectively. Fourteen patients (4.6%) developed distant metastasis. Multivariate analysis showed that Gleason score >7 (p = 0.001), pretreatment prostate-specific antigen (PSA) level >20 ng/mL (p = 0.037), higher radiation dose (p = 0.005), and the use of adjuvant LTAD vs. STAD (p = 0.011) were independent prognostic factors affecting bDFS in high-risk disease. The 5-year bDFS for patients treated with LTAD plus radiotherapy dose >78 Gy was 97%. Conclusions: For high-risk patients the present series showed that the use of LTAD in conjunction with higher doses (>78 Gy) of radiotherapy was associated with improved biochemical tumor control. We observed that the presence of Gleason sum >7 and pretreatment PSA level >20 ng/mL in the same patient represents a 6.8 times higher risk of PSA failure. These men could be considered for clinical trials with

  8. Emerging communities of child-healthcare practice in the management of long-term conditions such as chronic kidney disease: qualitative study of parents' accounts.

    Science.gov (United States)

    Carolan, Ian; Smith, Trish; Hall, Andy; Swallow, Veronica M

    2014-07-07

    Parents of children and young people with long-term conditions who need to deliver clinical care to their child at home with remote support from hospital-based professionals, often search the internet for care-giving information. However, there is little evidence that the information available online was developed and evaluated with parents or that it acknowledges the communities of practice that exist as parents and healthcare professionals share responsibility for condition management. The data reported here are part of a wider study that developed and tested a condition-specific, online parent information and support application with children and young people with chronic-kidney disease, parents and professionals. Semi-structured interviews were conducted with 19 fathers and 24 mothers who had recently tested the novel application. Data were analysed using Framework Analysis and the Communities of Practice concept. Evolving communities of child-healthcare practice were identified comprising three components and several sub components: (1) Experiencing (parents making sense of clinical tasks) through Normalising care, Normalising illness, Acceptance & action, Gaining strength from the affected child and Building relationships to formalise a routine; (2) Doing (Parents executing tasks according to their individual skills) illustrated by Developing coping strategies, Importance of parents' efficacy of care and Fear of the child's health failing; and (3) Belonging/Becoming (Parents defining task and group members' worth and creating a personal identity within the community) consisting of Information sharing, Negotiation with health professionals and Achieving expertise in care. Parents also recalled factors affecting the development of their respective communities of healthcare practice; these included Service transition, Poor parent social life, Psycho-social affects, Family chronic illness, Difficulty in learning new procedures, Shielding and avoidance, and

  9. Mathematical modelling long-term effects of replacing Prevnar7 with Prevnar13 on invasive pneumococcal diseases in England and Wales.

    Directory of Open Access Journals (Sweden)

    Yoon Hong Choi

    Full Text Available England and Wales recently replaced the 7-valent pneumococcal conjugate vaccine (PCV7 with its 13-valent equivalent (PCV13, partly based on projections from mathematical models of the long-term impact of such a switch compared to ceasing pneumococcal conjugate vaccination altogether.A compartmental deterministic model was used to estimate parameters governing transmission of infection and competition between different groups of pneumococcal serotypes prior to the introduction of PCV13. The best-fitting parameters were used in an individual based model to describe pneumococcal transmission dynamics and effects of various options for the vaccination programme change in England and Wales. A number of scenarios were conducted using (i different assumptions about the number of invasive pneumococcal disease cases adjusted for the increasing trend in disease incidence prior to PCV7 introduction in England and Wales, and (ii a range of values representing serotype replacement induced by vaccination of the additional six serotypes in PCV13.Most of the scenarios considered suggest that ceasing pneumococcal conjugate vaccine use would cause an increase in invasive pneumococcal disease incidence, while replacing PCV7 with PCV13 would cause an overall decrease. However, the size of this reduction largely depends on the level of competition induced by the additional serotypes in PCV13. The model estimates that over 20 years of PCV13 vaccination, around 5000-62000 IPD cases could be prevented compared to stopping pneumococcal conjugate vaccination altogether.Despite inevitable uncertainty around serotype replacement effects following introduction of PCV13, the model suggests a reduction in overall invasive pneumococcal disease incidence in all cases. Our results provide useful evidence on the benefits of PCV13 to countries replacing or considering replacing PCV7 with PCV13, as well as data that can be used to evaluate the cost-effectiveness of such a switch.

  10. A long-term echocardiographic study of the course of valvular dysfunctions following discontinuation of ergot-derived dopamine agonists in patients with Parkinson's disease

    Directory of Open Access Journals (Sweden)

    Walter Serra

    2015-03-01

    Conclusions: This long-term study confirms an improvement of the restrictive VHD after withdrawal of EDA in PD patients. However, only a partial reversibility of cardiac valvular abnormalities was observed.

  11. Very long-term sequelae of craniopharyngioma.

    Science.gov (United States)

    Wijnen, Mark; van den Heuvel-Eibrink, Marry M; Janssen, Joseph A M J L; Catsman-Berrevoets, Coriene E; Michiels, Erna M C; van Veelen-Vincent, Marie-Lise C; Dallenga, Alof H G; van den Berge, J Herbert; van Rij, Carolien M; van der Lely, Aart-Jan; Neggers, Sebastian J C M M

    2017-06-01

    Studies investigating long-term health conditions in patients with craniopharyngioma are limited by short follow-up durations and generally do not compare long-term health effects according to initial craniopharyngioma treatment approach. In addition, studies comparing long-term health conditions between patients with childhood- and adult-onset craniopharyngioma report conflicting results. The objective of this study was to analyse a full spectrum of long-term health effects in patients with craniopharyngioma according to initial treatment approach and age group at craniopharyngioma presentation. Cross-sectional study based on retrospective data. We studied a single-centre cohort of 128 patients with craniopharyngioma treated from 1980 onwards (63 patients with childhood-onset disease). Median follow-up since craniopharyngioma presentation was 13 years (interquartile range: 5-23 years). Initial craniopharyngioma treatment approaches included gross total resection ( n  = 25), subtotal resection without radiotherapy ( n  = 44), subtotal resection with radiotherapy ( n  = 25), cyst aspiration without radiotherapy ( n  = 8), and 90 Yttrium brachytherapy ( n  = 21). Pituitary hormone deficiencies (98%), visual disturbances (75%) and obesity (56%) were the most common long-term health conditions observed. Different initial craniopharyngioma treatment approaches resulted in similar long-term health effects. Patients with childhood-onset craniopharyngioma experienced significantly more growth hormone deficiency, diabetes insipidus, panhypopituitarism, morbid obesity, epilepsy and psychiatric conditions compared with patients with adult-onset disease. Recurrence-/progression-free survival was significantly lower after initial craniopharyngioma treatment with cyst aspiration compared with other therapeutic approaches. Survival was similar between patients with childhood- and adult-onset craniopharyngioma. Long-term health conditions were comparable after

  12. Long-term survival and causes of death in patients with ST-elevation acute coronary syndrome without obstructive coronary artery disease.

    Science.gov (United States)

    Andersson, Hedvig Bille; Pedersen, Frants; Engstrøm, Thomas; Helqvist, Steffen; Jensen, Morten Kvistholm; Jørgensen, Erik; Kelbæk, Henning; Räder, Sune Bernd Emil Werner; Saunamäki, Kari; Bates, Eric; Grande, Peer; Holmvang, Lene; Clemmensen, Peter

    2018-01-07

    We aimed to study survival and causes of death in patients with ST-elevation acute coronary syndrome (STE-ACS) with and without obstructive coronary artery disease (CAD). We included 4793 consecutive patients with STE-ACS triaged for acute coronary angiography at a large cardiac invasive centre (2009-2014). Of these, 88% had obstructive CAD (stenosis ≥50%), 6% had non-obstructive CAD (stenosis 1-49%), and 5% had normal coronary arteries. Patients without obstructive CAD were younger and more often female with fewer cardiovascular risk factors. Median follow-up time was 2.6 years. Compared with patients with obstructive CAD, the short-term hazard of death (≤30 days) was lower in both patients with non-obstructive CAD [hazard ratio (HR) 0.49, 95% confidence interval (CI) 0.27-0.89, P = 0.018] and normal coronary arteries (HR 0.31, 95% CI 0.11-0.83, P = 0.021). In contrast, the long-term hazard of death (>30 days) was similar in patients with non-obstructive CAD (HR 1.15, 95% CI 0.77-1.72, P = 0.487) and higher in patients with normal coronary arteries (HR 2.44, 95% CI 1.58-3.76, P Causes of death were cardiovascular in 70% of patients with obstructive CAD, 38% with non-obstructive CAD, and 32% with normal coronary arteries. Finally, patients without obstructive CAD had lower survival compared with an age and sex matched general population. STE-ACS patients without obstructive CAD had a long-term risk of death similar to or higher than patients with obstructive CAD. Causes of death were less often cardiovascular. This suggests that STE-ACS patients without obstructive CAD warrant medical attention and close follow-up. Published on behalf of the European Society of Cardiology. All rights reserved. © The Author 2017. For permissions, please email: journals.permissions@oup.com.

  13. Long-term prospective longitudinal evaluation of emotional distress and quality of life in cervical cancer patients who remained disease-free 2-years from diagnosis.

    Science.gov (United States)

    Mantegna, Giovanna; Petrillo, Marco; Fuoco, Gilda; Venditti, Laura; Terzano, Serena; Anchora, Luigi Pedone; Scambia, Giovanni; Ferrandina, Gabriella

    2013-03-18

    A long-term prospective assessment of QoL in cervical cancer patients is still lacking. Here, we provide the first 2-years prospective, longitudinal study evaluating emotional distress and QoL in early stage (ECC) and locally advanced (LACC) cervical cancer patients who remained disease-free 2-years from diagnosis. The questionnaires: Hospital Anxiety and Depression Scale (HADS), Global Health Status items of EORTC QLQ-C30 (GHS), and EORTC QLQ-CX24 (CX24) have been administered by a dedicated team of psycho-oncologists, administered at baseline, and after 3, 6, 12 and 24 months from surgery The Generalized Linear Model for repeated measure was used to analyze modifications of QoL measures over time. In both groups, an early reduction of the percentage of patients with anxiety levels ≥11 was observed at the 3-month evaluation (ECC: 25.7% at baseline Vs 14.7% after 3 months, p value=0.001; LACC: 22.2% at baseline Vs 15.4% after 3 months, p value=0.001). Despite this favorable trend, after 2 years from diagnosis, 11.9% of ECC and 15.6% of LACC patients still showed an anxiety score ≥11. No significant changes over time were observed in term of Depression levels. Focusing on QoL issues, mean GHS and Sexual Activity scores showed an improvement over time in both groups compared to baseline (GHS: 5.7% difference for ECC, p value=0.001, and 11.0% in LACC, p value=0.001; SXA: 13.9% difference for ECC, p value=0.001; and 6.1% in LACC, p value=0.008). On the other hand, Body Image mean scores were significantly impaired by chemoradiation administration in LACC patients, without long-term recovery (7.5% difference, p value=0.001). Finally, in both groups, lymphedema (LY) and menopausal symptoms (MS) showed an early worsening which persisted 2-year after surgery (LY: 19.5% difference for ECC, p value=0.014, and 27.3% in LACC, p value=0.001; MS: 14.4% difference for ECC, p value=0.004, and 16.0% in LACC, p value=0.002). Despite a significant improvement over time, elevated

  14. Long-Term Improvement of Neurological Signs and Metabolic Dysfunction in a Mouse Model of Krabbe's Disease after Global Gene Therapy.

    Science.gov (United States)

    Marshall, Michael S; Issa, Yazan; Jakubauskas, Benas; Stoskute, Monika; Elackattu, Vince; Marshall, Jeffrey N; Bogue, Wil; Nguyen, Duc; Hauck, Zane; Rue, Emily; Karumuthil-Melethil, Subha; Zaric, Violeta; Bosland, Maarten; van Breemen, Richard B; Givogri, Maria I; Gray, Steven J; Crocker, Stephen J; Bongarzone, Ernesto R

    2018-03-07

    We report a global adeno-associated virus (AAV)9-based gene therapy protocol to deliver therapeutic galactosylceramidase (GALC), a lysosomal enzyme that is deficient in Krabbe's disease. When globally administered via intrathecal, intracranial, and intravenous injections to newborn mice affected with GALC deficiency (twitcher mice), this approach largely surpassed prior published benchmarks of survival and metabolic correction, showing long-term protection of demyelination, neuroinflammation, and motor function. Bone marrow transplantation, performed in this protocol without immunosuppressive preconditioning, added minimal benefits to the AAV9 gene therapy. Contrasting with other proposed pre-clinical therapies, these results demonstrate that achieving nearly complete correction of GALC's metabolic deficiencies across the entire nervous system via gene therapy can have a significant improvement to behavioral deficits, pathophysiological changes, and survival. These results are an important consideration for determining the safest and most effective manner for adapting gene therapy to treat this leukodystrophy in the clinic. Copyright © 2018 The American Society of Gene and Cell Therapy. Published by Elsevier Inc. All rights reserved.

  15. Positive and negative early life experiences differentially modulate long term survival and amyloid protein levels in a mouse model of Alzheimer's disease.

    Science.gov (United States)

    Lesuis, Sylvie L; Maurin, Herve; Borghgraef, Peter; Lucassen, Paul J; Van Leuven, Fred; Krugers, Harm J

    2016-06-28

    Stress has been implicated as a risk factor for the severity and progression of sporadic Alzheimer's disease (AD). Early life experiences determine stress responsivity in later life, and modulate age-dependent cognitive decline. Therefore, we examined whether early life experiences influence AD outcome in a bigenic mouse model which progressively develops combined tau and amyloid pathology (biAT mice).Mice were subjected to either early life stress (ELS) or to 'positive' early handling (EH) postnatally (from day 2 to 9). In biAT mice, ELS significantly compromised long term survival, in contrast to EH which increased life expectancy. In 4 month old mice, ELS-reared biAT mice displayed increased hippocampal Aβ levels, while these levels were reduced in EH-reared biAT mice. No effects of ELS or EH were observed on the brain levels of APP, protein tau, or PSD-95. Dendritic morphology was moderately affected after ELS and EH in the amygdala and medial prefrontal cortex, while object recognition memory and open field performance were not affected. We conclude that despite the strong transgenic background, early life experiences significantly modulate the life expectancy of biAT mice. Parallel changes in hippocampal Aβ levels were evident, without affecting cognition of young adult biAT mice.

  16. Long-term mortality after parathyroidectomy among chronic kidney disease patients with secondary hyperparathyroidism: a systematic review and meta-analysis.

    Science.gov (United States)

    Chen, Lin; Wang, Kongbo; Yu, Shanlan; Lai, Liping; Zhang, Xiaoping; Yuan, Jingjing; Duan, Weifeng

    2016-08-01

    Parathyroidectomy (PTx) and medical treatments are both recommended for reducing serum intact parathyroid hormone (iPTH) and curing secondary hyperparathyroidism (sHPT) in patients with chronic kidney disease (CKD), but their therapeutic effects on long-term mortality are not well-known. Thus, we aim to assess such therapeutic effect of PTx. Electronic literatures published on Pubmed, Embase, and Cochrane Central Register of Controlled Trials in any language until 27 November 2015 were systematically searched. All literatures that compared outcomes (survival rate or mortality rate) between PTx-treated and medically-treated CKD patients with sHPT were included. Finally, 13 cohort studies involving 22053 patients were included. Data were extracted from all included literatures in a standard form. The outcomes of all-cause and cardiovascular mortalities were assessed using DerSimonian and Laird's random effects model. We find PTx-treated versus medically-treated patients had a 28% reduction in all-cause mortality and a 37% reduction in cardiovascular mortality. Thus, PTx versus medical treatments might reduce the risks of all-cause and cardiovascular mortalities in CKD patients with sHPT. Further studies with prospective and large-sample clinical trials are needed to find out the real effect of PTx and to assess whether mortality rates differ among patterns of PTx.

  17. Long-term coffee consumption and risk of cardiovascular disease: a systematic review and a dose-response meta-analysis of prospective cohort studies.

    Science.gov (United States)

    Ding, Ming; Bhupathiraju, Shilpa N; Satija, Ambika; van Dam, Rob M; Hu, Frank B

    2014-02-11

    Considerable controversy exists on the association between coffee consumption and cardiovascular disease (CVD) risk. A meta-analysis was performed to assess the dose-response relationship of long-term coffee consumption with CVD risk. PubMed and EMBASE were searched for prospective cohort studies of the relationship between coffee consumption and CVD risk, which included coronary heart disease, stroke, heart failure, and CVD mortality. Thirty-six studies were included with 1 279 804 participants and 36 352 CVD cases. A nonlinear relationship of coffee consumption with CVD risk was identified (P for heterogeneity=0.09, P for trend coffee consumption (median, 0 cups per day), the relative risk of CVD was 0.95 (95% confidence interval, 0.87-1.03) for the highest category (median, 5 cups per day) category, 0.85 (95% confidence interval, 0.80-0.90) for the second highest category (median, 3.5 cups per day), and 0.89 (95% confidence interval, 0.84-0.94) for the third highest category (median, 1.5 cups per day). Looking at separate outcomes, coffee consumption was nonlinearly associated with both coronary heart disease (P for heterogeneity=0.001, P for trend 0.05) risks. A nonlinear association between coffee consumption and CVD risk was observed in this meta-analysis. Moderate coffee consumption was inversely significantly associated with CVD risk, with the lowest CVD risk at 3 to 5 cups per day, and heavy coffee consumption was not associated with elevated CVD risk.

  18. Long-Term Results after Placement of Aortic Bifurcation Self-Expanding Stents: 10 Year Mortality, Stent Restenosis, and Distal Disease Progression

    International Nuclear Information System (INIS)

    Houston, J. Graeme; Bhat, Raj; Ross, Rose; Stonebridge, Peter A.

    2007-01-01

    Purpose. To retrospectively evaluate the 10 year follow-up results in patients who had 'kissing' self-expanding stent aortic bifurcation reconstruction. Methods. Forty-three patients were treated with 'kissing' self-expanding stents for aortoiliac occlusive disease. Early follow-up with clinical and ankle brachial pressure indices (ABPI) was performed at 3, 6, 12, and 24 months and with intra-arterial digital subtraction angiography at 12-24 months; clinical and angiographic follow-up was performed for symptom recurrence up to 10 years after treatment. Retrospective record review was performed to assess mortality, clinical patency, angiographic patency, and secondary assisted patency of both stents and downstream peripheral vessels at 5 and 10 years follow-up. Results. The 2 year primary angiographic and secondary assisted stent patencies were 89% and 93%, respectively. At 10 years follow-up in 40 patients the mortality was 38% (due to myocardial infarction, stroke, chronic renal failure, malignancy, and liver failure). At 5 and 10 years follow-up the primary clinical stent patency was 82% and 68%, and the secondary assisted stent patency 93% and 86%, respectively. At 5 and 10 years, the distal vessel patency was 86% and 72%, and the secondary assisted distal vessel patency treated by surgical or endovascular techniques was 94% and 88%, respectively. At 10 years there was no limb loss. Conclusion. The long-term (10 year) results of aortic bifurcation arterial self-expanding stent placement in patients with arterial occlusive disease show a 10 year primary stent patency rate of 68% but a secondary assisted patency rate of 86%. In addition there is a high overall mortality due to other cardiovascular causes and the rate of distal disease progression and loss of patency is similar to the loss of stent patency rate

  19. Long-term urethral catheterisation.

    Science.gov (United States)

    Turner, Bruce; Dickens, Nicola

    This article discusses long-term urethral catheterisation, focusing on the relevant anatomy and physiology, indications for the procedure, catheter selection and catheter care. It is important that nurses have a good working knowledge of long-term catheterisation as the need for this intervention will increase with the rise in chronic health conditions and the ageing population.

  20. Wildlife disease ecology from the individual to the population: Insights from a long-term study of a naturally infected European badger population.

    Science.gov (United States)

    McDonald, Jenni L; Robertson, Andrew; Silk, Matthew J

    2018-01-01

    Long-term individual-based datasets on host-pathogen systems are a rare and valuable resource for understanding the infectious disease dynamics in wildlife. A study of European badgers (Meles meles) naturally infected with bovine tuberculosis (bTB) at Woodchester Park in Gloucestershire (UK) has produced a unique dataset, facilitating investigation of a diverse range of epidemiological and ecological questions with implications for disease management. Since the 1970s, this badger population has been monitored with a systematic mark-recapture regime yielding a dataset of >15,000 captures of >3,000 individuals, providing detailed individual life-history, morphometric, genetic, reproductive and disease data. The annual prevalence of bTB in the Woodchester Park badger population exhibits no straightforward relationship with population density, and both the incidence and prevalence of Mycobacterium bovis show marked variation in space. The study has revealed phenotypic traits that are critical for understanding the social structure of badger populations along with mechanisms vital for understanding disease spread at different spatial resolutions. Woodchester-based studies have provided key insights into how host ecology can influence infection at different spatial and temporal scales. Specifically, it has revealed heterogeneity in epidemiological parameters; intrinsic and extrinsic factors affecting population dynamics; provided insights into senescence and individual life histories; and revealed consistent individual variation in foraging patterns, refuge use and social interactions. An improved understanding of ecological and epidemiological processes is imperative for effective disease management. Woodchester Park research has provided information of direct relevance to bTB management, and a better appreciation of the role of individual heterogeneity in disease transmission can contribute further in this regard. The Woodchester Park study system now offers a rare

  1. Telehealth Interventions to Support Self-Management of Long-Term Conditions: A Systematic Metareview of Diabetes, Heart Failure, Asthma, Chronic Obstructive Pulmonary Disease, and Cancer.

    Science.gov (United States)

    Hanlon, Peter; Daines, Luke; Campbell, Christine; McKinstry, Brian; Weller, David; Pinnock, Hilary

    2017-05-17

    Self-management support is one mechanism by which telehealth interventions have been proposed to facilitate management of long-term conditions. The objectives of this metareview were to (1) assess the impact of telehealth interventions to support self-management on disease control and health care utilization, and (2) identify components of telehealth support and their impact on disease control and the process of self-management. Our goal was to synthesise evidence for telehealth-supported self-management of diabetes (types 1 and 2), heart failure, asthma, chronic obstructive pulmonary disease (COPD) and cancer to identify components of effective self-management support. We performed a metareview (a systematic review of systematic reviews) of randomized controlled trials (RCTs) of telehealth interventions to support self-management in 6 exemplar long-term conditions. We searched 7 databases for reviews published from January 2000 to May 2016 and screened identified studies against eligibility criteria. We weighted reviews by quality (revised A Measurement Tool to Assess Systematic Reviews), size, and relevance. We then combined our results in a narrative synthesis and using harvest plots. We included 53 systematic reviews, comprising 232 unique RCTs. Reviews concerned diabetes (type 1: n=6; type 2, n=11; mixed, n=19), heart failure (n=9), asthma (n=8), COPD (n=8), and cancer (n=3). Findings varied between and within disease areas. The highest-weighted reviews showed that blood glucose telemonitoring with feedback and some educational and lifestyle interventions improved glycemic control in type 2, but not type 1, diabetes, and that telemonitoring and telephone interventions reduced mortality and hospital admissions in heart failure, but these findings were not consistent in all reviews. Results for the other conditions were mixed, although no reviews showed evidence of harm. Analysis of the mediating role of self-management, and of components of successful

  2. Long-term treatment of thalidomide ameliorates amyloid-like pathology through inhibition of β-secretase in a mouse model of Alzheimer's disease.

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    Ping He

    Full Text Available Thalidomide is a tumor necrosis factor alpha (TNFα inhibitor which has been found to have abilities against tumor growth, angiogenesis and inflammation. Recently, it has been applied in clinic for the treatment of multiple myeloma as well as some inflammatory diseases. However, whether thalidomide has any therapeutic effects on neurodegenerative disorders, i.e. Alzheimer's disease (AD is not clear. AD is characterized by excessive amount of amyloid β peptides (Aβ, which results in a significant release of inflammatory factors, including TNFα in the brain. Studies have shown that inhibition of TNFα reduces amyloid-associated pathology, prevents neuron loss and improves cognition. Our recent report showed that genetic inhibition of TNFα/TNF receptor signal transduction down-regulates β amyloid cleavage enzyme 1 (BACE1 activity, reduces Aβ generation and improves learning and memory deficits. However, the mechanism of thalidomide involving in the mitigation of AD neuropathological features remains unclear. Here, we chronically administrated thalidomide on human APPswedish mutation transgenic (APP23 mice from 9 months old (an onset of Aβ deposits and early stage of AD-like changes to 12 months old. We found that, in addition of dramatic decrease in the activation of both astrocytes and microglia, thalidomide significantly reduces Aβ load and plaque formation. Furthermore, we found a significant decrease in BACE1 level and activity with long-term thalidomide application. Interestingly, these findings cannot be observed in the brains of 12-month-old APP23 mice with short-term treatment of thalidomide (3 days. These results suggest that chronic thalidomide administration is an alternative approach for AD prevention and therapeutics.

  3. Long-Term Coffee Consumption and Risk of Cardiovascular Disease: A Systematic Review and a Dose-Response Meta-Analysis of Prospective Cohort Studies

    Science.gov (United States)

    Ding, Ming; Bhupathiraju, Shilpa N; Satija, Ambika; van Dam, Rob M; Hu, Frank B

    2013-01-01

    Background Considerable controversy exists regarding the association between coffee consumption and cardiovascular disease (CVD) risk. A meta-analysis was performed to assess the dose-response relationship of long-term coffee consumption with CVD risk. Methods and Results Pubmed and EMBASE were searched for prospective cohort studies of the relationship between coffee consumption and CVD risk, which included coronary heart disease, stroke, heart failure, and CVD mortality. Thirty-six studies were included with 1,279,804 participants and 36,352 CVD cases. A non-linear relationship of coffee consumption with CVD risk was identified (P for heterogeneity = 0.09, P for trend coffee consumption (median: 0 cups/d), the relative risk of CVD was 0.95 (95% CI, 0.87 to 1.03) for the highest (median: 5 cups/d) category, 0.85 (0.80 to 0.90) for the second highest (median: 3.5 cups/d), and 0.89 (0.84 to 0.94) for the third highest category (median: 1.5 cups/d). Looking at separate outcomes, coffee consumption was non-linearly associated with both CHD (P for heterogeneity = 0.001, P for trend 0.05). Conclusions A non-linear association between coffee consumption with CVD risk was observed in this meta-analysis. Moderate coffee consumption was inversely significantly associated with CVD risk, with the lowest CVD risk at 3 to 5 cups/d, and heavy coffee consumption was not associated with elevated CVD risk. PMID:24201300

  4. Long-term follow-up with Granulocyte and Monocyte Apheresis re-treatment in patients with chronically active inflammatory bowel disease

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    Karlsson Mats

    2010-07-01

    Full Text Available Abstract Background Patients with IBD and chronic inflammation refractory to conventional therapy often demonstrate higher risk of serious complications. Combinations of immunosuppression and biological treatment as well as surgical intervention are often used in this patient group. Hence, there is need for additional treatment options. In this observational study, focused on re-treatment and long-term results, Granulocyte/Monocyte Adsorption (GMA, Adacolumn® treatment has been investigated to study efficacy, safety and quality of life in IBD-patients with chronic activity. Methods Fifteen patients with ulcerative colitis and 25 patients with Crohn's disease, both groups with chronically active inflammation refractory to conventional medication were included in this observational study. The patients received 5-10 GMA sessions, and the clinical activity was assessed at baseline, after each completed course, and at week 10 and 20 by disease activity index, endoscopy and quality of life evaluation. Relapsed patients were re-treated by GMA in this follow-up study up to 58 months. Results Clinical response was seen in 85% and complete remission in 65% of the patients. Ten patients in the UC-group (66% and 16 patients in the CD-group (64% maintained clinical and endoscopic remission for an average of 14 months. Fourteen patients who relapsed after showing initial remission were re-treated with GMA and 13 (93% went into a second remission. Following further relapses, all of seven patients were successfully re-treated for the third time, all of three patients for the fourth time and one for a fifth time. Conclusions IBD-patients with chronic inflammation despite conventional therapy seem to benefit from GMA. Re-treatment of relapsing remission patients seems to be effective.

  5. Long-term safety and efficacy of adalimumab for intestinal Behçet's disease in the open label study following a phase 3 clinical trial

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    Nagamu Inoue

    2017-07-01

    Full Text Available Background/Aims: Intestinal Behçet's disease (BD is an immune-mediated inflammatory disorder. We followed up the patients and evaluated safety profile and effectiveness of adalimumab for the treatment of intestinal BD through 100 weeks rolled over from the 52 week clinical trial (NCT01243671.Methods: Patients initiated adalimumab therapy at 160 mg at week 0, followed by 80 mg at week 2, followed by 40 mg every other week until the end of the study. Long-term safety and all adverse events (AEs were examined. The efficacy was assessed on the basis of marked improvement (MI and complete remission (CR using a composite efficacy index, which combined global gastrointestinal symptoms and endoscopic assessments.Results: Twenty patients were enrolled in this study; 15 patients received adalimumab treatment until study completion. The incidence of AEs through week 100 was 544.4 events/100 person-years, which was comparable to the incidence through week 52 (560.4 events/100 person-years. No unexpected trend was observed and adalimumab was well tolerated. At weeks 52 and 100, 60.0% and 40.0% of patients showed MI, respectively, and 20.0% and 15.0% of patients showed CR, respectively.Conclusions: This report demonstrates 2 years safety and effectiveness of adalimumab in intestinal BD patients. Patients with intestinal BD refractory to conventional treatment receiving up to 2 years of adalimumab treatment demonstrated safety outcomes consistent with the known profile of adalimumab, and the treatment led to sustained reduction of clinical and endoscopic disease activity.

  6. Gastric Bypass Surgery Produces a Durable Reduction in Cardiovascular Disease Risk Factors and Reduces the Long-Term Risks of Congestive Heart Failure.

    Science.gov (United States)

    Benotti, Peter N; Wood, G Craig; Carey, David J; Mehra, Vishal C; Mirshahi, Tooraj; Lent, Michelle R; Petrick, Anthony T; Still, Christopher; Gerhard, Glenn S; Hirsch, Annemarie G

    2017-05-23

    Obesity and its association with reduced life expectancy are well established, with cardiovascular disease as one of the major causes of fatality. Metabolic surgery is a powerful intervention for severe obesity, resulting in improvement in comorbid diseases and in cardiovascular risk factors. This study investigates the relationship between metabolic surgery and long-term cardiovascular events. A cohort of Roux-en-Y gastric bypass surgery (RYGB) patients was tightly matched by age, body mass index, sex, Framingham Risk Score, smoking history, use of antihypertension medication, diabetes mellitus status, and calendar year with a concurrent cohort of nonoperated control patients. The primary study end points of major cardiovascular events (myocardial infarction, stroke, and congestive heart failure) were evaluated using Cox regression. Secondary end points of longitudinal cardiovascular risk factors were evaluated using repeated-measures regression. The RYGB and matched controls (N=1724 in each cohort) were followed for up to 12 years after surgery (overall median of 6.3 years). Kaplan-Meier analysis revealed a statistically significant reduction in incident major composite cardiovascular events ( P =0.017) and congestive heart failure (0.0077) for the RYGB cohort. Adjusted Cox regression models confirmed the reductions in severe composite cardiovascular events in the RYGB cohort (hazard ratio=0.58, 95% CI=0.42-0.82). Improvements of cardiovascular risk factors (eg, 10-year cardiovascular risk score, total cholesterol, high-density lipoprotein, systolic blood pressure, and diabetes mellitus) were observed within the RYGB cohort after surgery. Gastric bypass is associated with a reduced risk of major cardiovascular events and the development of congestive heart failure. © 2017 The Authors and Geisinger Clinic. Published on behalf of the American Heart Association, Inc., by Wiley.

  7. Influence of more extensive radiotherapy and adjuvant chemotherapy on long-term outcome of early-stage Hodgkin's disease: a meta-analysis of 23 randomized trials involving 3,888 patients. International Hodgkin's Disease Collaborative Group

    DEFF Research Database (Denmark)

    Specht, L.; Gray, R.G.; Clarke, M.J.

    1998-01-01

    PURPOSE: To assess the effect of more extensive radiotherapy and of adjuvant combination chemotherapy on long-term outcome of early-stage Hodgkin's disease. METHODS: In a collaborative worldwide systematic overview, individual patient data were centrally reviewed on 1,974 patients in eight.......7%; P counterbalanced by a nonsignificant excess of deaths from other...... causes (12.4% v 10.0% 10-year risk). CONCLUSION: More extensive radiotherapy fields or the addition of chemotherapy to radiotherapy in the initial treatment of early-stage Hodgkin's disease had a large effect on disease control, but only a small effect on overall survival. Recurrences could be prevented...

  8. Long term disease-free survival and T cell and antibody responses in women with high-risk Her2+ breast cancer following vaccination against Her2

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    Anders Carey

    2007-09-01

    Full Text Available Abstract Background The HER2-inhibiting antibody trastuzumab, in combination with chemotherapy, significantly improves survival of women with resected, HER2-overexpressing breast cancers, but is associated with toxicities including a risk of cardiomyopathy. Additionally, the beneficial effect of trastuzumab is expected to decrease once the drug is discontinued. We proposed to address these concerns by using cancer vaccines to stimulate HER2 intracellular domain (ICD-specific T cell and antibody responses. Methods Subjects with stage II (≥ 6 +LN, III, or stage IV breast cancerwith > 50% HER2 overexpressing tumor cells who were disease-free after surgery and adjuvant therapy were eligible. Vaccines consisted of immature, cultured DC (n = 3, mature cultured DC (n = 3, or mature Flt3-ligand mobilized peripheral blood DC (n = 1 loaded with ICD, or tetanus toxoid, keyhole limpet hemocyanin or CMV peptide as controls, and were administered intradermally/subcutaneously four times at 3 week intervals. ICD-specific T cell and antibody responses were measured. Cardiac function was determined by MUGA or ECHO; long term disease status was obtained from patient contact. Results All seven patients successfully underwent DC generation and five received all 4 immunizations. There were no toxicities greater than grade 1 or ejection fraction decrements below normal. Delayed-type hypersensitivity (DTH reactions at the injection site occurred in 6/7 patients and HER2 specificity was detected by cytokine flow cytometry or ELISPOT in 5 patients. At more than 5 years of follow-up, 6/7 had detectable anti-ICD antibodies. One patient experienced a pulmonary recurrence at 4 years from their study immunizations. This recurrence was resected and they are without evidence of disease. All patients are alive and disease-free at 4.6–6.7 years of follow-up. Conclusion Although this was a small pilot study, the well-tolerated nature of the vaccines, the lack of cardiac

  9. The long-term risk of smoking in diabetic patients with stable ischemic heart disease treated with intensive medical therapy and lifestyle modification.

    Science.gov (United States)

    Khan, Asrar A; Chung, Matthew J; Novak, Eric; Mori Brooks, Maria; Brown, David L

    2017-09-01

    Introduction The long-term risk of smoking in diabetic patients with stable ischemic heart disease (SIHD) is unknown. We sought to analyze the impact of smoking on outcomes of diabetic patients with SIHD when other cardiovascular risk factors are being aggressively treated. Methods The Bypass Angioplasty Revascularization Investigation 2 Diabetes (BARI 2D) trial randomized 2368 diabetics with SIHD to intensive medical therapy (IMT) with prompt revascularization or IMT alone. Smoking status was obtained at baseline, 6 months, and 1, 2, 3, 4 and 5 years. The primary endpoint of interest was all-cause mortality. Results Of 2360 patients, 33.1% of patients never smoked, 54.4% were former smokers, and 12.5% were current smokers. The rate of all-cause mortality was greater for current (2.5 deaths/100 patient-years) and former smokers (3.1 deaths/100 patient-years) than never smokers (2.1 deaths/100 patient-years) (P = 0.007). Cardiac death, cardiovascular death, fatal or nonfatal myocardial infarction, and fatal or nonfatal stroke were not increased in current or former smokers compared with never smokers. Compared with never smokers, current smokers experienced a 49% increased hazard of death (Hazard Ratio (HR) 1.49, 95% Confidence Interval (CI): 0.97-2.29, P = 0.07) whereas former smokers had a 37% increased hazard of death (HR 1.37, 95% CI: 1.04-2.79, P = 0.02) when considering smoking status as a time-dependent variable and adjusting for factors that differed by smoking status. Conclusions Current and former smoking are associated with increased all-cause mortality in diabetics with SIHD but not with increased cardiovascular morbidity or mortality.

  10. Long-Term Ursodeoxycholic Acid Therapy Does Not Alter Lithocholic Acid Levels in Patients with Cystic Fibrosis with Associated Liver Disease.

    Science.gov (United States)

    Colombo, Carla; Crosignani, Andrea; Alicandro, Gianfranco; Zhang, Wujuan; Biffi, Arianna; Motta, Valentina; Corti, Fabiola; Setchell, Kenneth D R

    2016-10-01

    To evaluate the fasting and postprandial serum bile acid composition in patients with cystic fibrosis-associated liver disease (CFLD) after chronic administration of ursodeoxycholic acid (UDCA) (20 mg/kg/day). The aim was to specifically focus on the extent of biotransformation of UDCA to its hepatotoxic metabolite, lithocholic acid, because of recent concerns regarding the safety of long-term, high-dose UDCA treatment for CFLD. Twenty patients with CFLD (median age 16 years, range: 2.4-35.0) prescribed UDCA therapy for at least 2 years were studied. Total and individual serum bile acids were measured by stable-isotope dilution mass spectrometry, in fasting and 2-hour postprandial samples taken during chronic UDCA (20 mg/kg/day) administration. During chronic UDCA administration (median duration 8 years, IQR: 6-16), UDCA became the predominant serum bile acid in all patients (median, IQR: 3.17, 1.25-5.56 μmol/L) and chenodeoxycholic acid concentrations were greater than cholic acid (1.86, 1.00-4.70 μmol/L vs 0.40, 0.24-2.71 μmol/L). The secondary bile acids, deoxycholate and lithocholate, were present in very low concentrations in fasted serum (acid significantly increased (P acid concentrations were observed. These data do not support recent suggestions that enhanced biotransformation of UDCA to the hepatotoxic secondary bile acid lithocholic occurs when patients with CFLD are treated with relatively high doses of UDCA. Copyright © 2016 Elsevier Inc. All rights reserved.

  11. Long Term Effects on Risk Factors for Cardiovascular Disease after 12-Months of Aerobic Exercise Intervention - A Worksite RCT among Cleaners.

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    Mette Korshøj

    Full Text Available Occupational groups exposed to high occupational physical activity have an increased risk for cardiovascular disease (CVD. This may be explained by the high relative aerobic workload. Enhanced cardiorespiratory fitness reduces the relative aerobic workload. Thus, the aim was to evaluate the 12-months effects of worksite aerobic exercise on risk factors for CVD among cleaners.One hundred and sixteen cleaners aged 18-65 years were randomized to a group performing aerobic exercise and a reference group receiving lectures. Outcomes were collected at baseline and after 12-months. A repeated measures 2×2 multi-adjusted mixed-model design was applied to compare the between-group differences using intention-to-treat analysis.Between-group differences (p<0.05 were found favouring the aerobic exercise group: cardiorespiratory fitness 2.15 (SE 1.03 mlO2/min/kg, aerobic workload -2.15 (SE 1.06 %HRR, resting HR -5.31 (SE 1.61 beats/min, high sensitive C-reactive protein -0.65 (SE 0.24 μg/ml. The blood pressure was unaltered. Stratified analyses on relative aerobic workload at baseline revealed that those with relative aerobic workloads ≥30% of HRR seems to impose a notable adverse effect on resting and ambulatory blood pressure.This long-term worksite aerobic exercise intervention among cleaners led to several beneficial effects, but also potential adverse effects among those with high relative aerobic workloads.Controlled-Trials.com ISRCTN86682076.

  12. A prospective study of long-term results and the red blood cell immune adherence function following 131I treatment of Graves' disease

    International Nuclear Information System (INIS)

    Sun Zengjun; Ma Yuqin; Liu Junqing; Zhang Xia

    2003-01-01

    Objective: To observe the long-term curative effect and red blood cell immune function in patients with Graves' Disease (GD) after radioactive iodine ( 131 I) treatment. Methods: To observe the hyperthyroid symptoms, physical signs and to measure the concentration of FT3, FT4, FSH by radioimmunoassay, the percent of RBC-C 3b R-YR(%), RBC-IC-YR(%). Results: Patients with GD treated by 131 I after three month, these hyperthyroid symptoms, Physical signs are alleviated and even vanished in patients who were treated by 131 I after 1.5 years, RBC-C 3b R-YR(%) are normal in all patients, but RBC-IC-YR(%) are higher than before treatment with 131 I, then become lower after three months, treatment by 131 I but they were higher than those in normal group, when treated with 131 I after 1.5 years, RBC-IC-YR(%) are normal, the relationship between RBC-C 3b R-YR(%) and RBC-IC-YR(%) are positively interacted. Conclusion: The 131 I treatment produces a good rapid curative effect for GD patients, the disorder state of the red cell immune adherence system is rapidly corrected and this effect is unidirectional. So RBC-C 3b R-YR(%), RBC-IC-YR(%) are the useful predictors of GD remission and their relationship, RBC-IC-YR(%) are helpful in explaining the mechanism of radioactive iodine therapy and are the predictors of GD remission after 131 I treatment

  13. Short-term to Long-term Results of Ahmed Glaucoma Valve Implantation for Uveitic Glaucoma Secondary to Behçet Disease.

    Science.gov (United States)

    Yakin, Mehmet; Eksioglu, Umit; Sungur, Gulten; Satana, Banu; Demirok, Gulizar; Ornek, Firdevs

    2017-01-01

    To evaluate short-term to long-term outcomes of Ahmed glaucoma valve (AGV) implantation in the management of uveitic glaucoma (UG) secondary to Behçet disease (BD). A retrospective chart review of 47 eyes of 35 patients with UG secondary to BD who underwent AGV implantation was conducted. Success was defined as having an intraocular pressure (IOP) between 6 and 21 mm Hg with (qualified success) or without (complete success) antiglaucomatous medications and without need for further glaucoma surgery. Mean postoperative follow-up was 57.72±26.13 months. Mean preoperative IOP was 35.40±8.33 mm Hg versus 12.28±2.90 mm Hg at the last follow-up visit (P<0.001). Mean number of preoperative topical antiglaucomatous medications was 2.96±0.29 versus 0.68±1.12 at the last follow-up visit (P<0.001). In all eyes, IOP could be maintained between 6 and 21 mm Hg with or without antiglaucomatous medications during follow-up. The cumulative probability of complete success was 46.8% at 6 months, 40.4% at 12 months, and 35.9% at 36 months, and the cumulative probability of eyes without complication was 53.2% at 6 months, 46.5% at 12 months, and 39.6% at 24 months postoperatively based on Kaplan-Meier survival analysis. No persistent or irreparable complications were observed. This study includes one of the largest series of AGV implantation in the management of UG with the longest follow-up reported. AGV implantation can be considered as a primary surgical option in the management of UG secondary to BD with 100% total success rate (with or without medications).

  14. Plasma stromal cell-derived factor 1α/CXCL12 level predicts long-term adverse cardiovascular outcomes in patients with coronary artery disease.

    Science.gov (United States)

    Ghasemzadeh, Nima; Hritani, Abdul Wahab; De Staercke, Christine; Eapen, Danny J; Veledar, Emir; Al Kassem, Hatem; Khayata, Mohamed; Zafari, A Maziar; Sperling, Laurence; Hooper, Craig; Vaccarino, Viola; Mavromatis, Kreton; Quyyumi, Arshed A

    2015-01-01

    Stromal derived factor-1α/CXCL12 is a chemoattractant responsible for homing of progenitor cells to ischemic tissues. We aimed to investigate the association of plasma CXCL12 with long-term cardiovascular outcomes in patients with coronary artery disease (CAD). 785 patients aged: 63 ± 12 undergoing coronary angiography were independently enrolled into discovery (N = 186) and replication (N = 599) cohorts. Baseline levels of plasma CXCL12 were measured using Quantikine CXCL12 ELISA assay (R&D systems). Patients were followed for cardiovascular death and/or myocardial infarction (MI) for a mean of 2.6 yrs. Cox proportional hazard was used to determine independent predictors of cardiovascular death/MI. The incidence of cardiovascular death/MI was 13% (N = 99). High CXCL12 level based on best discriminatory threshold derived from the ROC analysis predicted risk of cardiovascular death/MI (HR = 4.81, p = 1 × 10(-6)) independent of traditional risk factors in the pooled cohort. Addition of CXCL12 to a baseline model was associated with a significant improvement in c-statistic (AUC: 0.67-0.73, p = 0.03). Addition of CXCL12 was associated with correct risk reclassification of 40% of events and 10.5% of non-events. Similarly for the outcome of cardiovascular death, the addition of the CXCL12 to the baseline model was associated with correct reclassification of 20.7% of events and 9% of non-events. These results were replicated in two independent cohorts. Plasma CXCL12 level is a strong independent predictor of adverse cardiovascular outcomes in patients with CAD and improves risk reclassification. Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  15. A Meta-Analysis of Randomized Controlled Trials to Compare Long-Term Outcomes of Nissen and Toupet Fundoplication for Gastroesophageal Reflux Disease.

    Directory of Open Access Journals (Sweden)

    Zhi-chao Tian

    Full Text Available In recent years, several studies with large sample sizes and recent follow-up data have been published comparing outcomes between laparoscopic Nissen fundoplication and laparoscopic Toupet fundoplication. It is now timely to be re-evaluated and synthesized long-term efficacy and adverse events of both total and partial posterior fundoplication.Electronic searches for RCTs comparing the outcome after laparoscopic Nissen fundoplication and laparoscopic Toupet fundoplication were performed in the databases of MEDLINE, EMBASE, and the Cochrane Center Register of Controlled Trials. The data of evaluation in positive and adverse results of laparoscopic Nissen fundoplication and laparoscopic Nissen fundoplication were extracted and compared using meta-analysis.13 RCTs were ultimately identified involving 814 (52.05% and 750 (47.95% patients who underwent laparoscopic Nissen fundoplication and laparoscopic Toupet fundoplication, respectively. The operative time, perioperative complications, postoperative satisfaction, recurrence, and the rates of medication adoption or re-operation due to recurrence were not significantly different between two groups. The two types of fundoplication both reinforced the anti-reflux barrier and elevated the lower esophageal sphincter pressure. However, rates of adverse results involving dysphasia, gas-bloat syndrome, inability to belch and re-operation due to severe dysphasia were significantly higher after LNF. In the subgroup analysis of wrap length≤2 cm, laparoscopic Nissen fundoplication was associated with a significantly higher incidence of postoperative dysphagia. However, in the subgroup wrap length>2 cm, the difference was not statistically significant.Laparoscopic Toupet fundoplication might be the better surgery approach for gastroesophageal reflux disease with a lower rate of postoperative adverse results and equal effectiveness as Laparoscopic Nissen fundoplication.

  16. Long-term exposure to ambient air pollution and respiratory disease mortality in Shenyang, China: a 12-year population-based retrospective cohort study.

    Science.gov (United States)

    Dong, Guang-Hui; Zhang, Pengfei; Sun, Baijun; Zhang, Liwen; Chen, Xi; Ma, Nannan; Yu, Fei; Guo, Huimin; Huang, Hui; Lee, Yungling Leo; Tang, Naijun; Chen, Jie

    2012-01-01

    In China, both the levels and patterns of outdoor air pollution have altered dramatically with the rapid economic development and urbanization over the past two decades. However, few studies have investigated the association of outdoor air pollution with respiratory mortality, especially in the high pollution range. We conducted a retrospective cohort study of 9,941 residents aged ≥35 years old in Shenyang, China, to examine the association between outdoor air pollutants [particulate matter mortality using 12 years of data. We applied extended Cox proportional hazards modeling with time-dependent covariates to respiratory mortality. Analyses were also stratified by age, sex, educational level, smoking status, personal income, occupational exposure and body mass index (BMI) to examine the association of air pollution with mortality. We found significant associations between PM(10) and NO(2) levels and respiratory disease mortality. Our analysis found a relative risk of 1.67 [95% confidence interval (CI) 1.60-1.74] and 2.97 (95% CI 2.69-3.27) for respiratory mortality per 10 µg/m(3) increase in PM(10) and NO(2), respectively. The effects of air pollution were more apparent in women than in men. Age, sex, educational level, smoking status, personal income, occupational exposure, BMI and exercise frequency influenced the relationship between outdoor PM(10) and NO(2) and mortality. For SO(2), only smoking, little regular exercise and BMI above 18.5 influenced the relationship with mortality. These data contribute to the scientific literature on the long-term effects of air pollution for the high-exposure settings typical in developing countries. Copyright © 2011 S. Karger AG, Basel.

  17. A trade-off between natural and acquired antibody production in a reptile: implications for long-term resistance to disease

    Directory of Open Access Journals (Sweden)

    Franziska C. Sandmeier

    2012-08-01

    Vertebrate immune systems are understood to be complex and dynamic, with trade-offs among different physiological components (e.g., innate and adaptive immunity within individuals and among taxonomic lineages. Desert tortoises (Gopherus agassizii immunised with ovalbumin (OVA showed a clear trade-off between levels of natural antibodies (NAbs; innate immune function and the production of acquired antibodies (adaptive immune function. Once initiated, acquired antibody responses included a long-term elevation in antibodies persisting for more than one year. The occurrence of either (a high levels of NAbs or (b long-term elevations of acquired antibodies in individual tortoises suggests that long-term humoral resistance to pathogens may be especially important in this species, as well as in other vertebrates with slow metabolic rates, concomitantly slow primary adaptive immune responses, and long life-spans.

  18. Long term complications of diabetes

    Science.gov (United States)

    ... medlineplus.gov/ency/patientinstructions/000327.htm Long-term complications of diabetes To use the sharing features on this page, ... other tests. All these may help you keep complications of diabetes away. You will need to check your blood ...

  19. Long-term air humidification therapy is cost-effective for patients with moderate or severe chronic obstructive pulmonary disease or bronchiectasis.

    Science.gov (United States)

    Milne, Richard J; Hockey, Hans; Rea, Harry

    2014-06-01

    To establish the cost-effectiveness of long-term humidification therapy (LTHT) added to usual care for patients with moderate or severe chronic obstructive pulmonary disease or bronchiectasis. Resource usage in a 12-month clinical trial of LTHT was estimated from hospital records, patient diaries, and the equipment supplier. Health state utility values were derived from the St. Georges Respiratory Questionnaire (SGRQ) total score. All patients who remained in the trial for 12 months and who had at least 90 days of diary records were included (87 of 108). Clinical costs were NZ $3973 (95% confidence interval [CI] $1614-$6332) for the control group and NZ $3331 (95% CI $948-$6920) for the intervention group. The mean health benefit per patient was -6.9 SGRQ units (95% CI -13.0 to -7.2; P < 0.05) or +0.0678 quality-adjusted life-years (95% CI 0.001-0.135). With the intervention costing NZ $2059 annually, the mean cost per quality-adjusted life-year was NZ $20,902 (US $18,907) and the bootstrap median was NZ $19,749 (2.5th percentile -$40,923, 97.5th percentile $221,275). At a willingness-to-pay (WTP) threshold of NZ $30,000, the probability of cost-effectiveness was 61%, ranging from 49% to 72% as the cost of LTHT was varied by ±30%. At a WTP of NZ $20,000, the probability was 49% (range 34%-61%). LTHT is moderately cost-effective for patients with moderate to severe chronic obstructive pulmonary disease or bronchiectasis at a WTP threshold that is acceptable for public funding of medicines in New Zealand. These findings must be interpreted with caution because of the modest size of the clinical study, necessary lack of blinding in the clinical trial, and uncertainty in estimating health state utility from the SQRQ. Copyright © 2014 International Society for Pharmacoeconomics and Outcomes Research (ISPOR). Published by Elsevier Inc. All rights reserved.

  20. Predicting Long-term Ischemic Events Using Routine Clinical Parameters in Patients with Coronary Artery Disease: The OPT-CAD Risk Score.

    Science.gov (United States)

    Han, Yaling; Chen, Jiyan; Qiu, Miaohan; Li, Yi; Li, Jing; Feng, Yingqing; Qiu, Jian; Meng, Liang; Sun, Yihong; Tao, Guizhou; Wu, Zhaohui; Yang, Chunyu; Guo, Jincheng; Pu, Kui; Chen, Shaoliang; Wang, Xiaozeng

    2018-06-05

    The prognosis of patients with coronary artery disease (CAD) at hospital discharge was constantly varying, and post-discharge risk of ischemic events remain a concern. However, risk prediction tools to identify risk of ischemia for these patients has not yet been reported. We sought to develop a scoring system for predicting long-term ischemic events in CAD patients receiving antiplatelet therapy that would be beneficial in appropriate personalized decision-making for these patients. In this prospective Optimal antiPlatelet Therapy for Chinese patients with Coronary Artery Disease (OPT-CAD, NCT01735305) registry, a total of 14,032 patients with CAD receiving at least one kind of antiplatelet agent were enrolled from 107 centers across China, from January 2012 to March 2014. The risk scoring system was developed in a derivation cohort (enrolled initially 10,000 patients in the database) using a logistic regression model and was subsequently tested in a validation cohort (the last 4,032 patients). Points in risk score was assigned based on the multivariable odds ratio of each factor. Ischemic events were defined as the composite of cardiac death, myocardial infarction or stroke. Ischemic events occurred in 342 (3.4%) patients in the derivation cohort and 160 (4.0%) patients in the validation cohort during 1-year follow-up. The OPT-CAD score, ranging from 0-257 points, consist of 10 independent risk factors, including age (0-71 points), heart rates (0-36 points), hypertension (0-20 points), prior myocardial infarction (16 points), prior stroke (16 points), renal insufficient (21 points), anemia (19 points), low ejection fraction (22 points), positive cardiac troponin (23 points) and ST-segment deviation (13 points). In predicting 1-year ischemic events, the area under receiver operating characteristics curve were 0.73 and 0.72 in derivation and validation cohort, respectively. The incidences of ischemic events in low- (0-90 points), medium- (91-150 points) and

  1. Long-term outcomes after disease activity-guided dose reduction of TNF inhibition in rheumatoid arthritis: 3-year data of the DRESS study - a randomised controlled pragmatic non-inferiority strategy trial

    NARCIS (Netherlands)

    Bouman, Chantal Am; van Herwaarden, Noortje; van den Hoogen, Frank Hj; Fransen, Jaap; van Vollenhoven, Ronald F.; Bijlsma, Johannes Wj; Maas, Aatke van der; den Broeder, Alfons A.

    2017-01-01

    Tumour necrosis factor inhibitors (TNFi) are effective in rheumatoid arthritis (RA), but disadvantages include adverse events (AEs) and high costs. This can be improved by disease activity-guided dose reduction (DR). We aimed to assess long-term outcomes of TNFi DR in RA by using 3-year data from

  2. Long-term outcomes after disease activity-guided dose reduction of TNF inhibition in rheumatoid arthritis: 3-year data of the DRESS study - a randomised controlled pragmatic non-inferiority strategy trial

    NARCIS (Netherlands)

    Bouman, C.A.M.; Herwaarden, N. van; Hoogen, F.H.J. van den; Fransen, J.; Vollenhoven, R.F. van; Bijlsma, J.W.; Maas, A.V.; Broeder, A.A. den

    2017-01-01

    OBJECTIVE: Tumour necrosis factor inhibitors (TNFi) are effective in rheumatoid arthritis (RA), but disadvantages include adverse events (AEs) and high costs. This can be improved by disease activity-guided dose reduction (DR). We aimed to assess long-term outcomes of TNFi DR in RA by using 3-year

  3. Report from the Rockefellar Foundation Sponsored International Workshop on reducing mortality and improving quality of life in long-term survivors of Hodgkin's disease: July 9-16, 2003, Bellagio, Italy

    DEFF Research Database (Denmark)

    Mauch, Peter; Ng, Andrea; Aleman, Berthe

    2005-01-01

    A workshop, sponsored by the Rockefellar Foundation, was held between 9 to 16 July, 2003 to devise strategies to reduce mortality and improve quality of life of long-term survivors of Hodgkin's disease. Participants were selected for their clinical and research background on late effects after Ho...

  4. Juvenile idiopathic arthritis in adulthood: fulfilment of classification criteria for adult rheumatic diseases, long-term outcomes and predictors of inactive disease, functional status and damage.

    Science.gov (United States)

    Oliveira-Ramos, Filipa; Eusébio, Mónica; M Martins, Fernando; Mourão, Ana Filipa; Furtado, Carolina; Campanilho-Marques, Raquel; Cordeiro, Inês; Ferreira, Joana; Cerqueira, Marcos; Figueira, Ricardo; Brito, Iva; Canhão, Helena; Santos, Maria José; Melo-Gomes, José A; Fonseca, João Eurico

    2016-01-01

    To determine how adult juvenile idiopathic arthritis (JIA) patients fulfil classification criteria for adult rheumatic diseases, evaluate their outcomes and determine clinical predictors of inactive disease, functional status and damage. Patients with JIA registered on the Rheumatic Diseases Portuguese Register (Reuma.pt) older than 18 years and with more than 5 years of disease duration were included. Data regarding sociodemographic features, fulfilment of adult classification criteria, Health Assessment Questionnaire, Juvenile Arthritis Damage Index-articular (JADI-A) and Juvenile Arthritis Damage Index-extra-articular (JADI-E) damage index and disease activity were analysed. 426 patients were included. Most of patients with systemic JIA fulfilled criteria for Adult Still's disease. 95.6% of the patients with rheumatoid factor (RF)-positive polyarthritis and 57.1% of the patients with RF-negative polyarthritis matched criteria for rheumatoid arthritis (RA). 38.9% of the patients with extended oligoarthritis were classified as RA while 34.8% of the patients with persistent oligoarthritis were classified as spondyloarthritis. Patients with enthesitis-related arthritis fulfilled criteria for spondyloarthritis in 94.7%. Patients with psoriatic arthritis maintained this classification. Patients with inactive disease had lower disease duration, lower diagnosis delay and corticosteroids exposure. Longer disease duration was associated with higher HAQ, JADI-A and JADI-E. Higher JADI-A was also associated with biological treatment and retirement due to JIA disability and higher JADI-E with corticosteroids exposure. Younger age at disease onset was predictive of higher HAQ, JADI-A and JADI-E and decreased the chance of inactive disease. Most of the included patients fulfilled classification criteria for adult rheumatic diseases, maintain active disease and have functional impairment. Younger age at disease onset was predictive of higher disability and decreased the

  5. QUALITY-OF-LIFE IN A LONG-TERM MULTICENTER TRIAL IN CHRONIC NONSPECIFIC LUNG-DISEASE - ASSESSMENT AT BASE-LINE

    NARCIS (Netherlands)

    KAPTEIN, AA; BRAND, PLP; DEKKER, FW; KERSTJENS, HAM; POSTMA, DS; SLUITER, HJ

    1993-01-01

    Quality-of-life (QOL) in patients with respiratory illness is a topic of increasing interest to clinicians and researchers. In a multicentre trial, which studies the long-term effects of three medication regimens (beta-agonist plus either placebo, anti-cholinergic agent or corticosteroid, all by

  6. Health-related quality of life and disease specific symptoms in long-term thyroid cancer survivors : A study from the population-based PROFILES registry

    NARCIS (Netherlands)

    Husson, O.; Haak, H.R.; Buffart, L.M.; Nieuwlaat, W.-A.; Oranje, W.A.; Mols, F.; Kuijpens, J.L.; Coebergh, J.W.W.; van de Poll-Franse, L.V.

    2013-01-01

    Background. Given the longevity of thyroid cancer patients, any impairment in health-related quality of life (HRQoL) during the follow-up period is of considerable concern. Therefore, the first aim of this study was to assess (thyroid cancer specific) HRQoL among long-term thyroid cancer survivors

  7. Quality of Survivorship in a Rare Disease: Clinicofunctional Outcome and Physical Activity in an Observational Cohort Study of 618 Long-Term Survivors of Ewing Sarcoma

    NARCIS (Netherlands)

    Ranft, Andreas; Seidel, Corinna; Hoffmann, Christiane; Paulussen, Michael; Warby, Ann-Christin; van den Berg, Henk; Ladenstein, Ruth; Rossig, Claudia; Dirksen, Uta; Rosenbaum, Dieter; Juergens, Herbert

    2017-01-01

    PurposeSignificantly improved survival rates in patients with Ewing sarcoma have raised interest in accessing the quality of long-term survivorship. In this study, subjective and objective measurement tools, preclassified as physical or mental scores, were used to assess clinicofunctional outcome

  8. [Long-term psychiatric hospitalizations].

    Science.gov (United States)

    Plancke, L; Amariei, A

    2017-02-01

    Long-term hospitalizations in psychiatry raise the question of desocialisation of the patients and the inherent costs. Individual indicators were extracted from a medical administrative database containing full-time psychiatric hospitalizations for the period 2011-2013 of people over 16 years old living in the French region of Nord-Pas-de-Calais. We calculated the proportion of people who had experienced a hospitalization with a duration of 292 days or more during the study period. A bivariate analysis was conducted, then ecological data (level of health-care offer, the deprivation index and the size of the municipalities of residence) were included into a multilevel regression model in order to identify the factors significantly related to variability of long-term hospitalization rates. Among hospitalized individuals in psychiatry, 2.6% had had at least one hospitalization of 292 days or more during the observation period; the number of days in long-term hospitalization represented 22.5% of the total of days of full-time hospitalization in psychiatry. The bivariate analysis revealed that seniority in the psychiatric system was strongly correlated with long hospitalization rates. In the multivariate analysis, the individual indicators the most related to an increased risk of long-term hospitalization were: total lack of autonomy (OR=9.0; 95% CI: 6.7-12.2; P<001); diagnoses of psychological development disorders (OR=9.7; CI95%: 4.5-20.6; P<.001); mental retardation (OR=4.5; CI95%: 2.5-8.2; P<.001): schizophrenia (OR=3.0; CI95%: 1.7-5.2; P<.001); compulsory hospitalization (OR=1.7; CI95%: 1.4-2.1; P<.001); having experienced therapeutic isolation (OR=1.8; CI95%: 1.5-2.1; P<.001). Variations of long-term hospitalization rates depending on the type of establishment were very high, but the density of hospital beds or intensity of ambulatory activity services were not significantly linked to long-term hospitalization. The inhabitants of small urban units had

  9. Age at diagnosis of inflammatory bowel disease influences early development of colorectal cancer in inflammatory bowel disease patients: A nationwide, long-term survey

    NARCIS (Netherlands)

    J.E. Baars (Judith); E.J. Kuipers (Ernst); M. van Haastert (M.); J.J. Nicolai (Jan); A.C. Poen (Alexander); C.J. van der Woude (Janneke)

    2012-01-01

    textabstractBackground: Data on clinical characteristics of patients with inflammatory bowel disease (IBD)-related colorectal cancer (CRC) are scarce and mainly originate from tertiary referral centres. We studied patient and disease characteristics of IBD-related CRC in a nationwide IBD cohort in

  10. Improving outcomes of patients with Alzheimer's disease : long-term disease course and nutritional status in a 'real-life' setting

    NARCIS (Netherlands)

    Droogsma, Hinderika

    2015-01-01

    Alzheimer’s Disease (AD) is the most frequent cause of dementia and characterized by cognitive impairment (such as memory impairment), functional decline and neuropsychiatric symptoms (such as anxiety and depression). Since Alzheimer’s disease is one of the most disabling and burdensome health

  11. Impact of β-blocker selectivity on long-term outcomes in congestive heart failure patients with chronic obstructive pulmonary disease

    Directory of Open Access Journals (Sweden)

    Kubota Y

    2015-03-01

    Full Text Available Yoshiaki Kubota, Kuniya Asai, Erito Furuse, Shunichi Nakamura, Koji Murai, Yayoi Tetsuou Tsukada, Wataru Shimizu Department of Medicine (Division of Cardiology, Nippon Medical School, Bunkyo-ku, Tokyo, Japan Background: Chronic obstructive pulmonary disease (COPD is present in approximately one-third of all congestive heart failure (CHF patients, and is a key cause of underprescription and underdosing of β-blockers, largely owing to concerns about precipitating respiratory deterioration. For these reasons, the aim of this study was to evaluate the impact of β-blockers on the long-term outcomes in CHF patients with COPD. In addition, we compared the effects of two different β-blockers, carvedilol and bisoprolol. Methods: The study was a retrospective, non-randomized, single center trial. Acute decompensated HF patients with COPD were classified according to the oral drug used at discharge into β-blocker (n=86; carvedilol [n=52] or bisoprolol [n=34] and non-β-blocker groups (n=46. The primary endpoint was all-cause mortality between the β-blocker and non-β-blocker groups during a mean clinical follow-up of 33.9 months. The secondary endpoints were the differences in all-cause mortality and the hospitalization rates for CHF and/or COPD exacerbation between patients receiving carvedilol and bisoprolol. Results: The mortality rate was higher in patients without β-blockers compared with those taking β-blockers (log-rank P=0.039, and univariate analyses revealed that the use of β-blockers was the only factor significantly correlated with the mortality rate (hazard ratio: 0.41; 95% confidence interval: 0.17–0.99; P=0.047. Moreover, the rate of CHF and/or COPD exacerbation was higher in patients treated with carvedilol compared with bisoprolol (log-rank P=0.033. In the multivariate analysis, only a past history of COPD exacerbation significantly increased the risk of re-hospitalization due to CHF and/or COPD exacerbation (adjusted hazard

  12. Fus1 KO mouse as a model of oxidative stress-mediated sporadic Alzheimer’s disease: circadian disruption and long-term spatial and olfactory memory impairments.

    Directory of Open Access Journals (Sweden)

    Guillermo Coronas-Samano

    2016-11-01

    Full Text Available Insufficient advances in the development of effective therapeutic treatments of sporadic Alzheimer's Disease (sAD to date are largely due to the lack of sAD-relevant animal models. While the vast majority of models do recapitulate AD's hallmarks of plaques and tangles by virtue of tau and/or beta amyloid overexpression, these models do not reflect the fact that in sAD (unlike familial AD these genes are not risk factors per se and that other mechanisms like oxidative stress, metabolic dysregulation and inflammation play key roles in AD etiology. Here we characterize and propose the Fus1 KO mice that lack a mitochondrial protein Fus1/Tusc2 as a new sAD model. To establish sAD relevance, we assessed sAD related deficits in Fus1 KO and WT adult mice of 4-5 months old, the equivalent human age when the earliest cognitive and olfactory sAD symptoms arise. Fus1 KO mice showed oxidative stress (increased levels of ROS, decreased levels of PRDX1, disruption of metabolic homeostasis (decreased levels of ACC2, increased phosphorylation of AMPK, autophagy (decreased levels of LC3-II, PKC (decreased levels of RACK1 and calcium signaling (decreased levels of Calb2 in the olfactory bulb and/or hippocampus. Mice were behaviorally tested using objective and accurate video tracking (Noldus, in which Fus1 KO mice showed clear deficits in olfactory memory (decreased habituation/cross-habituation in the short and long term, olfactory guided navigation memory (inability to reduce their latency to find the hidden cookie, spatial memory (learning impairments on finding the platform in the Morris water maze and showed more sleep time during the diurnal cycle. Fus1 KO mice did not show clear deficits in olfactory perception (cross-habituation, association memory (passive avoidance or in species-typical behavior (nest building and no increased anxiety (open field, light-dark box or depression/anhedonia (sucrose preference at this relatively young age. These

  13. Short- and long-term risks of cardiovascular disease following radiotherapy in rectal cancer in four randomized controlled trials and a population-based register.

    Science.gov (United States)

    Chen, Lingjing; Eloranta, Sandra; Martling, Anna; Glimelius, Ingrid; Neovius, Martin; Glimelius, Bengt; Smedby, Karin E

    2018-03-01

    A population-based cohort and four randomized trials enriched with long-term register data were used to clarify if radiotherapy in combination with rectal cancer surgery is associated with increased risks of cardiovascular disease (CVD). We identified 14,901 rectal cancer patients diagnosed 1995-2009 in Swedish nationwide registers, of whom 9227 were treated with preoperative radiotherapy. Also, we investigated 2675 patients with rectal cancer previously randomized to preoperative radiotherapy or not followed by surgery in trials conducted 1980-1999. Risks of CVD overall and subtypes were estimated based on prospectively recorded hospital visits during relapse-free follow-up using multivariable Cox regression. Maximum follow-up was 18 and 33 years in the register and trials, respectively. We found no association between preoperative radiotherapy and overall CVD risk in the register (Incidence Rate Ratio, IRR = 0.99, 95% confidence interval (CI) 0.92-1.06) or in the pooled trials (IRR = 1.07, 95% CI 0.93-1.24). We noted an increased risk of venous thromboembolism among irradiated patients in both cohorts (IRR register  = 1.41, 95% CI 1.15-2.72; IRR trials  = 1.41, 95% CI 0.97-2.04), that remained during the first 6 months following surgery among patients treated 2006-2009, after the introduction of antithrombotic treatment (IRR 6 months  = 2.30, 95% CI 1.01-5.21). However, the absolute rate difference of venous thromboembolism attributed to RT was low (10 cases per 1000 patients and year). Preoperative radiotherapy did not affect rectal cancer patients' risk of CVD overall. Although an excess risk of short-term venous thromboembolism was noted, the small increase in absolute numbers does not call for general changes in routine prophylactic treatment, but might do so for patients already at high risk of venous thromboembolism. Copyright © 2017 Elsevier B.V. All rights reserved.

  14. Fus1 KO Mouse As a Model of Oxidative Stress-Mediated Sporadic Alzheimer's Disease: Circadian Disruption and Long-Term Spatial and Olfactory Memory Impairments.

    Science.gov (United States)

    Coronas-Samano, Guillermo; Baker, Keeley L; Tan, Winston J T; Ivanova, Alla V; Verhagen, Justus V

    2016-01-01

    Insufficient advances in the development of effective therapeutic treatments of sporadic Alzheimer's Disease (sAD) to date are largely due to the lack of sAD-relevant animal models. While the vast majority of models do recapitulate AD's hallmarks of plaques and tangles by virtue of tau and/or beta amyloid overexpression, these models do not reflect the fact that in sAD (unlike familial AD) these genes are not risk factors per se and that other mechanisms like oxidative stress, metabolic dysregulation and inflammation play key roles in AD etiology. Here we characterize and propose the Fus1 KO mice that lack a mitochondrial protein Fus1/Tusc2 as a new sAD model. To establish sAD relevance, we assessed sAD related deficits in Fus1 KO and WT adult mice of 4-5 months old, the equivalent human age when the earliest cognitive and olfactory sAD symptoms arise. Fus1 KO mice showed oxidative stress (increased levels of ROS, decreased levels of PRDX1), disruption of metabolic homeostasis (decreased levels of ACC2, increased phosphorylation of AMPK), autophagy (decreased levels of LC3-II), PKC (decreased levels of RACK1) and calcium signaling (decreased levels of Calb2) in the olfactory bulb and/or hippocampus. Mice were behaviorally tested using objective and accurate video tracking (Noldus), in which Fus1 KO mice showed clear deficits in olfactory memory (decreased habituation/cross-habituation in the short and long term), olfactory guided navigation memory (inability to reduce their latency to find the hidden cookie), spatial memory (learning impairments on finding the platform in the Morris water maze) and showed more sleep time during the diurnal cycle. Fus1 KO mice did not show clear deficits in olfactory perception (cross-habituation), association memory (passive avoidance) or in species-typical behavior (nest building) and no increased anxiety (open field, light-dark box) or depression/anhedonia (sucrose preference) at this relatively young age. These neurobehavioral

  15. [Neurological and psychomotor development of foetuses and children with congenital heart disease--causes and prevalence of disorders and long-term prognosis].

    Science.gov (United States)

    Herberg, U; Hövels-Gürich, H

    2012-06-01

    Children with severe congenital heart defects (CHD) requiring open heart surgery in the first year of life are at high risk for developing neurological and psychomotor abnormalities. Depending on the type and severity of the CHD, between 15 and over 50% of these children have deficits, which are usually confined to distinct domains of development, although formal intelligence tends to be normal. Children with mild CHD, who comprise the majority of congenital heart defects, have a far better developmental prognosis than those with complex CHD. This review concentrates on the impact of severe CHD on the developing brain of the foetus and infant. It also provides a summary of recent clinical and neuroimaging studies, and an overview of the long-term neurological prognosis. Advanced neuroimaging modalities indicate that, related to altered cerebral blood flow and oxygenation, foetuses with severe CHD show delayed third trimester brain maturation and increased vulnerability for hypoxic injury. Morphological and neurological abnormalities are present before surgery, commonly affecting the white matter. In the long-term, impaired neurological and developmental outcomes are related to the combination of prenatal, perinatal and additional perioperative risk factors. Therefore, new therapeutic approaches aim to optimise the intra- and perinatal management of foetuses and newborns with congenital heart defects. Identification and avoidance of risk factors, early neurodevelopmental assessment and therapy may optimise the long-term outcome in this high-risk population. © Georg Thieme Verlag KG Stuttgart · New York.

  16. Long-term impact of a chronic disease management program on hospital utilization and cost in an Australian population with heart disease or diabetes.

    Science.gov (United States)

    Hamar, G Brent; Rula, Elizabeth Y; Coberley, Carter; Pope, James E; Larkin, Shaun

    2015-04-22

    To evaluate the longitudinal value of a chronic disease management program, My Health Guardian (MHG), in reducing hospital utilization and costs over 4 years. The MHG program provides individualized support via telephonic nurse outreach and online tools for self-management, behavior change and well-being. In follow up to an initial 18-month analysis of MHG, the current study evaluated program impact over 4 years. A matched-cohort analysis retrospectively compared MHG participants with heart disease or diabetes (treatment, N = 4,948) to non-participants (comparison, N = 28,520) on utilization rates (hospital admission, readmission, total bed days) and hospital claims cost savings. Outcomes were evaluated using regression analyses, controlling for remaining demographic, disease, and pre-program admissions or cost differences between the study groups. Over the 4 year period, program participation resulted in significant reductions in hospital admissions (-11.4%, P hospital claims was $3,549 over 4-years; savings values for each program year were significant and increased with time (P = 0.003 to P hospital utilization and costs for individuals with heart disease or diabetes and demonstrate the increasing program effect with continued participation over time.

  17. Changes in bleeding patterns in von Willebrand disease after institution of long-term replacement therapy : results from the von Willebrand Disease Prophylaxis Network

    NARCIS (Netherlands)

    Holm, Elena; Abshire, Thomas C; Bowen, Joel; Álvarez, M Teresa; Bolton-Maggs, Paula; Carcao, Manuel; Federici, Augusto B; Gill, Joan Cox; Halimeh, Susan; Kempton, Christine; Key, Nigel S; Kouides, Peter; Lail, Alice; Landorph, Andrea; Leebeek, Frank; Makris, Michael; Mannucci, Pier; Mauser-Bunschoten, Eveline P; Nugent, Diane; Valentino, Leonard A; Winikoff, Rochelle; Berntorp, Erik

    Clinically, the leading symptom in von Willebrand disease (VWD) is bleeding, chiefly of mucosal type, for example, epistaxis, gingival, or gastrointestinal bleeding, and menorrhagia. In severe forms of VWD with secondary deficiency of factor VIII, spontaneous joint bleeding, resembling that observed

  18. Low-fat, high-carbohydrate parenteral nutrition (PN) may potentially reverse liver disease in long-term PN-dependent infants

    DEFF Research Database (Denmark)

    Jakobsen, Marianne Skytte; Jørgensen, Marianne Hørby; Husby, Steffen

    2015-01-01

    INTRODUCTION: Parenteral nutrition-associated cholestasis (PNAC) is a complication of long-term parenteral nutrition (PN). Removal of lipids may reverse PNAC but compromises the energy to ensure infant growth. The purpose of this study was to test whether a low-fat, high-carbohydrate PN regimen......, which prevents and reverses PNAC in adults, could do the same in infants. This regimen could potentially avoid the problem of diminished energy input after removing nutritional lipids. METHODS: Infants developing PNAC over a 2-year period were started on a low-fat PN regimen with calories primarily from...

  19. Influence of long-term colonoscopic surveillance on incidence of colorectal cancer and death from the disease in patients with precursors (adenomas)

    DEFF Research Database (Denmark)

    Jørgensen, Ole Dan; Kronborg, Ole; Fenger, Claus

    2007-01-01

    Case-control studies and short term prospective studies have suggested that selected groups of patients with precursors of colorectal cancer may benefit from colonoscopic surveillance after initial removal of adenomas. The aim of the present study was to demonstrate such a possible benefit from...... long term (1-24 years) colonoscopic surveillance in a population of patients with all types of adenomas regardless of size and way of removal. Two thousand and forty-one patients with a first time diagnosis of colorectal adenoma were included in prospective surveillance between year 1978 and 2002. All...

  20. Nuclear Energy, Long Term Requirements

    International Nuclear Information System (INIS)

    Knapp, V.

    2006-01-01

    There are serious warnings about depletion of oil and gas and even more serious warnings about dangers of climate change caused by emission of carbon dioxide. Should developed countries be called to replace CO2 emitting energy sources as soon as possible, and the time available may not be longer then few decades, can nuclear energy answer the call and what are the requirements? Assuming optimistic contribution of renewable energy sources, can nuclear energy expand to several times present level in order to replace large part of fossil fuels use? Paper considers intermediate and long-term requirements. Future of nuclear power depends on satisfactory answers on several questions. First group of questions are those important for near and intermediate future. They deal with economics and safety of nuclear power stations in the first place. On the same time scale a generally accepted concept for radioactive waste disposal is also required. All these issues are in the focus of present research and development. Safer and more economical reactors are targets of international efforts in Generation IV and INPRO projects, but aiming further ahead these innovative projects are also addressing issues such as waste reduction and proliferation resistance. However, even assuming successful technical development of these projects, and there is no reason to doubt it, long term and large-scale nuclear power use is thereby not yet secured. If nuclear power is to play an essential role in the long-term future energy production and in reduction of CO2 emission, than several additional questions must be replied. These questions will deal with long-term nuclear fuel sufficiency, with necessary contribution of nuclear power in sectors of transport and industrial processes and with nuclear proliferation safety. This last issue is more political then technical, thus sometimes neglected by nuclear engineers, yet it will have essential role for the long-term prospects of nuclear power. The

  1. Analysing long term discursive processes

    DEFF Research Database (Denmark)

    Horsbøl, Anders

    which extend beyond the single interaction, for instance negotiations or planning processes, seems to have played a less important role, with studies such as Iedema 2001 and Wodak 2000 as exceptions. These long term processes, however, are central to the constitution and workings of organizations......What do timescales - the notion that processes take place or can be viewed within a shorter or longer temporal range (Lemke 2005) - mean for the analysis of discourse? What are the methodological consequences of analyzing discourse at different timescales? It may be argued that discourse analysis...... in general has favored either the analysis of short term processes such as interviews, discussions, and lessons, or the analysis of non-processual entities such as (multimodal) texts, arguments, discursive repertoires, and discourses (in a Foucaultian sense). In contrast, analysis of long term processes...

  2. Comparing long term energy scenarios

    International Nuclear Information System (INIS)

    Cumo, M.; Simbolotti, G.

    2001-01-01

    Major projection studies by international organizations and senior analysts have been compared with reference to individual key parameters (population, energy demand/supply, resources, technology, emissions and global warming) to understand trends and implications of the different scenarios. Then, looking at the long term (i.e., 2050 and beyond), parameters and trends have been compared together to understand and quantify whether and when possible crisis or market turbulence might occur due to shortage of resources or environmental problems [it

  3. Delayed somatic effects following extended radiotherapy. Studies of 135 patients with Hodgkin's disease in long-term remission (Freiburg collective, treatment years 1948 to 1974). Untersuchungen ueber somatische Spaetschaeden nach ausgedehnter Strahlentherapie

    Energy Technology Data Exchange (ETDEWEB)

    Slanina, J

    1977-01-01

    A group of 135 patients who suffered from Hodgkin's disease with long-term remission following radiotherapy was investigated. By restricting the investigations to the patient group with long-term remission and correlative examinations, it was tried to differentiate between exclusive or principal delayed radiation effects and delayed effects due to other factors. The study reports about the most significant results obtained in the fields of catamnesis, laboratory diagnostics, hematology, pulmology, cardiology, thyroid function tests, neurology, andrology and dermatology. Due to the numerous detectable delayed effects the group of patients with long-term remission receives a special status. Although those effects are in no proportion to the deletary risks of an untreated Hodgkin's disease and though they are consequently tolerable in the present state of the therapeutic development, they must initiate the completion of the therapeutic concept including performance of radiotherapy and aftercare, because only then the risk for the patient provoked by these effects, which ranges beyond the malignity of this disease can be reduced or prevented.

  4. Long term radioactive waste management

    International Nuclear Information System (INIS)

    Lavie, J.M.

    1984-01-01

    In France, waste management, a sensitive issue in term of public opinion, is developing quickly, and due to twenty years of experience, is now reaching maturity. With the launching of the French nuclear programme, the use of radioactive sources in radiotherapy and industry, waste management has become an industrial activity. Waste management is an integrated system dealing with the wastes from their production to the long term disposal, including their identification, sortage, treatment, packaging, collection and transport. This system aims at guaranteing the protection of present and future populations with an available technology. In regard to their long term management, and the design of disposals, radioactive wastes are divided in three categories. This classification takes into account the different radioisotopes contained, their half life and their total activity. Presently short-lived wastes are stored in the shallowland disposal of the ''Centre de la Manche''. Set up within the French Atomic Energy Commission (CEA), the National Agency for waste management (ANDRA) is responsible within the framework of legislative and regulatory provisions for long term waste management in France [fr

  5. Setting the stage for long-term reproductive health.

    Science.gov (United States)

    Payne, Craig A; Vander Ley, Brian; Poock, Scott E

    2013-11-01

    This article discusses some of the aspects of heifer development that contribute to long-term health and productivity, such as disease prevention and control. Nutrition is also an important component of long-term health, and body condition score is discussed as a way to determine whether the nutrient demands of heifers are being met. Copyright © 2013 Elsevier Inc. All rights reserved.

  6. Inflammatory markers in relation to long-term air pollution

    NARCIS (Netherlands)

    Mostafavi Montazeri, Nahid|info:eu-repo/dai/nl/375290575; Vlaanderen, Jelle|info:eu-repo/dai/nl/31403160X; Chadeau-Hyam, Marc; Beelen, Rob|info:eu-repo/dai/nl/30483100X; Modig, Lars; Palli, Domenico; Bergdahl, Ingvar A; Vineis, Paolo; Hoek, Gerard|info:eu-repo/dai/nl/069553475; Kyrtopoulos, Soterios Α; Vermeulen, Roel|info:eu-repo/dai/nl/216532620

    Long-term exposure to ambient air pollution can lead to chronic health effects such as cancer, cardiovascular and respiratory disease. Systemic inflammation has been hypothesized as a putative biological mechanism contributing to these adverse health effects. We evaluated the effect of long-term

  7. Long-term effects of radiation

    International Nuclear Information System (INIS)

    Smith, J.; Smith, T.

    1981-01-01

    It is pointed out that sources of long-term damage from radiation are two-fold. People who have been exposed to doses of radiation from initial early fallout but have recovered from the acute effects may still suffer long-term damage from their exposure. Those who have not been exposed to early fallout may be exposed to delayed fallout, the hazards from which are almost exclusively from ingesting strontium, caesium and carbon isotopes present in food; the damage caused is relatively unimportant compared with that caused by the brief doses from initial radiation and early fallout. A brief discussion is presented of the distribution of delayed long-lived isotope fallout, and an outline is sketched of late biological effects, such as malignant disease, cataracts, retarded development, infertility and genetic effects. (U.K.)

  8. MANAGEMENT OF ENDOCRINE DISEASE: Atypical femoral fractures: risks and benefits of long-term treatment of osteoporosis with anti-resorptive therapy.

    Science.gov (United States)

    Adler, Robert A

    2018-03-01

    Modern osteoporosis treatment began in the mid-1990s with the approval of amino-bisphosphonates, anti-resorptive agents that have been shown to decrease osteoporotic fracture risk by about half. In 2005, the first cases of atypical femoral fractures (AFF), occurring in the shaft of the femur, were reported. Since then, more cases have been found, leading to great concern among patients and a dramatic decrease in bisphosphonate prescribing. The pathogenesis and incidence of AFF are reviewed herein. Management and an approach to prevention or early detection of AFF are also provided. Denosumab, a more recently approved anti-resorptive medication has also been associated with AFF. Long-term management of osteoporosis and prevention of fracture are challenging in light of this serious but uncommon side effect, yet with an aging population osteoporotic fracture is destined to increase in frequency. © 2018 European Society of Endocrinology.

  9. Long term presence of a single predominant tyrosinase-specific T-cell clone associated with disease control in a patient with metastatic melanoma.

    Science.gov (United States)

    Ochsenreither, Sebastian; Fusi, Alberto; Busse, Antonia; Letsch, Anne; Haase, Doreen; Thiel, Eckhard; Scheibenbogen, Carmen; Keilholz, Ulrich

    2010-05-15

    In an earlier study, we described a patient who developed an anti-tyrosinase T-cell response leading to long-term tumor control. Here we analyzed this response with regard to T-cell receptor (TCR) Vbeta family usage and clonality in order to further elucidate the nature of the T cell response in this patient. For identification of expanded specific cytotoxic T-cell (CTL) clones, tetramer enrichment of tyrosinase reactive T-cells was followed by comparative quantitative reverse transcribed PCR (qRT PCR) quantification of all TCR Vbeta-families and sequencing of family Vbeta4 elevated in the enriched fraction. The predominant specific clone was quantified by clonotypic qRT PCR in multiple samples from blood, bone marrow, and tumor tissue. FACS analyses with staining of TYR.A2 and TCR Vbeta4 were performed. Epitope specific enrichment revealed an isolated increase of Vbeta-family 4. FACS analysis showed a shift of specific CTLs to Vbeta-family 4 during tumor regression with a maximum of 80% of all TYR.A2 specific cells belonging to this family. Sequencing revealed a single predominant clone against polyclonal background coding for identical CDR3 loops. The predominant clone was highly expressed in bone marrow and tumor tissue, and was detectable in blood over a period of ten years. Considering the results of previous studies showing a specific effector phenotype in blood and a specific memory compartment in bone marrow of this patient, this data implicate the predominant clone featured all attributes of a sufficient CTL response including homing capacity and memory formation resulting in long term clonal persistence and tumor control.

  10. Long-term biodosimetry Redux

    International Nuclear Information System (INIS)

    Simon, Steven L.; Bouville, Andre

    2016-01-01

    This paper revisits and reiterates the needs, purposes and requirements of bio-dosimetric assays for long-term dose and health risk assessments. While the most crucial need for bio-dosimetric assays is to guide medical response for radiation accidents, the value of such techniques for improving our understanding of radiation health risk by supporting epidemiological (long-term health risk) studies is significant. As new cohorts of exposed persons are identified and new health risk studies are undertaken with the hopes that studying the exposed will result in a deeper understanding of radiation risk, the value of reliable dose reconstruction is underscored. The ultimate application of biodosimetry in long-term health risk studies would be to completely replace model-based dose reconstruction-a complex suite of methods for retrospectively estimating dose that is commonly fraught with large uncertainties due to the absence of important exposure-related information, as well as imperfect models. While biodosimetry could potentially supplant model-based doses, there are numerous limitations of presently available techniques that constrain their widespread application in health risk research, including limited ability to assess doses received far in the past, high cost, great inter-individual variability, invasiveness, higher than preferred detection limits and the inability to assess internal dose (for the most part). These limitations prevent the extensive application of biodosimetry to large cohorts and should be considered a challenge to researchers to develop new and more flexible techniques that meet the demands of long-term health risk research. Events in recent years, e.g. the Fukushima reactor accident and the increased threat of nuclear terrorism, underscore that any event that results in significant radiation exposures of a group of people will also produce a much larger population, exposed at lower levels, but that likewise needs (or demands) an exposure

  11. Long term stability of power systems

    Energy Technology Data Exchange (ETDEWEB)

    Kundur, P; Gao, B [Powertech Labs. Inc., Surrey, BC (Canada)

    1994-12-31

    Power system long term stability is still a developing subject. In this paper we provide our perspectives and experiences related to long term stability. The paper begins with the description of the nature of the long term stability problem, followed by the discussion of issues related to the modeling and solution techniques of tools for long term stability analysis. Cases studies are presented to illustrate the voltage stability aspect and plant dynamics aspect of long term stability. (author) 20 refs., 11 figs.

  12. Long-term sequelae of Farmer's lung disease in HRCT: a 14-year follow-up study of 88 patients and 83 matched control farmers

    International Nuclear Information System (INIS)

    Malinen, A.P.; Partanen, P.L.K.; Rytkoenen, H.T.; Vanninen, R.L.; Erkinjuntti-Pekkanen, R.A.

    2003-01-01

    The aim of this study was to compare high-resolution computed tomography (HRCT) findings of long-term farmer's lung (FL) patients and control farmers. We studied 88 FL patients and 83 matched control farmers with a mean follow-up time of 14 years. Emphysematous, fibrotic, and miliary changes were recorded by HRCT. The pattern of emphysema and location and distribution of other findings were evaluated in detail. Emphysema was found in 20 (23%) FL patients and in 6 (7%) controls (p=0.005). Recurrences of FL attacks increased (p=0.021) the risk of emphysema. Prevalence of fibrosis (17 vs 10%, p=0.16) and miliary changes (11 vs 4%, p=0.06) did not differ significantly in patients and controls. Among FL patients, emphysematous, fibrous, and miliary changes were more pronounced at the base than in the upper parts of the lung (p<0.02). In slice analysis, the pattern of emphysema was more polymorphous (p=0.001) and the distribution of fibrotic and miliary changes was more variable among FL patients than controls. Emphysema in HRCT is more common in FL patients than matched control farmers, and the occurrence is increased by recurrences of FL. Emphysematous, fibrous, and miliary changes in FL patients HRCT are multiform and predominate in the lower parts of the lung. (orig.)

  13. Long-Term Outcomes in Puerto Ricans with Rheumatoid Arthritis (RA) Receiving Early Treatment with Disease-Modifying Anti-Rheumatic Drugs using the American College of Rheumatology Definition of Early RA.

    Science.gov (United States)

    Varela-Rosario, Noemí; Arroyo-Ávila, Mariangelí; Fred-Jiménez, Ruth M; Díaz-Correa, Leyda M; Pérez-Ríos, Naydi; Rodríguez, Noelia; Ríos, Grissel; Vilá, Luis M

    2017-01-01

    Early treatment of rheumatoid arthritis (RA) results in better long-term outcomes. However, the optimal therapeutic window has not been clearly established. To determine the clinical outcome of Puerto Ricans with RA receiving early treatment with conventional and/or biologic disease-modifying anti-rheumatic drugs (DMARDs) based on the American College of Rheumatology (ACR) definition of early RA. A cross-sectional study was performed in a cohort of Puerto Ricans with RA. Demographic features, clinical manifestations, disease activity, functional status, and pharmacotherapy were determined. Early treatment was defined as the initiation of DMARDs (conventional and/or biologic) in less than 6 months from the onset of symptoms attributable to RA. Patients who received early (disease duration was 14.9 years and 337 (87.0%) patients were women. One hundred and twenty one (31.3%) patients received early treatment. In the multivariate analysis adjusted for age and sex, early treatment was associated with better functional status, lower probability of joint deformities, intra-articular injections and joint replacement surgeries, and lower scores in the physician's assessments of global health, functional impairment and physical damage of patients. Using the ACR definition of early RA, this group of patients treated with DMARDs within 6 months of disease had better long-term outcomes with less physical damage and functional impairment.

  14. Craniopharyngioma in Children: Long-term Outcomes

    Science.gov (United States)

    STEINBOK, Paul

    2015-01-01

    The survival rate for childhood craniopharyngioma has been improving, with more long-term survivors. Unfortunately it is rare for the patient to be normal, either from the disease itself or from the effects of treatment. Long-term survivors of childhood craniopharyngioma suffer a number of impairments, which include visual loss, endocrinopathy, hypothalamic dysfunction, cerebrovascular problems, neurologic and neurocognitive dysfunction. Pituitary insufficiency is present in almost 100%. Visual and hypothalamic dysfunction is common. There is a high risk of metabolic syndrome and increased risk of cerebrovascular disease, including stroke and Moyamoya syndrome. Cognitive, psychosocial, and emotional problems are prevalent. Finally, there is a higher risk of premature death among survivors of craniopharyngioma, and often this is not from tumor recurrence. It is important to consider craniopharyngioma as a chronic disease. There is no perfect treatment. The treatment has to be tailored to the individual patient to minimize dysfunction caused by tumor and treatments. So “cure” of the tumor does not mean a normal patient. The management of the patient and family needs multidisciplinary evaluation and should involve ophthalmology, endocrinology, neurosurgery, oncology, and psychology. Furthermore, it is also important to address emotional issues and social integration. PMID:26345668

  15. A Novel Study Paradigm for Long-term Prevention Trials in Alzheimer Disease: The Placebo Group Simulation Approach (PGSA): Application to MCI data from the NACC database.

    Science.gov (United States)

    Berres, M; Kukull, W A; Miserez, A R; Monsch, A U; Monsell, S E; Spiegel, R

    2014-01-01

    as determined for the original NTB score. An algorithm comprising a small number of baseline variables, notably cognitive performance at baseline, forecasts the group trajectory of cognitive decline in subsequent years with high accuracy. The current analysis of 3 independent subgroups of aMCI patients from the NACC database supports the validity of the PGSA longitudinal algorithm for a NTB. Use of the PGSA in long-term secondary AD prevention trials deserves consideration.

  16. Effect of enzyme replacement therapy on isokinetic strength for all major muscle groups in four patients with Pompe disease-a long-term follow-up

    DEFF Research Database (Denmark)

    Andreassen, Christer Swan; Schlütter, Jacob Mørup; Vissing, John

    2014-01-01

    Pompe disease is a rare, inherited metabolic myopathy characterized by progressive weakness of the proximal limb and respiratory muscles. We report the findings from four patients with late-onset Pompe disease treated with α-glucosidase (Myozyme) for 2 (n=2) and 6 (n=2) years, and monitored with ...

  17. The genetics of congenital heart disease… understanding and improving long-term outcomes in congenital heart disease: a review for the general cardiologist and primary care physician.

    Science.gov (United States)

    Simmons, M Abigail; Brueckner, Martina

    2017-10-01

    This review has two purposes: to provide an updated review of the genetic causes of congenital heart disease (CHD) and the clinical implications of these genetic mutations, and to provide a clinical algorithm for clinicians considering a genetics evaluation of a CHD patient. A large portion of congenital heart disease is thought to have a significant genetic contribution, and at this time a genetic cause can be identified in approximately 35% of patients. Through the advances made possible by next generation sequencing, many of the comorbidities that are frequently seen in patients with genetic congenital heart disease patients can be attributed to the genetic mutation that caused the congenital heart disease. These comorbidities are both cardiac and noncardiac and include: neurodevelopmental disability, pulmonary disease, heart failure, renal dysfunction, arrhythmia and an increased risk of malignancy. Identification of the genetic cause of congenital heart disease helps reduce patient morbidity and mortality by improving preventive and early intervention therapies to address these comorbidities. Through an understanding of the clinical implications of the genetic underpinning of congenital heart disease, clinicians can provide care tailored to an individual patient and continue to improve the outcomes of congenital heart disease patients.

  18. Long term study of mechanical

    Directory of Open Access Journals (Sweden)

    Ahmed M. Diab

    2016-06-01

    Full Text Available In this study, properties of limestone cement concrete containing different replacement levels of limestone powder were examined. It includes 0%, 5%, 10%, 15%, 20% and 25% of limestone powder as a partial replacement of cement. Silica fume was added incorporated with limestone powder in some mixes to enhance the concrete properties. Compressive strength, splitting tensile strength and modulus of elasticity were determined. Also, durability of limestone cement concrete with different C3A contents was examined. The weight loss, length change and cube compressive strength loss were measured for concrete attacked by 5% sodium sulfate using an accelerated test up to 525 days age. The corrosion resistance was measured through accelerated corrosion test using first crack time, cracking width and steel reinforcement weight loss. Consequently, for short and long term, the use of limestone up to 10% had not a significant reduction in concrete properties. It is not recommended to use blended limestone cement in case of sulfate attack. The use of limestone cement containing up to 25% limestone has insignificant effect on corrosion resistance before cracking.

  19. Long-term competence restoration.

    Science.gov (United States)

    Morris, Douglas R; DeYoung, Nathaniel J

    2014-01-01

    While the United States Supreme Court's Jackson v. Indiana decision and most state statutes mandate determinations of incompetent defendants' restoration probabilities, courts and forensic clinicians continue to lack empirical evidence to guide these determinations and do not yet have a consensus regarding whether and under what circumstances incompetent defendants are restorable. The evidence base concerning the restoration likelihood of those defendants who fail initial restoration efforts is even further diminished and has largely gone unstudied. In this study, we examined the disposition of a cohort of defendants who underwent long-term competence restoration efforts (greater than six months) and identified factors related to whether these defendants were able to attain restoration and adjudicative success. Approximately two-thirds (n = 52) of the 81 individuals undergoing extended restoration efforts were eventually deemed restored to competence. Lengths of hospitalization until successful restoration are presented with implications for the reasonable length of time that restoration efforts should persist. Older individuals were less likely to be restored and successfully adjudicated, and individuals with more severe charges and greater factual legal understanding were more likely to be restored and adjudicated. The significance of these findings for courts and forensic clinicians is discussed.

  20. Uranium ... long-term confidence

    International Nuclear Information System (INIS)

    Anon.

    1983-01-01

    Half way through 1983 the outlook for the world's uranium producers was far from bright if one takes a short term view. The readily accessible facts present a gloomy picture. The spot prices of uranium over the past few years decreased from a high of $42-$43/lb to a low of $17 in 1982. It now hovers between $23 and $24. the contract prices negotiated between producers and consumers are not so accessible but they do not reflect the spot price. The reasons why contractual uranium prices do not follow the usual dictates of supply and demand are related to the position in which uranium and associated power industries find themselves. There is public reaction with strong emotional overtones as well as much reduced expectations about the electric power needs of the world. Furthermore the supply of uranium is not guaranteed despite present over production. However the people in the industry, taking the medium- and long-term view, are not despondent

  1. Long-term corrosion studies

    International Nuclear Information System (INIS)

    Gdowski, G.

    1998-01-01

    The scope of this activity is to assess the long-term corrosion properties of metallic materials under consideration for fabricating waste package containers. Three classes of metals are to be assessed: corrosion resistant, intermediate corrosion resistant, and corrosion allowance. Corrosion properties to be evaluated are general, pitting and crevice corrosion, stress-corrosion cracking, and galvanic corrosion. The performance of these materials will be investigated under conditions that are considered relevant to the potential emplacement site. Testing in four aqueous solutions, and vapor phases above them, and at two temperatures are planned for this activity. (The environmental conditions, test metals, and matrix are described in detail in Section 3.0.) The purpose and objective of this activity is to obtain the kinetic and mechanistic information on degradation of metallic alloys currently being considered for waste package containers. This information will be used to provide assistance to (1) waste package design (metal barrier selection) (E-20-90 to E-20-92), (2) waste package performance assessment activities (SIP-PA-2), (3) model development (E-20-75 to E-20-89). and (4) repository license application

  2. Effect of discontinuation of long-term growth hormone treatment on carbohydrate metabolism and risk factors for cardiovascular disease in girls with Turner syndrome

    NARCIS (Netherlands)

    Y.K. van Pareren (Yvonne); S.M.P.F. de Muinck Keizer-Schrama (Sabine); Th. Stijnen (Theo); T.C.J. Sas (Theo); S.L.S. Drop (Stenvert)

    2002-01-01

    textabstractGH treatment increases insulin levels in girls with Turner syndrome (TS), who are already predisposed to develop diabetes mellitus and other risk factors for developing cardiovascular disease. Therefore, in the present study, we investigated carbohydrate metabolism and

  3. LONG-TERM BENEFITS OF REHABILITATION AT HOME ON QUALITY-OF-LIFE AND EXERCISE TOLERANCE IN PATIENTS WITH CHRONIC OBSTRUCTIVE PULMONARY-DISEASE

    NARCIS (Netherlands)

    WIJKSTRA, PJ; TENVERGERT, EM; VANALTENA, R; OTTEN, [No Value; KRAAN, J; POSTMA, DS; KOETER, GH

    Background - Pulmonary rehabilitation has been shown to have short term subjective and objective benefits for patients with chronic obstructive pulmonary disease (COPD). However, appropriately controlled studies have not previously been performed, nor have the benefits of different types of

  4. Long term risk factors for coronary heart disease and stroke: influence of duration of follow-up over four decades of mortality surveillance.

    Science.gov (United States)

    Batty, G David; Shipley, Martin; Smith, George Davey; Kivimaki, Mika

    2015-09-01

    While cohort studies have revealed a range of risk factors for coronary heart disease and stroke, the extent to which the strength of these associations varies according to duration of follow-up in studies with extended disease surveillance is unclear. This was the aim of the present study. Initiated in 1967/70, the original Whitehall study is an on-going cohort study of 15,402 male UK government workers free of coronary heart disease when they took part in a baseline medical examination during which a range of standard risk factors was measured. In analyses in which we stratified by duration of follow-up, there was evidence of time-dependency for most risk factor-disease relationships. Thus, the associations of systolic and diastolic blood pressure, total cholesterol and cigarette smoking with coronary heart disease and stroke diminished in strength with increasing duration of follow-up, whereas the magnitude of the body mass index-coronary heart disease relation was unchanged. For example, the age-adjusted hazard ratios (95% confidence interval) for cigarette smoking (versus never smoked) in relation to coronary heart disease were: 2.49 (1.80, 3.44), 1.65 (1.34, 2.03), 1.36 (1.15, 1.61) and 1.32 (1.10, 1.58) for follow-up periods 0-10, 10-20, 20-30 and 30+ years, respectively. Despite a general diminution in the strength of effect over time, even in the fourth decade of follow-up, classic risk factors retained some predictive capacity for coronary heart disease and, to a lesser degree, stroke. © The European Society of Cardiology 2014.

  5. Long-term outcome in patients with juvenile dermatomyositis

    DEFF Research Database (Denmark)

    Mathiesen, P; Hegaard, H; Herlin, Troels

    2012-01-01

    To evaluate a group of 53 patients with juvenile dermatomyositis (JDM), on average 13.9 years after disease onset, in order to describe the long-term disease outcome and to identify disease-related parameters associated with poor disease outcome....

  6. Association Between Change in Body Mass Index, Unified Parkinson's Disease Rating Scale Scores, and Survival Among Persons With Parkinson Disease: Secondary Analysis of Longitudinal Data From NINDS Exploratory Trials in Parkinson Disease Long-term Study 1.

    Science.gov (United States)

    Wills, Anne-Marie A; Pérez, Adriana; Wang, Jue; Su, Xiao; Morgan, John; Rajan, Suja S; Leehey, Maureen A; Pontone, Gregory M; Chou, Kelvin L; Umeh, Chizoba; Mari, Zoltan; Boyd, James

    2016-03-01

    Greater body mass index (BMI, calculated as weight in kilograms divided by height in meters squared) is associated with improved survival among persons with Huntington disease or amyotrophic lateral sclerosis. Weight loss is common among persons with Parkinson disease (PD) and is associated with worse quality of life. To explore the association between change in BMI, Unified Parkinson's Disease Rating Scale (UPDRS) motor and total scores, and survival among persons with PD and to test whether there is a positive association between BMI at randomization and survival. Secondary analysis (from May 27, 2014, to October 13, 2015) of longitudinal data (3-6 years) from 1673 participants who started the National Institute of Neurological Disorders and Stroke Exploratory Trials in PD Long-term Study-1 (NET-PD LS-1). This was a double-blind randomized placebo-controlled clinical trial of creatine monohydrate (10 g/d) that was performed at 45 sites throughout the United States and Canada. Participants with early (within 5 years of diagnosis) and treated (receiving dopaminergic therapy) PD were enrolled from March 2007 to May 2010 and followed up until September 2013. Change across time in motor UPDRS score, change across time in total UPDRS score, and time to death. Generalized linear mixed models were used to estimate the effect of BMI on the change in motor and total UPDRS scores after controlling for covariates. Survival was analyzed using Cox proportional hazards models of time to death. A participant's BMI was measured at randomization, and BMI trajectory groups were classified according to whether participants experienced weight loss ("decreasing BMI"), weight stability ("stable BMI"), or weight gain ("increasing BMI") during the study. Of the 1673 participants (mean [SD] age, 61.7 [9.6] years; 1074 [64.2%] were male), 158 (9.4%) experienced weight loss (decreasing BMI), whereas 233 (13.9%) experienced weight gain (increasing BMI). After adjusting for covariates, we

  7. Long term effectiveness of RA-1, a standardized Ayurvedic medicine as a monotherapy and in combination with disease modifying anti-rheumatic drugs in the treatment of rheumatoid arthritis.

    Science.gov (United States)

    Chopra, Arvind; Saluja, Manjit; Kianifard, Toktam; Chitre, Deepa; Venugopalan, Anuradha

    2018-03-08

    Data on long term use of Ayurvedic drugs is sparse. They may prove useful if combined with modern medicine in certain clinical situations (integrative medicine). We present the results of a long term observational study of RA-1 (Ayurvedic drug) used in the treatment of rheumatoid arthritis (RA). On completion of a 16 week randomized controlled study, 165 consenting volunteer patients were enrolled into a three year open label phase (OLP) study. Patients were symptomatic with persistent active disease and naïve for disease modifying anti-rheumatic drugs (DMARD). 57 patients were on fixed low dose prednisone. Patients were examined every 10-14 weeks in a routine rheumatology practice using standard care norms. They continued RA-1 (Artrex ™, 2 tablets twice daily) throughout the study period and were generally advised to lead a healthy life style. Based on clinical judgment, rheumatologist added DMARD and/or steroids (modified if already in use) to patients with inadequate response; chloroquine and/or methotrexate commonly used. Treatment response was assessed using American College of Rheumatology (ACR) efficacy measures and ACR 20% improvement index standard update statistical software (SAS and SPSS) were used; significant at p Ayurveda Foundation. Published by Elsevier B.V. All rights reserved.