Glycogen storage disease type II (Pompe disease in children

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A. N. Semyachkina

2014-01-01

Full Text Available The paper gives the data available in the literature, which reflect the manifestations, diagnosis, and current treatments of the rare (orphan inherited disease glycogen storage disease type II or Pomp disease in children, as well as its classification. The infant form is shown to be most severe, resulting in death from cardiovascular or pulmonary failure generally within the first year of a child’s life. Emphasis is laid on major difficulties in the differential and true diagnosis of this severe disease. Much attention is given to the new pathogenetic treatment — genetically engineered enzyme replacement drug Myozyme®. The authors describe their clinical case of a child with the juvenile form of glycogen storage disease type II (late-onset Pompe disease. Particular emphasis is laid on the clinical symptoms of the disease and its diagnostic methods, among which the morphological analysis of a muscle biopsy specimen by light and electron microscopies, and enzyme and DNA diagnoses are of most importance. The proband was found to have significant lysosomal glycogen accumulation in the muscle biopsy specimen, reduced lymphocyte acid α-1,4-glucosidase activity to 4,2 nM/mg/h (normal value, 13,0—53,6 nM/mg/h, described in the HGMD missense mutation database from 1000 G>A p.Gly334er of the GAA in homozygous state, which verified the diagnosis of Pompe disease. 

Facial-muscle weakness, speech disorders and dysphagia are common in patients with classic infantile Pompe disease treated with enzyme therapy.

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van Gelder, C M; van Capelle, C I; Ebbink, B J; Moor-van Nugteren, I; van den Hout, J M P; Hakkesteegt, M M; van Doorn, P A; de Coo, I F M; Reuser, A J J; de Gier, H H W; van der Ploeg, A T

2012-05-01

Classic infantile Pompe disease is an inherited generalized glycogen storage disorder caused by deficiency of lysosomal acid α-glucosidase. If left untreated, patients die before one year of age. Although enzyme-replacement therapy (ERT) has significantly prolonged lifespan, it has also revealed new aspects of the disease. For up to 11 years, we investigated the frequency and consequences of facial-muscle weakness, speech disorders and dysphagia in long-term survivors. Sequential photographs were used to determine the timing and severity of facial-muscle weakness. Using standardized articulation tests and fibreoptic endoscopic evaluation of swallowing, we investigated speech and swallowing function in a subset of patients. This study included 11 patients with classic infantile Pompe disease. Median age at the start of ERT was 2.4 months (range 0.1-8.3 months), and median age at the end of the study was 4.3 years (range 7.7 months -12.2 years). All patients developed facial-muscle weakness before the age of 15 months. Speech was studied in four patients. Articulation was disordered, with hypernasal resonance and reduced speech intelligibility in all four. Swallowing function was studied in six patients, the most important findings being ineffective swallowing with residues of food (5/6), penetration or aspiration (3/6), and reduced pharyngeal and/or laryngeal sensibility (2/6). We conclude that facial-muscle weakness, speech disorders and dysphagia are common in long-term survivors receiving ERT for classic infantile Pompe disease. To improve speech and reduce the risk for aspiration, early treatment by a speech therapist and regular swallowing assessments are recommended.

Childhood Pompe disease: clinical spectrum and genotype in 31 patients

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Capelle, C.I. van; Meijden, J.C. van der; Hout, J.M. van den; Jaeken, J.; Baethmann, M.; Voit, T.; Kroos, M.A.; Derks, T.G.; Rubio-Gozalbo, M.E.; Willemsen, M.A.A.P.; Lachmann, R.H.; Mengel, E.; Michelakakis, H.; Jongste, J.C. de; Reuser, A.J.; Ploeg, A.T. van der

2016-01-01

BACKGROUND: As little information is available on children with non-classic presentations of Pompe disease, we wished to gain knowledge of specific clinical characteristics and genotypes. We included all patients younger than 18 years, who had been evaluated at the Pompe Center in Rotterdam, the

Childhood Pompe disease: Clinical spectrum and genotype in 31 patients

NARCIS (Netherlands)

C.I. van Capelle (Carine); J.C. van der Meijden (Chris); J.M.P. van den Hout (Johanna); J. Jaeken; M. Baethmann; T. Voit; M.A. Kroos (Marian); T.G.J. Derks (Terry G J); M.E. Rubio-Gozalbo (Estela); M.A. Willemsen (Michél); R. Lachmann (Robin); E. Mengel; H. Michelakakis (Helen); J.C. de Jongste (Johan); A.J.J. Reuser (Arnold); A.T. van der Ploeg (Ans)

2016-01-01

textabstractBackground: As little information is available on children with non-classic presentations of Pompe disease, we wished to gain knowledge of specific clinical characteristics and genotypes. We included all patients younger than 18 years, who had been evaluated at the Pompe Center in

Childhood Pompe disease : clinical spectrum and genotype in 31 patients

NARCIS (Netherlands)

van Capelle, C I; van der Meijden, J C; van den Hout, J M P; Jaeken, J; Baethmann, M; Voit, T; Kroos, M A; Derks, T G J; Rubio-Gozalbo, M E; Willemsen, M A; Lachmann, R H; Mengel, E; Michelakakis, H; de Jongste, J C; Reuser, A J J; van der Ploeg, A T

2016-01-01

Background: As little information is available on children with non-classic presentations of Pompe disease, we wished to gain knowledge of specific clinical characteristics and genotypes. We included all patients younger than 18 years, who had been evaluated at the Pompe Center in Rotterdam, the

Effect of enzyme replacement therapy with alglucosidase alfa (Myozyme®) in 12 patients with advanced late-onset Pompe disease.

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Papadopoulos, Constantinos; Orlikowski, David; Prigent, Hélène; Lacour, Arnaud; Tard, Céline; Furby, Alain; Praline, Julien; Solé, Guilhem; Hogrel, Jean-Yves; De Antonio, Marie; Semplicini, Claudio; Deibener-Kaminsky, Joelle; Kaminsky, Pierre; Eymard, Bruno; Taouagh, Nadjib; Perniconi, Barbara; Hamroun, Dalil; Laforêt, Pascal

2017-09-01

The efficacy of enzyme replacement therapy (ERT) in patients at an advanced stage of Pompe disease has only been addressed in a few studies. Our objective was to assess the long term effects of ERT in a cohort of patients with severe Pompe disease. We identified patients from the French Pompe Registry with severe respiratory failure and permanent wheelchair use (assisted walk for a few meters was allowed) when starting ERT. Patients' medical records were collected and reviewed and respiratory and motor functions, before ERT initiation and upon last evaluation were compared. Twelve patients (7 males) were identified. Median age at symptom onset was 24years [IQR=15.5; 36.0]. At baseline ventilation was invasive in 11 patients and noninvasive in one, with a median ventilation time of 24h [IQR=21.88; 24.00] (min 20; max 24). ERT was initiated at a median age of 52.5years [IQR=35.75; 66.50]. Median treatment duration was 55months [IQR=39.5; 81.0]. During observational period no adverse reaction to ERT was recorded, five patients (41.67%) died, three decreased their ventilation time by 30, 60 and 90min and two increased their assisted walking distance, by 80 and 20m. Some patients at a very advanced stage of Pompe disease may show a mild benefit from ERT, in terms of increased time of autonomous ventilation and of enlarged distance in assisted walk. ERT can be initiated in these patients in order to retain their current level of independence and ability to perform daily life activities. Copyright © 2017 Elsevier Inc. All rights reserved.

Public support for neonatal screening for Pompe disease, a broad-phenotype condition

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Weinreich, S.S.; Rigter, T.; van El, C.G.; Dondorp, W.J.; Kostense, P.J.; van der Ploeg, A.T.; Reuser, A.J.; Cornel, M.C.; Hagemans, M.L.

2012-01-01

Background: Neonatal screening for Pompe disease has been introduced in Taiwan and a few U.S. states, while other jurisdictions including some European countries are piloting or considering this screening. First-tier screening flags both classic infantile and late-onset Pompe disease, which

Reevaluating Muscle Biopsies in the Diagnosis of Pompe Disease: A Corroborative Report.

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Genge, Angela; Campbell, Natasha

2016-07-01

Previous reports suggest that although a diagnostic muscle biopsy can confirm the presence of Pompe disease, the absence of a definitive biopsy result does not rule out the diagnosis. In this study, we reviewed patients with a limb-girdle syndrome who demonstrated nonspecific abnormalities of muscle, without evidence of the classical changes of acid maltase deficiency. These patients were rescreened for Pompe disease using dried blood spot (DBS) testing. Twenty-seven patients provided blood samples for the DBS test. Four patients underwent subsequent genetic testing. Genetic analysis demonstrated that one patient tested positive for Pompe disease and one patient had one copy of a pathogenic variant. In conclusion, the ability of a diagnostic muscle biopsy to definitively rule out the presence of Pompe disease is limited. There is a role for a screening DBS in all patients presenting with a limb-girdle syndrome without a clear diagnosis.

Enzyme replacement therapy in late-onset Pompe's disease : A three-year follow-up

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Winkel, LPF; Van den Hout, JMP; Kamphoven, JHJ; Disseldorp, JAM; Remmerswaal, M; Arts, WFM; Loonen, MCB; Vulto, AG; Van Doorn, PA; De Jong, G; Hop, W; Smit, GPA; Shapira, SK; Boer, MA; van Diggelen, OP; Reuser, AJJ; Van der Ploeg, AT

Pompe's disease is an autosomal recessive myopathy. The characteristic lysosomal storage of glycogen is caused by acid et-glucosidase deficiency. Patients with late-onset Pompe's disease present with progressive muscle weakness also affecting pulmonary function. In search of a treatment, we

Children with Pompe disease: clinical characteristics, peculiar features and effects of enzyme replacement therapy

NARCIS (Netherlands)

C.I. van Capelle (Carine)

2014-01-01

markdownabstract__Abstract__ Pompe disease is a metabolic myopathy. Since the first description of the disease in 1932 by J.C. Pompe,1 tremendous progress has been made from discovering the biochemical and genetic basis of the disease to developing enzyme replacement therapy (ERT). With this

Cognitive Development in Infantile-Onset Pompe Disease Under Very Early Enzyme Replacement Therapy.

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Lai, Chih-Jou; Hsu, Ting-Rong; Yang, Chia-Feng; Chen, Shyi-Jou; Chuang, Ya-Chin; Niu, Dau-Ming

2016-12-01

Most patients with infantile-onset Pompe disease die in early infancy before beginning enzyme replacement therapy, which has made it difficult to evaluate the impact of Pompe disease on cognitive development. Patients with infantile-onset Pompe disease can survive with enzyme replacement therapy, and physicians can evaluate cognitive development in these patients. We established an effective newborn screening program with quick clinical diagnostic criteria. Cognitive and motor development were evaluated using the Bayley Scales of Infant and Toddler Development-Third Edition at 6, 12, and 24 months of age. The patients who were treated very early demonstrate normal cognitive development with no significant change in cognition during this period (P = .18 > .05). The cognitive development was positively correlated with motor development (r = 0.533, P = .011). The results indicated that very early enzyme replacement therapy could protect cognitive development in patients with infantile-onset Pompe disease up to 24 months of age. © The Author(s) 2016.

[Adult form of Pompe disease].

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Ziółkowska-Graca, Bozena; Kania, Aleksander; Zwolińska, Grazyna; Nizankowska-Mogilnicka, Ewa

2008-01-01

Pompe disease (glycogen-storage disease type II) is an autosomal recessive disorder caused by a deficiency of lysosomal acid alpha-glucosidase (GAA), leading to the accumulation of glycogen in the lysosomes primarily in muscle cells. In the adult form of the disease, proximal muscle weakness is noted and muscle volume is decreased. The infantile form is usually fatal. In the adult form of the disease the prognosis is relatively good. Muscle weakness may, however, interfere with normal daily activities, and respiratory insufficiency may be associated with obstructive sleep apnea. Death usually results from respiratory failure. Effective specific treatment is not available. Enzyme replacement therapy with recombinant human GAA (rh-GAA) still remains a research area. We report the case of a 24-year-old student admitted to the Department of Pulmonary Diseases because of severe respiratory insufficiency. Clinical symptoms such as dyspnea, muscular weakness and increased daytime sleepiness had been progressing for 2 years. Clinical examination and increased blood levels of CK suggested muscle pathology. Histopathological analysis of muscle biopsy, performed under electron microscope, confirmed the presence of vacuoles containing glycogen. Specific enzymatic activity of alpha-glucosidase was analyzed confirming Pompe disease. The only effective method to treat respiratory insufficiency was bi-level positive pressure ventilation. Respiratory rehabilitation was instituted and is still continued by the patient at home. A high-protein, low-sugar diet was proposed for the patient. Because of poliglobulia low molecular weight heparin was prescribed. The patient is eligible for experimental replacement therapy with rh-GAA.

Fat and carbohydrate metabolism during exercise in late-onset Pompe disease

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Preisler, Nicolai; Laforet, Pascal; Madsen, Karen Lindhardt

2012-01-01

forearm exercise testing, and peak work capacity was determined. Fat and carbohydrate metabolism during cycle exercise was examined with a combination of indirect calorimetry and stable isotope methodology. Finally, the effects of an IV glucose infusion on heart rate, ratings of perceived exertion...... examined the metabolic response to exercise in patients with late-onset Pompe disease, in order to determine if a defect in energy metabolism may play a role in the pathogenesis of Pompe disease. We studied six adult patients with Pompe disease and 10 healthy subjects. The participants underwent ischemic......, and work capacity during exercise were determined. We found that peak oxidative capacity was reduced in the patients to 17.6 vs. 38.8 ml kg(-1) min(-1) in healthy subjects (p = 0.002). There were no differences in the rate of appearance and rate of oxidation of palmitate, or total fat and carbohydrate...

Stimulation of Respiratory Motor Output and Ventilation in a Murine Model of Pompe Disease by Ampakines.

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ElMallah, Mai K; Pagliardini, Silvia; Turner, Sara M; Cerreta, Anthony J; Falk, Darin J; Byrne, Barry J; Greer, John J; Fuller, David D

2015-09-01

Pompe disease results from a mutation in the acid α-glucosidase gene leading to lysosomal glycogen accumulation. Respiratory insufficiency is common, and the current U.S. Food and Drug Administration-approved treatment, enzyme replacement, has limited effectiveness. Ampakines are drugs that enhance α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor responses and can increase respiratory motor drive. Recent work indicates that respiratory motor drive can be blunted in Pompe disease, and thus pharmacologic stimulation of breathing may be beneficial. Using a murine Pompe model with the most severe clinical genotype (the Gaa(-/-) mouse), our primary objective was to test the hypothesis that ampakines can stimulate respiratory motor output and increase ventilation. Our second objective was to confirm that neuropathology was present in Pompe mouse medullary respiratory control neurons. The impact of ampakine CX717 on breathing was determined via phrenic and hypoglossal nerve recordings in anesthetized mice and whole-body plethysmography in unanesthetized mice. The medulla was examined using standard histological methods coupled with immunochemical markers of respiratory control neurons. Ampakine CX717 robustly increased phrenic and hypoglossal inspiratory bursting and reduced respiratory cycle variability in anesthetized Pompe mice, and it increased inspiratory tidal volume in unanesthetized Pompe mice. CX717 did not significantly alter these variables in wild-type mice. Medullary respiratory neurons showed extensive histopathology in Pompe mice. Ampakines stimulate respiratory neuromotor output and ventilation in Pompe mice, and therefore they have potential as an adjunctive therapy in Pompe disease.

Alkaptonuria and Pompe disease in one patient: metabolic and molecular analysis.

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Zouheir Habbal, Mohammad; Bou Assi, Tarek; Mansour, Hicham

2013-04-29

Pompe disease is characterised by deficiency of acid α-glucosidase that results in abnormal glycogen deposition in the muscles. Alkaptonuria is caused by a defect in the enzyme homogentisate 1,2-dioxygenase with subsequent accumulation of homogentisic acid. We report the case of a 6-year-old boy diagnosed with Pompe disease and alkaptonuria. Urine organic acids and α-glucosidase were measured. Homogentisate 1,2-dioxygenase (HGO) and acid alpha-glucosidase (GAA) genes were sequenced by Sanger DNA sequencing. The level of α-glucosidase in white blood cells was markedly decreased (4 nm/mg) while the level of homogentisic acid was markedly increased (15 027 mmol/mol creatine). GAA sequencing detected two heterozygous GAA mutations (C.670C>T and C.1064T>C) while HGO sequencing revealed three polymorphisms in exons 4, 5 and 6, respectively. To the best of our knowledge, this is the first reported instance of Pompe disease and alkaptonuria occurring in the same individual.

Enzyme replacement therapy for infantile-onset Pompe disease.

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Chen, Min; Zhang, Lingli; Quan, Shuyan

2017-11-20

Infantile-onset Pompe disease is a rare and progressive autosomal-recessive disorder caused by a deficiency of the lysosomal enzyme acid alpha-glucosidase (GAA). Current treatment involves enzyme replacement therapy (with recombinant human alglucosidase alfa) and symptomatic therapies (e.g. to control secretions). Children who are cross-reactive immunological material (CRIM)-negative require immunomodulation prior to commencing enzyme replacement therapy.Enzyme replacement therapy was developed as the most promising therapeutic approach for Pompe disease; however, the evidence is lacking, especially regarding the optimal dose and dose frequency. To assess the effectiveness, safety and appropriate dose regimen of enzyme replacement therapy for treating infantile-onset Pompe disease. We searched the Cochrane Cystic Fibrosis and Genetic Disorders Group's Inborn Errors of Metabolism Trials Register, which is compiled from electronic database searches and handsearching of journals and conference abstract books. We also searched the Cochrane Central Register of Controlled Trials (CENTRAL), Embase (Ovid), PubMed and LILACS, and CBM, CNKI, VIP, and WANFANG for literature published in Chinese. In addition, we searched three online registers: WHO International Clinical Trials Registry Platform ClinicalTrials.gov, and www.genzymeclinicalresearch.com. We also searched the reference lists of relevant articles and reviews.Date of last search of the Group's Inborn Errors of Metabolism Trials Register: 24 November 2016. Randomized and quasi-randomized controlled trials of enzyme replacement therapy in children with infantile-onset Pompe disease. Two authors independently selected relevant trials, assessed the risk of bias and extracted data. We contacted investigators to obtain important missing information. We found no trials comparing the effectiveness and safety of enzyme replacement therapy to another intervention, no intervention or placebo.We found one trial (18 participants

Cost-effectiveness of enzyme replacement therapy with alglucosidase alfa in classic-infantile patients with Pompe disease

NARCIS (Netherlands)

T.A. Kanters (Tim A.); I Hoogenboom-Plug (Iris); M.P.M.H. Rutten-van Mölken (Maureen); W.K. Redekop (Ken); A.T. van der Ploeg (Ans); L. van Hakkaart-van Roijen (Leona)

2014-01-01

textabstractBackground: Infantile Pompe disease is a rare metabolic disease. Patients generally do not survive the first year of life. Enzyme replacement therapy (ERT) has proven to have substantial effects on survival in infantile Pompe disease. However, the costs of therapy are very high. In this

Screening for late-onset Pompe disease in western Denmark

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Hansen, Julie Schjødtz; Pedersen, E G; Gaist, D

2018-01-01

OBJECTIVE: Late-onset Pompe disease (LOPD) is a rare autosomal recessively inherited metabolic myopathy caused by reduced activity of the lysosomal enzyme alpha-glucosidase. In a previous screening study at two large neuromuscular university clinics in Denmark, three patients with LOPD were...

Elevated Plasma Cardiac Troponin T Levels Caused by Skeletal Muscle Damage in Pompe Disease.

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Wens, Stephan C A; Schaaf, Gerben J; Michels, Michelle; Kruijshaar, Michelle E; van Gestel, Tom J M; In 't Groen, Stijn; Pijnenburg, Joon; Dekkers, Dick H W; Demmers, Jeroen A A; Verdijk, Lex B; Brusse, Esther; van Schaik, Ron H N; van der Ploeg, Ans T; van Doorn, Pieter A; Pijnappel, W W M Pim

2016-02-01

Elevated plasma cardiac troponin T (cTnT) levels in patients with neuromuscular disorders may erroneously lead to the diagnosis of acute myocardial infarction or myocardial injury. In 122 patients with Pompe disease, the relationship between cTnT, cardiac troponin I, creatine kinase (CK), CK-myocardial band levels, and skeletal muscle damage was assessed. ECG and echocardiography were used to evaluate possible cardiac disease. Patients were divided into classic infantile, childhood-onset, and adult-onset patients. cTnT levels were elevated in 82% of patients (median 27 ng/L, normal values normal in all patients, whereas CK-myocardial band levels were increased in 59% of patients. cTnT levels correlated with CK levels in all 3 subgroups (Pmass index measured with echocardiography was normal in all the 3 subgroups. cTnT mRNA expression in skeletal muscle was not detectable in controls but was strongly induced in patients with Pompe disease. cTnT protein was identified by mass spectrometry in patient-derived skeletal muscle tissue. Elevated plasma cTnT levels in patients with Pompe disease are associated with skeletal muscle damage, rather than acute myocardial injury. Increased cTnT levels in Pompe disease and likely other neuromuscular disorders should be interpreted with caution to avoid unnecessary cardiac interventions. © 2016 American Heart Association, Inc.

Survival, Quality of Life and Effects of Enzyme Replacement Therapy in Adults with Pompe Disease

OpenAIRE

Güngör, Deniz

2013-01-01

textabstractPompe disease, or glycogen storage disorder type II, is a rare inherited metabolic disorder caused by deficiency of the lysosomal enzyme acid α-glucosidase. This results in accumulation of glycogen in cells throughout the body, particularly muscle cells. The disease presents with (progressive) muscle weakness and can hence be categorized as a lysosomal storage disorder, a glycogen storage disorder and also a neuromuscular disorder. Pompe disease was the first neuromuscular disorde...

Late-onset Pompe disease with complicated intracranial aneurysm: a Chinese case report

Directory of Open Access Journals (Sweden)

Zhang B

2016-03-01

Full Text Available Bin Zhang,1,2,* Yuying Zhao,1,3,* Junling Liu,1,4 Ling Li,1 Jingli Shan,1 Dandan Zhao,1 Chuanzhu Yan1,3 1Laboratory of Neuromuscular Disorders and Department of Neurology, Qilu Hospital of Shandong University, Jinan, Shandong, 2Department of Neurology, Liaocheng People’s Hospital, Liaocheng, Shandong, 3Department of Neurology, Qilu Hospital of Shandong University, Key Laboratory for Experimental Teratology of the Ministry of Education, Brain Science Research Institute, Shandong University, Jinan, Shandong, 4Department of Neurology, Affiliated Hospital of Weifang Medical University, Weifang, Shandong, People’s Republic of China *These authors contributed equally to this work Abstract: Pompe disease is a rare autosomal recessive hereditary disease caused by genetic defects of acid maltase. This disease could be divided into two forms: infantile and late-onset, which mainly affect cardiac, respiratory, and skeletal muscle systems. Late-onset patients mainly show symptoms of skeletal muscle involvement, but recent reports have found that the central nervous system was also affected in some patients. Herein, we report a case of a female, adolescent-onset Pompe patient, who was diagnosed with complicated intracranial aneurysm in adulthood. Keywords: Pompe disease, glycogen storage disease II, acid maltase, acid alpha-glucosidase, cerebrovascular disorders

Skeletal muscle metabolism during prolonged exercise in Pompe disease

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Preisler, Nicolai; Laforêt, Pascal; Madsen, Karen Lindhardt

2017-01-01

of exercise, it is important in Pompe disease to acquire more information about muscle substrate use during exercise. METHODS: Seven adults with Pompe disease were matched to a healthy control group (1:1). We determined (1) peak oxidative capacity (VO2peak) and (2) carbohydrate and fatty acid metabolism...... during submaximal exercise (33 W) for 1 h, using cycle-ergometer exercise, indirect calorimetry and stable isotopes. RESULTS: In the patients, VO2peak was less than half of average control values; mean difference -1659 mL/min (CI: -2450 to -867, P = 0.001). However, the respiratory exchange ratio...... increased to >1.0 and lactate levels rose 5-fold in the patients, indicating significant glycolytic flux. In line with this, during submaximal exercise, the rates of oxidation (ROX) of carbohydrates and palmitate were similar between patients and controls (mean difference 0.226 g/min (CI: 0.611 to -0.078, P...

Use of cardiac magnetic resonance imaging to evaluate cardiac structure, function and fibrosis in children with infantile Pompe disease on enzyme replacement therapy.

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Barker, Piers C A; Pasquali, Sara K; Darty, Stephen; Ing, Richard J; Li, Jennifer S; Kim, Raymond J; DeArmey, Stephanie; Kishnani, Priya S; Campbell, Michael J

2010-12-01

/m(2) (range 43.8-334) vs. CMR median at most recent study 44.5 g/m(2) (range 34-303), p=0.44). In 5 patients with serial CMR scans over time, LVMI decreased in 2, was similar in 2, and increased in 1 patient with high sustained antibodies to exogenous enzyme. Delayed enhancement was noted in only l separate patient who also had high sustained antibodies to exogenous enzyme. CMR is an imaging tool that is feasible to use to serially follow LVMI and EF in children with Pompe disease on ERT. Real-time imaging is adequate for quantification purposes in these patients and minimizes the need for sedation. Quantitative CMR LVMI is generally lower than echo derived LVMI. Delayed enhancement appears to be a rare finding by CMR in Pompe disease. A further follow-up is necessary to better understand the long term effects of ERT in infantile Pompe survivors, especially those with high sustained antibody titers or advanced cardiac disease at treatment outset. Copyright © 2010 Elsevier Inc. All rights reserved.

Late onset Pompe disease- new genetic variant: Case report ...

African Journals Online (AJOL)

The patient was not given enzyme replacement therapy due to cost but received high protein therapy and Oxygen supplementation using Oxygen extractor machine. She is worsening due to respiratory failure. Conclusion: This is a new genetic variant isolated of late-onset Pompe disease which presents with almost pure ...

Quantification of diaphragm mechanics in Pompe disease using dynamic 3D MRI

DEFF Research Database (Denmark)

Mogalle, Katja; Perez-Rovira, Adria; Ciet, Pierluigi

2016-01-01

BACKGROUND: Diaphragm weakness is the main reason for respiratory dysfunction in patients with Pompe disease, a progressive metabolic myopathy affecting respiratory and limb-girdle muscles. Since respiratory failure is the major cause of death among adult patients, early identification of respira......BACKGROUND: Diaphragm weakness is the main reason for respiratory dysfunction in patients with Pompe disease, a progressive metabolic myopathy affecting respiratory and limb-girdle muscles. Since respiratory failure is the major cause of death among adult patients, early identification...... methodology relies on image registration and lung surface extraction to quantify lung kinematics during breathing. This allows for the extraction of geometry and motion features of the lung that characterize the independent contribution of the diaphragm and the thoracic muscles to the respiratory cycle....... RESULTS: Results in 16 3D+t MRI scans (10 Pompe patients and 6 controls) of a slow expiratory maneuver show that kinematic analysis from dynamic 3D images reveals important additional information about diaphragm mechanics and respiratory muscle involvement when compared to conventional pulmonary function...

Generalized glycogen storage and cardiomegaly in a knockout mouse model of Pompe disease

NARCIS (Netherlands)

A.G.A. Bijvoet (Agnes); A.T. van der Ploeg (Ans); E.H. van de Kamp; M.A. Kroos (Marian); J.-H. Ding (Jia-Huan); B.Z. Yang (Bing); P. Visser (Pim); C.E. Bakker (Cathy); M.Ph. Verbeet (Martin); B.A. Oostra (Ben); A.J.J. Reuser (Arnold)

1998-01-01

textabstractGlycogen storage disease type II (GSDII; Pompe disease), caused by inherited deficiency of acid alpha-glucosidase, is a lysosomal disorder affecting heart and skeletal muscles. A mouse model of this disease was obtained by targeted disruption of the

Assessment of toxicity and biodistribution of recombinant AAV8 vector–mediated immunomodulatory gene therapy in mice with Pompe disease

Directory of Open Access Journals (Sweden)

Gensheng Wang

2014-01-01

Full Text Available A preclinical safety study was conducted to evaluate the short- and long-term toxicity of a recombinant adeno-associated virus serotype 8 (AAV2/8 vector that has been developed as an immune-modulatory adjunctive therapy to recombinant human acid α-glucosidase (rhGAA, Myozyme enzyme replacement treatment (ERT for patients with Pompe disease (AAV2/8-LSPhGAApA. The AAV2/8-LSPhGAApA vector at 1.6 × 1013 vector particles/kg, after intravenous injection, did not cause significant short- or long-term toxicity. Recruitment of CD4+ (but not CD8+ lymphocytes to the liver was elevated in the vector-dosed male animals at study day (SD 15, and in group 8 animals at SD 113, in comparison to their respective control animals. Administration of the vector, either prior to or after the one ERT injection, uniformly prevented the hypersensitivity induced by subsequent ERT in males, but not always in female animals. The vector genome was sustained in all tissues through 16-week postdosing, except for in blood with a similar tissue tropism between males and females. Administration of the vector alone, or combined with the ERT, was effective in producing significantly increased GAA activity and consequently decreased glycogen accumulation in multiple tissues, and the urine biomarker, Glc4, was significantly reduced. The efficacy of the vector (or with ERT was better in males than in females, as demonstrated both by the number of tissues showing significantly effective responses and the extent of response in a given tissue. Given the lack of toxicity for AAV2/8LSPhGAApA, further consideration of clinical translation is warranted in Pompe disease.

[Tetra-saccharide glucose as a diagnostic biomarker for Pompe disease: a study with 35 patients].

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Bobillo Lobato, Joaquín; Durán Parejo, Pilar; Tejero Díez, Pedro; Jiménez Jiménez, Luis M

2013-08-04

Pompe disease is a disorder originating from an acid alpha-glycosidase (AAG) enzyme deficiency. This disease produces an accumulation of lysosomal glycogen in different tissues, whereby the skeletal and heart muscles are especially involved. The established diagnosis is achieved through the identification of the AAG deficiency. There are also other secondary diagnostic biomarkers, such as tetra-saccharide glucose (Glc4), which shows high levels in the urine of these patients. In this study it is highlighted the usefulness of Glc4 as a diagnostic biomarker for Pompe disease in its different forms of presentation, using a high-performance liquid chromatography with ultraviolet detection (HPLC/UV) adapted to the study. A total of 75 individuals have been analyzed: 40 healthy controls and 35 patients diagnosed with Pompe disease. Twenty-four hour samples of urine were collected from all of the patients and their Glc4 levels were determined by means of HPLC/UV. The evaluation of the urinary Glc4 shows a high discrimination ability between healthy/sick individuals. In addition, the results obtained have allowed to establish the most appropriate level of decision or cut-off point for the identification of sick people. Glc4 urinary levels are found to be high in patients suffering from Pompe disease and even though increased levels are also found in other conditions, the existence of a AAG deficiency together with a compatible clinical symptoms, prove very helpful for a correct diagnosis of this serious disease. Copyright © 2012 Elsevier España, S.L. All rights reserved.

Whole-body muscle MRI in 20 patients suffering from late onset Pompe disease: Involvement patterns.

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Carlier, Robert-Yves; Laforet, Pascal; Wary, Claire; Mompoint, Dominique; Laloui, Kenza; Pellegrini, Nadine; Annane, Djillali; Carlier, Pierre G; Orlikowski, David

2011-11-01

To describe muscle involvement on whole-body MRI scans in adult patients at different stages of late-onset Pompe disease. Twenty patients aged 37 to 75 were examined. Five were bedridden and required ventilatory support. Axial and coronal T1 turbo-spin-echo sequences were performed on 1.5T or 3T systems. MRI was scored for 47 muscles using Mercuri's classification. Whole-body scans were obtained with a mean in-room time of 29 min. Muscle changes consisted of internal bright signals of fatty replacement without severe retraction of the muscles' corpus. Findings were consistent with previous descriptions of spine extensors and pelvic girdle, but also provided new information on recurrent muscle changes particularly in the tongue and subscapularis muscle. Moreover thigh involvement was more heterogeneous than previously described, in terms of distribution across muscles as well as with respect to the overall clinical presentation. Whole-body MRI provides a very evocative description of muscle involvement in Pompe disease in adults. Copyright © 2011 Elsevier B.V. All rights reserved.

Quantification of Diaphragm Mechanics in Pompe Disease Using Dynamic 3D MRI.

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Katja Mogalle

Full Text Available Diaphragm weakness is the main reason for respiratory dysfunction in patients with Pompe disease, a progressive metabolic myopathy affecting respiratory and limb-girdle muscles. Since respiratory failure is the major cause of death among adult patients, early identification of respiratory muscle involvement is necessary to initiate treatment in time and possibly prevent irreversible damage. In this paper we investigate the suitability of dynamic MR imaging in combination with state-of-the-art image analysis methods to assess respiratory muscle weakness.The proposed methodology relies on image registration and lung surface extraction to quantify lung kinematics during breathing. This allows for the extraction of geometry and motion features of the lung that characterize the independent contribution of the diaphragm and the thoracic muscles to the respiratory cycle.Results in 16 3D+t MRI scans (10 Pompe patients and 6 controls of a slow expiratory maneuver show that kinematic analysis from dynamic 3D images reveals important additional information about diaphragm mechanics and respiratory muscle involvement when compared to conventional pulmonary function tests. Pompe patients with severely reduced pulmonary function showed severe diaphragm weakness presented by minimal motion of the diaphragm. In patients with moderately reduced pulmonary function, cranial displacement of posterior diaphragm parts was reduced and the diaphragm dome was oriented more horizontally at full inspiration compared to healthy controls.Dynamic 3D MRI provides data for analyzing the contribution of both diaphragm and thoracic muscles independently. The proposed image analysis method has the potential to detect less severe diaphragm weakness and could thus be used to determine the optimal start of treatment in adult patients with Pompe disease in prospect of increased treatment response.

Antibody-mediated enzyme replacement therapy targeting both lysosomal and cytoplasmic glycogen in Pompe disease.

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Yi, Haiqing; Sun, Tao; Armstrong, Dustin; Borneman, Scott; Yang, Chunyu; Austin, Stephanie; Kishnani, Priya S; Sun, Baodong

2017-05-01

Pompe disease is characterized by accumulation of both lysosomal and cytoplasmic glycogen primarily in skeletal and cardiac muscles. Mannose-6-phosphate receptor-mediated enzyme replacement therapy (ERT) with recombinant human acid α-glucosidase (rhGAA) targets the enzyme to lysosomes and thus is unable to digest cytoplasmic glycogen. Studies have shown that anti-DNA antibody 3E10 penetrates living cells and delivers "cargo" proteins to the cytosol or nucleus via equilibrative nucleoside transporter ENT2. We speculate that 3E10-mediated ERT with GAA will target both lysosomal and cytoplasmic glycogen in Pompe disease. A fusion protein (FabGAA) containing a humanized Fab fragment derived from the murine 3E10 antibody and the 110 kDa human GAA precursor was constructed and produced in CHO cells. Immunostaining with an anti-Fab antibody revealed that the Fab signals did not co-localize with the lysosomal marker LAMP2 in cultured L6 myoblasts or Pompe patient fibroblasts after incubation with FabGAA. Western blot with an anti-GAA antibody showed presence of the 150 kDa full-length FabGAA in the cell lysates, in addition to the 95- and 76 kDa processed forms of GAA that were also seen in the rhGAA-treated cells. Blocking of mannose-6-phosphate receptor with mannose-6-phosphate markedly reduced the 95- and the 76 kDa forms but not the 150 kDa form. In GAA-KO mice, FabGAA achieved similar treatment efficacy as rhGAA at an equal molar dose in reducing tissue glycogen contents. Our data suggest that FabGAA retains the ability of rhGAA to treat lysosomal glycogen accumulation and has the beneficial potential over rhGAA to reduce cytoplasmic glycogen storage in Pompe disease. FabGAA can be delivered to both the cytoplasm and lysosomes in cultured cells. FabGAA equally reduced lysosomal glycogen accumulation as rhGAA in GAA-KO mice. FabGAA has the beneficial potential over rhGAA to clear cytoplasmic glycogen. This study suggests a novel antibody-enzyme fusion protein therapy

Laboratory studies and Pompe disease: from suspicion to therapy monitoring

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K. V. Savost’yanov

2016-01-01

Full Text Available Pompe disease (PD is a rare, progressive, commonly fatal inherited autosomal recessive disease that is difficult to diagnose due to its obvious clinical heterogeneity and low awareness among physicians. Access to the laboratory diagnosis of rare diseases increases every year. In the past several years, Russian and foreign laboratories have achieved considerable success in accelerating and improving the diagnostic accuracy of PD. Unfortunately, the Russian-language literature contains scarce relevant information on the laboratory diagnosis of PD. This review is to fill up this gap. 

Clinical and biochemical observations in a patient with combined Pompe disease and cblC mutation

NARCIS (Netherlands)

Wijburg, F. A.; Rosenblatt, D. S.; Vos, G. D.; Oorthuys, J. W.; van't Hek, L. G.; Poorthuis, B. J.; Sanders, M. K.; Schutgens, R. B.

1992-01-01

Metabolic studies are described in a patient who presented at 3 weeks of age with severe anaemia, hyperbilirubinaemia and hypotonicity. Clinically, glycogen storage disease type II (Pompe disease) was suspected because of a massively enlarged heart and hepatosplenomegaly. This was confirmed

Survival, Quality of Life and Effects of Enzyme Replacement Therapy in Adults with Pompe Disease

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D. Güngör (Deniz)

2013-01-01

textabstractPompe disease, or glycogen storage disorder type II, is a rare inherited metabolic disorder caused by deficiency of the lysosomal enzyme acid α-glucosidase. This results in accumulation of glycogen in cells throughout the body, particularly muscle cells. The disease presents

Fatigue in neuromuscular disorders: Focus on Guillain-Barré syndrome and Pompe disease

NARCIS (Netherlands)

J.M. de Vries (Juna); M.L.C. Hagemans (Marloes); J.B.J. Bussmann (Hans); A.T. van der Ploeg (Ans); P.A. van Doorn (Pieter)

2010-01-01

textabstractFatigue accounts for an important part of the burden experienced by patients with neuromuscular disorders. Substantial high prevalence rates of fatigue are reported in a wide range of neuromuscular disorders, such as Guillain-Barré syndrome and Pompe disease. Fatigue can be subdivided

Prevalence of adult Pompe disease in patients with proximal myopathic syndrome and undiagnosed muscle biopsy.

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Golsari, Amir; Nasimzadah, Arzoo; Thomalla, Götz; Keller, Sarah; Gerloff, Christian; Magnus, Tim

2018-03-01

We examined patients with limb-girdle muscle weakness and/or hyper-CKaemia and undiagnosed muscle biopsy for late onset Pompe disease (LOPD). Patients with an inconclusive limb-girdle muscle weakness who presented at our neuromuscular centre between 2005 and 2015 with undiagnosed muscle biopsies were examined by dry blood spot testing (DBS) including determination of the enzyme activity of acid alpha-glucosidase (GAA). In the case of depressed enzyme activity, additional gene testing of the GAA gene was carried out. Of the 340 evaluated muscle biopsies, 69 patients fulfilled the inclusion criteria and were examined with DBS. Among those patients, 76% showed a limb-girdle muscle weakness and 14% showed a hyper-CKaemia. A diagnosis of LOPD could be established in the case of two patients (2.9%) with reduced GAA enzyme activity and proof of mutations in the GAA gene. One of the two patients presents in the muscle biopsy suggestive features of Pompe disease including vacuoles with positive acid phosphatase reaction. In summary, our results show that a muscle biopsy can be helpful in identifying LOPD patients, but vacuolation with glycogen storage can also be absent. An inconspicuous muscle biopsy does not rule out Pompe disease. Consequently, all patients with limb-girdle muscle weakness should be examined by DBS before conducting a muscle biopsy. Copyright © 2017 Elsevier B.V. All rights reserved.

The natural course of infantile Pompe's disease : 20 original cases compared with 133 cases from the literature

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van den Hout, HMP; Hop, W; van Diggelen, OP; Smeitink, JAM; Smit, GPA; Poll-The, BTT; Bakker, HD; Loonen, MCB; de Klerk, JBC; Reuser, AJJ; van der Ploeg, AT

Objective. Infantile Pompe's disease is a lethal cardiac and muscular disorder. Current developments toward enzyme replacement therapy are promising. The aim of our study is to delineate the natural course of the disease to verify endpoints of clinical studies. Methods. A total of 20 infantile

Divergent clinical outcomes of alpha-glucosidase enzyme replacement therapy in two siblings with infantile-onset Pompe disease treated in the symptomatic or pre-symptomatic state

OpenAIRE

Matsuoka, Takashi; Miwa, Yoshiyuki; Tajika, Makiko; Sawada, Madoka; Fujimaki, Koichiro; Soga, Takashi; Tomita, Hideshi; Uemura, Shigeru; Nishino, Ichizo; Fukuda, Tokiko; Sugie, Hideo; Kosuga, Motomichi; Okuyama, Torayuki; Umeda, Yoh

2016-01-01

Pompe disease is an autosomal recessive, lysosomal glycogen storage disease caused by acid ?-glucosidase deficiency. Infantile-onset Pompe disease (IOPD) is the most severe form and is characterized by cardiomyopathy, respiratory distress, hepatomegaly, and skeletal muscle weakness. Untreated, IOPD generally results in death within the first year of life. Enzyme replacement therapy (ERT) with recombinant human acid alpha glucosidase (rhGAA) has been shown to markedly improve the life expectan...

Late-onset Pompe disease: first clinical description in Russia

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S. S. Nikitin

2014-01-01

Full Text Available Late-onset Pompe-disease (LOPD is an adult form of the glycogenosis type II. The age of onset ranges from 1 till 75 y.o. and older. The diagnosis of LOPD is based on the presence of trunk and limb-girdle muscle weakness with hyperlordosis, respiratory failure, ocasionally accompanied by cardiomyopathy, persistent mild elevation of creatine kinase, dry blood spot test of the enzyme activity and DNA-analysis of GAA-gene. Early recognition of the LOPD and beginning of the enzyme replacement therapy is important in preventing severe motor and respiratory deficit, the patient disability and in increasing the survival in those patients.

Safety and efficacy of exercise training in adults with Pompe disease: Evalution of endurance, muscle strength and core stability before and after a 12 week training program

NARCIS (Netherlands)

L.E.M. van den Berg (Linda); M.M. Favejee (Marein); S.C.A. Wens (Stephan); M.E. Kruijshaar (Michelle); S.F.E. Praet (Stephan); A.J.J. Reuser (Arnold); J.B.J. Bussmann (Hans); P.A. van Doorn (Pieter); A.T. van der Ploeg (Ans)

2015-01-01

textabstractBackground: Pompe disease is a proximal myopathy. We investigated whether exercise training is a safe and useful adjuvant therapy for adult Pompe patients, receiving enzyme replacement therapy. Methods: Training comprised 36 sessions of standardized aerobic, resistance and core stability

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.)

Motor function and respiratory capacity in patients with late-onset pompe disease

DEFF Research Database (Denmark)

Illes, Zsolt; Mike, Andrea; Trauninger, Anita

2014-01-01

Introduction: The relationship between skeletal muscle strength and respiratory dysfunction in Pompe disease has not been examined by quantitative methods. We investigated correlations among lower extremity proximal muscle strength, respiratory function, and motor performance. Methods: Concentric...... strength of the knee extensor and flexor muscles were measured with a dynamometer, and pulmonary function was evaluated using spirometry in 7 adult patients. The six-minute walk test and the four-step stair-climb test were used for assessing aerobic endurance and anaerobic power, respectively. Results......: Anaerobic motor performance correlated with strength of both thigh muscles. Respiratory function did not correlate with either muscle strength or motor function performance. Conclusions: Respiratory and lower extremity proximal muscles could be differentially affected by the disease in individual patients...

Aspectos respiratórios da doença de Pompe: Relato de caso Respiratory aspects of Pompe disease: Case report

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Ewandro LR Moura

2008-02-01

Full Text Available Relatamos o caso de doente feminina, 38 anos, com diagnóstico de doença de Pompe há oito anos. Há cinco anos vinha com queixas respiratórias, evoluindo com insuficiência respiratória crónica. Em uso de CPAP (continuous positive airway pressure nocturno há um ano, apresentava agravamento clínico progressivo. Veio encaminhada ao Hospital Universitário de Brasília, com descompensação respiratória e cor pulmonale. Iniciou-se ventilação não invasiva com dois níveis de pressões (VNI e realizou-se polissonografia, que demonstrou a necessidade do ajuste de frequência respiratória associada aos ajustes das pressões para adequação da ventilação durante o sono. Evoluiu com melhora importante após uso da VNI.We report the case of a 38 years-old female patient with Pompe disease, diagnosed eight years ago. Respiratory complaints appeared five years ago progressing to chronic respiratory failure. Nocturnal CPAP (continuous positive airway pressure was prescribed last year, presenting progressive clinical worsening. She was referred to Hospital Universitário de Brasília, on account of respiratory failure and cor pulmonale. Thus, she began non-invasive ventilation (NIV with bi-level positive airways pressure, and a polysomnography showed the need of spontaneous/timed mode associated with adjustments in breathing pressures to improve ventilation during sleep. There was significant clinical improvement after NIV support was established.

Adult onset glycogen storage disease type II (adult onset Pompe disease): report and magnetic resonance images of two cases

International Nuclear Information System (INIS)

Del Gaizo, Andrew; Banerjee, Sima; Terk, Michael

2009-01-01

Glycogen storage disease type II (GSDII), also referred to as Pompe disease or acid maltase deficiency, is a rare inherited condition caused by a deficiency in acid alpha-glucosidase (GAA) enzyme activity. The condition is often classified by age of presentation, with infantile and late onset variants (Laforet et al. J Neurology 55:1122-8, 2000). Late onset tends to present with progressive proximal muscle weakness and respiratory insufficiency (Winkel et al. J Neurology 252:875-84, 2005). We report two cases of biopsy confirmed adult onset GSDII, along with key Magnetic Resonance (MR) images. (orig.)

Retrospective, Single Center Study of Clinical, Paraclinical and Natural Course of Infantile-Onset Pompe Disease

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Noormohammad Noori

2015-01-01

Full Text Available Background: Infantile-onset Pompe disease is a rare genetic and lethal disorder which is caused by the lack of acid alpha-glucosidase activity (GAA. The aim of our study was to identify the demographic and clinical characteristics, and natural history of these patients. Materials and Methods: In this retrospective study, clinical file of 15 patients diagnosed with infantile-onset Pompe disease whose symptoms started before the age of 12 months were studied. Diagnosis was based on clinical history, physical examination and diagnostic parameters in chest X-ray, echocardiogram, electrocardiogram and biochemical tests after rule out the other metabolic and neuromuscular disorders. Results: Sixty percent of the patients were male and 40% were female. The mean age at the onset of symptoms was 78 days (range: 3-150 days. Most frequent clinical and paraclinical symptoms were cardiomegaly, hypotonia, hyporeflexia, macroglossia, failure to thrive, hepatomegaly, and feeding problems, respectively. The mean age at the time of death was 5.96 months (range: 4-8 months, and all patients died before one year of age. Muscle enzymes including AST, ALT, LDH, and CPK were elevated in all patients. Due to the lack of availability, enzyme replacement therapy was not possible for any patient. Conclusion: The study showed that despite the supportive measures and no specific treatment, the clinical course is not significantly different with similar studies and the overall prognosis of this form of disease is very poor and disappointing.

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

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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.

Oral delivery of Acid Alpha Glucosidase epitopes expressed in plant chloroplasts suppresses antibody formation in treatment of Pompe mice.

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Su, Jin; Sherman, Alexandra; Doerfler, Phillip A; Byrne, Barry J; Herzog, Roland W; Daniell, Henry

2015-10-01

Deficiency of acid alpha glucosidase (GAA) causes Pompe disease in which the patients systemically accumulate lysosomal glycogen in muscles and nervous systems, often resulting in infant mortality. Although enzyme replacement therapy (ERT) is effective in treating patients with Pompe disease, formation of antibodies against rhGAA complicates treatment. In this report, we investigated induction of tolerance by oral administration of GAA expressed in chloroplasts. Because full-length GAA could not be expressed, N-terminal 410-amino acids of GAA (as determined by T-cell epitope mapping) were fused with the transmucosal carrier CTB. Tobacco transplastomic lines expressing CTB-GAA were generated through site-specific integration of transgenes into the chloroplast genome. Homoplasmic lines were confirmed by Southern blot analysis. Despite low-level expression of CTB-GAA in chloroplasts, yellow or albino phenotype of transplastomic lines was observed due to binding of GAA to a chloroplast protein that has homology to mannose-6 phosphate receptor. Oral administration of the plant-made CTB-GAA fusion protein even at 330-fold lower dose (1.5 μg) significantly suppressed immunoglobulin formation against GAA in Pompe mice injected with 500 μg rhGAA per dose, with several-fold lower titre of GAA-specific IgG1 and IgG2a. Lyophilization increased CTB-GAA concentration by 30-fold (up to 190 μg per g of freeze-dried leaf material), facilitating long-term storage at room temperature and higher dosage in future investigations. This study provides the first evidence that oral delivery of plant cells is effective in reducing antibody responses in ERT for lysosomal storage disorders facilitating further advances in clinical investigations using plant cell culture system or in vitro propagation. © 2015 Society for Experimental Biology, Association of Applied Biologists and John Wiley & Sons Ltd.

Revisão sistemática das escalas utilizadas para avaliação funcional na doença de Pompe Revisión sistemática de las escalas utilizadas para evaluación funcional en la enfermedad de Pompe Systematic review of functional assessment scales in Pompe disease

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Alana Karla Savegnago

2012-06-01

y 2010. Las palabras clave utilizadas en los idiomas portugués e inglés fueron: enfermedad de depósito de glucógeno tipo II, actividades cotidianas, evaluación. Los artículos fueron clasificados en nivel de evidencia y recomendación según Cook et al. SÍNTESIS DE LOS DATOS: Se incluyeron 14 estudios que evaluaron desde recién-nacidos a adultos (muestra total=449. Se encontraron las siguientes escalas en la literatura: Pediatric Evaluation of Disability Inventory (PEDI y su forma adaptada para 1DP (Pompe-PEDI, Alberta Infant Motor Scale (AIMS, Rotterdam Handiscap Scale (RHS, Functional Independence Measure (FIM, Gross Motor Function Measure (GMFM y Peabody Developmental Motor Scales (PDMS-II. La mayoría de los estudios presentó nivel de evidencia III, por tratarse de estudios no randomizados. Grado de recomendación de las escalas: C para AIMS y Pompe-PEDI; D para GMFM y PDMS-II; E para RHS y FIM. CONCLUSIONES: La mayoría de las escalas utilizadas para evaluación funcional en la EP presenta bajo nivel de evidencia y recomendación. Las que presentan mejor grado de recomendación (C son las escalas AIMS y Pompe-PEDI aplicadas en Pediatría.OBJECTIVE: To identify functional assessment scales used in Pompe disease (PD and to describe their levels of evidence and grades of recommendation. DATA SOURCE: Systematic review of the functional assessment scales used in PD. Review conducted in the databases Medline, Lilacs, Cochrane Central Register of Controlled Trials (CCTR, and SciELO including articles (except review articles published between 2000 and 2010. The key-words used in Portuguese and English were: glycogen storage disease type II, activities of daily living, assessment. The articles were classified according to their level of evidence and grade of recommendations. DATA SYNTHESIS: 14 studies assessing patients ranging from newborns to adults were included in the present review (total sample=449. The scales found in the literature were: Pediatric

Homozygotic intronic GAA mutation in three siblings with late-onset Pompe's disease Mutação homozigótica intrônica no gene GAA em três irmãos com doença de Pompe de início tardio

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Anderson Kuntz Grzesiuk

2010-04-01

Full Text Available Pompe's disease (PD is a metabolic myopathy caused by the accumulation of lysosomal glycogen, secondary to acid α-glucosidase (GAA enzyme deficiency. Childhood and late-onset forms are described, differing by the age of onset and symptoms. In this study were analyzed affected siblings with Pompe's disease (PD and their distinct clinical and pathological presentations. METHOD: Diagnosis was performed by the clinical presentation of limb-girdle dystrophies and respiratory compromise. Confirmatory diagnoses were conducted by muscle biopsy, GAA activity measurement and by GAA gene genotyping. RESULTS: The findings suggested muscular involvement due to GAA deficiency. GAA genotyping showed they are homozygous for the c.-32-3C>A mutation. CONCLUSION: Herein we reported a family where three out of five siblings were diagnosed with late-onset PD, although it is a rare metabolic disease inherited in an autossomal recessive manner. We emphasize the importance of including this presentation within the differential diagnoses of the limb-girdle dystrophies once enzyme replacement therapy is available.A doença de Pompe (DP é uma miopatia originada do acúmulo lisossomal de glicogênio, devido à deficiência da enzima α-glicosidase ácida (GAA, sendo descritas formas de inicio precoce e tardio. Neste estudo analisamos retrospectivamente o perfil clinico e patológico de 3 irmãos portadores de doença de Pompe de inicio tardio. MÉTODO: O diagnóstico foi realizado mediante apresentação clinica de distrofia de cinturas associado a comprometimento respiratório, sendo confirmado por biópsia muscular e análise da atividade e genotipagem da GAA. RESULTADOS: Os exames clínicos e laboratoriais demonstram envolvimento muscular devido à deficiência da GAA, com uma mutação c.-32-3C>A em homozigose. CONCLUSÃO: Relatamos os aspectos clínicos e laboratoriais de 3 irmãos afetados por doença de Pompe de início tardio. Enfatizamos a importância de

Prospective exploratory muscle biopsy, imaging, and functional assessment in patients with late-onset Pompe disease treated with alglucosidase alfa: The EMBASSY Study

NARCIS (Netherlands)

A.T. van der Ploeg (Ans); Carlier, P.G. (Pierre G.); Carlier, R.-Y. (Robert-Yves); Kissel, J.T. (John T.); B. Schoser; Wenninger, S. (Stephan); Pestronk, A. (Alan); Barohn, R.J. (Richard J.); Dimachkie, M.M. (Mazen M.); Goker-Alpan, O. (Ozlem); Mozaffar, T. (Tahseen); Pena, L.D.M. (Loren D.M.); Simmons, Z. (Zachary); V. Straub; Guglieri, M. (Michela); Young, P. (Peter); Boentert, M. (Matthias); Baudin, P.-Y. (Pierre-Yves); S.C.A. Wens (Stephan); Shafi, R. (Raheel); Bjartmar, C. (Carl); B. Thurberg

2016-01-01

textabstractBackground Late-onset Pompe disease is characterized by progressive skeletal myopathy followed by respiratory muscle weakness, typically leading to loss of ambulation and respiratory failure. In this population, enzyme replacement therapy (ERT) with alglucosidase alfa has been shown to

Respiratory muscle training with enzyme replacement therapy improves muscle strength in late - onset Pompe disease.

Science.gov (United States)

Jevnikar, Mitja; Kodric, Metka; Cantarutti, Fabiana; Cifaldi, Rossella; Longo, Cinzia; Della Porta, Rossana; Bembi, Bruno; Confalonieri, Marco

2015-12-01

Pompe disease is an autosomal recessive metabolic disorder caused by the deficiency of the lysosomal enzyme acid α-glucosidase. This deficiency leads to glycogen accumulation in the lysosomes of muscle tissue causing progressive muscular weakness particularly of the respiratory system. Enzyme replacement therapy (ERT) has demonstrated efficacy in slowing down disease progression in infants. Despite the large number of studies describing the effects of physical training in juvenile and adult late onset Pompe disease (LOPD). There are very few reports that analyze the benefits of respiratory muscle rehabilitation or training. The effectiveness of respiratory muscle training was investigated using a specific appliance with adjustable resistance (Threshold). The primary endpoint was effect on respiratory muscular strength by measurements of MIP and MEP. Eight late-onset Pompe patients (aged 13 to 58 years; 4 female, 4 male) with respiratory muscle deficiency on functional respiratory tests were studied. All patients received ERT at the dosage of 20 mg/kg/every 2 weeks and underwent training with Threshold at specified pressures for 24 months. A significant increase in MIP was observed during the follow-up of 24 month: 39.6 cm H 2 O (+ 25.0%) at month 3; 39.5 cm H 2 O (+ 24.9%) at month 6; 39.1 cm H 2 O (+ 23.7%) at month 9; 37.3 cm H 2 O (+ 18.2%) at month 12; and 37.3 cm H 2 O (+ 17.8%) at month 24. Median MEP values also showed a significant increase during the first 9 months: 29.8 cm H 2 O, (+ 14.3%) at month 3; 31.0 cm H 2 O (+ 18.6) at month 6; and 29.5 cm H 2 O (+ 12.9) at month 9. MEP was then shown to be decreased at months 12 and 24; median MEP was 27.2 cm H 2 O (+ 4.3%) at 12 months and 26.6 cm H 2 O (+ 1.9%) at 24 months. The FVC remain stable throughout the study. An increase in respiratory muscular strength was demonstrated with Threshold training when used in combination with ERT.

Divergent clinical outcomes of alpha-glucosidase enzyme replacement therapy in two siblings with infantile-onset Pompe disease treated in the symptomatic or pre-symptomatic state.

Science.gov (United States)

Matsuoka, Takashi; Miwa, Yoshiyuki; Tajika, Makiko; Sawada, Madoka; Fujimaki, Koichiro; Soga, Takashi; Tomita, Hideshi; Uemura, Shigeru; Nishino, Ichizo; Fukuda, Tokiko; Sugie, Hideo; Kosuga, Motomichi; Okuyama, Torayuki; Umeda, Yoh

2016-12-01

Pompe disease is an autosomal recessive, lysosomal glycogen storage disease caused by acid α-glucosidase deficiency. Infantile-onset Pompe disease (IOPD) is the most severe form and is characterized by cardiomyopathy, respiratory distress, hepatomegaly, and skeletal muscle weakness. Untreated, IOPD generally results in death within the first year of life. Enzyme replacement therapy (ERT) with recombinant human acid alpha glucosidase (rhGAA) has been shown to markedly improve the life expectancy of patients with IOPD. However, the efficacy of ERT in patients with IOPD is affected by the presence of symptoms and cross-reactive immunologic material (CRIM) status. We have treated two siblings with IOPD with ERT at different ages: the first was symptomatic and the second was asymptomatic. The female proband (Patient 1) was diagnosed with IOPD and initiated ERT at 4 months of age. Her younger sister (Patient 2) was diagnosed with IOPD at 10 days of age and initiated ERT at Day 12. Patient 1, now 6 years old, is alive but bedridden, and requires 24-hour invasive ventilation due to gradually progressive muscle weakness. In Patient 2, typical symptoms of IOPD, including cardiac failure, respiratory distress, progressive muscle weakness, hepatomegaly and myopathic facial features were largely absent during the first 12 months of ERT. Her cardiac function and mobility were well-maintained for the first 3 years, and she had normal motor development. However, she developed progressive hearing impairment and muscle weakness after 3 years of ERT. Both siblings have had low anti-rhGAA immunoglobulin G (IgG) antibody titers during ERT and have tolerated the treatment well. These results suggest that initiation of ERT during the pre-symptomatic period can prevent and/or attenuate the progression of IOPD, including cardiomyopathy, respiratory distress, and muscle weakness for first several years of ERT. However, to improve the long-term efficacy of ERT for IOPD, new strategies

The phenotype, genotype, and outcome of infantile-onset Pompe disease in 18 Saudi patients

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Zuhair N. Al-Hassnan

2018-06-01

Full Text Available Infantile-Onset Pompe Disease (IOPD is an autosomal recessive disorder of glycogen metabolism resulting from deficiency of the lysosomal hydrolase acid α-glucosidase encoded by GAA gene. Affected infants present before the age of 12 months with hypotonia, muscle weakness, and hypertrophic cardiomyopathy. Enzyme replacement therapy (ERT has been shown to improve survival, cardiac mass, and motor skills. In this work, we aim to illustrate the genotypes of IOPD and the outcome of ERT in our population. The medical records of infants with confirmed diagnosis of IOPD who received ERT were reviewed. Eighteen infants (7 males, 11 females were included in the study. The median age at presentation was 2 months and the median age at the start of ERT was 4.5 months. Fifteen (83.3% infants died with a median age at death of 12 months. The 3 alive infants (whose current ages are 6½ years, 6 years, and 10 years, who were initiated on ERT at the age of 3 weeks, 5 months, and 8 months respectively, has had variable response with requirement of assisted ventilation in one child and tracheostomy in another child. All infants were homozygous for GAA mutations except one infant who was compound heterozygous. All infants (n = 8 with truncating mutations died. Our work provides insight into the correlation of genotypes and outcome of ERT in IOPD in Saudi Arabia. Our data suggest that early detection of cases, through newborn screening, and immunomodulation before the initiation of ERT may improve the outcome of ERT in Saudi infants with IOPD. Keywords: Pompe disease, Glycogen storage disease type II, Enzyme replacement therapy, GAA

Antisense Oligonucleotides Promote Exon Inclusion and Correct the Common c.-32-13T>G GAA Splicing Variant in Pompe Disease

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Erik van der Wal

2017-06-01

Full Text Available The most common variant causing Pompe disease is c.-32-13T>G (IVS1 in the acid α-glucosidase (GAA gene, which weakens the splice acceptor of GAA exon 2 and induces partial and complete exon 2 skipping. It also allows a low level of leaky wild-type splicing, leading to a childhood/adult phenotype. We hypothesized that cis-acting splicing motifs may exist that could be blocked using antisense oligonucleotides (AONs to promote exon inclusion. To test this, a screen was performed in patient-derived primary fibroblasts using a tiling array of U7 small nuclear RNA (snRNA-based AONs. This resulted in the identification of a splicing regulatory element in GAA intron 1. We designed phosphorodiamidate morpholino oligomer-based AONs to this element, and these promoted exon 2 inclusion and enhanced GAA enzyme activity to levels above the disease threshold. These results indicate that the common IVS1 GAA splicing variant in Pompe disease is subject to negative regulation, and inhibition of a splicing regulatory element using AONs is able to restore canonical GAA splicing and endogenous GAA enzyme activity.

Pituitary diseases : long-term psychological consequences

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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

Mapping the T helper cell response to acid α-glucosidase in Pompe mice.

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Nayak, Sushrusha; Sivakumar, Ramya; Cao, Ou; Daniell, Henry; Byrne, Barry J; Herzog, Roland W

2012-06-01

Pompe disease is a neuromuscular disease caused by an inherited deficiency of the lysosomal enzyme acid α-glucosidase (GAA). The resulting accumulation of glycogen causes muscle weakness with the severe form of the disease resulting in death by cardiorespiratory failure in the first year of life. The only available treatment, enzyme replacement therapy (ERT) with recombinant GAA (rhGAA), is severely hampered by antibody responses that reduce efficacy and cause immunotoxicities. Currently, Pompe mice represent the only pre-clinical model for development of new treatments and for immunological studies. While antibody formation following ERT in this model has been described, the underlying T cell response has not been studied. In order to define the T helper response to rhGAA in Pompe mice, immunodominant CD4(+) T cell epitopes were mapped in GAA(-/-) 129SVE mice using ELISpot. Additionally, cytokine responses and antibody formation against rhGAA during ERT were measured. Among the three CD4(+) T cell epitopes identified, only epitope IFLGPEPKSVVQ, predicted to be the strongest MHC II binder, consistently contributed to IL-4 production. Frequencies of IL-4 producing T cells were considerably higher than those of IL-17 or IFN-γ producing cells, suggesting a predominantly Th2 cell mediated response. This is further supported by IgG1 being the prevalent antibody subclass against rhGAA during ERT and consistent with prior reports on IgE formation and anaphylaxis in this model. These results will facilitate mechanistic studies of the immune response to rhGAA in Pompe mice during development of new therapies and tolerance protocols. Copyright © 2012 Elsevier Inc. All rights reserved.

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....

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

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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.

Expanding our understanding of lower urinary tract symptoms and incontinence in adults with pompe disease.

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McNamara, Erin R; Austin, Stephanie; Case, Laura; Wiener, John S; Peterson, Andrew C; Kishnani, Priya S

2015-01-01

To study the prevalence of lower urinary tract symptoms (LUTS) and incontinence in late-onset Pompe disease (LOPD) Methods: Adult LOPD patients seen at the Duke Pompe Clinic were prospectively recruited and asked to complete validated questionnaires on LUTS and incontinence as part of an IRB-approved study. Patient demographics as well as previous urologic history were reviewed. 35 patients with LOPD were included in the study (17 males and 18 females). The median age was 51.8 (range 18-72 years of age). Of these patients, 27/35 were receiving enzyme replacement therapy (ERT) with median duration of 54 months (range 5-88 months). In the male patients, 9/17 (53%) described their stream as dribbling, weak, or intermittent, and 9/17 (53%) complained of post-void dribbling. In addition 38% of the men were unable to stop their urination midstream. In the female patients, the most common complaint was urinary incontinence, reported in 14/18 (78%). In addition, 7/18 (39%) complained of post-void dribbling, and 47% were unable to stop their urination midstream. Bowel incontinence was reported in 45% of patients. There was a significant association between urinary symptoms and lower extremity function scores and duration of ERT (p = 0.005 and p = 0.04, respectively) This is the first study in a large cohort of LOPD patients that demonstrates LUTS and incontinence occur at a high rate. This study emphasizes the spectrum of LOPD is beyond isolated gross motor and pulmonary involvement and has a significant effect on the lower urinary tract.

Long-term results of peripheral arterial disease rehabilitation

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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

As identidades ficcionais de Raul Pompéia

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Franco Baptista Sandanello

2014-12-01

Full Text Available A fortuna crítica da obra de Raul Pompéia – especialmente, de seu romance O Ateneu – foi marcada por uma forte aproximação entre a vida do escritor e sua ficção. No entanto, pouco se falou a respeito de um aspecto muito curioso desta relação complexa entre vida e arte: o papel subsequente de Pompéia na literatura brasileira não como escritor, mas como personagem. Curiosamente, o romancista integra a trama de obras ficcionais como, por exemplo, Tentação, de Adolfo Caminha, O canudo, de Afonso Schmidt, e Investigação sobre Ariel, de Sílvio Fiorani. Nesse sentido, a partir de uma comparação entre estas obras, será discutida a possível figuração ficcional de Pompéia, assim como a posição particular de sua subjetividade dentro da mecânica narrativa de cada texto. Do primeiro ao último, há, por assim dizer, um progressivo distanciamento do universo ficcional por ele   criado (como o do citado O Ateneu e uma ênfase cada vez    maior em suas crises e inquietações pessoais. The critical reception of Raul Pompéia’s work – especially that of O Ateneu – has strongly identified over the years his biography to his fiction. However, very little was said on a    very peculiar aspect of this complex link between life and art: the subsequent role of Pompéia in Brazilian literature not as a    writer, but as a fictional character. Curiously, the writer integrates the plot of fictional works such as Tentação, by Adolfo Caminha, O canudo, by Afonso Schmidt, and Investigação sobre Ariel, by Sílvio Fiorani. In this regard, from an initial comparison of these texts, this article discusses the fictional figure of Pompéia, as well as the particular standing of his subjectivity in each narrative. So to speak, in these texts there is a gradual detachment of the fictional universe of his own    works (as that of O Ateneu and an increasing emphasis on his personal dramas and crisis.

Molecular pomps at very low pressures. Generalization; Pompes moleculaires a tres basse pression

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Mercier, C; Benoist, P [Commissariat a l' Energie Atomique, Saclay (France). Centre d' Etudes Nucleaires

1956-07-01

In an earlier study it was shown by one of the authors that the theory could account for the experimental results on molecular pomps starting from simple assumption like the absence of collisions between molecules and diffuse reflection by the walls. In the present paper the formulas are extended to the case when the surface velocity of the rotor is no longer negligible compared with the velocity of the molecules; moreover the mathematical treatment is analysed in more details. (author) [French] Dans une precedente etude I'un des auteurs a montre que la theorie etait capable de fournir, a partir d'hypotheses simples telles que l'absence de chocs entre les molecules et la reflexion diffuse sur les parois, des resultats concernant les pompes moleculaires en accord avec les donnees experimentales. Dans le present rapport, les formules seront generalisees au cas ou la vitesse peripherique du rotor n'est plus negligeable devant la vitesse des molecules; de plus, le traitement mathematique utilise est analyse plus en detail. (auteur)

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

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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.

Lower Urinary Tract Symptoms and Incontinence in Children with Pompe Disease.

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Ajay, Divya; McNamara, Erin R; Austin, Stephanie; Wiener, John S; Kishnani, Priya

2016-01-01

Pompe disease (PD) is a disorder of lysosomal glycogen storage. The introduction of enzyme replacement therapy (ERT) has shifted the focus of care from survival to quality of life. The presence of lower urinary tract symptoms (LUTS) and incontinence has not been previously described in children with PD. Children with PD followed in the Duke Lysosomal Storage Disease Clinic completed a validated bladder control symptom score (BCSS) and additional questions regarding urinary tract infections (UTIs), giggle, and stress incontinence. Descriptive statistics were used to discriminate urinary symptoms between gender, age, and different types of PD. Sixteen of 23 children (aged 4-14 years) seen in our clinic participated. Seven were girls; ten had classic infantile PD, two atypical infantile PD, and four childhood presentation late-onset PD (LOPD). When stratified by PD subtype, median BCSS was worst for the classic PD subtype followed by atypical PD and LOPD. Daytime urinary incontinence accompanied by constipation was noted in six. Eight reported urinary incontinence with laughing: giggle incontinence in six and stress incontinence in two. Four girls reported a history of UTI. Longitudinal follow-up in 11 patients showed stable BCSS in six, improvement in three, and worsening in two. Worsening corresponded with changes in bowel function and improvement with increase in ERT dose or treatment of constipation. LUTS and incontinence are common in children with PD with greater symptoms noted with infantile-type PD. Improved bowel function and increase in ERT dose may lead to improvements in BCSS.

Control of neglected tropical diseases needs a long-term commitment

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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

Orthopedic Management of Patients with Pompe Disease: A Retrospective Case Series of 8 Patients

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Gerrit Haaker

2014-01-01

Full Text Available Introduction. Pompe disease (PD, a lysosomal storage disease as well as a neuromuscular disorder, is a rare disease marked by progressive muscle weakness. Enzyme replacement therapy (ERT in recent years allowed longer survival but brought new problems to the treatment of PD with increasing affection of the musculoskeletal system, particularly with a significantly higher prevalence of scoliosis. The present paper deals with the orthopedic problems in patients with PD and is the first to describe surgical treatment of scoliosis in PD patients. Patients and Methods. The orthopedic problems and treatment of eight patients with PD from orthopedic consultation for neuromuscular disorders are retrospectively presented. We analyzed the problems of scoliosis, hip dysplasia, feet deformities, and contractures and presented the orthopedic treatment options. Results. Six of our eight PD patients had scoliosis and two young patients were treated by operative spine stabilization with benefits for posture and sitting ability. Hip joint surgery, operative contracture release, and feet deformity correction were performed with benefits for independent activity. Conclusion. Orthopedic management gains importance due to extended survival and musculoskeletal involvement under ERT. Surgical treatment is indicated in distinct cases. Further investigation is required to evidence the effect of surgical spine stabilization in PD.

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

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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.

Autophagy and Mis-targeting of Therapeutic Enzyme in Skeletal Muscle in Pompe Disease

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Fukuda, Tokiko; Ahearn, Meghan; Roberts, Ashley; Mattaliano, Robert J.; Zaal, Kristien; Ralston, Evelyn; Plotz, Paul H.; Raben, Nina

2009-01-01

Enzyme replacement therapy (ERT) became a reality for patients with Pompe disease, a fatal cardiomyopathy and skeletal muscle myopathy caused by a deficiency of glycogen-degrading lysosomal enzyme acid alpha-glucosidase (GAA). The therapy, which relies on receptor-mediated endocytosis of recombinant human GAA (rhGAA), appears to be effective in cardiac muscle, but less so in skeletal muscle. We have previously shown a profound disturbance of the lysosomal degradative pathway (autophagy) in therapy-resistant muscle of GAA knockout mice (KO). Our findings here demonstrate a progressive age-dependent autophagic build-up in addition to enlargement of glycogen-filled lysosomes in multiple muscle groups in the KO. Trafficking and processing of the therapeutic enzyme along the endocytic pathway appear to be affected by the autophagy. Confocal microscopy of live single muscle fibers exposed to fluorescently labeled rhGAA indicates that a significant portion of the endocytosed enzyme in the KO was trapped as a partially processed form in the autophagic areas instead of reaching its target – the lysosomes. A fluid-phase endocytic marker was similarly mis-targeted and accumulated in vesicular structures within the autophagic areas. These findings may explain why ERT often falls short of reversing the disease process, and point to new avenues for the development of pharmacological intervention. PMID:17008131

Aspectos respiratórios da doença de Pompe: Relato de caso

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Ewandro L.R. Moura

2008-01-01

Full Text Available Resumo: Relatamos o caso de doente feminina, 38 anos, com diagnóstico de doença de Pompe há oito anos. Há cinco anos vinha com queixas respiratórias, evoluindo com insuficiência respiratória crónica. Em uso de CPAP (continuous positive airway pressure nocturno há um ano, apresentava agravamento clínico progressivo. Veio encaminhada ao Hospital Universitário de Brasília, com descompensação respiratória e cor pulmonale. Iniciou-se ventilação não invasiva com dois níveis de pressões (VNI e realizou-se polissonografia, que demonstrou a necessidade do ajuste de frequência respiratória associada aos ajustes das pressões para adequação da ventilação durante o sono. Evoluiu com melhora importante após uso da VNI.Rev Port Pneumol 2008; XIV (1: 159-164 Abstract: We report the case of a 38 years-old female patient with Pompe disease, diagnosed eight years ago. Respiratory complaints appeared five years ago progressing to chronic respiratory failure. Nocturnal CPAP (continuous positive airway pressure was prescribed last year, presenting progressive clinical worsening. She was referred to Hospital Universitário de Brasília, on account of respiratory failure and cor pulmonale. Thus, she began non-invasive ventilation (NIV with bi-level positive airways pressure, and a polysomnography showed the need of spontaneous/timed mode associated with adjustments in breathing pressures to improve ventilation during sleep. There was significant clinical improvement after NIV support was established.Rev Port Pneumol 2008; XIV (1: 159-164 Palavras-chave: Doença de depósito de glicogénio tipo II, doença de Pompe, miopatia, insuficiência respiratória, ventilação mecânica, Key-words: Glycogen storage disease type II, Pompe disease, myopathy, respiratory failure, ventilation, mechanical

Aspectos respiratórios da doença de Pompe: Relato de caso

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Ewandro L.R. Moura

2008-01-01

Full Text Available Resumo: Relatamos o caso de doente feminina, 38 anos, com diagnóstico de doença de Pompe há oito anos. Há cinco anos vinha com queixas respiratórias, evoluindo com insuficiência respiratória crónica. Em uso de CPAP (continuous positive airway pressure nocturno há um ano, apresentava agravamento clínico progressivo. Veio encaminhada ao Hospital Universitário de Brasília, com descompensação respiratória e cor pulmonale. Iniciou-se ventilação não invasiva com dois níveis de pressões (VNI e realizou-se polissonografia, que demonstrou a necessidade do ajuste de frequência respiratória associada aos ajustes das pressões para adequação da ventilação durante o sono. Evoluiu com melhora importante após uso da VNI Abstract: We report the case of a 38 years-old female patient with Pompe disease, diagnosed eight years ago. Respiratory complaints appeared five years ago progressing to chronic respiratory failure. Nocturnal CPAP (continuous positive airway pressure was prescribed last year, presenting progressive clinical worsening. She was referred to Hospital Universitário de Brasília, on account of respiratory failure and cor pulmonale. Thus, she began non-invasive ventilation (NIV with bi-level positive airways pressure, and a polysomnography showed the need of spontaneous/timed mode associated with adjustments in breathing pressures to improve ventilation during sleep. There was significant clinical improvement after NIV support was established Palavras-chave: Doença de depósito de glicogénio tipo II, doença de Pompe, miopatia, insuficiência respiratória, ventilação mecânica, Key-words: Glycogen storage disease type II, Pompe disease, myopathy, respiratory failure, ventilation, mechanical

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

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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.

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

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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.

Safety and efficacy of exercise training in adults with Pompe disease: evalution of endurance, muscle strength and core stability before and after a 12 week training program.

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van den Berg, Linda E M; Favejee, Marein M; Wens, Stephan C A; Kruijshaar, Michelle E; Praet, Stephan F E; Reuser, Arnold J J; Bussmann, Johannes B J; van Doorn, Pieter A; van der Ploeg, Ans T

2015-07-19

Pompe disease is a proximal myopathy. We investigated whether exercise training is a safe and useful adjuvant therapy for adult Pompe patients, receiving enzyme replacement therapy. Training comprised 36 sessions of standardized aerobic, resistance and core stability exercises over 12 weeks. Before and after, the primary outcome measures safety, endurance (aerobic exercise capacity and distance walked on the 6 min walk test) and muscle strength, and secondary outcome measures core stability, muscle function and body composition, were evaluated. Of 25 patients enrolled, 23 successfully completed the training. Improvements in endurance were shown by increases in maximum workload capacity (110 W before to 122 W after training, [95 % CI of the difference 6 · 0 to 19 · 7]), maximal oxygen uptake capacity (69 · 4 % and 75 · 9 % of normal, [2 · 5 to 10 · 4]), and maximum walking distance (6 min walk test: 492 meters and 508, [-4 · 4 to 27 · 7] ). There were increases in muscle strength of the hip flexors (156 · 4 N to 180 · 7 N [1 · 6 to 13 · 6) and shoulder abductors (143 · 1 N to 150 · 7 N [13 · 2 to 35 · 2]). As an important finding in secondary outcome measures the number of patients who were able to perform the core stability exercises rose, as did the core stability balancing time (p core stability exercises is feasible, safe and beneficial to adults with Pompe disease.

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

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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.

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

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

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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.

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

[Guidelines for monitoring late-onset Pompe disease.Sociedad Española de Medicina Interna (SEMI), Sociedad Española de Neurología (SEN) y Sociedad Española de Neumología y CirugíaTorácica (SEPAR)].

Science.gov (United States)

Gutiérrez-Rivas, Eduardo; Illa, Isabel; Pascual-Pascual, Samuel I; Pérez-López, Jordi; Vílchez-Padilla, Juan J; Bautista-Lorite, Juan; Barrot, Emilia; López de Munain, Adolfo

2015-04-01

Although treatment with alglucosidase alfa has helped improve the prognosis of patients with late-onset Pompe disease, both the development of the disease and the effectiveness of the treatment need to be monitored on a regular basis. This is the reason that has led a committee of Spanish experts to draw up a series of guidelines on how to follow up these patients. The committee proposes a model of follow-up tests for late-onset Pompe disease. First of all, the nutritional status and swallowing function must be evaluated. Second, and due to the variability of the clinical features, the committee recommends the simultaneous use of several scales to measure different functions and parameters. Thus, muscular force is assessed with the Medical Research Council scale; motor functioning, with the six-minute walk test and timed tests; disability, with the Rasch-built Pompe-specific Activity scale; respiratory functioning, with measurement of the forced vital capacity and oxygen saturation; and fatigue, with the fatigue intensity scale. Lastly, the safety and tolerability of enzyme replacement therapy are controlled by registering and treating the potential side effects and measurement of the anti-alglucosidase alfa antibodies. A number of different general recommendations are also included.

Mechanical pumps for liquid metals; Pompes mecaniques pour metaux liquides

Energy Technology Data Exchange (ETDEWEB)

Baumier, J; Gollion, H J [Commissariat a l' Energie Atomique, Fontenay-aux-Roses (France). Centre d' Etudes Nucleaires

1964-07-01

The pumping of liquid metals by centrifugal pumps poses two principal problems. These are hermetic sealing of the rotating shaft and, its guidance where immersed in liquid metal. The solutions to the problems used on 13 experimental pumps are given here. The resolution of the guidance problem consists in the majority of cases in the utilisation of hydrostatic bearings. Accordingly, a theoretical study was instituted for the first time to calculate the bearings of the earlier pumps. After this, an experimental study was carried out, to check the theory by water tests. A relation for bearing calculation of pumps with diffusers is proposed. Finally the influence of the bearing elasticity on the shafts critical speed is studied. (authors) [French] Le pompage des metaux liquides, par des pompes centrifuges, pose 2 principaux problemes, qui sont: d'une part, la realisation d'une excellente etancheite au passage de l'arbre, d'autre part, son guidage sur la partie immergee dans le metal liquide. Les solutions retenues pour resoudre ces problemes sur 13 pompes experimentees sont presentees. Le probleme du guidage de l'arbre, a dans la majorite des cas ete resolu en utilisant un palier hydrostatique, aussi l'etude en a d'abord ete approfondie de facon theorique pour calculer les paliers des premieres pompes, puis experimentale pour controler la theorie, en effectuant des essais a l'eau. On propose une relation pour calculer les paliers des pompes a diffuseurs. On a en outre effectue une etude de l'influence de l'elasticite du palier hydrostatique sur la vitesse critique de l'arbre. (auteurs)

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....

Enfermedad de Pompe de inicio temprano. A propósito de un caso

OpenAIRE

Grandez-Urbina, J. Antonio; Bustamante-Ubillus, Claudio; Farro-Calderon, Rossy; Linares-Alarcon, Ismael; Goyzueta-Knox, Silvana

2013-01-01

La enfermedad de Pompe es una enfermedad inusual de presentación temprana, causada por el déficit de la síntesis de α 1-4 glucosidasa ácida. Presentamos un caso de inicio insidioso descompensado evidenciándose dificultad respiratoria y cardiomiopatía. La enfermedad de Pompe es muy infrecuente, siendo el primer caso reportado en nuestro medio.

Enfermedad de Pompe de inicio temprano: A propósito de un caso

Directory of Open Access Journals (Sweden)

J. Antonio Grandez-Urbina

2013-10-01

Full Text Available La enfermedad de Pompe es una enfermedad inusual de presentación temprana, causada por el déficit de la síntesis de α 1-4 glucosidasa ácida. Presentamos un caso de inicio insidioso descompensado evidenciándose dificultad respiratoria y cardiomiopatía. La enfermedad de Pompe es muy infrecuente, siendo el primer caso reportado en nuestro medio.

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.

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...

Pompes à chaleur dans l'industrie Heat Pumps in Industry

Directory of Open Access Journals (Sweden)

Benchecroun N.

2006-11-01

Full Text Available Les pompes à chaleur pourraient permettre une réduction de la consommation énergétique de l'industrie française estimée à 3,5 Mtep à l'horizon 1990 (300 000 tep en 1982. Ces installations se situeront dans les domaines du chauffage des locaux industriels, du séchage à basse température, du réchauffage de liquides et de certaines opérations de fractionnement. Pour les utilisations dans des procédés industriels les secteurs concernés sont principalement les industries agroalimentaires et, à plus long terme, celui de la chimie (y compris pétrochimie. On constate que les ventes de machines de forte puissance restent faibles (environ 500 de 1973 à 1982. Le climat général d'investissement, la nécessité d'adapter les procédés à l'utilisation d'une pompe à chaleur, et le faible niveau de température Heat pumps could be used to reduce energy consumption by French industry which may attain 3. 5 million tOE by 1990 (300 000 tOE in 1982. Such installations will be used in the fields of industrial space heating, low-temperature drying, the heating of liquids and various fractionating operations. For uses in industrial processes, the sectors involved are mainly agribusiness and food industries and, in the longer range, the chemical industry (including petrochemicals. Sales of high-powered machinery are still low (about 500 from 1973 to 1982. The general investment climate, the need to adapt processes to the use of heat pumps, and the low temperature level (< 120 °C of the heat delivered are the main factors holding back the penetration of this type of equipment in industry. The use of a nonazeotropic mixture of fluids (particularly hydrocarbons gives compression heat pumps a flexibility of adaptation which should help remove these technicalobstacles and thus make it easier for heat pumps to penetrate, especially in the chemical sector.

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.

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 =

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

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.

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

El rol del fisioterapeuta en el manejo de la Enfermedad de Pompe

OpenAIRE

Rocío del Pilar Ruiz Agudelo; Laura Lorena Alba; Rosmary Martínez-Rueda

2015-01-01

Antecedentes: La enfermedad de Pompe está catalogada dentro del grupo de enfermedades huérfanas.  A pesar de existir tratamiento, quienes la padecen pueden sufrir secuelas relacionadas con la misma, en parte por la dificultad de un diagnóstico temprano y adecuado de la patología. Objetivo: Describir las diferentes alternativas de tratamiento fisioterapéutico para la enfermedad de Pompe. Metodología: Se realizó una revisión bibliométrica. Se consultaron en bases de datos y portales científicos...

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.

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...

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)

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

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.

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.

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

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....

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.

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.

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

El rol del fisioterapeuta en el manejo de la Enfermedad de Pompe

Directory of Open Access Journals (Sweden)

Rocío del Pilar Ruiz Agudelo

2015-06-01

Full Text Available Antecedentes: La enfermedad de Pompe está catalogada dentro del grupo de enfermedades huérfanas.  A pesar de existir tratamiento, quienes la padecen pueden sufrir secuelas relacionadas con la misma, en parte por la dificultad de un diagnóstico temprano y adecuado de la patología. Objetivo: Describir las diferentes alternativas de tratamiento fisioterapéutico para la enfermedad de Pompe. Metodología: Se realizó una revisión bibliométrica. Se consultaron en bases de datos y portales científicos como PubMed, sCielo, Elsevier, Lilacs, MediGraphic, InfoMed, portales de asociaciones o fundaciones de carácter público y privadas, ONG's, con una ventana de tiempo entre 1993 y 2014; artículos y documentos que describieran intervenciones en estos pacientes, clasificándolas según los dominios de la American Physical Therapy Association (APTA. Resultados: Se encontraron 50 artículos que cumplían con los criterios de búsqueda. Se pudo evidenciar que el abordaje terapéutico tardío dificulta la habilitación y rehabilitación del paciente por las características de las secuelas propias de la enfermedad. Dado el compromiso cardiorrespiratorio, osteomuscular, neuromotor y tegumentario, la prescripción del ejercicio debe ser analizada según las condiciones clínicas y fisiológicas del paciente, pero en todos los casos (Pompe tardío y Pompe infantil la intensidad de las actividades debe ser leve de bajo impacto. Conclusiones: Las dimensiones de mayor compromiso y que requieren por tanto un mayor trabajo fisioterapéutico se centran en el dominio cardiorrespiratorio y osteomuscular. Se recomienda realizar más estudios relacionados con esta temática que permitan una adecuada intervención temprana en este tipo de pacientes.

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.

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

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....

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)....

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)

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

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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.

Long-term risks of kidney living donation

DEFF Research Database (Denmark)

Maggiore, Umberto; Budde, Klemens; Heemann, Uwe

2017-01-01

Two recent matched cohort studies from the USA and Norway published in 2014 have raised some concerns related to the long-term safety of kidney living donation. Further studies on the long-term risks of living donation have since been published. In this position paper, Developing Education Science...... and Care for Renal Transplantation in European States (DESCARTES) board members critically review the literature in an effort to summarize the current knowledge concerning long-term risks of kidney living donation to help physicians for decision-making purposes and for providing information...... to the prospective live donors. Long-term risk of end-stage renal disease (ESRD) can be partially foreseen by trying to identify donors at risk of developing ‘de novo’ kidney diseases during life post-donation and by predicting lifetime ESRD risk. However, lifetime risk may be difficult to assess in young donors...

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

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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.

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

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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.

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

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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.

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

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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

Guía clínica de la enfermedad de Pompe de inicio tardío

OpenAIRE

Barba-Romero, Miguel A.; Barrot, Emilia; Bautista-Lorite, Juan; Gutiérrez-Rivas, Eduardo; Illa, Isabel; Jiménez, Luis M.; Ley-Martos, Myriam; López de Munain, Adolfo; Pardo, Julio; Pascual-Pascual, Samuel I.; Pérez-López, Jordi; Solera, Jesús; Vílchez-Padilla, Juan J.

2012-01-01

English version available at www.neurologia.com Hasta 2006, la enfermedad de Pompe o glucogenosis tipo II era una enfermedad incurable y con tratamiento meramente paliativo. El desarrollo de la terapia de sustitución con la enzima α-glucosidasa recombinante humana ha constituido el primer tratamiento específico para esta enfermedad. El objetivo de esta guía es servir de referencia en el manejo de la variedad de inicio tardío de la enfermedad de Pompe, es decir, la que aparece d...

Identificación y caracterización de las mutaciones en el gen GAA en pacientes colombianos afectados por la enfermedad de Pompe

OpenAIRE

Niño Martínez, Monica Yasmin

2013-01-01

Pompe disease (PD) is a recessive metabolic disorder characterized by acid α-glucosidase (GAA) deficiency, an enzyme that catalyzes the hydrolysis of α-1,4 and α-1,6 glucosidic bonds of glycogen. This deficiency results in lysosomal accumulation of glycogen in all tissues, especially in skeletal muscles. PD patients display a large spectrum of phenotypes with regard the age of onset, the disease progression rate and the severity of symptoms. The clinical course of the disease is mainly determ...

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

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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

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

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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.

Lysosomal storage diseases

Science.gov (United States)

Ferreira, Carlos R.; Gahl, William A.

2016-01-01

Lysosomes are cytoplasmic organelles that contain a variety of different hydrolases. A genetic deficiency in the enzymatic activity of one of these hydrolases will lead to the accumulation of the material meant for lysosomal degradation. Examples include glycogen in the case of Pompe disease, glycosaminoglycans in the case of the mucopolysaccharidoses, glycoproteins in the cases of the oligosaccharidoses, and sphingolipids in the cases of Niemann-Pick disease types A and B, Gaucher disease, Tay-Sachs disease, Krabbe disease, and metachromatic leukodystrophy. Sometimes, the lysosomal storage can be caused not by the enzymatic deficiency of one of the hydrolases, but by the deficiency of an activator protein, as occurs in the AB variant of GM2 gangliosidosis. Still other times, the accumulated lysosomal material results from failed egress of a small molecule as a consequence of a deficient transporter, as in cystinosis or Salla disease. In the last couple of decades, enzyme replacement therapy has become available for a number of lysosomal storage diseases. Examples include imiglucerase, taliglucerase and velaglucerase for Gaucher disease, laronidase for Hurler disease, idursulfase for Hunter disease, elosulfase for Morquio disease, galsulfase for Maroteaux-Lamy disease, alglucosidase alfa for Pompe disease, and agalsidase alfa and beta for Fabry disease. In addition, substrate reduction therapy has been approved for certain disorders, such as eliglustat for Gaucher disease. The advent of treatment options for some of these disorders has led to newborn screening pilot studies, and ultimately to the addition of Pompe disease and Hurler disease to the Recommended Uniform Screening Panel (RUSP) in 2015 and 2016, respectively. PMID:29152458

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.

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

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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.

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.

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

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.

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

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

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

Comparative impact of AAV and enzyme replacement therapy on respiratory and cardiac function in adult Pompe mice

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Darin J Falk

Full Text Available Pompe disease is an autosomal recessive genetic disorder characterized by a deficiency of the enzyme responsible for degradation of lysosomal glycogen (acid α-glucosidase (GAA. Cardiac dysfunction and respiratory muscle weakness are primary features of this disorder. To attenuate the progressive and rapid accumulation of glycogen resulting in cardiorespiratory dysfunction, adult Gaa−/− mice were administered a single systemic injection of rAAV2/9-DES-hGAA (AAV9-DES or bimonthly injections of recombinant human GAA (enzyme replacement therapy (ERT. Assessment of cardiac function and morphology was measured 1 and 3 months after initiation of treatment while whole-body plethysmography and diaphragmatic contractile function was evaluated at 3 months post-treatment in all groups. Gaa−/− animals receiving either AAV9-DES or ERT demonstrated a significant improvement in cardiac function and diaphragmatic contractile function as compared to control animals. AAV9-DES treatment resulted in a significant reduction in cardiac dimension (end diastolic left ventricular mass/gram wet weight; EDMc at 3 months postinjection. Neither AAV nor ERT therapy altered minute ventilation during quiet breathing (eupnea. However, breathing frequency and expiratory time were significantly improved in AAV9-DES animals. These results indicate systemic delivery of either strategy improves cardiac function but AAV9-DES alone improves respiratory parameters at 3 months post-treatment in a murine model of Pompe disease.

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

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...

Long-term Morbidity of Testicular Cancer Treatment.

Science.gov (United States)

Fung, Chunkit; Fossa, Sophie D; Williams, Annalynn; Travis, Lois B

2015-08-01

Second malignant neoplasms, cardiovascular disease, neurotoxicity and ototoxicity, pulmonary complications, hypogonadism, and nephrotoxicity are potentially life-threatening long-term complications of testicular cancer and its therapy. This article describes the pathogenesis, risks, and management of these late effects experienced by long-term testicular cancer survivors, who are defined as individuals who are disease free 5 years or more after primary treatment. Testicular cancer survivors should follow applicable national guidelines for cancer screening and management of cardiovascular disease risk factors. In addition, health care providers should capitalize on the time of cancer diagnosis as a teachable moment to introduce and promote lifestyle changes. Copyright © 2015 Elsevier Inc. All rights reserved.

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

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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

Guía clínica de la enfermedad de Pompe de inicio tardío

OpenAIRE

Barba-Romero, Miguel A.; Barrot, Emilia; Bautista-Lorite, Juan; Gutiérrez-Rivas, E.; Illa, Isabel; Jiménez, Luis M.; Ley-Martos, Myriam; López de Munain, Adolfo; Pardo, Julio; Pascual-Pascual, Samuel I.; Pérez-López, Jordi; Solera, Jesús; Vílchez-Padilla, Juan J.

2012-01-01

Hasta 2006, la enfermedad de Pompe o glucogenosis tipo II era una enfermedad incurable y con tratamiento meramente paliativo. El desarrollo de la terapia de sustitución con la enzima α-glucosidasa recombinante humana ha constituido el primer tratamiento específico para esta enfermedad. El objetivo de esta guía es servir de referencia en el manejo de la variedad de inicio tardío de la enfermedad de Pompe, es decir, la que aparece después del primer año de vida. En la guía, un grupo de exper...

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.

The influence of a polymorphism in the gene encoding angiotensin converting enzyme (ACE on treatment outcomes in late-onset Pompe patients receiving alglucosidase alfa

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Rena C. Baek

2016-09-01

Full Text Available Correlations between angiotensin-converting enzyme (ACE genotype (I/I, I/D, D/D, disease severity at baseline and response to enzyme replacement therapy (ERT were assessed in the Pompe disease Late-Onset Treatment Study (LOTS. No correlations were observed between ACE genotype and disease severity at baseline. However, D/D patients appeared to have a reduced response to alglucosidase alfa treatment than I/I or I/D patients, suggesting that ACE polymorphisms may influence the response to alglucosidase alfa treatment and warrants further investigation.

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

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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

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.

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.)

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.)

The Long-Term Public Health Benefits of Breastfeeding.

Science.gov (United States)

Binns, Colin; Lee, MiKyung; Low, Wah Yun

2016-01-01

Breastfeeding has many health benefits, both in the short term and the longer term, to infants and their mothers. There is an increasing number of studies that report on associations between breastfeeding and long-term protection against chronic disease. Recent research evidence is reviewed in this study, building on previous authoritative reviews. The recent World Health Organization reviews of the short- and long-term benefits of breastfeeding concluded that there was strong evidence for many public health benefits of breastfeeding. Cognitive development is improved by breastfeeding, and infants who are breastfed and mothers who breastfeed have lower rates of obesity. Other chronic diseases that are reduced by breastfeeding include diabetes (both type 1 and type 2), obesity, hypertension, cardiovascular disease, hyperlipidemia, and some types of cancer. © 2015 APJPH.

Long-term mortality after Staphylococcus aureus spondylodiscitis

DEFF Research Database (Denmark)

Aagaard, Theis; Roed, Casper; Larsen, Anders R

2014-01-01

Patients diagnosed with Staphylococcus aureus spondylodiscitis have increased long-term mortality compared with the background population mainly due to infectious, endocrine, cardiovascular, gastrointestinal and alcohol and drug abuse-related diseases.......Patients diagnosed with Staphylococcus aureus spondylodiscitis have increased long-term mortality compared with the background population mainly due to infectious, endocrine, cardiovascular, gastrointestinal and alcohol and drug abuse-related diseases....

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)

Long-term mortality and causes of death associated with Staphylococcus aureus bacteremia

DEFF Research Database (Denmark)

Gotland, N; Uhre, M L; Mejer, N

2016-01-01

OBJECTIVES: Data describing long-term mortality in patients with Staphylococcus aureus bacteremia (SAB) is scarce. This study investigated risk factors, causes of death and temporal trends in long-term mortality associated with SAB. METHODS: Nationwide population-based matched cohort study...... respiratory disease, nervous system disease, unknown causes, psychiatric disorders, cardiovascular disease and senility. Over time, rates of death decreased or were stable for all disease categories except for musculoskeletal and skin disease where a trend towards an increase was seen. CONCLUSION: Long......-term mortality after SAB was high but decreased over time. SAB cases were more likely to die of eight specific causes of death and less likely to die of five other causes of death compared to controls. Causes of death decreased for most disease categories. Risk factors associated with long-term mortality were...

Lack of vigilance is the main factor of late diagnosis of glycogen storage disease type II in the Republic of Kazakhstan

Directory of Open Access Journals (Sweden)

L. A. Kuzina

2018-01-01

Full Text Available Pompe disease is a hereditary autosomal recessive disease characterized by accumulation of glycogen due to decreased activity of acid α-glucosidase enzyme in lysosomes. The disease can develop at any age. Cases with onset after the 1st year of life are attributed to late-onset Pompe disease (LOPD. LOPD has a very wide age range when clinical manifestations appear which significantly complicates diagnosis in adults. A case of verified Pompe disease in the Republic of Kazakhstan is presented. A chronology of clinical manifestations and symptoms, results of paraclinical examinations helping to suspect LOPD and verify the diagnosis by decreased activity of acid α-glucosidase in dry blood spot are described.

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

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.

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....

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...

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...

Longitudinal follow-up to evaluate speech disorders in early-treated patients with infantile-onset Pompe disease.

Science.gov (United States)

Zeng, Yin-Ting; Hwu, Wuh-Liang; Torng, Pao-Chuan; Lee, Ni-Chung; Shieh, Jeng-Yi; Lu, Lu; Chien, Yin-Hsiu

2017-05-01

Patients with infantile-onset Pompe disease (IOPD) can be treated by recombinant human acid alpha glucosidase (rhGAA) replacement beginning at birth with excellent survival rates, but they still commonly present with speech disorders. This study investigated the progress of speech disorders in these early-treated patients and ascertained the relationship with treatments. Speech disorders, including hypernasal resonance, articulation disorders, and speech intelligibility, were scored by speech-language pathologists using auditory perception in seven early-treated patients over a period of 6 years. Statistical analysis of the first and last evaluations of the patients was performed with the Wilcoxon signed-rank test. A total of 29 speech samples were analyzed. All the patients suffered from hypernasality, articulation disorder, and impairment in speech intelligibility at the age of 3 years. The conditions were stable, and 2 patients developed normal or near normal speech during follow-up. Speech therapy and a high dose of rhGAA appeared to improve articulation in 6 of the 7 patients (86%, p = 0.028) by decreasing the omission of consonants, which consequently increased speech intelligibility (p = 0.041). Severity of hypernasality greatly reduced only in 2 patients (29%, p = 0.131). Speech disorders were common even in early and successfully treated patients with IOPD; however, aggressive speech therapy and high-dose rhGAA could improve their speech disorders. Copyright © 2016 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.

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...

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…

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.)

Enfermedad de pompe: presentación de un caso

OpenAIRE

Azrak, M. Angeles; Fernández, Zulma; Peruffo, M.Virginia; Specola, Norma; Nuñez, M.; Pardo, A.

2009-01-01

Presentamos el caso de una niña de 6 meses de ascendencia china con enfermedad de Pompe diagnosticada a partir de una miocardiopatía hipertrófica biventricular e hipotonía global. En esta enfermedad el acúmulo de glucógeno lisosomal principalmente en el corazón y músculo esquelético ocasiona la mayoría de la sintomatología clínica. En esta paciente la hipotonía generalizada, macroglosia, debil...

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

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.

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)

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.

Revisão sistemática das escalas utilizadas para avaliação funcional na doença de Pompe Revisión sistemática de las escalas utilizadas para evaluación funcional en la enfermedad de Pompe Systematic review of functional assessment scales in Pompe disease

OpenAIRE

Alana Karla Savegnago; Rosângela Maria da Silva; Cíntia Jonhston; Ana Maria Martins; Ana Paula L. de Melo; Werther Brunow de Carvalho

2012-01-01

OBJETIVO: Identificar as escalas utilizadas para avaliação funcional na doença de Pompe (DP) e descrever seu nível de evidência e recomendação. FONTES DE DADOS: Revisão sistemática sobre as escalas de avaliação funcional na DP. Pesquisa realizada nos bancos de dados Medline, Lilacs, Registro Cochrane de Ensaios Controlados Central (CCTR) e SciELO com artigos (exceto artigos de revisão) publicados entre 2000 e 2010. As palavras-chave utilizadas nos idiomas português e inglês foram: doença de d...

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.

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

The long-term effect of ambulatory oxygen in normoxaemic COPD patients

DEFF Research Database (Denmark)

Ringbaek, Thomas; Martinez, Gerd; Lange, Peter

2013-01-01

To study the long-term benefits of ambulatory oxygen (AO) in combination with pulmonary rehabilitation (PR) in chronic obstructive pulmonary disease (COPD) patients experiencing exertional desaturation.......To study the long-term benefits of ambulatory oxygen (AO) in combination with pulmonary rehabilitation (PR) in chronic obstructive pulmonary disease (COPD) patients experiencing exertional desaturation....

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....

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

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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.

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

长时程增强与长时程抑制的研究%Advances in long - term potentiation and long - term depression

Institute of Scientific and Technical Information of China (English)

张敬军; 夏作理

2000-01-01

The impairment of learning and memory function in the central nervous system(CNS)is one of the main features of aging and Alzheimer' s disease (AD). Many experimental results have showed that long- term memory(LTM) is related to long- term potentiation(LTP) and long- term depression (LTD). They intluenee each other. The relationship between LTP and LTD is complex. Therefore, it is important to study the learning and memory mechanism from LTP、LTD and gene expression in the CNS.

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

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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.

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

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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.

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)

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

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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.

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

NARCIS (Netherlands)

Hout, J.M. van den; Kamphoven, J.H.; Winkel, L.P.; Arts, W.F.M.; Klerk, J.B.C. de; Loonen, M.C.B.; Vulto, A.G.; Cromme-Dijkhuis, A.H.; Weisglas-Kuperus, N.; Hop, W.C.J.; Hirtum, H. van; Diggelen, O.P. van; Boer, M. de; Kroos, M.A.; Doorn, P.A. van; Voort, E.I. van der; Sibbles, B.; Corven, E.J. van; Brakenhoff, J.P.; Hove, J.L. van; Smeitink, J.A.M.; Jong, G. de; Reuser, A.J.J.; Ploeg, A.T. van der

2004-01-01

OBJECTIVE: 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,

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

NARCIS (Netherlands)

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

2004-01-01

textabstractOBJECTIVE: 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

Trastuzumab use during pregnancy: long-term survival after locally advanced breast cancer and long-term infant follow-up.

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Andrade, Jurandyr M de; Brito, Luiz G O; Moises, Elaine C D; Amorim, Andréa C; Rapatoni, Liane; Carrara, Hélio H A; Tiezzi, Daniel G

2016-04-01

Here, we describe the case of a patient diagnosed with locally advanced breast cancer 8 years ago. Her treatment course was neoadjuvant chemotherapy, followed by mastectomy and then adjuvant radiotherapy and trastuzumab (TTZ). During the use of adjuvant targeted therapy, an incidental pregnancy was diagnosed. Four years later, she developed bone and cerebral metastases, and since then, she has received courses of TTZ, capecitabine, lapatinib, and radiotherapy with intermittent control of the disease. Her 7-year-old son presents a normal physical and long-term neurological developmental curve according to specialized evaluation. This case is unique for several reasons: the patient received the highest dose of TTZ yet described during pregnancy (4400 mg); there has been a long period of disease-free survival after treatment for locally advanced breast cancer and long overall survival despite successive disease progressions during the metastatic phase of the disease (97 months), and there was a monitored pediatric follow-up period (7 years).

Fiber type conversion by PGC-1α activates lysosomal and autophagosomal biogenesis in both unaffected and Pompe skeletal muscle.

Directory of Open Access Journals (Sweden)

Shoichi Takikita

2010-12-01

Full Text Available PGC-1α is a transcriptional co-activator that plays a central role in the regulation of energy metabolism. Our interest in this protein was driven by its ability to promote muscle remodeling. Conversion from fast glycolytic to slow oxidative fibers seemed a promising therapeutic approach in Pompe disease, a severe myopathy caused by deficiency of the lysosomal enzyme acid alpha-glucosidase (GAA which is responsible for the degradation of glycogen. The recently approved enzyme replacement therapy (ERT has only a partial effect in skeletal muscle. In our Pompe mouse model (KO, the poor muscle response is seen in fast but not in slow muscle and is associated with massive accumulation of autophagic debris and ineffective autophagy. In an attempt to turn the therapy-resistant fibers into fibers amenable to therapy, we made transgenic KO mice expressing PGC-1α in muscle (tgKO. The successful switch from fast to slow fibers prevented the formation of autophagic buildup in the converted fibers, but PGC-1α failed to improve the clearance of glycogen by ERT. This outcome is likely explained by an unexpected dramatic increase in muscle glycogen load to levels much closer to those observed in patients, in particular infants, with the disease. We have also found a remarkable rise in the number of lysosomes and autophagosomes in the tgKO compared to the KO. These data point to the role of PGC-1α in muscle glucose metabolism and its possible role as a master regulator for organelle biogenesis - not only for mitochondria but also for lysosomes and autophagosomes. These findings may have implications for therapy of lysosomal diseases and other disorders with altered autophagy.

Clinical trials with rasagiline: evidence for short-term and long-term effects.

Science.gov (United States)

Siderowf, Andrew; Stern, Matthew

2006-05-23

Rasagiline (N-propargyl-1 (R)-aminoindan) is a selective, potent irreversible inhibitor of MAO-B that possesses neuroprotective and anti-apoptotic properties in a variety of in vitro and in vivo animal models relevant to Parkinson's disease (PD). Several randomized controlled clinical trials have demonstrated the safety and efficacy of rasagiline as monotherapy in PD and as adjunctive therapy for patients receiving levodopa. In addition, the 1-year randomized, delayed-start analysis of the TEMPO study suggests that rasagiline may slow the rate of progression of PD. The randomized delayed-start paradigm has potential to differentiate short-term symptomatic effects from long-term effects of anti-parkinsonian agents. In the future, long-term trials to examine the potential disease-modifying effects of rasagiline, which incorporate biological markers as well as clinical endpoints, may further elucidate the role of rasagiline in the treatment of both early and advanced PD.

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...

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

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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.

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.

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.

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

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

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)

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

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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

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.

Long-term outcome of eosinophilic fasciitis : A cross-sectional evaluation of 35 patients

NARCIS (Netherlands)

Mertens, JS; Thurlings, Rogier M; Kievit, Wietske; Seyger, Marieke M B; Radstake, Timothy R D; de Jong, Elke M G J

BACKGROUND: Eosinophilic fasciitis (EF) is a connective tissue disease with an unknown long-term course. OBJECTIVE: To evaluate presence and determinants of residual disease damage in patients with EF after long-term follow-up. METHODS: Patients with biopsy-proven EF were included for this

Long-term outcome of eosinophilic fasciitis: A cross-sectional evaluation of 35 patients

NARCIS (Netherlands)

Mertens, J.S.; Thurlings, R.M.; Kievit, W.; Seyger, M.M.B.; Radstake, T.R.D.J.; Jong, E.M.G.J. de

2017-01-01

BACKGROUND: Eosinophilic fasciitis (EF) is a connective tissue disease with an unknown long-term course. OBJECTIVE: To evaluate presence and determinants of residual disease damage in patients with EF after long-term follow-up. METHODS: Patients with biopsy-proven EF were included for this

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

AB073. Classic infantile-onset Pompe disease: phenotypes and outcomes of 5 Vietnamese patients receiving enzyme replacement therapy

Science.gov (United States)

Nguyen, Khanh Ngoc; Do, Mai Thi Thanh; Can, Ngoc Thi Bich; Hwu, Wuh-Liang; Vu, Dung Chi

2017-01-01

Background Pompe disease (PD) or glycogen storage disease type II is a lysosomal storage disorder, caused by mutations of GAA gene which results in deficiency of acid alpha-glucosidase (GAA) enzyme that involves in metabolism of glycogen in the lysosomes. Its incidence is 1/14,000–1/100,000. PD is divided into three types: classic infantile onset, non-classic infantile onset, and late onset. Early enzyme replacement therapy (ERT) before developing respiratory distress may lead to good outcome. Since 2013, we have identified 16 cases with classic infantile-onset and 5 cases were treated with ERT. Herein, we describe phenotypes and outcomes of five infantile-onset PD patients who received ERT. Methods GAA enzyme assay was done at National Taiwan University Hospital. Results Ages of diagnosis were 12, 38 and 70 days, 5 and 9 months of age. Clinical presentations included macroglossia (5/5), hypertrophic cardiomyopathy (5/5), failure to thrive (5/5), facial weakness and hypotonia (3 patients diagnosed after 70 days of age), respiratory failure (1 patient diagnosed at 9 months of age). All patients had mildly elevated plasma CK (270–380 UI/L) and transaminase (60–260 UI/l). Ages at starting ERT were 28 and 58 days, 3, 6 and 10 months. The time intervals from diagnosis to starting ERT were between 14 days and 1 month. The durations of ERT were 4–22 months. The outcomes were good. All patients had improvement of cardiac functions shown on echocardiography, respiratory status, and motor development. The patient who first received ERT at 10 months of age was reportedly dead at home due to food obstruction at 18 months of age. Current ages of the survivors were 5–24 months. Conclusions Patients with classic infantile-onset PD will have good outcomes if ERT is started early. Newborn screening for this disease is necessary to yield an early diagnosis.

Strategies to meet the need for long-term data.

Science.gov (United States)

Chalmers, John; Woodward, Mark; Borghi, Claudio; Manolis, Athanasios; Mancia, Giuseppe

2016-08-01

Chronic diseases afflict patients for many years, often to the end of life, and there is increasing need for estimating lifelong risk and for evaluating the effects of treatment in the long term. Yet recommendations for lifelong treatment are most frequently based on findings from randomized clinical trials lasting only a few years. There is therefore a clear need for much longer term data, and here we present the advantages and disadvantages of many strategies, including the use of long-term posttrial follow-up, of long-term prospective cohort studies, registry databases, and of administrative databases. We also emphasize the need for long-term cost-effectiveness studies. One of the most promising strategies comes from linkage of data gathered through the ever-expanding pool of administrative databases worldwide with data from other sources, including randomized trials and the many forms of observational study.

The developmental environment, epigenetic biomarkers and long-term health.

Science.gov (United States)

Godfrey, K M; Costello, P M; Lillycrop, K A

2015-10-01

Evidence from both human and animal studies has shown that the prenatal and early postnatal environments influence susceptibility to chronic disease in later life and suggests that epigenetic processes are an important mechanism by which the environment alters long-term disease risk. Epigenetic processes, including DNA methylation, histone modification and non-coding RNAs, play a central role in regulating gene expression. The epigenome is highly sensitive to environmental factors in early life, such as nutrition, stress, endocrine disruption and pollution, and changes in the epigenome can induce long-term changes in gene expression and phenotype. In this review we focus on how the early life nutritional environment can alter the epigenome leading to an altered susceptibility to disease in later life.

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.

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.

Sobre o feminino e a cidadania em Pompéia

OpenAIRE

Feitosa, Lourdes M. G. C.; Faversani, Fábio

2002-01-01

Nos últimos anos, novos trabalhos tem questionado os antigos paradigmas interpretativos que excluíam a mulher do espaço público e do grupo de cidadãos. O objetivo deste artigo é refletir sobre a ideia equivocada do confinamento feminino ao lar e de sua exclusão da vida pública e do poder, durante o século 1 d.C. A análise de inscrições parietais de Pompéia tem sido importante para questionar esta imagem sobre as mulheres romanas. _______________________________________________________________...

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...

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.

Mothers with HIP - the short term and long-term impact, what is new?

Science.gov (United States)

Mecacci, Federico; Ottanelli, Serena; Petraglia, Felice

2018-05-03

Hyperglycemia is one of the most common medical conditions that women encounter during pregnancy and it is due to gestational diabetes (GDM) in the majority of cases [1]. GDM is associated with a higher incidence of maternal morbidity in pregnancy in term of hypertensive disorders/preclampsia and higher rate of cesarean delivery but also with long-term risk of type 2 diabetes and cardiovascular disease. Pregnancy can therefore be considered a stress test; diagnosis of HIP can unmask a preexisting susceptibility and consequently a future risk for type 2 diabetes and can be a useful marker of future cardiovascular risk. Postpartum follow up provides an excellent opportunity to implement healthy lifestyle behaviors to prevent or delay the development of diabetes or cardiovascular disease. The aim of the current review is to focus on short and long term maternal morbidity of HIP. Copyright © 2018. Published by Elsevier B.V.

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....

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

Scientific Understanding from Long Term Observations: Insights from the Long Term Ecological Research (LTER) Program

Science.gov (United States)

Gosz, J.

2001-12-01

estuaries below by removing all incoming freshwater. At Toolik Lake, long-term experiments of removing top predators from the good web of lakes showed dramatic alterations of lake populations of small fish and zooplankton. In New Mexico, LTER research on small mammal populations is successfully predicting rodent increases and the potential for increased zoonotic diseases such as Hantavirus and bubonic plague. This ability to forecast based on El Nino prediction is being used to increase scientific awareness and public health awareness through media based communication with the public. In Oregon, the Andrews Forest LTER program has had long, strong links with natural resource policy and management. Basic understanding of forest-stream interactions, characteristics of old-growth forests, roles of woody debris in temperate forest ecosystems, invertebrate biodiversity and ecosystem function have been incorporated in management guidelines, plans and regulations for public and private lands throughout the Pacific Northwest. Other examples of the values of long-term research and monitoring will be presented.

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)

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

Audit of long-term and short-term liabilities

Directory of Open Access Journals (Sweden)

Korinko M.D.

2017-03-01

Full Text Available The article determines the importance of long-term and short-term liabilities for the management of financial and material resources of an enterprise. It reviews the aim, objects and information generators for realization of audit of short-term and long-term obligations. The organizing and methodical providing of audit of long-term and short-term liabilities of an enterprise are generalized. The authors distinguish the stages of realization of audit of long-term and short-term liabilities, the aim of audit on each of the presented stages, and recommend methodical techniques. It is fixed that it is necessary to conduct the estimation of the systems of internal control and record-keeping of an enterprise by implementation of public accountant procedures for determination of volume and maintenance of selection realization. After estimating the indicated systems, a public accountant determines the methodology for realization of public accountant verification of long-term and short-term liabilities. The analytical procedures that public accountants are expedient to use for realization of audit of short-term and long-term obligations are determined. The authors suggest the classification of the educed defects on the results of the conducted public accountant verification of short-term and long-term obligations.

Impact of long-term and short-term therapies on seminal parameters

Directory of Open Access Journals (Sweden)

Jlenia Elia

2013-04-01

Full Text Available Aim: The aim of this work was: i to evaluate the prevalence of male partners of subfertile couples being treated with long/short term therapies for non andrological diseases; ii to study their seminal profile for the possible effects of their treatments on spermatogenesis and/or epididymal maturation. Methods: The study group was made up of 723 subjects, aged between 25 and 47 years. Semen analysis was performed according to World Health Organization (WHO guidelines (1999. The Superimposed Image Analysis System (SIAS, which is based on the computerized superimposition of spermatozoa images, was used to assess sperm motility parameters. Results: The prevalence of subjects taking pharmacological treatments was 22.7% (164/723. The prevalence was 3.7% (27/723 for the Short-Term Group and 18.9% (137/723 for the Long-Term Group. The subjects of each group were also subdivided into subgroups according to the treatments being received. Regarding the seminal profile, we did not observe a significant difference between the Long-Term, Short-Term or the Control Group. However, regarding the subgroups, we found a significant decrease in sperm number and progressive motility percentage in the subjects receiving treatment with antihypertensive drugs compared with the other subgroups and the Control Group. Conclusions: In the management of infertile couples, the potential negative impact on seminal parameters of any drugs being taken as Long-Term Therapy should be considered. The pathogenic mechanism needs to be clarified.

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.

Long-term studies of the natural history of asthma in childhood

DEFF Research Database (Denmark)

Bisgaard, Hans; Bønnelykke, Klaus

2010-01-01

secondary prevention through the use of inhaled corticosteroids can effectively halt the long-term disease progression in childhood. In conclusion, the natural history of asthma and the associated airway changes is still poorly understood, and we have not managed to translate findings from long-term studies...

Short-term memory binding deficits in Alzheimer's disease

OpenAIRE

Parra, Mario; Abrahams, S.; Fabi, K.; Logie, R.; Luzzi, S.; Della Sala, Sergio

2009-01-01

Alzheimer's disease impairs long term memories for related events (e.g. faces with names) more than for single events (e.g. list of faces or names). Whether or not this associative or ‘binding’ deficit is also found in short-term memory has not yet been explored. In two experiments we investigated binding deficits in verbal short-term memory in Alzheimer's disease. Experiment 1 : 23 patients with Alzheimer's disease and 23 age and education matched healthy elderly were recruited. Participants...

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....

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

The long-term psychiatric and medical prognosis of perinatal mental illness.

Science.gov (United States)

Meltzer-Brody, Samantha; Stuebe, Alison

2014-01-01

The perinatal period provides an important window into a woman's long-term health. Perinatal mental illness is a common condition conferring potential serious long-term psychiatric and medical consequences for the mother and family. It is known that childbirth acts as a powerful trigger for depressive episodes in some women, and that women with histories of a mood disorder are particularly vulnerable. Some evidence links perinatal mental illness with obstetrical complications and reduced lactation initiation and duration. Therefore, perinatal mental illness may be a marker for long-term risk, and may contribute directly to subsequent cardiometabolic disease through both neuroendocrine mechanisms and the effects of mental illness on health behaviours. In clinical practice, these associations underscore the importance of screening and treating women with perinatal mental illness to ensure best possible long-term outcomes. Early screening and treatment may both mitigate the primary disease process and reduce the risk of comorbid medical conditions. Copyright © 2013 Elsevier Ltd. All rights reserved.

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.

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.

Long-term follow-up study and long-term care of childhood cancer survivors

Directory of Open Access Journals (Sweden)

Hyeon Jin Park

2010-04-01

Full Text Available The number of long-term survivors is increasing in the western countries due to remarkable improvements in the treatment of childhood cancer. The long-term complications of childhood cancer survivors in these countries were brought to light by the childhood cancer survivor studies. In Korea, the 5-year survival rate of childhood cancer patients is approaching 70%; therefore, it is extremely important to undertake similar long-term follow-up studies and comprehensive long-term care for our population. On the basis of the experiences of childhood cancer survivorship care of the western countries and the current Korean status of childhood cancer survivors, long-term follow-up study and long-term care systems need to be established in Korea in the near future. This system might contribute to the improvement of the quality of life of childhood cancer survivors through effective intervention strategies.

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)

Pompes à chaleur dans l'industrie Heat Pumps in Industry

OpenAIRE

Benchecroun N.

2006-01-01

Les pompes à chaleur pourraient permettre une réduction de la consommation énergétique de l'industrie française estimée à 3,5 Mtep à l'horizon 1990 (300 000 tep en 1982). Ces installations se situeront dans les domaines du chauffage des locaux industriels, du séchage à basse température, du réchauffage de liquides et de certaines opérations de fractionnement. Pour les utilisations dans des procédés industriels les secteurs concernés sont principalement les industries agroalimentaires et, à pl...

Pathophysiology of the GH/IGF-1 axis : long-term consequences on joints and bone

NARCIS (Netherlands)

Claessen, Kim Maria Johanna Aldegonda

2014-01-01

In this thesis, a number of observations are described in acromegaly patients with cured or biochemically well-controlled disease during long-term follow-up. These observations focus on the long-term consequences of the disease on joints and bone. In addition, we investigated the role of the Growth

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.

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.

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

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

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)

Long-term prognosis and causes of death after spondylodiscitis

DEFF Research Database (Denmark)

Aagaard, Theis; Roed, Casper; Dahl, Benny

2016-01-01

BACKGROUND: Data on long-term prognosis after spondylodiscitis are scarce. The purpose of this study was to determine long-term mortality and the causes of death after spondylodiscitis. METHODS: A nationwide, population-based cohort study using national registries of patients diagnosed with non.......62), respiratory (MRR = 1.71), gastrointestinal (MRR = 3.35), musculoskeletal (MRR = 5.39) and genitourinary diseases (MRR = 3.37), but also due to trauma, poisoning and external causes (MRR = 2.78), alcohol abuse-related diseases (MRR = 5.59) and drug abuse-related diseases (6 vs 0 deaths, MRR not calculable...... ratios (MRR). RESULTS: Three hundred and sixty-five patients (24%) and 1115 individuals from the comparison cohort (15%) died. Unadjusted MRR for spondylodiscitis patients was 1.76 (95% CI = 1.57-1.98) and 1.47 (95% CI = 1.30-1.66) after adjustment for comorbidity. No deaths were observed in 128 patients...

Epigenetics as a mechanism linking developmental exposures to long-term toxicity

DEFF Research Database (Denmark)

Barouki, R; Melén, E; Herceg, Z

2018-01-01

A variety of experimental and epidemiological studies lend support to the Developmental Origin of Health and Disease (DOHaD) concept. Yet, the actual mechanisms accounting for mid- and long-term effects of early-life exposures remain unclear. Epigenetic alterations such as changes in DNA methylat......A variety of experimental and epidemiological studies lend support to the Developmental Origin of Health and Disease (DOHaD) concept. Yet, the actual mechanisms accounting for mid- and long-term effects of early-life exposures remain unclear. Epigenetic alterations such as changes in DNA...

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

Long-term cortisol levels measured in scalp hair of obese patients.

Science.gov (United States)

Wester, Vincent L; Staufenbiel, Sabine M; Veldhorst, Margriet A B; Visser, Jenny A; Manenschijn, Laura; Koper, Jan W; Klessens-Godfroy, Francoise J M; van den Akker, Erica L T; van Rossum, Elisabeth F C

2014-09-01

In obese subjects a relatively high cortisol output in urine has been observed compared to nonobese individuals. However, cortisol levels in blood, saliva, and urine in association with obesity have been inconsistent across studies, possibly due to the high variability of systemic cortisol levels. Cortisol levels measured in scalp hair provide a marker for long-term cortisol exposure, and have been associated with cardiovascular disease in an elderly population and to disease course in Cushing's disease. We aimed to compare hair cortisol levels between obese patients and nonobese controls. Hair cortisol levels of 47 obese patients (median BMI 38.8, range 31.1-65.8), 41 overweight, and 87 normal-weight subjects using an enzyme-linked immunosorbent assay (ELISA) were measured. Obese patients had higher hair cortisol levels than overweight and normal weight subjects (respectively 30.8 vs 8.5 and 8.4 pg/mg hair, P cortisol levels was found between normal weight and overweight subjects. Our results suggest a higher long-term cortisol exposure in obese patients, which may contribute to cardiovascular disease risk. Future research will determine whether long-term cortisol levels provide a novel treatment target in the management of cardiovascular disease risk in obesity. © 2014 The Obesity Society.

Long-term management of GERD in the elderly with pantoprazole

Directory of Open Access Journals (Sweden)

Carlo Calabrese

2007-04-01

Full Text Available Carlo Calabrese, Anna Fabbri, Giulio Di FeboDepartment of Internal Medicine and Gastroenterology, University of Bologna, ItalyAbstract: The prevalence of gastroesophageal reflux disease (GERD increases with age and elderly are more likely to develop severe disease. Older patients often complain of less severe or frequent heartburn than younger patients and they may present with atypical symptoms such as dysphagia, weight loss, or extraesophageal symptoms. Proton pump inhibitors (PPIs are central in the management of GERD and are unchallenged with regards to their efficacy. They are considered safe and more effective than histamine receptor antagonists for healing esophagitis and for preventing its recurrence using a long term maintenance treatment. PPI have minimal side effects and few slight drug interactions and are considered safe for long term treatment. Pantoprazole is significantly effective both for acute and long-term treatment with excellent control of relapse and symptoms. It is well tolerated even for long-term therapy and its tolerability is optimal. Pantoprazole shows to have minimal interactions with other drugs because of a lower affinity for cytocrome P450 than older PPIs. Although the majority of elderly has concomitant illnesses and receive other drugs, this does not adversely effect the efficacy of pantoprazole because of its pharmacokinetics, which are independent of patient age. Clinical practice suggests that a low dose maintenance of PPIs should be used in older patients with GERD.Keywords: GERD, long-term management, pantoprazole, safety, efficacy, tolerability

Lysosomal Storage Diseases To date

OpenAIRE

HOFFMANN, Björn; MAYATEPEK, Ertan

2011-01-01

New therapeutic options and progress of approved therapies have made Lysosomal Storage Diseases (LSDs) one of the most exciting group of diseases. This review aims to summarize current achievements in these particular disorders and to give an outlook towards possible future treatment options. Enzyme replacement therapy is the gold standard for Gaucher disease, Fabry disease, Mucopolysaccharidosis type I, II, and VI, and for Pompe disease. Besides this, substrate reduction has been approved fo...

[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

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.

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.

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.

Long-term complications in Hodgkin's lymphoma survivors.

Science.gov (United States)

Kilickap, Saadettin; Barista, Ibrahim; Ulger, Sukran; Celik, Ismail; Selek, Ugur; Güllü, Ibrahim; Yildiz, Ferah; Kars, Ayse; Ozisik, Yavuz; Tekuzman, Gülten

2012-01-01

Background. Although patients with Hodgkin's lymphoma (HL) achieve prolonged survival, long-term complications are a major cause of morbidity and mortality among long-term survivors of HL. Methods. We retrospectively evaluated long-term complications in 336 HL survivors treated between January 1990 and January 2006 at the Department of Medical Oncology of the Hacettepe University Institute of Oncology who were >16 years old at presentation. All patients were regularly followed up every 3 months for the first 2 years after complete response, biannually for 3 years, and annually after 5 years. Results. Median follow-up was 8.5 years. The mean age (±SD) of the patients at the time of diagnosis was 35.7 ± 13.1 years. The male to female ratio was 61%/39%. During follow-up, 29 second malignancies (8.6%) were diagnosed in 28 patients with HL; 22 were solid tumors and 7 were hematological malignancies. Forty-seven (14.0%) of all patients with HL were found to have thyroid abnormalities. During follow-up, 54 (16.1%) patients developed cardiovascular complications. Overall, 29 (8.6%) patients developed late pulmonary toxicities. The cumulative number of chronic viral infections was 13 (3.9%). Conclusions. Long-term survivors of HL need to be properly followed up not only for disease control but also for evaluation of possible late morbidities to minimize the consequences.

Long-term potentiation and long-term depression: a clinical perspective

Directory of Open Access Journals (Sweden)

Timothy V.P. Bliss

2011-01-01

Full Text Available Long-term potentiation and long-term depression are enduring changes in synaptic strength, induced by specific patterns of synaptic activity, that have received much attention as cellular models of information storage in the central nervous system. Work in a number of brain regions, from the spinal cord to the cerebral cortex, and in many animal species, ranging from invertebrates to humans, has demonstrated a reliable capacity for chemical synapses to undergo lasting changes in efficacy in response to a variety of induction protocols. In addition to their physiological relevance, long-term potentiation and depression may have important clinical applications. A growing insight into the molecular mechanisms underlying these processes, and technological advances in non-invasive manipulation of brain activity, now puts us at the threshold of harnessing long-term potentiation and depression and other forms of synaptic, cellular and circuit plasticity to manipulate synaptic strength in the human nervous system. Drugs may be used to erase or treat pathological synaptic states and non-invasive stimulation devices may be used to artificially induce synaptic plasticity to ameliorate conditions arising from disrupted synaptic drive. These approaches hold promise for the treatment of a variety of neurological conditions, including neuropathic pain, epilepsy, depression, amblyopia, tinnitus and stroke.

Considerations on long-term immuno-intervention in the treatment of multiple sclerosis: an expert opinion.

Science.gov (United States)

Grigoriadis, Nikolaos; Linnebank, Michael; Alexandri, Nektaria; Muehl, Sarah; Hofbauer, Günther F L

2016-10-01

As management of multiple sclerosis (MS) requires life-long treatment with disease-modifying agents, any risks associated with long-term use should be considered when evaluating therapeutic options. Immune cells of the innate and adaptive immune systems play various roles in the pathogenesis of MS. MS therapies affect the immune system, each with a unique mode of action, and consequently possess different long-term safety profiles. Rare, but serious safety concerns, including an increased risk of infection and cancer, have been associated with immunosuppressant use. The risks associated with newer immunosuppressive agents, which target specific elements of MS disease pathophysiology, are not yet fully established as the duration of clinical trials is relatively short and post-marketing experience is limited. Non-immunosuppressants used to treat MS have well-defined safety profiles established over a large number of patient-years demonstrating them to be well-tolerated long-term treatment options. When considering the long-term use of disease-modifying agents for treating MS, classification as immunosuppressants or non-immunosuppressants can be useful when evaluating potential risks associated with chronic use. A successful therapeutic strategy for any serious, chronic disease such as MS should weigh effectiveness versus long-term safety of available treatments.

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.

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...

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

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...

Pediatric polytrauma : Short-term and long-term outcomes

NARCIS (Netherlands)

vanderSluis, CK; Kingma, J; Eisma, WH; tenDuis, HJ

Objective: To assess the short-term and long-term outcomes of pediatric polytrauma patients and to analyze the extent to which short-term outcomes can predict long-term outcomes. Materials and Methods: Ail pediatric polytrauma patients (Injury Severity Score of greater than or equal to 16, less than

Protective effects of long-term lithium administration in a slowly progressive SMA mouse model.

Science.gov (United States)

Biagioni, Francesca; Ferrucci, Michela; Ryskalin, Larisa; Fulceri, Federica; Lazzeri, Gloria; Calierno, Maria Teresa; Busceti, Carla L; Ruffoli, Riccardo; Fornai, Francesco

2017-12-01

In the present study we evaluated the long-term effects of lithium administration to a knock-out double transgenic mouse model (Smn-/-; SMN1A2G+/-; SMN2+/+) of Spinal Muscle Atrophy type III (SMA-III). This model is characterized by very low levels of the survival motor neuron protein, slow disease progression and motor neuron loss, which enables to detect disease-modifying effects at delayed time intervals. Lithium administration attenuates the decrease in motor activity and provides full protection from motor neuron loss occurring in SMA-III mice, throughout the disease course. In addition, lithium prevents motor neuron enlargement and motor neuron heterotopy and suppresses the occurrence of radial-like glial fibrillary acidic protein immunostaining in the ventral white matter of SMA-III mice. In SMA-III mice long-term lithium administration determines a dramatic increase of survival motor neuron protein levels in the spinal cord. These data demonstrate that long-term lithium administration during a long-lasting motor neuron disorder attenuates behavioural deficit and neuropathology. Since low level of survival motor neuron protein is bound to disease severity in SMA, the robust increase in protein level produced by lithium provides solid evidence which calls for further investigations considering lithium in the long-term treatment of spinal muscle atrophy.

Conversion of short-term to long-term memory in the novel object recognition paradigm.

Science.gov (United States)

Moore, Shannon J; Deshpande, Kaivalya; Stinnett, Gwen S; Seasholtz, Audrey F; Murphy, Geoffrey G

2013-10-01

It is well-known that stress can significantly impact learning; however, whether this effect facilitates or impairs the resultant memory depends on the characteristics of the stressor. Investigation of these dynamics can be confounded by the role of the stressor in motivating performance in a task. Positing a cohesive model of the effect of stress on learning and memory necessitates elucidating the consequences of stressful stimuli independently from task-specific functions. Therefore, the goal of this study was to examine the effect of manipulating a task-independent stressor (elevated light level) on short-term and long-term memory in the novel object recognition paradigm. Short-term memory was elicited in both low light and high light conditions, but long-term memory specifically required high light conditions during the acquisition phase (familiarization trial) and was independent of the light level during retrieval (test trial). Additionally, long-term memory appeared to be independent of stress-mediated glucocorticoid release, as both low and high light produced similar levels of plasma corticosterone, which further did not correlate with subsequent memory performance. Finally, both short-term and long-term memory showed no savings between repeated experiments suggesting that this novel object recognition paradigm may be useful for longitudinal studies, particularly when investigating treatments to stabilize or enhance weak memories in neurodegenerative diseases or during age-related cognitive decline. Copyright © 2013 Elsevier Inc. All rights reserved.

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....

Long-term follow-up of bronchus-associated lymphoid tissue lymphomas (BALTOMA)

International Nuclear Information System (INIS)

Gaffke, G.; Jost, D.; Stroszcynski, C.; Puls, R.; Schlecht, I.; Felix, R.; Ludwig, W.D.; Hosten, N.

2002-01-01

Purpose: The purpose of this work was to describe the findings and the long term follow up of pathologically confirmed bronchus-associated lymphoid tissue lymphoma (BALTOMA) in 6 patients. Methods: CT examinations and conventional radiological examinations were reviewed and compared to describe typical radiological findings and patterns of pulmonary manifestations. It were described the number of lesions and characteristics like presence of airspace consolidation, ground-glass attenuation, bubble-like radio-lucencies, air bronchogram, bronchial dilatation, Infiltration and the long term behaviour of the manifestations. Results: Lesions with a positive air bronchogram, no infiltration of extrapulmonary tissue or extrapulmonary manifestations were revealed as typical findings. Only a slow or no progression of disease was shown in most patients over a term of up to twelve years. Conclusions: The lymphoma of the bronchus-associated lymphoid tissue of the lung is a rare tumor. A positive air bronchogram, a multiplicity of disease, bilateral lesions, a fibrotic transformation of the lung tissue and no growth or only a slow groth over al long term of observation are typical radiological findings. (orig.) [de

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.

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

Near-Term Actions to Address Long-Term Climate Risk

Science.gov (United States)

Lempert, R. J.

2014-12-01

Addressing climate change requires effective long-term policy making, which occurs when reflecting on potential events decades or more in the future causes policy makers to choose near-term actions different than those they would otherwise pursue. Contrary to some expectations, policy makers do sometimes make such long-term decisions, but not as commonly and successfully as climate change may require. In recent years however, the new capabilities of analytic decision support tools, combined with improved understanding of cognitive and organizational behaviors, has significantly improved the methods available for organizations to manage longer-term climate risks. In particular, these tools allow decision makers to understand what near-term actions consistently contribute to achieving both short- and long-term societal goals, even in the face of deep uncertainty regarding the long-term future. This talk will describe applications of these approaches for infrastructure, water, and flood risk management planning, as well as studies of how near-term choices about policy architectures can affect long-term greenhouse gas emission reduction pathways.

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.

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...

Long-Term Fine Particulate Matter Exposure and Major Depressive Disorder in a Community-Based Urban Cohort

Science.gov (United States)

Kim, Kyoung-Nam; Lim, Youn-Hee; Bae, Hyun Joo; Kim, Myounghee; Jung, Kweon; Hong, Yun-Chul

2016-01-01

Background: Previous studies have associated short-term air pollution exposure with depression. Although an animal study showed an association between long-term exposure to particulate matter ≤ 2.5 μm (PM2.5) and depression, epidemiological studies assessing the long-term association are scarce. Objective: We aimed to determine the association between long-term PM2.5 exposure and major depressive disorder (MDD). Methods: A total of 27,270 participants 15–79 years of age who maintained an address within the same districts in Seoul, Republic of Korea, throughout the entire study period (between 2002 and 2010) and without a previous MDD diagnosis were analyzed. We used three district-specific exposure indices as measures of long-term PM2.5 exposure. Cox proportional hazards models adjusted for potential confounding factors and measured at district and individual levels were constructed. We further conducted stratified analyses according to underlying chronic diseases such as diabetes mellitus, cardiovascular disease, and chronic obstructive pulmonary disease. Results: The risk of MDD during the follow-up period (2008–2010) increased with an increase of 10 μg/m3 in PM2.5 in 2007 [hazard ratio (HR) = 1.44; 95% CI: 1.17, 1.78], PM2.5 between 2007 and 2010 (HR = 1.59; 95% CI: 1.02, 2.49), and 12-month moving average of PM2.5 until an event or censor (HR = 1.47; 95% CI: 1.14, 1.90). The association between long-term PM2.5 exposure and MDD was greater in participants with underlying chronic diseases than in participants without these diseases. Conclusion: Long-term PM2.5 exposure increased the risk of MDD among the general population. Individuals with underlying chronic diseases are more vulnerable to long-term PM2.5 exposure. Citation: Kim KN, Lim YH, Bae HJ, Kim M, Jung K, Hong YC. 2016. Long-term fine particulate matter exposure and major depressive disorder in a community-based urban cohort. Environ Health Perspect 124:1547–1553; http://dx.doi.org/10

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.

Long-term respiratory health effects in textile workers.

Science.gov (United States)

Lai, Peggy S; Christiani, David C

2013-03-01

Over 60 million people worldwide work in the textile or clothing industry. Recent studies have recognized the contribution of workplace exposures to chronic lung diseases, in particular chronic obstructive pulmonary disease (COPD). Early studies in textile workers have focused on the relationship between hemp or cotton dust exposure and the development of a syndrome termed byssinosis. The purpose of this review is to evaluate the effect of long-term exposure to organic dust in textile workers on chronic respiratory disease in the broader context of disease classifications, such as reversible or irreversible obstructive lung disease (i.e. asthma or COPD), and restrictive lung disease. Cessation of exposure to cotton dust leads to improvement in lung function. Recent animal models have suggested a shift in the lung macrophage:dendritic cell population ratio as a potential mechanistic explanation for persistent inflammation in the lung due to repeated cotton dust-related endotoxin exposure. Other types of textile dust, such as silk, may contribute to COPD in textile workers. Textile dust-related obstructive lung disease has characteristics of both asthma and COPD. Significant progress has been made in the understanding of chronic lung disease due to organic dust exposure in textile workers.

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.

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.

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...

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

Prognostic Factors and Decision Tree for Long-term Survival in Metastatic Uveal Melanoma.

Science.gov (United States)

Lorenzo, Daniel; Ochoa, María; Piulats, Josep Maria; Gutiérrez, Cristina; Arias, Luis; Català, Jaum; Grau, María; Peñafiel, Judith; Cobos, Estefanía; Garcia-Bru, Pere; Rubio, Marcos Javier; Padrón-Pérez, Noel; Dias, Bruno; Pera, Joan; Caminal, Josep Maria

2017-12-04

The purpose of this study was to demonstrate the existence of a bimodal survival pattern in metastatic uveal melanoma. Secondary aims were to identify the characteristics and prognostic factors associated with long-term survival and to develop a clinical decision tree. The medical records of 99 metastatic uveal melanoma patients were retrospectively reviewed. Patients were classified as either short (≤ 12 months) or long-term survivors (> 12 months) based on a graphical interpretation of the survival curve after diagnosis of the first metastatic lesion. Ophthalmic and oncological characteristics were assessed in both groups. Of the 99 patients, 62 (62.6%) were classified as short-term survivors, and 37 (37.4%) as long-term survivors. The multivariate analysis identified the following predictors of long-term survival: age ≤ 65 years (p=0.012) and unaltered serum lactate dehydrogenase levels (p=0.018); additionally, the size (smaller vs. larger) of the largest liver metastasis showed a trend towards significance (p=0.063). Based on the variables significantly associated with long-term survival, we developed a decision tree to facilitate clinical decision-making. The findings of this study demonstrate the existence of a bimodal survival pattern in patients with metastatic uveal melanoma. The presence of certain clinical characteristics at diagnosis of distant disease is associated with long-term survival. A decision tree was developed to facilitate clinical decision-making and to counsel patients about the expected course of disease.

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.

Long-term mortality and causes of death associated with Staphylococcus aureus bacteremia. A matched cohort study.

Science.gov (United States)

Gotland, N; Uhre, M L; Mejer, N; Skov, R; Petersen, A; Larsen, A R; Benfield, T

2016-10-01

Data describing long-term mortality in patients with Staphylococcus aureus bacteremia (SAB) is scarce. This study investigated risk factors, causes of death and temporal trends in long-term mortality associated with SAB. Nationwide population-based matched cohort study. Mortality rates and ratios for 25,855 cases and 258,547 controls were analyzed by Poisson regression. Hazard ratio of death was computed by Cox proportional hazards regression analysis. The majority of deaths occurred within the first year of SAB (44.6%) and a further 15% occurred within the following 2-5 years. The mortality rate was 14-fold higher in the first year after SAB and 4.5-fold higher overall for cases compared to controls. Increasing age, comorbidity and hospital contact within 90 days of SAB was associated with an increased risk of death. The overall relative risk of death decreased gradually by 38% from 1992-1995 to 2012-2014. Compared to controls, SAB patients were more likely to die from congenital malformation, musculoskeletal/skin disease, digestive system disease, genitourinary disease, infectious disease, endocrine disease, injury and cancer and less likely to die from respiratory disease, nervous system disease, unknown causes, psychiatric disorders, cardiovascular disease and senility. Over time, rates of death decreased or were stable for all disease categories except for musculoskeletal and skin disease where a trend towards an increase was seen. Long-term mortality after SAB was high but decreased over time. SAB cases were more likely to die of eight specific causes of death and less likely to die of five other causes of death compared to controls. Causes of death decreased for most disease categories. Risk factors associated with long-term mortality were similar to those found for short-term mortality. To improve long-term survival after SAB, patients should be screened for comorbidity associated with SAB. Copyright © 2016 The British Infection Association. Published by

Human acid alpha-glucosidase from rabbit milk has therapeutic effect in mice with glycogen storage disease type II

NARCIS (Netherlands)

A.G.A. Bijvoet (Agnes); A.J.J. Reuser (Arnold); H. van Hirtum (Hans); M.A. Kroos (Marian); E.H. van de Kamp; O. Schoneveld; P. Visser (Pim); J.P. Brakenhoff (Just); M. Weggeman (Miranda); E.J.J.M. van Corven (Emiel); A.T. van der Ploeg (Ans)

1999-01-01

textabstractPompe's disease or glycogen storage disease type II (GSDII) belongs to the family of inherited lysosomal storage diseases. The underlying deficiency of acid alpha-glucosidase leads in different degrees of severity to glycogen storage in heart, skeletal

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

Long-term survival in small-cell lung cancer

DEFF Research Database (Denmark)

Lassen, U; Osterlind, K; Hansen, M

1995-01-01

PURPOSE: To describe in patients with small-cell lung cancer (SCLC) the characteristics of those who survive for > or = 5 years, to identify long-term prognostic factors, to analyze survival data of 5-year survivors, and to study 10-year survival in patients entered before 1981. PATIENTS......, especially tobacco-related cancers and other tobacco-related diseases....

Acute and long-term survival in chronically critically ill surgical patients: a retrospective observational study.

Science.gov (United States)

Hartl, Wolfgang H; Wolf, Hilde; Schneider, Christian P; Küchenhoff, Helmut; Jauch, Karl-Walter

2007-01-01

Various cohort studies have shown that acute (short-term) mortality rates in unselected critically ill patients may have improved during the past 15 years. Whether these benefits also affect acute and long-term prognosis in chronically critically ill patients is unclear, as are determinants relevant to prognosis. We conducted a retrospective analysis of data collected from March 1993 to February 2005. A cohort of 390 consecutive surgical patients requiring intensive care therapy for more than 28 days was analyzed. The intensive care unit (ICU) survival rate was 53.6%. Survival rates at one, three and five years were 61.8%, 44.7% and 37.0% among ICU survivors. After adjustment for relevant covariates, acute and long-term survival rates did not differ significantly between 1993 to 1999 and 1999 to 2005 intervals. Acute prognosis was determined by disease severity during ICU stay and by primary diagnosis. However, only the latter was independently associated with long-term prognosis. Advanced age was an independent prognostic determinant of poor short-term and long-term survival. Acute and long-term prognosis in chronically critically ill surgical patients has remained unchanged throughout the past 12 years. After successful surgical intervention and intensive care, long-term outcome is reasonably good and is mainly determined by age and underlying disease.

Long term mortality study of chromate pigment workers who suffered lead poisoning

Energy Technology Data Exchange (ETDEWEB)

Davies, J.M.

1984-05-01

Long term mortality was studied in a group of 57 chromate pigment workers who suffered clinical lead poisoning, mostly between 1930 and 1945. One death was attributed to lead poisoning and there were significant excesses of deaths from nephritis (observed/expected 3/0.24) and cerebrovascular disease (9/2.20), as well as non-significant excesses for respiratory diseases (7/3.59) and accidents and violence (3/1.13). The deaths from nephritis followed long spells of service exceeding 10 years. Poisoning appeared to have more adverse long term effects on older workers: 15 men aged 40 or over at the time of acute poisoning experienced generally high mortality, and 30 years later or by the end of 1981 only two survived instead of the seven expected. The risk of cerebrovascular disease appeared to be unrelated to duration of exposure and affected even men employed for under one year. Excluding the 57 lead poisoned men, other contemporary workers at the factories showed no excess mortality from cerebrovascular disease.

Preeclampsia: long-term consequences for vascular health

Directory of Open Access Journals (Sweden)

Amaral LM

2015-07-01

Full Text Available Lorena M Amaral, Mark W Cunningham Jr, Denise C Cornelius, Babbette LaMarca Department of Pharmacology, University of Mississippi Medical Center, Jackson, MS, USA Abstract: Preeclampsia (PE is a pregnancy-specific syndrome and one of the leading causes of preterm birth, neonatal and maternal morbidity and mortality. This disease is characterized by new onset hypertension usually in the third trimester of pregnancy and is sometimes associated with proteinuria, although proteinuria is not a requirement for the diagnosis of PE. In developing countries, women have a higher risk of death due to PE than more affluent countries and one of the most frequent causes of death is high blood pressure and stroke. Although PE only affects approximately 2%–8% of pregnancies worldwide it is associated with severe complications such as eclampsia, hemorrhagic stroke, hemolysis, elevated liver enzymes and low platelets (HELLP syndrome, renal failure and pulmonary edema. Importantly, there is no “cure” for the disease except for early delivery of the baby and placenta, leaving PE a health care risk for babies born from PE moms. In addition, PE is linked to the development of cardiovascular disease and stroke in women after reproductive age, leaving PE a risk factor for long-term health in women. This review will highlight factors implicated in the pathophysiology of PE that may contribute to long-term effects in women with preeclamptic pregnancies. Keywords: preeclampsia, endothelial dysfunction, AT1-AA, CD4+ T helper cells

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.

Long-term survival and causes of death after stroke

DEFF Research Database (Denmark)

Brønnum-Hansen, Henrik; Davidsen, M; Thorvaldsen, P

2001-01-01

As part of the Danish contribution to the World Health Organization (WHO) MONICA (Monitoring Trends and Determinants in Cardiovascular Disease) Project, a register of patients with stroke was established in 1982. The purpose of the present study was to analyze long-term survival and causes of death...

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.)

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.)

Muscle MRI of classic infantile pompe patients: Fatty substitution and edema-like changes.

Science.gov (United States)

Pichiecchio, Anna; Rossi, Marta; Cinnante, Claudia; Colafati, Giovanna Stefania; De Icco, Roberto; Parini, Rossella; Menni, Francesca; Furlan, Francesca; Burlina, Alberto; Sacchini, Michele; Donati, Maria Alice; Fecarotta, Simona; Casa, Roberto Della; Deodato, Federica; Taurisano, Roberta; Di Rocco, Maja

2017-06-01

The aim of this study was to evaluate the muscle MRI pattern of 9 patients (median age: 6.5 ± 2.74 years) affected by classic infantile-onset Pompe disease who were treated with enzyme replacement therapy. We performed and qualitatively scored T1-weighted (T1-w) sequences of the facial, shoulder girdle, paravertebral, and lower limb muscles and short-tau inversion recovery (STIR) sequences of the lower limbs using the Mercuri and Morrow scales, respectively. On T1-w images, mild (grade 1) or moderate (grade 2) involvement was found in the tongue in 6 of 6 patients and in the adductor magnus muscle in 6 of 9. STIR hyperintensity was detected in all areas examined and was categorized as limited to mild in 5 of 8 patients. On T1-w sequences, mild/moderate adipose substitution in the adductor magnus and tongue muscles was documented. STIR edema-like alterations of thigh and calf muscles are novel findings. Correlations with biopsy findings and clinical parameters are needed to fully understand these findings. Muscle Nerve 55: 841-848, 2017. © 2016 Wiley Periodicals, Inc.

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.

Long-term mortality after IPD and bacteremic versus non-bacteremic pneumococcal pneumonia

NARCIS (Netherlands)

Wagenvoort, Gertjan H. J.; Sanders, Elisabeth A. M.; de Melker, Hester E.; van der Ende, Arie; Vlaminckx, Bart J.; Knol, Mirjam J.

2017-01-01

Short-term mortality after invasive pneumococcal disease (IPD) and pneumococcal pneumonia is high but data on long-term mortality (including the comparison between bacteremic and non-invasive/non-bacteremic pneumococcal pneumonia) within the first years after diagnosis are scarce. Adult patients

The long-term health of vegetarians and vegans.

Science.gov (United States)

Appleby, Paul N; Key, Timothy J

2016-08-01

Vegetarians, who do not eat any meat, poultry or fish, constitute a significant minority of the world's population. Lacto-ovo-vegetarians consume dairy products and/or eggs, whereas vegans do not eat any foods derived wholly or partly from animals. Concerns over the health, environmental and economic consequences of a diet rich in meat and other animal products have focussed attention on those who exclude some or all of these foods from their diet. There has been extensive research into the nutritional adequacy of vegetarian diets, but less is known about the long-term health of vegetarians and vegans. We summarise the main findings from large cross-sectional and prospective cohort studies in western countries with a high proportion of vegetarian participants. Vegetarians have a lower prevalence of overweight and obesity and a lower risk of IHD compared with non-vegetarians from a similar background, whereas the data are equivocal for stroke. For cancer, there is some evidence that the risk for all cancer sites combined is slightly lower in vegetarians than in non-vegetarians, but findings for individual cancer sites are inconclusive. Vegetarians have also been found to have lower risks for diabetes, diverticular disease and eye cataract. Overall mortality is similar for vegetarians and comparable non-vegetarians, but vegetarian groups compare favourably with the general population. The long-term health of vegetarians appears to be generally good, and for some diseases and medical conditions it may be better than that of comparable omnivores. Much more research is needed, particularly on the long-term health of vegans.

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

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

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

Long-term air pollution exposure and cardio- respiratory mortality: a review

Science.gov (United States)

2013-01-01

Current day concentrations of ambient air pollution have been associated with a range of adverse health effects, particularly mortality and morbidity due to cardiovascular and respiratory diseases. In this review, we summarize the evidence from epidemiological studies on long-term exposure to fine and coarse particles, nitrogen dioxide (NO2) and elemental carbon on mortality from all-causes, cardiovascular disease and respiratory disease. We also summarize the findings on potentially susceptible subgroups across studies. We identified studies through a search in the databases Medline and Scopus and previous reviews until January 2013 and performed a meta-analysis if more than five studies were available for the same exposure metric. There is a significant number of new studies on long-term air pollution exposure, covering a wider geographic area, including Asia. These recent studies support associations found in previous cohort studies on PM2.5. The pooled effect estimate expressed as excess risk per 10 μg/m3 increase in PM2.5 exposure was 6% (95% CI 4, 8%) for all-cause and 11% (95% CI 5, 16%) for cardiovascular mortality. Long-term exposure to PM2.5 was more associated with mortality from cardiovascular disease (particularly ischemic heart disease) than from non-malignant respiratory diseases (pooled estimate 3% (95% CI −6, 13%)). Significant heterogeneity in PM2.5 effect estimates was found across studies, likely related to differences in particle composition, infiltration of particles indoors, population characteristics and methodological differences in exposure assessment and confounder control. All-cause mortality was significantly associated with elemental carbon (pooled estimate per 1 μg/m3 6% (95% CI 5, 7%)) and NO2 (pooled estimate per 10 μg/m3 5% (95% CI 3, 8%)), both markers of combustion sources. There was little evidence for an association between long term coarse particulate matter exposure and mortality, possibly due to the small number of

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.

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.

Preeclampsia; short and long-term consequences for mother and neonate.

Science.gov (United States)

Bokslag, Anouk; van Weissenbruch, Mirjam; Mol, Ben Willem; de Groot, Christianne J M

2016-11-01

Preeclampsia is a common pregnancy specific disease, that presents with hypertension and a variety of organ failures, including malfunction of kidneys, liver and lungs. At present, the only definitive treatment of preeclampsia is end the pregnancy and deliver the neonate and placenta. For women with mild preeclampsia in the preterm phase of pregnancy, expectant management is generally indicated to improve fetal maturity, often requiring maternal medical treatment. Last decades, more evidence is available that the underlying mechanism of preeclampsia, endothelial disease, is not limited to pregnancy but increases cardiovascular risk in later life. In this review, we present the most recent insight in preeclampsia with focus on impact on the fetus, short and long-term outcome of offspring's, and long-term outcome of women with a history of preeclampsia. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

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.

Long term potentiation, but not depression, in interlamellar hippocampus CA1.

Science.gov (United States)

Sun, Duk-Gyu; Kang, Hyeri; Tetteh, Hannah; Su, Junfeng; Lee, Jihwan; Park, Sung-Won; He, Jufang; Jo, Jihoon; Yang, Sungchil; Yang, Sunggu

2018-03-26

Synaptic plasticity in the lamellar CA3 to CA1 circuitry has been extensively studied while interlamellar CA1 to CA1 connections have not yet received much attention. One of our earlier studies demonstrated that axons of CA1 pyramidal neurons project to neighboring CA1 neurons, implicating information transfer along a longitudinal interlamellar network. Still, it remains unclear whether long-term synaptic plasticity is present within this longitudinal CA1 network. Here, we investigate long-term synaptic plasticity between CA1 pyramidal cells, using in vitro and in vivo extracellular recordings and 3D holography glutamate uncaging. We found that the CA1-CA1 network exhibits NMDA receptor-dependent long-term potentiation (LTP) without direction or layer selectivity. By contrast, we find no significant long-term depression (LTD) under various LTD induction protocols. These results implicate unique synaptic properties in the longitudinal projection suggesting that the interlamellar CA1 network could be a promising structure for hippocampus-related information processing and brain diseases.

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.

Long-term studies of the natural history of asthma in childhood

DEFF Research Database (Denmark)

Bisgaard, Hans; Bønnelykke, Klaus

2010-01-01

Segmentation of children with asthma and other wheezy disorders remains the main research challenge today, as it was when described 2 centuries ago. Early childhood wheezy disorders follow different temporal trajectories, probably representing different underlying mechanisms (endophenotypes...... secondary prevention through the use of inhaled corticosteroids can effectively halt the long-term disease progression in childhood. In conclusion, the natural history of asthma and the associated airway changes is still poorly understood, and we have not managed to translate findings from long-term studies......). Prospective identification of endophenotypes allowing accurate prediction of the clinical course is currently not possible. The variability of the clinical course remains an enigma and difficult to predict. Three of 4 school-aged children with asthma have outgrown disease by midadulthood. The risk...

A conceptual framework for a long-term economic model for the treatment of attention-deficit/hyperactivity disorder.

Science.gov (United States)

Nagy, Balázs; Setyawan, Juliana; Coghill, David; Soroncz-Szabó, Tamás; Kaló, Zoltán; Doshi, Jalpa A

2017-06-01

Models incorporating long-term outcomes (LTOs) are not available to assess the health economic impact of attention-deficit/hyperactivity disorder (ADHD). Develop a conceptual modelling framework capable of assessing long-term economic impact of ADHD therapies. Literature was reviewed; a conceptual structure for the long-term model was outlined with attention to disease characteristics and potential impact of treatment strategies. The proposed model has four layers: i) multi-state short-term framework to differentiate between ADHD treatments; ii) multiple states being merged into three core health states associated with LTOs; iii) series of sub-models in which particular LTOs are depicted; iv) outcomes collected to be either used directly for economic analyses or translated into other relevant measures. This conceptual model provides a framework to assess relationships between short- and long-term outcomes of the disease and its treatment, and to estimate the economic impact of ADHD treatments throughout the course of the disease.

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.

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.

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

Long-term associative learning predicts verbal short-term memory performance.

Science.gov (United States)

Jones, Gary; Macken, Bill

2018-02-01

Studies using tests such as digit span and nonword repetition have implicated short-term memory across a range of developmental domains. Such tests ostensibly assess specialized processes for the short-term manipulation and maintenance of information that are often argued to enable long-term learning. However, there is considerable evidence for an influence of long-term linguistic learning on performance in short-term memory tasks that brings into question the role of a specialized short-term memory system separate from long-term knowledge. Using natural language corpora, we show experimentally and computationally that performance on three widely used measures of short-term memory (digit span, nonword repetition, and sentence recall) can be predicted from simple associative learning operating on the linguistic environment to which a typical child may have been exposed. The findings support the broad view that short-term verbal memory performance reflects the application of long-term language knowledge to the experimental setting.

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.

Long-term survival and function after suspected gram-negative sepsis.

Science.gov (United States)

Perl, T M; Dvorak, L; Hwang, T; Wenzel, R P

1995-07-26

To determine the long-term (> 3 months) survival of septic patients, to develop mathematical models that predict patients likely to survive long-term, and to measure the health and functional status of surviving patients. A large tertiary care university hospital and an associated Veterans Affairs Medical Center. From December 1986 to December 1990, a total of 103 patients with suspected gram-negative sepsis entered a double-blind, placebo-controlled efficacy trial of monoclonal antiendotoxin antibody. Of these, we followed up 100 patients for 7667 patient-months. Beginning in May 1992, we reviewed hospital records and contacted all known survivors. We measured the health status of all surviving patients. The determinants of long-term survival (up to 6 years) were identified through two Cox proportional hazard regression models: one that included patient characteristics identified at the time of sepsis (bedside model) and another that included bedside, infection-related, and treatment characteristics (overall model). Of the 60 patients in the cohort who died at a median interval of 30.5 days after sepsis, 32 died within the first month of the septic episode, seven died within 3 months, and four more died within 6 months. In the bedside multivariate model constructed to predict long-term survival, large hazard ratios (HRs) were associated with severity of underlying illness as classified by McCabe and Jackson criteria (for rapidly fatal disease, HR = 30.4, P respiratory distress syndrome (HR = 2.3; P = .02) predicted patients most likely to die. The Acute Physiology and Chronic Health Evaluation II score was not a significant predictor of outcome when either model included the simpler McCabe and Jackson classification of underlying disease severity. We compared the health status scores with norms for the general population and found that patients with resolved sepsis reported more physical dysfunction (P bedridden), suggesting that the patients' physical function

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

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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

Long-Term Refugee Health: Health Behaviors and Outcomes of Cambodian Refugee and Immigrant Women

Science.gov (United States)

Nelson-Peterman, Jerusha L.; Toof, Robin; Liang, Sidney L.; Grigg-Saito, Dorcas C.

2015-01-01

Refugees in the United States have high rates of chronic disease. Both long-term effects of the refugee experience and adjustment to the U.S. health environment may contribute. While there is significant research on health outcomes of newly resettled refugees and long-term mental health experiences of established refugees, there is currently…

Impact of previous vascular burden on in-hospital and long-term mortality in patients with ST-segment elevation myocardial infarction.

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Consuegra-Sánchez, Luciano; Melgarejo-Moreno, Antonio; Galcerá-Tomás, José; Alonso-Fernández, Nuria; Díaz-Pastor, Angela; Escudero-García, Germán; Jaulent-Huertas, Leticia; Vicente-Gilabert, Marta

2014-06-01

Patients with a current acute coronary syndrome and previous ischemic heart disease, peripheral arterial disease, and/or cerebrovascular disease are reported to have a poorer outcome than those without these previous conditions. It is uncertain whether this association with outcome is observed at long-term follow-up. Prospective observational study, including 4247 patients with ST-segment elevation myocardial infarction. Detailed clinical data and information on previous ischemic heart disease, peripheral arterial disease, and cerebrovascular disease ("vascular burden") were recorded. Multivariate models were performed for in-hospital and long-term (median, 7.2 years) all-cause mortality. One vascular territory was affected in 1131 (26.6%) patients and ≥ 2 territories in 221 (5.2%). The total in-hospital mortality rate was 12.3% and the long-term incidence density was 3.5 deaths per 100 patient-years. A background of previous ischemic heart disease (odds ratio = 0.83; P = .35), peripheral arterial disease (odds ratio = 1.30; P = .34), or cerebrovascular disease (stroke) (odds ratio = 1.15; P = .59) was not independently predictive of in-hospital death. In an adjusted model, previous cerebrovascular disease and previous peripheral arterial disease were both predictors of mortality at long-term follow-up (hazard ratio = 1.57; P cerebrovascular disease and peripheral arterial disease were predictors of mortality at long-term after hospital discharge. Copyright © 2013 Sociedad Española de Cardiología. Published by Elsevier Espana. All rights reserved.

Bentall operation in 375 patients: long-term results and predictors of death.

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Varrica, Alessandro; Satriano, Angela; de Vincentiis, Carlo; Biondi, Andrea; Trimarchi, Santi; Ranucci, Marco; Menicanti, Lorenzo; Frigiola, Alessandro

2014-01-01

The Bentall operation is a 40-year-old standardized procedure for treating aortic valve diseases and aneurysms involving the aortic root. The study aim was to analyze the results and predictors of long-term outcome after the Bentall procedure for aortic root diseases. Between January 1990 and December 2007, a total of 375 patients (296 males, 79 females) underwent the Bentall operation at the authors' institution. Bicuspid aortic valve (BAV) was present in 91 patients, and Marfan syndrome in 13. Thirty-six patients were treated as emergencies, and 30 for acute dissection. A concomitant surgical procedure was performed in 78 patients. The operative procedure included both classic Bentall and button techniques. Follow up data were obtained from hospital and office records and from telephone contacts. Kaplan-Meier survival analysis and Cox regression analysis were performed to investigate the predictors of long-term outcome. The overall in-hospital mortality was 4.5%, and after elective operations was 2.3%. A 20-year long-term follow up included 32 late deaths, of which 14 were cardiac-related. Freedom from late all-cause mortality at 5, 10, and 15 years was 97.1%, 81.9%, and 53.9%, respectively. At univariate analysis, long-term mortality was associated with age, diabetes, BAV, NYHA class III/IV, emergency treatment, cardiopulmonary bypass time, and coronary artery bypass grafting. Independent predictors of long-term mortality were age (OR 1.16; CI: 1.08-1.23), emergency surgery (OR 28; CI: 4-192) and BAV (OR 3; CI: 1.3-6.9). The Bentall procedure is a safe and durable operation, with a very good early and long-term results and a low rate of reoperation. In the present series, age, BAV and emergency surgery were important independent predictors of mortality.

Long-term results after repair of ruptured and non-ruptured abdominal aortic aneurysm

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Kuzmanović Ilija B.

2004-01-01

Full Text Available INTRODUCTION Abdominal aortic aneurysm can be repaired by elective procedure while asymptomatic, or immediately when it is complicated - mostly due to rupture. Treating abdominal aneurysm electively, before it becomes urgent, has medical and economical reason. Today, the first month mortality after elective operations of the abdominal aorta aneurysm is less than 3%; on the other hand, significant mortality (25%-70% has been recorded in patients operated immediately because of rupture of the abdominal aneurysm. In addition, the costs of elective surgical treatment are significantly lower. OBJECTIVE The objective of this study is to compare long-term survival of patients that underwent elective or immediate repair of abdominal aortic aneurysm (due to rupture, and to find out the factors influencing the long-term survival of these patients. MATERIAL AND METHODS Through retrospective review of prospectively collected data of the Institute for Cardiovascular Diseases of Clinical Center of Serbia, Belgrade, 56 patients that had elective surgery and 35 patients that underwent urgent operation due to rupture of abdominal aneurysm were followed up. Only the patients that survived 30 postoperative days were included in this review, and were followed up (ranging from 2 to 126 months. Electively operated patients were followed during 58.82 months on the average (range 7 to 122, and urgently operated were followed over 52.26 months (range 2 to 126. There was no significant difference of the length of postoperative follow-up between these two groups. RESULTS During this period, out of electively operated and immediately operated patients, 27 and 22 cases died, respectively. There was no significant difference (p>0,05a of long-term survival between these two groups. Obesity and early postoperative complications significantly decreased long-term survival of both electively and immediately operated patients. Graft infection, ventral hernia, aneurysm of

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...

[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.

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-

Long-term outcomes of nasopharyngectomy using partial maxillectomy approach.

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Ng, Li Shia; Lim, Chwee Ming; Loh, Kwok Seng

2016-05-01

To determine the long-term outcomes of a prospective cohort of 20 patients who had nasopharyngectomy using an open partial-maxillectomy approach. A prospective cohort study of the surgical outcomes of patients with recurrent T1 (rT1) and rT2 nasopharyngeal carcinoma recurrence. There were 14 male and six female patients. The mean age was 49 years. The mean duration of follow-up was 60.4 months. All of the surgical margins were free of malignancy. Ten patients (50%) developed recurrences. The 5-year local control rate, disease-free, disease-specific, and overall survivals were 70%, 48.9%, 70.2%, and 66.7%, respectively. There were no major intraoperative complications. The most common morbidity was pain. The median length of hospitalization was 4 days. Early local recurrence of nasopharyngeal carcinoma can be treated surgically using a partial maxillectomy approach. The long-term outcomes are similar to nasopharyngectomy performed using the more common approaches. 4. Laryngoscope, 126:1103-1107, 2016. © 2015 The American Laryngological, Rhinological and Otological Society, Inc.

Cognition, Emotional Health, and Immunological Markers in Children With Long-Term Nonprogressive HIV

NARCIS (Netherlands)

Paul, Robert; Apornpong, Tanakorn; Prasitsuebsai, Wasana; Puthanakit, Thanyawee; Saphonn, Vonthanak; Aurpibul, Linda; Kosalaraksa, Pope; Kanjanavanit, Suparat; Luesomboon, Wicharn; Ngampiyaskul, Chaiwat; Suwanlerk, Tulathip; Chettra, Kea; Shearer, William T.; Valcour, Victor; Ananworanich, Jintanat; Kerr, Stephen

2018-01-01

HIV-infected children with long-term nonprogressive (LTNP) disease eventually convert to a progressive disease type, yet the extent to which these children experience the cognitive and emotional symptoms observed in typical progressive HIV (Progressors) is unknown. Eighty-eight LTNPs, 53

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

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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.

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

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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.

Reforming Long-Term Care Funding in Alberta.

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Crump, R Trafford; Repin, Nadya; Sutherland, Jason M

2015-01-01

Like many provinces across Canada, Alberta is facing growing demand for long-term care. Issues with the mixed funding model used to pay long-term care providers had Alberta Health Services concerned that it was not efficiently meeting the demand for long-term care. Consequently, in 2010, Alberta Health Services introduced the patient/care-based funding (PCBF) model. PCBF is similar to activity-based funding in that it directly ties the complexity and care needs of long-term care residents to the payment received by long-term care providers. This review describes PCBF and discusses some of its strengths and weaknesses. In doing so, this review is intended to inform other provinces faced with similar long-term care challenges and contemplating their own funding reforms.

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...

Adjuvant psychological therapy in long-term endocrine conditions.

Science.gov (United States)

Daniels, J; Turner-Cobb, J M

2017-06-01

Consideration of psychological distress in long-term endocrine conditions is of vital importance given the prevalence of anxiety and depression in such disorders. Poor mental health can lead to compromised self-care, higher utilization of health services, lower rates of adherence, reduced quality of life and ultimately poorer outcomes. Adjuvant psychological therapy offers an effective resource to reduce distress in endocrine conditions. While the vast majority of work in this area has focused on psychological screening and intervention in diabetes, identification and recognition of psychological distress are equally important in other endocrinological conditions, with supportive evidence in polycystic ovary syndrome and Addison's disease. Referral pathways and recommendations set out by UK guidelines and the Department of Health mandate requires greater attention across a wider range of long-term endocrine conditions to facilitate improved quality of life and health outcome. © 2017 John Wiley & Sons Ltd.

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

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

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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.

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.

[Long-term follow-up of patients with suprasellar germinomas].

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Bauditz, Juergen; Lochs, Herbert; Ventz, Manfred

2007-10-15

Suprasellar germinomas are rare intracranial neoplasms, which mainly occur in children and adolescents and manifest with endocrine symptoms and/or compression syndromes. The clinical, hormonal and morphological findings as well as treatment and complications were investigated in seven patients (six male, one female) with germinomas. Mean age at diagnosis was 19.7 years (range 15-32 years). First disease-related symptoms were diabetes insipidus (three patients), loss of libido (two patients), pseudopubertas praecox (one patient), and dwarfism (one patient). However, decisive symptoms leading to final diagnosis were visual disturbances (five patients), pubertas tarda (one patient), and hypogonadism (one patient). All patients were treated by transcranial radiation with a dose of 40-54 Gy. One patient received additional chemotherapy with cisplatin, etoposide, and ifosfamide (PEI). Patients were followed up for 14.6 years (range 7-27 years). Intracranial and pulmonary relapses were observed in two patients. Panhypopituitarism and diabetes insipidus were seen in all patients after treatment. Two patients suffered from loss of vision, two further patients from unilateral amaurosis. One patient developed epilepsy and persistent cognitive impairment. Long-term follow-up shows that two patients died from recurrent disease and decompensated liver cirrhosis, respectively. The other patients are long-term survivors. Full social integration with employment was possible in one case. Suprasellar germinomas cause endocrine symptoms during early tumor stages, however, diagnosis is generally established when ocular symptoms related to tumor compression are already present. Long-term survival is characterized by panhypopituitarism, diabetes insipidus and, partly, ocular or cerebral defects.

Industrial Foundations as Long-Term Owners

DEFF Research Database (Denmark)

Thomsen, Steen; Poulsen, Thomas; Børsting, Christa Winther

Short-termism has become a serious concern for corporate governance, and this has inspired a search for institutional arrangements to promote long-term decision-making. In this paper, we call attention to long-term ownership by industrial foundations, which is common in Northern Europe but little...... known in the rest of the world. We use a unique Danish data set to document that industrial foundations are long-term owners that practice long-term governance. We show that foundation ownership is highly stable compared to other ownership structures. Foundation-owned companies replace managers less...... frequently. They have conservative capital structures with low financial leverage. They score higher on an index of long-termism in finance, investment, and employment. They survive longer. Overall, our paper supports the hypothesis that corporate time horizons are influenced by ownership structures...

Long-term associative learning predicts verbal short-term memory performance

OpenAIRE

Jones, Gary; Macken, Bill

2017-01-01

Studies using tests such as digit span and nonword repetition have implicated short-term memory across a range of developmental domains. Such tests ostensibly assess specialized processes for the short-term manipulation and maintenance of information that are often argued to enable long-term learning. However, there is considerable evidence for an influence of long-term linguistic learning on performance in short-term memory tasks that brings into question the role of a specialized short-term...

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.

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

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.

Long-term course of orthostatic tremor in serial posturographic measurement.

Science.gov (United States)

Feil, K; Böttcher, N; Guri, F; Krafczyk, S; Schöberl, F; Zwergal, A; Strupp, M

2015-08-01

Primary orthostatic tremor (OT) is a rare neurological disease of unknown pathophysiology characterized by a high-frequency tremor mainly of the legs when standing. The aim of this study was to examine its long-term course by subjective estimation and objective recording by serial posturography and to obtain further standardized epidemiological and clinical data on patients with OT. A clinical cohort of 37 patients with the diagnosis of primary OT was screened for this longitudinal follow-up study. Eighteen patients consented to participate. During study visit all patients underwent a standardized neurological examination and completed subjective scales and scores. Posturographic recordings at follow-up were compared to prior clinical posturographic measurements in 15 cases. In our cohort the mean duration of symptoms was 14.1 ± 6.8 years. Subjectively, 78% of patients reported progression of the disease. Posturographic data (5.4 ± 4.0 years) revealed a significant increase of the total sway path (standing on firm ground with eyes open) from 2.4 ± 1.3 to 3.4 ± 1.4 m/min (p = 0.022) and of the total root mean square values from 9.8 ± 4.3 to 12.4 ± 4.8 mm (p = 0.028). None of these observations are explained by aging of the patients. Mean frequency of the tremor did not change over time (14.7 ± 1.9 Hz vs. 14.9 ± 2.0 Hz at follow-up). Clinically, most patients had signs of cerebellar dysfunction and a substantial portion also showed proprioceptive deficits in the long-term course. This long-term follow-up study indicates, that primary OT is a progressive disorder. Furthermore, the clinical observation of cerebellar dysfunction in most OT patients in the long-term course might indicate an important role of the cerebellum in its pathophysiology. Copyright © 2015 Elsevier Ltd. All rights reserved.

Bias due to withdrawal in long-term randomised trials in COPD

DEFF Research Database (Denmark)

Vestbo, Jørgen; Anderson, Julie Anne; Calverley, Peter Mark Anthony

2011-01-01

Randomised controlled trials (RCTs) are considered the least biased method for evaluating drug efficacy and several large long-term RCTs in chronic obstructive pulmonary disease have been published. These usually include drugs with symptomatic benefits and have significant withdrawal rates....

Radiotherapy in early-stage Dupuytren's contracture. Long-term results after 13 years

International Nuclear Information System (INIS)

Betz, Nicolas; Ott, Oliver J.; Sauer, Rolf; Fietkau, Rainer; Adamietz, Boris; Keilholz, Ludwig

2010-01-01

Background and Purpose: In early-stage Dupuytren's contracture, radiotherapy is applied to prevent disease progression. Long-term outcome and late toxicity of the treatment were evaluated in a retrospective analysis. Patients and Methods: Between 12/1982 and 02/2006, 135 patients (208 hands) were irradiated with orthovoltage (120 kV; 20 mA; 4-mm Al filter), in two courses with five daily fractions of 3.0 Gy to a total dose of 30 Gy; separated by a 6- to 8-week interval. The extent of disease was described according to a modified classification of Tubiana et al. Long-term outcome was analyzed at last follow-up between 02/2008 and 05/2008 with a median follow-up of 13 years (range, 2-25 years). Late treatment toxicity and objective reduction of symptoms as change in stage and numbers of nodules and cords were evaluated and used as evidence to assess treatment response. Results: According to the individual stages, 123 cases (59%) remained stable, 20 (10%) improved, and 65 (31%) progressed. In stage N 87% and in stage N/I 70% remained stable or even regressed. In more advanced stages, the rate of disease progression increased to 62% (stage I) or 86% (stage II). 66% of the patients showed a long-term relief of symptoms (i.e., burning sensations, itching and scratching, pressure and tension). Radiotherapy did not increase the complication rate after surgery in case of disease progression and only minor late toxicity (skin atrophy, dry desquamation) could be observed in 32% of the patients. There was no evidence for a second malignancy induced by radiotherapy. Conclusion: After a mean follow-up of 13 years radiotherapy is effective in prevention of disease progression and improves patients' symptoms in early-stage Dupuytren's contracture (stage N, N/I). In case of disease progression after radiotherapy, a ''salvage'' operation is still feasible. (orig.)

Long-term fundus changes in acquired partial lipodystrophy.

Science.gov (United States)

Jansen, Joyce; Delaere, Lien; Spielberg, Leigh; Leys, Anita

2013-11-18

We describe long-term fundus changes in a patient with partial lipodystrophy (PL). Retinal pigment alterations, drusen and subretinal neovascularisation were seen without evidence for membranoproliferative glomerulonephritis. Fundus alterations similar to those seen in age-related macular degeneration can occur at an earlier age in patients with PL, even without renal disease. Dysregulation of an alternative complement pathway with low serum levels of C3 has been implicated as a pathogenetic mechanism.

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.

Vertebral sarcoidosis: long-term follow-up with MRI

Energy Technology Data Exchange (ETDEWEB)

Lefere, M. [University Hospitals Gasthuisberg, Department of Radiology, Leuven (Belgium); Larbi, A.; Malghem, J.; Vande Berg, B.; Dallaudiere, B. [University Hospitals St Luc, Department of Radiology, Brussels (Belgium)

2014-08-15

Vertebral involvement in sarcoidosis is rare and its clinical and imaging features are non-specific. Indeed, because the lesions are hard to differentiate from metastatic disease based on imaging alone, a histological confirmation is advised. Fatty replacement is a well-known finding indicating stabilization and healing in both benign and malignant conditions. It can be used as an indicator of a favorable disease course and response to treatment. We report the case of a 43-year-old woman with multifocal vertebral sarcoidosis lesions and long-term follow-up showing progressive and gradual fatty involution on magnetic resonance imaging (MRI) during 4 years of steroid treatment with a final favorable outcome. (orig.)

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

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.

A fly-wheel drive with controlled-torque clutch for a reactors cooling circuit pumps; Entrainement des pompes du circuit de refrigeration d'un reacteur par volant a embrayage sous couple controle

Energy Technology Data Exchange (ETDEWEB)

Riettini, A [Commissariat a l' Energie Atomique, Grenoble (France). Centre d' Etudes Nucleaires

1964-10-15

After a theoretical study on the slowing down of a centrifugal pump, the motion equations have been checked by means of experimental tests. In order to have important slowing down times (which is the case of the cooling pumps of a research reactor) it is necessary to add a fly-wheel. To prevent troubles when starting, a block pump-fly-wheel with clutch under controlled torque was developed. It is so possible to start the fly-wheel progressively without increasing too much power of the driving motor. (author) [French] Apres une etude theorique sur le mouvement de ralentissement d'une pompe centrifuge, les equations du mouvement ont ete verifiees par des essais pratiques. Pour obtenir des temps de ralentissement importants (cas des pompes de refrigeration d'un reacteur de recherche) il est necessaire d'y adjoindre un volant d'inertie. Pour eviter les inconvenients au demarrage, on a etudie un ensemble pompe-volant avec embrayage sous couple controle. Cette solution permet de lancer progressivement le volant sans augmentation appreciable de la puissance du moteur d'entrainement. (auteur)

Pompe Disease

Science.gov (United States)

... enzyme performs its function in intracellular compartments called lysosomes. Lysosomes are known to function as cellular clearinghouses; they ... enzyme performs its function in intracellular compartments called lysosomes. Lysosomes are known to function as cellular clearinghouses; ...

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.

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.

Recent and long-term occupational noise exposure and salivary cortisol level

DEFF Research Database (Denmark)

Stokholm, Zara Ann; Hansen, Åse Marie; Grynderup, Matias Brødsgaard

2014-01-01

Environmental and occupational noise exposure have been related to increased risk of cardiovascular disease, hypothetically mediated by stress-activation of the hypothalamic-pituitary-adrenal (HPA) axis. The objective of this study was to investigate the relation between recent and long-term occu...

Annular electromagnetic pumps-construction and testing-theory, and comparison with experimental results; Pompes electromagnetiques annulaires - construction et essais - theorie et confrontation avec l'experience

Energy Technology Data Exchange (ETDEWEB)

Cambillard, E P; Schwab, B L [Commissariat a l' Energie Atomique, Fontenay-aux-Roses (France). Centre d' Etudes Nucleaires

1964-07-01

This report consists of three sections. The first is concerned with the description of different pumps which have been constructed, tests on these which have been completed and the results obtained. The second section presents a theoretical method for the determination of the coefficients, taking in account the break of the magnetic circuit. It is shown that the preliminary design calculations of the annular pumps can be made, neglecting the break of the magnetic circuit, by further assigning essential magnitudes (pressure, losses) with easily calculated coefficients. The third section of this report uses the theoretical bases exposed in the second section, and develop a new annular pump calculation method which takes-into account both the current out of balance and any type of winding. (authors) [French] Ce rapport comprend trois parties. La premiere est consacree a la description des differentes pompes construites, aux essais,effectues et aux resultats obtenus. La deuxieme partie expose une methode theorique de determination de coefficients tenant compte de l'ouverture du circuit magnetique. On montre que le calcul d'avant-projet des pompes annulaires peut s'effectuer comme si le circuit magnetique n'etait pas coupe, en affectant ensuite toutes les grandeurs essentielles (pression, pertes) de coefficients aisement calculables. La troisieme partie de ce rapport utilise les bases de la theorie exposee dans la deuxieme partie et developpe une nouvelle methode de calcul des pompes annulaires qui tient compte a la fois du desequilibre des courants et d'un bobinage quelconque. (auteurs)

Persistent negative illness perceptions despite long-term biochemical control of acromegaly: novel application of the drawing test.

Science.gov (United States)

Tiemensma, Jitske; Pereira, Alberto M; Romijn, Johannes A; Broadbent, Elizabeth; Biermasz, Nienke R; Kaptein, Adrian A

2015-05-01

Patients with acromegaly have persistent complaints despite long-term biochemical control. Drawings can be used to assess patients' perceptions about their disease. We aimed to explore the utility of the drawing test and its relation to illness perceptions and quality of life (QoL) in patients after long-term remission of acromegaly. A cross-sectional study was conducted to evaluate the utility of the drawing test. A total of 50 patients after long-term remission (mean±s.e.m., 16±1.2 years) of acromegaly were included in this study. Patients completed the drawing test (two retrospective drawings of their body perception before acromegaly and during the active phase of acromegaly, and one drawing on the current condition after long-term remission), Illness Perception Questionnaire-Revised, Physical Symptom Checklist, EuroQoL-5D, and AcroQoL. Patients perceived a dramatic change in body size during the active state of the disease compared with the healthy state before the awareness of acromegaly. Patients reported that their body did not completely return to the original proportions after long-term remission. In addition, larger drawings indicated more negative consequences (Pdrawings also indicated more impaired QoL, especially disease-specific QoL (all Pdrawing test, illness perceptions, and QoL. The drawing test appears to be a novel and relatively easy tool to assess the perception of patients after long-term remission of acromegaly. The assessment of drawings may enable health care providers to appreciate the perceptions of patients with long-term remission of acromegaly, and enable discussion of symptoms and remission. © 2015 European Society of Endocrinology.

Plasma Aluminum Concentrations in Pediatric Patients Receiving Long-Term Parenteral Nutrition.

Science.gov (United States)

Courtney-Martin, Glenda; Kosar, Christina; Campbell, Alison; Avitzur, Yaron; Wales, Paul W; Steinberg, Karen; Harrison, Debra; Chambers, Kathryn

2015-07-01

Patients receiving long-term parenteral nutrition (PN) are at increased risk of aluminium (Al) toxicity because of bypass of the gastrointestinal tract during PN infusion. Complications of Al toxicity include metabolic bone disease (MBD), Al-associated encephalopathy in adults, and impaired neurological development in preterm infants. Unlike the United States, there are no regulations regarding Al content of large- and small-volume parenterals in Canada. We, therefore, aimed to present our data on plasma Al concentration and Al intake from our cohort of pediatric patients receiving long-term PN. Plasma Al concentration was retrospectively gathered from the patient charts of all 27 patients with intestinal failure (IF) receiving long-term PN at The Hospital for Sick Children, Toronto, Canada, and compared with age- and sex-matched controls recruited for comparison. In addition, Al concentration was measured in PN samples collected from 10 randomly selected patients with IF and used to determine their Al intake. The plasma Al concentration of patients with IF receiving long-term PN was significantly higher than that of control participants (1195 ± 710 vs 142 ± 63 nmol/L; P Parenteral and Enteral Nutrition.

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

Huntingtin is critical both pre- and postsynaptically for long-term learning-related synaptic plasticity in Aplysia.

Directory of Open Access Journals (Sweden)

Yun-Beom Choi

Full Text Available Patients with Huntington's disease exhibit memory and cognitive deficits many years before manifesting motor disturbances. Similarly, several studies have shown that deficits in long-term synaptic plasticity, a cellular basis of memory formation and storage, occur well before motor disturbances in the hippocampus of the transgenic mouse models of Huntington's disease. The autosomal dominant inheritance pattern of Huntington's disease suggests the importance of the mutant protein, huntingtin, in pathogenesis of Huntington's disease, but wild type huntingtin also has been shown to be important for neuronal functions such as axonal transport. Yet, the role of wild type huntingtin in long-term synaptic plasticity has not been investigated in detail. We identified a huntingtin homolog in the marine snail Aplysia, and find that similar to the expression pattern in mammalian brain, huntingtin is widely expressed in neurons and glial cells. Importantly the expression of mRNAs of huntingtin is upregulated by repeated applications of serotonin, a modulatory transmitter released during learning in Aplysia. Furthermore, we find that huntingtin expression levels are critical, not only in presynaptic sensory neurons, but also in the postsynaptic motor neurons for serotonin-induced long-term facilitation at the sensory-to-motor neuron synapse of the Aplysia gill-withdrawal reflex. These results suggest a key role for huntingtin in long-term memory storage.

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

Compensation for PKMζ in long-term potentiation and spatial long-term memory in mutant mice.

Science.gov (United States)

Tsokas, Panayiotis; Hsieh, Changchi; Yao, Yudong; Lesburguères, Edith; Wallace, Emma Jane Claire; Tcherepanov, Andrew; Jothianandan, Desingarao; Hartley, Benjamin Rush; Pan, Ling; Rivard, Bruno; Farese, Robert V; Sajan, Mini P; Bergold, Peter John; Hernández, Alejandro Iván; Cottrell, James E; Shouval, Harel Z; Fenton, André Antonio; Sacktor, Todd Charlton

2016-05-17

PKMζ is a persistently active PKC isoform proposed to maintain late-LTP and long-term memory. But late-LTP and memory are maintained without PKMζ in PKMζ-null mice. Two hypotheses can account for these findings. First, PKMζ is unimportant for LTP or memory. Second, PKMζ is essential for late-LTP and long-term memory in wild-type mice, and PKMζ-null mice recruit compensatory mechanisms. We find that whereas PKMζ persistently increases in LTP maintenance in wild-type mice, PKCι/λ, a gene-product closely related to PKMζ, persistently increases in LTP maintenance in PKMζ-null mice. Using a pharmacogenetic approach, we find PKMζ-antisense in hippocampus blocks late-LTP and spatial long-term memory in wild-type mice, but not in PKMζ-null mice without the target mRNA. Conversely, a PKCι/λ-antagonist disrupts late-LTP and spatial memory in PKMζ-null mice but not in wild-type mice. Thus, whereas PKMζ is essential for wild-type LTP and long-term memory, persistent PKCι/λ activation compensates for PKMζ loss in PKMζ-null mice.

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...

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.

Dienogest in long-term treatment of endometriosis

Directory of Open Access Journals (Sweden)

Schindler AE

2011-07-01

Full Text Available Adolf E SchindlerInstitute for Medical Research and Education, Essen, GermanyAbstract: Endometriosis is a chronic disease primarily affecting women of childbearing age, in which endometriotic lesions form outside the uterus, typically leading to painful symptoms, fatigue, and infertility. The symptoms of endometriosis may cause significant impairment in quality of life and represent a substantial economic burden to patients, families, and society. There is no cure for endometriosis; management consists of alleviating pain and other symptoms, reducing endometriotic lesions, and improving quality of life. Recurrence after surgical intervention is common, while the clinical evidence to support the efficacy and safety of many medications currently used in endometriosis is limited. Dienogest is an oral progestin that has been investigated extensively in the treatment of endometriosis in two clinical programs performed in Europe and Japan, including dose-ranging, placebo-controlled, active comparator-controlled, and long-term (up to 65 weeks studies. These studies demonstrated that dienogest 2 mg daily effectively alleviates the painful symptoms of endometriosis, reduces endometriotic lesions, and improves indices of quality of life. Dienogest showed a favorable safety and tolerability profile in these studies, with predictable adverse effects, high rates of patient compliance, and low withdrawal rates. This review article describes the clinical trial evidence that characterizes the efficacy and safety of dienogest in endometriosis, including two studies characterizing dienogest in long-term use. The relevance of these findings to the management of endometriosis in clinical practice is discussed.Keywords: dienogest, endometriosis, progestins, long-term treatment, quality of life, symptoms, pain

Long-Term Results of Mitral Valve Repair

Directory of Open Access Journals (Sweden)

Francisco Diniz Affonso da Costa

Full Text Available Abstract Introduction: Current guidelines state that patients with severe mitral regurgitation should be treated in reference centers with a high reparability rate, low mortality rate, and durable results. Objective: To analyze our global experience with the treatment of organic mitral regurgitation from various etiologies operated in a single center. Methods: We evaluated all surgically treated patients with organic mitral regurgitation from 2004-2017. Patients were evaluated clinically and by echocardiography every year. We determined early and late survival rates, valve related events and freedom from recurrent mitral regurgitation and tricuspid regurgitation. Valve failure was defined as any mitral regurgitation ≥ moderate degree or the need for reoperation for any reason. Results: Out of 133 patients with organic mitral regurgitation, 125 (93.9% were submitted to valve repair. Mean age was 57±15 years and 52 patients were males. The most common etiologies were degenerative disease (73 patients and rheumatic disease (34 patients. Early mortality was 2.4% and late survival was 84.3% at 10 years, which are similar to the age- and gender-matched general population. Only two patients developed severe mitral regurgitation, and both were reoperated (95.6% at 10 years. Freedom from mitral valve failure was 84.5% at 10 years, with no difference between degenerative and rheumatic valves. Overall, late ≥ moderate tricuspid regurgitation was present in 34% of the patients, being more common in the rheumatic ones. The use of tricuspid annuloplasty abolished this complication. Conclusion: We have demonstrated that mitral regurgitation due to organic mitral valve disease from various etiologies can be surgically treated with a high repair rate, low early mortality and long-term survival that are comparable to the matched general population. Concomitant treatment of atrial fibrillation and tricuspid valve may be important adjuncts to optimize long-term

Causes of death in long-term lung cancer survivors: a SEER database analysis.

Science.gov (United States)

Abdel-Rahman, Omar

2017-07-01

Long-term (>5 years) lung cancer survivors represent a small but distinct subgroup of lung cancer patients and information about the causes of death of this subgroup is scarce. The Surveillance, Epidemiology and End Results (SEER) database (1988-2008) was utilized to determine the causes of death of long-term survivors of lung cancer. Survival analysis was conducted using Kaplan-Meier analysis and multivariate analysis was conducted using a Cox proportional hazard model. Clinicopathological characteristics and survival outcomes were assessed for the whole cohort. A total of 78,701 lung cancer patients with >5 years survival were identified. This cohort included 54,488 patients surviving 5-10 years and 24,213 patients surviving >10 years. Among patients surviving 5-10 years, 21.8% were dead because of primary lung cancer, 10.2% were dead because of other cancers, 6.8% were dead because of cardiac disease and 5.3% were dead because of non-malignant pulmonary disease. Among patients surviving >10 years, 12% were dead because of primary lung cancer, 6% were dead because of other cancers, 6.9% were dead because of cardiac disease and 5.6% were dead because of non-malignant pulmonary disease. On multivariate analysis, factors associated with longer cardiac-disease-specific survival in multivariate analysis include younger age at diagnosis (p death from primary lung cancer is still significant among other causes of death even 20 years after diagnosis of lung cancer. Moreover, cardiac as well as non-malignant pulmonary causes contribute a considerable proportion of deaths in long-term lung cancer survivors.

Toxicities Associated with Cisplatin-Based Chemotherapy and Radiotherapy in Long-Term Testicular Cancer Survivors

Directory of Open Access Journals (Sweden)

Chunkit Fung

2018-01-01

Full Text Available Testicular cancer has become the paradigm of adult-onset cancer survivorship, due to the young age at diagnosis and 10-year relative survival of 95%. This clinical review presents the current status of various treatment-related complications experienced by long-term testicular cancer survivors (TCS free of disease for 5 or more years after primary treatment. Cardiovascular disease and second malignant neoplasms represent the most common potentially life-threatening late effects. Other long-term adverse outcomes include neuro- and ototoxicity, pulmonary complications, nephrotoxicity, hypogonadism, infertility, and avascular necrosis. Future research efforts should focus on delineation of the genetic underpinning of these long-term toxicities to understand their biologic basis and etiopathogenetic pathways, with the goal of developing targeted prevention and intervention strategies to optimize risk-based care and minimize chronic morbidities. In the interim, health care providers should advise TCS to adhere to national guidelines for the management of cardiovascular disease risk factors, as well as to adopt behaviors consistent with a healthy lifestyle, including smoking cessation, a balanced diet, and a moderate to vigorous intensity exercise program. TCS should also follow national guidelines for cancer screening as currently applied to the general population.

Toxicities Associated with Cisplatin-Based Chemotherapy and Radiotherapy in Long-Term Testicular Cancer Survivors

Science.gov (United States)

Dinh, Paul; Ardeshir-Rouhani-Fard, Shirin; Fossa, Sophie D.; Travis, Lois B.

2018-01-01

Testicular cancer has become the paradigm of adult-onset cancer survivorship, due to the young age at diagnosis and 10-year relative survival of 95%. This clinical review presents the current status of various treatment-related complications experienced by long-term testicular cancer survivors (TCS) free of disease for 5 or more years after primary treatment. Cardiovascular disease and second malignant neoplasms represent the most common potentially life-threatening late effects. Other long-term adverse outcomes include neuro- and ototoxicity, pulmonary complications, nephrotoxicity, hypogonadism, infertility, and avascular necrosis. Future research efforts should focus on delineation of the genetic underpinning of these long-term toxicities to understand their biologic basis and etiopathogenetic pathways, with the goal of developing targeted prevention and intervention strategies to optimize risk-based care and minimize chronic morbidities. In the interim, health care providers should advise TCS to adhere to national guidelines for the management of cardiovascular disease risk factors, as well as to adopt behaviors consistent with a healthy lifestyle, including smoking cessation, a balanced diet, and a moderate to vigorous intensity exercise program. TCS should also follow national guidelines for cancer screening as currently applied to the general population. PMID:29670654

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.

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.

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

Science.gov (United States)

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.

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

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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

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

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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).

Sexuality and Physical Intimacy in Long Term Care: Sexuality, long term care, capacity assessment

OpenAIRE

Lichtenberg, Peter A.

2014-01-01

Sexuality and sexual needs in older adults remains a neglected area of clinical intervention, particularly so in long term care settings. Because older adults in medical rehabilitation and long term care beds present with significant frailties, and often significant neurocognitive disorders it makes it difficult for occupational therapists and other staff to evaluate the capacity of an older adult resident to participate in sexual relationships. The current paper reviews the current literatur...

Acute and long-term management of food allergy

DEFF Research Database (Denmark)

de Silva, D; Geromi, M; Panesar, S S

2014-01-01

BACKGROUND: Allergic reactions to food can have serious consequences. This systematic review summarizes evidence about the immediate management of reactions and longer-term approaches to minimize adverse impacts. METHODS: Seven bibliographic databases were searched from their inception to September...... management for non-life-threatening reactions. H1-antihistamines may be of benefit, but this evidence was in part derived from studies on those with cross-reactive birch pollen allergy. Regarding long-term management, avoiding the allergenic food or substituting an alternative was commonly recommended...... helpful, but allergen-specific immunotherapy may be disease modifying and therefore warrants further exploration. CONCLUSIONS: Food allergy can be debilitating and affects a significant number of people. However, the evidence base about acute and longer-term management is weak and needs to be strengthened...

Differences in health status between long-term and short-term benzodiazepine users.

NARCIS (Netherlands)

Zandstra, S.M.; Furer, J.W.; Lisdonk, E.H. van de; Bor, J.H.J.; Zitman, F.G.; Weel, C. van

2002-01-01

BACKGROUND: Despite generally accepted advice to keep treatment short, benzodiazepines are often prescibed for more than six months. Prevention of long-term benzodiazepine use could be facilitated by the utilisation of risk indicators for long-term use. However, the characteristics of long-term

Blood status in the long-term radiation syndrome

Directory of Open Access Journals (Sweden)

Galstyan I.A.

2013-12-01

Full Text Available Aims: to reveal regularities of change of peripheral blood indicators in the long-term acute radiation syndrome (ARS depending on its initial severity and existence of various somatic diseases. Material and methods. Clinical he-matologic materials from 114 patients in the long-term ARS (from 1,5 to more than 20 years after radiation accident, developed as a result of gamma beta exposure are presented. Results. In the period of the late radiation effect average values of peripheral blood are in physiological norm limits. However at some patients there are temporary superficial cytopenic conditions (thrombocytopenia — 22,7%, a leukopenia — 12,2%, a neutropenia — 13,1%, a lymphocytope-nia — 10,5%. Conclusion. As a result of a dynamic research it is revealed that in the remote terms of ARS (from 1,5 to more than 20 years after radiation average group levels of erythrocytes, leukocytes, neutrophils and lymphocytes in blood are in limits of physiological norm. However in the individual analysis of a functional state of blood at a part of patients are noted not stable cytopenic states.

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

Long-Term Outcome of Steroid-Resistant Nephrotic Syndrome in Children.

Science.gov (United States)

Trautmann, Agnes; Schnaidt, Sven; Lipska-Ziętkiewicz, Beata S; Bodria, Monica; Ozaltin, Fatih; Emma, Francesco; Anarat, Ali; Melk, Anette; Azocar, Marta; Oh, Jun; Saeed, Bassam; Gheisari, Alaleh; Caliskan, Salim; Gellermann, Jutta; Higuita, Lina Maria Serna; Jankauskiene, Augustina; Drozdz, Dorota; Mir, Sevgi; Balat, Ayse; Szczepanska, Maria; Paripovic, Dusan; Zurowska, Alexandra; Bogdanovic, Radovan; Yilmaz, Alev; Ranchin, Bruno; Baskin, Esra; Erdogan, Ozlem; Remuzzi, Giuseppe; Firszt-Adamczyk, Agnieszka; Kuzma-Mroczkowska, Elzbieta; Litwin, Mieczyslaw; Murer, Luisa; Tkaczyk, Marcin; Jardim, Helena; Wasilewska, Anna; Printza, Nikoleta; Fidan, Kibriya; Simkova, Eva; Borzecka, Halina; Staude, Hagen; Hees, Katharina; Schaefer, Franz

2017-10-01

We investigated the value of genetic, histopathologic, and early treatment response information in prognosing long-term renal outcome in children with primary steroid-resistant nephrotic syndrome. From the PodoNet Registry, we obtained longitudinal clinical information for 1354 patients (disease onset at >3 months and children, respectively, with the highest remission rates achieved with calcineurin inhibitor-based protocols. Ten-year ESRD-free survival rates were 43%, 94%, and 72% in children with IIS resistance, complete remission, and partial remission, respectively; 27% in children with a genetic diagnosis; and 79% and 52% in children with histopathologic findings of minimal change glomerulopathy and FSGS, respectively. Five-year ESRD-free survival rate was 21% for diffuse mesangial sclerosis. IIS responsiveness, presence of a genetic diagnosis, and FSGS or diffuse mesangial sclerosis on initial biopsy as well as age, serum albumin concentration, and CKD stage at onset affected ESRD risk. Our findings suggest that responsiveness to initial IIS and detection of a hereditary podocytopathy are prognostic indicators of favorable and poor long-term outcome, respectively, in children with steroid-resistant nephrotic syndrome. Children with multidrug-resistant sporadic disease show better renal survival than those with genetic disease. Furthermore, histopathologic findings may retain prognostic relevance when a genetic diagnosis is established. Copyright © 2017 by the American Society of Nephrology.

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...

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.

Chronically impaired autoregulation of cerebral blood flow in long-term diabetics

DEFF Research Database (Denmark)

Bentsen, N; Larsen, B; Lassen, N A

1975-01-01

Using the arteriovenous oxygen difference method autoregulation of cerebral blood flow (CBF) was tested in 16 long-term diabetics and eight control patients. Blood pressure was raised by angiotensin infusion and lowered by trimethaphan camsylate infusion, in some cases combined with head-up tilting...... of the patient. Regression analysis was carried out on the results in order to quantify autoregulatory capacity. In the control patients CBF did not vary with moderate blood pressure variations, indicating normal autoregulation. In four of the 16 diabetic patients CBF showed significant pressure dependency......, indicating impaired autoregulation. The cause of impaired autoregulation in some long-term diabetics is believed to be diffuse or multifocal dysfunction of cerebral arterioles due to diabetic vascular disease. Other conditions with impaired autoregulation are discussed and compared with that seen in long...

Long-term exposure to indoor air pollution and wheezing symptoms in infants

DEFF Research Database (Denmark)

Raaschou-Nielsen, O.; Hermansen, M.N.; Loland, L.

2010-01-01

Long-term exposure to air pollution is suspected to cause recurrent wheeze in infants. The few previous studies have had ambiguous results. The objective of this study was to estimate the impact of measured long-term exposure to indoor air pollution on wheezing symptoms in infants. We monitored......-point 'any symptom-day' (yes/no) and by standard linear regression with the end-point 'number of symptom-days'. The results showed no systematic association between risk for wheezing symptoms and the levels of these air pollutants with various indoor and outdoor sources. In conclusion, we found no evidence...... of an association between long-term exposure to indoor air pollution and wheezing symptoms in infants, suggesting that indoor air pollution is not causally related to the underlying disease. Practical Implications Nitrogen oxides, formaldehyde and fine particles were measured in the air in infants' bedrooms...

Long-Term Retrievability of IVC Filters: Should We Abandon Permanent Devices?

International Nuclear Information System (INIS)

Berczi, V.; Bottomley, J. R.; Thomas, S. M.; Taneja, S.; Gaines, P. A.; Cleveland, T. J.

2007-01-01

Thromboembolic disease produces a considerable disease burden, with death from pulmonary embolism in the UK alone estimated at 30,000-40,000 per year. Whilst it is unproven whether filters actually improve longevity, the morbidity and mortality associated with thromboembolic disease in the presence of contraindications to anticoagulation is high. Thus complications associated with filter insertion, and whilst they remain in situ, must be balanced against the alternatives. Permanent filters remain in situ for the remainder of the patient's life and any complications from the filters are of significant concern. Filters that are not permanent are therefore attractive in these circumstances. Retrievable filters, to avoid or decrease long-term filter complications, appear to be a significant advance in the prevention of pulmonary embolism. In this review, we discuss the safety and effectiveness of both permanent and retrievable filters as well as the retrievability of retrievable inferior vena cava (IVC) filters, to explore whether the use of permanent IVC filters can be abandoned in favor of retrievable filters. Currently four types of retrievable filters are available: the Recovery filter (Bard Peripheral Vascular, Tempe, AZ, USA), the Guenther Tulip filter (Cook, Bloomington, IN, USA), the OptEase Filter (Cordis, Roden, The Netherlands), and the ALN filter (ALN Implants Chirurgicaux, Ghisonaccia, France). Efficacy and safety data for retrievable filters are as yet based on small series, with a total number of fewer than 1,000 insertions, and follow-up is mostly short term. Current long-term data are poor and insufficient to warrant the long-term implantation of these devices into humans. The case of fractured wire from a Recovery filter that migrated to the heart causing pericardial tamponade requiring open heart surgery is a reminder that any new endovascular device remaining in situ in the long term may produce unexpected problems. We should also bear in mind that

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

Long-term prisoner in prison isolation

Directory of Open Access Journals (Sweden)

Karolina Grudzińska

2013-06-01

Full Text Available Long-term prisoner belongs to a particular category of people who are imprisoned in prisons. On the one hand in this group are often heavily demoralized people who committed the most serious crimes, on the other hand it is a group of prisoners, who should be well thought out and programmed the impact of rehabilitation. The situation of man trapped for years poses in a complicated situation not only the prisoners, but also the entire prison staff. They have to take care of the fact that the prison isolation did not cause the state in which convicts form itself in learned helplessness and lack of skills for self-planning and decision-making. In addition, planning the rehabilitation impact of long-term prisoners should not be forgotten that these prisoners in the short or the long term will return to the libertarian environment therefore, should prevent any negative effects of long-term imprisonment. This article presents the main issues related to the execution of imprisonment against long-term prisoners. It is an attempt to systematize the knowledge of this category of people living in prison isolation.

Radiologic features of a pyrophosphate-like arthropathy associated with long-term dialysis

Energy Technology Data Exchange (ETDEWEB)

Braunstein, E.M.; Martel, W.; Menerey, K.; Fox, I.H.; Swartz, R.

1987-08-01

In a series of 28 long-term dialysis patients with musculoskeletal complaints, the radiologic findings in six cases resembled those occurring in the arthropathy of idiopathic calcium pyrophosphate dihydrate deposition (CPPD) disease. These findings included osteophytes, subchondral cysts, and cartilage loss in the metacarpophalangeal joints, patellofemoral joints, wrists, and shoulders. Chondrocalcinosis was present in three of the six cases. There were no significant differences in renal function or levels of serum calcium, phosphorus, iron, ferritin, aluminum, or parathormone between these patients and a control group matched for sex and age. Long-term dialysis may be associated with a metabolic arthritis similar to the arthritis which occurs in CPPD deposition disease. The etiology may include deposition of CPPD crystals, hydroxyapatite, or other calcium-containing substances in joints, or it may be related to a number of dialysis-induced metabolic abnormalities.

Radiologic features of a pyrophosphate-like arthropathy associated with long-term dialysis

International Nuclear Information System (INIS)

Braunstein, E.M.; Martel, W.; Menerey, K.; Fox, I.H.; Swartz, R.

1987-01-01

In a series of 28 long-term dialysis patients with musculoskeletal complaints, the radiologic findings in six cases resembled those occurring in the arthropathy of idiopathic calcium pyrophosphate dihydrate deposition (CPPD) disease. These findings included osteophytes, subchondral cysts, and cartilage loss in the metacarpophalangeal joints, patellofemoral joints, wrists, and shoulders. Chondrocalcinosis was present in three of the six cases. There were no significant differences in renal function or levels of serum calcium, phosphorus, iron, ferritin, aluminum, or parathormone between these patients and a control group matched for sex and age. Long-term dialysis may be associated with a metabolic arthritis similar to the arthritis which occurs in CPPD deposition disease. The etiology may include deposition of CPPD crystals, hydroxyapatite, or other calcium-containing substances in joints, or it may be related to a number of dialysis-induced metabolic abnormalities. (orig.)

Competitive short-term and long-term memory processes in spatial habituation.

Science.gov (United States)

Sanderson, David J; Bannerman, David M

2011-04-01

Exposure to a spatial location leads to habituation of exploration such that, in a novelty preference test, rodents subsequently prefer exploring a novel location to the familiar location. According to Wagner's (1981) theory of memory, short-term and long-term habituation are caused by separate and sometimes opponent processes. In the present study, this dual-process account of memory was tested. Mice received a series of exposure training trials to a location before receiving a novelty preference test. The novelty preference was greater when tested after a short, rather than a long, interval. In contrast, the novelty preference was weaker when exposure training trials were separated by a short, rather than a long interval. Furthermore, it was found that long-term habituation was determined by the independent effects of the amount of exposure training and the number of exposure training trials when factors such as the intertrial interval and the cumulative intertrial interval were controlled. A final experiment demonstrated that a long-term reduction of exploration could be caused by a negative priming effect due to associations formed during exploration. These results provide evidence against a single-process account of habituation and suggest that spatial habituation is determined by both short-term, recency-based memory and long-term, incrementally strengthened memory.

Long- and short-term exposure to PM2.5 and mortality: using novel exposure models.

Science.gov (United States)

Kloog, Itai; Ridgway, Bill; Koutrakis, Petros; Coull, Brent A; Schwartz, Joel D

2013-07-01

Many studies have reported associations between ambient particulate matter (PM) and adverse health effects, focused on either short-term (acute) or long-term (chronic) PM exposures. For chronic effects, the studied cohorts have rarely been representative of the population. We present a novel exposure model combining satellite aerosol optical depth and land-use data to investigate both the long- and short-term effects of PM2.5 exposures on population mortality in Massachusetts, United States, for the years 2000-2008. All deaths were geocoded. We performed two separate analyses: a time-series analysis (for short-term exposure) where counts in each geographic grid cell were regressed against cell-specific short-term PM2.5 exposure, temperature, socioeconomic data, lung cancer rates (as a surrogate for smoking), and a spline of time (to control for season and trends). In addition, for long-term exposure, we performed a relative incidence analysis using two long-term exposure metrics: regional 10 × 10 km PM2.5 predictions and local deviations from the cell average based on land use within 50 m of the residence. We tested whether these predicted the proportion of deaths from PM-related causes (cardiovascular and respiratory diseases). For short-term exposure, we found that for every 10-µg/m increase in PM 2.5 exposure there was a 2.8% increase in PM-related mortality (95% confidence interval [CI] = 2.0-3.5). For the long-term exposure at the grid cell level, we found an odds ratio (OR) for every 10-µg/m increase in long-term PM2.5 exposure of 1.6 (CI = 1.5-1.8) for particle-related diseases. Local PM2.5 had an OR of 1.4 (CI = 1.3-1.5), which was independent of and additive to the grid cell effect. We have developed a novel PM2.5 exposure model based on remote sensing data to assess both short- and long-term human exposures. Our approach allows us to gain spatial resolution in acute effects and an assessment of long-term effects in the entire population rather than a

Long term liquidity analysis of the firm

Directory of Open Access Journals (Sweden)

Jaroslav Gonos

2009-09-01

Full Text Available Liquidity control is a very difficult and important function. If the business is not liquid in the long term, it is under threatof bankruptcy, and on the other hand surplus of the cash in hand threaten its future efficiency, because the cash in hand is a sourceof only limited profitability. Long term liquidity is related to the ability of the short term and long term liabilities payment. Articleis trying to point out to the monitoring and analyzing of the long term liquidity in the concrete business, in this case the printing industrycompany. Hereby at the end of the article mentioned monitored and analyzed liquidity is evaluated in the five years time period.

Drosophila Neprilysins Are Involved in Middle-Term and Long-Term Memory.

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Turrel, Oriane; Lampin-Saint-Amaux, Aurélie; Préat, Thomas; Goguel, Valérie

2016-09-14

Neprilysins are type II metalloproteinases known to degrade and inactivate a number of small peptides. Neprilysins in particular are the major amyloid-β peptide-degrading enzymes. In mouse models of Alzheimer's disease, neprilysin overexpression improves learning and memory deficits, whereas neprilysin deficiency aggravates the behavioral phenotypes. However, whether these enzymes are involved in memory in nonpathological conditions is an open question. Drosophila melanogaster is a well suited model system with which to address this issue. Several memory phases have been characterized in this organism and the neuronal circuits involved are well described. The fly genome contains five neprilysin-encoding genes, four of which are expressed in the adult. Using conditional RNA interference, we show here that all four neprilysins are involved in middle-term and long-term memory. Strikingly, all four are required in a single pair of neurons, the dorsal paired medial (DPM) neurons that broadly innervate the mushroom bodies (MBs), the center of olfactory memory. Neprilysins are also required in the MB, reflecting the functional relationship between the DPM neurons and the MB, a circuit believed to stabilize memories. Together, our data establish a role for neprilysins in two specific memory phases and further show that DPM neurons play a critical role in the proper targeting of neuropeptides involved in these processes. Neprilysins are endopeptidases known to degrade a number of small peptides. Neprilysin research has essentially focused on their role in Alzheimer's disease and heart failure. Here, we use Drosophila melanogaster to study whether neprilysins are involved in memory. Drosophila can form several types of olfactory memory and the neuronal structures involved are well described. Four neprilysin genes are expressed in adult Drosophila Using conditional RNA interference, we show that all four are specifically involved in middle-term memory (MTM) and long-term

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

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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.

Influence of long-term treatment with glyceryl trinitrate on remote ischemic conditioning

DEFF Research Database (Denmark)

Hauerslev, Marie; Mørk, Sivagowry Rasalingam; Pryds, Kasper

2018-01-01

Remote ischemic conditioning (RIC) protects against sustained myocardial ischemia. Due to overlapping mechanisms this protection may be altered by glyceryl trinitrate (GTN), which is commonly used in the treatment of patients with chronic ischemic heart disease. We investigated whether long-term ...

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...

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.

LONG-TERM OUTCOMES OF DECEASED DONOR LIVER TRANSPLANTATION

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S. V. Gautier

2014-01-01

Full Text Available Aim of the study was to evaluate patient and graft survival after liver transplantation (LT and to determine if primary disease diagnosis, early graft dysfunction or other factors affect it. Furthermore, we analyzed the reasonsof short-term and long-term deaths or retransplantations.Materials and methods. 192 LTs from donors with brain death were performed from December 2004 until June 2014. Recipient age varied from 5 to 71 years. Most frequent diagnosis was liver cirrhosis (mainly due to hepatitis C, then hepatocellular carcinoma (HCC, liver graft dysfunction, etc.Results and discussion. 1-year patient survival is 89.5%, graft survival is 87.7%, 3-year –87% and 84.6%, respectively, and 5-year – 83.5% and 83.0%, respectively. Early mortality (in fi rst 30 days after transplantation was 8%, long-term mortality – 5.9%. Primary non-function graft (PNF was the reason of 66.7% early deaths. In the long term, infections and oncology were the reasons of death with the same frequency – 36.4%. Early graft dysfunction including primary non-function signifi cantly decreases short term survival (p = 0.0002. Nevertheless, in the majority of cases graft function improves and doesn’t affect survival. Donor factors play role in outcomes: early dysfunction is higher (40.6% in extended criteria donor group than in standard donor group (р = 0.0431. PNF has the same trend – 8.5% and 0.0%, respectively, but without signifi cance (р =0.0835. 5-year survival is remarkably lower in HCC group 40.8% (p = 0.003 than in other groups.Conclusion: survival after liver transplantation in our Center is comparable with the results of the world’s centers.

Early Life Fructose Exposure and Its Implications for Long-Term Cardiometabolic Health in Offspring.

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Zheng, Jia; Feng, Qianyun; Zhang, Qian; Wang, Tong; Xiao, Xinhua

2016-11-01

It has become increasingly clear that maternal nutrition can strongly influence the susceptibility of adult offspring to cardiometabolic disease. For decades, it has been thought that excessive intake of fructose, such as sugar-sweetened beverages and foods, has been linked to increased risk of obesity, type 2 diabetes, and cardiovascular disease in various populations. These deleterious effects of excess fructose consumption in adults are well researched, but limited data are available on the long-term effects of high fructose exposure during gestation, lactation, and infancy. This review aims to examine the evidence linking early life fructose exposure during critical periods of development and its implications for long-term cardiometabolic health in offspring.

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

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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

Long-term contracts vs. short-term trade of natural gas - a European perspective

International Nuclear Information System (INIS)

Neuhoff, Karsten; Hirschhausen, Christian von

2005-01-01

This paper analyses the economics of long-term gas contracts under changing institutional conditions, mainly gas sector liberalisation. The paper is motivated by the increasingly tense debate in continental Europe, UK and the US on the security of long-term gas supply. We discuss the main issues regarding long-term contracts, i.e. the changing role of the flexibility clause, the effect of abandoning the destination clause, and the strategic behaviour of producers between long-term sales and spot-sales. The literature suggests consumers and producers benefit from risk hedging through long-term contracts. Furthermore long-term contracts may reduce exercise of market power. Our analysis adds an additional benefit if the long-run demand elasticity is significantly lower than the short-run elasticity, both strategic producers and consumers benefit from lower prices and larger market volume. Some policy implications of the findings are also discussed. (Author)

The uranium industry: long-term planning for short-term competition

International Nuclear Information System (INIS)

Vottero, X.; Georges Capus, G.

2001-01-01

Long term planning for short term competition Today, uranium producers face new challenges in terms of both production (new regulatory, environmental and social constraints) and market conditions (new sources of uranium supply, very low prices and tough competition). In such a context, long-term planning is not just a prerequisite to survive in the nuclear fuel cycle industry. In fact, it also contributes to sustaining nuclear electricity generation facing fierce competition from other energy sources in increasingly deregulated markets. Firstly, the risk of investing in new mining projects in western countries is growing because, on the one hand, of very erratic market conditions and, on the other hand, of increasingly lengthy, complex and unpredictable regulatory conditions. Secondly, the supply of other sources of uranium (uranium derived from nuclear weapons, uranium produced in CIS countries, ...) involve other risks, mainly related to politics and commercial restrictions. Consequently, competitive uranium supply requires not only technical competence but also financial strength and good marketing capabilities in order to anticipate long-term market trends, in terms of both demand and supply. It also requires taking into account new parameters such as politics, environment, regulations, etc. Today, a supplier dedicated to the sustainable production of nuclear electricity must manage a broad range of long-term risks inherent to the procurement of uranium. Taking into account all these parameters in a context of short-term, fast-changing market is a great challenge for the future generation. World Uranium Civilian Supply and Demand. (authors)

Recycling of extracorporeally irradiated autograft for malignant bone tumors: long-term follow-up.

Science.gov (United States)

Kotb, Samir Z; Mostafa, Mohamed F

2013-11-01

This study was conducted to evaluate the long-term oncological and functional outcomes. Forty-two patients (29 men and 13 women) with primary malignant bone tumors were included in this study. The procedure consisted of wide en bloc resection, clearing the extraosseous soft tissue and medullary content, extracorporeal irradiation with a single dose of 50 Gy using linear accelerator, and reimplantation using suitable fixation devices. The mean survivor follow-up was 54 months (24-174 months). There were 32 (76.2%) patients continuously disease free, 7 (16.7%) died of disease, and 3 (7.1%) alive with disease. Local recurrence was encountered in 4 (9.5%) patients. Nonunion occurred at 3 (6.4%) osteotomy sites. Deep infection developed in 4 (9.5%) cases. There were 13 patients rated excellent, 17 good, 10 fair, and 2 failures according to the Mankin scoring system. The mean ratings of the Musculoskeletal Tumor Society score and the Toronto Extremity Salvage Score were 77 and 81, respectively. The long-term oncological and functional results are encouraging and suggest that extracorporeal irradiation and reimplantation can be a long-lasting biological reconstructive technique in properly selected patients.

Long-term particulate matter exposure and mortality: a review of European epidemiological studies

Directory of Open Access Journals (Sweden)

Boffetta Paolo

2009-12-01

Full Text Available Abstract Background Several studies considered the relation between long-term exposure to particulate matter (PM and total mortality, as well as mortality from cardiovascular and respiratory diseases. Our aim was to provide a comprehensive review of European epidemiological studies on the issue. Methods We searched the Medline database for epidemiological studies on air pollution and health outcomes published between January 2002 and December 2007. We also examined the reference lists of individual papers and reviews. Two independent reviewers classified the studies according to type of air pollutant, duration of exposure and health outcome considered. Among European investigations that examined long-term PM exposure we found 4 cohort studies (considering total and cardiopulmonary mortality, 1 case-control study (considering mortality from myocardial infarction, and 4 ecologic studies (2 studies considering total and cardiopulmonary mortality and 2 studies focused on cardiovascular mortality. Results Measurement indicators of PM exposure used in European studies, including PM10, PM2.5, total suspended particulate and black smoke, were heterogeneous. This notwithstanding, in all analytic studies total mortality was directly associated with long-term exposure to PM. The excesses in mortality were mainly due to cardiovascular and respiratory causes. Three out of 4 ecologic studies found significant direct associations between PM indexes and mortality. Conclusion European studies on long-term exposure to PM indicate a direct association with mortality, particularly from cardiovascular and respiratory diseases.

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

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

Modeling long-term dynamics of electricity markets

International Nuclear Information System (INIS)

Olsina, Fernando; Garces, Francisco; Haubrich, H.-J.

2006-01-01

In the last decade, many countries have restructured their electricity industries by introducing competition in their power generation sectors. Although some restructuring has been regarded as successful, the short experience accumulated with liberalized power markets does not allow making any founded assertion about their long-term behavior. Long-term prices and long-term supply reliability are now center of interest. This concerns firms considering investments in generation capacity and regulatory authorities interested in assuring the long-term supply adequacy and the stability of power markets. In order to gain significant insight into the long-term behavior of liberalized power markets, in this paper, a simulation model based on system dynamics is proposed and the underlying mathematical formulations extensively discussed. Unlike classical market models based on the assumption that market outcomes replicate the results of a centrally made optimization, the approach presented here focuses on replicating the system structure of power markets and the logic of relationships among system components in order to derive its dynamical response. The simulations suggest that there might be serious problems to adjust early enough the generation capacity necessary to maintain stable reserve margins, and consequently, stable long-term price levels. Because of feedback loops embedded in the structure of power markets and the existence of some time lags, the long-term market development might exhibit a quite volatile behavior. By varying some exogenous inputs, a sensitivity analysis is carried out to assess the influence of these factors on the long-run market dynamics

Short-term memory and long-term memory are still different.

Science.gov (United States)

Norris, Dennis

2017-09-01

A commonly expressed view is that short-term memory (STM) is nothing more than activated long-term memory. If true, this would overturn a central tenet of cognitive psychology-the idea that there are functionally and neurobiologically distinct short- and long-term stores. Here I present an updated case for a separation between short- and long-term stores, focusing on the computational demands placed on any STM system. STM must support memory for previously unencountered information, the storage of multiple tokens of the same type, and variable binding. None of these can be achieved simply by activating long-term memory. For example, even a simple sequence of digits such as "1, 3, 1" where there are 2 tokens of the digit "1" cannot be stored in the correct order simply by activating the representations of the digits "1" and "3" in LTM. I also review recent neuroimaging data that has been presented as evidence that STM is activated LTM and show that these data are exactly what one would expect to see based on a conventional 2-store view. (PsycINFO Database Record (c) 2017 APA, all rights reserved).

Effect of BRCA1/2 mutation on short-term and long-term breast cancer survival: a systematic review and meta-analysis.

Science.gov (United States)

Lee, Eun-Ha; Park, Sue K; Park, Boyoung; Kim, Sung-Won; Lee, Min Hyuk; Ahn, Sei Hyun; Son, Byung Ho; Yoo, Keun-Young; Kang, Daehee

2010-07-01

Reports of BRCA genetic mutations and risk of death or recurrence are inconsistent. This study aimed to compare overall and disease-free breast cancer survival rates between BRCA1/2 mutation carriers and non-carriers for short-term and long-term outcomes separately. We searched the PUBMED and EMBASE databases and retrieved 452 articles using keywords that included breast cancer, BRCA mutation, and survival. Seventeen articles were selected for systematic review and among them 11 were included in our meta-analysis. We used the random-effects model to calculate the summary hazard ratio and corresponding 95% confidence interval. BRCA1 mutation carriers had significantly lower short-term and long-term overall survival rates (OSR) relative to non-carriers (HR = 1.92 [95% CI = 1.45-2.53]; 1.33 [1.12-1.58], respectively), while both short-term and long-term OSR of BRCA2 carriers did not differ from non-carriers (HR = 1.30 [95% CI = 0.95-1.76]; 1.12 [95% CI = 0.86-1.45], respectively). For short-term progression-free survival rate (PFSR), BRCA1 mutation carriers had a significantly lower rate than non-carriers (HR = 1.54 [95% CI = 1.12-2.12]), while BRCA2 mutation carriers had a similar PFSR (HR = 1.23 [95% CI = 0.96-1.58]). For long-term PFSRs, we found no significant results. Our results suggest that BRCA1 mutation decreases short-term and long-term OSRs and short-term PFSR, however, BRCA2 mutation does not affect either short-term or long-term survival rate, which is attributed to the different carcinogenic pathways for BRCA1 and BRCA2.

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.

Drawings reflect a new dimension of the psychological impact of long-term remission of Cushing's syndrome.

Science.gov (United States)

Tiemensma, Jitske; Daskalakis, Nikolaos P; van der Veen, Else M; Ramondt, Steven; Richardson, Stephanie K; Broadbent, Elizabeth; Romijn, Johannes A; Pereira, Alberto M; Biermasz, Nienke R; Kaptein, Adrian A

2012-09-01

Drawings can be used to assess perceptions of patients about their disease. We aimed to explore the utility of the drawing test and its relation to illness perceptions, quality of life (QoL), and clinical disease severity in patients after long-term remission of Cushing's syndrome. We conducted a cross-sectional study including 47 patients with long-term remission of Cushing's syndrome. Patients completed the drawing test, the Illness Perception Questionnaire-Revised, the Short-Form 36, the EuroQoL-5D, and the Cushing QoL. The Cushing's syndrome severity index was scored based on medical records. Characteristics of the drawings were strongly associated with the Cushing's syndrome severity index and severity ratings of health professionals (all P Cushing's syndrome because drawings do not share common properties with parameters of QoL or illness perceptions, but do represent the clinical severity of the disease. The assessment of drawings may enable doctors to appreciate the perceptions of patients with long-term remission of Cushing's syndrome and will lead the way in dispelling idiosyncratic beliefs.

Long-term Non-Invasive Ventilation in Infants: A Systematic Review and Meta-Analysis.

Science.gov (United States)

Bedi, Prabhjot K; Castro-Codesal, Maria Luisa; Featherstone, Robin; AlBalawi, Mohammed M; Alkhaledi, Bashar; Kozyrskyj, Anita L; Flores-Mir, Carlos; MacLean, Joanna E

2018-01-01

The use of long-term non-invasive ventilation (NIV) to treat sleep and breathing disorders in children has increased substantially in the last decade; however, less data exist about its use in infants. Given that infants have distinct sleep and breathing patterns when compared to older children, the outcomes of infants on long-term NIV may differ as well. The aim of this study is to systematically review the use and outcomes of long-term NIV in infants. Ovid Medline, Ovid Embase, CINAHL (via EbscoHOST), PubMed, and Wiley Cochrane Library were systematically searched from January 1990 to July 2017. Studies on infants using long-term NIV outside of an acute care setting were included. Data were extracted on study design, population characteristics, and NIV outcomes. A total of 327 studies were full-text reviewed, with final inclusion of 60. Studies were distributed across airway (40%), neuromuscular (28%), central nervous system (10%), cardio-respiratory (2%), and multiple (20%) disease categories. Of the 18 airway studies reporting on NIV outcomes, 13 (72%) reported improvements in respiratory parameters. Of the 12 neuromuscular studies exclusively on spinal muscular atrophy type 1 (SMA1), six (50%) reported decreased hospitalizations and nine (75%) reported on mortality outcomes. Risk of bias was moderate to serious, and quality of the evidence was low to very low for all studies. Most studies had an observational design with no control group, limiting the potential for a meta-analysis. The outcomes reported in studies differed by the disease category being studied. Studies on airway conditions showed improvements in respiratory parameters for infants using NIV. Studies on neuromuscular disorder, which were almost exclusively on SMA1, reported decreased hospitalizations and prolonged survival. Overall, it appears that NIV is an effective long-term therapy for infants. However, the high risk of bias and low quality of the available evidence limited strong

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.

Long-Term Memory Performance in Adult ADHD.

Science.gov (United States)

Skodzik, Timo; Holling, Heinz; Pedersen, Anya

2017-02-01

Memory problems are a frequently reported symptom in adult ADHD, and it is well-documented that adults with ADHD perform poorly on long-term memory tests. However, the cause of this effect is still controversial. The present meta-analysis examined underlying mechanisms that may lead to long-term memory impairments in adult ADHD. We performed separate meta-analyses of measures of memory acquisition and long-term memory using both verbal and visual memory tests. In addition, the influence of potential moderator variables was examined. Adults with ADHD performed significantly worse than controls on verbal but not on visual long-term memory and memory acquisition subtests. The long-term memory deficit was strongly statistically related to the memory acquisition deficit. In contrast, no retrieval problems were observable. Our results suggest that memory deficits in adult ADHD reflect a learning deficit induced at the stage of encoding. Implications for clinical and research settings are presented.

A systematic review of potential long-term effects of sport-related concussion

Science.gov (United States)

Gardner, Andrew J; Schneider, Kathryn J; Guskiewicz, Kevin M; Bailes, Julian; Cantu, Robert C; Castellani, Rudolph J; Turner, Michael; Jordan, Barry D; Randolph, Christopher; Dvořák, Jiří; Hayden, K. Alix; Tator, Charles H; McCrory, Paul; Iverson, Grant L

2017-01-01

Objective Systematic review of possible long-term effects of sports-related concussion in retired athletes. Data sources Ten electronic databases. Study selection Original research; incidence, risk factors or causation related to long-term mental health or neurological problems; individuals who have suffered a concussion; retired athletes as the subjects and possible long-term sequelae defined as >10 years after the injury. Data extraction Study population, exposure/outcome measures, clinical data, neurological examination findings, cognitive assessment, neuroimaging findings and neuropathology results. Risk of bias and level of evidence were evaluated by two authors. Results Following review of 3819 studies, 47 met inclusion criteria. Some former athletes have depression and cognitive deficits later in life, and there is an association between these deficits and multiple prior concussions. Former athletes are not at increased risk for death by suicide (two studies). Former high school American football players do not appear to be at increased risk for later life neurodegenerative diseases (two studies). Some retired professional American football players may be at increased risk for diminishment in cognitive functioning or mild cognitive impairment (several studies), and neurodegenerative diseases (one study). Neuroimaging studies show modest evidence of macrostructural, microstructural, functional and neurochemical changes in some athletes. Conclusion Multiple concussions appear to be a risk factor for cognitive impairment and mental health problems in some individuals. More research is needed to better understand the prevalence of chronic traumatic encephalopathy and other neurological conditions and diseases, and the extent to which they are related to concussions and/or repetitive neurotrauma sustained in sports. PMID:28455362

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.)

Long-term outcomes of laparoscopic surgery for advanced transverse colon cancer.

Science.gov (United States)

Zhao, Liying; Wang, Yanan; Liu, Hao; Chen, Hao; Deng, Haijun; Yu, Jiang; Xue, Qi; Li, Guoxin

2014-05-01

The role of laparoscopic surgery for advanced transverse colon cancer (TCC) remains controversial, especially in terms of long-term oncologic outcomes. This retrospective cohort study enrolled 157 consecutive patients who underwent curable resections for advanced TCC between January 2002 and June 2011 (laparoscopic-assisted colectomy (LAC), n = 74; open colectomy (OC), n = 83). Short-term outcomes and oncologic long-term outcomes were compared between the two groups. Compared to the OC group, patients in the LAC group had less blood loss (LAC vs. OC, 79.6 ± 70.3 vs. 158.4 ± 89.3 ml, p < 0.001), faster return of bowel function (2.6 ± 0.7 vs. 3.8 ± 0.8 days, p < 0.001), and shorter postoperative hospital stay (10.3 ± 3.7 vs. 12.6 ± 6.0 days, p = 0.007). Conversions were required in four (5.4%) patients. Rates of short-term complication, mortality, and long-term complication were comparable between the two groups. The median follow-up time was 54 (26-106) months in the LAC group and 58 (29-113) months in the OC group (p = 0.407). There were no statistical differences in the rates of 5-year overall survival (73.6 vs. 71.1%, p = 0.397) and 5-year disease-free survival (70.5 vs. 66.7%, p = 0.501) between the two groups. Laparoscopic surgery for advanced TCC yield short-term benefits while achieving equivalent long-term oncologic outcomes.

Long-term medical outcomes in survivors of extra-ocular retinoblastoma: the Memorial Sloan-Kettering Cancer Center (MSKCC) experience.

Science.gov (United States)

Friedman, Danielle Novetsky; Sklar, Charles A; Oeffinger, Kevin C; Kernan, Nancy A; Khakoo, Yasmin; Marr, Brian P; Wolden, Suzanne L; Abramson, David H; Dunkel, Ira J

2013-04-01

Data on long-term outcomes of survivors of extra-ocular retinoblastoma are lacking. The authors sought to provide the first report characterizing long-term outcomes among survivors of extra-ocular retinoblastoma. Retrospective analysis of long-term medical outcomes in 19 survivors of extra-ocular retinoblastoma treated between 1992 and 2009. Severity of outcomes was graded using Common Terminology Criteria for Adverse Events. All patients received intensive multimodality therapy for their extra-ocular disease after management of their primary intra-ocular disease, including conventional chemotherapy (n = 19, 100%), radiotherapy (n = 15, 69%), and/or high-dose chemotherapy with autologous stem cell transplant (n = 17, 89%). The median follow-up was 7.8 years from diagnosis of extra-ocular retinoblastoma (range 2-17.8 years). The most common long-term non-visual outcomes were hearing loss (n = 15, 79%), short stature (n = 7, 37%), and secondary malignancies [SMN] (n = 6, 31%). Sixty-eight percent of survivors exhibited ≥2 non-visual long-term outcomes of any grade. Except short stature, which was not graded for severity, Grade 3-4 outcomes were limited to: ototoxicity (n = 8; n = 4 require hearing aids), SMNs (n = 6), and unequal limb length (n = 1). Five patients who developed SMNs carried a known RB1 mutation. SMNs developed at a median of 11.1 years after initial diagnosis; two patients died of their SMN. Long-term cardiac, pulmonary, hepatobiliary, or renal conditions were not identified in any survivors. Long-term outcomes are commonly seen in extra-ocular retinoblastoma survivors but the majority are mild-moderate in their severity. Longer comprehensive follow-up is needed to fully assess treatment-related outcomes but the information collected to date may affect management decisions for children with extra-ocular disease. Copyright © 2012 Wiley Periodicals, Inc.

A Long-term Plan for Kalk

DEFF Research Database (Denmark)

2017-01-01

In this case, the author demonstrates together with the owner-manager of KALK A/S, Mr Rasmus Jorgensen, how to use the Family Business Map to frame a constructive discussion about long-term planning. The Family Business Map is a tool for long-term planning in family firms developed by Professor...

Virtual Models of Long-Term Care

Science.gov (United States)

Phenice, Lillian A.; Griffore, Robert J.

2012-01-01

Nursing homes, assisted living facilities and home-care organizations, use web sites to describe their services to potential consumers. This virtual ethnographic study developed models representing how potential consumers may understand this information using data from web sites of 69 long-term-care providers. The content of long-term-care web…

Sleep facilitates long-term face adaptation

OpenAIRE

Ditye, Thomas; Javadi, Amir Homayoun; Carbon, Claus-Christian; Walsh, Vincent

2013-01-01

Adaptation is an automatic neural mechanism supporting the optimization of visual processing on the basis of previous experiences. While the short-term effects of adaptation on behaviour and physiology have been studied extensively, perceptual long-term changes associated with adaptation are still poorly understood. Here, we show that the integration of adaptation-dependent long-term shifts in neural function is facilitated by sleep. Perceptual shifts induced by adaptation to a distorted imag...

Prevalence of Long-Term Opioid Use in Long-Stay Nursing Home Residents.

Science.gov (United States)

Hunnicutt, Jacob N; Chrysanthopoulou, Stavroula A; Ulbricht, Christine M; Hume, Anne L; Tjia, Jennifer; Lapane, Kate L

2018-01-01

Overall and long-term opioid use among older adults have increased since 1999. Less is known about opioid use in older adults in nursing homes (NHs). Cross-sectional. U.S. NHs (N = 13,522). Long-stay NH resident Medicare beneficiaries with a Minimum Data Set 3.0 (MDS) assessment between April 1, 2012, and June 30, 2012, and 120 days of follow-up (N = 315,949). We used Medicare Part D claims to measure length of opioid use in the 120 days from the index assessment (short-term: ≤30 days, medium-term: >30-89 days, long-term: ≥90 days), adjuvants (e.g., anticonvulsants), and other pain medications (e.g., corticosteroids). MDS assessments in the follow-up period were used to measure nonpharmacological pain management use. Modified Poisson models were used to estimate adjusted prevalence ratios (aPR) and 95% confidence intervals (CI) for age, gender, race and ethnicity, cognitive and physical impairment, and long-term opioid use. Of all long-stay residents, 32.4% were prescribed any opioid, and 15.5% were prescribed opioids long-term. Opioid users (versus nonusers) were more commonly prescribed pain adjuvants (32.9% vs 14.9%), other pain medications (25.5% vs 11.0%), and nonpharmacological pain management (24.5% vs 9.3%). Long-term opioid use was higher in women (aPR = 1.21, 95% CI = 1.18-1.23) and lower in racial and ethnic minorities (non-Hispanic blacks vs whites: APR = 0.93, 95% CI = 0.90-0.94) and those with severe cognitive impairment (vs no or mild impairment, aPR = 0.82, 95% CI = 0.79-0.83). One in seven NH residents was prescribed opioids long-term. Recent guidelines on opioid prescribing for pain recommend reducing long-term opioid use, but this is challenging in NHs because residents may not benefit from nonpharmacological and nonopioid interventions. Studies to address concerns about opioid safety and effectiveness (e.g., on pain and functional status) in NHs are needed. © 2017, Copyright the Authors Journal compilation © 2017, The American Geriatrics

Radiological and clinical predictors of long-term outcome in rotator cuff calcific tendinitis.

Science.gov (United States)

de Witte, Pieter Bas; van Adrichem, Raymond A; Selten, Jasmijn W; Nagels, Jochem; Reijnierse, M; Nelissen, Rob G H H

2016-10-01

Knowledge on the epidemiology and long-term course of rotator cuff calcific tendinitis (RCCT) is scarce. We assessed demographics, radiological characteristics, and their association with long-term outcomes in a large patient group. Baseline demographics, radiological characteristics and treatment were recorded in 342 patients. Interobserver agreement of radiological measures was analyzed. Long-term outcome was evaluated with questionnaires (WORC, DASH). The association of baseline characteristics with outcome was assessed. Mean age was 49.0 (SD = 10.0), and 59.5 % were female. The dominant arm was affected in 66.0 %, and 21.3 % had bilateral disease. Calcifications were on average 18.7 mm (SD = 10.1, ICC = 0.84 (p < 0.001)) and located 10.1 mm (SD = 11.8) medially to the acromion (ICC = 0.77 (p < 0.001)). Gärtner type I calcifications were found in 32.1 % (Kappa = 0.47 (p < 0.001)). After 14 years (SD = 7.1) of follow-up, median WORC was 72.5 (range, 3.0-100.0; WORC < 60 in 42 %) and median DASH 17.0 (range, 0.0-82.0). Female gender, dominant arm involvement, bilateral disease, longer duration of symptoms, and multiple calcifications were associated with inferior WORC. DASH results were similar. Many subjects have persisting shoulder complaints years after diagnosis, regardless of treatment. Female gender, dominant arm involvement, bilateral disease, longer duration of symptoms, and multiple calcifications were associated with inferior outcome. Radiological measures had moderate-to-good reliability and no prognostic value. • Most RCCT studies report on short-term outcome and/or small patients groups. • In this large, long-term observational study, RCCT appeared to not be self-limiting in many subjects. • Negative prognostic factors included female gender, more calcifications, dominant arm affected, and longer duration of symptoms. • Interobserver agreement of general radiological RCCT measures is

Short-term memory binding deficits in Alzheimer's disease.

Science.gov (United States)

Parra, Mario A; Abrahams, Sharon; Fabi, Katia; Logie, Robert; Luzzi, Simona; Della Sala, Sergio

2009-04-01

Alzheimer's disease impairs long term memories for related events (e.g. faces with names) more than for single events (e.g. list of faces or names). Whether or not this associative or 'binding' deficit is also found in short-term memory has not yet been explored. In two experiments we investigated binding deficits in verbal short-term memory in Alzheimer's disease. Experiment 1: 23 patients with Alzheimer's disease and 23 age and education matched healthy elderly were recruited. Participants studied visual arrays of objects (six for healthy elderly and four for Alzheimer's disease patients), colours (six for healthy elderly and four for Alzheimer's disease patients), unbound objects and colours (three for healthy elderly and two for Alzheimer's disease patients in each of the two categories), or objects bound with colours (three for healthy elderly and two for Alzheimer's disease patients). They were then asked to recall the items verbally. The memory of patients with Alzheimer's disease for objects bound with colours was significantly worse than for single or unbound features whereas healthy elderly's memory for bound and unbound features did not differ. Experiment 2: 21 Alzheimer's disease patients and 20 matched healthy elderly were recruited. Memory load was increased for the healthy elderly group to eight items in the conditions assessing memory for single or unbound features and to four items in the condition assessing memory for the binding of these features. For Alzheimer's disease patients the task remained the same. This manipulation permitted the performance to be equated across groups in the conditions assessing memory for single or unbound features. The impairment in Alzheimer's disease patients in recalling bound objects reported in Experiment 1 was replicated. The binding cost was greater than that observed in the healthy elderly group, who did not differ in their performance for bound and unbound features. Alzheimer's disease grossly impairs the

Long-Term Stewardship Baseline Report and Transition Guidance

Energy Technology Data Exchange (ETDEWEB)

Kristofferson, Keith

2001-11-01

Long-term stewardship consists of those actions necessary to maintain and demonstrate continued protection of human health and the environment after facility cleanup is complete. As the Department of Energy’s (DOE) lead laboratory for environmental management programs, the Idaho National Engineering and Environmental Laboratory (INEEL) administers DOE’s long-term stewardship science and technology efforts. The INEEL provides DOE with technical, and scientific expertise needed to oversee its long-term environmental management obligations complexwide. Long-term stewardship is administered and overseen by the Environmental Management Office of Science and Technology. The INEEL Long-Term Stewardship Program is currently developing the management structures and plans to complete INEEL-specific, long-term stewardship obligations. This guidance document (1) assists in ensuring that the program leads transition planning for the INEEL with respect to facility and site areas and (2) describes the classes and types of criteria and data required to initiate transition for areas and sites where the facility mission has ended and cleanup is complete. Additionally, this document summarizes current information on INEEL facilities, structures, and release sites likely to enter long-term stewardship at the completion of DOE’s cleanup mission. This document is not intended to function as a discrete checklist or local procedure to determine readiness to transition. It is an overarching document meant as guidance in implementing specific transition procedures. Several documents formed the foundation upon which this guidance was developed. Principal among these documents was the Long-Term Stewardship Draft Technical Baseline; A Report to Congress on Long-Term Stewardship, Volumes I and II; Infrastructure Long-Range Plan; Comprehensive Facility Land Use Plan; INEEL End-State Plan; and INEEL Institutional Plan.

Women with Chronic Physical Disabilities: Correlates of Their Long-Term Psychosocial Adaptation.

Science.gov (United States)

Dangoor, Nira; Florian, Victor

1994-01-01

This study examined the effects of demographic factors, disability status, and individual internal resources to the long-term psychosocial adjustment of 88 married women with orthopedic, neurological, and internal chronic diseases. Results suggest that sense of coherence and socioeconomic status, rather than disability status variables, accounted…

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

Long-Term Prognosis of Plantar Fasciitis

DEFF Research Database (Denmark)

Hansen, Liselotte; Krogh, Thøger Persson; Ellingsen, Torkell

2018-01-01

, exercise-induced symptoms, bilateral heel pain, fascia thickness, and presence of a heel spur) could predict long-term outcomes, (3) to assess the long-term ultrasound (US) development in the fascia, and (4) to assess whether US-guided corticosteroid injections induce atrophy of the heel fat pad. Study....... The risk was significantly greater for women (P heel...... regardless of symptoms and had no impact on prognosis, and neither did the presence of a heel spur. Only 24% of asymptomatic patients had a normal fascia on US at long-term follow-up. A US-guided corticosteroid injection did not cause atrophy of the heel fat pad. Our observational study did not allow us...

Long-term dependence in exchange rates

Directory of Open Access Journals (Sweden)

A. Karytinos

2000-01-01

Full Text Available The extent to which exchange rates of four major currencies against the Greek Drachma exhibit long-term dependence is investigated using a R/S analysis testing framework. We show that both classic R/S analysis and the modified R/S statistic if enhanced by bootstrapping techniques can be proven very reliable tools to this end. Our findings support persistence and long-term dependence with non-periodic cycles for the Deutsche Mark and the French Franc series. In addition a noisy chaos explanation is favored over fractional Brownian motion. On the contrary, the US Dollar and British Pound were found to exhibit a much more random behavior and lack of any long-term structure.

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.

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.

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.

Short-term versus long-term contracting for uranium enrichment services

International Nuclear Information System (INIS)

Rudy, G.P.

1990-01-01

The US Department of Energy (US DOE) is the world's largest and most experienced supplier of uranium enrichment services. Through the late 1970s and early 1980s, emerging market forces transformed what was once a monopoly into a highly competitive industry. In the early 1980's the DOE lost market share. But as we enter the 1990s, new market forces have emerged. The US DOE believes a responsible balance between long-term and short-term contracting will be the key to success and the key to assuring the long-term health and reliability of the nuclear fuel industry. The US DOE intends to be in this nuclear business for a long time and will continue to offer reliable and responsive services second to none

Biochemical effects on long-term frozen human costal cartilage

Energy Technology Data Exchange (ETDEWEB)

Santin, Stefany P.; Martinho Junior, Antonio C.; Yoshito, Daniele; Soares, Fernando A.N.; Mathor, Monica B., E-mail: mathor@ipen.b [Instituto de Pesquisas Energeticas e Nucleares (IPEN/CNEN-SP), Sao Paulo, SP (Brazil)

2011-07-01

Currently, the progresses on treatment of musculoskeletal diseases with the evolving of artificial implants and the success of tissue transplantation between genetically different individuals have conducted to an increase in radiosterilization. Regarding to tissue transplantation, it is essential to have sterile tissue and many tissue banks use radiosterilization as an effective method to sterilize these tissues. However, high doses of ionizing radiation and the preservation method may induce structural modifications in the tissues, as degradation of structural scaffold, decreasing its mechanical properties. Particularly, cartilage have been preserved in high concentrations of glycerol or deep-frozen at -70 degree C for storage after radiosterilization. Therefore, it is important to study the modifications induced in cartilage by preservation methods and by radiosterilization to determine the appropriated parameters for high quality of human allografts. Costal cartilages were obtained from cadaveric donors and were frozen at -20 degree C for 2 years long in order to compare with previous studies for fresh, deep-frozen and glycerolised cartilages. The mechanical tests were carried out in a universal testing machine until sample failure. According our results, there is no significant statistical difference between stress at break of fresh, long-term - 20 degree C frozen cartilages and deep-frozen cartilage. This early result suggests, regarding to tensile property, that long-term - 20 degree C frozen cartilages corresponds to glycerolised costal cartilages irradiated with 25 kGy or deep-frozen cartilages irradiated with 25 and 50 kGy. Thus, this long-term frozen cartilages may be used for tissue banks, but more studies about effects of ionizing radiation are necessary. (author)

Biochemical effects on long-term frozen human costal cartilage

International Nuclear Information System (INIS)

Santin, Stefany P.; Martinho Junior, Antonio C.; Yoshito, Daniele; Soares, Fernando A.N.; Mathor, Monica B.

2011-01-01

Currently, the progresses on treatment of musculoskeletal diseases with the evolving of artificial implants and the success of tissue transplantation between genetically different individuals have conducted to an increase in radiosterilization. Regarding to tissue transplantation, it is essential to have sterile tissue and many tissue banks use radiosterilization as an effective method to sterilize these tissues. However, high doses of ionizing radiation and the preservation method may induce structural modifications in the tissues, as degradation of structural scaffold, decreasing its mechanical properties. Particularly, cartilage have been preserved in high concentrations of glycerol or deep-frozen at -70 degree C for storage after radiosterilization. Therefore, it is important to study the modifications induced in cartilage by preservation methods and by radiosterilization to determine the appropriated parameters for high quality of human allografts. Costal cartilages were obtained from cadaveric donors and were frozen at -20 degree C for 2 years long in order to compare with previous studies for fresh, deep-frozen and glycerolised cartilages. The mechanical tests were carried out in a universal testing machine until sample failure. According our results, there is no significant statistical difference between stress at break of fresh, long-term - 20 degree C frozen cartilages and deep-frozen cartilage. This early result suggests, regarding to tensile property, that long-term - 20 degree C frozen cartilages corresponds to glycerolised costal cartilages irradiated with 25 kGy or deep-frozen cartilages irradiated with 25 and 50 kGy. Thus, this long-term frozen cartilages may be used for tissue banks, but more studies about effects of ionizing radiation are necessary. (author)

Neo bladder long term follow-up

International Nuclear Information System (INIS)

Fakhr, I.; Mohamed, A. M.; Moustafa, A.; Al-Sherbiny, M.; Salama, M.

2013-01-01

One of the commonest forms of orthotopic bladder substitution for bladder cancer surivors, used in our institute, is the use of ileocecal segment. Sometimes, the need for Indiana pouch heterotropic continent diversion arises. Aim: To compare the long-term effect of orthotopic ileocecal bladder and heterotropic Indiana pouch following radical cystectomy in bladder cancer patients. Patients and methods: Between January 2008 and December 2011, 91 patients underwent radical cystectomy/anterior pelvic exentration and ortho topic ileocecal bladder reconstruction (61 patients) and Indiana pouch (30 patients), when orthotopic diversion could not be technically or oncologically feasible. Results: Convalescence was uneventful in most patients. All minor and major urinary leakage cases, in both diversions groups, where successfully conservatively treated. Only one patient in the ileocecal group with major urinary leak required re-exploration with successful revision of uretro-colonic anastomosis. Only one patient in the Indiana pouch group had accidentally discovered sub-centimetric stone, which was simply expelled. The overall survival proportion of ileocecal group was 100% compared to 80% in the Indiana pouch group (p < 0.001). The disease free survival proportion of ileocecal group was 90.8% compared to 80% in the Indiana pouch group (p = 0.076). Effective comparative daytime and nighttime urinary continence as well as renal function deterioration were not statistically significant between both reconstruction types. Conclusion: Both ileocecal bladder and Indiana pouch are safe procedures in regard to long-term effects over kidney function following radical cystectomy

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.

Long-Term Dynamics of Autonomous Fractional Differential Equations

Science.gov (United States)

Liu, Tao; Xu, Wei; Xu, Yong; Han, Qun

This paper aims to investigate long-term dynamic behaviors of autonomous fractional differential equations with effective numerical method. The long-term dynamic behaviors predict where systems are heading after long-term evolution. We make some modification and transplant cell mapping methods to autonomous fractional differential equations. The mapping time duration of cell mapping is enlarged to deal with the long memory effect. Three illustrative examples, i.e. fractional Lotka-Volterra equation, fractional van der Pol oscillator and fractional Duffing equation, are studied with our revised generalized cell mapping method. We obtain long-term dynamics, such as attractors, basins of attraction, and saddles. Compared with some existing stability and numerical results, the validity of our method is verified. Furthermore, we find that the fractional order has its effect on the long-term dynamics of autonomous fractional differential equations.

The Womanly World of Long Term Care: The Plight of the Long Term Care Worker. Gray Paper.

Science.gov (United States)

Older Women's League, Washington, DC.

Long-term care workers (those who are paid to provide custodial care for long-term patients in nursing homes or at home) must care for a growing number of increasingly disabled or dependent persons. They are working for agencies and institutions under growing pressure to increase productivity. They face new training and competency requirements,…

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.)

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.)

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.

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

Anastomotic Leak Increases Distant Recurrence and Long-Term Mortality After Curative Resection for Colonic Cancer

DEFF Research Database (Denmark)

Krarup, Peter-Martin; Nordholm-Carstensen, Andreas; Jorgensen, Lars N

2014-01-01

OBJECTIVE: To investigate the impact of anastomotic leak (AL) on disease recurrence and long-term mortality in patients alive 120 days after curative resection for colonic cancer. BACKGROUND: There is no solid data as to whether AL after colonic cancer surgery increases the risk of disease...

Long term wet spent nuclear fuel storage

International Nuclear Information System (INIS)

1987-04-01

The meeting showed that there is continuing confidence in the use of wet storage for spent nuclear fuel and that long-term wet storage of fuel clad in zirconium alloys can be readily achieved. The importance of maintaining good water chemistry has been identified. The long-term wet storage behaviour of sensitized stainless steel clad fuel involves, as yet, some uncertainties. However, great reliance will be placed on long-term wet storage of spent fuel into the future. The following topics were treated to some extent: Oxidation of the external surface of fuel clad, rod consolidation, radiation protection, optimum methods of treating spent fuel storage water, physical radiation effects, and the behaviour of spent fuel assemblies of long-term wet storage conditions. A number of papers on national experience are included

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