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Sample records for suspected idiopathic normal

  1. Idiopathic Normal Pressure Hydrocephalus

    Directory of Open Access Journals (Sweden)

    Basant R. Nassar BS

    2016-04-01

    Full Text Available Idiopathic normal pressure hydrocephalus (iNPH is a potentially reversible neurodegenerative disease commonly characterized by a triad of dementia, gait, and urinary disturbance. Advancements in diagnosis and treatment have aided in properly identifying and improving symptoms in patients. However, a large proportion of iNPH patients remain either undiagnosed or misdiagnosed. Using PubMed search engine of keywords “normal pressure hydrocephalus,” “diagnosis,” “shunt treatment,” “biomarkers,” “gait disturbances,” “cognitive function,” “neuropsychology,” “imaging,” and “pathogenesis,” articles were obtained for this review. The majority of the articles were retrieved from the past 10 years. The purpose of this review article is to aid general practitioners in further understanding current findings on the pathogenesis, diagnosis, and treatment of iNPH.

  2. Vascular factors in suspected normal pressure hydrocephalus

    Science.gov (United States)

    Agerskov, Simon; Rabiei, Katrin; Marlow, Thomas; Jensen, Christer; Guo, Xinxin; Kern, Silke; Wikkelsø, Carsten; Skoog, Ingmar

    2016-01-01

    Objective: We examined clinical and imaging findings of suspected idiopathic normal pressure hydrocephalus (iNPH) in relation to vascular risk factors and white matter lesions (WMLs), using a nested case-control design in a representative, population-based sample. Methods: From a population-based sample, 1,235 persons aged 70 years or older were examined with CT of the brain between 1986 and 2000. We identified 55 persons with hydrocephalic ventricular enlargement, i.e., radiologic findings consistent with iNPH. Among these, 26 had clinical signs that fulfilled international guideline criteria for probable iNPH. These cases were labeled suspected iNPH. Each case was matched to 5 controls from the same sample, based on age, sex, and study cohort. Data on risk factors were obtained from clinical examinations and the Swedish Hospital Discharge Register. History of hypertension, diabetes mellitus (DM), smoking, overweight, history of coronary artery disease, stroke/TIA, and WMLs on CT were examined. Risk factors associated with iNPH with a p value <0.1 in χ2 tests were included in conditional logistic regression models. Results: In the regression analyses, suspected iNPH was related to moderate to severe WMLs (odds ratio [OR] 5.2; 95% confidence interval [CI]: 1.5–17.6), while hydrocephalic ventricular enlargement was related to hypertension (OR 2.7; 95% CI: 1.1–6.8), moderate to severe WMLs (OR 6.5; 95% CI: 2.1–20.3), and DM (OR 4.3; 95% CI: 1.1–16.3). Conclusions: Hypertension, WMLs, and DM were related to clinical and imaging features of iNPH, suggesting that vascular mechanisms are involved in the pathophysiology. These findings might have implications for understanding disease mechanisms in iNPH and possibly prevention. PMID:26773072

  3. Glymphatic MRI in idiopathic normal pressure hydrocephalus.

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    Ringstad, Geir; Vatnehol, Svein Are Sirirud; Eide, Per Kristian

    2017-10-01

    The glymphatic system has in previous studies been shown as fundamental to clearance of waste metabolites from the brain interstitial space, and is proposed to be instrumental in normal ageing and brain pathology such as Alzheimer's disease and brain trauma. Assessment of glymphatic function using magnetic resonance imaging with intrathecal contrast agent as a cerebrospinal fluid tracer has so far been limited to rodents. We aimed to image cerebrospinal fluid flow characteristics and glymphatic function in humans, and applied the methodology in a prospective study of 15 idiopathic normal pressure hydrocephalus patients (mean age 71.3 ± 8.1 years, three female and 12 male) and eight reference subjects (mean age 41.1 + 13.0 years, six female and two male) with suspected cerebrospinal fluid leakage (seven) and intracranial cyst (one). The imaging protocol included T1-weighted magnetic resonance imaging with equal sequence parameters before and at multiple time points through 24 h after intrathecal injection of the contrast agent gadobutrol at the lumbar level. All study subjects were kept in the supine position between examinations during the first day. Gadobutrol enhancement was measured at all imaging time points from regions of interest placed at predefined locations in brain parenchyma, the subarachnoid and intraventricular space, and inside the sagittal sinus. Parameters demonstrating gadobutrol enhancement and clearance in different locations were compared between idiopathic normal pressure hydrocephalus and reference subjects. A characteristic flow pattern in idiopathic normal hydrocephalus was ventricular reflux of gadobutrol from the subarachnoid space followed by transependymal gadobutrol migration. At the brain surfaces, gadobutrol propagated antegradely along large leptomeningeal arteries in all study subjects, and preceded glymphatic enhancement in adjacent brain tissue, indicating a pivotal role of intracranial pulsations for glymphatic function. In

  4. Circadian rhythm in idiopathic normal pressure hydrocephalus.

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    Eleftheriou, Andreas; Ulander, Martin; Lundin, Fredrik

    2018-01-01

    The pathogenesis of idiopathic normal pressure hydrocephalus (iNPH) takes place in structures close to the cerebral ventricular system. Suprachiasmatic nucleus (SCN), situated close to the third ventricle, is involved in circadian rhythm. Diurnal disturbances are well-known in demented patients. The cognitive decline in iNPH is potentially reversible after a shunt operation. Diurnal rhythm has never been studied in iNPH. We hypothesize that there is a disturbance of circadian rhythm in iNPH-patients and the aim was to study any changes of the diurnal rhythm (mesor and circadian period) as well as any changes of the diurnal amplitude and acrophase of the activity in iNPH-patients before and after a shunt operation. Twenty consecutive iNPH-patients fulfilling the criteria of the American iNPH-guidelines, 9 males and 11 females, mean age 73 (49-81) years were included. The patients underwent a pre-operative clinical work-up including 10m walk time (w10mt) steps (w10ms), TUG-time (TUGt) and steps (TUGs) and for cognitive function an MMSE score was measured. In order to receive circadian rhythm data actigraphic recordings were performed using the SenseWear 2 (BodyMedia Inc Pittsburgh, PA, USA) actigraph. Cosinor analyses of accelerometry data were performed in "R" using non-linear regression with Levenburg- Marquardt estimation. Pre- and post-operative data regarding mesor, amplitude and circadian period were compared using Wilcoxon-Mann-Whitney test for paired data. Twenty patients were evaluated before and three month post-operatively. Motor function (w10mt, w10ms, TUGt, TUGs) was significantly improved while MMSE was not significantly changed. Actigraphic measurements (mesor, amplitude and circadian period) showed no significant changes after shunt operation. This is the first systematic study of circadian rhythm in iNPH-patients. We found no significant changes in circadian rhythm after shunt surgery. The conceptual idea of diurnal rhythm changes in hydrocephalus is

  5. Endoscopic third ventriculostomy in idiopathic normal pressure ...

    African Journals Online (AJOL)

    Mohammed Ahmed Eshra

    2013-12-22

    Dec 22, 2013 ... system of the brain causing ventricular enlargement. This is followed by gradual .... sion, not to decrease the pressure (which is already normal).8–15 ... So ETV must be performed in patients with clinical evolution of not more.

  6. Comparison of CSF Distribution between Idiopathic Normal Pressure Hydrocephalus and Alzheimer Disease.

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    Yamada, S; Ishikawa, M; Yamamoto, K

    2016-07-01

    CSF volumes in the basal cistern and Sylvian fissure are increased in both idiopathic normal pressure hydrocephalus and Alzheimer disease, though the differences in these volumes in idiopathic normal pressure hydrocephalus and Alzheimer disease have not been well-described. Using CSF segmentation and volume quantification, we compared the distribution of CSF in idiopathic normal pressure hydrocephalus and Alzheimer disease. CSF volumes were extracted from T2-weighted 3D spin-echo sequences on 3T MR imaging and quantified semi-automatically. We compared the volumes and ratios of the ventricles and subarachnoid spaces after classification in 30 patients diagnosed with idiopathic normal pressure hydrocephalus, 10 with concurrent idiopathic normal pressure hydrocephalus and Alzheimer disease, 18 with Alzheimer disease, and 26 control subjects 60 years of age or older. Brain to ventricle ratios at the anterior and posterior commissure levels and 3D volumetric convexity cistern to ventricle ratios were useful indices for the differential diagnosis of idiopathic normal pressure hydrocephalus or idiopathic normal pressure hydrocephalus with Alzheimer disease from Alzheimer disease, similar to the z-Evans index and callosal angle. The most distinctive characteristics of the CSF distribution in idiopathic normal pressure hydrocephalus were small convexity subarachnoid spaces and the large volume of the basal cistern and Sylvian fissure. The distribution of the subarachnoid spaces in the idiopathic normal pressure hydrocephalus with Alzheimer disease group was the most deformed among these 3 groups, though the mean ventricular volume of the idiopathic normal pressure hydrocephalus with Alzheimer disease group was intermediate between that of the idiopathic normal pressure hydrocephalus and Alzheimer disease groups. The z-axial expansion of the lateral ventricle and compression of the brain just above the ventricle were the common findings in the parameters for differentiating

  7. Ongoing search for diagnostic biomarkers in idiopathic normal pressure hydrocephalus.

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    Tarnaris, Andrew; Toma, Ahmed K; Kitchen, Neil D; Watkins, Laurence D

    2009-12-01

    Idiopathic normal pressure hydrocephalus is a syndrome, which typically has a clinical presentation of gait/balance disturbance, often accompanied by cognitive decline and/or urinary incontinence. Its diagnosis is based on relevant history and clinical examination, appropriate imaging findings and physiological testing. The clinical picture of idiopathic normal pressure hydrocephalus may occasionally be difficult to distinguish from that of Alzheimer's dementia, subcortical ischemic vascular dementia and Parkinson's disease. The aim of this article is to systematically review the literature from the last 29 years in order to identify cerebrospinal fluid (CSF) or imaging biomarkers that may aid in the diagnosis of the syndrome. The authors concluded that no CSF or imaging biomarker is currently fulfilling the criteria required to aid in the diagnosis of the condition. However, a few studies have revealed promising CSF and imaging markers that need to be verified by independent groups. The reasons that the progress in this field has been slow so far is also commented on, as well as steps required to apply the current evidence in the design of future studies within the field.

  8. Idiopathic chondrolysis - diagnostic difficulties

    International Nuclear Information System (INIS)

    Kozlowski, K.; Scougall, J.; Royal Alexandra Hospital for Children, Sydney

    1984-01-01

    Four cases of idiopathic chondrolysis of the hip in three white girls and one Maori girl are reported. The authors stress the causes why a disease with characteristic clinical and radiographic appearances and normal biochemical findings presents diagnostic difficulties. It is suspected that idiopathic chondrolysis is a metabolic disorder of chondrocytes, triggered by environment circumstances in susceptible individuals. Idiopathic chondrolysis is probably one of the most common causes of coxarthrosis in women. (orig.)

  9. The APOE Genotype in Idiopathic Normal Pressure Hydrocephalus.

    Directory of Open Access Journals (Sweden)

    Yi Yang

    Full Text Available Amyloid plaque has been reported in brain biopsies from patients with idiopathic normal-pressure hydrocephalus (iNPH and proposed as a significant feature of the pathophysiology. Presence of the apolipoprotein E ε4 (APOE ε4 allele is associated with increased risk of Alzheimer's disease (AD.To compare the distribution of APOE genotype in iNPH patients with an age-matched population-based control group and with Alzheimer's disease (AD patients.APOE genotype frequencies were determined in 77 iNPH patients (50 men and 27 women, mean age 71.7 years diagnosed with iNPH, a sample of 691 AD patients and 638 age-matched population controls (299 men and 339 women from the INTERGENE cohort.The APOE distribution did not differ significantly between the iNPH patients and the control population. The per e4-allele odds-ratio (OR of iNPH was given by OR = 0.90, 95% confidence interval (CI = (0.50, 1.60 that was considerably smaller than the per-allele OR of AD, OR = 5.34 (4.10, 7.00.The results suggest that the APOE-related risk of AD in patients with iNPH is not higher than in the general population.

  10. Cerebrospinal fluid biomarkers profile of idiopathic normal pressure hydrocephalus.

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    Schirinzi, Tommaso; Sancesario, Giulia Maria; Di Lazzaro, Giulia; D'Elia, Alessio; Imbriani, Paola; Scalise, Simona; Pisani, Antonio

    2018-04-01

    Idiopathic normal pressure hydrocephalus (iNPH) is a disabling neurological disorder whose potential treatability is significantly limited by diagnostic uncertainty. In fact, typical clinical presentation occurs at late phases of disease, when CSF shunting could be ineffective. In recent years, measurement of different CSF proteins, whose concentration directly reflects neuropathological changes of CNS, has significantly improved both diagnostic timing and accuracy of neurodegenerative disease. Unfortunately iNPH lacks neuropathological hallmarks allowing the identification of specific disease biomarkers. However, neuropathology of iNPH is so rich and heterogeneous that many processes can be tracked in CSF, including Alzheimer's disease core pathology, subcortical degeneration, neuroinflammation and vascular dysfunction. Indeed, a huge number of CSF biomarkers have been analyzed in iNPH patients, but a unifying profile has not been provided yet. In this brief survey, we thus attempted to summarize the main findings in the field of iNPH CSF biomarkers, aimed at outlining a synthetic model. Although defined cut-off values for biomarkers are not available, a better knowledge of CSF characteristics may definitely assist in diagnosing the disease.

  11. Pathophysiology of cerebral circulatory disorders in idiopathic normal pressure hydrocephalus

    International Nuclear Information System (INIS)

    Takeuchi, Totaro; Goto, Hiromi; Izaki, Kenji

    2007-01-01

    This study was conducted to elucidate the pathologic conditions of cerebral circulatory disorders in idiopathic normal pressure hydrocephalus (iNPH). Among 44 possible iNPH patients, 40 patients underwent shunt surgery based on diagnostic flow charts plotted by the Southern Tohoku method and were evaluated to be shunt-effective at the end of the first post-surgical month. The cerebral blood flow (CBF) was measured by N-isopropyl-( 123 I)-P-iodo-amphetamine single photon emission computed tomography (mean, mCBF; cortical region, cCBF; thalamus-basal ganglia region, tbCBF on autoradiography [ARG] method) and the perfusion patterns of the cerebral cortex were measured based on three-dimensional stereotactic surface projection (3D-SSP) Z-score images, before and 1 month after the surgery in all 40 subjects. The mCBF rose significantly from 32.1±2.74 ml/100 g/min before surgery to 39.8±3.02 ml/100 g/min after surgery (p<0.03). Investigation of the change of CBF revealed reductions in the cCBF (3 cases), tbCBF (9 cases), and cCBF+tbCBF (28 cases), with the reduced-cCBF group totaling 31 cases and the reduced-tbCBF group totaling 37 cases. Investigation of cerebral cortex hypoperfusion by 3D-SSP Z-score revealed 31 cases with hypoperfusion (frontal lobe type [19 cases], occipitotemporal lobe type [5 cases], mixed type [7 cases]) and nine cases with cortical normoperfusion (N). The pattern of reduction of the cortical blood flow on ARG method was favorably correlated with the pattern of hypoperfusion of the cerebral cortex on 3D-SSP Z-score images before surgery. A reduction of blood flow was found in the thalamus-basal ganglia region of all N type cases. The blood flow improved in 19 of 31 (61.3%) cases of the reduced-cCBF group and in 32 of 37 (86.5%) cases of the reduced-tbCBF group. All of the cases without detectable improvement exhibited increased blood flow in non-reduction areas. Investigation of the hypoperfusion patterns of the cerebral cortex on 3D-SSP Z

  12. Is it possible to make a diagnosis of idiopathic normal pressure hydrocephalus on MRI/CT?

    International Nuclear Information System (INIS)

    Ishikawa, Masatsune; Suzuki, Takayuki; Ohwaki, Hisayuki; Matsumoto, Atsuhito

    2006-01-01

    In 2004, Japanese guidelines for idiopathic normal pressure hydrocephalus (iNPH) were created to diagnose it more precisely and treat it more effectively. The guidelines suggested the importance of a tight cerebrospinal fluid (CSF) space at the high convexity area. We reviewed MRI/CT findings in 38 cases of probable iNPH during Jan. 1998 to Dec. 2003. We noted the tightness in the interhemispheric fissure on axial MRI/CT view. Thirty-three cases were shunt-responsive among 38 cases (shunt effectiveness: 86.8%). MRI/CT findings of iNPH on axial view were classified into typical, almost typical and atypical. Major findings of typical cases showed: 1) ventricular dilatation, 2) tight interhemispheric fissure, 3) dilated Sylvian fissure. Almost all of the typical cases were a little bit questionable on findings for 2) and/or 3). Atypical cases showed an absence of finding 2) or showed aqueductal stenosis. Among 33 the shunt-responding cases, ten cases (30%) were typical, 16 cases (49%) were almost typical and seven cases were atypical. Among the 5 cases of non-responders, they were divided into 2, 2 and 1 cases, respectively. Atypical cases included 2 cases of aqueductal stenosis and 5 cases of open interhemispheric fissure; the latter were difficult to differentiate form brain atrophy. Thus, about 80% of iNPH cases could be diagnosed or highly suspected on axial MRI/CT findings. Although MRI/CT findings were not definitive for making a diagnosis of iNPH, they were highly useful to idenitify patients for a CSF tap test. (author)

  13. Delayed clearance of cerebrospinal fluid tracer from entorhinal cortex in idiopathic normal pressure hydrocephalus: A glymphatic magnetic resonance imaging study.

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    Eide, Per K; Ringstad, Geir

    2018-01-01

    The glymphatic system plays a key role for clearance of waste solutes from the rodent brain. We recently found evidence of glymphatic circulation in the human brain when using magnetic resonance imaging (MRI) contrast agent as cerebrospinal fluid (CSF) tracer in conjunction with multiple MRI acquisitions (gMRI). The present study explored the hypothesis that reduced glymphatic clearance in entorhinal cortex (ERC) may be instrumental in idiopathic normal pressure hydrocephalus (iNPH) dementia. gMRI acquisitions were obtained over a 24-48 h time span in cognitively affected iNPH patients and non-cognitively affected patients with suspected CSF leaks. The CSF tracer enrichment was determined as changes in normalized MRI T1 signal units. The study included 30 patients with iNPH and 8 individuals with suspected CSF leaks (i.e. reference individuals). Compared to reference individuals, iNPH patients presented with higher medial temporal lobe atrophy score and Evan's index and inferior ERC thickness. We found delayed clearance of the intrathecal CSF tracer gadobutrol from CSF, the ERC and adjacent white matter, suggesting impaired glymphatic circulation. Reduced clearance and accumulation of toxic waste product such as amyloid-β may be a mechanism behind dementia in iNPH. Glymphatic MRI (gMRI) may become a tool for assessment of early dementia.

  14. Treatment and clinical outcome in patients with idiopathic normal pressure hydrocephalus - a systematic review

    DEFF Research Database (Denmark)

    Torsnes, Linnea; Blåfjelldal, Vibeke; Poulsen, Frantz Rom

    2014-01-01

    INTRODUCTION: Treatment of idiopathic normal pressure hydrocephalus (iNPH) is challenging. It is well known that patients with iNPH experience short-term symptom relief after shunt implantation, but the long-term effect of shunting has yielded diverging results. The objective of the present study...

  15. CTA/V detection of bilateral sigmoid sinus dehiscence and suspected idiopathic intracranial hypertension in unilateral pulsatile tinnitus

    International Nuclear Information System (INIS)

    Xu, Shuaishuai; Xu, Jianrong; Ruan, Shidong; Liu, Shanfeng; Gong, Ruozhen

    2018-01-01

    This aimed to evaluate the prevalence and extent of bilateral sigmoid sinus dehiscence (SSD) and to explore the presence of idiopathic intracranial hypertension (IIH) in patients with unilateral pulsatile tinnitus (PT) with CTA/V. Sixty PT patients (52 females; 40.4 ± 11.6 years [20-72]) who underwent CTA/V and 30 non-PT patients (27 females; 38.4 ± 14.7 years [12-62]) were enrolled in this study. The primary outcome measure was the radiographic presence of SSD. The index of transverse sinus stenosis (ITSS) was obtained by multiplying the stenosis scale values for each transverse sinus, and once was ≥ 4, the presence of IIH was suspected. The prevalence and extent of SSD on symptomatic side (78%; maximum transverse diameter, MTD 0.49 ± 0.23; maximum vertical diameter, MVD 0.50 ± 0.26 cm) were significantly higher and larger than those on asymptomatic side (50%, P < 0.001; MTD 0.35 ± 0.18, P = 0.006; MVD 0.30 ± 0.15 cm, P < 0.001) in the study group and those (20%, P < 0.001; MTD 0.36 ± 0.18, P = 0.073; MVD 0.30 ± 0.22 cm, P < 0.048) in the control group. The presence of SSD showed significant correlation with both PT (logistic regression analysis, OR 4.167 [1.450-11.97]; P = 0.008) and suspected IIH (OR 16.25 [1.893-139.5]; P = 0.011). In PT patients, SSD has a significant correlation with PT and a potential correlation with IIH. (orig.)

  16. A Girl with Idiopathic Epilepsy Showing Forced Normalization after Levetiracetam Administration.

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    Kawakami, Yasuhiko; Okazaki, Tetsuya; Takase, Masato; Fujino, Osamu; Itoh, Yasuhiko

    2015-01-01

    Forced normalization has been reported in association with almost all anti-epileptic drugs. We report on a 9-year-old girl with idiopathic epilepsy who showed forced normalization after administration of levetiracetam (LEV). She initially presented with generalized tonic-clonic seizures when she was 4 years old. Diffuse sharp and slow wave complexes (SWCs) were observed on electroencephalography (EEG). We prescribed sodium valproate (VPA) and benzodiazepines, but the seizures and EEG findings worsened gradually. Although subsequent administration of LEV stopped the seizures, the patient became subject to episodes of rage and violent behavior. Forced normalization was confirmed by the disappearance of SWCs on EEG. We reduced the dose of LEV and tried in various ways to resolve the situation, but finally we had to abandon LEV. To the best of our knowledge, this is the first report of a patient with idiopathic epilepsy but without disabilities in everyday life showing forced normalization associated with LEV administration.

  17. FDG-PET of patients with suspected renal failure. Standardized uptake values in normal tissues

    International Nuclear Information System (INIS)

    Minamimoto, Ryogo; Takahashi, Nobukazu; Inoue, Tomio

    2007-01-01

    This study aims to clarify the effect of renal function on 2-[ 18 F] fluoro-2-deoxy-D-glucose positron emission tomography (FDG-PET) imaging and determine the clinical significance of renal function in this setting. We compared FDG distribution between normal volunteers and patients with suspected renal failure. Twenty healthy volunteers and 20 patients with suspected renal failure who underwent FDG-PET between November 2002 and May 2005 were selected for this study. We define ''patients with suspected renal failure'' as having a blood serum creatinine level in excess of 1.1 mg/dl. The serum creatinine level was examined once in 2 weeks of the FDG-PET study. Regions of interest were placed over 15 regions for semi-quantitative analysis: the white matter, cortex, both upper lung fields, both middle lung fields, both lower lung fields, mediastinum, myocardium of the left ventricle, the left atrium as a cardiac blood pool, central region of the right lobe of the liver, left kidney, and both femoris muscles. The mean standardized uptake values (SUVs) of brain cortex and white matter were higher in healthy volunteers than in renal patients. The mean SUVs of the mediastinum at the level of the aortic arch and left atrium as a cardiac blood pool were lower in healthy volunteers than in patients with suspected renal failure. These regions differed between healthy volunteers and patients with suspected renal failure (P<0.05). We found decreasing brain accumulation and increasing blood pool accumulation of FDG in patients with high plasma creatinine. Although the difference is small, this phenomenon will not have a huge effect on the assessment of FDG-PET imaging in patients with suspected renal failure. (author)

  18. Prevalence of idiopathic normal-pressure hydrocephalus in the elderly population of a Japanese rural community

    International Nuclear Information System (INIS)

    Hiraoka, Kotaro; Meguro, Kenichi; Mori, Etsuro

    2008-01-01

    The prevalence of idiopathic normal-pressure hydrocephalus (NPH) in a community was investigated by retrospective analysis of data from a previous community-based study of 170 randomly selected elderly residents aged 65 years or older. Magnetic resonance (MR) images of the subjects were reviewed for the specific structural features of idiopathic NPH, i.e. ventricular enlargement and narrow cerebrospinal fluid (CSF) space at high convexity and high midline areas. The clinical features of idiopathic NPH, gait disturbance, urinary incontinence, and cognitive impairment, were evaluated on the basis of records of the subjects' neurological examinations, a health questionnaire, the Mini-Mental State Examination, and Clinical Dementia Rating. Thirteen of the 170 subjects showed lateral ventricular enlargement greater than 0.3 on Evans' index. Five subjects (2.9%) demonstrated both ventricular enlargement and narrow CSF space at the high convexity/midline. All five subjects with these MR imaging signs had cognitive impairment, one had gait disturbance, and one had urinary incontinence. The present study found 2.9% of community-dwelling elderly subjects showed radiological and clinical features consistent with idiopathic NPH. (author)

  19. Idiopathic normal pressure hydrocephalus, quantitative EEG findings, and the cerebrospinal fluid tap test: a pilot study.

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    Seo, Jong-Geun; Kang, Kyunghun; Jung, Ji-Young; Park, Sung-Pa; Lee, Maan-Gee; Lee, Ho-Won

    2014-12-01

    In this pilot study, we analyzed relationships between quantitative EEG measurements and clinical parameters in idiopathic normal pressure hydrocephalus patients, along with differences in these quantitative EEG markers between cerebrospinal fluid tap test responders and nonresponders. Twenty-six idiopathic normal pressure hydrocephalus patients (9 cerebrospinal fluid tap test responders and 17 cerebrospinal fluid tap test nonresponders) constituted the final group for analysis. The resting EEG was recorded and relative powers were computed for seven frequency bands. Cerebrospinal fluid tap test nonresponders, when compared with responders, showed a statistically significant increase in alpha2 band power at the right frontal and centrotemporal regions. Higher delta2 band powers in the frontal, central, parietal, and occipital regions and lower alpha1 band powers in the right temporal region significantly correlated with poorer cognitive performance. Higher theta1 band powers in the left parietal and occipital regions significantly correlated with gait dysfunction. And higher delta1 band powers in the right frontal regions significantly correlated with urinary disturbance. Our findings may encourage further research using quantitative EEG in patients with ventriculomegaly as a potential electrophysiological marker for predicting cerebrospinal fluid tap test responders. This study additionally suggests that the delta, theta, and alpha bands are statistically correlated with the severity of symptoms in idiopathic normal pressure hydrocephalus patients.

  20. [Normal lung volumes in patients with idiopathic pulmonary fibrosis and emphysema].

    Science.gov (United States)

    Casas, Juan Pablo; Abbona, Horacio; Robles, Adriana; López, Ana María

    2008-01-01

    Pulmonary function tests in idiopathic pulmonary fibrosis characteristically show a restrictive pattern, resulting from reduction of pulmonary compliance due to diffuse fibrosis. Conversely, an obstructive pattern with hyperinflation results in emphysema by loss of elastic recoil, expiratory collapse of the peripheral airways and air trapping. Previous reports suggest that when both diseases coexist, pulmonary volumes are compensated and a smaller than expected reduction or even normal lung volumes can be found. We report 4 male patients of 64, 60, 73 and 70 years, all with heavy cigarette smoking history and progressive breathlessness. Three of them had severe limitation in their quality of life. All four showed advanced lung interstitial involvement, at high resolution CT scan, fibrotic changes predominantly in the subpleural areas of lower lung fields and concomitant emphysema in the upper lobes. Emphysema and pulmonary fibrosis was confirmed by open lung biopsy in one patient. The four patients showed normal spirometry and lung volumes with severe compromise of gas exchange and poor exercise tolerance evaluated by 6 minute walk test. Severe pulmonary arterial hypertension was also confirmed in three patients. Normal lung volumes does not exclude diagnosis of idiopathic pulmonary fibrosis in patients with concomitant emphysema. The relatively preserved lung volumes may underestimate the severity of idiopathic pulmonary fibrosis and attenuate its effects on lung function parameters.

  1. [Idiopathic normal pressure hydrocephalus: High incidence in people over 80 years of age].

    Science.gov (United States)

    Aragonès, Josep Maria; Altimiras, Jacint; Alonso, Francisco; Roura, Pere; Alfonso, Sebastián; Bajo, Lorena

    Idiopathic normal pressure hydrocephalus is usually observed in adults over 60 years of age. The highest incidence of cases is between 70 and 80 years-old, and it could be under-diagnosed in over 80 year-olds. A description is presented on the overall incidence and age group incidence, the delay in the diagnosis, and main outcomes. A descriptive study was performed on patients with idiopathic normal pressure hydrocephalus, in the population of Osona County during the years 2010-2015. The annual incidence rate was 4.43 per 100,000 inhabitants. The incidence increased with age; from 8.09 per 100,000 in the 60 to 69 years age group, to 23.61 per 100,000 in the 70-79 years age group of, and to 37.02 per 100,000 in the 80-89 years age. The delay in the diagnosis was 15.01 ± 10.35 months. All the patients improved after surgery, but only 73.3% of the patients maintained the improvement after one year. Idiopathic normal pressure hydrocephalus is an age related disease and probably underdiagnosed in the elderly. An early diagnosis and a clinical suspicion are essential in patients over 80 years old. Copyright © 2017 SEGG. Publicado por Elsevier España, S.L.U. All rights reserved.

  2. International Veterinary Epilepsy Task Force's current understanding of idiopathic epilepsy of genetic or suspected genetic origin in purebred dogs

    DEFF Research Database (Denmark)

    Hülsmeyer, Velia-Isabel; Fischer, Andrea; Mandigers, Paul J. J.

    2015-01-01

    Canine idiopathic epilepsy is a common neurological disease affecting both purebred and crossbred dogs. Various breed-specific cohort, epidemiological and genetic studies have been conducted to date, which all improved our knowledge and general understanding of canine idiopathic epilepsy, and in ...

  3. Survey of Saccadic Parameters Using Videonystagmography in Patients with Idiopathic Parkinson's Disease and Normal Subjects

    Directory of Open Access Journals (Sweden)

    Reza Hosseinabadi

    2008-06-01

    Full Text Available Background and Aim: Patients with Parkinson’s disease manifest oculomotor abnormalities. This is the consequence of basal ganglia impairment. The most common abnormalities include increased saccade latency, hypometric saccades and decreased saccade velocity. The purpose of this study was comparison of saccadic parameters using videonystagmography in patients with idiopathic Parkinson’s disease and normal subjects.Materials and Methods: In this cross sectional study, saccadic movements were investigated in thirty patients with idiopathic Parkinson’s disease and thirty age matched subjects were 35-70 years old. Saccade latency, velocity and accuracy were quantitatively analyzed. Results: Results of this study indicated increased saccade latency, reduction of saccade velocity and accuracy in patients with Parkinson’s disease(P<0.001.Conclusion: This study showed that patients with Parkinson’s disease manifest saccadic deficits. This suggests dopaminergic control of these ocular movements.

  4. Fluid Distribution Pattern in Adult-Onset Congenital, Idiopathic, and Secondary Normal-Pressure Hydrocephalus: Implications for Clinical Care.

    Science.gov (United States)

    Yamada, Shigeki; Ishikawa, Masatsune; Yamamoto, Kazuo

    2017-01-01

    In spite of growing evidence of idiopathic normal-pressure hydrocephalus (NPH), a viewpoint about clinical care for idiopathic NPH is still controversial. A continuous divergence of viewpoints might be due to confusing classifications of idiopathic and adult-onset congenital NPH. To elucidate the classification of NPH, we propose that adult-onset congenital NPH should be explicitly distinguished from idiopathic and secondary NPH. On the basis of conventional CT scan or MRI, idiopathic NPH was defined as narrow sulci at the high convexity in concurrent with enlargement of the ventricles, basal cistern and Sylvian fissure, whereas adult-onset congenital NPH was defined as huge ventricles without high-convexity tightness. We compared clinical characteristics and cerebrospinal fluid distribution among 85 patients diagnosed with idiopathic NPH, 17 patients with secondary NPH, and 7 patients with adult-onset congenital NPH. All patients underwent 3-T MRI examinations and tap-tests. The volumes of ventricles and subarachnoid spaces were measured using a 3D workstation based on T2-weighted 3D sequences. The mean intracranial volume for the patients with adult-onset congenital NPH was almost 100 mL larger than the volumes for patients with idiopathic and secondary NPH. Compared with the patients with idiopathic or secondary NPH, patients with adult-onset congenital NPH exhibited larger ventricles but normal sized subarachnoid spaces. The mean volume ratio of the high-convexity subarachnoid space was significantly less in idiopathic NPH than in adult-onset congenital NPH, whereas the mean volume ratio of the basal cistern and Sylvian fissure in idiopathic NPH was >2 times larger than that in adult-onset congenital NPH. The symptoms of gait disturbance, cognitive impairment, and urinary incontinence in patients with adult-onset congenital NPH tended to progress more slowly compared to their progress in patients with idiopathic NPH. Cerebrospinal fluid distributions and

  5. Psychiatric symptoms are present in most of the patients with idiopathic normal pressure hydrocephalus

    Directory of Open Access Journals (Sweden)

    Matheus F. Oliveira

    2014-06-01

    Full Text Available Normal pressure hydrocephalus (NPH is characterized by gait disturbance, dementia and/or urinary incontinence associated with dilation of ventricular system with normal opening cerebrospinal fluid pressure. Wide scientifical evidence confirms association between NPH and psychiatric symptoms. We selected 35 patients with idiopathic normal pressure hydrocephalus from January 2010 to January 2012 in a Brazilian tertiary hospital and performed a formal psychiatric evaluation to identify psychiatric disorders. Psychiatric disorders were present in 71% of these patients, especially anxiety, depression and psychotic syndromes. NPH patients may develop symptoms with frontal dominance, such as personality changes, anxiety, depression, psychotic syndromes, obsessive compulsive disorder, Othello syndrome; shoplifting and mania. Unusual appearances of NPH symptoms may hinder early diagnosis and consequently proper treatment.

  6. Clinical validity of a normal pulmonary angiogram in patients with suspected pulmonary embolism - A critical review

    International Nuclear Information System (INIS)

    Beek, Edwin J.R. van; Brouwers, Elise M.J.; Song Bin; Stein, Paul D.; Oudkerk, Matthijs

    2001-01-01

    AIM: To determine the validity of a normal pulmonary angiogram in the exclusion of pulmonary embolism (PE), based on the safety of withholding anticoagulant therapy in patients with a normal pulmonary angiogram. MATERIALS AND METHODS: A review of English reports published between 1965 and April 1999 was carried out. Eligible articles described prospective studies in patients with suspected PE and a normal pulmonary angiogram, who remained untreated and were followed-up for a minimum of 3 months. Articles were evaluated by two authors, using pre-defined criteria for strength of design. End points consisted of fatal and non-fatal recurrent thromboembolic events. A sensitivity analysis was performed, by removing one study at a time from the overall results and by comparing pre- and post-1990 publications. RESULTS: Among 1050 patients in eight articles included in the analysis, recurrent thromboembolic events were described in 18 patients (1.7% 95% CI: 1.0-2.7%). These were fatal in three patients (0.3% 95% CI: 0.02-0.7%). The recurrence rate of PE decreased from 2.9% (95% CI: 1.4-6.8%) before 1990 to 1.1% (95% CI: 0.5-2.2%) after 1990. CONCLUSION: It would appear that the ability to exclude PE by angiography has improved over the years, as indicated by recurrence rate of PE. The low recurrence rate of PE supports the validity of a normal pulmonary angiogram for the exclusion of PE. Beek, E.J.R. van et al. (2001)

  7. Foetal radiography for suspected skeletal dysplasia: technique, normal appearances, diagnostic approach

    Energy Technology Data Exchange (ETDEWEB)

    Calder, Alistair D. [Great Ormond Street Hospital for Children NHS Foundation Trust, Radiology Department, London (United Kingdom); Offiah, Amaka C. [Sheffield Children' s NHS Foundation Trust, Academic Unit of Child Health, Sheffield (United Kingdom)

    2015-04-01

    Despite advances in antenatal imaging and genetic techniques, post-delivery post-mortem foetal radiography remains the key investigation in accurate diagnosis of skeletal dysplasia manifesting in the foetus. Foetal radiography is best performed using pathology-specimen radiography equipment and is often carried out in the pathology department without involvement of the radiology unit. However, paediatric radiologists may be asked to interpret post-mortem foetal radiographs when an abnormality is suspected. Many foetal radiographs are carried out before 20 weeks' gestation, and the interpreting radiologist needs to be familiar with the range of normal post-mortem foetal appearances at different gestational ages, as well as the appearances of some of the more commonly presenting skeletal dysplasias, and will benefit from a systematic approach when assessing more challenging cases. In this pictorial essay, we illustrate various normal post-mortem foetal radiographic appearances, give examples of commonly occurring skeletal dysplasias, and describe an approach to establishing more difficult diagnoses. (orig.)

  8. Foetal radiography for suspected skeletal dysplasia: technique, normal appearances, diagnostic approach

    International Nuclear Information System (INIS)

    Calder, Alistair D.; Offiah, Amaka C.

    2015-01-01

    Despite advances in antenatal imaging and genetic techniques, post-delivery post-mortem foetal radiography remains the key investigation in accurate diagnosis of skeletal dysplasia manifesting in the foetus. Foetal radiography is best performed using pathology-specimen radiography equipment and is often carried out in the pathology department without involvement of the radiology unit. However, paediatric radiologists may be asked to interpret post-mortem foetal radiographs when an abnormality is suspected. Many foetal radiographs are carried out before 20 weeks' gestation, and the interpreting radiologist needs to be familiar with the range of normal post-mortem foetal appearances at different gestational ages, as well as the appearances of some of the more commonly presenting skeletal dysplasias, and will benefit from a systematic approach when assessing more challenging cases. In this pictorial essay, we illustrate various normal post-mortem foetal radiographic appearances, give examples of commonly occurring skeletal dysplasias, and describe an approach to establishing more difficult diagnoses. (orig.)

  9. Lymphoscintigraphy in paediatric patients for suspected lymphoedema: normal variants, abnormal and syndromic patterns

    International Nuclear Information System (INIS)

    Pascual, T.; Howman-Giles, R.; Martin, H.

    2009-01-01

    Full text: Background: Lymphoscintigraphy (LS) is the diagnostic test of choice differentiating lymphoedema from other causes of extremity swelling. The LS patterns in normal and congenital lymphoedema in the paediatric population are not well defined. The impact of LS on clinical decision making is also not well reported. Aims: 1. define normal LS patterns in the pediatric population 2. describe types of abnormality (aplasia, hypoplasia, hyperplasia/dilated system) 3. describe LS patterns in syndromic lymphatic vascular disease 4. correlate LS with clinical impact on patient management. Methods: Retrospective review of all paediatric patients who had LS from July 1996-April 2008 was undertaken. Indications, sites of abnormality, LS patterns and clinical outcome were reviewed. Results: 118 patients (3mths-21yrs, mean 6 yrs) underwent LS. Normal LS patterns and variations were identified in 57 pts (48%). Sixty-one scans (52%) were abnormal showing lymph node aplasia (11%), hypoplasia (17%), mixed-pattern (8%), hyperplasia/dilated system (4%) and other patterns ie intestinal/pulmonary lymphangiectasia and vascual lymphatic malformations (11%). Patients with no signs of lymphoedema may still have aplasia or hypoplasia on LS. Dermal back flow is not always seen in lymphoedema. Management impact related to diagnosis and potential for development of lymphoedema in other limbs, differentiation of lymphoedema in patients with other vascular anomalies, stratification for lymphoedema therapy or referral to the dysmorphology clinic. Conclusion: LS is a valuable tool in the evaluation of lymphoedema in pediatric patients. Recognition of scan patterns in patients with suspected lymphoedema or lymphatic vascular diseases is essential as it plays a major role in the clinical management.

  10. Parkinsonian Symptomatology May Correlate with CT Findings before and after Shunting in Idiopathic Normal Pressure Hydrocephalus

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    Mitsuaki Ishii

    2010-01-01

    Full Text Available We aimed to investigate the characteristics of Parkinsonian features assessed by the unified Parkinson's disease rating scale (UPDRS and determine their correlations with the computed tomography (CT findings in patients with idiopathic normal pressure hydrocephalus (iNPH. The total score and the scores for arising from chair, gait, postural stability, and body hypokinesia in the motor examination section of UPDRS were significantly improved after shunt operations. Stepwise multiple regression analysis revealed that postural stability was the determinant of the gait domain score of the iNPH grading scale. The canonical correlation analysis between the CT findings and the shunt-responsive Parkinsonian features indicated that Evans index rather than midbrain diameters had a large influence on the postural stability. Thus, the pathophysiology of postural instability as a cardinal feature of gait disturbance may be associated with impaired frontal projections close to the frontal horns of the lateral ventricles in the iNPH patients.

  11. Astrogliosis and impaired aquaporin-4 and dystrophin systems in idiopathic normal pressure hydrocephalus.

    Science.gov (United States)

    Eide, P K; Hansson, H-A

    2017-06-19

    Idiopathic normal pressure hydrocephalus (iNPH) is one subtype of dementia that may improve following drainage of cerebrospinal fluid (CSF). This prospective observational study explored whether expression of the water channel aquaporin-4 (AQP4) and the anchoring molecule dystrophin 71 (Dp71) are altered at astrocytic perivascular endfeet and in adjacent neuropil of iNPH patient. Observations were related to measurements of pulsatile and static intracranial pressure (ICP). The study included iNPH patients undergoing overnight monitoring of the pulsatile/static ICP in whom a biopsy was taken from the frontal cerebral cortex during placement of the ICP sensor. Reference (Ref) biopsies were sampled from 13 patients who underwent brain surgery for epilepsy, tumours or cerebral aneurysms. The brain tissue specimens were examined by light microscopy, immunohistochemistry, densitometry and morphometry. iNPH patients responding to surgery (n = 44) had elevated pulsatile ICP, indicative of impaired intracranial compliance. As compared to the Ref patients, the cortical biopsies of iNPH patients revealed prominent astrogliosis and reduced expression of AQP4 and Dp71 immunoreactivities in the astrocytic perivascular endfeet and in parts of the adjacent neuropil. There was a significant correlation between degree of astrogliosis and reduction of AQP4 and Dp71 at astrocytic perivascular endfeet. Idiopathic normal pressure hydrocephalus patients responding to CSF diversion present with abnormal pulsatile ICP, indicative of impaired intracranial compliance. A main histopathological finding was astrogliosis and reduction of AQP4 and of Dp71 in astrocytic perivascular endfeet. We propose that the altered AQP4 and Dp71 complex contributes to the subischaemia prevalent in the brain tissue of iNPH. © 2017 British Neuropathological Society.

  12. Pulmonary epithelial permeability in normal subjects and patients with idiopathic interstitial pneumonia

    International Nuclear Information System (INIS)

    Anazawa, Yoshiki; Isawa, Toyoharu; Teshima, Takeo; Miki, Makoto; Motomiya, Masakichi

    1991-01-01

    99m Tc-DTPA is a low molecular weight substance, which is believed to pass through the pulmonary epithelium when it is inhaled as an aerosol. We performed 99m Tc-DTPA inhalation studies in 10 nonsmoking normal subjects and 10 patients with biopsy proven idiopathic interstitial pneumonia prior to therapy. 99m Tc-DTPA aerosol was inhaled for 3 min with the subject in the supine position and radioactivity was measured anteriorly with a gamma camera and recorded on computer. Measurements were performed for 3 min with the subject inhaling aerosol and for the subsequent 30 min with the subject in the same position. Time activity curves from the five regions of interest (ROIs) including the entire left lung, the entire right lung, and the upper, middle and lower third of the right lung were separately fitted to a single exponential function for the initial 7 min following cessation of inhalation, and the respective clearance half life (t1/2) in min was calculated. Lung function data, arterial blood gas tensions and blood chemistry were also obtained for comparison with the t1/2 values. The t1/2 values were significantly smaller in all ROIs in patients with idiopathic interstitial pneumonia than in normal subjects, indicating an increased pulmonary epithelial permeability in these patients. There was no relationship between t1/2 and %DL co , %DL co /V A , PaO 2 , or LDH. Although the true pathophysiologic significance of t1/2 measured using 99m Tc-DTPA aerosol is still not known, we consider that this measurement may be an important indicator of nonrespiratory lung function, in particular the degree of alveolar epithelial damage. (author)

  13. Serial quantitative CT evaluation for patients with idiopathic pulmonary fibrosis (IPF) using Gaussian Histogram Normalized Correlation (GHNC)

    International Nuclear Information System (INIS)

    Iwasawa, Tae; Ogura, Takashi; Nishimura, Junichi; Asakura, Akira; Gotoh, Toshiyuki; Yazawa, Takuya; Inoue, Tomio

    2006-01-01

    We assessed serial changes in high-resolution CT findings quantitatively using originally developed software Gaussian Histogram Normalized Correlation (GHNC) in 15 patients with idiopathic pulmonary fibrosis (IPF). Mean follow-up period was 1.4 years. The volume of honeycombing increased with 0.8±0.9%TLC (predicted Total lung capacity) per year, the normal lung volume reduced by 4.1±7.3%TLC per year. GHNC is useful for the quantitative evaluation. (author)

  14. Unusual idiopathic normal pressure hydrocephalus patient with marked asymmetric and upper body parkinsonism

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    Kyunghun Kang

    2016-01-01

    Full Text Available Asymmetry of parkinsonian symptoms is strong evidence toward the diagnosis of Parkinson's disease (PD. Lower body parkinsonism is characteristic in idiopathic normal pressure hydrocephalus (INPH. We report an unusual INPH patient with marked asymmetric and upper body parkinsonism. An 83-year-old man presented with gait impairment and asymmetric clumsiness of movement. According to the Unified Parkinson's Disease Rating Scale (UPDRS, the motor subscore was 12 in the left limb and 8 in the right. The score was 14 for both the upper and lower body. After the cerebrospinal fluid tap test (CSFTT, he showed marked improvement in the upper body score. A loss of asymmetry of parkinsonian signs, with greater improvement in the left limb, was presented. Fluorinated N-3-fluoropropyl-2β-carbomethoxy-3β-(4-iodophenyl-nortropane (F-18 FP-CIT positron emission tomography (PET imaging was normal. In the differential diagnosis of elderly patients presenting with parkinsonism compatible with PD, we might need to consider a diagnosis of INPH.

  15. Responsiveness of cardiodynamics to exercise loading in normal subjects and in patients with idiopathic cardiomyopathy

    International Nuclear Information System (INIS)

    Fukuoka, Yukie; Matsuda, Takeshi

    1996-01-01

    In the present study, we examined the responses of the left ventricular systolic function and diastolic function to exertion by cardiac blood pool scintigraphy using 99mTc in reference to changes with aging and changes in the cases of idiopathic cardiomyopathy. In order to study the functional response with aging to exertion, 38 normal subjects were divided by age. Subsequently, 28 hypertrophic cardiomyopathy cases (Group H), 13 dilated cardiomyopathy cases (Group D) and 14 normal cases (Group N) were studied. Ejection Fraction (EF) and Peak Ejection Rate (PER) were used as the indicators for the systolic function. Peak Filling Rate (PER), 1/3 Filling Rate (1/3FR) and Time to Peak Filling (TPF) were used as the indicators for the diastolic function. When comparison was made among the normal subjects by age, the systolic function and diastolic function at rest, varied as they were, showed no significant change with aging. The %delta EF and %delta PER tended to decrease linearly with aging (Y=-4E-X+28, p<0.0001; Y=-X+57, p<0.0001). As to the %delta PFR, %delta 1/3FR and %delta TPF, however, correlation with aging was not found. In comparing hypertrophic cardiomyopathy and dilated cardiomyopathy, Group D showed significantly lower values before exertion and after maximum exertion than the other two groups. A difference in the response of the systolic function and diastolic function to exertion was noted in both the comparative study on aging of the normal subjects and the study of cases of cardiomyopathy. The presence of the compensatory mechanism due to elevation of the left atrial pressure and an increase in the left atrial contractile power may be mentioned as a reservoir of blood and also as a booster pump. Particularly, the effect of the latter compensates for the decline in the left ventricular compliance, which may lead to the maintenance of the diastolic function. (K.H.)

  16. Adolescent Idiopathic Scoliosis: From Normal Spinal Anatomy To Three-Dimensional Deformity

    NARCIS (Netherlands)

    Schlösser, T.P.C.

    2014-01-01

    Adolescent idiopathic scoliosis is a three-dimensional deformity of the spine and trunk that primarily affects previously healthy children. It is a classic orthopedic disorder. Despite many years of dedicated research into the etio-pathogenesis of idiopathic scoliosis, there is not one distinct

  17. Mechanism of right thoracic adolescent idiopathic scoliosis at risk for progression; a unifying pathway of development by normal growth and imbalance

    DEFF Research Database (Denmark)

    Wong, Christian

    2015-01-01

    Adolescent idiopathic scoliosis is regarded as a multifactorial disease and none of the many suggested causal etiologies have yet prevailed. I will suggest that adolescent idiopathic scoliosis has one common denominator, namely that initial curve development is mediated through one common normal...

  18. Diffusion tensor imaging of idiopathic normal pressure hydrocephalus. A voxel-based fractional anisotropy study

    International Nuclear Information System (INIS)

    Koyama, Tetsuo; Ohmura, Takehisa; Miyake, Hiroji; Marumoto, Kohei; Domen, Kazuhisa

    2012-01-01

    Diffusion tensor imaging (DTI) using a 3.0 tesla magnetic resonance scanner was used to investigate white matter changes caused by idiopathic normal pressure hydrocephalus (INPH) in 10 patients diagnosed by clinical symptoms (gait disturbance, dementia, and/or urinary incontinence) and Evans index >0.3, and compared with findings for 10 age-matched controls (≥60 years). Then, using a computer-automated method, fractional anisotropy (FA) brain maps were generated and finally transformed into the standard space. Voxel-based FA values within two regions of interests (ROIs), the forceps minor and corticospinal tracts, were then separately evaluated. Within each ROI, statistical comparisons of results from the INPH and control groups were performed. In addition, for INPH patients, grading scores for clinical symptoms and FA values were correlated. The forceps minor mean FA value was much smaller for the INPH group (0.504) than for the control group (0.631). The corticospinal tract mean FA value was slightly smaller for the INPH group (0.588) than for the control group (0.632). Additional analyses indicated that lower FA values within the forceps minor tended to be associated with clinical symptoms such as urinary incontinence and gait disturbance. Our findings indicate FA values decreased in the forceps minor of INPH patients. We also found that lower values were associated with severer clinical symptoms, implying that DTI techniques may be developed for more accurate diagnosis. (author)

  19. 18F-FDG PET-CT pattern in idiopathic normal pressure hydrocephalus.

    Science.gov (United States)

    Townley, Ryan A; Botha, Hugo; Graff-Radford, Jonathan; Boeve, Bradley F; Petersen, Ronald C; Senjem, Matthew L; Knopman, David S; Lowe, Val; Jack, Clifford R; Jones, David T

    2018-01-01

    Idiopathic normal pressure hydrocephalus (iNPH) is an important and treatable cause of neurologic impairment. Diagnosis is complicated due to symptoms overlapping with other age related disorders. The pathophysiology underlying iNPH is not well understood. We explored FDG-PET abnormalities in iNPH patients in order to determine if FDG-PET may serve as a biomarker to differentiate iNPH from common neurodegenerative disorders. We retrospectively compared 18 F-FDG PET-CT imaging patterns from seven iNPH patients (mean age 74 ± 6 years) to age and sex matched controls, as well as patients diagnosed with clinical Alzheimer's disease dementia (AD), Dementia with Lewy Bodies (DLB) and Parkinson's Disease Dementia (PDD), and behavioral variant frontotemporal dementia (bvFTD). Partial volume corrected and uncorrected images were reviewed separately. Patients with iNPH, when compared to controls, AD, DLB/PDD, and bvFTD, had significant regional hypometabolism in the dorsal striatum, involving the caudate and putamen bilaterally. These results remained highly significant after partial volume correction. In this study, we report a FDG-PET pattern of hypometabolism in iNPH involving the caudate and putamen with preserved cortical metabolism. This pattern may differentiate iNPH from degenerative diseases and has the potential to serve as a biomarker for iNPH in future studies. These findings also further our understanding of the pathophysiology underlying the iNPH clinical presentation.

  20. Relevances between cerebral circulatory disorder and symptom in idiopathic normal pressure hydrocephalus

    International Nuclear Information System (INIS)

    Takeuchi, Totaro; Shimizu, Tsuneo; Watanabe, Kazuo

    2010-01-01

    The subjects were shunt-effective idiopathic normal pressure hydrocephalus (iNPH) 40 patients. Before and one year after surgery, measurements of the cerebral circulatory dynamics (the cerebral blood flow pattern by region of interest (ROI) setting using 123 I-iofetamine (IMP) single photon emission computed tomography (SPECT)), and an evaluation of symptoms (gait disturbance: G, dementia: D, urinary incontinence: U) by grading scale (JNPHGS-R). Preoperative cerebral circulation and symptoms: As for the each symptoms and blood flow patterns, the seventies of G (p=0.017) in whole cortex non-reduction group (thalamus-basal ganglia reduction group) and D (p=0.021) in whole cortex reduction group were obviously high compared with other symptoms particularly. Circulation and symptoms one year after surgery: As the circulatory dynamics at different improvement sites and each symptoms, it was obviously mild for G (p=0.003) in the group with an increased only thalamus-basal ganglia blood flow and increased both whole cortex and thalamus-basal ganglia blood flow and tend to mild for D (p=0.091) in the group with an increased only whole cortex blood flow and increased both whole cortex and thalamus-basal ganglia blood flow compared with other symptoms. (author)

  1. 18F-FDG PET-CT pattern in idiopathic normal pressure hydrocephalus

    Directory of Open Access Journals (Sweden)

    Ryan A. Townley

    Full Text Available Background: Idiopathic normal pressure hydrocephalus (iNPH is an important and treatable cause of neurologic impairment. Diagnosis is complicated due to symptoms overlapping with other age related disorders. The pathophysiology underlying iNPH is not well understood. We explored FDG-PET abnormalities in iNPH patients in order to determine if FDG-PET may serve as a biomarker to differentiate iNPH from common neurodegenerative disorders. Methods: We retrospectively compared 18F-FDG PET-CT imaging patterns from seven iNPH patients (mean age 74 ± 6 years to age and sex matched controls, as well as patients diagnosed with clinical Alzheimer's disease dementia (AD, Dementia with Lewy Bodies (DLB and Parkinson's Disease Dementia (PDD, and behavioral variant frontotemporal dementia (bvFTD. Partial volume corrected and uncorrected images were reviewed separately. Results: Patients with iNPH, when compared to controls, AD, DLB/PDD, and bvFTD, had significant regional hypometabolism in the dorsal striatum, involving the caudate and putamen bilaterally. These results remained highly significant after partial volume correction. Conclusions: In this study, we report a FDG-PET pattern of hypometabolism in iNPH involving the caudate and putamen with preserved cortical metabolism. This pattern may differentiate iNPH from degenerative diseases and has the potential to serve as a biomarker for iNPH in future studies. These findings also further our understanding of the pathophysiology underlying the iNPH clinical presentation. Keywords: FDG-PET, Normal pressure hydrocephalus, Hypometabolism, Caudate, Biomarker

  2. Impact of cerebrospinal fluid shunting for idiopathic normal pressure hydrocephalus on the amyloid cascade.

    Directory of Open Access Journals (Sweden)

    Masao Moriya

    Full Text Available The aim of this study was to determine whether the improvement of cerebrospinal fluid (CSF flow dynamics by CSF shunting, can suppress the oligomerization of amyloid β-peptide (Aβ, by measuring the levels of Alzheimer's disease (AD-related proteins in the CSF before and after lumboperitoneal shunting. Lumbar CSF from 32 patients with idiopathic normal pressure hydrocephalus (iNPH (samples were obtained before and 1 year after shunting, 15 patients with AD, and 12 normal controls was analyzed for AD-related proteins and APLP1-derived Aβ-like peptides (APL1β (a surrogate marker for Aβ. We found that before shunting, individuals with iNPH had significantly lower levels of soluble amyloid precursor proteins (sAPP and Aβ38 compared to patients with AD and normal controls. We divided the patients with iNPH into patients with favorable (improvement ≥ 1 on the modified Rankin Scale and unfavorable (no improvement on the modified Rankin Scale outcomes. Compared to the unfavorable outcome group, the favorable outcome group showed significant increases in Aβ38, 40, 42, and phosphorylated-tau levels after shunting. In contrast, there were no significant changes in the levels of APL1β25, 27, and 28 after shunting. After shunting, we observed positive correlations between sAPPα and sAPPβ, Aβ38 and 42, and APL1β25 and 28, with shifts from sAPPβ to sAPPα, from APL1β28 to 25, and from Aβ42 to 38 in all patients with iNPH. Our results suggest that Aβ production remained unchanged by the shunt procedure because the levels of sAPP and APL1β were unchanged. Moreover, the shift of Aβ from oligomer to monomer due to the shift of Aβ42 (easy to aggregate to Aβ38 (difficult to aggregate, and the improvement of interstitial-fluid flow, could lead to increased Aβ levels in the CSF. Our findings suggest that the shunting procedure can delay intracerebral deposition of Aβ in patients with iNPH.

  3. Automatic Volumetry of the Cerebrospinal Fluid Space in Idiopathic Normal Pressure Hydrocephalus

    Directory of Open Access Journals (Sweden)

    Kazunari Ishii

    2013-12-01

    Full Text Available Objectives: To measure the cerebrospinal fluid (CSF space volume in idiopathic normal pressure hydrocephalus (INPH, we developed a software that allows us to automatically measure the regional CSF space and compared the volumes of the ventricle systems (VS, Sylvian fissures (SF and sulci at high convexity and midline (SHM among INPH patients, Alzheimer's disease (AD patients and healthy volunteers (HVs. Methods: Fifteen INPH patients, 15 AD patients and 15 HVs were retrospectively selected for this study. 3D-T1 MR images were obtained. We improved upon an automatic gray matter volume system to measure CSF spaces, adopting new regions for the template of INPH-characteristic CSF spaces and measured them. The VS, SF and SHM volumes were calculated relative to the intracranial volume. Results: The relative SHM volume of the INPH group (0.0237 ± 0.0064 was the smallest among the 3 groups (AD: 0.0477 ± 0.0109, HV: 0.0542 ± 0.0045. The VS (0.0499 ± 0.0135 and SF (0.0187 ± 0.0037 volumes of the INPH group were significantly larger than those of the AD (VS: 0.0311 ± 0.0075, SF: 0.0146 ± 0.0026 and HV groups (VS: 0.0167 ± 0.0065, SF: 0.0111 ± 0.017. Conclusion: Automatic volume measurement can be used to delineate the characteristic changes in CSF space in patients with INPH and is useful in the diagnosis of INPH.

  4. Quantifying response to intracranial pressure normalization in idiopathic intracranial hypertension via dynamic neuroimaging.

    Science.gov (United States)

    Lublinsky, Svetlana; Kesler, Anat; Friedman, Alon; Horev, Anat; Shelef, Ilan

    2018-04-01

    Idiopathic intracranial hypertension (IIH) is characterized by elevated intracranial pressure without a clear cause. To investigate dynamic imaging findings in IIH and their relation to mechanisms underlying intracranial pressure normalization. Prospective. Eighteen IIH patients and 30 healthy controls. T 1 -weighted, venography, fluid attenuation inversion recovery, and apparent diffusion coefficients were acquired on 1.5T scanner. The dural sinus was measured before and after lumbar puncture (LP). The degree of sinus occlusion was evaluated, based on 95% confidence intervals of controls. We studied a number of neuroimaging biomarkers associated with IIH (sinus occlusion; optic nerve; distribution of cerebrospinal fluid into the subarachnoid space, sulci and lateral ventricles (LVs); Meckel's caves; arachnoid granulation; pituitary and choroid plexus), before and after LP, using a set of specially developed quantification techniques. Relationships among various biomarkers were investigated (Pearson correlation coefficient) and linked to long-term disease outcomes (logistic regression). The t-test and the Wilcoxon rank test were used to compare between controls and before and after LP data. As a result of LP, the following were found to be in good accordance with the opening pressure: relative compression of cerebrospinal fluid (R = -0.857, P < 0.001) and brain volumes (R = -0.576, P = 0.012), LV expansion (R = 0.772, P < 0.001) and venous volume (R = 0.696, P = 0.001), enlargement of the pituitary (R = 0.640, P = 0.023), and shrinkage of subarachnoid space (R = -0.887, P < 0.001). The only parameter that had an impact on long-term prognosis was cross-sectional size of supplemental drainage veins after LP (sensitivity of 92%, specificity of 20%, and area under the curve of 0.845, P < 0.001). We present an approach for quantitative characterization of the intracranial venous system and its implementation as a diagnostic assistance

  5. Prevalence of lateral ventricle asymmetry in brain MRI studies of neurologically normal dogs and dogs with idiopathic epilepsy.

    Science.gov (United States)

    Pivetta, Mauro; De Risio, Luisa; Newton, Richard; Dennis, Ruth

    2013-01-01

    Asymmetry of the cerebral lateral ventricles is a common finding in cross-sectional imaging of otherwise normal canine brains and has been assumed to be incidental. The purpose of this retrospective study was to compare the prevalence of ventricular asymmetry in brain MRI studies of normal dogs and dogs with idiopathic epilepsy. Brain MRI archives were searched for 100 neurologically normal dogs (Group 1) and 100 dogs with idiopathic epilepsy (Group 2). For each dog, asymmetry of the lateral ventricles was subjectively classified as absent, mild, moderate, and severe based on a consensus of two observers who were unaware of group status. Ventricular areas were measured from transverse T1W images at the level of the interthalamic adhesion. An asymmetry ratio was calculated as the ratio of the larger to smaller ventricular transverse area. There was excellent agreement between subjective assessments of ventricular asymmetry and quantitative assessments using asymmetry ratios (k = 0.995). The prevalence of asymmetry was 38% in Group 1 dogs and 44% in Group 2 dogs. Assymmetry was scored as mild in the majority of Group 2 dogs. There was no significant association between presence/absence and degree of ventricular asymmetry vs. dog group, age, gender, or skull conformation. Findings from the current study supported previously published assumptions that asymmetry of the lateral cerebral ventricles is an incidental finding in MRI studies of the canine brain. © 2013 Veterinary Radiology & Ultrasound.

  6. The European iNPH Multicentre Study on the predictive values of resistance to CSF outflow and the CSF Tap Test in patients with idiopathic normal pressure hydrocephalus

    DEFF Research Database (Denmark)

    Wikkelsø, Carsten; Hellström, Per; Klinge, Petra Margarete

    2013-01-01

    The objective was to determine the sensitivity, specificity, and positive and negative predictive values of the CSF Tap Test (CSF TT) and resistance to CSF outflow (Rout) for the outcome of shunting in a sample of patients with idiopathic normal pressure hydrocephalus (iNPH).......The objective was to determine the sensitivity, specificity, and positive and negative predictive values of the CSF Tap Test (CSF TT) and resistance to CSF outflow (Rout) for the outcome of shunting in a sample of patients with idiopathic normal pressure hydrocephalus (iNPH)....

  7. Volúmenes pulmonares normales en pacientes con fibrosis pulmonar idiopática y enfisema Normal lung volumes in patients with idiopathic pulmonary fibrosis and emphysema

    Directory of Open Access Journals (Sweden)

    Juan Pablo Casas

    2008-08-01

    Full Text Available La fibrosis pulmonar idiopática (FPI es una enfermedad que se caracteriza por presentar un compromiso pulmonar de tipo restrictivo, resultante de una reducción en la complacencia pulmonar secundaria a fibrosis difusa. En el enfisema, la pérdida de elasticidad pulmonar y el colapso de las vías aéreas periféricas generan obstrucción e hiperinflación. El efecto simultáneo que ambas enfermedades producen sobre la fisiología pulmonar no es del todo claro y se han descripto volúmenes pulmonares normales o casi normales. Presentamos 4 pacientes de sexo masculino de 64, 60, 73 y 70 años, con antecedentes de tabaquismo e historia de disnea progresiva, tres de ellos con grave limitación en su calidad de vida al momento de la consulta. En la tomografía de tórax de alta resolución todos los pacientes presentaban signos de enfermedad intersticial pulmonar avanzada, con cambios de tipo fibrótico con predominio basal y subpleural, que coexistían con enfisema centroacinar con predominio en lóbulos superiores. Uno de ellos tuvo confirmación diagnóstica de ambas condicioes por biopsia pulmonar a cielo abierto. En los cuatro pacientes la espirometría y volúmenes pulmonares fueron normales, pero tenían importante compromiso del intercambio gaseoso evaluado mediante el test de caminata de 6 minutos. Tres de los pacientes tenían hipertensión pulmonar grave diagnosticado por ecocardiograma. La presencia de volúmenes pulmonares normales no excluye un diagnóstico de fibrosis pulmonar idiopática en pacientes fumadores si coexisten evidencias tomográficas de enfisema. En estos pacientes el grado de compromiso funcional, determinado por la reducción de los volúmenes pulmonares, no debería ser considerado en la evaluación de la gravedad.Pulmonary function tests in idiopathic pulmonary fibrosis characteristically show a restrictive pattern, resulting from reduction of pulmonary compliance due to diffuse fibrosis. Conversely, an obstructive

  8. Improvement in social function and health-related quality of life after shunt surgery for idiopathic normal-pressure hydrocephalus.

    Science.gov (United States)

    Petersen, Jakob; Hellström, Per; Wikkelsø, Carsten; Lundgren-Nilsson, Asa

    2014-10-01

    To investigate the impact of shunt surgery on the activity, participation, autonomy, and health-related quality of life (HRQOL) of patients with idiopathic normal-pressure hydrocephalus (iNPH) as well as the effect on caregiver burden. Thirty-seven patients (median age 70 years, range 50-89 years) with iNPH were evaluated before and 6 months after surgery. Symptoms and signs were assessed by the iNPH scale, activities of daily living (ADL) with the Functional Independence Measure (FIM) and Assessment of Motor and Process Skills (AMPS), autonomy and participation with Impact on Participation and Autonomy (IPA), and caregiver burden with the Caregiver Burden Scale (CBS). HRQOL was evaluated with the EQ-5D (EuroQol Group-5 Dimension health survey). Twenty-four patients (65%) improved clinically (iNPH scale score) and 31 (86%) improved their HRQOL after surgery, almost to the same level as found in the normal population. The patients became more independent in physical and cognitive activities, and participation and autonomy improved. The caregiver burden was decreased among caregivers to male patients but remained unchanged on the overall group level. After shunt surgery, patients with iNPH showed improvement in most aspects of social life, they became more independent, and their quality of life returned to nearly normal.

  9. Diffusion imaging of reversible and irreversible microstructural changes within the corticospinal tract in idiopathic normal pressure hydrocephalus

    Directory of Open Access Journals (Sweden)

    Kouhei Kamiya

    2017-01-01

    Full Text Available The symptoms of idiopathic normal pressure hydrocephalus (iNPH can be improved by shunt surgery, but prediction of treatment outcome is not established. We investigated changes of the corticospinal tract (CST in iNPH before and after shunt surgery by using diffusion microstructural imaging, which infers more specific tissue properties than conventional diffusion tensor imaging. Two biophysical models were used: neurite orientation dispersion and density imaging (NODDI and white matter tract integrity (WMTI. In both methods, the orientational coherence within the CSTs was higher in patients than in controls, and some normalization occurred after the surgery in patients, indicating axon stretching and recovery. The estimated axon density was lower in patients than in controls but remained unchanged after the surgery, suggesting its potential as a marker for irreversible neuronal damage. In a Monte-Carlo simulation that represented model axons as undulating cylinders, both NODDI and WMTI separated the effects of axon density and undulation. Thus, diffusion MRI may distinguish between reversible and irreversible microstructural changes in iNPH. Our findings constitute a step towards a quantitative image biomarker that reflects pathological process and treatment outcomes of iNPH.

  10. The effect of backpack weight on the standing posture and balance of schoolgirls with adolescent idiopathic scoliosis and normal controls.

    Science.gov (United States)

    Chow, Daniel H K; Kwok, Monica L Y; Cheng, Jack C Y; Lao, Miko L M; Holmes, Andrew D; Au-Yang, Alexander; Yao, Fiona Y D; Wong, M S

    2006-10-01

    Concerns have been raised regarding the effect of carrying a backpack on adolescent posture and balance, but the effect of backpack loading combined with other factors affecting balance, such as adolescent idiopathic scoliosis (AIS), has not been determined. This study examines the effects of backpack load on the posture and balance of schoolgirls with AIS and normal controls. The standing posture of 26 schoolgirls with mild AIS (mean age 13, Cobb angle 10-25 degrees ) and 20 age-matched normal schoolgirls were recorded without a backpack and while carrying a standard dual-strap backpack loaded at 7.5%, 10%, 12.5% and 15% of the subject's bodyweight (BW). Kinematics of the pelvis, trunk and head were recorded using a motion analysis system and centre of pressure (COP) data were recorded using a force platform. Reliable COP data could only be derived for 13 of the subjects with AIS. Increasing backpack load causes a significantly increased flexion of the trunk in relation to the pelvis and extension of the head in relation to the trunk, as well as increased antero-posterior range of COP motion. While backpack load appears to affect balance predominantly in the antero-posterior direction, differences between groups were more evident in the medio-lateral direction, with AIS subjects showing poor balance in this direction. Overall, carrying a backpack causes similar sagittal plane changes in posture and balance in both normal and AIS groups. Load size or subject group did not influence balance, but the additive effect of backpack carrying and AIS on postural control alters the risk of fall in this population. Therefore, load limit recommendations based on normal subjects should not be applicable to subjects with AIS.

  11. Usefulness of the convexity apparent hyperperfusion sign in 123I-iodoamphetamine brain perfusion SPECT for the diagnosis of idiopathic normal pressure hydrocephalus.

    Science.gov (United States)

    Ohmichi, Takuma; Kondo, Masaki; Itsukage, Masahiro; Koizumi, Hidetaka; Matsushima, Shigenori; Kuriyama, Nagato; Ishii, Kazunari; Mori, Etsuro; Yamada, Kei; Mizuno, Toshiki; Tokuda, Takahiko

    2018-03-16

    OBJECTIVE The gold standard for the diagnosis of idiopathic normal pressure hydrocephalus (iNPH) is the CSF removal test. For elderly patients, however, a less invasive diagnostic method is required. On MRI, high-convexity tightness was reported to be an important finding for the diagnosis of iNPH. On SPECT, patients with iNPH often show hyperperfusion of the high-convexity area. The authors tested 2 hypotheses regarding the SPECT finding: 1) it is relative hyperperfusion reflecting the increased gray matter density of the convexity, and 2) it is useful for the diagnosis of iNPH. The authors termed the SPECT finding the convexity apparent hyperperfusion (CAPPAH) sign. METHODS Two clinical studies were conducted. In study 1, SPECT was performed for 20 patients suspected of having iNPH, and regional cerebral blood flow (rCBF) of the high-convexity area was examined using quantitative analysis. Clinical differences between patients with the CAPPAH sign (CAP) and those without it (NCAP) were also compared. In study 2, the CAPPAH sign was retrospectively assessed in 30 patients with iNPH and 19 healthy controls using SPECT images and 3D stereotactic surface projection. RESULTS In study 1, rCBF of the high-convexity area of the CAP group was calculated as 35.2-43.7 ml/min/100 g, which is not higher than normal values of rCBF determined by SPECT. The NCAP group showed lower cognitive function and weaker responses to the removal of CSF than the CAP group. In study 2, the CAPPAH sign was positive only in patients with iNPH (24/30) and not in controls (sensitivity 80%, specificity 100%). The coincidence rate between tight high convexity on MRI and the CAPPAH sign was very high (28/30). CONCLUSIONS Patients with iNPH showed hyperperfusion of the high-convexity area on SPECT; however, the presence of the CAPPAH sign did not indicate real hyperperfusion of rCBF in the high-convexity area. The authors speculated that patients with iNPH without the CAPPAH sign, despite showing

  12. Microstructural changes of the corticospinal tract in idiopathic normal pressure hydrocephalus: a comparison of diffusion tensor and diffusional kurtosis imaging

    International Nuclear Information System (INIS)

    Nakanishi, Atsushi; Hori, Masaaki; Aoki, Shigeki; Fukunaga, Issei; Masutani, Yoshitaka; Takaaki, Hattori; Miyajima, Masakazu

    2013-01-01

    The goals of this study were to examine the usefulness of diffusional kurtosis imaging (DKI) for assessing microstructural changes in the compressed corticospinal tract (CST) among patients with idiopathic normal pressure hydrocephalus (iNPH). Eleven patients with iNPH (mean age: 73.6 years, range: 65-84), who underwent 3-T magnetic resonance imaging, including DKI before surgery, were recruited. Six age-matched, healthy subjects (mean age: 69.8 years, range: 60-75) served as the control group. DKI and diffusion tensor imaging parameters were calculated and compared between the iNPH and the control groups using tract-specific analysis of the CST at the level of the lateral ventricle. Mean diffusional kurtosis (DK) and axial diffusion kurtosis were significantly lower in iNPH patients. However, apparent diffusion coefficient, fractional anisotropy, and axial eigenvalue (λ 1 ) were significantly higher in the iNPH group than in the control group. The mechanical pressure caused by ventricular enlargement in iNPH patients might induce formation of well-aligned fiber tracts and increased fiber density in the CST, resulting in decreased DK. DKI is able to depict both the altered microstructure and water molecule movement within neural axons and intra- or extracellular space. In addition, the investigated DKI parameters provide different information about white matter relative to conventional diffusional metrics for iNPH. (orig.)

  13. Microstructural changes of the corticospinal tract in idiopathic normal pressure hydrocephalus: a comparison of diffusion tensor and diffusional kurtosis imaging

    Energy Technology Data Exchange (ETDEWEB)

    Nakanishi, Atsushi; Hori, Masaaki; Aoki, Shigeki [Juntendo University, Department of Radiology, School of Medicine, Bunkyo-ku, Tokyo (Japan); Fukunaga, Issei [Juntendo University, Department of Radiology, School of Medicine, Bunkyo-ku, Tokyo (Japan); Tokyo Metropolitan University, Department of Health Science, Graduate School of Human Health Sciences, Arakawa, Tokyo (Japan); Masutani, Yoshitaka [The University of Tokyo, Division of Radiology and Biomedical Engineering, Graduate School of Medicine, Bunkyo-ku, Tokyo (Japan); Takaaki, Hattori [Tokyo Medical and Dental University, Department of Neurology and Neurological Science, Graduate School, Bunkyo-ku, Tokyo (Japan); Miyajima, Masakazu [Juntendo University, Department of Neurosurgery, School of Medicine, Bunkyo-ku, Tokyo (Japan)

    2013-08-15

    The goals of this study were to examine the usefulness of diffusional kurtosis imaging (DKI) for assessing microstructural changes in the compressed corticospinal tract (CST) among patients with idiopathic normal pressure hydrocephalus (iNPH). Eleven patients with iNPH (mean age: 73.6 years, range: 65-84), who underwent 3-T magnetic resonance imaging, including DKI before surgery, were recruited. Six age-matched, healthy subjects (mean age: 69.8 years, range: 60-75) served as the control group. DKI and diffusion tensor imaging parameters were calculated and compared between the iNPH and the control groups using tract-specific analysis of the CST at the level of the lateral ventricle. Mean diffusional kurtosis (DK) and axial diffusion kurtosis were significantly lower in iNPH patients. However, apparent diffusion coefficient, fractional anisotropy, and axial eigenvalue ({lambda} {sub 1}) were significantly higher in the iNPH group than in the control group. The mechanical pressure caused by ventricular enlargement in iNPH patients might induce formation of well-aligned fiber tracts and increased fiber density in the CST, resulting in decreased DK. DKI is able to depict both the altered microstructure and water molecule movement within neural axons and intra- or extracellular space. In addition, the investigated DKI parameters provide different information about white matter relative to conventional diffusional metrics for iNPH. (orig.)

  14. Mediastinal and extrapleural fat hypertrophy in idiopathic pulmonary fibrosis on high-resolution CT: comparison with normal individuals

    International Nuclear Information System (INIS)

    Son, Kyu Ri; Lee, Hyun Ju; Lim, Kun Young; Lee, Chang Hyun; Goo, Jin Mo; Im, Jung Gi

    2004-01-01

    We wished to compare the amount of mediastinal and extrapleural fat on high resolution CT for patients with idiopathic pulmonary fibrosis (IPF) with that of normal individuals, and we wished to evaluate the correlation between the amount of fat and the degree of pulmonary fibrosis. We selected a group of 25 patients with radiologically and clinically diagnosed IPF and we also selected another group of 25 age and gender-matched patients having no abnormalities on pulmonary function testing as well as HRCT as controls from our radiologic database search (mean age: 59 years, M:F= 11:14). We measured the area of mediastinal and extrapleural fat at the levels of the aortic arch and at the origin of the right pulmonary artery and right inferior pulmonary vein on three sections of HRCT by using software (Rapidia; 3DMED, Seoul, Korea). The total amount of fat was calculated by summing up the areas of the mediastinal and extrapleural fat, which is corrected by the body mass index; we also evaluated statistical differences between the two groups. At same sections of CT, the ratio (%) of the honeycombing area to the total areas of the lung was calculated. We evaluated the relationship between the amount of extrapleural or mediastinal fat with the ratio (%) of the honeycombing area. The total amount of fat in patients with IPF and normal individuals were 67.24±19.03 cm 2 and 32.55±11.91 cm 2 , respectively. The fat amount corrected by body mass index was 280.48±74.43 mm 2 /kg/m 2 in the IPF patients and 137.06±41.76 mm 2 /kg/m 2 in normal individuals. The differences between two groups for the total amount of fat and fat amount, as corrected for by the body mass index, were statistically significant (p<0.0001). The ratio (%) of the honeycombing area and the total amount of fat showed a moderate correlation (rho= 0.43, p=0.032). Patients with IPF have a larger amount of mediastinal and extrapleural fat than normal individuals. The hypertrophy of mediastinal and extrapleural

  15. Mediastinal and extrapleural fat hypertrophy in idiopathic pulmonary fibrosis on high-resolution CT: comparison with normal individuals

    Energy Technology Data Exchange (ETDEWEB)

    Son, Kyu Ri; Lee, Hyun Ju; Lim, Kun Young; Lee, Chang Hyun; Goo, Jin Mo; Im, Jung Gi [College of Medicine, Seoul National Univ., Seoul (Korea, Republic of)

    2004-12-01

    We wished to compare the amount of mediastinal and extrapleural fat on high resolution CT for patients with idiopathic pulmonary fibrosis (IPF) with that of normal individuals, and we wished to evaluate the correlation between the amount of fat and the degree of pulmonary fibrosis. We selected a group of 25 patients with radiologically and clinically diagnosed IPF and we also selected another group of 25 age and gender-matched patients having no abnormalities on pulmonary function testing as well as HRCT as controls from our radiologic database search (mean age: 59 years, M:F= 11:14). We measured the area of mediastinal and extrapleural fat at the levels of the aortic arch and at the origin of the right pulmonary artery and right inferior pulmonary vein on three sections of HRCT by using software (Rapidia; 3DMED, Seoul, Korea). The total amount of fat was calculated by summing up the areas of the mediastinal and extrapleural fat, which is corrected by the body mass index; we also evaluated statistical differences between the two groups. At same sections of CT, the ratio (%) of the honeycombing area to the total areas of the lung was calculated. We evaluated the relationship between the amount of extrapleural or mediastinal fat with the ratio (%) of the honeycombing area. The total amount of fat in patients with IPF and normal individuals were 67.24{+-}19.03 cm{sup 2} and 32.55{+-}11.91 cm{sup 2}, respectively. The fat amount corrected by body mass index was 280.48{+-}74.43 mm{sup 2}/kg/m{sup 2} in the IPF patients and 137.06{+-}41.76 mm{sup 2}/kg/m{sup 2} in normal individuals. The differences between two groups for the total amount of fat and fat amount, as corrected for by the body mass index, were statistically significant (p<0.0001). The ratio (%) of the honeycombing area and the total amount of fat showed a moderate correlation (rho= 0.43, p=0.032). Patients with IPF have a larger amount of mediastinal and extrapleural fat than normal individuals. The

  16. Gait and Balance Measures Can Identify Change From a Cerebrospinal Fluid Tap Test in Idiopathic Normal Pressure Hydrocephalus.

    Science.gov (United States)

    Gallagher, Ryan; Marquez, Jodie; Osmotherly, Peter

    2018-04-25

    To identify in patients with idiopathic normal pressure hydrocephalus (iNPH) undergoing a cerebrospinal fluid (CSF) tap test (TT) for consideration of a ventricular peritoneal (VP) shunt: (1) gait and balance measures, which identify symptom change; (2) differences present between pre- and post-CSF TT scores between patients classified as responders and nonresponder; (3) ability of patients with iNPH to accurately quantify change in their gait and balance symptoms from a CSF TT. Prospective observational study. Post-CSF TT assessment was completed 2-4 hours post. Tertiary referral neurological and neurosurgical hospital. Patients (N=74) with iNPH receiving a 30 mL CSF TT for consideration of a VP shunt. Patients underwent a battery of gait and balance measures pre- and post-CSF TT and indicated their perceived change on a global rating of change (GRC). Patients deemed to improve and offered VP shunt insertion by a neurologist or neurosurgeon were labeled responders. Performance oriented mobility assessment (Tinetti), Berg Balance Scale (BBS), timed Up and Go (TUG), 10-meter walk test (10MWT), GRC. Forty patients were classified responders, 34 nonresponders. Significant differences were identified for responders: Tinetti (3.88 points), TUG (3.98 seconds), 10MWT (0.08 m/sec), and BBS (5.29 points). Significant differences were found for nonresponders for the Tinetti (0.91 points) and BBS (2.06 points). Change scores for responders and nonresponders were significantly different for all tests between responders and nonresponders. GRC scores for gait (+2 for responders, 0 for nonresponders) and balance (+2.5 for responders, 0 for nonresponders) were both significantly different. The Tinetti, BBS, and TUG can identify change in patients undergoing a CSF TT for iNPH. Patients appear to be able to accurately identify if change has occurred. Copyright © 2018 American Congress of Rehabilitation Medicine. Published by Elsevier Inc. All rights reserved.

  17. Automated method to compute Evans index for diagnosis of idiopathic normal pressure hydrocephalus on brain CT images

    Science.gov (United States)

    Takahashi, Noriyuki; Kinoshita, Toshibumi; Ohmura, Tomomi; Matsuyama, Eri; Toyoshima, Hideto

    2017-03-01

    The early diagnosis of idiopathic normal pressure hydrocephalus (iNPH) considered as a treatable dementia is important. The iNPH causes enlargement of lateral ventricles (LVs). The degree of the enlargement of the LVs on CT or MR images is evaluated by using a diagnostic imaging criterion, Evans index. Evans index is defined as the ratio of the maximal width of frontal horns (FH) of the LVs to the maximal width of the inner skull (IS). Evans index is the most commonly used parameter for the evaluation of ventricular enlargement. However, manual measurement of Evans index is a time-consuming process. In this study, we present an automated method to compute Evans index on brain CT images. The algorithm of the method consisted of five major steps: standardization of CT data to an atlas, extraction of FH and IS regions, the search for the outmost points of bilateral FH regions, determination of the maximal widths of both the FH and the IS, and calculation of Evans index. The standardization to the atlas was performed by using linear affine transformation and non-linear wrapping techniques. The FH regions were segmented by using a three dimensional region growing technique. This scheme was applied to CT scans from 44 subjects, including 13 iNPH patients. The average difference in Evans index between the proposed method and manual measurement was 0.01 (1.6%), and the correlation coefficient of these data for the Evans index was 0.98. Therefore, this computerized method may have the potential to accurately compute Evans index for the diagnosis of iNPH on CT images.

  18. Detection of changes in cerebrospinal fluid space in idiopathic normal pressure hydrocephalus using voxel-based morphometry

    Energy Technology Data Exchange (ETDEWEB)

    Yamashita, Fumio; Asada, Takashi [University of Tsukuba, Clinical Neuroscience, Medical Sciences for Control of Pathological Process, Graduate School of Comprehensive Human Sciences, Tsukuba-shi, Ibaraki-ken (Japan); Sasaki, Makoto; Kudo, Kohsuke [Iwate Medical University, Advanced Medical Research Center, Morioka (Japan); Takahashi, Satoshi; Narumi, Shinsuke; Terayama, Yasuo [Iwate Medical University, Department of Neurology, Morioka (Japan); Matsuda, Hiroshi [Saitama Medical University International Medical Center, Department of Nuclear Medicine, Saitama (Japan)

    2010-05-15

    We attempted to detect alterations in the cerebrospinal fluid (CSF) space in patients with idiopathic normal pressure hydrocephalus (iNPH) using voxel-based morphometry (VBM). We obtained sagittal volume images of the entire head by three-dimensional T1-weighted magnetic resonance imaging and compared the regional distribution of CSF in 12 patients with iNPH, 14 patients with Alzheimer's disease (AD), and 17 healthy individuals using VBM with automatically extracted CSF objects. VBM demonstrated significant widening at the lateral ventricles and Sylvian fissures and narrowing of the CSF space at the high convexity/midline areas in iNPH patients, compared to the AD patients and healthy controls (p<0.05, after correction with a false-discovery rate). In addition, the ratio of the CSF volume in the lateral ventricle/Sylvian fissure area to that in the high convexity/midline area in iNPH patients (3.9{+-}1.2) was remarkably greater than that in AD patients (1.2{+-}0.3) and controls (0.9{+-}0.3; one-way ANOVA, p <0.001; post hoc Tukey's test, p < 0.001); we could discriminate iNPH patients from those in the other two groups without any overlap, when using a cutoff level of 1.9. VBM using CSF objects can be used to delineate the characteristic alteration of the CSF space in iNPH patients, which has been evaluated by visual interpretation. (orig.)

  19. Meta-analysis of Pentacam vs. ultrasound pachymetry in central corneal thickness measurement in normal, post-LASIK or PRK, and keratoconic or keratoconus-suspect eyes.

    Science.gov (United States)

    Wu, Wenjing; Wang, Yan; Xu, Lulu

    2014-01-01

    The aim of this meta-analysis is to evaluate the central corneal thickness (CCT) measurement differences between Pentacam (Oculus Inc., Germany) and Ultrasound Pachymetry (USP) in normal (unoperated eyes , myopic and astigmatic eyes without corneal disease or topographic irregularity), after laser in situ keratomileusis (LASIK) or photorefractive keratectomy (PRK), and keratoconic or keratoconus suspected eyes. We assess whether Pentacam and USP have similar CCT differences in normal, thinner corneas after LASIK or PRK procedures, and kerotoconic or keratoconus suspected eyes. Data sources, including PubMed, Medline, EMBASE, and Cochrane Central Registry of Controlled Trials on the Cochrane Library, were searched to find the relevant studies. Primary outcome measures were CCT measurement between Pentacam and USP. Three groups of eyes were analyzed: normal; LASIK or PRK eyes; and keratoconus suspected or keratoconic eyes. Nineteen studies describing 1,908 eyes were enrolled in the normal group. Pentacam results were 1.47 μm ,95 % confidence interval (CI) -2.32 to 5.27, higher than USP without statistically significant difference (P = 0.45). Nine studies with total 539 eyes were included in the corneas after LASIK or PRK. The mean difference in the CCT measurement with Pentacam and ultrasound pachymetry was 1.03 μm, with the 95 % CI -3.36 to 5.42, there was no statistically difference (P = 0.64). Four studies with a total of 185 eyes were included in the keratoconic eyes or keratoconus-suspect group, however,the mean difference was -6.33 μm (95 % CI -9.17 to-3.49), which was statistically different between Pentacam and ultrasound pachymetry in the CCT measurement (P LASIK or PRK procedures. However, in keratoconic or keratoconus-suspect eyes, Pentacam slightly underestimates the central corneal thickness than does ultrasound pachymetry, which may result from the difficulty in fixation of keratoconic eyes, misalignment of Pentacam and the variation

  20. A case of idiopathic omental hemorrhage

    Directory of Open Access Journals (Sweden)

    Toshimitsu Hosotani

    2016-05-01

    Full Text Available With the exception of trauma, intraperitoneal hemorrhage in young women is caused by the high frequency of ectopic pregnancy and ovarian bleeding. Here, we describe a case of idiopathic omental hemorrhage, which is a rare cause of intraperitoneal hemorrhage. Intraperitoneal hemorrhage was suspected in a 38-year-old Japanese woman based on contrast-enhanced computed tomography. Her last menstrual period was 23 days prior, and ovarian bleeding was considered based on bloody ascites revealed by culdocentesis. She underwent emergency surgery for hypovolemic shock. Although both ovaries were of normal size and no abnormal findings were observed, we performed a partial omentectomy because multiple clots were attached only to the greater omentum. Postoperatively, no rebleeding occurred, and she was discharged 11 days after the surgery. Because she did not have a clear history of trauma and underlying disease, idiopathic omental hemorrhage was diagnosed.

  1. Idiopathic and normal lateral lumbar curves: muscle effects interpreted by 12th rib length asymmetry with pathomechanic implications for lumbar idiopathic scoliosis

    Directory of Open Access Journals (Sweden)

    Theodoros B. Grivas

    2016-10-01

    Full Text Available Abstract Background The historical view of scoliosis as a primary rotation deformity led to debate about the pathomechanic role of paravertebral muscles; particularly multifidus, thought by some to be scoliogenic, counteracting, uncertain, or unimportant. Here, we address lateral lumbar curves (LLC and suggest a pathomechanic role for quadrates lumborum, (QL in the light of a new finding, namely of 12th rib bilateral length asymmetry associated with idiopathic and small non-scoliosis LLC. Methods Group 1: The postero-anterior spinal radiographs of 14 children (girls 9, boys 5 aged 9–18, median age 13 years, with right lumbar idiopathic scoliosis (IS and right LLC less that 10°, were studied. The mean Cobb angle was 12° (range 5–22°. Group 2: In 28 children (girls 17, boys 11 with straight spines, postero-anterior spinal radiographs were evaluated similarly to the children with the LLC, aged 8–17, median age 13 years. The ratio of the right/left 12th rib lengths and it’s reliability was calculated. The difference of the ratio between the two groups was tested; and the correlation between the ratio and the Cobb angle estimated. Statistical analysis was done using the SPSS package. Results The ratio’s reliability study showed intra-observer +/−0,036 and the inter-observer error +/−0,042 respectively in terms of 95 % confidence limit of the error of measurements. The 12th rib was longer on the side of the curve convexity in 12 children with LLC and equal in two patients with lumbar scoliosis. The 12th rib ratios of the children with lumbar curve were statistically significantly greater than in those with straight spines. The correlation of the 12th rib ratio with Cobb angle was statistically significant. The 12th thoracic vertebrae show no axial rotation (or minimal in the LLC and no rotation in the straight spine group. Conclusions It is not possible, at present, to determine whether the 12th convex rib lengthening is

  2. Evaluation of lumbar tap test combined with mean cerebral blood flow measurement and electroencephalographic topography on idiopathic normal pressure hydrocephalus patients

    International Nuclear Information System (INIS)

    Takeuchi, Totaro; Iwasaki, Mitsuyoshi; Shirata, Kanji

    2004-01-01

    The purpose of this study was to improve the diagnostic precision of the lumbar tap test (LTT). Thirty one patients (mean age, 65.4 years; male to female ratio, 12: 19) suspected of having idiopathic normal pressure hydrocephalus (INPH) were used in the study. They underwent LTT (20-30 ml of cerebrospinal fluid was drained through a puncture with a 18 G needle; evaluation within 3 days after LTT). Based on changes in symptoms after LTT, including dementia (evaluated according to the duration of time needed to accomplish No. 7 items in the N method psychofunction test) and gait disturbance (evaluated according to the duration and the number of steps needed to walk 4 m in a straight line), mean cerebral blood flow (mCBF) monitored with 133Xe-CT, and electroencephalographic topography (EEG-T). The patients were divided into symptom-improved [LTT(+)] and no change [LTT(-)] groups. Changing patterns of EEG-T and mCBF after LTT and the efficacy of V-P shunt at 1 month after shunt administration (effective: E; non-effective: NE) were analyzed and compared. Changing patterns of EEG-T and mCBF after LTT were categorized as improvement of both parameters (type A), that of mCBF only (type B), that of EEG-T only (type C), and no change in either parameter (type D). Twenty patients were classified as LTT(+) and eleven as LTT(-). The false positive rate was 25% (5/20) for LTT(+); the false negative rate was 27.3% (3/11) for LTT(-). In all the patients, in relation to changing patterns of EEG-T and mCBF, 100% of type A patients (9/9), 75% of type B (6/8), 42.9% of type C (3/7), and 0% of type D (0/7) responded to shunting. Increased rates (IR) of mCBF in 17 patients with improvement of mCBF were 24.2±10.6% in E patients and 8.9±5.2% in NE patients, demonstrating a significantly higher percentage in E patients (p<0.005). The borderline of IR between E and NE was around 15%. Although quantitative evaluation of symptoms (dementia and gait disturbance) before and after LTT, 27

  3. Lipid composition of hepatic and adipose tissues from normal cats and from cats with idiopathic hepatic lipidosis.

    Science.gov (United States)

    Hall, J A; Barstad, L A; Connor, W E

    1997-01-01

    The purpose of this study was to characterize the lipid classes in hepatic and adipose tissues from cats with idiopathic hepatic lipidosis (IHL). Concentrations of triglyceride, phospholipid phosphorus, and free and total cholesterol were determined in lipid extracts of liver homogenates from 5 cats with IHL and 5 healthy control cats. Total fatty acid composition of liver and adipose tissue was also compared. Triglyceride accounted for 34% of liver by weight in cats with IHL (338 +/- 38 mg/g wet liver) versus 1% in control cats (9.9 +/- 1.0 mg/g wet liver, P hepatic tissue in the 2 groups differed; palmitate was higher (19.5 +/- 1.1% of total fatty acids in cats with IHL versus 9.2 +/- 2.7% in controls, P hepatic triglyceride in cats with IHL is the mobilization of fatty acids from adipose tissue.

  4. Asymmetrical trunk movement during walking improved to normal range at 3 months after corrective posterior spinal fusion in adolescent idiopathic scoliosis.

    Science.gov (United States)

    Wong-Chung, Daniel A C F; Schimmel, Janneke J P; de Kleuver, Marinus; Keijsers, Noël L W

    2018-02-01

    To investigate the effects of posterior spinal fusion (PSF) and curve type on upper body movements in Adolescent Idiopathic Scoliosis (AIS) patients during gait. Twenty-four girls (12-18 years) with AIS underwent PSF. 3D-Gait-analyses were performed preoperatively, at 3 months and 1 year postoperatively. Mean position (0° represents symmetry) and range of motion (ROM) of the trunk (thorax-relative-to-pelvis) in all planes were assessed. Lower body kinematics and spatiotemporal parameters were also evaluated. Mean trunk position improved from 7.0° to 2.9° in transversal plane and from 5.0° to - 0.8° in frontal plane at 3 months postoperative (p maintenance of normal gait can explain the rapid recovery and well functioning in daily life of AIS patients, despite undergoing a fusion of large parts of their spine.

  5. Measurement of blood flow in the superior sagittal sinus in healthy volunteers, and in patients with normal pressure hydrocephalus and idiopathic intracranial hypertension with phase-contrast cine MR imaging

    DEFF Research Database (Denmark)

    Gideon, P; Thomsen, C; Gjerris, F

    1996-01-01

    PURPOSE: To measure blood flow and velocity in the superior sagittal ++sinus. MATERIAL AND METHODS: MR velocity mapping was used to examine 14 healthy volunteers, 15 patients with normal pressure hydrocephalus (NPH), 3 patients with high pressure hydrocephalus (HPH), and 11 patients with idiopathic...

  6. MRI of the wrist in juvenile idiopathic arthritis: erosions or normal variants? A prospective case-control study

    International Nuclear Information System (INIS)

    Ording Muller, Lil-Sofie; Boavida, Peter; Avenarius, Derk; Eldevik, Odd Petter; Damasio, Beatrice; Malattia, Clara; Lambot-Juhan, Karen; Tanturri, Laura; Owens, Catherine M.; Rosendahl, Karen

    2013-01-01

    Bony depressions at the wrist resembling erosions are frequently seen on MRI in healthy children. The accuracy of MRI in detecting early bony destruction is therefore questionable. We compared findings on MRI of the wrist in healthy children and those with juvenile idiopathic arthritis (JIA) to investigate markers for true disease. We compared the number and localisation of bony depressions at the wrist in 85 healthy children and 68 children with JIA, ages 5-15 years. The size of the wrist was assessed from a radiograph of the wrist performed on the same day as the MRI. No significant difference in the number of bony depressions in the carpal bones was seen between healthy children and children with JIA at any age. Depressions are found in similar locations in the two groups, except for a few sites, where bony depressions were seen exclusively in the JIA group, particularly at the CMC joints. The wrist was significantly smaller in children with JIA (P < 0.001). Using adult scoring systems and standard MR sequences in the assessment of bone destruction in children may lead to overstaging or understaging of disease. At present, standard MRI sequences cannot easily be used for assessment of early signs of erosions in children. (orig.)

  7. MRI of the wrist in juvenile idiopathic arthritis: erosions or normal variants? A prospective case-control study

    Energy Technology Data Exchange (ETDEWEB)

    Ording Muller, Lil-Sofie [University Hospital North Norway, Department of Radiology, Tromsoe (Norway); Great Ormond Street Hospital for Children, Department of Radiology, London (United Kingdom); Boavida, Peter [Homerton University Hospital, Department of Radiology, London (United Kingdom); Avenarius, Derk; Eldevik, Odd Petter [University Hospital North Norway, Department of Radiology, Tromsoe (Norway); Damasio, Beatrice [Ospedale Pediatrico Gaslini, Department of Radiology, Genoa (Italy); Malattia, Clara [Ospedale Pediatrico Gaslini, Department of Rhematology, Genoa (Italy); Lambot-Juhan, Karen [Hopital Necker Enfants Malades, Department of Radiology, Paris (France); Tanturri, Laura [Ospedale Pediatrico Bambino Gesu, Department of Radiology, Rome (Italy); Owens, Catherine M. [Great Ormond Street Hospital for Children, Department of Radiology, London (United Kingdom); UCL, Institute of Child Health, London (United Kingdom); Rosendahl, Karen [Great Ormond Street Hospital for Children, Department of Radiology, London (United Kingdom); UCL, Institute of Child Health, London (United Kingdom); Haukeland University Hospital, Department of Radiology, Bergen (Norway); University of Bergen, Department of Surgical Sciences, Bergen (Norway)

    2013-07-15

    Bony depressions at the wrist resembling erosions are frequently seen on MRI in healthy children. The accuracy of MRI in detecting early bony destruction is therefore questionable. We compared findings on MRI of the wrist in healthy children and those with juvenile idiopathic arthritis (JIA) to investigate markers for true disease. We compared the number and localisation of bony depressions at the wrist in 85 healthy children and 68 children with JIA, ages 5-15 years. The size of the wrist was assessed from a radiograph of the wrist performed on the same day as the MRI. No significant difference in the number of bony depressions in the carpal bones was seen between healthy children and children with JIA at any age. Depressions are found in similar locations in the two groups, except for a few sites, where bony depressions were seen exclusively in the JIA group, particularly at the CMC joints. The wrist was significantly smaller in children with JIA (P < 0.001). Using adult scoring systems and standard MR sequences in the assessment of bone destruction in children may lead to overstaging or understaging of disease. At present, standard MRI sequences cannot easily be used for assessment of early signs of erosions in children. (orig.)

  8. Comparison of untreated adolescent idiopathic scoliosis with normal controls: a review and statistical analysis of the literature.

    Science.gov (United States)

    Rushton, Paul R P; Grevitt, Michael P

    2013-04-20

    Review and statistical analysis of studies evaluating health-related quality of life (HRQOL) in adolescents with untreated adolescent idiopathic scoliosis (AIS) using Scoliosis Research Society (SRS) outcomes. To apply normative values and minimum clinical important differences for the SRS-22r to the literature. Identify whether the HRQOL of adolescents with untreated AIS differs from unaffected peers and whether any differences are clinically relevant. The effect of untreated AIS on adolescent HRQOL is uncertain. The lack of published normative values and minimum clinical important difference for the SRS-22r has so far hindered our interpretation of previous studies. The publication of this background data allows these studies to be re-examined. Using suitable inclusion criteria, a literature search identified studies examining HRQOL in untreated adolescents with AIS. Each cohort was analyzed individually. Statistically significant differences were identified by using 95% confidence intervals for the difference in SRS-22r domain mean scores between the cohorts with AIS and the published data for unaffected adolescents. If the lower bound of the confidence interval was greater than the minimum clinical important difference, the difference was considered clinically significant. Of the 21 included patient cohorts, 81% reported statistically worse pain than those unaffected. Yet in only 5% of cohorts was this difference clinically important. Of the 11 cohorts included examining patient self-image, 91% reported statistically worse scores than those unaffected. In 73% of cohorts this difference was clinically significant. Affected cohorts tended to score well in function/activity and mental health domains and differences from those unaffected rarely reached clinically significant values. Pain and self-image tend to be statistically lower among cohorts with AIS than those unaffected. The literature to date suggests that it is only self-image which consistently differs

  9. Analysis of Morphological Characteristics and Origins of Idiopathic Premature Ventricular Contractions Under a 12-Lead Electrocardiogram in Children with Structurally Normal Hearts.

    Science.gov (United States)

    Jiang, Jianbin; He, Yuee; Qiu, Huixian; Zhang, Yuanhai; Chu, Maoping; Li, Yuechun; Chen, Qi

    2017-10-21

    Up to 40% of healthy children have premature ventricular complexes or contractions (PVCs) detected with 24-hour Holter monitoring. We aimed to investigate the morphological characteristics and origins of idiopathic PVCs under a 12-lead electrocardiogram in children with structurally normal hearts. All asymptomatic monomorphic PVC patients with structurally normal hearts under 18 years of age were included in this retrospective study. Characteristics of PVCs in lead V 1 under a 12-lead electrocardiogram were classified as left bundle branch block (PVC-LBBB) or right bundle branch block (PVC-RBBB). According to limb leads, PVC-LBBB or PVC-RBBB was divided into: PVCs-LBBB type I; PVCs-LBBB type II; PVCs-RBBB type I; PVCs-RBBB type II; and PVCs-RBBB type III. Out of 178 PVC patients, 94 cases of PVCs-LBBB (PVCs-LBBB type I = 60; PVCs-LBBB type II = 34) and 84 cases of PVCs-RBBB (PVCs-RBBB type I = 3; PVCs-RBBB type II = 55; PVCs-RBBB type III = 26) were identified. The frequency of PVCs-LBBB type I increased with age and the frequency of PVCs-RBBB type II and III decreased with age. Among the children monitor tested, from 1 years old to 18 years old, PVCs originating from the left or right ventricular outflow tract gradually increased with age, while PVCs originating from the branch sources decreased with age.

  10. Idiopathic anaphylaxis.

    Science.gov (United States)

    Fenny, Nana; Grammer, Leslie C

    2015-05-01

    Idiopathic anaphylaxis is a diagnosis of exclusion after other causes have been thoroughly evaluated and excluded. The pathogenesis of idiopathic anaphylaxis remains uncertain, although increased numbers of activated lymphocytes and circulating histamine-releasing factors have been implicated. Signs and symptoms of patients diagnosed with idiopathic anaphylaxis are indistinguishable from the manifestations of other forms of anaphylaxis. Treatment regimens are implemented based on the frequency and severity of patient symptoms and generally include the use of epinephrine autoinjectors, antihistamines, and steroids. The prognosis of idiopathic anaphylaxis is generally favorable with well-established treatment regimens and effective patient education. Copyright © 2015 Elsevier Inc. All rights reserved.

  11. Spooky Suspects

    Science.gov (United States)

    Pacifici, Lara

    2011-01-01

    This activity presents an option for covering biology content while engaging students in an investigation that highlights the spirit of Halloween. Students are engaged in the story line and have fun trying to solve the mystery kidnapping by using science skills to examine the evidence and eliminate some ghoulish suspects. (Contains 1 figure.)

  12. Investigation and clinical applications of muscle strength change in cerebrospinal fluid tap test in cases of idiopathic normal pressure hydrocephalus: A retrospective study.

    Science.gov (United States)

    Matsuoka, Tsuyoshi; Iida, Jun-Ichi; Kawahara, Makoto; Uchiyama, Yoshitomo

    2016-12-15

    The cerebrospinal fluid tap test (CSFTT) is widely used to diagnose idiopathic normal pressure hydrocephalus (iNPH) and predict the therapeutic effectiveness of shunting. However, the ability to walk cannot be quantified for patients who are unable to walk. Therefore, we examined whether the iNPH diagnostic aid is possible using dynamometry, even for patients who are unable to walk. In this study, 45 patients underwent grip strength assessment, quadriceps strength assessment, 10-m walk test, and 3-m Timed Up and Go test before and after CSFTT. Our investigation of physical functions indicated that the CSFTT-positive group demonstrated significant improvements in grip and bilateral quadriceps muscle strength. The results of the receiver operating characteristic analysis indicated that leg muscle strength measurement reliability was high and that the area under the curve was 0.754-0.811. Our investigation of the clinically effective cutoff point for the rate of change indicated that it was 13.6% for right quadriceps muscle strength and 15.3% for left quadriceps muscle strength. Comparing CSFTT results in cases of iNPH with the observed rate of change in muscle strength can aid in the diagnosis of iNPH. Copyright © 2016 Elsevier B.V. All rights reserved.

  13. Clinical significance of cerebrospinal fluid tap test and magnetic resonance imaging/computed tomography findings of tight high convexity in patients with possible idiopathic normal pressure hydrocephalus

    International Nuclear Information System (INIS)

    Ishikawa, Masatsune; Furuse, Motomasa; Nishida, Namiko; Oowaki, Hisayuki; Matsumoto, Atsuhito; Suzuki, Takayuki

    2010-01-01

    Idiopathic normal pressure hydrocephalus (iNPH) is a treatable syndrome with a classical triad of symptoms. The Japanese iNPH guidelines indicate that the cerebrospinal fluid (CSF) tap test and tight high-convexity on magnetic resonance (MR) imaging are important for the diagnosis. The relationships between the effectiveness of CSF shunt surgery in possible iNPH patients, the tap test result, and the MR imaging/computed tomography (CT) findings of tight high-convexity were evaluated in 88 possible iNPH patients (mean age 75 years) with one or more of the classical triad of symptoms, and mild to moderate ventricular dilation. All patients underwent the tap test in the outpatient clinic, and patients and caregivers assessed the clinical changes during one week. The tap test was positive in 47 patients and negative in 41 patients. Surgery was performed in 19 patients with positive tap test, and was effective in 17 patients. Although the findings were inconsistent in some patients, the result of the tap test was found to be highly correlated with the MR imaging/CT finding of tight high-convexity (p<0.0001), confirming that both these diagnostic tests are promising predictors of shunt effectiveness. (author)

  14. Narrow CSF space at high convexity and high midline areas in idiopathic normal pressure hydrocephalus detected by axial and coronal MRI

    Energy Technology Data Exchange (ETDEWEB)

    Sasaki, Makoto [Iwate Medical University, Department of Radiology, Morioka (Japan); Honda, Satoshi [St. Luke' s International Hospital, Department of Radiology, Tokyo (Japan); Yuasa, Tatsuhiko; Iwamura, Akihide [Kohnodai Hospital, National Center of Neurology and Psychiatry, Department of Neurology, Ichikawa (Japan); Shibata, Eri [Iwate Medical University, Department of Neuropsychiatry, Morioka (Japan); Ohba, Hideki [Iwate Medical University, Department of Neurology, Morioka (Japan)

    2008-02-15

    The aim of this study was to determine the performance of axial and coronal magnetic resonance imaging (MRI) in detecting the narrowing of the cerebrospinal fluid (CSF) space at the high convexity and high midline areas, which is speculated to be one of the clinical characteristics of idiopathic normal pressure hydrocephalus (iNPH). We retrospectively examined axial and coronal T1-weighted images of 14 iNPH patients and 12 age-matched controls. The narrowness of the CSF space at the high convexity/midline was blindly evaluated by five raters using a continuous confidence rating scale for receiver operating characteristic (ROC) analysis. Axial and coronal imaging accurately determined the presence of the narrow cisterns/sulci at the high convexity/midline and was capable of predicting probable/definite iNPH with a high degree of accuracy. there were also no significant differences in the detection of this finding between the axial and coronal images. Both axial and coronal T1-weighted MRI can detect the narrow CSF space at the high convexity/midline accurately and may therefore facilitate clinicians in choosing a management strategy for iNPH patients. (orig.)

  15. Narrow CSF space at high convexity and high midline areas in idiopathic normal pressure hydrocephalus detected by axial and coronal MRI

    International Nuclear Information System (INIS)

    Sasaki, Makoto; Honda, Satoshi; Yuasa, Tatsuhiko; Iwamura, Akihide; Shibata, Eri; Ohba, Hideki

    2008-01-01

    The aim of this study was to determine the performance of axial and coronal magnetic resonance imaging (MRI) in detecting the narrowing of the cerebrospinal fluid (CSF) space at the high convexity and high midline areas, which is speculated to be one of the clinical characteristics of idiopathic normal pressure hydrocephalus (iNPH). We retrospectively examined axial and coronal T1-weighted images of 14 iNPH patients and 12 age-matched controls. The narrowness of the CSF space at the high convexity/midline was blindly evaluated by five raters using a continuous confidence rating scale for receiver operating characteristic (ROC) analysis. Axial and coronal imaging accurately determined the presence of the narrow cisterns/sulci at the high convexity/midline and was capable of predicting probable/definite iNPH with a high degree of accuracy. there were also no significant differences in the detection of this finding between the axial and coronal images. Both axial and coronal T1-weighted MRI can detect the narrow CSF space at the high convexity/midline accurately and may therefore facilitate clinicians in choosing a management strategy for iNPH patients. (orig.)

  16. The Effect of Spinal Tap Test on Different Sensory Modalities of Postural Stability in Idiopathic Normal Pressure Hydrocephalus

    Directory of Open Access Journals (Sweden)

    Katrin Abram

    2016-09-01

    Full Text Available Background/Aims: Postural instability in patients with normal pressure hydrocephalus (NPH is a most crucial symptom leading to falls with secondary complications. The aim of the current study was to evaluate the therapeutic effect of spinal tap on postural stability in these patients. Methods: Seventeen patients with clinical symptoms of NPH were examined using gait scale, computerized dynamic posturography (CDP, and neuropsychological assessment. Examinations were done before and after spinal tap test. Results: The gait score showed a significant improvement 24 h after spinal tap test in all subtests and in the sum score (p Conclusion: Postural stability in NPH is predominantly affected by deficient vestibular functions, which did not improve after spinal tap test. Conditions which improved best were mainly independent from visual control and are based on proprioceptive functions.

  17. Idiopathic intracranial hypertension

    DEFF Research Database (Denmark)

    Yri, Hanne M; Jensen, Rigmor H

    2015-01-01

    AIMS: The aims of this article are to characterize the headache in idiopathic intracranial hypertension (IIH) and to field-test the ICHD diagnostic criteria for headache attributed to IIH. MATERIALS AND METHODS: We included 44 patients with new-onset IIH. Thirty-four patients with suspected but u...... tinnitus may suggest intracranial hypertension. Based on data from a well-defined IIH cohort, we propose a revision of the ICDH-3 beta diagnostic criteria with improved clinical applicability and increased sensitivity and specificity....

  18. The role of diffusion tensor imaging and fractional anisotropy in the evaluation of patients with idiopathic normal pressure hydrocephalus: a literature review.

    Science.gov (United States)

    Siasios, Ioannis; Kapsalaki, Eftychia Z; Fountas, Kostas N; Fotiadou, Aggeliki; Dorsch, Alexander; Vakharia, Kunal; Pollina, John; Dimopoulos, Vassilios

    2016-09-01

    OBJECTIVE Diffusion tensor imaging (DTI) for the assessment of fractional anisotropy (FA) and involving measurements of mean diffusivity (MD) and apparent diffusion coefficient (ADC) represents a novel, MRI-based, noninvasive technique that may delineate microstructural changes in cerebral white matter (WM). For example, DTI may be used for the diagnosis and differentiation of idiopathic normal pressure hydrocephalus (iNPH) from other neurodegenerative diseases with similar imaging findings and clinical symptoms and signs. The goal of the current study was to identify and analyze recently published series on the use of DTI as a diagnostic tool. Moreover, the authors also explored the utility of DTI in identifying patients with iNPH who could be managed by surgical intervention. METHODS The authors performed a literature search of the PubMed database by using any possible combinations of the following terms: "Alzheimer's disease," "brain," "cerebrospinal fluid," "CSF," "diffusion tensor imaging," "DTI," "hydrocephalus," "idiopathic," "magnetic resonance imaging," "normal pressure," "Parkinson's disease," and "shunting." Moreover, all reference lists from the retrieved articles were reviewed to identify any additional pertinent articles. RESULTS The literature search retrieved 19 studies in which DTI was used for the identification and differentiation of iNPH from other neurodegenerative diseases. The DTI protocols involved different approaches, such as region of interest (ROI) methods, tract-based spatial statistics, voxel-based analysis, and delta-ADC analysis. The most studied anatomical regions were the periventricular WM areas, such as the internal capsule (IC), the corticospinal tract (CST), and the corpus callosum (CC). Patients with iNPH had significantly higher MD in the periventricular WM areas of the CST and the CC than had healthy controls. In addition, FA and ADCs were significantly higher in the CST of iNPH patients than in any other patients with other

  19. Practice guideline: Idiopathic normal pressure hydrocephalus: Response to shunting and predictors of response: Report of the Guideline Development, Dissemination, and Implementation Subcommittee of the American Academy of Neurology.

    Science.gov (United States)

    Halperin, John J; Kurlan, Roger; Schwalb, Jason M; Cusimano, Michael D; Gronseth, Gary; Gloss, David

    2015-12-08

    We evaluated evidence for utility of shunting in idiopathic normal pressure hydrocephalus (iNPH) and for predictors of shunting effectiveness. We identified and classified relevant published studies according to 2004 and 2011 American Academy of Neurology methodology. Of 21 articles, we identified 3 Class I articles. Shunting is possibly effective in iNPH (96% chance subjective improvement, 83% chance improvement on timed walk test at 6 months) (3 Class III). Serious adverse event risk was 11% (1 Class III). Predictors of success included elevated Ro (1 Class I, multiple Class II), impaired cerebral blood flow reactivity to acetazolamide (by SPECT) (1 Class I), and positive response to either external lumbar drainage (1 Class III) or repeated lumbar punctures. Age may not be a prognostic factor (1 Class II). Data are insufficient to judge efficacy of radionuclide cisternography or aqueductal flow measurement by MRI. Clinicians may choose to offer shunting for subjective iNPH symptoms and gait (Level C). Because of significant adverse event risk, risks and benefits should be carefully weighed (Level B). Clinicians should inform patients with iNPH with elevated Ro and their families that they have an increased chance of responding to shunting compared with those without such elevation (Level B). Clinicians may counsel patients with iNPH and their families that (1) positive response to external lumbar drainage or to repeated lumbar punctures increases the chance of response to shunting, and (2) increasing age does not decrease the chance of shunting being successful (both Level C). © 2015 American Academy of Neurology.

  20. Influence of comorbidities in idiopathic normal pressure hydrocephalus — research and clinical care. A report of the ISHCSF task force on comorbidities in INPH

    Science.gov (United States)

    2013-01-01

    Idiopathic normal pressure hydrocephalus (INPH) is a syndrome of ventriculomegaly, gait impairment, cognitive decline and incontinence that occurs in an elderly population prone to many types of comorbidities. Identification of the comorbidities is thus an important part of the clinical management of INPH patients. In 2011, a task force was appointed by the International Society for Hydrocephalus and Cerebrospinal Fluid Disorders (ISHCSF) with the objective to compile an evidence-based expert analysis of what we know and what we need to know regarding comorbidities in INPH. This article is the final report of the task force. The expert panel conducted a comprehensive review of the literature. After weighing the evidence, the various proposals were discussed and the final document was approved by all the task force members and represents a consensus of expert opinions. Recommendations regarding the following topics are given: I. Musculoskeletal conditions; II. Urinary problems; III. Vascular disease including risk factors, Binswanger disease, and white matter hyperintensities; IV. Mild cognitive impairment and Alzheimer disease including biopsies; V. Other dementias (frontotemporal dementia, Lewy body, Parkinson); VI. Psychiatric and behavioral disorders; VII. Brain imaging; VIII. How to investigate and quantify. The task force concluded that comorbidity can be an important predictor of prognosis and post-operative outcome in INPH. Reported differences in outcomes among various INPH cohorts may be partly explained by variation in the rate and types of comorbidities at different hydrocephalus centers. Identification of comorbidities should thus be a central part of the clinical management of INPH where a detailed history, physical examination, and targeted investigations are the basis for diagnosis and grading. Future INPH research should focus on the contribution of comorbidity to overall morbidity, mortality and long-term outcomes. PMID:23758953

  1. Upper midbrain profile sign and cingulate sulcus sign. MRI findings on sagittal images in idiopathic normal-pressure hydrocephalus, Alzheimer's disease, and progressive supranuclear palsy

    International Nuclear Information System (INIS)

    Adachi, Michito; Ohshima, Fumi; Kawanami, Toru; Kato, Takeo

    2006-01-01

    On magnetic resonance imaging (MRI) sagittal sections, we sometimes encounter abnormal aspects of the superior profile of the midbrain and the cingulate sulcus in patients with dementia. In this preliminary study, we refer to these findings as the ''upper midbrain profile sign'' and the cingulate sulcus sign.'' We prospectively evaluated the usefulness of these signs for the diagnosis of idiopathic normal-pressure hydrocephalus (iNPH), Alzheimer's disease (AD) and progressive supranuclear palsy (PSP). We evaluated the upper midbrain profile sign and the cingulate sulcus sign on MRI sagittal images obtained from 21 people with headaches but no neurological deficit (controls), 10 iNPH patients, 11 AD patients, and 5 PSP patients. The upper midbrain profile sign indicated a concave shape to the superior profile of the midbrain on mid-sagittal images, and the cingulate sulcus sign indicated a narrow, tight aspect of the posterior part of the cingulate sulcus on paramedian-sagittal images. These signs were never seen in any images from the controls. The upper midbrain profile sign was seen in 7 of 10 patients with iNPH, 5 of 11 with AD, and 3 of 5 with PSP. The cingulate sulcus sign was seen in all 10 patients with iNPH but was never seen in any patient with AD or PSP. The upper midbrain profile sign could support a diagnosis of PSP but cannot discriminate among iNPH, AD, and PSP. In contrast, the cingulate sulcus sign has a very high sensitivity for iNPH and should facilitate the distinction of iNPH from other dementias. In the clinical setting, it is momentous to evaluate these signs easily by one simple MRI sequence. (author)

  2. Idiopathic granulomatous mastitis

    International Nuclear Information System (INIS)

    Ozturk, E.; Akin, M.; Can, Mehmet F.; Ozrehan, I.; Yagci, G.; Tufan, T.; Kurt, B.

    2009-01-01

    Objective was to discuss the clinical and radiological features and treatment approaches in 14 patients diagnosed with idiopathic granulomatous mastitis (GM). We retrospectively evaluated the clinical features, radiological findings and treatment approaches in 14 patients with idiopathic GM in the General Surgery Department, Gulhane School of Medicine, Ankara, Turkey between April 2000 and June 2006. The mean age of the patients was 34.5 years (range 27-41 years). The complaints at admission were a mass in the breast in 7 (50%) patients, an abscess and a mass in 6 (42.8%) and a skin fisculain one (7.2%). Granulomatous mastitis was unilateral in all subjects (on the right in 5 patients and on the left in 9). All of the patients underwent ultrasonographic evaluation. Mammography was performed in 8 and magnetic resonance imaging in 5 patients. Seven patients (50%) were suspected to have breast carcinoma according radiological findings. We performed the large excision in 11, incisional biopsy plus abscess drainage in one, and incisional biopsy plus abscess drainage plus medical treatment (prednisolone, methotrexate) in 2 patients. Due to the development of abscess after 9 months, drainage and large excision were performed in one patient who received medical treatment. Idiopathic GM is a disease that generally affects young women of reproductive age and may be mistaken for breast carcinoma in clinical and radiological evaluations. The gold standard for the diagnosis is histopathologic evaluation. (author)

  3. Juvenile Idiopathic Arthritis

    Directory of Open Access Journals (Sweden)

    Kenan Barut

    2017-04-01

    Full Text Available Juvenile idiopathic arthritis is the most common chronic rheumatic disease of unknown aetiology in childhood and predominantly presents with peripheral arthritis. The disease is divided into several subgroups, according to demographic characteristics, clinical features, treatment modalities and disease prognosis. Systemic juvenile idiopathic arthritis, which is one of the most frequent disease subtypes, is characterized by recurrent fever and rash. Oligoarticular juvenile idiopathic arthritis, common among young female patients, is usually accompanied by anti-nuclear antibodie positivity and anterior uveitis. Seropositive polyarticular juvenile idiopathic arthritis, an analogue of adult rheumatoid arthritis, is seen in less than 10% of paediatric patients. Seronegative polyarticular juvenile idiopathic arthritis, an entity more specific for childhood, appears with widespread large- and small-joint involvement. Enthesitis-related arthritis is a separate disease subtype, characterized by enthesitis and asymmetric lower-extremity arthritis. This disease subtype represents the childhood form of adult spondyloarthropathies, with human leukocyte antigen-B27 positivity and uveitis but commonly without axial skeleton involvement. Juvenile psoriatic arthritis is characterized by a psoriatic rash, accompanied by arthritis, nail pitting and dactylitis. Disease complications can vary from growth retardation and osteoporosis secondary to treatment and disease activity, to life-threatening macrophage activation syndrome with multi-organ insufficiency. With the advent of new therapeutics over the past 15 years, there has been a marked improvement in juvenile idiopathic arthritis treatment and long-term outcome, without any sequelae. The treatment of juvenile idiopathic arthritis patients involves teamwork, including an experienced paediatric rheumatologist, an ophthalmologist, an orthopaedist, a paediatric psychiatrist and a physiotherapist. The primary goals

  4. Hyperdynamic CSF motion profiles found in idiopathic normal pressure hydrocephalus and Alzheimer's disease assessed by fluid mechanics derived from magnetic resonance images.

    Science.gov (United States)

    Takizawa, Ken; Matsumae, Mitsunori; Hayashi, Naokazu; Hirayama, Akihiro; Yatsushiro, Satoshi; Kuroda, Kagayaki

    2017-10-18

    Magnetic resonance imaging (MRI) does not only ascertain morphological features, but also measures physiological properties such as fluid velocity or pressure gradient. The purpose of this study was to investigate cerebrospinal fluid (CSF) dynamics in patients with morphological abnormalities such as enlarged brain ventricles and subarachnoid spaces. We used a time-resolved three dimensional phase contrast (3D-PC) MRI technique to quantitatively evaluate CSF dynamics in the Sylvian aqueduct of healthy elderly individuals and patients with either idiopathic normal pressure hydrocephalus (iNPH) or Alzheimer's disease (AD) presenting with ventricular enlargement. Nineteen healthy elderly individuals, ten iNPH patients, and seven AD patients (all subjects ≥ 60 years old) were retrospectively evaluated 3D-PC MRI. The CSF velocity, pressure gradient, and rotation in the Sylvian aqueduct were quantified and compared between the three groups using Kolmogorov-Smirnov and Mann-Whitney U tests. There was no statistically significant difference in velocity among the three groups. The pressure gradient was not significantly different between the iNPH and AD groups, but was significantly different between the iNPH group and the healthy controls (p < 0.001), and similarly, between the AD group and the healthy controls (p < 0.001). Rotation was not significantly different between the iNPH and AD groups, but was significantly different between the iNPH group and healthy controls (p < 0.001), and similarly, between the AD group and the healthy controls (p < 0.001). Quantitative analysis of CSF dynamics with time resolved 3D-PC MRI revealed differences and similarities in the Sylvian aqueduct between healthy elderly individuals, iNPH patients, and AD patients. The results showed that CSF motion is in a hyperdynamic state in both iNPH and AD patient groups compared to healthy elderly individuals, and that iNPH patients and AD patients display similar CSF motion profiles.

  5. Normal Lung Quantification in Usual Interstitial Pneumonia Pattern: The Impact of Threshold-based Volumetric CT Analysis for the Staging of Idiopathic Pulmonary Fibrosis.

    Directory of Open Access Journals (Sweden)

    Hirotsugu Ohkubo

    Full Text Available Although several computer-aided computed tomography (CT analysis methods have been reported to objectively assess the disease severity and progression of idiopathic pulmonary fibrosis (IPF, it is unclear which method is most practical. A universal severity classification system has not yet been adopted for IPF.The purpose of this study was to test the correlation between quantitative-CT indices and lung physiology variables and to determine the ability of such indices to predict disease severity in IPF.A total of 27 IPF patients showing radiological UIP pattern on high-resolution (HR CT were retrospectively enrolled. Staging of IPF was performed according to two classification systems: the Japanese and GAP (gender, age, and physiology staging systems. CT images were assessed using a commercially available CT imaging analysis workstation, and the whole-lung mean CT value (MCT, the normally attenuated lung volume as defined from -950 HU to -701 Hounsfield unit (NL, the volume of the whole lung (WL, and the percentage of NL to WL (NL%, were calculated.CT indices (MCT, WL, and NL closely correlated with lung physiology variables. Among them, NL strongly correlated with forced vital capacity (FVC (r = 0.92, P <0.0001. NL% showed a large area under the receiver operating characteristic curve for detecting patients in the moderate or advanced stages of IPF. Multivariable logistic regression analyses showed that NL% is significantly more useful than the percentages of predicted FVC and predicted diffusing capacity of the lungs for carbon monoxide (Japanese stage II/III/IV [odds ratio, 0.73; 95% confidence intervals (CI, 0.48 to 0.92; P < 0.01]; III/IV [odds ratio. 0.80; 95% CI 0.59 to 0.96; P < 0.01]; GAP stage II/III [odds ratio, 0.79; 95% CI, 0.56 to 0.97; P < 0.05].The measurement of NL% by threshold-based volumetric CT analysis may help improve IPF staging.

  6. Normal Lung Quantification in Usual Interstitial Pneumonia Pattern: The Impact of Threshold-based Volumetric CT Analysis for the Staging of Idiopathic Pulmonary Fibrosis.

    Science.gov (United States)

    Ohkubo, Hirotsugu; Kanemitsu, Yoshihiro; Uemura, Takehiro; Takakuwa, Osamu; Takemura, Masaya; Maeno, Ken; Ito, Yutaka; Oguri, Tetsuya; Kazawa, Nobutaka; Mikami, Ryuji; Niimi, Akio

    2016-01-01

    Although several computer-aided computed tomography (CT) analysis methods have been reported to objectively assess the disease severity and progression of idiopathic pulmonary fibrosis (IPF), it is unclear which method is most practical. A universal severity classification system has not yet been adopted for IPF. The purpose of this study was to test the correlation between quantitative-CT indices and lung physiology variables and to determine the ability of such indices to predict disease severity in IPF. A total of 27 IPF patients showing radiological UIP pattern on high-resolution (HR) CT were retrospectively enrolled. Staging of IPF was performed according to two classification systems: the Japanese and GAP (gender, age, and physiology) staging systems. CT images were assessed using a commercially available CT imaging analysis workstation, and the whole-lung mean CT value (MCT), the normally attenuated lung volume as defined from -950 HU to -701 Hounsfield unit (NL), the volume of the whole lung (WL), and the percentage of NL to WL (NL%), were calculated. CT indices (MCT, WL, and NL) closely correlated with lung physiology variables. Among them, NL strongly correlated with forced vital capacity (FVC) (r = 0.92, P <0.0001). NL% showed a large area under the receiver operating characteristic curve for detecting patients in the moderate or advanced stages of IPF. Multivariable logistic regression analyses showed that NL% is significantly more useful than the percentages of predicted FVC and predicted diffusing capacity of the lungs for carbon monoxide (Japanese stage II/III/IV [odds ratio, 0.73; 95% confidence intervals (CI), 0.48 to 0.92; P < 0.01]; III/IV [odds ratio. 0.80; 95% CI 0.59 to 0.96; P < 0.01]; GAP stage II/III [odds ratio, 0.79; 95% CI, 0.56 to 0.97; P < 0.05]). The measurement of NL% by threshold-based volumetric CT analysis may help improve IPF staging.

  7. Idiopathic short stature

    Directory of Open Access Journals (Sweden)

    Vlaški Jovan

    2013-01-01

    Full Text Available Growth is a complex process and the basic characteristic of child- hood growth monitoring provides insight into the physiological and pathological events in the body. Statistically, the short stature means departure from the values of height for age and sex (in a particular environment, which is below -2 standard deviation score, or less than -2 standard deviation, i.e. below the third percentile. Advances in molecular genetics have contributed to the improvement of diagnostics in endocrinology. Analysis of patients’ genotypes should not be performed before taking a classical history, detailed clinical examination and appropriate tests. In patients with idiopathic short stature specific causes are excluded, such as growth hormone deficiency, Turner syndrome, short stature due to low birth weight, intrauterine growth retardation, small for gestational age, dysmorphology syndromes and chronic childhood diseases. The exclusion of abovementioned conditions leaves a large number of children with short stature whose etiology includes patients with genetic short stature or familial short stature and those who are low in relation to genetic potential, and who could also have some unrecognized endocrine defect. Idiopathic short stature represents a short stature of unknown cause of heterogeneous etiology, and is characterized by a normal response of growth hormone during stimulation tests (>10 ng/ml or 20 mJ/l, without other disorders, of normal body mass and length at birth. In idiopathic short stature standard deviation score rates <-2.25 (-2 to -3 or <1.2 percentile. These are also criteria for the initiation of growth hormone therapy. In children with short stature there is also the presence of psychological and social suffering. Goals of treatment with growth hormone involve achieving normal height and normal growth rate during childhood.

  8. Presentation of suspected pediatric uveitis.

    Science.gov (United States)

    Saeed, Muhammad Usman; Raza, Syed Hamid; Goyal, Sudeshna; Cleary, Gavin; Newman, William David; Chandna, Arvind

    2014-01-01

    Presentation of suspected pediatric uveitis: Pediatric uveitis is usually managed in specialized ophthalmic centers in the UK. Meaningful data acquisition in these clinics may be helpful in clinical governance, and healthcare planning in a specialty that is gradually changing due to changes in treatment choices. Retrospective analysis of prospectively acquired data in the Liverpool pediatric uveitis database was performed. Analysis of our data, based on 147 patients, with a mean age of 10 years, indicated a female to male ratio of 2:1. 99% of patients were Caucasian. Our data indicates 86% of all patients attending the uveitis clinic were diagnosed with juvenile idiopathic arthritis, followed by intermediate uveitis 5% and idiopathic uveitis 4%. 46% of patients required treatment. Systemic treatment included methotrexate (34%), prednisolone (14%), etanercept (6%), ciclosporin (6%), mycophenolate (3%), and infliximab (1%). Severe visual loss (defined by counting fingers or below vision) was seen in 10 eyes despite appropriately treated chronic uveitis. Our data shows uveitis-related ocular morbidity in a predominantly pediatric Caucasian population. Patients with severe and chronic uveitis may experience significant uveitis-related complications and subsequent visual loss despite aggressive treatment.

  9. Genetics Home Reference: juvenile idiopathic arthritis

    Science.gov (United States)

    ... disease to fight microbial invaders and facilitate tissue repair. Normally, the body stops the inflammatory response after healing is complete to prevent damage to its own cells and tissues. In people with juvenile idiopathic arthritis , the inflammatory ...

  10. Idiopathic Scoliosis

    Directory of Open Access Journals (Sweden)

    Jens Ivar Brox

    2014-07-01

    Full Text Available Idiopathic scoliosis (IS is a lifetime condition and is defined as a structural, lateral rotated curvature of the spine of >10° on standing coronal plane radiographs. It should be distinguished from other causes of scoliosis. It can be classified as infantile, juvenile, and adolescent according to age. As a rule of thumb, about 80% of all curves are idiopathic, right convex thoracic, and present in otherwise healthy girls at the beginning of puberty. A family member most commonly detects scoliosis. The structural asymmetry of the spine is best observed by asking the patient to bend forward. IS is often seen in more than one member of a family, but the aetiology remains unknown. Multiple genes are likely to be involved with incomplete penetrance and variable expressivity. Early detection by screening allows for monitoring curve progression and timely initiation of bracing, but school screening is controversial and practises vary worldwide. Most patients have minor scoliosis and treatment is generally not recommended for patients with curves 45°. Scoliosis surgery was not successful until the introduction of Harrington’s instrumentation in the 1960s. Modern instrumentation has evolved from the Cotrel-Dubousset system in the 1980s, and a variety of methods are available today. Although scoliosis may be a burden, long-term studies suggest that a good quality of life is maintained in most patients.

  11. Idiopathic Bilateral Bloody Tearing

    Directory of Open Access Journals (Sweden)

    Emrullah Beyazyıldız

    2015-01-01

    Full Text Available Bloody tear is a rare and distinct clinic phenomenon. We report a case presenting with the complaint of recurrent episodes of bilateral bloody tearing. A 16-year-old girl presented to our clinic with complaint of bloody tearing in both eyes for 3 months. Bloody tearing was not associated with her menses. A blood-stained discharge from the punctum was not observed during the compression of both nasolacrimal ducts. Nasolacrimal passage was not obstructed. Imaging studies such as dacryocystography and gradient-echo magnetic resonance imaging (MRI of nasolacrimal canal were normal. Intranasal endoscopic evaluation was normal. We collected samples from bloody tears two times and pathological examination was performed. Pathological analysis showed lots of squamous cells and no endometrial cells; dysplastic cells were found. Further evaluations for underlying causes were unremarkable. No abnormalities were found in ophthalmologic, radiologic, and pathologic investigations. This condition is likely a rare abnormality and the least recognized aetiology for the idiopathic phenomenon.

  12. Idiopathic hirsutism: excessive bodily and facial hair in women.

    Science.gov (United States)

    Elghblawi, Ebtisam

    Hirsutism is the excessive and increased bodily and facial hair growth in women in locations where hair is normally minimal or absent. It refers to the growth of hair in a pattern normally occurring only in men, and therefore primarily raises psychological, cosmetic and social concerns. Idiopathic hirsutism (IH), where the cause of excessive hair growth is unknown, is considered to be the most common form of hirsutism. It is suspected that this type of hirsutism may be familial, as there is often a family history of the condition. Women with IH will generally have normal menses and normal levels of testosterone. There are many treatment modalities that fall into two broad groups: medical and mechanical treatment. An example of a medical treatment is when an agent is used, which interferes with the synthesis of androgen at the ovarian or adrenal level, or by inhibiting the effect of androgen at the receptor level. An example of a mechanical treatment is laser hair removal, where the hair follicle is destroyed; however, much depends on the on the skill of the treating practitioner, laser type, laser spot size, skin type, hair colour, and the stage at which the hair follicles were during their hair growth cycle, and the delivered wavelength. Laser offers the fastest method of hair loss. Other mechanical treatments include electrolysis, depilatory creams, plucking and waxing. This article presents a general overview of IH, including a definition, diagnostic measures, clinical manifestations, normal and abnormal physiology, and treatment options.

  13. Adolescent idiopathic scoliosis (AIS, environment, exposome and epigenetics: a molecular perspective of postnatal normal spinal growth and the etiopathogenesis of AIS with consideration of a network approach and possible implications for medical therapy

    Directory of Open Access Journals (Sweden)

    Burwell R Geoffrey

    2011-12-01

    Full Text Available Abstract Genetic factors are believed to play an important role in the etiology of adolescent idiopathic scoliosis (AIS. Discordant findings for monozygotic (MZ twins with AIS show that environmental factors including different intrauterine environments are important in etiology, but what these environmental factors may be is unknown. Recent evidence for common chronic non-communicable diseases suggests epigenetic differences may underlie MZ twin discordance, and be the link between environmental factors and phenotypic differences. DNA methylation is one important epigenetic mechanism operating at the interface between genome and environment to regulate phenotypic plasticity with a complex regulation across the genome during the first decade of life. The word exposome refers to the totality of environmental exposures from conception onwards, comprising factors in external and internal environments. The word exposome is used here also in relation to physiologic and etiopathogenetic factors that affect normal spinal growth and may induce the deformity of AIS. In normal postnatal spinal growth we propose a new term and concept, physiologic growth-plate exposome for the normal processes particularly of the internal environments that may have epigenetic effects on growth plates of vertebrae. In AIS, we propose a new term and concept pathophysiologic scoliogenic exposome for the abnormal processes in molecular pathways particularly of the internal environment currently expressed as etiopathogenetic hypotheses; these are suggested to have deforming effects on the growth plates of vertebrae at cell, tissue, structure and/or organ levels that are considered to be epigenetic. New research is required for chromatin modifications including DNA methylation in AIS subjects and vertebral growth plates excised at surgery. In addition, consideration is needed for a possible network approach to etiopathogenesis by constructing AIS diseasomes. These approaches may

  14. Normal pressure hydrocephalus

    Science.gov (United States)

    Hydrocephalus - occult; Hydrocephalus - idiopathic; Hydrocephalus - adult; Hydrocephalus - communicating; Dementia - hydrocephalus; NPH ... Ferri FF. Normal pressure hydrocephalus. In: Ferri FF, ed. ... Elsevier; 2016:chap 648. Rosenberg GA. Brain edema and disorders ...

  15. Idiopathic portal hypertension

    International Nuclear Information System (INIS)

    Han, Tae Kyun; Ryu, Dae Sik; Kim, Heung Chul; Hur, Hun; Eom, Kyeung Tae; Namkung, Sook; Park, Man Soo; Hwang, Woo Chul; Lee, Kwan Seop

    1996-01-01

    To describe the radiologic findings of idiopathic portal hypertension and to find the points of differentiation between idiopathic portal hypertension and liver cirrhosis. Four portograms in five patients who for four years had suffered from pathologically confirmed idiopathic portal hypertension were retrospectively analyzed and compared with a portogram obtained from a control subject with liver cirrhosis. Portographic finding s of idiopathic portal hypertension were paucity of medium-sized portal branches, irregular and obtuse-angled division of peripheral branches, abrupt interruption and an avascular area beneath the liver margin. A portogram of idiopathic portal hypertension may be useful in differentiation this and liver cirrhosis

  16. Idiopathic scoliosis; a biomechanical and functional anatomical study.

    NARCIS (Netherlands)

    Veldhuizen, Albert Gerrit

    1985-01-01

    Although many types of causes of scoliosis are known, the idiopathic variety comprises the largest group and as its name indicates, its a etiology is unknown. Idiopathics coliosis develops in a previously normal vertebral column and has been recognized as an entity for more than a century.

  17. Is there a correlation between operative results and change in ventricular volume after shunt placement? A study of 60 cases of idiopathic normal-pressure hydrocephalus

    Energy Technology Data Exchange (ETDEWEB)

    Meier, U.; Graewe, A.; Hajdukova, A. [Department of Neurosurgery, Unfallkrankenhaus Berlin, Warener Strasse 7, 12683, Berlin (Germany); Paris, S.; Stockheim, D.; Mutze, S. [Department of Radiology, Unfallkrankenhaus Berlin, Warener Strasse 7, 12683, Berlin (Germany)

    2003-06-01

    In patients with communicating or normal-pressure hydrocephalus, ventricular volume decreases following implantation of differential pressure valves. We implanted hydrostatic (Miethke dual-switch) valves in 60 patients with normal-pressure hydrocephalus (NPH) between September 1997 and December 2001. The patients underwent CT 1 year after operation, and we measured the Evans index. Although 83% of the patients showed no change in ventricular volume as assessed by this index, 72% nevertheless showed good to excellent and 16% satisfactory clinical improvement, while 12% showed no improvement. Moderate or marked reduction in ventricular size was observed in 17%, of whom 40% of these patients showed good to excellent and 20% satisfactory clinical improvement; 40% showed unsatisfactory improvement. The favourable outcome following implantation of a hydrostatic shunt thus did not correlate with decreased ventricular volume 1 year after operation, better outcomes being observed in patients with little or no alteration in ventricular size than in those with a marked decrease. Postoperative change in ventricular volume in NPH thus does not have the same significance as in patients with high-pressure hydrocephalus. (orig.)

  18. Is there a correlation between operative results and change in ventricular volume after shunt placement? A study of 60 cases of idiopathic normal-pressure hydrocephalus

    International Nuclear Information System (INIS)

    Meier, U.; Graewe, A.; Hajdukova, A.; Paris, S.; Stockheim, D.; Mutze, S.

    2003-01-01

    In patients with communicating or normal-pressure hydrocephalus, ventricular volume decreases following implantation of differential pressure valves. We implanted hydrostatic (Miethke dual-switch) valves in 60 patients with normal-pressure hydrocephalus (NPH) between September 1997 and December 2001. The patients underwent CT 1 year after operation, and we measured the Evans index. Although 83% of the patients showed no change in ventricular volume as assessed by this index, 72% nevertheless showed good to excellent and 16% satisfactory clinical improvement, while 12% showed no improvement. Moderate or marked reduction in ventricular size was observed in 17%, of whom 40% of these patients showed good to excellent and 20% satisfactory clinical improvement; 40% showed unsatisfactory improvement. The favourable outcome following implantation of a hydrostatic shunt thus did not correlate with decreased ventricular volume 1 year after operation, better outcomes being observed in patients with little or no alteration in ventricular size than in those with a marked decrease. Postoperative change in ventricular volume in NPH thus does not have the same significance as in patients with high-pressure hydrocephalus. (orig.)

  19. Early evaluation of cerebral metabolic rate of glucose (CMRglu) with {sup 18}F-FDG PET/CT and clinical assessment in idiopathic normal pressure hydrocephalus (INPH) patients before and after ventricular shunt placement: preliminary experience

    Energy Technology Data Exchange (ETDEWEB)

    Calcagni, Maria Lucia; Lavalle, Mariadea; Leccisotti, Lucia; Giordano, Alessandro [Universita Cattolica del Sacro Cuore, Institute of Nuclear Medicine, Rome (Italy); Mangiola, Annunziato; De Bonis, Pasquale; Anile, Carmelo [Universita Cattolica del Sacro Cuore, Institute of Neurosurgery, Rome (Italy); Indovina, Luca [Universita Cattolica del Sacro Cuore, Institute of Physics, Rome (Italy); Marra, Camillo [Universita Cattolica del Sacro Cuore, Institute of Neurology, Rome (Italy); Pelliccioni, Armando [Istituto Nazionale per l' Assicurazione contro gli Infortuni sul Lavoro (INAIL), Rome (Italy)

    2012-02-15

    We evaluated the relationships between the cerebral metabolic rate of glucose (CMRglu) measured by dynamic {sup 18}F-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) and the clinical and neuropsychological assessment before and after the surgical procedure in idiopathic normal pressure hydrocephalus (INPH) patients. Eleven selected INPH patients underwent clinical assessment (modified Rankin scale, Krauss scale, Larsson categorization system and Stein-Langfitt scale), cognitive evaluation (Mini-Mental State Examination, MMSE) and dynamic {sup 18}F-FDG PET/CT scan 3 days before and 1 week after ventricular shunt placement. After shunting, the global CMRglu significantly increased (2.95 {+-} 0.44 vs 4.38 {+-} 0.68, p = 10{sup -7}) in all INPH patients with a mean percentage value of 48.7%. After shunting, no significant change was found in the Evans ratio whereas a significant decrease in all clinical scale scores was observed. Only a slight reduction in the MMSE was found. After shunting, a significant correlation between the global CMRglu value and clinical assessment was found (R {sup 2} = 0.75, p = 0.024); indeed all clinical scale scores varied (decreasing) and the CMRglu value also varied (increasing) in all INPH patients. Our preliminary data show that changes in the CMRglu are promptly reversible after surgery and that there is a relationship between the early metabolic changes and clinical symptoms, independently from the simultaneous changes in the ventricular size. The remarkable and prompt improvement in the global CMRglu and in symptoms may also have important implications for the current concept of ''neuronal plasticity'' and for the cells' reactivity in order to recover their metabolic function. (orig.)

  20. Early evaluation of cerebral metabolic rate of glucose (CMRglu) with 18F-FDG PET/CT and clinical assessment in idiopathic normal pressure hydrocephalus (INPH) patients before and after ventricular shunt placement: preliminary experience

    International Nuclear Information System (INIS)

    Calcagni, Maria Lucia; Lavalle, Mariadea; Leccisotti, Lucia; Giordano, Alessandro; Mangiola, Annunziato; De Bonis, Pasquale; Anile, Carmelo; Indovina, Luca; Marra, Camillo; Pelliccioni, Armando

    2012-01-01

    We evaluated the relationships between the cerebral metabolic rate of glucose (CMRglu) measured by dynamic 18 F-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) and the clinical and neuropsychological assessment before and after the surgical procedure in idiopathic normal pressure hydrocephalus (INPH) patients. Eleven selected INPH patients underwent clinical assessment (modified Rankin scale, Krauss scale, Larsson categorization system and Stein-Langfitt scale), cognitive evaluation (Mini-Mental State Examination, MMSE) and dynamic 18 F-FDG PET/CT scan 3 days before and 1 week after ventricular shunt placement. After shunting, the global CMRglu significantly increased (2.95 ± 0.44 vs 4.38 ± 0.68, p = 10 -7 ) in all INPH patients with a mean percentage value of 48.7%. After shunting, no significant change was found in the Evans ratio whereas a significant decrease in all clinical scale scores was observed. Only a slight reduction in the MMSE was found. After shunting, a significant correlation between the global CMRglu value and clinical assessment was found (R 2 = 0.75, p = 0.024); indeed all clinical scale scores varied (decreasing) and the CMRglu value also varied (increasing) in all INPH patients. Our preliminary data show that changes in the CMRglu are promptly reversible after surgery and that there is a relationship between the early metabolic changes and clinical symptoms, independently from the simultaneous changes in the ventricular size. The remarkable and prompt improvement in the global CMRglu and in symptoms may also have important implications for the current concept of ''neuronal plasticity'' and for the cells' reactivity in order to recover their metabolic function. (orig.)

  1. Idiopathic noncirrhotic portal hypertension: current perspectives.

    Science.gov (United States)

    Riggio, Oliviero; Gioia, Stefania; Pentassuglio, Ilaria; Nicoletti, Valeria; Valente, Michele; d'Amati, Giulia

    2016-01-01

    The term idiopathic noncirrhotic portal hypertension (INCPH) has been recently proposed to replace terms, such as hepatoportal sclerosis, idiopathic portal hypertension, incomplete septal cirrhosis, and nodular regenerative hyperplasia, used to describe patients with a hepatic presinusoidal cause of portal hypertension of unknown etiology, characterized by features of portal hypertension (esophageal varices, nonmalignant ascites, porto-venous collaterals), splenomegaly, patent portal, and hepatic veins and no clinical and histological signs of cirrhosis. Physicians should learn to look for this condition in a number of clinical settings, including cryptogenic cirrhosis, a disease known to be associated with INCPH, drug administration, and even chronic alterations in liver function tests. Once INCPH is clinically suspected, liver histology becomes mandatory for the correct diagnosis. However, pathologists should be familiar with the histological features of INCPH, especially in cases in which histology is not only requested to exclude liver cirrhosis.

  2. Idiopathic Retroperitoneal Hematoma

    African Journals Online (AJOL)

    6. Stewart BT, McLaughlin SJ, Thompson GA. Spontaneous retroperitoneal haemorrhage:a general surgeon's perspective. Aust N. Z J Surg 1998;68:371-3. Monib, et al.: Idiopathic retroperitoneal hematoma. How to cite this article: Monib S, Ritchie A, Thabet E. Idiopathic retroperitoneal hematoma. J Surg Tech Case Report ...

  3. Idiopathic hypertrophic subaortic stenosis. I

    International Nuclear Information System (INIS)

    Kvam, G.

    1980-01-01

    Biplane left ventricular cineangiographies in 4 patients with typical obstructive idiopathic hypertrophic subaortic stenosis (IHSS) and in control patients with normal left ventricles were analysed. In the protruding hypertrophic muscular interventricular septum of IHSS a markedly reduced shortening occurs in either direction during the systolic contraction. It does not bend towards the right ventricle. It is suggested that the septum of IHSS acts as a suspender during the systolic contraction, thereby accounting for the fast stroke volume ejection and the high ejection fraction of IHSS. (Auth.)

  4. Girl with idiopathic childhood hypercalcemia reveals new disease-causing CYP24A1 mutation

    DEFF Research Database (Denmark)

    Madsen, Jens Otto Broby; Sauer, Sabrina; Beck, Bodo

    2018-01-01

    of a 21 months old girl initially hospitalized due to excessive consumption of water and behavioral difficulties. Blood tests showed hypercalcemia, borderline high vitamin-D levels, and renal ultrasound revealed medullary nephrocalcinosis. An abnormality within the vitamin-D metabolism was suspected......CONTEXT: Idiopathic Infantile Hypercalcemia (IHH) was associated with vitamin-D supplementation in the 1950's. 50 years later mutations in the CYP241A gene, involved in the degradation of vitamin-D, have been identified as being a part of the etiology. CASE DESCRIPTION: We hereby report a case...... and genetic testing was performed. This revealed the patient to be compound heterozygous for a common (p.E143del) and a novel (likely) disease-causing mutation (p.H83D) in the CYP24A1 gene. The hypercalcemia normalized after calcium depleted diet and discontinuation of vitamin-D supplementation. CONCLUSIONS...

  5. Idiopathic pulmonary fibrosis

    Directory of Open Access Journals (Sweden)

    Noble Paul W

    2008-03-01

    Full Text Available Abstract Idiopathic pulmonary fibrosis (IPF is a non-neoplastic pulmonary disease that is characterized by the formation of scar tissue within the lungs in the absence of any known provocation. IPF is a rare disease which affects approximately 5 million persons worldwide. The prevalence is estimated to be slightly greater in men (20.2/100,000 than in women (13.2/100,000. The mean age at presentation is 66 years. IPF initially manifests with symptoms of exercise-induced breathless and dry coughing. Auscultation of the lungs reveals early inspiratory crackles, predominantly located in the lower posterior lung zones upon physical exam. Clubbing is found in approximately 50% of IPF patients. Cor pulmonale develops in association with end-stage disease. In that case, classic signs of right heart failure may be present. Etiology remains incompletely understood. Some environmental factors may be associated with IPF (cigarette smoking, exposure to silica and livestock. IPF is recognized on high-resolution computed tomography by peripheral, subpleural lower lobe reticular opacities in association with subpleural honeycomb changes. IPF is associated with a pathological lesion known as usual interstitial pneumonia (UIP. The UIP pattern consists of normal lung alternating with patches of dense fibrosis, taking the form of collagen sheets. The diagnosis of IPF requires correlation of the clinical setting with radiographic images and a lung biopsy. In the absence of lung biopsy, the diagnosis of IPF can be made by defined clinical criteria that were published in guidelines endorsed by several professional societies. Differential diagnosis includes other idiopathic interstitial pneumonia, connective tissue diseases (systemic sclerosis, polymyositis, rheumatoid arthritis, forme fruste of autoimmune disorders, chronic hypersensitivity pneumonitis and other environmental (sometimes occupational exposures. IPF is typically progressive and leads to significant

  6. Adolescent Idiopathic Scoliosis

    Science.gov (United States)

    ... Radiation Exposure in Scoliosis Kyphosis Adolescent Back Pain Spondylolysis For Adolescents For Adults Common Questions & Glossary Resources ... Radiation Exposure in Scoliosis Kyphosis Adolescent Back Pain Spondylolysis For Adolescents For Adults Juvenile Idiopathic Scoliosis Diagnosed ...

  7. Adolescent Idiopathic Scoliosis

    Directory of Open Access Journals (Sweden)

    Safak Ekinci

    2014-06-01

    Full Text Available Scoliosis is called idiopathic when no other underlying disease can be identified. The etiology of adolescent idiopathic scoliosis (AIS is still unknown despite many years of research effort. Theories on AIS's etiology have included mechanical, hormonal, metabolic, neuromuscular, growth, and genetic abnormalities. Skeletally immature patients with adolescent idiopathic scoliosis are at risk of curve progression. The adolescent onset of severe idiopathic scoliosis has traditionally been evaluated using standing posteroanterior radiographs of the full spine to assess lateral curvature with the Cobb method. Scoliosis in children of school age and above primarily occurs in girls. The therapeutic goal in children is to prevent progression. In children, scoliosis of 20 and deg; or more should be treated with a brace, and scoliosis of 45 and deg; or more with surgery. [Arch Clin Exp Surg 2014; 3(3.000: 174-182

  8. Understanding idiopathic intracranial hypertension

    DEFF Research Database (Denmark)

    Markey, Keira A; Mollan, Susan P; Jensen, Rigmor H

    2016-01-01

    Idiopathic intracranial hypertension is a disorder characterised by raised intracranial pressure that predominantly affects young, obese women. Pathogenesis has not been fully elucidated, but several causal factors have been proposed. Symptoms can include headaches, visual loss, pulsatile tinnitus...

  9. Graves' disease and idiopathic intracranial hypertension

    OpenAIRE

    Manish Gutch; Annesh Bhattacharjee; Sukriti Kumar; Durgesh Pushkar

    2017-01-01

    Idiopathic intracranial hypertension (IIH) is a central nervous system disorder characterized by raised intracranial pressure with normal cerebrospinal fluid composition and absence of any structural anomaly on neuroimaging. Among all endocrine disorders associated with the development of IIH, the association of hyperthyroidism and IIH is very rare with few cases reported till date. Thyroid disturbances have a unique association with IIH. Hypo- and hyper-thyroidism have been reported in assoc...

  10. PATTERN OF INHERITANCE OF IDIOPATHIC HYPERCALCIURIA IN TWO FAMILIES

    Directory of Open Access Journals (Sweden)

    A. Nickavar

    2006-09-01

    Full Text Available Idiopathic hypercalciuria is a leading cause of frequency-dysuria syndrome in childhood. Different modes of inheritance have been suggested in this disease. This article presents the occurrence of idiopathic hypercalciuria in all children of two families. In the first family, a 5.5 year old girl with a history of renal stones and dysuria due to hypercalciuria, had two involved brothers and one sister. In the second family, hypercalciuria and medullary nephrocalcinosis were detected in two siblings who were admitted for polyuria and dysuria. Idiopathic type of hypercalciuria was diagnosed in these two families by normal laboratory exams and exclusion of other causes of normocalcemic hypercalciuria. According to the involvement of all offsprings (both sexes in these two families, it is suggested that idiopathic hypercalciuria is an autosomal dominant disease with complete penetration.

  11. Skin perfusion pressure measured with a photo sensor in an air-filled plastic balloon: validity and reproducibility on the lower leg in normal subjects and patients suspected of obliterative arterial disease

    DEFF Research Database (Denmark)

    Nielsen, Steen Levin; Nielsen, Anne Lerberg; Vind, Susanne Haase

    2011-01-01

    An inflatable small plastic bag including a photo sensor was constructed for measurement of skin perfusion pressure avoiding the rim of the photo sensor over bony and tendineous surfaces of the tibia below the knee, at the ankle, and on the dorsal forefoot. Compression was obtained using a conical...... blood pressure cuff with continuous decrease from suprasystolic arm pressure. The validity of skin perfusion pressure with the new device was compared to that of isotope washout below the knee in normal subjects and in patients with an ischemic forefoot with acceptable agreement. The method had a high...... reproducibility within and between days in normal subjects. Compared to systolic arterial pressure measured using a strain gauge with a cuff on the ankle in normal subjects and patients with intermittent claudication the new device showed blood pressure in the skin closer to the diastolic pressure. The new...

  12. Idiopathic ophthalmodynia and idiopathic rhinalgia: two topographic facial pain syndromes.

    Science.gov (United States)

    Pareja, Juan A; Cuadrado, María L; Porta-Etessam, Jesús; Fernández-de-las-Peñas, César; Gili, Pablo; Caminero, Ana B; Cebrián, José L

    2010-09-01

    To describe 2 topographic facial pain conditions with the pain clearly localized in the eye (idiopathic ophthalmodynia) or in the nose (idiopathic rhinalgia), and to propose their distinction from persistent idiopathic facial pain. Persistent idiopathic facial pain, burning mouth syndrome, atypical odontalgia, and facial arthromyalgia are idiopathic facial pain syndromes that have been separated according to topographical criteria. Still, some other facial pain syndromes might have been veiled under the broad term of persistent idiopathic facial pain. Through a 10-year period we have studied all patients referred to our neurological clinic because of facial pain of unknown etiology that might deviate from all well-characterized facial pain syndromes. In a group of patients we have identified 2 consistent clinical pictures with pain precisely located either in the eye (n=11) or in the nose (n=7). Clinical features resembled those of other localized idiopathic facial syndromes, the key differences relying on the topographic distribution of the pain. Both idiopathic ophthalmodynia and idiopathic rhinalgia seem specific pain syndromes with a distinctive location, and may deserve a nosologic status just as other focal pain syndromes of the face. Whether all such focal syndromes are topographic variants of persistent idiopathic facial pain or independent disorders remains a controversial issue.

  13. Hypercalcemia in idiopathic myelofibrosis

    DEFF Research Database (Denmark)

    Voss, A; Schmidt, K; Hasselbalch, H

    1992-01-01

    A case of idiopathic myelofibrosis (IMF) presenting with hypercalcemia and hypercalcitriolemia is reported. It is proposed that ectopic production of the active vitamin D metabolite related to ongoing clonal expansion in the bone marrow accounts for the hypercalcemic state. Consistently low level...

  14. Idiopathic scrotal elephantiasis.

    Science.gov (United States)

    Hornberger, Brad J; Elmore, James M; Roehrborn, Claus G

    2005-02-01

    Scrotal lymphedema (scrotal elephantiasis) is a condition that has historically been described in areas endemic to filariasis. We present a unique case of a 22-year-old man with idiopathic lymphedema isolated to the scrotum. After acquired causes of lymphedema were ruled out, the patient was treated with scrotectomy and scrotal reconstruction.

  15. Idiopathic central diabetes Insipidus.

    Science.gov (United States)

    Grace, Mary; Balachandran, Venu; Menon, Sooraj

    2011-10-01

    Idiopathic central diabetes insipidus (CDI) is a rare disorder characterized clinically by polyuria and polydipsia, and an abnormal urinary concentration without any identified etiology. We report a case of central diabetes insipidus in a 60-year-old lady in the absence of secondary causes like trauma, infection, and infiltrative disorders of brain.

  16. Idiopathic epiretinal membrane

    NARCIS (Netherlands)

    Bu, Shao-Chong; Kuijer, Roelof; Li, Xiao-Rong; Hooymans, Johanna M M; Los, Leonoor I

    2014-01-01

    Background: Idiopathic epiretinal membrane (iERM) is a fibrocellular membrane that proliferates on the inner surface of the retina at the macular area. Membrane contraction is an important sight-threatening event and is due to fibrotic remodeling. Methods: Analysis of the current literature

  17. Skin perfusion pressure measured with a photo sensor in an air-filled plastic balloon: validity and reproducibility on the lower leg in normal subjects and patients suspected of obliterative arterial disease

    International Nuclear Information System (INIS)

    Nielsen, Steen Levin; Nielsen, Anne Lerberg; Vind, Susanne Haase; Thomassen, Anders

    2011-01-01

    An inflatable small plastic bag including a photo sensor was constructed for measurement of skin perfusion pressure avoiding the rim of the photo sensor over bony and tendineous surfaces of the tibia below the knee, at the ankle, and on the dorsal forefoot. Compression was obtained using a conical blood pressure cuff with continuous decrease from suprasystolic arm pressure. The validity of skin perfusion pressure with the new device was compared to that of isotope washout below the knee in normal subjects and in patients with an ischemic forefoot with acceptable agreement. The method had a high reproducibility within and between days in normal subjects. Compared to systolic arterial pressure measured using a strain gauge with a cuff on the ankle in normal subjects and patients with intermittent claudication the new device showed blood pressure in the skin closer to the diastolic pressure. The new pressure device thus had acceptable validity and reproducibility for estimation of the skin perfusion pressure and can be used on bony and tendineous sites on the lower limb in regions where critical wound healing is frequent, e.g. ankle and forefoot

  18. Idiopathic intracranial hypertension in pediatric patients

    Directory of Open Access Journals (Sweden)

    Nada Jirásková

    2008-11-01

    Full Text Available Nada Jirásková, Pavel RozsívalDepartment of Ophthalmology, University Hospital, Hradec Králové, Czech RepublicPurpose: To evaluate retrospectively the features, treatment, and outcome of idiopathic intracranial hypertension (IIH in children.Methods: Nine patients, 15 years and younger, diagnosed with IIH. Inclusion criteria were papilledema, normal brain computer tomography or magnetic resonance imaging, cerebrospinal fluid pressure greater than 250 mm H2O, normal cerebrospinal fluid content, and a nonfocal neurologic examination except for sixth nerve palsy.Results: Of the nine patients, eight were girls. Five girls were overweight and one boy was obese. The most common presenting symptom was headache (5 patients. Diplopia or strabismus did not occur in our group. Visual field abnormalities were present in all eyes, and severe visual loss resulting in light perception vision occurred in both eyes of one patient. Eight patients were treated medically with acetazolamide alone, and one girl needed a combination of acetazolamide and corticosteroids. This girl also required optic nerve sheath decompression surgery. Resolution of papilledema and recovery of visual function occurred in all patients.Conclusions: Idiopathic intracranial hypertension in prepubertal children is rather uncommon. Prompt diagnosis and management are important to prevent permanent visual loss.Keywords: idiopathic intracranial hypertension, pediatric, treatment

  19. Genetics Home Reference: adolescent idiopathic scoliosis

    Science.gov (United States)

    ... Facebook Twitter Home Health Conditions Adolescent idiopathic scoliosis Adolescent idiopathic scoliosis Printable PDF Open All Close All ... Javascript to view the expand/collapse boxes. Description Adolescent idiopathic scoliosis is an abnormal curvature of the ...

  20. Idiopathic gingival fibromatosis

    Directory of Open Access Journals (Sweden)

    Sujata Rath

    2011-01-01

    This article presents a case report of a 14-year-old female patient with idiopathic gingival fibromatosis in the maxillary region with radiographic feature of congenitally missing maxillary permanent left lateral incisor, maxillary left and right permanent canine, mandibular right second premolar, all third molars along with overretained primary maxillary left lateral incisor and primary mandibular second molar. The treatment rendered in this patient comprised of surgical excision of the hyperplasia under general anesthesia.

  1. Acute Idiopathic Scrotal Edema

    Directory of Open Access Journals (Sweden)

    Micheál Breen

    2013-01-01

    Full Text Available We report a case of acute idiopathic scrotal edema (AISE in a 4-year-old boy who presented with acute scrotal pain and erythema. The clinical features, ultrasound appearance, and natural history of this rare diagnosis are reviewed. In this report, we highlight the importance of good ultrasound technique in differentiating the etiology of the acute scrotum and demonstrate the color Doppler “Fountain Sign” that is highly suggestive of AISE.

  2. Chronic idiopathic intestinal pseudo-obstruction in an English bulldog.

    Science.gov (United States)

    Dvir, E; Leisewitz, A L; Van der Lugt, J J

    2001-05-01

    A case of chronic idiopathic intestinal pseudo-obstruction in an English bulldog is described. The dog was presented with chronic weight loss and vomiting. An intestinal obstruction was suspected based on clinical and radiological findings. A diagnosis of chronic idiopathic intestinal pseudo-obstruction was made on the basis of full thickness intestinal biopsies. The dog was refractory to any antiemetic therapy. Necropsy revealed marked atrophy and fibrosis of the tunica muscularis, together with a mononuclear cell infiltrate extending from the duodenum to the colon. This case was presented with clinical findings consistent with visceral myopathy in humans--namely, atony and dilatation of the whole gut--but the histological findings resembled sclerosis limited to the gastrointestinal tract.

  3. Idiopathic Intracranial Hypertension (Pseudotumor Cerebri)

    Science.gov (United States)

    ... cause is determined and is referred to as “secondary” intracranial hypertension. What are the risk factors for idiopathic intracranial ... clotting disorders, anemia and malnutrition. Can idiopathic intracranial ... to be “secondary” which affects males and females equally. The second ...

  4. Magnetic resonance angiography in suspected cerebral vasculitis

    International Nuclear Information System (INIS)

    Demaerel, Philippe; De Ruyter, Nele; Wilms, Guido; Maes, Frederik; Velghe, Beatrijs

    2004-01-01

    The purpose of this study was to determine the technical capacity and diagnostic accuracy of 3D time-of-flight magnetic resonance angiography (MRA) in suspected cerebral vasculitis in a retrospective analysis of MRA and digital subtraction angiography (DSA) in 14 young patients with clinical and/or radiological suspicion of cerebral vasculitis. A total of nine arteries were evaluated in each patient. Consensus review of DSA by three observers was the reference standard. The sensitivity for detecting a stenosis varied from 62 to 79% for MRA and from 76 to 94% for DSA, depending on the observer. The specificity for detecting a stenosis varied from 83 to 87% for MRA and from 83 to 97% for DSA. Using the criterion ''more than two stenoses in at least two separate vascular distributions'' to consider the examination as being true positive, the false-positive rates for MRA and DSA were comparable. MRA plays a role as the first angiographical examination in the diagnostic work-up of suspected cerebral vasculitis. When more than two stenoses in at least two separate vascular distributions are depicted on MRA, DSA is not expected to add a significant diagnostic contribution in a patient with suspected cerebral vasculitis. DSA remains necessary when MRA is normal or when less than three stenoses are seen. (orig.)

  5. Magnetic resonance angiography in suspected cerebral vasculitis

    Energy Technology Data Exchange (ETDEWEB)

    Demaerel, Philippe; De Ruyter, Nele; Wilms, Guido [Department of Radiology, Universitair Ziekenhuis, KU Leuven, 3000, Leuven (Belgium); Maes, Frederik [Department of Medical Imaging Computing, Universitair Ziekenhuis, KU Leuven, 3000, Leuven (Belgium); Velghe, Beatrijs [Department of Radiology, Ziekenhuis Oost-Limburg, Schiepse Bos 6, 3600, Genk (Belgium)

    2004-06-01

    The purpose of this study was to determine the technical capacity and diagnostic accuracy of 3D time-of-flight magnetic resonance angiography (MRA) in suspected cerebral vasculitis in a retrospective analysis of MRA and digital subtraction angiography (DSA) in 14 young patients with clinical and/or radiological suspicion of cerebral vasculitis. A total of nine arteries were evaluated in each patient. Consensus review of DSA by three observers was the reference standard. The sensitivity for detecting a stenosis varied from 62 to 79% for MRA and from 76 to 94% for DSA, depending on the observer. The specificity for detecting a stenosis varied from 83 to 87% for MRA and from 83 to 97% for DSA. Using the criterion ''more than two stenoses in at least two separate vascular distributions'' to consider the examination as being true positive, the false-positive rates for MRA and DSA were comparable. MRA plays a role as the first angiographical examination in the diagnostic work-up of suspected cerebral vasculitis. When more than two stenoses in at least two separate vascular distributions are depicted on MRA, DSA is not expected to add a significant diagnostic contribution in a patient with suspected cerebral vasculitis. DSA remains necessary when MRA is normal or when less than three stenoses are seen. (orig.)

  6. Idiopathic megarectum in children.

    Science.gov (United States)

    Godbole, P P; Pinfield, A; Stringer, M D

    2001-02-01

    There is scant information about the management of idiopathic megarectum in childhood. Children with idiopathic megarectum referred to a single institution between 1994 and 1998 were identified prospectively. Those with Hirschsprung's disease or an anorectal malformation were excluded. The remaining patient group, 22 boys and 7 girls, had a median age of 8.0 years (range 3.5-14.0 y). Median duration of symptoms prior to referral was 2.0 years (range 0.4-11 y). Chronic soiling was the dominant complaint in 28/29 (97%) cases. 23 children had received regular stimulant laxatives for periods ranging from 1 month to 11 years, and 9 children had been treated with regular enemas. The degree of megarectum assessed by both abdominal palpation and plain radiography was: grade 1 (below umbilical level) n=6; grade 2 (at umbilical level) n=15; and grade 3 (above umbilical level) n=8. Hirschsprung's disease was specifically excluded by rectal biopsy in all cases and no patient had evidence of spinal dysraphism. Three boys with massive megarectums and intractable symptoms were treated by a staged Duhamel sigmoid pull-through with excellent functional results. Fifteen patients (52%) were treated by a single manual evacuation under general anaesthesia followed by a daily Bisacodyl 5-10 mg suppository. After a median follow-up of 16 months, 13 continue to respond well with a daily bowel action and no soiling (4 of the 13 have discontinued treatment and remain well). The remaining 11 patients (38%) have continued conventional treatment with oral laxatives but with limited success. Idiopathic megarectum is poorly described in children. It is more common in boys and is often resistant to laxative therapy alone. After appropriate preparation, treatment with stimulant suppositories can be effective. Surgery has a valuable role in selected patients with a massive megarectum.

  7. Idiopathic granulomatous lobular mastitis.

    Science.gov (United States)

    Pereira, Frederick A; Mudgil, Adarsh V; Macias, Edgar S; Karsif, Karen

    2012-02-01

    Idiopathic granulomatous lobular mastitis (IGLM) is a rare breast condition with prominent skin findings. It is typically seen in young parous women. Painful breast masses, draining sinuses, scarring, and breast atrophy are the main clinical manifestations. IGLM can resemble a variety of other inflammatory and neoplastic processes of the breast. It is thought to result from obstruction and rupture of breast lobules. Extravasated breast secretions then induce an inflammatory reaction. Corynebacteria have also been implicated in the pathogenesis. Treatment is surgical, but systemic corticosteroids, methotrexate, and antibiotics also play a role. © 2012 The International Society of Dermatology.

  8. Graves' disease and idiopathic intracranial hypertension

    Directory of Open Access Journals (Sweden)

    Manish Gutch

    2017-01-01

    Full Text Available Idiopathic intracranial hypertension (IIH is a central nervous system disorder characterized by raised intracranial pressure with normal cerebrospinal fluid composition and absence of any structural anomaly on neuroimaging. Among all endocrine disorders associated with the development of IIH, the association of hyperthyroidism and IIH is very rare with few cases reported till date. Thyroid disturbances have a unique association with IIH. Hypo- and hyper-thyroidism have been reported in association with this disorder. We present a rare case of a 25-year-old man with Graves' disease with intractable headache that was later investigated and attributed to development of IIH.

  9. Idiopathic pulmonary fibrosis.

    Science.gov (United States)

    Xaubet, Antoni; Ancochea, Julio; Molina-Molina, María

    2017-02-23

    Idiopathic pulmonary fibrosis is a fibrosing interstitial pneumonia associated with the radiological and/or histological pattern of usual interstitial pneumonia. Its aetiology is unknown, but probably comprises the action of endogenous and exogenous micro-environmental factors in subjects with genetic predisposition. Its diagnosis is based on the presence of characteristic findings of high-resolution computed tomography scans and pulmonary biopsies in absence of interstitial lung diseases of other aetiologies. Its clinical evolution is variable, although the mean survival rate is 2-5 years as of its clinical presentation. Patients with idiopathic pulmonary fibrosis may present complications and comorbidities which modify the disease's clinical course and prognosis. In the mild-moderate disease, the treatment consists of the administration of anti-fibrotic drugs. In severe disease, the best therapeutic option is pulmonary transplantation. In this paper we review the diagnostic and therapeutic aspects of the disease. Copyright © 2016 Elsevier España, S.L.U. All rights reserved.

  10. Pneumothorax and idiopathic pulmonary fibrosis

    International Nuclear Information System (INIS)

    Iwasawa, Tae; Ogura, Takashi; Takahashi, Hiroshi; Asakura, Akira; Gotoh, Toshiyuki; Yazawa, Takuya; Inoue, Tomio

    2010-01-01

    We evaluated the relation between the severity of idiopathic pulmonary fibrosis (IPF) and the incidence of pneumothorax on computed tomography (CT) images. In this retrospective study, we evaluated the presence of pneumothorax in 56 consecutive patients who died of IPF from the initial CT to death. We quantitatively analyzed a total of 207 CT images and measured the volume of the normal pattern (N-pattern) and each lesion pattern on the initial CT and their serial changes. The effects of pneumothorax and clinical and CT features on survival were evaluated using Cox regression analysis. Pneumothorax occurred in 17 of 56 patients. Comparison of the pneumothorax (+) and (-) groups showed the initial vital capacity (VC) was lower (P=0.005) and the follow-up period was shorter (P=0.03) in the former group. The decrease in the N-pattern volume in the pneumothorax (+) group was significantly faster than in the pneumothorax (-) group (P=0.013). Cox regression analyses identified a rapid decrease in N-pattern volume (P=0.008) and a rapid decrease in VC (P=0.002), but not pneumothorax, as significant predictors of poor survival. Pneumothorax in IPF patients is associated with lower VC and rapid deterioration of CT findings. The findings suggest that pneumothorax is a complication of advanced IPF. (author)

  11. Changes in plasma atrial natriuretic factor in patients with idiopathic atrial fibrillation

    International Nuclear Information System (INIS)

    Du Tongxin; Xia Xiaojie; Qu Wei; Wang Shukui; Sun Junjiang

    2002-01-01

    To observe the changes in plasma atrial natriuretic factor (AFN) in patients with idiopathic atrial fibrillation and investigate its mechanism, plasma ANF, platelet count and hematocrit were detected in 21 cases with transient idiopathic atrial fibrillation (group A, A1 representing attack, while A2 termination), 28 with persistent idiopathic atrial fibrillation (group B), 27 suffered from rheumatic heart disease with mitral stenosis and persistent atrial fibrillation (group C), 32 with transient supraventricular tachycardia (group D) and 20 normal controls (group E). It was found that the level of ANF was significantly higher in patients with attacking transient idiopathic atrial fibrillation than that in group A2, D and E (P 0.05), while there was significant difference in hematocrit in group A1 compared with group A2, D, E (P < 0.01). It suggested that ANF and hematocrit play an important role in the attack of idiopathic atrial fibrillation

  12. Association between adolescent idiopathic scoliosis prevalence and age at menarche in different geographic latitudes

    Directory of Open Access Journals (Sweden)

    Mihas Constantinos

    2006-05-01

    Full Text Available Abstract Background Age at menarche is considered a reliable prognostic factor for idiopathic scoliosis and varies in different geographic latitudes. Adolescent idiopathic scoliosis prevalence has also been reported to be different in various latitudes and demonstrates higher values in northern countries. A study on epidemiological reports from the literature was conducted to investigate a possible association between prevalence of adolescent idiopathic scoliosis and age at menarche among normal girls in various geographic latitudes. An attempt is also made to implicate a possible role of melatonin in the above association. Material-methods 20 peer-reviewed published papers reporting adolescent idiopathic scoliosis prevalence and 33 peer-reviewed papers reporting age at menarche in normal girls from most geographic areas of the northern hemisphere were retrieved from the literature. The geographic latitude of each centre where a particular study was originated was documented. The statistical analysis included regression of the adolescent idiopathic scoliosis prevalence and age at menarche by latitude. Results The regression of prevalence of adolescent idiopathic scoliosis and age at menarche by latitude is statistically significant (p Conclusion Late age at menarche is parallel with higher prevalence of adolescent idiopathic scoliosis. Pubarche appears later in girls that live in northern latitudes and thus prolongs the period of spine vulnerability while other pre-existing or aetiological factors are contributing to the development of adolescent idiopathic scoliosis. A possible role of geography in the pathogenesis of idiopathic scoliosis is discussed, as it appears that latitude which differentiates the sunlight influences melatonin secretion and modifies age at menarche, which is associated to the prevalence of idiopathic scoliosis.

  13. Prime Suspect, Second Row Center

    Science.gov (United States)

    Laird, Ellen A.

    2011-01-01

    His father had been hacked to death in his own bed with an ax the previous November. His mother was similarly brutalized and left for dead with her husband but survived. On the last Monday of that August, after several months and many investigative twists, turns, and fumbles, there sat the son--the prime suspect--in Ellen Laird's literature class,…

  14. Idiopathic thrombocytopenic purpura

    Directory of Open Access Journals (Sweden)

    L Kayal

    2014-01-01

    Full Text Available Idiopathic thrombocytopenic purpura (ITP is defined as a hematologic disorder, characterized by isolated thrombocytopenia without a clinically apparent cause. The major causes of accelerated platelet consumption include immune thrombocytopenia, decreased bone marrow production, and increased splenic sequestration. The clinical presentation may be acute with severe bleeding, or insidious with slow development with mild or no symptoms. The initial laboratory tests useful at the first visit to predict future diagnosis were erythrocyte count, leukocyte count, anti-glycoprotein IIb/IIIa antibodies, reticulated platelets, plasma thrombopoietin level. Treatment should be restricted to those patients with moderate or severe thrombocytopenia who are bleeding or at risk of bleeding. We present a case report on ITP with clinical presentation, diagnosis and management.

  15. Persistent idiopathic facial pain

    DEFF Research Database (Denmark)

    Maarbjerg, Stine; Wolfram, Frauke; Heinskou, Tone Bruvik

    2017-01-01

    Introduction: Persistent idiopathic facial pain (PIFP) is a poorly understood chronic orofacial pain disorder and a differential diagnosis to trigeminal neuralgia. To address the lack of systematic studies in PIFP we here report clinical characteristics and neuroimaging findings in PIFP. Methods...... pain 7 (13%), hypoesthesia 23 (48%), depression 16 (30%) and other chronic pain conditions 17 (32%) and a low prevalence of stabbing pain 21 (40%), touch-evoked pain 14 (26%) and remission periods 10 (19%). The odds ratio between neurovascular contact and the painful side was 1.4 (95% Cl 0.4–4.4, p = 0.......565) and the odds ratio between neurovascular contact with displacement of the trigeminal nerve and the painful side was 0.2 (95% Cl 0.0–2.1, p = 0.195). Conclusion: PIFP is separated from trigeminal neuralgia both with respect to the clinical characteristics and neuroimaging findings, as NVC was not associated...

  16. ADHD in idiopathic epilepsy

    Directory of Open Access Journals (Sweden)

    Marcos H. C. Duran

    2014-01-01

    Full Text Available Our aim was to clarify the correlation of attention deficit hyperactivity disorder (ADHD with epilepsy and behavior problems. This was a cross-sectional study. Sixty children with idiopathic epilepsy were interviewed using the MTA-SNAP IV Teacher and Parent Rating Scale, Vineland Adaptive Behavior Scales and Conners’ Rating Scales. We used the chi-square test to analyze the correlation of epilepsy variables in patients with and without ADHD with a significance level of 0.05. Eight patients had ADHD symptoms (13%, seven had the inattentive ADHD subtype and only three had behavioral problems. When epileptic patients with and without ADHD symptoms were compared we found no significant difference in regard to epilepsy variables. All patients were controlled and 43% were either without AED or undergoing withdrawal. Our study revealed a low comorbidity of ADHD symptoms and epilepsy due to low interference of seizures and drug treatment on the comorbid condition.

  17. Atypical idiopathic inflammatory demyelinating lesions

    DEFF Research Database (Denmark)

    Wallner-Blazek, Mirja; Rovira, Alex; Fillipp, Massimo

    2013-01-01

    Atypical lesions of a presumably idiopathic inflammatory demyelinating origin present quite variably and may pose diagnostic problems. The subsequent clinical course is also uncertain. We, therefore, wanted to clarify if atypical idiopathic inflammatory demyelinating lesions (AIIDLs) can be class......Atypical lesions of a presumably idiopathic inflammatory demyelinating origin present quite variably and may pose diagnostic problems. The subsequent clinical course is also uncertain. We, therefore, wanted to clarify if atypical idiopathic inflammatory demyelinating lesions (AIIDLs) can...... be classified according to previously suggested radiologic characteristics and how this classification relates to prognosis. Searching the databases of eight tertiary referral centres we identified 90 adult patients (61 women, 29 men; mean age 34 years) with ≥1 AIIDL. We collected their demographic, clinical...

  18. Idiopathic ventricular tachycardia and fibrillation.

    Science.gov (United States)

    Belhassen, B; Viskin, S

    1993-06-01

    Important data have recently been added to our understanding of sustained ventricular tachyarrhythmias occurring in the absence of demonstrable heart disease. Idiopathic ventricular tachycardia (VT) is usually of monomorphic configuration and can be classified according to its site of origin as either right monomorphic (70% of all idiopathic VTs) or left monomorphic VT. Several physiopathological types of monomorphic VT can be presently individualized, according to their mode of presentation, their relationship to adrenergic stress, or their response to various drugs. The long-term prognosis is usually good. Idiopathic polymorphic VT is a much rarer type of arrhythmia with a less favorable prognosis. Idiopathic ventricular fibrillation may represent an underestimated cause of sudden cardiac death in ostensibly healty patients. A high incidence of inducibility of sustained polymorphic VT with programmed ventricular stimulation has been found by our group, but not by others. Long-term prognosis on Class IA antiarrhythmic medications that are highly effective at electrophysiologic study appears excellent.

  19. Idiopathic pulmonary fibrosis: treatment update.

    LENUS (Irish Health Repository)

    O'Connell, Oisin J

    2011-11-01

    Idiopathic pulmonary fibrosis (IPF) is the most common of the idiopathic interstitial pneumonias. Despite multiple recent clinical trials, there is no strong evidence supporting a survival advantage for any agent in the management of patients with IPF. The limited effectiveness of current treatment regimes has led to a search for novel therapies including antifibrotic strategies. This article reviews the evidence supporting the treatments currently used in the management of IPF.

  20. Evaluation of idiopathic transverse myelitis revealing specific myelopathy diagnoses.

    Science.gov (United States)

    Zalewski, Nicholas L; Flanagan, Eoin P; Keegan, B Mark

    2018-01-09

    To evaluate specific myelopathy diagnoses made in patients with suspected idiopathic transverse myelitis (ITM). A total of 226 patients 18 years and older were referred to Mayo Clinic Neurology for suspected ITM from December 1, 2010, to December 31, 2015. Electronic medical records were reviewed for detailed clinical presentation and course, laboratory and electrophysiologic investigations, and neuroimaging to determine the etiology. Current diagnostic criteria for ITM and alternative myelopathy diagnoses were applied. All cases where any discrepancy was suspected from the final reported clinical diagnosis were reviewed by each author and a consensus final diagnosis was made. The diagnostic criteria for ITM were met in 41 of 226 patients (18.1%). In 158 patients (69.9%), an alternative specific myelopathy diagnosis was made: multiple sclerosis or clinically isolated syndrome, 75; vascular myelopathy, 41; neurosarcoidosis, 12; neuromyelitis optica spectrum disorder, 12; myelin oligodendrocyte glycoprotein myelopathy, 5; neoplastic, 4; compressive, 3; nutritional, 3; infectious, 2; and other, 2. A myelopathy was not confirmed in 27 patients. Time from symptom onset to final clinical diagnosis in patients without ITM was a median of 9 months (range 0-288). Fifty-five patients (24%) required treatment changes according to their final clinical diagnosis. The majority of patients with suspected ITM have an alternative specific myelopathy diagnosis. A presumptive diagnosis of ITM can lead to premature diagnostic conclusions affecting patient treatment. Copyright © 2017 American Academy of Neurology.

  1. Idiopathic precocious puberty in girls: Psychosexual development.

    Science.gov (United States)

    Meyer-Bahlburg, H F; Ehrhardt, A A; Bell, J J; Cohen, S F; Healey, J M; Feldman, J F; Morishima, A; Baker, S W; New, M I

    1985-08-01

    A promising model syndrome for the examination of the role of physical maturation in the development of female sexuality is idiopathic precocious puberty (IPP). In this first controlled study of psychosexual development in IPP females, 16 females between 13 and 20 years of age with a history of IPP were compared to 16 control subjects with a history of normal puberty pair-matched to the index subjects on the basis of sex, race, age, socioeconomic level, and menarcheal status. The psychosexual history and the current psychosexual status were assessed by a systematic half-structured interview. The IPP females on average passed the psychosexual milestones at an earlier age than their normal maturing peers, with a particularly early onset of masturbation. Those who were sociosexually active tended to report a higher total orgasmic outlet and a higher sex drive. There was no increase in homosexuality among IPP girls. The timing of puberty has a (modest) influence on psychosexual development in females.

  2. Idiopathic intracranial hypertension: case report

    Directory of Open Access Journals (Sweden)

    Iacob G.

    2015-12-01

    Full Text Available Idiopathic intracranial hypertension - IIH (synonymous old terms: benign intracranial hypertension - BIH, pseudotumor-cerebri - PTC it’s a syndrome, related to elevated intracranial pressure, of unknown cause, sometimes cerebral emergency, occuring in all age groups, especially in children and young obese womans, in the absence of an underlying expansive intracranial lesion, despite extensive investigations. Although initial symptoms can resolve, IIH displays a high risk of recurrence several months or years later, even if initial symptoms resolved. Results: A 20-year-old male, obese since two years (body mass index 30, 9, was admitted for three months intense headache, vomiting, diplopia, progressive visual acuity loss. Neurologic examination confirmed diplopia by left abducens nerve palsy, papilledema right > left. At admission, cerebral CT scan and cerebral MRI with angio MRI 3DTOF and 2D venous TOF was normal. Despite treatment with acetazolamide (Diamox, corticosteroid, antidepressants (Amitriptyline, anticonvulsivants (Topiramate three weeks later headache, diplopia persist and vision become worse, confirmed by visual field assessment, visual evoked potential (VEP. A cerebral arteriography demonstrate filling defect of the superior sagittal sinus in the 1/3 proximal part and very week filling of the transverse right sinus on venous time. Trombophylic profile has revealed a heterozygote V factor Leyden mutation, a homozygote MTHFR and PAI mutation justifying an anticoagulant treatment initiated to the patient. The MRI showed a superior sagittal sinus, right transverse and sigmoid sinus thrombosis, dilatation and buckling of the optic nerve sheaths with increased perineural fluid especially retrobulbar, discrete flattening of the posterior segment of the eyeballs, spinal MRI showed posterior epidural space with dilated venous branches, with mass effect on the spinal cord, that occurs pushed anterior on sagittal T1/T2 sequences cervical and

  3. Drug therapy for chronic idiopathic axonal polyneuropathy

    NARCIS (Netherlands)

    Vrancken, A. F. J. E.; van Schaik, I. N.; Hughes, R. A. C.; Notermans, N. C.

    2004-01-01

    BACKGROUND: Chronic idiopathic axonal polyneuropathy is an insidiously progressive sensory or sensorimotor polyneuropathy that affects elderly people. Although severe disability or handicap does not occur, it reduces quality of life. OBJECTIVES: To assess whether drug therapy for chronic idiopathic

  4. Idiopathic inflammatory myositis.

    Science.gov (United States)

    Tieu, Joanna; Lundberg, Ingrid E; Limaye, Vidya

    2016-02-01

    Knowledge on idiopathic inflammatory myopathy (IIM) has evolved with the identification of myositis-associated and myositis-specific antibodies, development of histopathological classification and the recognition of how these correlate with clinical phenotype and response to therapy. In this paper, we outline key advances in diagnosis and histopathology, including the more recent identification of antibodies associated with immune-mediated necrotising myopathy (IMNM) and inclusion body myositis (IBM). Ongoing longitudinal observational cohorts allow further classification of these patients with IIM, their predicted clinical course and response to specific therapies. Registries have been developed worldwide for this purpose. A challenging aspect in IIM, a multisystem disease with multiple clinical subtypes, has been defining disease status and clinically relevant improvement. Tools for assessing activity and damage are now recognised to be important in determining disease activity and guiding therapeutic decision-making. The International Myositis Assessment and Clinical Studies (IMACS) group has developed such tools for use in research and clinical settings. There is limited evidence for specific treatment strategies in IIM. With significant development in the understanding of IIM and improved classification, longitudinal observational cohorts and trials using validated outcome measures are necessary, to provide important information for evidence-based care in the clinical setting. Crown Copyright © 2016. Published by Elsevier Ltd. All rights reserved.

  5. Idiopathic (primary achalasia

    Directory of Open Access Journals (Sweden)

    Vaezi Michael F

    2007-09-01

    Full Text Available Abstract Idiopathic achalasia is a primary esophageal motor disorder characterized by esophageal aperistalsis and abnormal lower esophageal sphincter (LES relaxation in response to deglutition. It is a rare disease with an annual incidence of approximately 1/100,000 and a prevalence rate of 1/10,000. The disease can occur at any age, with a similar rate in men and women, but is usually diagnosed between 25 and 60 years. It is characterized predominantly by dysphagia to solids and liquids, bland regurgitation, and chest pain. Weight loss (usually between 5 to 10 kg is present in most but not in all patients. Heartburn occurs in 27%–42% of achalasia patients. Etiology is unknown. Some familial cases have been reported, but the rarity of familial occurrence does not support the hypothesis that genetic inheritance is a significant etiologic factor. Association of achalasia with viral infections and auto-antibodies against myenteric plexus has been reported, but the causal relationship remains unclear. The diagnosis is based on history of the disease, radiography (barium esophagogram, and esophageal motility testing (esophageal manometry. Endoscopic examination is important to rule out malignancy as the cause of achalasia. Treatment is strictly palliative. Current medical and surgical therapeutic options (pneumatic dilation, surgical myotomy, and pharmacologic agents aimed at reducing the LES pressure and facilitating esophageal emptying by gravity and hydrostatic pressure of retained food and liquids. Although it cannot be permanently cured, excellent palliation is available in over 90% of patients.

  6. Idiopathic (primary) achalasia

    Science.gov (United States)

    Farrokhi, Farnoosh; Vaezi, Michael F

    2007-01-01

    Idiopathic achalasia is a primary esophageal motor disorder characterized by esophageal aperistalsis and abnormal lower esophageal sphincter (LES) relaxation in response to deglutition. It is a rare disease with an annual incidence of approximately 1/100,000 and a prevalence rate of 1/10,000. The disease can occur at any age, with a similar rate in men and women, but is usually diagnosed between 25 and 60 years. It is characterized predominantly by dysphagia to solids and liquids, bland regurgitation, and chest pain. Weight loss (usually between 5 to 10 kg) is present in most but not in all patients. Heartburn occurs in 27%–42% of achalasia patients. Etiology is unknown. Some familial cases have been reported, but the rarity of familial occurrence does not support the hypothesis that genetic inheritance is a significant etiologic factor. Association of achalasia with viral infections and auto-antibodies against myenteric plexus has been reported, but the causal relationship remains unclear. The diagnosis is based on history of the disease, radiography (barium esophagogram), and esophageal motility testing (esophageal manometry). Endoscopic examination is important to rule out malignancy as the cause of achalasia. Treatment is strictly palliative. Current medical and surgical therapeutic options (pneumatic dilation, surgical myotomy, and pharmacologic agents) aimed at reducing the LES pressure and facilitating esophageal emptying by gravity and hydrostatic pressure of retained food and liquids. Although it cannot be permanently cured, excellent palliation is available in over 90% of patients. PMID:17894899

  7. Idiopathic Retroperitoneal Hematoma

    Directory of Open Access Journals (Sweden)

    Tomoyuki Abe

    2010-09-01

    Full Text Available A 34-year-old female presented with sudden onset of severe abdominal pain in a flank distribution. A large mass was palpable in the right upper quadrant on physical examination. Abdominal contrast-enhanced computed tomography showed a well-defined, right-sided, retroperitoneal cystic lesion located between the abdominal aorta and the inferior vena cava (IVC. The tumor size was 55 × 58 mm, and it compressed the gallbladder and the duodenum. Upper gastrointestinal radiography revealed a stricture of the second portion of the duodenum by the tumor. T2-weighted magnetic resonance imaging showed that the whole part was hyperintense with hypointense rims, but the inner was partially hypointense. Based on the radiological findings, the preoperative differential diagnosis included retroperitoneal teratoma, Schwannoma, abscess, and primary retroperitoneal tumor. On laparotomy, the tumor was located in the right retroperitoneal cavity. Kocher maneuver and medial visceral rotation, which consists of medial reflection of the upper part of right colon and duodenum by incising their lateral peritoneal attachments, were performed. Although a slight adhesion to the IVC was detected, the tumor was removed safely. Thin-section histopathology examination detected neither tumor tissues nor any tissues such as adrenal gland, ovarian tissue, or endometrial implants. The final pathological diagnosis was idiopathic retroperitoneal hematoma; the origin of the bleeding was unclear. The patient was discharged without any complication 5 days after the operation.

  8. Idiopathic non-cirrhotic portal hypertension

    Directory of Open Access Journals (Sweden)

    CHEN Jie

    2013-07-01

    Full Text Available The pathogenesis of idiopathic non-cirrhotic portal hypertension (INCPH remains unknown and the disease is diagnosed by the absence of recognized clinical indicators of cirrhosis and of any other known etiologies of portal hypertension. To promote understanding of this disease, a comprehensive overview of potential etiologies, clinical manifestations, histopathological features, methods of diagnosis and potential differential diagnoses, and outcome of clinical management is presented in this review. In particular, we discuss the findings from INCPH studies and their implications in regards to each of the above-mentioned categories. For example, associations with various comorbidities have suggested a possible immune system component to INCPH development and/or progression. In addition, the common clinical characteristics of patients upon presentation can not only help to recognize disease suspects but may also provide insights into the pathogenesis and prognosis. Finally, prognosis following the various intervention strategies appears to depend mainly on severity of the portal hypertension, as well as its various accompanying complications.

  9. Aetiology of idiopathic granulomatous mastitis.

    Science.gov (United States)

    Altintoprak, Fatih; Kivilcim, Taner; Ozkan, Orhan Veli

    2014-12-16

    Idiopathic granulomatous mastitis is a rare chronic inflammatory lesion of the breast that can clinically and radiographically mimic breast carcinoma. The most common clinical presentation is an unilateral, discrete breast mass, nipple retraction and even a sinus formation often associated with an inflammation of the overlying skin. The etiology of idiopathic granulomatous mastitis is still obscure. Its treatment remains controversial. The cause may be the autoimmune process, infection, a chemical reaction associated with oral contraceptive pills, or even lactation. Various factors, including hormonal imbalance, autoimmunity, unknown microbiological agents, smoking and α 1-antitrypsin deficiency have been suggested to play a role in disease aetiology. In this review, causing factors in the aetiology of idiopathic granulomatous mastitis are reviewed in detail.

  10. Debate: idiopathic short stature should be treated with growth hormone.

    Science.gov (United States)

    Ambler, Geoffrey R; Fairchild, Jan; Wilkinson, Dominic J C

    2013-03-01

    In this paper we outline the case for and against the treatment of idiopathic short stature with growth hormone. Drs Ambler and Fairchild argue that many of those with 'idiopathic' short stature are not 'short, normal children' and will ultimately receive molecular diagnoses. They also argue that there is a subset of children who suffer negative psychosocial consequences of their stature for whom growth hormone therapy is effective. Growth hormone has a very good safety record and is likely to be as cost-effective in idiopathic short-stature as in some other conditions that are currently funded. Dr Wilkinson counters that short stature is not associated with physical or psychological illness, and that there is no evidence that growth hormone improves psychological or physical wellbeing. Moreover, growth hormone for idiopathic short stature represents a form of enhancement rather than treatment, and is not a fair use of resources. Socially mediated disadvantage should be treated by attention to prejudice and not by hormone treatment. © 2012 The Authors. Journal of Paediatrics and Child Health © 2012 Paediatrics and Child Health Division (Royal Australasian College of Physicians).

  11. Pediatric Idiopathic Intracranial Hypertension Presenting With Sensorineural Hearing Loss.

    Science.gov (United States)

    Reitsma, Sietze; Stokroos, Robert; Weber, Jacobiene W; van Tongeren, Joost

    2015-12-01

    To present the rare case of a young boy with idiopathic intracranial hypertension presenting with bilateral sensorineural hearing loss developing over several months. This was accompanied by headaches, otalgia, tinnitus, and vertigo. Furthermore, we aim to provide a concise review on this matter, as this report represents the second case in literature of pediatric idiopathic intracranial hypertension presenting with hearing loss. Workup of a 9-year-old boy with bilateral sensorineural hearing loss, including (among others) physical examination, audiometry, diagnostic imaging, and lumbar puncture. Physical examination including fundoscopy as well as imaging showed no abnormalities. At presentation, pure tone audiometry revealed bone conduction thresholds of about 30 dB HL in both ears. Two months later, this declined to about 35 dB HL in both ears. Lumbar puncture revealed an increased intracranial pressure. The boy was thus diagnosed with idiopathic intracranial hypertension. After the lumbar puncture, the otological complaints gradually resolved, and the hearing normalized (bone conduction thresholds of 0-5 dB HL). Although rare, sensorineural hearing loss in the pediatric population together with otalgia, tinnitus, and vertigo can be due to idiopathic intracranial hypertension and as such can be reversible. © The Author(s) 2015.

  12. Syphilis mimicking idiopathic intracranial hypertension

    DEFF Research Database (Denmark)

    Yri, Hanne; Wegener, Marianne; Jensen, Rigmor

    2011-01-01

    Idiopathic intracranial hypertension (IIH) is a condition of yet unknown aetiology affecting predominantly obese females of childbearing age. IIH is a diagnosis of exclusion as raised cerebrospinal fluid pressure may occur secondary to numerous other medical conditions. An atypical phenotype...... or an atypical disease course should alert the physician to reevaluate a presumed IIH-diagnosis. The authors report a case of a 32-year-old non-obese male with intracranial hypertension, secondary to a syphilitic central nervous system infection, initially misdiagnosed as being idiopathic. Upon relevant...

  13. Transient long thoracic nerve injury during posterior spinal fusion for adolescent idiopathic scoliosis: A report of two cases

    Directory of Open Access Journals (Sweden)

    Athanasios I Tsirikos

    2013-01-01

    Full Text Available We present the transient long thoracic nerve (LTN injury during instrumented posterior spinal arthrodesis for idiopathic scoliosis. The suspected mechanism of injury, postoperative course and final outcome is discussed. The LTN is susceptible to injury due to its long and relatively superficial course across the thoracic wall through direct trauma or tension. Radical mastectomies with resection of axillary lymph nodes, first rib resection to treat thoracic outlet syndrome and cardiac surgery can be complicated with LTN injury. LTN injury producing scapular winging has not been reported in association with spinal deformity surgery. We reviewed the medical notes and spinal radiographs of two adolescent patients with idiopathic scoliosis who underwent posterior spinal arthrodesis and developed LTN neuropraxia. Scoliosis surgery was uneventful and intraoperative spinal cord monitoring was stable throughout the procedure. Postoperative neurological examination was otherwise normal, but both patients developed winging of the scapula at 4 and 6 days after spinal arthrodesis, which did not affect shoulder function. Both patients made a good recovery and the scapular winging resolved spontaneously 8 and 11 months following surgery with no residual morbidity. We believe that this LTN was due to positioning of our patients with their head flexed, tilted and rotated toward the contralateral side while the arm was abducted and extended. The use of heavy retractors may have also applied compression or tension to the nerve in one of our patients contributing to the development of neuropraxia. This is an important consideration during spinal deformity surgery to prevent potentially permanent injury to the nerve, which can produce severe shoulder dysfunction and persistent pain.

  14. [Idiopathic rabbit syndrome: a case report].

    Science.gov (United States)

    Miwa, H; Sasaki, Y; Hatori, K; Tanaka, S; Mizuno, Y

    1999-10-01

    We report a patient with idiopathic oromandibular tremor resembling rabbit syndrome. The patient is a 36-year-old Japanese woman without any past and medical histories. On neurological examination, there was no abnormal finding except the oromandibular tremor. The tremor was confined to the jaw and perioral muscles. There was no extremity tremor. Laboratory findings were all normal, as well as her MRI and EEG. Surface EMG studies revealed that regular grouped discharges at a frequency of about 6 Hz appeared in the masseter, the orbicularis oris, and the digastric, and that the alternative contractions were found between the masseter and the digastric. Oral administration of tiapride was effective, but diazepam, trihexyphenydil, levodopa, and a beta-blocker were without effect. Although she had not taken neuroleptics, the appearance of the tremor was identical to the rabbit syndrome. The efficacy of the dopamine blockade may suggest that an abnormal basal ganglia function contributes to the pathophysiologic mechanism underlying this type of tremor.

  15. Idiopathic epileptic syndromes and cognition.

    Science.gov (United States)

    Hommet, Caroline; Sauerwein, Hannelore C; De Toffol, Bertrand; Lassonde, Maryse

    2006-01-01

    Epilepsy is frequently associated with cognitive impairments which result from various interacting factors. The present paper deals with the contribution of neuropsychology to the characterization of the type of epilepsy and the possible mechanisms underlying idiopathic epileptic syndromes. The non-lesional, so-called idiopathic epilepsies, constitute an interesting model for assessing the relationship between epileptiform EEG discharges and cognition. Among the idiopathic generalized epilepsies, disorders of social integration and personality have been frequently reported in juvenile myoclonic epilepsy (JME). Since similar disturbances are observed in frontal-lobe-lesioned patients, impairments in other frontal lobe functions (e.g. executive functions) might be expected in JME. This gives rise to speculation about the possible underlying pathophysiological mechanisms in JME. With regard to partial idiopathic epilepsies, benign childhood epilepsy with centrotemporal spikes (BCECTS) may provide a useful model for the study of the relationship between epileptiform EEG discharges in the peri-sylvian region and language functions. Furthermore, the description of mild cognitive dysfunctions in BCECTS, and their persistence into adulthood, can provide information about compensatory mechanisms and may allow for the generation of remedial strategies. Thus, 'lesional' neuropsychology has given way to 'dynamic' neuropsychology based on specific postulates. By using the cognitive profile to specify the mechanism underlying the behavioral disturbances observed in different types of epilepsy, neuropsychology may eventually contribute to a revision of the present classification of epileptic syndromes. In addition, the neuropsychological data may help predict the extent and limits of functional recovery and cerebral plasticity.

  16. Antioxidant therapy in idiopathic oligoasthenoteratozoospermia

    Directory of Open Access Journals (Sweden)

    Ahmad Majzoub

    2017-01-01

    Conclusion: Additional randomized controlled studies are required to confirm the efficacy and safety of antioxidant supplementation in the medical treatment of idiopathic male infertility as well as the dosage required to improve semen parameters, fertilization rates, and pregnancy outcomes in iOAT.

  17. Areas of normal pulmonary parenchyma on HRCT exhibit increased FDG PET signal in IPF patients

    International Nuclear Information System (INIS)

    Win, Thida; Thomas, Benjamin A.; Lambrou, Tryphon; Hutton, Brian F.; Endozo, Raymondo; Shortman, Robert I.; Afaq, Asim; Ell, Peter J.; Groves, Ashley M.; Screaton, Nicholas J.; Porter, Joanna C.; Maher, Toby M.; Lukey, Pauline

    2014-01-01

    Patients with idiopathic pulmonary fibrosis (IPF) show increased PET signal at sites of morphological abnormality on high-resolution computed tomography (HRCT). The purpose of this investigation was to investigate the PET signal at sites of normal-appearing lung on HRCT in IPF. Consecutive IPF patients (22 men, 3 women) were prospectively recruited. The patients underwent 18 F-FDG PET/HRCT. The pulmonary imaging findings in the IPF patients were compared to the findings in a control population. Pulmonary uptake of 18 F-FDG (mean SUV) was quantified at sites of morphologically normal parenchyma on HRCT. SUVs were also corrected for tissue fraction (TF). The mean SUV in IPF patients was compared with that in 25 controls (patients with lymphoma in remission or suspected paraneoplastic syndrome with normal PET/CT appearances). The pulmonary SUV (mean ± SD) uncorrected for TF in the controls was 0.48 ± 0.14 and 0.78 ± 0.24 taken from normal lung regions in IPF patients (p 18 F-FDG on PET in areas of lung with a normal morphological appearance on HRCT. This may have implications for determining disease mechanisms and treatment monitoring. (orig.)

  18. Areas of normal pulmonary parenchyma on HRCT exhibit increased FDG PET signal in IPF patients

    Energy Technology Data Exchange (ETDEWEB)

    Win, Thida [Lister Hospital, Respiratory Medicine, Stevenage (United Kingdom); Thomas, Benjamin A.; Lambrou, Tryphon; Hutton, Brian F.; Endozo, Raymondo; Shortman, Robert I.; Afaq, Asim; Ell, Peter J.; Groves, Ashley M. [University College London, Institute of Nuclear Medicine, University College Hospital, London (United Kingdom); Screaton, Nicholas J. [Papworth Hospital, Radiology Department, Papworth Everard (United Kingdom); Porter, Joanna C. [University College London, Centre for Respiratory Diseases, University College Hospital, London (United Kingdom); Maher, Toby M. [Royal Brompton Hospital, Interstitial Lung Disease Unit, London (United Kingdom); Lukey, Pauline [GSK, Fibrosis DPU, Research and Development, Stevenage (United Kingdom)

    2014-02-15

    Patients with idiopathic pulmonary fibrosis (IPF) show increased PET signal at sites of morphological abnormality on high-resolution computed tomography (HRCT). The purpose of this investigation was to investigate the PET signal at sites of normal-appearing lung on HRCT in IPF. Consecutive IPF patients (22 men, 3 women) were prospectively recruited. The patients underwent {sup 18}F-FDG PET/HRCT. The pulmonary imaging findings in the IPF patients were compared to the findings in a control population. Pulmonary uptake of {sup 18}F-FDG (mean SUV) was quantified at sites of morphologically normal parenchyma on HRCT. SUVs were also corrected for tissue fraction (TF). The mean SUV in IPF patients was compared with that in 25 controls (patients with lymphoma in remission or suspected paraneoplastic syndrome with normal PET/CT appearances). The pulmonary SUV (mean ± SD) uncorrected for TF in the controls was 0.48 ± 0.14 and 0.78 ± 0.24 taken from normal lung regions in IPF patients (p < 0.001). The TF-corrected mean SUV in the controls was 2.24 ± 0.29 and 3.24 ± 0.84 in IPF patients (p < 0.001). IPF patients have increased pulmonary uptake of {sup 18}F-FDG on PET in areas of lung with a normal morphological appearance on HRCT. This may have implications for determining disease mechanisms and treatment monitoring. (orig.)

  19. Locomotor skills and balance strategies in adolescents idiopathic scoliosis.

    Science.gov (United States)

    Mallau, Sophie; Bollini, Gérard; Jouve, Jean-Luc; Assaiante, Christine

    2007-01-01

    Locomotor balance control assessment was performed to study the effect of idiopathic scoliosis on head-trunk coordination in 17 patients with adolescent idiopathic scoliosis (AIS) and 16 control subjects. The aim of this study was to explore the functional effects of structural spinal deformations like idiopathic scoliosis on the balance strategies used during locomotion. Up to now, the repercussion of the idiopathic scoliosis on head-trunk coordination and balance strategies during locomotion is relatively unknown. Seventeen patients with AIS (mean age 14 years 3 months, 10 degrees 30 degrees) and 16 control subjects (mean age 14 years 1 month) were tested during various locomotor tasks: walking on the ground, walking on a line, and walking on a beam. Balance control was examined in terms of rotation about the vertical axis (yaw) and on a frontal plane (roll). Kinematics of foot, pelvis, trunk, shoulder, and head rotations were measured with an automatic optical TV image processor in order to calculate angular dispersions and segmental stabilizations. Decreasing the walking speed is the main adaptive strategy used in response to balance problems in control subjects as well as patients with AIS. However, patients with AIS performed walking tasks more slowly than normal subjects (around 15%). Moreover, the pelvic stabilization is preserved, despite the structural changes affecting the spine. Lastly, the biomechanical defect resulting from idiopathic scoliosis mainly affects the yaw head stabilization during locomotion. Patients with AIS show substantial similarities with control subjects in adaptive strategies relative to locomotor velocity as well as balance control based on segmental stabilization. In contrast, the loss of the yaw head stabilization strategies, mainly based on the use of vestibular information, probably reflects the presence of vestibular deficits in the patients with AIS.

  20. [Idiopathic facial paralysis in children].

    Science.gov (United States)

    Achour, I; Chakroun, A; Ayedi, S; Ben Rhaiem, Z; Mnejja, M; Charfeddine, I; Hammami, B; Ghorbel, A

    2015-05-01

    Idiopathic facial palsy is the most common cause of facial nerve palsy in children. Controversy exists regarding treatment options. The objectives of this study were to review the epidemiological and clinical characteristics as well as the outcome of idiopathic facial palsy in children to suggest appropriate treatment. A retrospective study was conducted on children with a diagnosis of idiopathic facial palsy from 2007 to 2012. A total of 37 cases (13 males, 24 females) with a mean age of 13.9 years were included in this analysis. The mean duration between onset of Bell's palsy and consultation was 3 days. Of these patients, 78.3% had moderately severe (grade IV) or severe paralysis (grade V on the House and Brackmann grading). Twenty-seven patients were treated in an outpatient context, three patients were hospitalized, and seven patients were treated as outpatients and subsequently hospitalized. All patients received corticosteroids. Eight of them also received antiviral treatment. The complete recovery rate was 94.6% (35/37). The duration of complete recovery was 7.4 weeks. Children with idiopathic facial palsy have a very good prognosis. The complete recovery rate exceeds 90%. However, controversy exists regarding treatment options. High-quality studies have been conducted on adult populations. Medical treatment based on corticosteroids alone or combined with antiviral treatment is certainly effective in improving facial function outcomes in adults. In children, the recommendation for prescription of steroids and antiviral drugs based on adult treatment appears to be justified. Randomized controlled trials in the pediatric population are recommended to define a strategy for management of idiopathic facial paralysis. Copyright © 2015 Elsevier Masson SAS. All rights reserved.

  1. MRI for clinically suspected pediatric appendicitis: case interpretation

    International Nuclear Information System (INIS)

    Moore, Michael M.; Brian, James M.; Methratta, Sosamma T.; Hulse, Michael A.; Choudhary, Arabinda K.; Eggli, Kathleen D.; Boal, Danielle K.B.

    2014-01-01

    As utilization of MRI for clinically suspected pediatric appendicitis becomes more common, there will be increased focus on case interpretation. The purpose of this pictorial essay is to share our institution's case interpretation experience. MRI findings of appendicitis include appendicoliths, tip appendicitis, intraluminal fluid-debris level, pitfalls of size measurements, and complications including abscesses. The normal appendix and inguinal appendix are also discussed. (orig.)

  2. MRI for clinically suspected pediatric appendicitis: case interpretation

    Energy Technology Data Exchange (ETDEWEB)

    Moore, Michael M.; Brian, James M.; Methratta, Sosamma T.; Hulse, Michael A.; Choudhary, Arabinda K.; Eggli, Kathleen D.; Boal, Danielle K.B. [Penn State Milton S. Hershey Medical Center, Division of Pediatric Radiology, Department of Radiology, Hershey, PA (United States)

    2014-05-15

    As utilization of MRI for clinically suspected pediatric appendicitis becomes more common, there will be increased focus on case interpretation. The purpose of this pictorial essay is to share our institution's case interpretation experience. MRI findings of appendicitis include appendicoliths, tip appendicitis, intraluminal fluid-debris level, pitfalls of size measurements, and complications including abscesses. The normal appendix and inguinal appendix are also discussed. (orig.)

  3. Idiopathic pulmonary fibrosis misdiagnosed as sputum-negative pulmonary tuberculosis.

    Science.gov (United States)

    Isah, Muhammad Danasabe; Abbas, Aminu; Abba, Abdullahi A; Umar, Mohammed

    2016-01-01

    Idiopathic pulmonary fibrosis (IPF), also known as cryptogenic fibrosing alveolitis, is one of a spectrum of idiopathic interstitial pneumonia. IPF is an increasingly common condition which poses many diagnostic and therapeutic challenges leading to misdiagnosis and mismanagement. We presented a 55-year-old male textile trader who was initially managed as sputum-negative pulmonary tuberculosis before histology report. He presented to our clinic with Breathlessness and cough of 3 years and 2.5 years, respectively. He had commenced anti-tuberculosis two months before presentation without significant relief. General Physical examination and vital signs were essentially normal. SPO2 was 96% on room air. Chest Examination revealed end-inspiratory bi-basal velcro-like crackles. Other systemic examinations were normal. Radiological examination by way of chest X- ray and chest CT showed features suggestive of IPF. The patient also had open Lung biopsy for histology and spirometry which demonstrated restrictive ventilatory function pattern. A diagnosis of Interstitial lung disease probably Idiopathic Pulmonary Fibrosis was entertained. He was commenced on Tab prednisolone, Tab Rabeprazole, with minimal improvement. IPF have often been misdiagnosed and treated as pulmonary tuberculosis with unfavorable outcome.

  4. Mineralogical microanalysis of idiopathic pulmonary fibrosis

    International Nuclear Information System (INIS)

    Monso, E.; Tura, J.M.; Marsal, M.; Morell, F.; Pujadas, J.; Morera, J.

    1990-01-01

    A mineralogical analysis of lung tissue was conducted on 25 samples from patients who had been diagnosed as having idiopathic pulmonary fibrosis (IPF). Scanning electron microscopy (SEM) at low magnification and energy-dispersive x-ray analysis (EDXA) was used. In all samples, the surface silicon/sulfur (Si/S) ratio was calculated. The Si/S ratio for 25 samples of normal lung and 6 samples of pneumoconiotic lung was also determined (upper limit of normal Si/S ratio = 0.3). The difference between the Si/S ratio in the group with IPF and group with normal lung tissue was significantly significant (p less than .007, Wilcoxon test). Six of 12 patients with a previous diagnosis of IPF and a Si/S ratio greater than 0.3 had an exposure history that could imply inhalation of silica/silicates, and the correct diagnosis for these patients is most probably pneumoconiosis. The silica/silicate deposits detected in patients with IPF, and who had a ratio and no past exposure to dusts, could be either a cause or an effect of the disease

  5. Mineralogical microanalysis of idiopathic pulmonary fibrosis

    Energy Technology Data Exchange (ETDEWEB)

    Monso, E.; Tura, J.M.; Marsal, M.; Morell, F.; Pujadas, J.; Morera, J. (Hospital Germans Trias i Pujol, Badalona (Spain))

    1990-05-01

    A mineralogical analysis of lung tissue was conducted on 25 samples from patients who had been diagnosed as having idiopathic pulmonary fibrosis (IPF). Scanning electron microscopy (SEM) at low magnification and energy-dispersive x-ray analysis (EDXA) was used. In all samples, the surface silicon/sulfur (Si/S) ratio was calculated. The Si/S ratio for 25 samples of normal lung and 6 samples of pneumoconiotic lung was also determined (upper limit of normal Si/S ratio = 0.3). The difference between the Si/S ratio in the group with IPF and group with normal lung tissue was significantly significant (p less than .007, Wilcoxon test). Six of 12 patients with a previous diagnosis of IPF and a Si/S ratio greater than 0.3 had an exposure history that could imply inhalation of silica/silicates, and the correct diagnosis for these patients is most probably pneumoconiosis. The silica/silicate deposits detected in patients with IPF, and who had a ratio and no past exposure to dusts, could be either a cause or an effect of the disease.

  6. Epidemiology of idiopathic pulmonary fibrosis

    Directory of Open Access Journals (Sweden)

    Ley B

    2013-11-01

    Full Text Available Brett Ley, Harold R Collard Department of Medicine, Division of Pulmonary and Critical Care Medicine, University of California San Francisco, San Francisco, California, USA Abstract: Idiopathic pulmonary fibrosis is a chronic fibrotic lung disease of unknown cause that occurs in adults and has a poor prognosis. Its epidemiology has been difficult to study because of its rarity and evolution in diagnostic and coding practices. Though uncommon, it is likely underappreciated both in terms of its occurrence (ie, incidence, prevalence and public health impact (ie, health care costs and resource utilization. Incidence and mortality appear to be on the rise, and prevalence is expected to increase with the aging population. Potential risk factors include occupational and environmental exposures, tobacco smoking, gastroesophageal reflux, and genetic factors. An accurate understanding of its epidemiology is important, especially as novel therapies are emerging. Keywords: idiopathic pulmonary fibrosis, epidemiology, incidence, prevalence, mortality, risk factors

  7. A case of idiopathic portalhypertension

    International Nuclear Information System (INIS)

    Serizawa, Ken; Yajima, Yoshiaki; Onodera, Hiroyoshi; Hirata, Toru; Sugawara, Hiroshi

    1982-01-01

    A 40-year-old man was referred to our clinic for esophageal varices. Histological examination of the liver biopsy samples revealed no sign of liver cirrhosis. Celiac angiography and ultrasound showed no obstruction of portal vein. A diagnosis of idiopathic portalhypertension was established. Splenomegaly and collateral circulation from spleen to left retroperitoneum were shown on CT scan and confirmed by surgical operation. CT scan following operation showed no collateral circulation. (author)

  8. Syphilis mimicking idiopathic intracranial hypertension

    DEFF Research Database (Denmark)

    Yri, Hanne; Wegener, Marianne; Jensen, Rigmor

    2011-01-01

    Idiopathic intracranial hypertension (IIH) is a condition of yet unknown aetiology affecting predominantly obese females of childbearing age. IIH is a diagnosis of exclusion as raised cerebrospinal fluid pressure may occur secondary to numerous other medical conditions. An atypical phenotype or a...... antibiotic treatment, signs and symptoms of elevated intracranial pressure resolved completely. Syphilis is a rare, but very important, differential diagnosis that in this case was clinically indistinguishable from IIH....

  9. Intestinal Volvulus in Idiopathic Steatorrhea

    Science.gov (United States)

    Warner, H. A.; Kinnear, D. G.; Cameron, D. G.

    1963-01-01

    Volvulus of the intestine has recently been observed in three patients with idiopathic steatorrhea in relapse. Two patients gave a history of intermittent abdominal pain, distension and obstipation. Radiographic studies during these attacks revealed obstruction at the level of the sigmoid colon. Reduction under proctoscopic control was achieved in one instance, spontaneous resolution occurring in the other. The third patient presented as a surgical emergency and underwent operative reduction of a small intestinal volvulus. Persistence of diarrhea and weight loss postoperatively led to further investigation and a diagnosis of idiopathic steatorrhea. In all cases, treatment resulted in clinical remission with a coincident disappearance of obstructive intestinal symptoms. The pathogenesis of volvulus in sprue is poorly understood. Atonicity and dilatation of the bowel and stretching of the mesentery likely represent important factors. The symptoms of recurrent abdominal pain and distension in idiopathic steatorrhea necessitate an increased awareness of intestinal volvulus as a complication of this disease. ImagesFig. 1Fig. 2Fig. 3Figs. 4 and 5Fig. 6 PMID:13998948

  10. Idiopathic great saphenous phlebosclerosis.

    Directory of Open Access Journals (Sweden)

    Ahmadreza Jodati

    2013-06-01

    Full Text Available Arterial sclerosis has been extensively described but reports on venous sclerosis are very sparse. Phlebosclerosis refers to the thickening and hardening of the venous wall. Despite its morphological similarities with arteriosclerosis and potential morbid consequences, phlebosclerosis has gained only little attention. We report a 72 year old male with paralysis and atrophy of the right leg due to childhood poliomyelitis who was referred for coronary artery bypass surgery. The great saphenous vein, harvested from the left leg, showed a hardened cord-like obliterated vein. Surprisingly, harvested veins from the atrophic limb were normal and successfully used for grafting.

  11. Suspected synthetic cannabinoid toxicosis in a dog.

    Science.gov (United States)

    Williams, Keysa; Wells, Raegan J; McLean, Mary Kay

    2015-01-01

    To describe the effects of suspected synthetic cannabinoid (SC) toxicosis and the response to intravenous lipid emulsion (ILE) therapy in a dog. A 2-year-8-month-old male Boxer dog was evaluated at an emergency hospital for progressive ataxia and inappropriate mentation. The initial physical examination identified marked hypothermia (32.7°C [90.9°F]), intermittent sinus bradycardia (60/min), stuporous mentation with intermittent aggression, and severe ataxia. Neurologic status deteriorated to comatose mentation within 2 hours of presentation. The initial diagnostic evaluation (eg, CBC, serum biochemistry profile, venous blood gas, and electrolyte determination) revealed a respiratory acidosis and thrombocytopenia. The owner reported that the dog was exposed to an SC containing Damiana leaf, Marshmallow leaf, and Athaea leaves. Initial treatment included IV fluids and supplemental oxygen. Mechanical ventilation was provided due to hypoventilation and periods of apnea. Intravenous lipid emulsion therapy was administered as a bolus (1.5 mL/kg) and continued as a continuous rate infusion (0.5 mL/kg/h) for a total of 6 hours. The dog became rousable and was weaned from mechanical ventilation approximately 15 hours following presentation. The dog was eating and walking with no ataxia, had a normal mentation at approximately 33 hours following presentation, and was discharged home at that time. Communication with the owners 5 days following discharge revealed that the dog was apparently normal. Based on this case and other reports in the literature regarding human exposures, SC ingestion may result in more severe clinical signs than marijuana ingestion in dogs. Significant clinical intervention may be necessary. Intravenous lipid emulsion treatment may be beneficial due to the lipophilicity of SC. © Veterinary Emergency and Critical Care Society 2015.

  12. Guidelines for identifying suspect/counterfeit material

    Energy Technology Data Exchange (ETDEWEB)

    NONE

    1995-09-01

    These guidelines are intended to assist users of products in identifying: substandard, misrepresented, or fraudulently marked items. The guidelines provide information about such topics as: precautions, inspection and testing, dispositioning identified items, installed inspection and reporting suspect/counterfeit materials. These guidelines apply to users who are developing procurement documents, product acceptance/verification methods, company procedures, work instructions, etc. The intent of these SM guidelines in relation to the Quality Assurance Program Description (QAPD) and implementing company Management Control Procedures is not to substitute or replace existing requirements, as defined in either the QAPD or company implementing instructions (Management Control Procedures). Instead, the guidelines are intended to provide a consolidated source of information addressing the issue of Suspect/Counterfeit materials. These guidelines provide an extensive suspect component listing and suspect indications listing. Users can quickly check their suspect items against the list of manufacturers products (i.e., type, LD. number, and nameplate information) by consulting either of these listings.

  13. Altered sensory-weighting mechanisms is observed in adolescents with idiopathic scoliosis

    Directory of Open Access Journals (Sweden)

    Allard Paul

    2006-10-01

    Full Text Available Abstract Background Scoliosis is the most common type of spinal deformity. In North American children, adolescent idiopathic scoliosis (AIS makes up about 90% of all cases of scoliosis. While its prevalence is about 2% to 3% in children aged between 10 to 16 years, girls are more at risk than boys for severe progression with a ratio of 3.6 to 1. The aim of the present study was to test the hypothesis that idiopathic scoliosis interferes with the mechanisms responsible for sensory-reweighting during balance control. Methods Eight scoliosis patients (seven female and one male; mean age: 16.4 years and nine healthy adolescents (average age 16.5 years participated in the experiment. Visual and ankle proprioceptive information was perturbed (eyes closed and/or tendon vibration suddenly and then returned to normal (eyes open and/or no tendon vibration. An AMTI force platform was used to compute centre of pressure root mean squared velocity and sway density curve. Results For the control condition (eyes open and no tendon vibration, adolescent idiopathic scoliosis patients had a greater centre of pressure root mean squared velocity (variability than control participants. Reintegration of ankle proprioception, when vision was either available or removed, led to an increased centre of pressure velocity variability for the adolescent idiopathic scoliosis patients whereas the control participants reduced their centre of pressure velocity variability. Moreover, in the absence of vision, adolescent idiopathic scoliosis exhibited an increased centre of pressure velocity variability when ankle proprioception was returned to normal (i.e. tendon vibration stopped. The analysis of the sway density plot suggests that adolescent idiopathic scoliosis patients, during sensory reintegration, do not scale appropriately their balance control commands. Conclusion Altogether, the present results demonstrate that idiopathic scoliosis adolescents have difficulty in

  14. Functional neuroimaging abnormalities in idiopathic generalized epilepsy

    Directory of Open Access Journals (Sweden)

    Megan L. McGill

    2014-01-01

    Full Text Available Magnetic resonance imaging (MRI techniques have been used to quantitatively assess focal and network abnormalities. Idiopathic generalized epilepsy (IGE is characterized by bilateral synchronous spike–wave discharges on electroencephalography (EEG but normal clinical MRI. Dysfunctions involving the neocortex, particularly the prefrontal cortex, and thalamus likely contribute to seizure activity. To identify possible morphometric and functional differences in the brains of IGE patients and normal controls, we employed measures of thalamic volumes, cortical thickness, gray–white blurring, fractional anisotropy (FA measures from diffusion tensor imaging (DTI and fractional amplitude of low frequency fluctuations (fALFF in thalamic subregions from resting state functional MRI. Data from 27 patients with IGE and 27 age- and sex-matched controls showed similar thalamic volumes, cortical thickness and gray–white contrast. There were no differences in FA values on DTI in tracts connecting the thalamus and prefrontal cortex. Functional analysis revealed decreased fALFF in the prefrontal cortex (PFC subregion of the thalamus in patients with IGE. We provide minimum detectable effect sizes for each measure used in the study. Our analysis indicates that fMRI-based methods are more sensitive than quantitative structural techniques for characterizing brain abnormalities in IGE.

  15. Osteoporosis in men with idiopathic hypogonadotropic hypogonadism

    International Nuclear Information System (INIS)

    Finkelstein, J.S.; Klibanski, A.; Neer, R.M.; Greenspan, S.L.; Rosenthal, D.I.; Crowley, W.F. Jr.

    1987-01-01

    To assess the effect of testosterone deficiency on skeletal integrity in men, we determined bone density in 23 hypogonadal men with isolated gonadotropin-releasing hormone deficiency and compared those values with ones from controls. Cortical bone density, as assessed by single-photon absorptiometry of the nondominant radius, ranged from 0.57 to 0.86 g/cm2 (mean +/- SE, 0.71 +/- 0.02) in patients with fused epiphyses and from 0.57 to 0.67 g/cm2 (mean, 0.61 +/- 0.01) in patients with open epiphyses, both of which were significantly (p less than 0.001) lower than normal. Spinal trabecular bone density, as assessed by computed tomography, was similarly decreased (p less than 0.0001) and ranged from 42 to 177 mg K2HPO4/cm3 (mean, 112 +/- 7). Cortical bone density was at least 2 SD below normal in 16 of 23 men, and 8 men had spinal bone densities below the fracture threshold of 80 to 100 mg K2HPO4/cm3. Osteopenia was equally severe in men with immature and mature bone ages, suggesting that abnormal bone development plays an important role in the osteopenia of men with idiopathic hypogonadotropic hypogonadism

  16. Auditory verbal memory and psychosocial symptoms are related in children with idiopathic epilepsy.

    Science.gov (United States)

    Schaffer, Yael; Ben Zeev, Bruria; Cohen, Roni; Shuper, Avinoam; Geva, Ronny

    2015-07-01

    Idiopathic epilepsies are considered to have relatively good prognoses and normal or near normal developmental outcomes. Nevertheless, accumulating studies demonstrate memory and psychosocial deficits in this population, and the prevalence, severity and relationships between these domains are still not well defined. We aimed to assess memory, psychosocial function, and the relationships between these two domains among children with idiopathic epilepsy syndromes using an extended neuropsychological battery and psychosocial questionnaires. Cognitive abilities, neuropsychological performance, and socioemotional behavior of 33 early adolescent children, diagnosed with idiopathic epilepsy, ages 9-14years, were assessed and compared with 27 age- and education-matched healthy controls. Compared to controls, patients with stabilized idiopathic epilepsy exhibited higher risks for short-term memory deficits (auditory verbal and visual) (pmemory deficits (plong-term memory deficits (pmemory deficits was related to severity of psychosocial symptoms among the children with epilepsy but not in the healthy controls. Results suggest that deficient auditory verbal memory may be compromising psychosocial functioning in children with idiopathic epilepsy, possibly underscoring that cognitive variables, such as auditory verbal memory, should be assessed and treated in this population to prevent secondary symptoms. Copyright © 2015 Elsevier Inc. All rights reserved.

  17. Addison's disease presenting with idiopathic intracranial hypertension in 24-year-old woman: a case report

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    Moore Peter

    2010-02-01

    Full Text Available Abstract Introduction Idiopathic intracranial hypertension can rarely be associated with an underlying endocrine disorder such as Cushing's syndrome, hyperthyroidism, or with administration of thyroxine or growth hormone. Though cases of idiopathic intracranial hypertension associated with Addison's disease in children have been reported, there is only one documented case report of this association in adults. We describe a case of an acute adrenal insufficiency precipitated by idiopathic intracranial hypertension in a Caucasian female. Case presentation A 24-year-old Caucasian woman was acutely unwell with a background of several months of generalised fatigue and intermittent headaches. She had unremarkable neurological and systemic examination with a normal computerised tomography and magnetic resonance imaging of the brain. Normal cerebrospinal fluid but increased opening pressure at lumbar puncture suggested intracranial hypertension. A flat short synacthen test and raised level of adrenocorticotrophic hormone were consistent with primary adrenal failure. Conclusion Addison's disease can remain unrecognised until precipitated by acute stress. This case suggests that idiopathic intracranial hypertension can rarely be associated with Addison's disease and present as an acute illness. Idiopathic intracranial hypertension is possibly related to an increase in the levels of arginine vasopressin peptide in serum and cerebrospinal fluid secondary to a glucocorticoid deficient state.

  18. Interventions for suspected placenta praevia.

    Science.gov (United States)

    Neilson, J P

    2003-01-01

    . Available data should, however, encourage further work to address the safety of more conservative policies of hospitalisation for women with suspected placenta praevia, and the possible value of insertion of a cervical suture.

  19. Clinical efficiency of cyclosporine in chronic idiopathic urticaria in adults

    Directory of Open Access Journals (Sweden)

    V.I. Petrov

    2010-06-01

    Full Text Available The purpose of the research is to evaluate the clinical effectiveness of cyclosporine and other antihistamines in patients with chronic forms of urticaria resistant to basic first-line therapy. Open randomized controlled study has been performed in parallel groups. 53 patients with chronic idiopathic urticaria ages 18-50 years have been examined. In case of ineffectiveness of previous therapy, patients have been randomized into 2 groups: group I receiving cyclosporine (Sandimmune Neoral ® 2,5 mg/kg/day, group II receiving cetirizine (Zyrtec ® 10 mg/day and ranitidine (Zantac ® 300 mg/day orally. It has been found that the administration of cyclosporine in patients with severe chronic idiopathic urticaria provides a more rapid achievement of clinical effect than the therapy with H1/H2 histamine antagonists. It is confirmed by a significant decrease of total index of severity of illness and major symptoms of skin lesions. This tendency towards normalization of quality of life of patients taking cyclosporine remains during 8 weeks after the medication. Thus administration of cyclosporine can be considered as therapy of choice in patients with chronic idio-pathic urticaria with a severe course and ineffective long-term therapy with antihistamines / systemic corticosteroids

  20. Coexistence of chronic renal failure, hashimoto thyroiditis and idiopathic hypoparathyroidism: a rare case report.

    Science.gov (United States)

    Yildiz, Saliha; Soyoral, Yasemin; Demirkiran, Davut; Ozturk, Mustafa

    2014-04-01

    Hypoparathyroidism is an uncommon disease and its coexistence with chronic renal failure is quite rare. Hypocalcemia and hyperphosphatemia are seen in both diseases. Diagnosis of hypoparathyroidism may be overlooked when parathormone response is not evaluated in patients with chronic renal failure. A 19-year-old female patient who had been receiving hemodialysis for 3 years because of chronic renal failure was diagnosed as idiopathic hypoparathyroidism and hashimoto thyroiditis. When her medical records on the first admission and medical history were evaluated, hypoparathyroidism and hashimoto thyroiditis were seen to be present also when she was started hemodialysis. Idiopathic hypoparathyroidism should be suspected in case as absence of parathormone response to hypocalcemia in patients with chronic renal failure. It should be taken into consideration that hashimoto thyroiditis may accompany and required analysis should be done.

  1. Genetics Home Reference: idiopathic pulmonary fibrosis

    Science.gov (United States)

    ... these health problems has idiopathic pulmonary fibrosis . Other respiratory diseases, some of which are less serious, can cause similar signs and symptoms. In people with idiopathic pulmonary fibrosis , scarring of the lungs increases over time until the lungs can no longer ...

  2. Idiopathic pulmonary fibrosis: current understanding of the pathogenesis and the status of treatment

    OpenAIRE

    Khalil, Nasreen; O'Connor, Robert

    2004-01-01

    IDIOPATHIC PULMONARY FIBROSIS (IPF) is a progressive and lethal pulmonary fibrotic lung disease. The diagnostic histological changes are called usual interstitial pneumonia and are characterized by histological temporal heterogeneity, whereby normal lung tissue is interspersed with interstitial fibrosis, honeycomb cysts and fibroblast foci. Pulmonary functions show restricted volumes and capacities, preserved flows and evidence of decreased gas exchange. High-resolution computed axial tomogra...

  3. Anterior lamina cribrosa surface position in idiopathic intracranial hypertension and glaucoma

    DEFF Research Database (Denmark)

    Villarruel, Jenni Martinez; Li, Xiao Q.; Bach-Holm, Daniella

    2017-01-01

    Purpose: To compare the anterior lamina cribrosa (LC) surface position in patients with idiopathic intracranial hypertension (IIH), primary open-angle glaucoma (high-tension glaucoma [HTG] and normal-tension glaucoma [NTG]), and healthy controls using enhanced depth imaging spectral-domain optical...

  4. Idiopathic focal epilepsies: the "lost tribe".

    Science.gov (United States)

    Pal, Deb K; Ferrie, Colin; Addis, Laura; Akiyama, Tomoyuki; Capovilla, Giuseppe; Caraballo, Roberto; de Saint-Martin, Anne; Fejerman, Natalio; Guerrini, Renzo; Hamandi, Khalid; Helbig, Ingo; Ioannides, Andreas A; Kobayashi, Katsuhiro; Lal, Dennis; Lesca, Gaetan; Muhle, Hiltrud; Neubauer, Bernd A; Pisano, Tiziana; Rudolf, Gabrielle; Seegmuller, Caroline; Shibata, Takashi; Smith, Anna; Striano, Pasquale; Strug, Lisa J; Szepetowski, Pierre; Valeta, Thalia; Yoshinaga, Harumi; Koutroumanidis, Michalis

    2016-09-01

    epigenetic factors involved that might also explain low observed twin concordance? The genetic (and epigenetic) models for different IFEs, their comorbidities, and their similarities to other neurodevelopmental disorders deserve investigation in the coming years. In so doing, we will probably learn much about normal brain functioning. This is because these disorders, perhaps more than any other human brain disease, are disorders of functional brain systems (even though these functional networks may not yet be fully defined). In June 2012, an international group of clinical and basic science researchers met in London under the auspices of the Waterloo Foundation to discuss and debate these issues in relation to IFEs. This Waterloo Foundation Symposium on the Idiopathic Focal Epilepsies: Phenotype to Genotype witnessed presentations that explored the clinical phenomenology, phenotypes and endophenotypes, and genetic approaches to investigation of these disorders. In parallel, the impact of these epilepsies on children and their families was reviewed. The papers in this supplement are based upon these presentations. They represent an updated state-of-the-art thinking on the topics explored. The symposium led to the formation of international working groups under the umbrella of "Luke's Idiopathic Focal Epilepsy Project" to investigate various aspects of the idiopathic focal epilepsies including: semiology and classification, genetics, cognition, sleep, high-frequency oscillations, and parental resources (see www.childhood-epilepsy.org). The next sponsored international workshop, in June 2014, was on randomised controlled trials in IFEs and overnight learning outcome measures.

  5. Idiopathic pulmonary fibrosis: evolving concepts.

    Science.gov (United States)

    Ryu, Jay H; Moua, Teng; Daniels, Craig E; Hartman, Thomas E; Yi, Eunhee S; Utz, James P; Limper, Andrew H

    2014-08-01

    Idiopathic pulmonary fibrosis (IPF) occurs predominantly in middle-aged and older adults and accounts for 20% to 30% of interstitial lung diseases. It is usually progressive, resulting in respiratory failure and death. Diagnostic criteria for IPF have evolved over the years, and IPF is currently defined as a disease characterized by the histopathologic pattern of usual interstitial pneumonia occurring in the absence of an identifiable cause of lung injury. Understanding of the pathogenesis of IPF has shifted away from chronic inflammation and toward dysregulated fibroproliferative repair in response to alveolar epithelial injury. Idiopathic pulmonary fibrosis is likely a heterogeneous disorder caused by various interactions between genetic components and environmental exposures. High-resolution computed tomography can be diagnostic in the presence of typical findings such as bilateral reticular opacities associated with traction bronchiectasis/bronchiolectasis in a predominantly basal and subpleural distribution, along with subpleural honeycombing. In other circumstances, a surgical lung biopsy may be needed. The clinical course of IPF can be unpredictable and may be punctuated by acute deteriorations (acute exacerbation). Although progress continues in unraveling the mechanisms of IPF, effective therapy has remained elusive. Thus, clinicians and patients need to reach informed decisions regarding management options including lung transplant. The findings in this review were based on a literature search of PubMed using the search terms idiopathic pulmonary fibrosis and usual interstitial pneumonia, limited to human studies in the English language published from January 1, 2000, through December 31, 2013, and supplemented by key references published before the year 2000. Copyright © 2014 Mayo Foundation for Medical Education and Research. Published by Elsevier Inc. All rights reserved.

  6. Detection of sex hormone in serum and semen of patients with idiopathic oligospermia and its significance

    International Nuclear Information System (INIS)

    Wang Guohong; Xu Ruiji; Zhang Zhongshu; Wang Youji

    2005-01-01

    The objective of this study was to detect testosterone(T), free testosterone (FT) and sex hormone-binding globulin(SHBG) in serum and semen of patients with idiopathic oligospermia, and further analyze the relationship between T, FT, SHBG and idiopathic oligospermia. Blood and semen samples were collected from males of a normal control group and an idiopathic oligospermia group at 8:00-10:00 am. The sperm density in semen was detected by routine semen analysis, while T, FT, SHBG in serum and semen were detected by RIA. There were no significant differences in serum concentrations of T, FT, SHBG between normal control group [(30.03±13.07)nmol/L,(97.50±46.96)pmol/L, (40.37±16.73)nmol/L, respectively] and idiopathic oligspermia group [(28.11±11.54) nmol/L, (94.88±42.04) pmol/L, (41.61± 18.86)noml/LJ(all P>0. 05). There were significant differences in semsn concentrations of FT and SHBG between normal control group[(2.01±0.32) pmol/L, (0.17±0.21)nmol/L] and idiopathic oligspermia group [ (0.52±0.44) pmol/L, (0.22±0.15) nmol/LJ (P 0.05). Therefore, measurement of semsn FT, SHBG concentration could early reflect the function of tesis, which is useful for early diagnosis and treatment of idiopathic oligospermia. (authors)

  7. Detection of equine herpesvirus in horses with idiopathic keratoconjunctivitis and comparison of three sampling techniques.

    Science.gov (United States)

    Hollingsworth, Steven R; Pusterla, Nicola; Kass, Philip H; Good, Kathryn L; Brault, Stephanie A; Maggs, David J

    2015-09-01

    To determine the role of equine herpesvirus (EHV) in idiopathic keratoconjunctivitis in horses and to determine whether sample collection method affects detection of EHV DNA by quantitative polymerase chain reaction (qPCR). Twelve horses with idiopathic keratoconjunctivitis and six horses without signs of ophthalmic disease. Conjunctival swabs, corneal scrapings, and conjunctival biopsies were collected from 18 horses: 12 clinical cases with idiopathic keratoconjunctivitis and six euthanized controls. In horses with both eyes involved, the samples were taken from the eye judged to be more severely affected. Samples were tested with qPCR for EHV-1, EHV-2, EHV-4, and EHV-5 DNA. Quantity of EHV DNA and viral replicative activity were compared between the two populations and among the different sampling techniques; relative sensitivities of the sampling techniques were determined. Prevalence of EHV DNA as assessed by qPCR did not differ significantly between control horses and those with idiopathic keratoconjunctivitis. Sampling by conjunctival swab was more likely to yield viral DNA as assessed by qPCR than was conjunctival biopsy. EHV-1 and EHV-4 DNA were not detected in either normal or IKC-affected horses; EHV-2 DNA was detected in two of 12 affected horses but not in normal horses. EHV-5 DNA was commonly found in ophthalmically normal horses and horses with idiopathic keratoconjunctivitis. Because EHV-5 DNA was commonly found in control horses and in horses with idiopathic keratoconjunctivitis, qPCR was not useful for the etiological diagnosis of equine keratoconjunctivitis. Conjunctival swabs were significantly better at obtaining viral DNA samples than conjunctival biopsy in horses in which EHV-5 DNA was found. © 2015 American College of Veterinary Ophthalmologists.

  8. Idiopathic thrombocytopenic purpura during pregnancy

    Directory of Open Access Journals (Sweden)

    Tânia Regina Padovani

    2012-04-01

    Full Text Available ABSTRACT This essay is based on a medical case of idiopathic thrombocytopenic purpura (ITP during pregnancy. The cause of ITP is unknown, who suffer from this disorder, generate antibodies that destroy thrombocytes from their blood. ITP affects women of childbearing age and is associated to maternal and fetal complications. The management of a pregnant patient is difficult and requires the combined care of an obstetrician, a hematologist, and a neonatologist. The main therapeutic options for ITP in pregnant women include glucocorticoids and intravenous immunoglobulin. Splenectomy may be (performed in refractory cases. There is no concerning the management and treatment of pregnant women.

  9. Wind turbines and idiopathic symptoms

    DEFF Research Database (Denmark)

    Blanes-Vidal, Victoria; Schwartz, Joel

    2016-01-01

    Whether or not wind turbines pose a risk to human health is a matter of heated debate. Personal reactions to other environmental exposures occurring in the same settings as wind turbines may be responsible of the reported symptoms. However, these have not been accounted for in previous studies. We...... investigated whether there is an association between residential proximity to wind turbines and idiopathic symptoms, after controlling for personal reactions to other environmental co-exposures. We assessed wind turbine exposures in 454 residences as the distance to the closest wind turbine (Dw) and number...... of wind turbines

  10. Colon transit scintigraphy by 67 Ga citrate for idiopathic constitution

    International Nuclear Information System (INIS)

    Neshandar Asll, I.; Ehsani, M.J.; Javadi, H.

    2005-01-01

    Background/objective: segmental colonic transit studies are important in patients with severe constipation. This study is the first Iranian preliminary survey of colonic transit scintigraphy using 67 Ga -citrate as a new method in constipated patients with normal radiographic and colonoscopic evaluations. Patients and methods: thirteen patients with idiopathic constipation underwent colon transit scintigraphy. After oral administration of 6-7 MBq Ga-citrates, serial abdominal images were taken up to 72 hours. Pattern classification wa s performed visually according to the distribution of radioactivity, Scintigraphic parameters such as geometric mean center of seq mental retention of tracer, as well as mean ac activity profiles and colonic tracer half-clearance time were calculated Results: Three patterns of colonic transit scintigraphy were recognized. Nine patients had the normal pattern, i.e. excellent propagation of ac activity. Three patients had the colonic inertia pattern with marked retention of activity in the transverse colon and splenic flexure at 48 hours, One patient had significant retention of activity in the recto sigmoid at 72 hours, defined as functional recto sigmoid obstruction . No significant difference was seen in GMC24h between the normal pattern and colonic inertia (P4.053), but GMC48h and GMC72h markedly differed between the two groups (P50.0 16 and 0.025 respectively). 'The mean half clearance time of the two groups was di different (P4.017). Our results are well compatible with scintigraphic diagnostic criteria in different patterns of colonic transit defined by other studies with different radiotracer. Conclusion: oral 67 Ga -citrate colon transit scintigraphy is a feasible method to evaluate idiopathic constipation and seems to be a suitable surrogate for radio-opaque markers. Keywords: oral 67 Ga -citrate, colonic transit study, idiopathic constipation, scintigraphy

  11. Handbook for Response to Suspect Radioactive Materials

    International Nuclear Information System (INIS)

    Cliff, William C.; Pappas, Richard A.; Arthur, Richard J.

    2005-01-01

    This document provides response actions to be performed following the initial port, airport, or border crossing discovery of material that is suspected of being radioactive. The purpose of this guide is to provide actions appropriate for handling radioactive material

  12. Clinical Case of Tocilizumab Use in a Patient with Systemic Juvenile Idiopathic Arthritis

    Directory of Open Access Journals (Sweden)

    Y. M. Spivakovskiy

    2015-01-01

    Full Text Available The article presents a case of using genetically engineered biopharmaceutical tocilizumab in a child with systemic juvenile idiopathic arthritis. On the initial stage, the treatment was characterized by resistance to high doses of glucocorticoids and cytostatic drugs. Successful termination of visceral and articular manifestations of systemic juvenile idiopathic arthritis and normalization of laboratory indicators of disease activity in the setting of use of interleukin 6 receptor blocker were described. We observed stable improvement of the child’s condition during a year-long follow-up in the setting of the selected anti-inflammatory therapy pattern. 

  13. Comparison of clinically suspected injuries with injuries detected at whole-body CT in suspected multi-trauma victims

    International Nuclear Information System (INIS)

    Shannon, L.; Peachey, T.; Skipper, N.; Adiotomre, E.; Chopra, A.; Marappan, B.; Kotnis, N.

    2015-01-01

    Aim: To assess the accuracy of the trauma team leader's clinical suspicion of injury in patients who have undergone whole-body computed tomography (WBCT) for suspected polytrauma, and to assess the frequency of unsuspected injuries and specific patterns of injury at WBCT. Materials and methods: Requests for patients who underwent WBCT for suspected polytrauma from April 2011 to March 2014 were reviewed and each body area that was clinically suspected to be injured was recorded. Body areas were divided into the following groups based on anatomical regions covered in each segment of the WBCT examination: head (including facial bones); neck (including cervical spine); chest (including thoracic spine); and abdomen/pelvis (including lumbar spine). The formal radiology report for each study was reviewed and injuries found at CT were grouped into the same body areas. For each patient, the number of clinically suspected injured areas was compared to the number of confirmed injured areas at WBCT. Results: Five hundred and eighty-eight patients were included in the study. Thirty-two percent (186/588) had a normal scan. Ninety-three percent (546/588) had fewer injured body areas at WBCT than suspected. Four percent (27/588) had the equivalent number of injured areas at WBCT as suspected. Three percent (15/588) had more injured areas at WBCT than suspected. Fifty percent (263/527) with clinically suspected chest injuries were confirmed to have chest injuries at WBCT. This was lower for other areas: abdomen/pelvis 31% (165/535); head 29% (155/533); neck 13% (66/513). Four percent of (24/588) patients had unsuspected injuries found at WBCT. Seventy-five percent (18/24) of unsuspected injuries were considered as serious, where failure to treat would have the potential for significant morbidity. Most of these patients had severe injuries to other body areas that were correctly suspected. Of the 165 with abdominal/pelvic region injuries, there were associated injuries in the

  14. MRI of idiopathic orbital inflammation and lymphoid disease with lesions in extraocular muscle

    International Nuclear Information System (INIS)

    Matsuda, Chiharu; Kotake, Fumio; Kawanishi, Masayuki; Saito, Kazuhiro; Abe, Kimihiko

    2004-01-01

    Of the disorders accompanied by hypertrophy of the extraocular muscles, differentiating between idiopathic orbital inflammation and malignant lymphoma is difficult but important to treatment and prognosis. In this study using MRI, shape, signal intensity, and enhancement effects were compared between idiopathic orbital inflammation and lymphoproliferative lesions. The subjects were 27 patients (8 with idiopathic orbital inflammation, 1 with reactive lymphoid hyperplasia, 3 with atypical lymphoid hyperplasia, and 15 with malignant lymphoma) and 10 normal controls. The evaluation items were: thickness of extraocular muscles, number of extraocular muscles involved signal intensity of extraocular muscles, and enhancement effects on extraocular muscles. When compared to control subjects (p<0.05) the attachment portion of extraocular muscles were significantly thicker in the patients with idiopathic orbital inflammation, atypical lymphoid hyperplasia, or malignant lymphoma; the most marked hypertrophy was observed in patients with malignant lymphoma. The number of extraocular muscles involved was 1.5 (mean) in the patients with idiopathic orbital inflammation, 1 in the patient with reactive lymphoid hyperplasia, 1.7 (mean) in the patients with atypical lymphoid hyperplasia, and 5.1 (mean) in those with malignant lymphoma. The signal intensity ratio on T1W-images did not significantly differ between the patients and controls for all the disorders investigated. Signal intensity ratio on T2W-images significantly differed between patients with atypical lymphoid hyperplasia or malignant lymphoma and the controls (p<0.05) but not between patients with idiopathic orbital inflammation and controls. Signal intensity ratio after contrast enhancement differed significantly only between patients with idiopathic orbital inflammation and controls (p<0.05). (author)

  15. Atherosclerosis in Juvenile Idiopathic Arthritis

    Directory of Open Access Journals (Sweden)

    Ewa Jednacz

    2012-01-01

    Full Text Available Atherosclerosis is a chronic inflammatory disease of the arteries. Clinical consequences of the atherosclerotic process occur in the adult population, however atherosclerotic process begins in childhood. The classic risk factors for atherosclerosis include obesity, dyslipidaemia, age, gender or family history. In recent years, attention has been drawn to the similarity between atherosclerotic inflammatory processes and inflammatory changes in the course of systemic connective tissue disease, in particular systemic lupus etythematosus (SLE or rheumatoid arthritis (RA. There is also observed the similarity of the pathogenetic background of development of atherosclerosis and juvenile idiopathic arthritis (JIA. Elevated levels of pro-inflammatory cytokines are observed in the course of juvenile idiopathic arthritis. Also homocysteine concentrations, which may play a significant role in the development of atherosclerotic lesions, are observed higher in patients with JIA. Some studies revealed higher carotid intima-media thickness (IMT index values in children with JIA. In view of the fact that atherosclerotic process begins as early as in childhood, the introduction of appropriate preventive measures in children is a matter of utmost importance.

  16. Thoracic computed tomography in patients with suspected malignant pleural effusions

    International Nuclear Information System (INIS)

    Traill, Zoee C.; Davies, Robert J.O.; Gleeson, Fergus V.

    2001-01-01

    AIM: To assess the role of contrast-enhanced computed tomography (CT) prospectively in patients with suspected malignant pleural effusions. MATERIALS AND METHODS: Forty consecutive patients referred for the investigation of a suspected malignant pleural effusion had contrast-enhanced thoracic CT, thoracoscopy, thoraco-centesis and pleural biopsy, either percutaneously or at thoracoscopy. Final diagnoses were based on histopathological or cytological analysis (n = 30), autopsy findings (n = 3) or clinical follow-up (n = 7). The pleural surfaces were classified at contrast-enhanced CT as normal or abnormal and, if abnormal, as benign or malignant in appearance using previously established CT criteria for malignant pleural thickening by two observers unaware of the pathological diagnosis. RESULTS: Pleural effusions were malignant in 32 patients and benign in eight patients. Pleural surfaces assessed at CT showed features of malignancy in 27 out of 32 patients with a malignant effusion (sensitivity 84%, specificity 100%). Overall, CT appearances indicated the presence of malignancy in 28 of 32 (87%) patients. All eight patients with benign pleural disease were correctly diagnosed by CT. CONCLUSION: Contrast-enhanced CT is of value in patients with suspected malignant pleural effusions. The previously established criteria for malignant pleural thickening of nodularity, irregularity and pleural thickness >1 cm are reliable in the presence of a pleural effusion. Traill, Z.C. et al. (2001)

  17. Idiopathic intracranial hypertension: A typical presentation

    International Nuclear Information System (INIS)

    Algahtani, Hussein A.; Obeid, Tahir H.; Abuzinadah, Ahmad R.; Baeesa, Saleh S.

    2007-01-01

    Objective was to describe the clinical features of 5 patients with rare atypical presentation of idiopathic intracranial hypertension (IIH), and propose the possible mechanism of this atypical presentation. We carried out a retrospective study of 5 patients, admitted at King Khalid National Guard Hospital, Jeddah, Kingdom of Saudi Arabia with IIH during the period from January 2001 to December 2005. All were females with their age ranges from 24 to 40 years. The clinical presentations, the laboratory and imaging studies were analyzed. The opening pressures of the lumbar puncture tests were documented. All patients were presented with headache. One had typical pain of trigeminal neuralgia and one with neck pain and radiculopathy. Facial diplegia was present in one patient and two patients had bilateral 6th cranial neuropathy. Papilledema was present in all patients except in one patient. Imaging study was normal in all patients, and they had a very high opening pressure during lumbar puncture, except in one patient. All patients achieved full recovery with medical therapy in 6 to 12 weeks with no relapse during the mean follow up of 2 years. Atypical finding in IIH are rare and require a high index of suspicion for early diagnosis. (author)

  18. Lymphatics in lymphangioleiomyomatosis and idiopathic pulmonary fibrosis

    Directory of Open Access Journals (Sweden)

    Souheil El-Chemaly

    2012-09-01

    Full Text Available The primary function of the lymphatic system is absorbing and transporting macromolecules and immune cells to the general circulation, thereby regulating fluid, nutrient absorption and immune cell trafficking. Lymphangiogenesis plays an important role in tissue inflammation and tumour cell dissemination. Lymphatic involvement is seen in lymphangioleiomyomatosis (LAM and idiopathic pulmonary fibrosis (IPF. LAM, a disease primarily affecting females, involves the lung (cystic destruction, kidney (angiomyolipoma and axial lymphatics (adenopathy and lymphangioleiomyoma. LAM occurs sporadically or in association with tuberous sclerosis complex (TSC. Cystic lung destruction results from proliferation of LAM cells, which are abnormal smooth muscle-like cells with mutations in the TSC1 or TSC2 gene. Lymphatic abnormalities arise from infiltration of LAM cells into the lymphatic wall, leading to damage or obstruction of lymphatic vessels. Benign appearing LAM cells possess metastatic properties and are found in the blood and other body fluids. IPF is a progressive lung disease resulting from fibroblast proliferation and collagen deposition. Lymphangiogenesis is associated with pulmonary destruction and disease severity. A macrophage subset isolated from IPF bronchoalveolar lavage fluid (BALF express lymphatic endothelial cell markers in vitro, in contrast to the same macrophage subset from normal BALF. Herein, we review lymphatic involvement in LAM and IPF.

  19. Positron emission tomography suggests that the rate of progression of idiopathic parkinsonism is slow

    International Nuclear Information System (INIS)

    Bhatt, M.H.; Snow, B.J.; Martin, W.R.; Pate, B.D.; Ruth, T.J.; Calne, D.B.

    1991-01-01

    The authors performed sequential positron emission tomography scans with 6-[18F]fluoro-L-dopa in 9 patients with idiopathic parkinsonism and 7 age-matched normal control subjects to compare changes in the nigrostriatal dopaminergic pathway over time. The mean interval between the scans was 3.3 years for the group with idiopathic parkinsonism and 3.9 years for the control subjects. The scans were analyzed by calculating the ratio of striatal to background radioactivity. Both groups showed statistically significant reductions of striatal uptake over the interval. The rate of decrease was almost identical in each group (p = 0.6). They infer that the usual rate of loss of integrity of the dopaminergic nigrostriatal pathway in patients with idiopathic parkinsonism is slow and the rate of change between the two groups was comparable

  20. Total spine and posterior fossa MRI screening in adolescent idiopathic scoliosis (177 cases

    Directory of Open Access Journals (Sweden)

    MR Etemadifar

    2005-05-01

    Full Text Available Background: MRI screening for idiopathic scoliosis is controversial. Considering our clinical experiences, the results of MRI in all patients with idiopathic scoliosis were evaluated. Methods: In a prospective clinical study, all neurologically normal patients with idiopathic scoliosis screened by posterior fossa and total spine MRI. Results: After excluding 9 patients for mild neurological findings, in other 177 patients (132 female, 45 male, the average age and curve angle was 15±2 years and 59±17º (30 to 135º, respectively. Convexity was to right in 146 and to left in 31 cases. MRI was positive in 12 cases (6.8%. In 5 cases (2.8%, neurosurgical intervention was necessary prior to scoliosis surgery. There was no relation between age, sex, presence of pain or curve angle and positive MRI findings (P>0.05. Left convexity was significantly related to positive MRI findings (P=0.013. In males with left convex curves, the probability of positive MRI findings was 8.8 folds other patients. Conclusion: Considering our results and other reported articles, it seems that routine MRI screening of all patients presenting as idiopathic scoliosis is necessary for detection of underlying pathologies. Key words: Idiopathic Scoliosis, MRI, Spine Syrinx, Chiari

  1. [Regulation of airway stem cell proliferation in idiopathic pulmonary fibrosis].

    Science.gov (United States)

    Yang, S X; Wu, Q; Sun, X; Li, X; Li, K; Xu, L; Li, Y; Zhang, Q Y; Zhang, Y C; Chen, H Y

    2016-09-01

    To investigate the effect of fibroblasts on regulating airway stem cell proliferation in idiopathic pulmonary fibrosis. Lung cell suspension was prepared from β-actin-GFP mice. Airway stem cells were obtained by fluorescence activated cell sorting and co-cultured with lung fibroblasts. The fibroblasts were treated with TGF-β inhibitor SB43142. The expression of growth factors FGF1/2 and the effect of FGF1/2 on stem cell proliferation were observed. The cloning efficiency of airway stem cells, when co-cultured with normal lung fibroblast cells for 8 days, was (3.5±1.1)%, while the cloning efficiency was reduced to (0.04±0.04)% when co-cultured with lung fibroblasts from idiopathic pulmonary fibrosis patients. The difference between the 2 groups was statistically significant(P=0.002 5). TGF-β receptor inhibitor SB431542 increased lung fibroblast growth factors FGF1/2 expression.FGF1 mRNA expression was increased to the experimental group 0.005 5 from 0.000 2 in the control group.FGF2 mRNA expression of the amount raised to the experimental group 0.000 15 from 0.000 8 in the control group.FGF1/2 promoted the growth of airway stem cells. After FGF1/2 was co-cultured with normal lung fibroblast cells for 8 days, the cloning efficiency of airway stem cells was (0.3±0.1)%. During the development of idiopathic pulmonary fibrosis, fibroblast secreted FGF1/2 regulate airway stem cell proliferation.

  2. [Physiotherapy for juvenile idiopathic arthritis].

    Science.gov (United States)

    Spamer, M; Georgi, M; Häfner, R; Händel, H; König, M; Haas, J-P

    2012-07-01

    Control of disease activity and recovery of function are major issues in the treatment of children and adolescents suffering from juvenile idiopathic arthritis (JIA). Functional therapies including physiotherapy are important components in the multidisciplinary teamwork and each phase of the disease requires different strategies. While in the active phase of the disease pain alleviation is the main focus, the inactive phase requires strategies for improving motility and function. During remission the aim is to regain general fitness by sports activities. These phase adapted strategies must be individually designed and usually require a combination of different measures including physiotherapy, occupational therapy, massage as well as other physical procedures and sport therapy. There are only few controlled studies investigating the effectiveness of physical therapies in JIA and many strategies are derived from long-standing experience. New results from physiology and sport sciences have contributed to the development in recent years. This report summarizes the basics and main strategies of physical therapy in JIA.

  3. Cough in idiopathic pulmonary fibrosis

    Directory of Open Access Journals (Sweden)

    Mirjam J.G. van Manen

    2016-09-01

    Full Text Available Many patients with idiopathic pulmonary fibrosis (IPF complain of chronic refractory cough. Chronic cough is a distressing and disabling symptom with a major impact on quality of life. During recent years, progress has been made in gaining insight into the pathogenesis of cough in IPF, which is most probably “multifactorial” and influenced by mechanical, biochemical and neurosensory changes, with an important role for comorbidities as well. Clinical trials of cough treatment in IPF are emerging, and cough is increasingly included as a secondary end-point in trials assessing new compounds for IPF. It is important that such studies include adequate end-points to assess cough both objectively and subjectively. This article summarises the latest insights into chronic cough in IPF. It describes the different theories regarding the pathophysiology of cough, reviews the different methods to assess cough and deals with recent and future developments in the treatment of cough in IPF.

  4. Pathogenesis of Idiopathic Pulmonary Fibrosis

    Science.gov (United States)

    Wolters, Paul J.; Collard, Harold R.; Jones, Kirk D.

    2014-01-01

    Idiopathic pulmonary fibrosis (IPF) is a fibrosing interstitial lung disease associated with aging that is characterized by the histopathological pattern of usual interstitial pneumonia. Although an understanding of the pathogenesis of IPF is incomplete, recent advances delineating specific clinical and pathologic features of IPF have led to better definition of the molecular pathways that are pathologically activated in the disease. In this review we highlight several of these advances, with a focus on genetic predisposition to IPF and how genetic changes, which occur primarily in epithelial cells, lead to activation of profibrotic pathways in epithelial cells. We then discuss the pathologic changes within IPF fibroblasts and the extracellular matrix, and we conclude with a summary of how these profibrotic pathways may be interrelated. PMID:24050627

  5. Idiopathic Inflammatory Myopathies: An update

    Directory of Open Access Journals (Sweden)

    Bulent KURT

    2016-06-01

    Full Text Available Idiopathic inflammatory myopathies (IIM are a heterogeneous group of disease with complex clinical features. It has been sub-classified as: (1 Dermatomyositis, (2 Polymyositis, and (3 Inclusion body myositis (IBM. Nowadays, there are some studies in literature suggest necrotizing autoimmune myopathy and immune-mediated necrotizing myopathy should also be added to this group of disease. There is a debate in the diagnosis of IIMs and up until now, about 12 criteria systems have been proposed. Some of the criteria systems have been used widely such as Griggs et al.'s proposal for IBM. Clinical findings, autoantibodies, enzymes, electrophysiological, and muscle biopsy findings are diagnostic tools. Because of diseases' complexity, none of the findings are diagnostic alone. In this study, we discussed the diagnostic criteria of IMMs and described detailed morphological features. [J Interdiscipl Histopathol 2016; 4(2.000: 41-45

  6. Fahr's syndrome - Idiopathic Bilateral Striopallidodentate Calcinosis ...

    African Journals Online (AJOL)

    Log in or Register to get access to full text downloads. ... Fehr's disease, also known as Idiopathic Calcification of the Basal Ganglia (ICBG) or ... ferrocalcinosis (and many others), is a rare sporadic or familial neurological disorder whose ...

  7. Genetics Home Reference: idiopathic inflammatory myopathy

    Science.gov (United States)

    ... stumble while walking and find it difficult to grasp items. As in dermatomyositis and polymyositis, swallowing can ... and development? More about Mutations and Health Inheritance Pattern Most cases of idiopathic inflammatory myopathy are sporadic, ...

  8. Increased 5α-reductase activity in idiopathic hirsutism

    International Nuclear Information System (INIS)

    Serafini, P.; Lobo, R.A.

    1985-01-01

    In vitro, genital skin 5α-reductase activity (5α-RA) was measured in ten hirsute women with normal androgen levels (idiopathic hirsutism (IH)) and in ten hirsute women with elevated androgen levels (polycystic ovary syndrome (PCO)) in order to determine the influence of secreted androgens on 5α-RA. In vitro 5α-RA was assessed by incubations of skin with 14 C-testosterone (T) for 2 hours, after which steroids were separated and the radioactivity of dihydrotestosterone (DHT) and 5α-androstane 3α-17β-estradiol (3α-diol) in specific eluates were determined. All androgens were normal in IH with the exception of higher levels of 3α-diol glucuronide which were similar to the levels of PCO. The conversion ratio (CR) of T to DHT in IH and PCO were similar, yet significantly greater than the CR of control subjects. The CR of T to 3α-diol in IH and PCO were similar, yet higher than in control subjects. Serum androgens showed no correlation with 5α-RA, while the CR of T to DHT showed a significant positive correlation with the Ferriman and Gallwey score. The increased 5α-RA in IH appears to be independent of serum androgen levels and is, therefore, an inherent abnormality. The term idiopathic is a misnomer, because hirsutism in these patients may be explained on the basis of increased skin 5α-RA

  9. Family history of idiopathic REM behavior disorder

    DEFF Research Database (Denmark)

    Dauvilliers, Yves; Postuma, Ronald B; Ferini-Strambi, Luigi

    2013-01-01

    To compare the frequency of proxy-reported REM sleep behavior disorder (RBD) among relatives of patients with polysomnogram-diagnosed idiopathic RBD (iRBD) in comparison to controls using a large multicenter clinic-based cohort.......To compare the frequency of proxy-reported REM sleep behavior disorder (RBD) among relatives of patients with polysomnogram-diagnosed idiopathic RBD (iRBD) in comparison to controls using a large multicenter clinic-based cohort....

  10. Adolescent idiopathic scoliosis: natural history and long term treatment effects

    Directory of Open Access Journals (Sweden)

    Asher Marc A

    2006-03-01

    Full Text Available Abstract Adolescent idiopathic scoliosis is a lifetime, probably systemic condition of unknown cause, resulting in a spinal curve or curves of ten degrees or more in about 2.5% of most populations. However, in only about 0.25% does the curve progress to the point that treatment is warranted. Untreated, adolescent idiopathic scoliosis does not increase mortality rate, even though on rare occasions it can progress to the >100° range and cause premature death. The rate of shortness of breath is not increased, although patients with 50° curves at maturity or 80° curves during adulthood are at increased risk of developing shortness of breath. Compared to non-scoliotic controls, most patients with untreated adolescent idiopathic scoliosis function at or near normal levels. They do have increased pain prevalence and may or may not have increased pain severity. Self-image is often decreased. Mental health is usually not affected. Social function, including marriage and childbearing may be affected, but only at the threshold of relatively larger curves. Non-operative treatment consists of bracing for curves of 25° to 35° or 40° in patients with one to two years or more of growth remaining. Curve progression of ≥ 6° is 20 to 40% more likely with observation than with bracing. Operative treatment consists of instrumentation and arthrodesis to realign and stabilize the most affected portion of the spine. Lasting curve improvement of approximately 40% is usually achieved. In the most completely studied series to date, at 20 to 28 years follow-up both braced and operated patients had similar, significant, and clinically meaningful reduced function and increased pain compared to non-scoliotic controls. However, their function and pain scores were much closer to normal than patient groups with other, more serious conditions. Risks associated with treatment include temporary decrease in self-image in braced patients. Operated patients face the usual

  11. Multiplex assessment of serum cytokine and chemokine levels in idiopathic morphea and vitamin K1-induced morphea.

    Science.gov (United States)

    Cox, Lori Ann; Webster, Guy F; Piera-Velazquez, Sonsoles; Jimenez, Sergio A

    2017-05-01

    The levels of 63 cytokines, chemokines, and growth factors were measured in the serum of four patients with idiopathic morphea and of one patient with vitamin K 1 -induced morphea employing a multiplex assay to identify the role of inflammatory/immunologic events in their pathogenesis. Full-thickness skin biopsies of affected skin were analyzed by histopathology. Luminex assays for 63 cytokines, chemokines, and growth factors were performed in the sera from four patients with idiopathic morphea and in two different samples of serum obtained in two separate occasions from one patient with vitamin K 1 -induced morphea. The serum values of numerous inflammatory cytokines and growth factors including IL-2, IL-4, IL-6, and IFNβ were markedly increased in the serum of patients with idiopathic morphea, whereas, these values were normal in the serum of the patient with vitamin K 1 -induced morphea. In contrast, serum eotaxin levels were greater than threefold higher in the patient with vitamin K 1 -induced morphea compared to patients with idiopathic morphea. The results demonstrated remarkable increases in the levels of numerous cytokines and chemokines in the serum samples of all patients with idiopathic morphea indicative of a prominent role of inflammatory/immunologic events in its pathogenesis. The results also showed statistically significant differences between idiopathic morphea and vitamin K 1 -induced morphea suggesting that their development involves different pathogenetic mechanisms.

  12. Idiopathic Ophthalmodynia and Idiopathic Rhinalgia: A Prospective Series of 16 New Cases.

    Science.gov (United States)

    Pareja, Juan A; Montojo, Teresa; Guerrero, Ángel L; Álvarez, Mónica; Porta-Etessam, Jesús; Cuadrado, María L

    2015-01-01

    Idiopathic ophthalmodynia and idiopathic rhinalgia were described a few years ago. These conditions seem specific pain syndromes with a distinctive location in the eye or in the nose. We aimed to present a new prospective series in order to verify the consistency of these syndromes. We performed a descriptive study of all patients referred to our regional neurologic clinics from 2010 to 2014 because of facial pain exclusively felt in the eye or in the nose fulfilling the proposed diagnostic criteria for idiopathic ophthalmodynia and idiopathic rhinalgia. There were 9 patients with idiopathic ophthalmodynia and 7 patients with idiopathic rhinalgia, with a clear female preponderance, and a mean age at onset in the fifth decade. The pain was usually moderate and the temporal pattern was generally chronic. Only one patient reported accompaniments (hypersensitivity to the light and to the flow of air in the symptomatic eye). Preventive treatment with amitriptyline, pregabalin, or gabapentin was partially or totally effective. The clinical features of this new series parallels those of the original description, thus indicating that both idiopathic ophthalmodynia and idiopathic rhinalgia have clear-cut clinical pictures with excellent consistency both inter- and intra-individually. © 2015 American Headache Society.

  13. Low density in liver of idiopathic portal hypertension

    International Nuclear Information System (INIS)

    Ishito, Hiroyuki

    1988-01-01

    In order to evaluate the diagnostic value of low density in liver on computed tomography (CT), CT scans of 11 patients with idiopathic portal hypertension (IPH) were compared with those from 22 cirrhotic patients, two patients with scarred liver and 16 normal subjects. Low densities on plain CT scans in patients with IPH were distinctly different from those observed in normal liver. Some of the low densities had irregular shape with unclear margin and were scattered near the liver surface, and others had vessel-like structures with unclear margin and extended as far as near the liver surface. Ten of the 11 patients with IPH had low densities mentioned above, while none of the 22 cirrhotic patients had such low densities. The present results suggest that the presence of low densities in liver on plain CT scan is clinically beneficial in diagnosis of IPH. (author)

  14. Bone density determination using I125 densitometry with idiopathic scoliosis

    International Nuclear Information System (INIS)

    Weinberger, N.

    1984-01-01

    Based on the assumption that radiographs from patients with idiopathic scoliosis show osteoporotic changes in the curved area, investigation with I 125 -densitometry were made, and specifically with measurement points at the ulna and the calcaneus. A difference in the bone density between patients with scoliosis and normal controls could not be proven. The mineral-salt content of the scoliosis patients lay on the average 6.5 to 9.3% lower than the normal controls. No relation could be found between the degree of curvature of the scoliosis and the peripheral bone density, from which it can be concluded that no generalized mineral-salt deficiency exists. Radiographs show only local changes (photo densitometry, computed tomography). (TRV) [de

  15. Idiopathic combined, autoantibody-mediated ADAMTS-13/factor H deficiency in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome in a 17-year-old woman: a case report

    Directory of Open Access Journals (Sweden)

    Patschan Daniel

    2011-12-01

    Full Text Available Abstract Introduction Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome is a life-threatening condition with various etiopathogeneses. Without therapy approximately 90% of all patients die from the disease. Case presentation We report the case of a 17-year-old Caucasian woman with widespread hematomas and headache. Due to hemolytic anemia, thrombocytopenia, and schistocytosis, thrombotic thrombocytopenic purpura-hemolytic uremic syndrome was suspected and plasma exchange therapy was initiated immediately. Since her thrombocyte level did not increase during the first week of therapy, plasma treatment had to be intensified to a twice-daily schedule. Further diagnostics showed markedly reduced activities of both ADAMTS-13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 - also known as von Willebrand factor-cleaving protease and factor H. Test results for antibodies against both proteins were positive. While plasma exchange therapy was continued, rituximab was given once weekly for four consecutive weeks. After the last dose, thrombocytes and activities of ADAMTS-13 and factor H increased into the normal range. Our patient improved and was discharged from the hospital. Conclusions Since no clinical symptoms/laboratory findings indicated a malignant or specific autoimmune-mediated disorder, the diagnosis made was thrombotic thrombocytopenic purpura-hemolytic uremic syndrome due to idiopathic combined, autoantibody-mediated ADAMTS-13/factor H deficiency.

  16. Suspected Child Maltreatment: Recognize and Respond

    Science.gov (United States)

    Kemple, Kristen Mary; Kim, Hae Kyoung

    2011-01-01

    Early childhood educators spend extensive amounts of time with young children, so they are often the first adults to notice signs that a child may be abused or neglected. All educators are required by law to report suspected maltreatment, and can play an important role in preventing and responding to abuse and neglect of young children. What is…

  17. Suspecting Neurological Dysfunction From E Mail Messages ...

    African Journals Online (AJOL)

    A non medical person suspected and confirmed neurological dysfunction in an individual, based only on e mail messages sent by the individual. With email communication becoming rampant “peculiar” email messages may raise the suspicion of neurological dysfunction. Organic pathology explaining the abnormal email ...

  18. Cervical spinal canal narrowing in idiopathic syringomyelia

    International Nuclear Information System (INIS)

    Struck, Aaron F.; Carr, Carrie M.; Shah, Vinil; Hesselink, John R.; Haughton, Victor M.

    2016-01-01

    The cervical spine in Chiari I patient with syringomyelia has significantly different anteroposterior diameters than it does in Chiari I patients without syringomyelia. We tested the hypothesis that patients with idiopathic syringomyelia (IS) also have abnormal cervical spinal canal diameters. The finding in both groups may relate to the pathogenesis of syringomyelia. Local institutional review boards approved this retrospective study. Patients with IS were compared to age-matched controls with normal sagittal spine MR. All subjects had T1-weighted spin-echo (500/20) and T2-weighted fast spin-echo (2000/90) sagittal cervical spine images at 1.5 T. Readers blinded to demographic data and study hypothesis measured anteroposterior diameters at each cervical level. The spinal canal diameters were compared with a Mann-Whitney U test. The overall difference was assessed with a Friedman test. Seventeen subjects were read by two reviewers to assess inter-rater reliability. Fifty IS patients with 50 age-matched controls were studied. IS subjects had one or more syrinxes varying from 1 to 19 spinal segments. Spinal canal diameters narrowed from C1 to C3 and then enlarged from C5 to C7 in both groups. Diameters from C2 to C4 were narrower in the IS group (p < 0.005) than in controls. The ratio of the C3 to the C7 diameters was also smaller (p = 0.004) in IS than controls. Collectively, the spinal canal diameters in the IS were significantly different from controls (Friedman test p < 0.0001). Patients with IS have abnormally narrow upper and mid cervical spinal canal diameters and greater positive tapering between C3 and C7. (orig.)

  19. Cervical spinal canal narrowing in idiopathic syringomyelia

    Energy Technology Data Exchange (ETDEWEB)

    Struck, Aaron F. [Massachusetts General Hospital, Department of Neurology, Boston, MA (United States); Carr, Carrie M. [Mayo Clinic, Department of Radiology, Rochester, MN (United States); Shah, Vinil [University of California San Francisco, Department of Radiology, San Francisco, CA (United States); Hesselink, John R. [University of California San Diego, Department of Radiology, San Diego, CA (United States); Haughton, Victor M. [University of Wisconsin, Department of Radiology, Madison, WI (United States)

    2016-08-15

    The cervical spine in Chiari I patient with syringomyelia has significantly different anteroposterior diameters than it does in Chiari I patients without syringomyelia. We tested the hypothesis that patients with idiopathic syringomyelia (IS) also have abnormal cervical spinal canal diameters. The finding in both groups may relate to the pathogenesis of syringomyelia. Local institutional review boards approved this retrospective study. Patients with IS were compared to age-matched controls with normal sagittal spine MR. All subjects had T1-weighted spin-echo (500/20) and T2-weighted fast spin-echo (2000/90) sagittal cervical spine images at 1.5 T. Readers blinded to demographic data and study hypothesis measured anteroposterior diameters at each cervical level. The spinal canal diameters were compared with a Mann-Whitney U test. The overall difference was assessed with a Friedman test. Seventeen subjects were read by two reviewers to assess inter-rater reliability. Fifty IS patients with 50 age-matched controls were studied. IS subjects had one or more syrinxes varying from 1 to 19 spinal segments. Spinal canal diameters narrowed from C1 to C3 and then enlarged from C5 to C7 in both groups. Diameters from C2 to C4 were narrower in the IS group (p < 0.005) than in controls. The ratio of the C3 to the C7 diameters was also smaller (p = 0.004) in IS than controls. Collectively, the spinal canal diameters in the IS were significantly different from controls (Friedman test p < 0.0001). Patients with IS have abnormally narrow upper and mid cervical spinal canal diameters and greater positive tapering between C3 and C7. (orig.)

  20. Effect of acid suppression therapy on gastroesophageal reflux and cough in idiopathic pulmonary fibrosis: an intervention study.

    Science.gov (United States)

    Kilduff, Claire E; Counter, Melanie J; Thomas, Gareth A; Harrison, Nicholas K; Hope-Gill, Benjamin D

    2014-01-01

    Chronic cough affects more than 70 percent of patients with Idiopathic Pulmonary Fibrosis and causes significant morbidity. Gastroesophageal reflux is the cause of some cases of chronic cough; and also has a postulated role in the aetiology of Idiopathic Pulmonary Fibrosis. A high prevalence of acid; and more recently non-acid, reflux has been observed in Idiopathic Pulmonary Fibrosis cohorts. Therefore, gastroesophageal reflux may be implicated in the pathogenesis of cough in Idiopathic Pulmonary Fibrosis. Eighteen subjects with Idiopathic Pulmonary Fibrosis underwent 24-hour oesophageal impedance and cough count monitoring after the careful exclusion of causes of chronic cough other than gastroesophageal reflux. All 18 were then treated with high dose acid suppression therapies. Fourteen subjects underwent repeat 24-hour oesophageal impedance and cough count monitoring after eight weeks. Total reflux and acid reflux frequencies were within the normal range in the majority of this cohort. The frequencies of non-acid and proximal reflux events were above the normal range. Following high dose acid suppression therapy there was a significant decrease in the number of acid reflux events (p = 0.02), but an increase in the number of non-acid reflux events (p = 0.01). There was no change in cough frequency (p = 0.70). This study confirms that non-acid reflux is prevalent; and that proximal oesophageal reflux occurs in the majority, of subjects with Idiopathic Pulmonary Fibrosis. It is the first study to investigate the effect of acid suppression therapy on gastroesophageal reflux and cough in patients with Idiopathic Pulmonary Fibrosis. The observation that cough frequency does not improve despite verifiable reductions in oesophageal acid exposure challenges the role of acid reflux in Idiopathic Pulmonary Fibrosis associated cough. The finding that non-acid reflux is increased following the use of acid suppression therapies cautions against the widespread use

  1. Idiopathic Gingival Fibromatosis: Case Report and Its Management

    Directory of Open Access Journals (Sweden)

    Prashant P. Jaju

    2009-01-01

    Full Text Available Idiopathic gingival fibromatosis is a rare condition. We present a case of idiopathic gingival fibromatosis with its multidisciplinary approach of management. The clinical, radiographic, and histopathological features have been described in detail.

  2. Juvenile idiopathic arthritis – an update on its diagnosis and ...

    African Journals Online (AJOL)

    2015-12-03

    Dec 3, 2015 ... Juvenile idiopathic arthritis (JIA) is the most common form of chronic arthritis in children and the most ... A swollen knee and uveitis in a young girl, for instance, is ..... Methotrexate for treating juvenile idiopathic arthritis.

  3. Idiopathic portal hypertension regarding thiopurine treatment in patients with inflammatory bowel disease.

    Science.gov (United States)

    Suárez Ferrer, Cristina; Llop Herrera, Elba; Calvo Moya, Marta; Vera Mendoza, María Isabel; González Partida, Irene; González Lama, Yago; Matallana Royo, Virginia; Calleja Panero, José Luis; Abreu García, Luis

    2016-02-01

    The possibility of developing idiopathic portal hypertension has been described with thiopurine treatment despite compromises the prognosis of these patients, the fact its true prevalence is unknown. A cross-sectional study was conducted in a cohort of inflammatory bowel disease (IBD) patients followed at our unit, to determine the prevalence of diagnosis of idiopathic portal hypertension (IPH) and its relationship with thiopurine treatment. At the time of the analysis, 927/1,419 patients were under treatment with thiopurine drugs (65%). A total of 4 patients with IBD type Crohn's disease with idiopathic portal hypertension probably related to the thiopurine treatment were identified (incidence of 4.3 cases per 1,000). Seventy-five percent of patients started with signs or symptoms of portal hypertension. Only one patient was asymptomatic but the diagnosis of IPH because of isolated thrombocytopenia is suspected. However, note that all patients had thrombocytopenia previously. Abdominal ultrasound with fibroscan, hepatic vein catheterization and liver biopsy were performed on all of them as part of the etiology of portal hypertension. In the abdominal ultrasound, indirect portal hypertension data were observed in all patients (as splenomegaly) cirrhosis was also ruled out. The fibroscan data showed significant liver fibrosis (F2-F3). Idiopathic portal hypertension following thiopurine treatment in IBD patients is a rare occurrence, but it must be borne in mind in the differential diagnosis for early diagnosis, especially in patients undergoing thiopurine treatment over a long period. The presence of thrombocytopenia is often the only predictor of its development in the preclinical stage.

  4. Telltale teeth: Idiopathic Hypergonadotropic Hypogonadism

    Directory of Open Access Journals (Sweden)

    G S Lele

    2014-01-01

    Full Text Available The detection of any atypical extraoral or intraoral features warrants a thorough investigation, even if the patient is asymptomatic or unaware of these. At times, dental findings help in the diagnosis of an underlying systemic problem. These findings may or may not be associated with any syndrome. Thus, thorough examination and exhaustive investigations should be carried out for every atypical finding to ensure optimal oral and general health for the patient. Case Description: This is a case report of seventeen year old male who presented with peculiar/atypical dentition which ′told the tale′ and led to the diagnosis of underlying endocrinological problem about which the parents were totally unaware. The patient was short with central obesity and microcephaly. Intraorally, there was presence of thirty six microdonts. Consultation with pediatrician and endocrinologist, and thorough investigations confirmed the condition to be of ′Idiopathic Hypergonadotropic Hypogonadism′. The patient underwent not only oral rehabilitation, but also timely consultation and treatment from a pediatrician and an endocrinologist.

  5. Idiopathic aneurysm of pulmonary artery

    Energy Technology Data Exchange (ETDEWEB)

    Pacheco, Julio B. Cota; Pimentel, Patricia N.; Knust, Beatriz S., E-mail: jcota@uol.com.br [Clinica de Cardiologia Cota Pacheco, Mogi das Cruzes, SP (Brazil)

    2015-07-15

    Because it is a very rare isolated lesion, we decided to present this case of idiopathic pulmonary artery aneurysm (IPAA) and review the cases published in the literature in order to correlate our clinical and imaging findings, as well as management based on patient data. IPAA was first described in a case of autopsy by Bristowe in 1860 and later in 1947 by Deterling and Claggett, whose prevalence was lower than eight to one hundred thousand. Although the use of diagnostic imaging methods has been applied in a very large population in recent decades, this lesion has been most often described in postmortem examinations. Therefore, it is important to be aware of possible clinical symptoms, at times non-specific, as well as the signs on imaging studies. In this study, therefore, the report of a case of an asymptomatic patient whose diagnosis was done through color Doppler echocardiography in a routine test in 2012, later confirmed by simple chest computed tomography (chest CT) and cardiac catheterization as IPAA and its branches. We discussed the literature available and the possibilities for treatment and the use of color Doppler echocardiography as an initial diagnostic tool for such a rare and intriguing disease. (author)

  6. Magnetic resonance imaging of posterior pituitary for evaluation of the neurohypophyseal function in idiopathic and autosomal dominant neurohypophyseal diabetes insipidus

    International Nuclear Information System (INIS)

    Ozata, M.; Tayfun, C.; Kurtaran, K.; Yetkin, I.; Beyhan, Z.; Corakci, A.; Caglayan, S.; Alemdaroglu, A.; Guendogan, M.A.

    1997-01-01

    We investigated the role of MR imaging for evaluation of the functional status of the neurohypophyseal system in both idiopathic central diabetes insipidus (DI) and familial autosomal dominant neurohypophyseal DI. The patients and family with DI were analyzed retrospectively for the presence or absence of posterior pituitary gland hyperintense signal on MR images. A total of 19 adult patients with idiopathic central DI, 7 members of a family with autosomal dominant DI and 20 control subjects were included in the study. Diagnosis of idiopathic DI was based on the presence of central DI in the absence of any alteration that is known to be responsible for DI. The patients were studied retrospectively and the morphology and intensity of the posterior lobe by MR imaging was assessed by blinded reading. In all patients with idiopathic central DI and the affected members of the family, the posterior bright signal was absent while the stalk was normal on MR images. In contrast, normal posterior pituitary bright signal and stalk were found in unaffected members of the family and all control subjects. We conclude that MR imaging of the posterior pituitary lobe can be used to evaluate the functional status of the neurohypophyseal system in idiopathic central DI and familial autosomal dominant DI. (orig.). With 3 figs., 1 tab

  7. Scintigraphic measurement of regional gut transit in idiopathic constipation

    International Nuclear Information System (INIS)

    Stivland, T.; Camilleri, M.; Vassallo, M.; Proano, M.; Rath, D.; Brown, M.; Thomforde, G.; Pemberton, J.; Phillips, S.

    1991-01-01

    In this study, total gut transit and regional colonic transit in patients with idiopathic constipation were measured scintigraphically. Eight patients with severe constipation were studied, none of whom had evidence of abnormal function of the pelvic floor. 99mTc-radiolabeled Amberlite resin particles with a mixed meal were used to assess gastric emptying and small bowel transit; similar particles labeled with 111In were ingested in a coated capsule that dispersed in the ileocecal region. These were used to quantify colonic transit. Five healthy volunteers were also studied. Two patients showed delayed gastric emptying and two had slow small bowel transit. Seven of the eight patients had slow colonic transit. In five, delay affected the whole colon (pancolonic inertia); in two, transit in the ascending and transverse colon was normal, but solids moved through the left colon slowly. Mean colonic transit was also measured using radiopaque markers; this technique identified the patients with slow transit, as shown by measurements of overall colonic transit by simultaneous scintigraphy. However, estimated transit through the ascending and transverse colons was considerably shorter by the radiopaque marker technique. In conclusion, idiopathic constipation is characterized by either exaggerated reservoir functions of the ascending and transverse colons and/or impairment of propulsive function in the descending colon. Particle size may influence the result of regional colonic transit tests. Transit delays in other parts of the gut suggest that, in some patients, the condition may be a more generalized motor dysfunction

  8. Scintigraphic measurement of regional gut transit in idiopathic constipation

    Energy Technology Data Exchange (ETDEWEB)

    Stivland, T.; Camilleri, M.; Vassallo, M.; Proano, M.; Rath, D.; Brown, M.; Thomforde, G.; Pemberton, J.; Phillips, S. (Gastroenterology Research Unit, Mayo Clinic, Rochester, Minnesota (USA))

    1991-07-01

    In this study, total gut transit and regional colonic transit in patients with idiopathic constipation were measured scintigraphically. Eight patients with severe constipation were studied, none of whom had evidence of abnormal function of the pelvic floor. 99mTc-radiolabeled Amberlite resin particles with a mixed meal were used to assess gastric emptying and small bowel transit; similar particles labeled with 111In were ingested in a coated capsule that dispersed in the ileocecal region. These were used to quantify colonic transit. Five healthy volunteers were also studied. Two patients showed delayed gastric emptying and two had slow small bowel transit. Seven of the eight patients had slow colonic transit. In five, delay affected the whole colon (pancolonic inertia); in two, transit in the ascending and transverse colon was normal, but solids moved through the left colon slowly. Mean colonic transit was also measured using radiopaque markers; this technique identified the patients with slow transit, as shown by measurements of overall colonic transit by simultaneous scintigraphy. However, estimated transit through the ascending and transverse colons was considerably shorter by the radiopaque marker technique. In conclusion, idiopathic constipation is characterized by either exaggerated reservoir functions of the ascending and transverse colons and/or impairment of propulsive function in the descending colon. Particle size may influence the result of regional colonic transit tests. Transit delays in other parts of the gut suggest that, in some patients, the condition may be a more generalized motor dysfunction.

  9. Clarifying Normalization

    Science.gov (United States)

    Carpenter, Donald A.

    2008-01-01

    Confusion exists among database textbooks as to the goal of normalization as well as to which normal form a designer should aspire. This article discusses such discrepancies with the intention of simplifying normalization for both teacher and student. This author's industry and classroom experiences indicate such simplification yields quicker…

  10. The role of MRI in suspected inner ear malformations

    International Nuclear Information System (INIS)

    Koesling, S.; Juettemann, S.; Amaya, B.; Rasinski, C.; Bloching, M.; Koenig, E.

    2003-01-01

    Purpose: This is a prospective analysis of the value of MRI in suspected inner ear malformations. Materials and Methods: In 50 patients (43 children and young adults, 7 adults) with suspected inner ear malformation MRI (1.5 T) was performed. In addition, 42 of these patients underwent CT. For the analysis of the inner ear structures, the constructive interference in steady state (CISS) sequence with 0.7 mm slice thickness was used. Functional tests revealed a sensorineural hearing loss or deafness in 82 temporal bones (TB) and a combined hearing loss in 4 TB. The hearing loss was unilateral in 14 patients. MRI and CT findings were compared. Results: Imaging findings were normal in 58 TB. The pathological findings included inner ear malformations (35 TB), inflammatory changes (4 TB), partial obliteration of labyrinth (2 TB) and congenital aural atresia (1 TB). An isolated absence of the cochlear nerve (1 TB) could only be found by MRI. In the remaining cases, an inner ear malformation was diagnosed by MRI and CT with the same confidence but MRI was superior in displaying the fine details. Conclusions: MRI will become the method of choice in the diagnosis of inner ear malformations. (orig.) [de

  11. Value of noncontrast spiral CT for suspected acute appendicitis

    International Nuclear Information System (INIS)

    Choi, Pil Yeob; Lee, Sang Wook; Kwon, Jae Soo; Sung, Young Soon; Rho, Myoung Ho; Chang, Jeong A.

    1998-01-01

    To assess the diagnostic accuracy and clinical efficacy of noncontrast spiral CT in patients with suspected acute appendicitis. Over a six-month period, 100 patients with suspected acute appendicitis were prospectively evaluated with noncontrast spiral CT. All scans were obtained from the lower body of L3 to the symphysis pubis, with 5mm or 10mm collimation and pitch of 1 or 1.5, and without intravenous or oral contrast material. Diagnosis was established by means of surgical or clinical follow-up. Prospective diagnosis based on CT findings was compared with surgical results and clinical follow-up. Acute appendicitis was confirmed in 47 of 100 patients. On the basis of the Ct findings, SI patients were prospectively interpreted as positive for appendicitis, but in six the diagnosis was false-positive. Two of the 47 with acute appendicitis were prospectively interpreted as normal. The preoperative diagnosis of acute appendicitis was, thus, 45 true-positive, 47 true-negative, six false-positive and two false-negative yielding a sensitivity of 96%, a specificity of 89%, an accuracy of 92%, a positive predictive value of 88%, and a negative predictive value of 96%. Using CT, an alternative diagnosis was established in 14 patients. Noncontrast spiral CT is a useful technique for diagnosing acute appendicitis. =20

  12. Suspects in criminal investigations of rape

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    Marinković Darko

    2014-01-01

    Full Text Available Investigations of sexual assaults mostly focus on victims and their credibility, which may cause lack of firm evidence in relation to suspects. Given the fact that the criminal offence of rape is characterised by a high incidence of false reports and accusations, frequently indicating specific persons as the perpetrators, certain caution is necessary in the investigation in order to avoid false accusations and/or convictions. As regards the personality of the rapist and motives for committing a forcible sexual act, certain types or rather certain categories of perpetrators can be distinguished, although it should be noted that a large number of rapists do not belong to one category only, but rather combine characteristics of several different types. During a criminal investigation it is of vital importance to differentiate between a rape as a surprise attack and a rape as abuse of trust, as they are compatible with the nature of the suspect's defence. The suspect shall be subjected to a forensic examination in the course of the investigation in order to find traces which prove vaginal, anal or oral penetration, coerced sexual intercourse and identity of the rapist. While conducting an interrogation of a suspected rapist, a crime investigating officer shall use either factual or emotional approach to his interviewee, depending on his psychological and motivational characteristics. In this regard, the factual approach is believed to be more efficient with anger rapists and sadistic rapists, whereas the compassionate approach gives good results with the gentlemen-rapists and partly with the power asserting rapists.

  13. Management of idiopathic nephrotic syndrome in childhood

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    Peco-Antić Amira

    2004-01-01

    Full Text Available The management of idiopathic nephrotic syndrome (INS in children includes immunosuppressive and symptomatic treatment. The response to corticosteroid therapy is the best prognostic marker of the disease. The majority of children with INS (about 85% are steroid-sensitive as they normalize proteinuria within 4 weeks of daily, oral prednisone administration. The most of steroid-sensitive patients (94% has minimal change of nephrotic syndrome, while the majority (80.5%-94.4% of those who are steroid-resistant has focal segmental glomerulosderosis or mesangioproliferative glomerulonephritis. Initial therapy of INS consists of 60 mg/m2/day prednisone daily for 4 weeks followed by 40 mg/m2 on alternate days for 4 weeks, thereafter decreasing alternate day therapy every month by 25% over the next 4 months. Thus, the overall duration of the initial cortico-steroids course is 6 months that may be significantly protective against the future development of frequent relapses. Approximately 30% of patients experience only one attack and are cured after the first course of therapy; 10-20% have only 3 or 4 steroid-responsive episodes before permanent cure; the remaining 40-50% of patients are frequent relapsers, or steroid-dependent. Standard relapse therapy consists of 60 mg/m2/ day prednisone until urine is protein free for at least 3 days, followed by 40 mg/m2 on alternate days for 4 weeks. The treatment of frequent-relapses and steroid-dependent INS includes several different regimens: maintenance (6 months alternate steroid therapy just above steroid threshold (0.1-0.5 mg/kg/ 48h, levamisole, alkylating agents (cyclophosphamide or chlorambucil or cyclosporine. The worse prognosis is expected in steroid-resistant patients who are the most difficult to treat. Renal biopsy should be performed in them. At present, there is no consensus on therapeutic regimen for steroid-resistant patients. The following immunosuppressive drugs have been used with varying

  14. Chronic idiopathic constipation: a psychological enquiry.

    Science.gov (United States)

    Dykes, S; Smilgin-Humphreys, S; Bass, C

    2001-01-01

    Intractable idiopathic constipation in women is often associated with psychosocial problems. To determine the past and current psychological factors associated with slow and normal transit constipation. Twenty-eight consecutive patients referred for biofeedback treatment were interviewed before the procedure. All were women. Transit studies revealed that 12 had slow transit constipation (STC) and 16 had normal transit constipation (NTC). Patients were assessed for evidence of previous and current psychiatric diagnoses using a standardized diagnostic interview schedule. A full family and social history was noted. Self-rating scales were used to measure psychological distress, abnormal attitudes to eating and current psychosocial functioning. The mean age of the 28 patients was 38.2 years (SD = 10.8) with a mean duration of symptoms of 17.5 years (SD = 16.9). Seventeen (61%) had a current psychiatric disorder and 18 (64%) a previous episode of psychiatric illness. The mean age of the 16 NTC patients was 38.4 years (SD = 10.1) with a mean duration of symptoms of 12.4 years (SD = 15.9). By contrast, the 12 STC patients had a much longer mean duration of constipation (24.3 years; SD = 16.4), a mean age of 37.9 years (SD = 12.1), with half having an onset in childhood. The STC patients reported more psychosocial distress on the rating scales than those with NTC, and only one did not experience some form of adverse life event or gynaecological procedure in the 6 months before the onset of constipation. Eleven (39%) of the 28 women had had a hysterectomy at a mean age of 36 years, but only four (14%) reported a history of sexual abuse. Of the nine (32%) patients who reported markedly distorted attitudes to food, six had NTC and three had STC. Of consecutive patients undergoing psychological assessment for intractable constipation, three fifths had evidence of current, and two thirds a previous, affective disorder. One third reported distorted attitudes to food. Although

  15. Evaluation of the pituitary gland in idiopathic hypogonadotropic hypogonadism

    International Nuclear Information System (INIS)

    Jayasundar, R.; Raghunathan, P.; Ammini, A.C.; Gupta, R.

    1999-01-01

    Purpose: Evaluation of the pituitary gland has been carried out in idiopathic hypogonadotropic hypogonadism (IHH) to test the potential of MR imaging in differentiating IHH patients from normals. Material and Methods: Thirty-seven patients (aged 18-30 years), and 20 volunteers (aged 20-30 years) were studied by T1-weighted MR imaging. Length (L A and L P ), height (H), width (W), area (A A and A P ), and volume (V 1A , V 1P , V 2A , V 2P ) of the pituitary gland were determined. (Subscripts P and A refer to measurements made with and without the posterior lobe, respectively.) V 1 and V 2 were estimated using two different methods. Results: in the control group, L P , W and V 2A exhibited significant differences between female and male volunteers. While W was the only parameter that did not show significant difference between normals and patients (in both men and women), all other parameters except L P , H and A P showed statistically significant differences between normals and IHH patients in both males and females. While L P was significantly different between normals and patients (men), H and A P were significantly different between normals and patients only in the female group. Conclusion: Correlation analysis between various parameters has shown that L A can be used for evaluating the pituitary in both the male and female IHH patients. (orig.)

  16. Anterior segment changes after pharmacologic mydriasis using Pentacam and optical coherence tomography in angle closure suspects

    Directory of Open Access Journals (Sweden)

    Jing-Min Guo

    2015-10-01

    Full Text Available AIM:To compare the dynamic changes of anterior segment parameters especially iris morphology induced by pharmacologic mydriasis between angle closure suspects and normal controls.METHODS:The study group comprised 19 eyes of 19 angle closure suspects and 19 eyes of 19 age- and sex-matched normal open-angle eyes. Pentacam and optical coherence tomography measurements before and 30min after instillation of compound tropicamide eye drop were performed and compared. Biometric evaluations of iris tomography and anterior chamber angle were estimated by a customized image-processing software.RESULTS:Baseline axial length, iris cross sectional area and volume did not differ significantly between angle closure suspects and normal controls. Angle closure suspects had smaller pupil size, narrower anterior segment dimension and axial length, thinner iris with greater curve in comparison with normal controls. Pharmacologic mydriasis led to significant increments in iris thickness at 750 μm, anterior chamber depth and volume, whereas significant decrements in iris curve, cross sectional area and volume in both groups. Angle opening distance at 500 μm was increased significantly in normal controls (from 0.465±0.115 mm to 0.539±0.167 mm, P=0.009, but not in angle closure suspects (from 0.125±0.100 mm to 0.145±0.131 mm, P=0.326. Iris volume change per millimeter of pupil dilation (△IV/△PD decreased significantly less in angle closure suspects than normal controls (-2.47±1.33 mm2 vs -3.63±1.58 mm2, P=0.019. Linear regression analysis showed that the change of angle opening distance at 500 μm was associated most with the change of central anterior chamber depth (β=0.841, P=0.002 and △IV/△PD (β=0.028, P=0.002, followed by gender (β=0.062, P=0.032.CONCLUSION:Smaller iris volume decrement per millimeter of pupil dilation is related significantly with the less anterior angle opening in angle closure suspects after pharmacologic mydriasis. Dynamic

  17. Total fertilization failure and idiopathic subfertility

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    Goverde Angelique J

    2009-01-01

    Full Text Available Abstract Background To gain more insight in whether failure of intrauterine insemination (IUI treatment in patients with idiopathic subfertility could be related to diminished fertilization, the aim of this study is to compare the fertilization of an initial IVF procedure after six cycles of IUI and the fertilization of an initial IVF procedure without preceding IUI cycles in couples with idiopathic subfertility. Methods We performed a complimentary analysis of a randomized controlled trial, in which the number of total fertilization failure (TFF in the first IVF procedure after unsuccessful IUI was compared to those of IVF without preceding IUI in patients with idiopathic subfertility. These patients participated in a previous study that assessed the cost effectiveness of IUI versus IVF in idiopathic subfertility and were randomized to either IUI or IVF treatment. Results 45 patients underwent IVF after 6 cycles of unsuccessful IUI and 58 patients underwent IVF immediately without preceding IUI. In 7 patients the IVF treatment was cancelled before ovum pick. In the IVF after unsuccessful IUI group TFF was seen in 2 of the 39 patients (5% versus 7 of the 56 patients (13% in the immediate IVF group. After correction for confounding factors the TFF rate was not significantly different between the two groups (p = 0.08, OR 7.4; 95% CI: 0.5–14.9. Conclusion Our data showed that TFF and the fertilization rate in the first IVF treatment were not significantly different between couples with idiopathic subfertility undergoing IVF after failure of IUI versus those couples undergoing IVF immediately without prior IUI treatment. Apparently, impaired fertilization does not play a significant role in the success rate of IUI in patients with idiopathic subfertility.

  18. A neonate with intestinal volvulus without malrotation exhibiting early jaundice with a suspected fetal onset.

    Science.gov (United States)

    Hara, Kaori; Kinoshita, Mari; Kin, Takane; Arimitsu, Takeshi; Matsuzaki, Yohei; Ikeda, Kazushige; Tomita, Hiroshi; Fujino, Akihiro; Kuroda, Tatsuo

    2015-01-01

    Intestinal volvulus without malrotation is a rare disease that causes volvulus of the small intestine despite normal intestinal rotation and fixation. We encountered a neonate with this disease who developed early jaundice and was suspected to have a fetal onset. This patient was characterized by early jaundice complicating intestinal volvulus without malrotation and is considered to have exhibited reduced fetal movement and early jaundice as a result of volvulus, necrosis, and hemorrhage of the small intestine in the fetal period. If abdominal distention accompanied by early jaundice is noted in a neonate, intestinal volvulus without malrotation and associated intraabdominal hemorrhage should be suspected and promptly treated.

  19. Management strategies for idiopathic urethritis.

    Science.gov (United States)

    Henderson, L; Farrelly, P; Dickson, A P; Goyal, A

    2016-02-01

    Williams and Mikhael (1971) described idiopathic urethritis (IU) as a self-limiting condition that affects boys aged 5-15 years, with symptoms of urethrorrhagia, dysuria and haematuria. However, a proportion of boys will remain symptomatic for several years, and may develop urethral stricture (Poch et al., 2007; Palagiri et al., 2003). There is no universally effective treatment for IU, although various strategies have been employed. To review the presentation and long-term outcomes of boys with IU, and present the efficacy of management strategies that have been utilised. A retrospective review was performed of all boys with IU. It was based on clinical and cystoscopic findings for presentation, medical history, management and clinical progress. Fifty-four boys were included, with a median age of 11 years (range 5-15 years) at presentation. The median duration of symptoms was 18 months (range 2-132 months). The median follow-up was 18.5 months (range 1-120 months). Seven (13.0%) boys had early urethral stricture at initial cystourethroscopy, and one (1.9%) developed stricture during follow-up. Thirty-six boys (66.7%) had previous circumcision and four (7.4%) had meatal stenosis. Eight (14.8%) had previous hypospadias repair. Whilst 50% of boys with IU do not require any specific treatment, those with severe/unremitting symptoms may benefit from a trial of urethral steroids or short-term urethral catheterisation. The mechanisms of benefit from these modalities are unclear and they require further evaluation. Copyright © 2015 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.

  20. Idiopathic thoracic transdural intravertebral spinal cord herniation

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    Mazda K Turel

    2017-01-01

    Full Text Available Idiopathic spinal cord herniation is a rare and often missed cause of thoracic myelopathy. The clinical presentation and radiological appearance is inconsistent and commonly confused with a dorsal arachnoid cyst and often is a misdiagnosed entity. While ventral spinal cord herniation through a dural defect has been previously described, intravertebral herniation is a distinct entity and extremely rare. We present the case of a 70-year old man with idiopathic thoracic transdural intravertebral spinal cord herniation and discuss the clinico-radiological presentation, pathophysiology and operative management along with a review the literature of this unusual entity.

  1. [Diagnostic strategy in patients with clinically suspected deep vein thrombosis

    DEFF Research Database (Denmark)

    Mantoni, Margit Yvonne; Kristensen, M.; Brogaard, M.H.

    2008-01-01

    INTRODUCTION: The standard method for diagnosing deep vein thrombosis (DVT) involves determination of D-dimer and ultrasound scanning. In an attempt to reduce the number of ultrasound examinations we have supplemented this with a clinical probability estimate for DVT (DVT-score) over one year....... MATERIALS AND METHODS: A total of 508 consecutive patients presenting in the emergency room with suspected DVT had D-dimer and DVT-score performed. Patients with non-elevated D-dimer and a low or moderate DVT score received no treatment. The remainder had ultrasound scanning from the groin to the popliteal...... patients with normal D-dimer had high DVT-scores, none had DVT, so that the benefit from determining DVT-scores was modest. Ultrasound scanning revealed DVT in 85 out of 397 patients with elevated D-dimer. A repeat examination was performed in 91 patients with persisting symptoms, and disclosed DVT in two...

  2. Idiopathic portal hypertension with regard to thiopurine treatment.

    Science.gov (United States)

    Machlab, Salvador; Miquel, Mireia; Vergara, Mercedes

    2018-04-16

    Idiopathic portal hypertension (IPH) is an infrequent adverse reaction to the use of thiopurines that tends to be overlooked. Herein, we present a patient with ileocolic Crohn's disease treated with azathioprine who presented ascites, esophageal varices and splenomegaly without any signs of liver cirrhosis. A portal hemodynamics study revealed a normal portosystemic gradient compatible with presinusoidal portal hypertension. Finally, IPH was diagnosed after a liver biopsy. IPH secondary to thiopurines is due to a 6-thioguanine nucleotide (6-TGN)-dependent reaction and occurs predominantly between three months and three years after the start of treatment. The onset is usually insidious and thrombocytopenia is the first manifestation. The definitive diagnosis is obtained by liver biopsy.

  3. [Idiopathic elephantiasis of the penis: a case report].

    Science.gov (United States)

    Yamamoto, Keisuke; Matsuoka, Yasuhiro; Takao, Tetsuya; Tsujimur, Akira; Okuyama, Akihiko; Kubo, Tateki; Hosokawa, Kou; Kakuta, Yoichi; Yamaguchi, Seiji

    2009-03-01

    We report a case of idiopathic elephantiasis of penis. A 41-year-old man was refered to our hospital with a painful penile swelling and severe miction pain. He had no particular past or familial history, and had never been to tropical or semitropical region. Physical examination showed a prominent swelling and flection of his penis. Magnetic resonance imaging revealed a significant thickening of the penile skin and subcutaneous tissue. Due to the difficulty of voiding, an urethral catheter was placed. The penile skin biopsy showed no malignancy. Under the diagnosis of penile elephantiasis, the resection of abnormal penile skin and penile plasty with the split skin graft from his thigh was performed. Histopathological findings showed nonspecific inflammation. The skin graft was successfully adapted. He could urinate smoothly without pain after the urethral catheter was removed. There has been no recurrence 6 months after the operation. He recovered normal micturition and sexual function.

  4. Mitochondria in the spotlight of aging and idiopathic pulmonary fibrosis

    Science.gov (United States)

    Mora, Ana L.; Rojas, Mauricio

    2017-01-01

    Idiopathic pulmonary fibrosis (IPF) is a chronic age-related lung disease with high mortality that is characterized by abnormal scarring of the lung parenchyma. There has been a recent attempt to define the age-associated changes predisposing individuals to develop IPF. Age-related perturbations that are increasingly found in epithelial cells and fibroblasts from IPF lungs compared with age-matched cells from normal lungs include defective autophagy, telomere attrition, altered proteostasis, and cell senescence. These divergent processes seem to converge in mitochondrial dysfunction and metabolic distress, which potentiate maladaptation to stress and susceptibility to age-related diseases such as IPF. Therapeutic approaches that target aging processes may be beneficial for halting the progression of disease and improving quality of life in IPF patients. PMID:28145905

  5. Gastroesophageal Reflux and Idiopathic Pulmonary Fibrosis: A Review

    Directory of Open Access Journals (Sweden)

    Ahmed Fahim

    2011-01-01

    Full Text Available The histological counterpart of idiopathic pulmonary fibrosis is usual interstitial pneumonia, in which areas of fibrosis of various ages are interspersed with normal lung. This pattern could be explained by repeated episodes of lung injury followed by abnormal wound healing responses. The cause of the initiating alveolar epithelial injury is unknown, but postulated mechanisms include immunological, microbial, or chemical injury, including aspirated gastric refluxate. Reflux is promoted by low basal pressure in the lower oesophageal sphincter and frequent relaxations, potentiated by hiatus hernia or oesophageal dysmotility. In susceptible individuals, repeated microaspiration of gastric refluxate may contribute to the pathogenesis of IPF. Microaspiration of nonacid or gaseous refluxate is poorly detected by current tests for gastroesophageal reflux which were developed for investigating oesophageal symptoms. Further studies using pharyngeal pH probes, high-resolution impedance manometry, and measurement of pepsin in the lung should clarify the impact of reflux and microaspiration in the pathogenesis of IPF.

  6. Suppressed without a Cause: A Case of Idiopathic Immune Deficiency.

    Science.gov (United States)

    Ayub, Muhammad Talha; Jafar, Munnam S; Khalid, Muhammad; Baig, Muhammad A; Mba, Benjamin

    2018-01-01

    We report a case of a 45-year-old male who presented with a headache, fever, vomiting, somnolence, and difficulty walking for 10 days. His cerebrospinal fluid studies revealed cryptococcal meningitis. Chest and abdominal computed tomography (CT) scans showed splenomegaly along with mediastinal, retroperitoneal and inguinal lymphadenopathy. CD4 count turned out to be 208 μL -1 . Human immunodeficiency virus (HIV) testing, serum protein electrophoresis, serum light chains and quantitative immunoglobulins were non-diagnostic and CD4 lymphopenia was attributed to acute infection. However, a persistent CD4 lymphopenia was seen in subsequent outpatient testing, which prompted a detailed workup for secondary causes of immunodeficiency. Repeated lymph node biopsies with analytic cytometric immunophenotypic analysis were normal, as was the bone marrow biopsy with detailed immunophenotypic and cytogenetic studies. The patient was hence being treated as a case of idiopathic CD4 lymphocytopenia.

  7. Idiopathic Perforation of the Sigmoid Colon in a 2.5 Years Old Girl: A Case Report

    Directory of Open Access Journals (Sweden)

    Sanaz Mehrabani

    2017-09-01

    Full Text Available Idiopathic colon perforation is rare in children. It is more common at the extremes of age. Splenic flextures, ileocecal and lower sigmoid regions are the most common sites of perforation. Delay in proper management of this condition is associated with high mortality and morbidity rate. We report on the case of a 2.5 years old girl who presented with fever, diarrhea, nausea and vomiting and progressive abdominal distention.Finally, she underwent an exploratory laparotomy for suspected peritonitis because of the patient's worsening condition. An area of perforation was found in the sigmoid colon that segmental resection and an end double barrel colostomy was done.

  8. Increased 5. cap alpha. -reductase activity in idiopathic hirsutism

    Energy Technology Data Exchange (ETDEWEB)

    Serafini, P.; Lobo, R.A.

    1985-01-01

    In vitro, genital skin 5..cap alpha..-reductase activity (5..cap alpha..-RA) was measured in ten hirsute women with normal androgen levels (idiopathic hirsutism (IH)) and in ten hirsute women with elevated androgen levels (polycystic ovary syndrome (PCO)) in order to determine the influence of secreted androgens on 5..cap alpha..-RA. In vitro 5..cap alpha..-RA was assessed by incubations of skin with /sup 14/C-testosterone (T) for 2 hours, after which steroids were separated and the radioactivity of dihydrotestosterone (DHT) and 5..cap alpha..-androstane 3..cap alpha..-17..beta..-estradiol (3..cap alpha..-diol) in specific eluates were determined. All androgens were normal in IH with the exception of higher levels of 3..cap alpha..-diol glucuronide which were similar to the levels of PCO. The conversion ratio (CR) of T to DHT in IH and PCO were similar, yet significantly greater than the CR of control subjects. The CR of T to 3..cap alpha..-diol in IH and PCO were similar, yet higher than in control subjects. Serum androgens showed no correlation with 5..cap alpha..-RA, while the CR of T to DHT showed a significant positive correlation with the Ferriman and Gallwey score. The increased 5..cap alpha..-RA in IH appears to be independent of serum androgen levels and is, therefore, an inherent abnormality. The term idiopathic is a misnomer, because hirsutism in these patients may be explained on the basis of increased skin 5..cap alpha..-RA.

  9. Birkhoff normalization

    NARCIS (Netherlands)

    Broer, H.; Hoveijn, I.; Lunter, G.; Vegter, G.

    2003-01-01

    The Birkhoff normal form procedure is a widely used tool for approximating a Hamiltonian systems by a simpler one. This chapter starts out with an introduction to Hamiltonian mechanics, followed by an explanation of the Birkhoff normal form procedure. Finally we discuss several algorithms for

  10. Nuclear Pedigree Criteria of Suspected HNPCC

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    Kładny Józef

    2003-01-01

    Full Text Available Abstract The criteria for the diagnosis of HNPCC established by the ICG-HNPCC are very restrictive as they do not allow for the diagnosis of a large number of "suspected HNPCC" cases - these are families which do no fulfill the strict diagnostic "Amsterdam criteria", but do present with several pedigree and clinical features characteristic for HNPCC. Several series of families suspected of harboring germline mutations in DNA mismatch repair genes have been studied for germline changes in DNA mismatch repair genes and a mutation rate of somewhere between 8-60% was found. Therefore a subgroup of members of the ICG-HNPCC has been working on pedigree/clinical diagnostic criteria for suspected HNPCC. Materials and methods Part I The study was based on two series of colorectal cancer (CRC cases: 1 HNPCC - this group comprised 190 patients affected by CRC from randomly selected families which fulfilled the Amsterdam II criteria registered in Düsseldorf, Germany (102 cases of CRC, Denmark (18 CRCs, Leiden, Holland (23 CRCs and Szczecin, Poland (47 CRCs. 2 Consecutive CRCs - this group comprised 629 (78.0% of 806 individuals with CRC diagnosed in 1991-1997 in the city of Szczecin (ca. 400,000 of inhabitants, Poland. Nuclear pedigrees in both groups were compared for frequency of occurrence of clinical features, that have been shown to be associated with HNPCC. Part II 52 consecutive CRC cases from Szczecin, matching the criteria recognized in part I as appropriate for diagnosis of cases "suspected of HNPCC" were studied for the occurrence of germline hMSH2/hMLH1 constitutional mutations using "exon by exon" sequencing. Results The combination of features - i.e. the occurrence of an HNPCC associated cancer (CRC or cancer of the endometrium, small bowel or urinary tract in a 1st degree relative of a CRC patient; at least one of the patients being diagnosed under age of 50 - appeared to be strongly associated to HNPCC with an OR - 161. Constitutional

  11. Exercises for adolescent idiopathic scoliosis.

    Science.gov (United States)

    Romano, Michele; Minozzi, Silvia; Bettany-Saltikov, Josette; Zaina, Fabio; Chockalingam, Nachiappan; Kotwicki, Tomasz; Maier-Hennes, Axel; Negrini, Stefano

    2012-08-15

    Adolescent idiopathic scoliosis (AIS) is a three-dimensional deformity of the spine . While AIS can progress during growth and cause a surface deformity, it is usually not symptomatic. However, in adulthood, if the final spinal curvature surpasses a certain critical threshold, the risk of health problems and curve progression is increased. The use of scoliosis-specific exercises (SSE) to reduce progression of AIS and postpone or avoid other more invasive treatments is controversial. To evaluate the efficacy of SSE in adolescent patients with AIS. The following databases (up to 30 March 2011) were searched with no language limitations: CENTRAL (The Cochrane Library 2011, issue 2), MEDLINE (from January 1966), EMBASE (from January 1980), CINHAL (from January 1982), SportDiscus (from January 1975), PsycInfo (from January 1887), PEDro (from January 1929). We screened reference lists of articles and also conducted an extensive handsearch of grey literature. Randomised controlled trials and prospective cohort studies with a control group comparing exercises with no treatment, other treatment, surgery, and different types of exercises. Two review authors independently selected studies, assessed risk of bias and extracted data. Two studies (154 participants) were included. There is low quality evidence from one randomised controlled study that exercises as an adjunctive to other conservative treatments increase the efficacy of these treatments (thoracic curve reduced: mean difference (MD) 9.00, (95% confidence interval (CI) 5.47 to 12.53); lumbar curve reduced:MD 8.00, (95% CI 5.08 to 10.92)). There is very low quality evidence from a prospective controlled cohort study that scoliosis-specific exercises structured within an exercise programme can reduce brace prescription (risk ratio (RR) 0.24, (95% CI 0.06 to1.04) as compared to usual physiotherapy (many different kinds of general exercises according to the preferences of the single therapists within different facilities

  12. Idiopathic Thrombocytopenic Purpura Misdiagnosed as Hereditary Angioedema

    DEFF Research Database (Denmark)

    Andersen, Michelle Fog; Bygum, Anette

    2015-01-01

    however not always have to be caused by angioedema but can relate to other concomitant disorders. In this report we are focusing on misdiagnosis in a patient with known hereditary angioedema, whose bleeding episode caused by idiopathic thrombocytopenic purpura was mistaken for an acute attack...

  13. Idiopathic intracranial hypertension and transverse sinus stenoses

    DEFF Research Database (Denmark)

    Skyrman, Simon; Fytagoridis, Anders; Andresen, Morten

    2013-01-01

    An 18-year-old woman was diagnosed with idiopathic intracranial hypertension (IIH) and bilateral transverse sinus stenoses (TSS), after presenting with papilledema and decreased visual acuity. Lumbar puncture revealed an opening pressure of >60 cm H2O. MRI showed bilateral TSS believed to be asso...

  14. Exercise therapy in juvenile idiopathic arthritis

    NARCIS (Netherlands)

    Takken, T.; van Brussel, M.; Engelbert, R. H. H.; van der Net, J.; Kuis, W.; Helders, P. J. M.

    2008-01-01

    Exercise therapy is considered an important component of the treatment of arthritis. The efficacy of exercise therapy has been reviewed in adults with rheumatoid arthritis but not in children with juvenile idiopathic arthritis (JIA). To assess the effects of exercise therapy on functional ability,

  15. Idiopathic trigeminal neuropathy in a poodle

    Directory of Open Access Journals (Sweden)

    Carlos Eduardo Aparicio

    2010-12-01

    Full Text Available A seven years old, male poodle is examined presenting acute mandible paralysis (dropped jaw, drooling and difficulty for the apprehension and chewing; not evidence of an other alteration of cranial nerves. The muscular biopsy rules out a myositisof masticatory muscles. The disorder is resolved completely in 3 weeks confirming diagnosis of idiopathic trigeminal neuropathy.

  16. Biological agents in polyarticular juvenile idiopathic arthritis

    DEFF Research Database (Denmark)

    Amarilyo, Gil; Tarp, Simon; Foeldvari, Ivan

    2016-01-01

    BACKGROUND AND OBJECTIVE: Although various biological agents are in use for polyarticular juvenile idiopathic arthritis (pJIA), head-to-head trials comparing the efficacy and safety among them are lacking. We aimed to compare the efficacy and safety of biological agents in pJIA using all currently...

  17. GENETIC PREDICTORS OF IDIOPATHIC SICK SINUS SYNDROME

    Directory of Open Access Journals (Sweden)

    A. A. Chernova

    2012-01-01

    Full Text Available Published data demonstrating genetic determination of sick sinus syndrome is presented. The definition of this pathology is presented; the main symptoms are described, as well as genes that influence the development of idiopathic sick sinus syndrome, their polymorphisms and role in disorders of the cardiovascular system.

  18. Acute surgical management in idiopathic intracranial hypertension.

    LENUS (Irish Health Repository)

    Zakaria, Zaitun

    2012-01-01

    Idiopathic intracranial hypertension is a headache syndrome with progressive symptoms of raised intracranial pressure. Most commonly, it is a slow process where surveillance and medical management are the main treatment modalities. We describe herein an acute presentation with bilateral sixth nerve palsies, papilloedema and visual deterioration, where acute surgical intervention was a vision-saving operation.

  19. Histological characteristics of diffuse idiopathic skeletal hyperostosis

    NARCIS (Netherlands)

    Kuperus, JS; Westerveld, L Anneloes; Rutges, Joost A; Alblas, Jacqueline; van Rijen, Mattie H; Bleys, Ronald L A W; Oner, F Cumhur; Verlaan, JJ

    Diffuse idiopathic skeletal hyperostosis (DISH) is a predominantly radiographic diagnosis and histological knowledge of DISH is limited. The aim of this study was to describe the histological characteristics of DISH in the spinal column and to study the relation between DISH and intervertebral disc

  20. Pirfenidone treatment in idiopathic pulmonary fibrosis

    DEFF Research Database (Denmark)

    Salih, Goran Nadir; Shaker, Saher Burhan; Madsen, Helle Dall

    2016-01-01

    BACKGROUND: Pirfenidone was approved by the European Medicines Agency and introduced in most European countries in 2011 for treatment of idiopathic pulmonary fibrosis (IPF). OBJECTIVE: To describe the national Danish experiences of pirfenidone treatment for IPF during 30 months with respect...

  1. Diffuse idiopathic skeletal hyperostosis in ancient clergymen.

    NARCIS (Netherlands)

    Verlaan, J.J.; Oner, F.C.; Maat, G.J.

    2007-01-01

    Diffuse idiopathic skeletal hyperostosis (DISH) is a common but often unrecognized systemic disorder observed mainly in the elderly. DISH is diagnosed when the anterior longitudinal ligament of the spine is ossified on at least four contiguous spinal levels or when multiple peripheral enthesopathies

  2. Diffuse idiopathic skeletal hyperostosis in ancient clergymen

    NARCIS (Netherlands)

    Verlaan, J.J.; Oner, F.C.; Maat, G.R.J.

    2007-01-01

    Diffuse idiopathic skeletal hyperostosis (DISH) is a common but often unrecognized systemic disorder observed mainly in the elderly. DISH is diagnosed when the anterior longitudinal ligament of the spine is ossified on at least four contiguous spinal levels or when multiple peripheral

  3. Drug therapy for chronic idiopathic axonal polyneuropathy

    NARCIS (Netherlands)

    Warendorf, Janna; Vrancken, Alexander F.J.E.; van Schaik, Ivo N.; Hughes, Richard A.C.; Notermans, Nicolette C.

    2017-01-01

    Background: Chronic idiopathic axonal polyneuropathy (CIAP) is an insidiously progressive sensory or sensorimotor polyneuropathy that affects elderly people. Although severe disability or handicap does not occur, CIAP reduces quality of life. CIAP is diagnosed in 10% to 25% of people referred for

  4. Drug therapy for chronic idiopathic axonal polyneuropathy

    NARCIS (Netherlands)

    Warendorf, Janna; Vrancken, Alexander F. J. E.; van Schaik, Ivo N.; Hughes, Richard A. C.; Notermans, Nicolette C.

    2017-01-01

    Chronic idiopathic axonal polyneuropathy (CIAP) is an insidiously progressive sensory or sensorimotor polyneuropathy that affects elderly people. Although severe disability or handicap does not occur, CIAP reduces quality of life. CIAP is diagnosed in 10% to 25% of people referred for evaluation of

  5. Acute Lymphoblastic Leukaemia presenting as Juvenile Idiopathic ...

    African Journals Online (AJOL)

    Background: Acute Lymphoblastic Leukaemia in children commonly presents with osteo articular manifestations that may mimic Juvenile Idiopathic Arthritis. This may create considerable diagnostic difficulty and lead to delay in commencing appropriate treatment. Case: An eight year old boy who presented with multiple ...

  6. Contemporary Management of Idiopathic Laryngotracheal Stenosis.

    Science.gov (United States)

    Donahoe, Laura; Keshavjee, Shaf

    2018-05-01

    Idiopathic laryngotracheal stenosis is a rare but well-described indication for subglottic tracheal resection. Initially described by Pearson in 1975, the 1-stage subglottic tracheal resection with reconstruction of the airway ensures preservation of the recurrent laryngeal nerves while resulting in an effective and durable repair of the stenosis. Copyright © 2018 Elsevier Inc. All rights reserved.

  7. Unusual Presentation Of Idiopathic Retroperitoneal Fibrosis: Case ...

    African Journals Online (AJOL)

    Idiopathic retroperitoneal fibrosis (IRF) is an uncommon entity described as progressive proliferation of connective tissues leading to a fibrous plaque-like lesions that encases the aorta and inferior vena cava inferior to the level of the renal arteries. Mass forming retroperitoneal fibrosis is rare. We present a rare case of a ...

  8. International Suspect Screening: NORMAN Suspect Exchange meets the US EPA CompTox Chemistry Dashboard (ICCE 2017 Oslo)

    Science.gov (United States)

    Members of the European NORMAN Network of Environmental Laboratories (www.norman-network.com) have many substance lists, including targets, suspects, surfactants, perfluorinated substances and regulated, partially confidential data sets of complex mixtures. The NORMAN Suspect Lis...

  9. Optimal management of idiopathic scoliosis in adolescence

    Directory of Open Access Journals (Sweden)

    Kotwicki T

    2013-07-01

    Full Text Available Tomasz Kotwicki,1 Joanna Chowanska,1,2 Edyta Kinel,3 Dariusz Czaprowski,4,5 Marek Tomaszewski,1 Piotr Janusz1 1Department of Pediatric Orthopedics, University of Medical Sciences, Poznan, Poland; 2National Scoliosis Foundation, Stoughton, MA, USA; 3Department of Rehabilitation, University of Medical Sciences, Poznan Poland; 4Department of Physiotherapy, Józef Rusiecki University College, Olsztyn, 5Rehasport Clinic, Poznan, Poland Abstract: Idiopathic scoliosis is a three-dimensional deformity of the growing spine, affecting 2%–3% of adolescents. Although benign in the majority of patients, the natural course of the disease may result in significant disturbance of body morphology, reduced thoracic volume, impaired respiration, increased rates of back pain, and serious esthetic concerns. Risk of deterioration is highest during the pubertal growth spurt and increases the risk of pathologic spinal curvature, increasing angular value, trunk imbalance, and thoracic deformity. Early clinical detection of scoliosis relies on careful examination of trunk shape and is subject to screening programs in some regions. Treatment options are physiotherapy, corrective bracing, or surgery for mild, moderate, or severe scoliosis, respectively, with both the actual degree of deformity and prognosis being taken into account. Physiotherapy used in mild idiopathic scoliosis comprises general training of the trunk musculature and physical capacity, while specific physiotherapeutic techniques aim to address the spinal curvature itself, attempting to achieve self-correction with active trunk movements developed in a three-dimensional space by an instructed adolescent under visual and proprioceptive control. Moderate but progressive idiopathic scoliosis in skeletally immature adolescents can be successfully halted using a corrective brace which has to be worn full time for several months or until skeletal maturity, and is able to prevent more severe deformity and avoid

  10. Rituximab in treatment of idiopathic glomerulopathy

    Directory of Open Access Journals (Sweden)

    Kamel El-Reshaid

    2012-01-01

    Full Text Available The aim of our study was to assess the role of rituximab (Mabthera in the treatment of patients with corticosteroid-resistant and calcineurin-inhibitors ± cellcept refractory idiopathic nephrotic syndrome (INS. A total of 83 patients who had required the previous treatment for a minimum of two years were included in the study. Our protocol included the use of rituximab in four-weekly slow infusions. Five patients were excluded as they could not tolerate rituximab infusion for allergic reaction. As expected, none of the patients had a decline in the total circulating lymphocyte counts yet all had achieved decline of their initially normal CD20 to < 0.5% one month after infusion. The decline persisted for eight to ten months later. In the minimal change disease (MCD group, 31 of the 32 patients had complete remission (CR and were off any immunosuppressive therapy and one of the previous non-responders (NR did not respond. Excluding two patients who had required retreatment, the others remained in CR (17 up to 28 months and six up to 36 months. Treatment with rituximab resulted in amelioration of NS in 17 of the 18 patients with focal segmental glomerulosclerosis (FSGS, while only one patient remained NR. Although renal function remained stable, proteinuria reappeared by eight to 12 months. Retreatment with rituximab resulted in a similar response with stable kidney function. In the 28 patients with membranous glomerulopathy (MG, 24 had achieved CR. Two patients failed to respond and two had partial remission. By 12 months, all patients relapsed. The response was within one month following treatment in patient with MCD, but was gradual within three months in FSGS and MG. Relapsers in all groups responded in a similar pattern to repeat dosing with the drug subsequently. Our prospective study represents an adequate number of patients with biopsy-proven subgroups of INS in both children and adults with long-term follow-up of treatment with rituximab

  11. Bone mineral density of girls with idiopathic scoliosis: a comparative study

    Directory of Open Access Journals (Sweden)

    Ameri E

    2012-11-01

    Full Text Available Background: Several studies have suggested higher incidence of osteoporosis in patients with idiopathic scoliosis in comparison with the normal population. The aim of this study was to assess the prevalence of low bone mass among adolescent girls with idiopathic scoliosis.Methods: In this cross-sectional study performed in shafa Hospital in Tehran, Iran during 2011-2012, we recruited fifty-seven 12- to-20-year old girls with idiopathic scoliosis and compared them with 100 age-matched healthy girls. The patients had no other diseases including neuromuscular disorders, congenital vertebral anomalies or a history of spinal surgery. Bone mineral densities (BMD of the hip and spine were evaluated and compared in all 157 participants using dual X-ray absorptiometry (DXA. Standard BMD (sBMD was also calculated at the lumbar spine. Results: Analysis of the data revealed that hip BMD was significantly (P=0.004 lower in patients with idiopathic scoliosis versus the controls. Moreover, BMD and sBMD of the Spine were also significantly lower in the patients (respectively, P=0.030 and P=0.030. Curve location had no effect on the values of hip BMD, spine BMD or spine sBMD (respectively, P=0.061 and P=0.274 and P=0.208.Finally, with more severe curves a lower bone mass was detected for sBMD and spine BMD (respectively, P=0.017 and P=0.016, but it was not significant for hip BMD (P=0.069.Conclusion: Adolescent girls with idiopathic scoliosis had lower bone mass compared with their healthy peers. The lower bone mass was correlated with the severity of the curve but not its location.

  12. Adult-onset photosensitivity: clinical significance and epilepsy syndromes including idiopathic (possibly genetic) photosensitive occipital epilepsy.

    Science.gov (United States)

    Koutroumanidis, Michalis; Tsirka, Vasiliki; Panayiotopoulos, Chrysostomos

    2015-09-01

    To evaluate the clinical associations of adult-onset photosensitivity, we studied the clinical and EEG data of patients who were referred due to a possible first seizure and who had a photoparoxysmal response on their EEG. Patients with clinical evidence of photosensitivity before the age of 20 were excluded. Of a total of 30 patients, four had acute symptomatic seizures, two had vasovagal syncope, and 24 were diagnosed with epilepsy. Nine of the 24 patients had idiopathic (genetic) generalized epilepsies and predominantly generalized photoparoxysmal response, but also rare photically-induced seizures, while 15 had exclusively, or almost exclusively, reflex photically-induced occipital seizures with frequent secondary generalization and posterior photoparoxysmal response. Other important differences included a significantly older age at seizure onset and paucity of spontaneous interictal epileptic discharges in patients with photically-induced occipital seizures; only a quarter of these had occasional occipital spikes, in contrast to the idiopathic (genetic) generalized epilepsy patients with typically generalized epileptic discharges. On the other hand, both groups shared a positive family history of epilepsy, common seizure threshold modulators (such as tiredness and sleep deprivation), normal neurological examination and MRI, a generally benign course, and good response to valproic acid. We demonstrated that photosensitivity can first occur in adult life and manifest, either as idiopathic (possibly genetic) photosensitive occipital epilepsy with secondary generalization or as an EEG, and less often, a clinical/EEG feature of idiopathic (genetic) generalized epilepsies. Identification of idiopathic photosensitive occipital epilepsy fills a diagnostic gap in adult first-seizure epileptology and is clinically important because of its good response to antiepileptic drug treatment and fair prognosis.

  13. Satisfactory patient-based outcomes after surgical treatment for idiopathic clubfoot: includes surgeon's individualized technique.

    Science.gov (United States)

    Mahan, Susan T; Spencer, Samantha A; Kasser, James R

    2014-09-01

    Treatment of idiopathic clubfoot has shifted towards Ponseti technique, but previously surgical management was standard. Outcomes of surgery have varied, with many authors reporting discouraging results. Our purpose was to evaluate a single surgeon's series of children with idiopathic clubfoot treated with a la carte posteromedial and lateral releases using the Pediatric Outcomes Data Collection Instrument (PODCI) with a minimum of 2-year follow-up. A total of 148 patients with idiopathic clubfoot treated surgically by a single surgeon over 15 years were identified, and mailed PODCI questionnaires. Fifty percent of the patients were located and responded, resulting in 74 complete questionnaires. Median age at surgery was 10 months (range, 5.3 to 84.7 mo), male sex 53/74 (71.6%), bilateral surgery 31/74 (41.9%), and average follow-up of 9.7 years. PODCI responses were compared with previously published normal healthy controls using t test for each separate category. Included in the methods is the individual surgeon's operative technique. In PODCIs where a parent reports for their child or adolescent, there was no difference between our data and the healthy controls in any of the 5 categories. In PODCI where an adolescent self-reports, there was no difference in 4 of 5 categories; significant difference was only found between our data (mean = 95.2; SD = 7.427) and normal controls (mean = 86.3; SD = 12.5) in Happiness Scale (P = 0.0031). In this group of idiopathic clubfoot patients, treated with judicious posteromedial release by a single surgeon, primarily when surgery was treatment of choice for clubfoot, patient-based outcomes are not different from their normal healthy peers through childhood and adolescence. While Ponseti treatment has since become the treatment of choice for clubfoot, surgical treatment, in some hands, has led to satisfactory results. Level III.

  14. Idiopathic dilated cardiomyopathy: computerized anatomic study of relashionship between septal and free left ventricle wall.

    Science.gov (United States)

    Juliani, Paulo Sérgio; Costa, Eder França da; Correia, Aristides Tadeu; Monteiro, Rosangela; Jatene, Fabio Biscegli

    2014-01-01

    A feature of dilated cardiomyopathy is the deformation of ventricular cavity, which contributes to systolic dysfunction. Few studies have evaluated this deformation bearing in mind ventricular regions and segments of the ventricle, which could reveal important details of the remodeling process, supporting a better understanding of its role in functional impairment and the development of new therapeutic strategies. To evaluate if, in basal, equatorial and apical regions, increased internal transverse perimeter of left ventricle in idiopathic dilated cardiomyopathy occurs proportionally between the septal and non-septal segment. We performed an anatomical study with 28 adult hearts from human cadavers. One group consisted of 18 hearts with idiopathic dilated cardiomyopathy and another group with 10 normal hearts. After lamination and left ventricle digital image capture, in three different regions (base, equator and apex), the transversal internal perimeter of left ventricle was divided into two segments: septal and not septal. These segments were measured by proper software. It was established an index of proportionality between these segments, called septal and non-septal segment index. Then we determined whether this index was the same in both groups. Among patients with normal hearts and idiopathic dilated cardiomyopathy, the index of proportionality between the two segments (septal and non-septal) showed no significant difference in the three regions analyzed. The comparison results of the indices NSS/SS among normal and enlarged hearts were respectively: in base 1.99 versus 1.86 (P=0.46), in equator 2.22 versus 2.18 (P=0.79) and in apex 2.96 versus 3.56 (P=0.11). In the idiopathic dilated cardiomyopathy, the transversal dilatation of left ventricular internal perimeter occurs proportionally between the segments corresponding to the septum and free wall at the basal, equatorial and apical regions of this chamber.

  15. Phenotypic spectrum of probable and genetically-confirmed idiopathic basal ganglia calcification.

    Science.gov (United States)

    Nicolas, Gaël; Pottier, Cyril; Charbonnier, Camille; Guyant-Maréchal, Lucie; Le Ber, Isabelle; Pariente, Jérémie; Labauge, Pierre; Ayrignac, Xavier; Defebvre, Luc; Maltête, David; Martinaud, Olivier; Lefaucheur, Romain; Guillin, Olivier; Wallon, David; Chaumette, Boris; Rondepierre, Philippe; Derache, Nathalie; Fromager, Guillaume; Schaeffer, Stéphane; Krystkowiak, Pierre; Verny, Christophe; Jurici, Snejana; Sauvée, Mathilde; Vérin, Marc; Lebouvier, Thibaud; Rouaud, Olivier; Thauvin-Robinet, Christel; Rousseau, Stéphane; Rovelet-Lecrux, Anne; Frebourg, Thierry; Campion, Dominique; Hannequin, Didier

    2013-11-01

    Idiopathic basal ganglia calcification is characterized by mineral deposits in the brain, an autosomal dominant pattern of inheritance in most cases and genetic heterogeneity. The first causal genes, SLC20A2 and PDGFRB, have recently been reported. Diagnosing idiopathic basal ganglia calcification necessitates the exclusion of other causes, including calcification related to normal ageing, for which no normative data exist. Our objectives were to diagnose accurately and then describe the clinical and radiological characteristics of idiopathic basal ganglia calcification. First, calcifications were evaluated using a visual rating scale on the computerized tomography scans of 600 consecutively hospitalized unselected controls. We determined an age-specific threshold in these control computerized tomography scans as the value of the 99th percentile of the total calcification score within three age categories: 60 years. To study the phenotype of the disease, patients with basal ganglia calcification were recruited from several medical centres. Calcifications that rated below the age-specific threshold using the same scale were excluded, as were patients with differential diagnoses of idiopathic basal ganglia calcification, after an extensive aetiological assessment. Sanger sequencing of SLC20A2 and PDGFRB was performed. In total, 72 patients were diagnosed with idiopathic basal ganglia calcification, 25 of whom bore a mutation in either SLC20A2 (two families, four sporadic cases) or PDGFRB (one family, two sporadic cases). Five mutations were novel. Seventy-one per cent of the patients with idiopathic basal ganglia calcification were symptomatic (mean age of clinical onset: 39 ± 20 years; mean age at last evaluation: 55 ± 19 years). Among them, the most frequent signs were: cognitive impairment (58.8%), psychiatric symptoms (56.9%) and movement disorders (54.9%). Few clinical differences appeared between SLC20A2 and PDGFRB mutation carriers. Radiological analysis

  16. Believable Suspect Agents: Response and Interpersonal Style Selection for an Artificial Suspect

    NARCIS (Netherlands)

    Bruijnes, Merijn

    2016-01-01

    The social skills necessary to properly and successfully conduct a police interrogation can and need to be trained. In the thesis I will describe the steps I took towards a virtual character that can play the role of a suspect in a police interrogation training. Students of the police academy will

  17. Comparison of endogenous and radiolabeled bile acid excretion in patients with idiopathic chronic diarrhea

    International Nuclear Information System (INIS)

    Schiller, L.R.; Bilhartz, L.E.; Santa Ana, C.A.

    1990-01-01

    Fecal recovery of radioactivity after ingestion of a bolus of radiolabeled bile acid is abnormally high in most patients with idiopathic chronic diarrhea. To evaluate the significance of this malabsorption, concurrent fecal excretion of both exogenous radiolabeled bile acid and endogenous (unlabeled) bile acid were measured in patients with idiopathic chronic diarrhea. Subjects received a 2.5-microCi oral dose of taurocholic acid labeled with 14C in the 24th position of the steroid moiety. Endogenous bile acid excretion was measured by a hydroxysteroid dehydrogenase assay on a concurrent 72-h stool collection. Both radiolabeled and endogenous bile acid excretion were abnormally high in most patients with chronic diarrhea compared with normal subjects, even when equivoluminous diarrhea was induced in normal subjects by ingestion of osmotically active solutions. The correlation between radiolabeled and endogenous bile acid excretion was good. However, neither radiolabeled nor endogenous bile acid excretion was as abnormal as is typically seen in patients with ileal resection, and none of these diarrhea patients responded to treatment with cholestyramine with stool weights less than 200 g. These results suggest (a) that this radiolabeled bile acid excretion test accurately reflects excess endogenous bile acid excretion; (b) that excess endogenous bile acid excretion is not caused by diarrhea per se; (c) that spontaneously occurring idiopathic chronic diarrhea is often associated with increased endogenous bile acid excretion; and (d) that bile acid malabsorption is not likely to be the primary cause of diarrhea in most of these patients

  18. Suspected invasive placenta: evaluation with magnetic resonance imaging

    International Nuclear Information System (INIS)

    Bour, Laurence; Place, Vinciane; Bendavid, Sandra; Fargeaudou, Yann; Portal, Jean-Jacques; Ricbourg, Aude; Sebbag, Delphine; Dohan, Anthony; Soyer, Philippe; Vicaut, Eric

    2014-01-01

    To determine the utility of magnetic resonance imaging (MRI) in diagnosing invasive placenta (IP). MRI findings in 32 women with suspected IP were evaluated independently by four readers. Interobserver agreement was calculated with kappa (κ) statistics. Associations between MRI findings and IP were assessed by univariate and multivariate analyses. Sensitivity, specificity and accuracy of MRI for the diagnosis of IP were estimated. Sixteen women (16/32; 50 %) had confirmed IP. Interobserver correlation for the diagnosis of IP was fair (κ = 0.40). Univariate analysis revealed that thinning or focal defect of the uteroplacental interface (P < 0.0001) was the most discriminating MRI variable in the differentiation between normal and IP. Overall sensitivity and specificity of MRI for the diagnosis of IP were 84 % [95 % CI: 75-94 %] and 80 % [95 % CI: 66-93 %], respectively. Thinning or focal defect of the uteroplacental interface was the most accurate finding (88 %) in the diagnosis of IP. Multivariate analysis revealed that thinning or focal defect of the uteroplacental interface was the single independent predictor of IP (P = 0.0006; OR = 64.99). MR imaging has 84 % sensitivity [95 % CI: 75-94 %] and 80 % specificity [95 % CI: 66-93 %] for the diagnosis of IP. Thinning or focal defect of the uteroplacental interface is the most discriminating independent MR variable in differentiating between normal placenta and IP. (orig.)

  19. Cost-effectiveness of routine imaging of suspected appendicitis.

    Science.gov (United States)

    D'Souza, N; Marsden, M; Bottomley, S; Nagarajah, N; Scutt, F; Toh, S

    2018-01-01

    Introduction The misdiagnosis of appendicitis and consequent removal of a normal appendix occurs in one in five patients in the UK. On the contrary, in healthcare systems with routine cross-sectional imaging of suspected appendicitis, the negative appendicectomy rate is around 5%. If we could reduce the rate in the UK to similar numbers, would this be cost effective? This study aimed to calculate the financial impact of negative appendicectomy at the Queen Alexandra Hospital and to explore whether a policy of routine imaging of such patients could reduce hospital costs. Materials and methods We performed a retrospective analysis of all appendicectomies over a 1-year period at our institution. Data were extracted on outcomes including appendix histology, operative time and length of stay to calculate the negative appendicectomy rate and to analyse costs. Results A total of 531 patients over 5 years of age had an appendicectomy. The negative appendicectomy rate was 22% (115/531). The additional financial costs of negative appendicectomy to the hospital during this period were £270,861. Universal imaging of all patients with right iliac fossa pain that could result in a 5% negative appendicectomy rate would cost between £67,200 and £165,600 per year but could save £33,896 (magnetic resonance imaging), £105,896 (computed tomography) or £132,296 (ultrasound) depending on imaging modality used. Conclusions Negative appendicectomy is still too frequent and results in additional financial burden to the health service. Routine imaging of patients with suspected appendicitis would not only reduce the negative appendicectomy rate but could lead to cost savings and a better service for our patients.

  20. Idiopathic pulmonary hemosiderosis: Alveoli are an answer to anemia

    Directory of Open Access Journals (Sweden)

    S Bhatia

    2011-01-01

    Full Text Available Idiopathic pulmonary hemosiderosis (IPH is a rare disorder (triad of iron-deficiency anemia, hemoptysis, and alveolar infiltrates. A 3-year-old male presented with mild fever, breathlessness, dry cough, and bluish nail discoloration for 8 days. He had required five blood transfusions in the past 1 year (last transfusion was given 4 months ago. He had a respiratory rate of 58/min with respiratory distress, cyanosis, and grade III clubbing. Respiratory system examination was normal. Several previous reports of hemoglobin were as low as 3.6 g/dl with hypochromic and microcytic anemia. There were transient increases in the hemoglobin and normalization of red cell morphology with blood transfusions. Serum iron, G6PD enzyme assay, hemoglobin electrophoresis, the sickling test, Coomb′s test, stool and urine analysis, and a Meckel′s scan were normal. HIV antibody and dsDNA were negative. The chest radiograph revealed symmetrical patchy infiltrates sparing lung apices (confirmed on high-resolution computed tomography. Lung biopsy diagnosed pulmonary hemosiderosis (interstitial lung disease with hemosiderin-laden macrophages scattered in the alveoli and areas of fibrosis in the alveolar septa. The patient showed marked clinical improvement in 10 days of therapy with prednisolone. IPH should be listed in the differential diagnosis of a child presenting with unexplained hypochromic, microcytic anemia and respiratory symptoms.

  1. The distribution of immunomodulatory cells in the lungs of patients with idiopathic pulmonary fibrosis

    Science.gov (United States)

    Nuovo, Gerard J.; Hagood, James S.; Magro, Cynthia M.; Chin, Nena; Kapil, Rubina; Davis, Luke; Marsh, Clay B.; Folcik, Virginia A.

    2011-01-01

    We have characterized the immune system involvement in the disease processes of idiopathic pulmonary fibrosis in novel ways. To do so, we analyzed lung tissue from 21 cases of idiopathic pulmonary fibrosis and 21 (non-fibrotic, non-cancerous) controls for immune cell and inflammation-related markers. The immunohistochemical analysis of the tissue was grouped by patterns of severity in disease pathology. There were significantly greater numbers of CD68+ and CD80+ cells, and significantly fewer CD3+, CD4+, and CD45RO+ cells in areas of relatively (histologically) normal lung in biopsies from idiopathic pulmonary fibrosis patients compared to controls. In zones of active disease, characterized by epithelial cell regeneration and fibrosis, there were significantly more cells expressing CD4, CD8, CD20, CD68, CD80, CCR6, S100, IL-17, tumor necrosis factor-α, and retinoic acid-related orphan receptors compared to histologically normal lung areas from idiopathic pulmonary fibrosis patients. Inflammation was implicated in these active regions by the cells that expressed retinoid orphan receptor-α, -β, and -γ, CCR6, and IL-17. The regenerating epithelial cells predominantly expressed these pro-inflammatory molecules, as evidenced by co-expression analyses with epithelial cytokeratins. Macrophages in pseudo-alveoli and CD3+ T cells in the fibrotic interstitium also expressed IL-17. Co-expression of IL-17 with retinoid orphan receptors, and epithelial cytoskeletal proteins, CD68, and CD3 in epithelial cells, macrophages, and T-cells, respectively, confirmed the production of IL-17 by these cell types. There was little staining for Foxp3, CD56, or CD34 in any idiopathic pulmonary fibrosis lung regions. The fibrotic regions had fewer immune cells overall. In summary, our study shows participation of innate and adaptive mononuclear cells in active-disease regions of idiopathic pulmonary fibrosis lung, where the regenerating epithelial cells appear to propagate inflammation

  2. Optimal management of idiopathic scoliosis in adolescence

    Science.gov (United States)

    Kotwicki, Tomasz; Chowanska, Joanna; Kinel, Edyta; Czaprowski, Dariusz; Tomaszewski, Marek; Janusz, Piotr

    2013-01-01

    Idiopathic scoliosis is a three-dimensional deformity of the growing spine, affecting 2%–3% of adolescents. Although benign in the majority of patients, the natural course of the disease may result in significant disturbance of body morphology, reduced thoracic volume, impaired respiration, increased rates of back pain, and serious esthetic concerns. Risk of deterioration is highest during the pubertal growth spurt and increases the risk of pathologic spinal curvature, increasing angular value, trunk imbalance, and thoracic deformity. Early clinical detection of scoliosis relies on careful examination of trunk shape and is subject to screening programs in some regions. Treatment options are physiotherapy, corrective bracing, or surgery for mild, moderate, or severe scoliosis, respectively, with both the actual degree of deformity and prognosis being taken into account. Physiotherapy used in mild idiopathic scoliosis comprises general training of the trunk musculature and physical capacity, while specific physiotherapeutic techniques aim to address the spinal curvature itself, attempting to achieve self-correction with active trunk movements developed in a three-dimensional space by an instructed adolescent under visual and proprioceptive control. Moderate but progressive idiopathic scoliosis in skeletally immature adolescents can be successfully halted using a corrective brace which has to be worn full time for several months or until skeletal maturity, and is able to prevent more severe deformity and avoid the need for surgical treatment. Surgery is the treatment of choice for severe idiopathic scoliosis which is rapidly progressive, with early onset, late diagnosis, and neglected or failed conservative treatment. The psychologic impact of idiopathic scoliosis, a chronic disease occurring in the psychologically fragile period of adolescence, is important because of its body distorting character and the onerous treatment required, either conservative or surgical

  3. Idiopathic facial pain related with dental implantation

    Directory of Open Access Journals (Sweden)

    Tae-Geon Kwon

    2016-06-01

    Full Text Available Chronic pain after dental implantation is rare but difficult issue for the implant practitioner. Patients with chronic pain who had been performed previous implant surgery or related surgical intervention sometimes accompany with psychological problem and difficult to adequately manage. According to the International Classification of Headache Disorders (ICHD 3rd eds, Cepalagia 2013, painful neuropathies and other facial pains are subdivided into the 12 subcategories; 13.1. Trigeminal neuralgia; 13.2 Glossopharyngeal neuralgia; 13.3 Nervus intermedius (facial nerve neuralgia; 13.4 Occipital neuralgia; 13.5 Optic neuritis; 13.6 Headache attributed to ischaemic ocular motor nerve palsy; 13.7 Tolosa-Hunt syndrome; 13.8 Paratrigeminal oculo-sympathetic (Raeder’s syndrome; 13.9 Recurrent painful ophthalmoplegic neuropathy; 13.10 Burning Mouth Syndrome (BMS; 13.11 Persistent Idiopathic Facial Pain (PIFP; 13.12 Central neuropathic pain. Chronic orofacial pain after dental implant surgery can be largely into the two main categories that can be frequently encountered in clinical basis ; 1 Neuropathic pain, 2 Idiopathic pain. If there is no direct evidence of the nerve injury related with the implant surgery, the clinician need to consider the central cause of pain instead of the peripheral cause of the pain. There might be several possibilities; 1 Anaesthesia dolorosa, 2 Central post-stroke pain, 3 Facial pain attributed to multiple sclerosis, 4 Persistent idiopathic facial pain (PIFP, 5 Burning mouth syndrome. In this presentation, Persistent idiopathic facial pain (PIFP, the disease entity that can be frequently encountered in the clinic would be discussed. Persistent idiopathic facial pain (PIFP can be defined as “persistent facial and/or oral pain, with varying presentations but recurring daily for more than 2 hours per day over more than 3 months, in the absence of clinical neurological deficit”. ‘Atypical’ pain is a diagnosis of

  4. Identification of a novel idiopathic epilepsy locus in Belgian Shepherd dogs

    DEFF Research Database (Denmark)

    Seppälä, Eija H.; Koskinen, Lotta L.E.; Gulløv, Christina Hedal

    2012-01-01

    Epilepsy is the most common neurological disorder in dogs, with an incidence ranging from 0.5% to up to 20% in particular breeds. Canine epilepsy can be etiologically defined as idiopathic or symptomatic. Epileptic seizures may be classified as focal with or without secondary generalization...... collected 159 cases and 148 controls and confirmed the presence of epilepsy through epilepsy questionnaires and clinical examinations. The MRI was normal while interictal EEG revealed abnormalities and variable foci in the clinically examined affected dogs. A genome-wide association study using Affymetrix...... mutation. It would establish the affected breed as a novel therapeutic model, help to develop a DNA test for breeding purposes and introduce a novel candidate gene for human idiopathic epilepsies....

  5. Nucleated red blood cells count in pregnancies with idiopathic intra-uterine growth restriction.

    Directory of Open Access Journals (Sweden)

    Fatemeh Davari-Tanha

    2014-06-01

    Full Text Available Elevated nucleated red blood cell (NRBC count is introduced as a potential marker of intra-uterine growth restriction (IUGR. To investigate the probable association regardless of any known underlying disease, we aimed to study disturbances in NRBC count in infants experiencing idiopathic IUGR.Twenty three infants regarded IUGR without any known cause were chosen to be compared to 48 normal neonates. Blood samples were collected instantly after birth and the same measurements were done in both groups.NRBC count/100 white blood cells was significantly higher in the IUGR group (P value < 0.001. pH measurements did not reveal any significant difference.Increased NRBC count in cases of idiopathic IUGR in absence of chronic hypoxia could strengthen its predictive value suggested in previous studies. It could help early IUGR detection and beneficial intervention.

  6. Cardiopulmonary Exercise Testing in Patients with Idiopathic Scoliosis.

    Science.gov (United States)

    Shen, Jianxiong; Lin, Youxi; Luo, Jinmei; Xiao, Yi

    2016-10-05

    Scoliosis causes impairment of the respiratory and cardiovascular systems. Traditional pulmonary function tests only examine patients under static conditions. The aim of our study was to investigate the correlation between radiographic parameters and dynamic cardiopulmonary capacity in patients with idiopathic scoliosis. Forty patients with idiopathic scoliosis were included in this prospective study from January 2014 to February 2016. The patients underwent full radiographic assessment of deformity, pulmonary function testing, and cardiopulmonary bicycle ergometer testing. The impact of the severity of thoracic curvature and kyphosis on pulmonary function and physical capacity was investigated. Thirty-three female patients with a mean age of 15.5 years (range, 11 to 35 years) and coronal thoracic curvature of 49.4° (range, 24° to 76°) and 7 male subjects with a mean age of 15.9 years (range, 13 to 18 years) and coronal thoracic curvature of 47.1°(range, 22° to 80°) were included. No correlation was found between coronal thoracic curvature and pulmonary function test results in the female patients. Female patients with a thoracic curve of ≥60° had lower blood oxygen saturation at maximal exercise in the cardiopulmonary exercise test (p = 0.032). Female patients with a thoracic curve of ≥50° had a higher respiratory rate (p = 0.041) and ventilation volume per minute (p = 0.046) and lower breathing reserve at maximal exercise (p = 0.038). Thoracic kyphosis in female patients was positively correlated with pulmonary function, as shown by the forced expiratory volume in 1 second (r = 0.456, p = 0.01), forced vital capacity (r = 0.366, p = 0.043), vital capacity (r = 0.525, p = 0.006), and total lung capacity (r = 0.388, p = 0.031), as well as with tidal volume (r = 0.401, p = 0.025) in cardiopulmonary exercise testing. Female patients who engaged in regular exercise had better peak oxygen intake normalized by body weight (p rate (p = 0.020), and heart rate

  7. IIH with normal CSF pressures?

    Directory of Open Access Journals (Sweden)

    Soh Youn Suh

    2013-01-01

    Full Text Available Idiopathic intracranial hypertension (IIH is a condition of raised intracranial pressure (ICP in the absence of space occupying lesions. ICP is usually measured by lumbar puncture and a cerebrospinal fluid (CSF pressure above 250 mm H 2 O is one of the diagnostic criteria of IIH. Recently, we have encountered two patients who complained of headaches and exhibited disc swelling without an increased ICP. We prescribed acetazolamide and followed both patients frequently; because of the definite disc swelling with IIH related symptoms. Symptoms and signs resolved in both patients after they started taking acetazolamide. It is generally known that an elevated ICP, as measured by lumbar puncture, is the most important diagnostic sign of IIH. However, these cases caution even when CSF pressure is within the normal range, that suspicion should be raised when a patient has papilledema with related symptoms, since untreated papilledema may cause progressive and irreversible visual loss.

  8. Exome Sequencing in Suspected Monogenic Dyslipidemias

    Science.gov (United States)

    Stitziel, Nathan O.; Peloso, Gina M.; Abifadel, Marianne; Cefalu, Angelo B.; Fouchier, Sigrid; Motazacker, M. Mahdi; Tada, Hayato; Larach, Daniel B.; Awan, Zuhier; Haller, Jorge F.; Pullinger, Clive R.; Varret, Mathilde; Rabès, Jean-Pierre; Noto, Davide; Tarugi, Patrizia; Kawashiri, Masa-aki; Nohara, Atsushi; Yamagishi, Masakazu; Risman, Marjorie; Deo, Rahul; Ruel, Isabelle; Shendure, Jay; Nickerson, Deborah A.; Wilson, James G.; Rich, Stephen S.; Gupta, Namrata; Farlow, Deborah N.; Neale, Benjamin M.; Daly, Mark J.; Kane, John P.; Freeman, Mason W.; Genest, Jacques; Rader, Daniel J.; Mabuchi, Hiroshi; Kastelein, John J.P.; Hovingh, G. Kees; Averna, Maurizio R.; Gabriel, Stacey; Boileau, Catherine; Kathiresan, Sekar

    2015-01-01

    Background Exome sequencing is a promising tool for gene mapping in Mendelian disorders. We utilized this technique in an attempt to identify novel genes underlying monogenic dyslipidemias. Methods and Results We performed exome sequencing on 213 selected family members from 41 kindreds with suspected Mendelian inheritance of extreme levels of low-density lipoprotein (LDL) cholesterol (after candidate gene sequencing excluded known genetic causes for high LDL cholesterol families) or high-density lipoprotein (HDL) cholesterol. We used standard analytic approaches to identify candidate variants and also assigned a polygenic score to each individual in order to account for their burden of common genetic variants known to influence lipid levels. In nine families, we identified likely pathogenic variants in known lipid genes (ABCA1, APOB, APOE, LDLR, LIPA, and PCSK9); however, we were unable to identify obvious genetic etiologies in the remaining 32 families despite follow-up analyses. We identified three factors that limited novel gene discovery: (1) imperfect sequencing coverage across the exome hid potentially causal variants; (2) large numbers of shared rare alleles within families obfuscated causal variant identification; and (3) individuals from 15% of families carried a significant burden of common lipid-related alleles, suggesting complex inheritance can masquerade as monogenic disease. Conclusions We identified the genetic basis of disease in nine of 41 families; however, none of these represented novel gene discoveries. Our results highlight the promise and limitations of exome sequencing as a discovery technique in suspected monogenic dyslipidemias. Considering the confounders identified may inform the design of future exome sequencing studies. PMID:25632026

  9. Retrospective panoramic radiographic analysis for idiopathic osteosclerosis in Indians

    Directory of Open Access Journals (Sweden)

    Srikanth H Srivathsa

    2016-01-01

    Full Text Available Introduction: Idiopathic osteosclerosis is an area of increased radiodensity observed on panoramic radiographs. The prevalence of this entity is not known, especially in Indians. Aims and Objectives: To determine the prevalence and epidemiological characteristics of idiopathic osteosclerosis. Materials and Methods: Six hundred and forty panoramic radiographs were retrospectively analyzed for the presence of idiopathic osteosclerosis by a single trained oral radiologist. Statistical Analysis: The data obtained were analyzed using Microsoft Excel (Version 2007 for Windows. Results: Idiopathic osteosclerosis was identified in 32 individuals with a prevalence of 5%. There were 21 female (65.7% and 11 male (34.3% participants. There were 31 single, unilateral (96.85% osteosclerotic lesions and 1 (3.15% bilateral lesion. Right side localization was noted in 19 participants (57.57% and left side localization in 14 participants (42.42%. Conclusion: This study illustrates the prevalence of idiopathic osteosclerosis in Indians. Further, it depicts the characteristics of idiopathic osteosclerotic lesions.

  10. Radiofrequency Catheter Ablation of Coexistent Idiopathic Left Ventricular Tachycardia and Atrioventricular Nodal Reentrant Tachycardia

    Directory of Open Access Journals (Sweden)

    Ken-Pen Weng

    2005-10-01

    Full Text Available A healthy 15-year-old male patient presented with a 6-month history of recurrent attacks of palpitations. On multiple emergency room visits, a sustained wide QRS complex tachycardia with a right bundle branch block and northwest axis deviation was documented. The tachycardia was not terminated by intravenous adenosine, but was suppressed with intravenous verapamil. There was no evidence of structural heart disease, myocarditis, long QT syndrome, or electrolyte imbalance after a series of standard examinations. Idiopathic left ventricular tachycardia (ILVT was suspected. Electrophysiologic studies revealed 2 inducible tachycardias, which were shown to represent atrioventricular nodal reentrant tachycardia (AVNRT and ILVT. Transformation from AVNRT to ILVT occurred spontaneously following atrial pacing. Successful ablation of ILVT and the slow atrioventricular nodal pathway resulted in cure of the double tachycardia.

  11. Duchenne muscular dystrophy and idiopathic hyperCKemia segregating in a family

    Energy Technology Data Exchange (ETDEWEB)

    Frydman, M.; Straussberg, R.; Shomrat, R.; Legum, C. [Tel Aviv Univ. (Israel)] [and others

    1995-09-11

    A 7-month-old boy with gross motor delay and failure to thrive presented with rhabdomyolysis following an acute asthmatic episode. During hospitalization an electrocardiographic conversion to a Wolff-Parkinson-White type 1 (WPW) pattern took place. Duchenne muscular dystrophy (DMD) was suspected based on elevated creatine kinase (CK) serum levels, muscle biopsy, and family history. The diagnosis was confirmed by molecular analysis, which documented a deletion corresponding to cDNA probe 1-2a in the dystrophin gene, in the propositus and in an affected male cousin of his mother. {open_quotes}Idiopathic{close_quotes} hyperCKemia was found in the propositus, his father, and 5 of his relatives. We suggest that the unusually early and severe manifestations of DMD in this patient may be related to the coincidental inheritance of the maternal DMD gene and of a paternal gene, causing hyperCKemia. 13 refs., 3 figs., 1 tab.

  12. Food-stimulated cholescintigraphy as a supplement to ERC in patients with suspected bile flow obstruction

    International Nuclear Information System (INIS)

    Hansen, H.H.; Toftgaard, C.; Rokkjor, M.J.; Kruse, A.; Funch-Jensen, P.; Thommesen, P.; Municipal Hospital, Aarhus

    1990-01-01

    Cholescintigraphy after food stimulation was carried out in 40 patients (13 patients with biliary enteric bypass, 14 patients with bile duct stenosis, demonstrated by ERC, 5 patients with endoprothesis and 8 patients with clinically suspected post-cholecystectomy syndrome). Biliary-bowel transit time of one hour or less was considered to be normal. It is concluded that in patients with biliary enteric bypass (hepatico-jejunostomia) or biliary strictures a biliary-bowel transit time of one hour will be discriminatory between normal and abnormal conditions. This is in contrast to patients with endoprothesis and suspected sphincter of Oddi dysmotility, where a transit time of one hour only will have limited predictive value. (orig.) [de

  13. Sacral root neuromodulation in idiopathic nonobstructive chronic urinary retention.

    Science.gov (United States)

    Shaker, H S; Hassouna, M

    1998-05-01

    Sacral root neuromodulation is becoming a superior alternative to the standard treatment of idiopathic nonobstructive urinary retention. We report results in 20 successive patients who underwent sacral foramen implantation to restore bladder function. After an initial, thorough baseline assessment 20 patients 19.43 to 55.66 years old with idiopathic nonobstructive urinary retention underwent percutaneous nerve evaluation. Response was assessed by a detailed voiding diary. Responders underwent implantation with an S3 foramen implant, and were followed 1, 3 and 6 months postoperatively, and every 6 months thereafter. Sacral root neuromodulation restored voiding capability in these patients. Bladders were emptied with minimal post-void residual urine, which decreased from 78.3 to 5.5 to 10.2% of the total voided volume from baseline to postoperative followup. These results were reflected in uroflowmetry and pressure-flow studies, which were almost normal after implantation. Furthermore, the urinary tract infection rate decreased significantly and associated pelvic pain improved substantially. The Beck depression inventory and SF-36 quality of life questionnaire indicated some improvement but reached significance in only 1 item. In addition, cystometrography showed no significant difference after 6 months of implantation compared with baseline values. Complications were minimal and within expectations. Sacral root neuromodulation is an appealing, successful modality for nonobstructive urinary retention. Only patients who have a good response to percutaneous nerve evaluation are candidates for implantation. The high efficacy in patients who undergo implantation, relative simplicity of the procedure and low complication rate make this a treatment breakthrough in this difficult group.

  14. Sex steroid receptor expression in idiopathic pulmonary fibrosis.

    Science.gov (United States)

    Mehrad, Mitra; Trejo Bittar, Humberto E; Yousem, Samuel A

    2017-08-01

    Usual interstitial pneumonia (UIP) is characterized by progressive scarring of the lungs and is associated with high morbidity and mortality despite therapeutic interventions. Sex steroid receptors have been demonstrated to play an important role in chronic lung conditions; however, their significance is unknown in patients with UIP. We retrospectively reviewed 40 idiopathic UIP cases for the expression of hormonal receptors. Forty cases including 10 normal lung, 10 cryptogenic organizing pneumonia, 10 idiopathic organizing diffuse alveolar damage, 7 hypersensitivity pneumonitis, and 3 nonspecific interstitial pneumonitis served as controls. Immunohistochemistry for estrogen receptor α, progesterone receptor (PR), and androgen receptor was performed in all groups. Expression of these receptors was assessed in 4 anatomic/pathologic compartments: alveolar and bronchiolar epithelium, arteries/veins, fibroblastic foci/airspace organization, and old scar. All UIPs (100%) stained positive for PR in myofibroblasts in the scarred areas, whereas among the control cases, only 1 nonspecific interstitial pneumonitis case stained focally positive and the rest were negative. PR was positive in myocytes of the large-sized arteries within the fibrotic areas in 31 cases (77.5%). PR was negative within the alveolar and bronchial epithelium, airspace organization, and center of fibroblastic foci; however, weak PR positivity was noted in the peripheral fibroblasts of the fibroblastic foci where they merged with dense fibrous connective tissue scar. All UIP and control cases were negative for androgen receptor and estrogen receptor α. This is the first study to show the expression of PR within the established fibrotic areas of UIP, indicating that progesterone may have profibrotic effects in UIP patients. Hormonal therapy by targeting PR could be of potential benefit in patients with UIP/IPF. Copyright © 2017 Elsevier Inc. All rights reserved.

  15. Genetic mutation in Korean patients of sudden cardiac arrest as a surrogating marker of idiopathic ventricular arrhythmia.

    Science.gov (United States)

    Son, Myoung Kyun; Ki, Chang-Seok; Park, Seung-Jung; Huh, June; Kim, June Soo; On, Young Keun

    2013-07-01

    Mutation or common intronic variants in cardiac ion channel genes have been suggested to be associated with sudden cardiac death caused by idiopathic ventricular tachyarrhythmia. This study aimed to find mutations in cardiac ion channel genes of Korean sudden cardiac arrest patients with structurally normal heart and to verify association between common genetic variation in cardiac ion channel and sudden cardiac arrest by idiopathic ventricular tachyarrhythmia in Koreans. Study participants were Korean survivors of sudden cardiac arrest caused by idiopathic ventricular tachycardia or fibrillation. All coding exons of the SCN5A, KCNQ1, and KCNH2 genes were analyzed by Sanger sequencing. Fifteen survivors of sudden cardiac arrest were included. Three male patients had mutations in SCN5A gene and none in KCNQ1 and KCNH2 genes. Intronic variant (rs2283222) in KCNQ1 gene showed significant association with sudden cardiac arrest (OR 4.05). Four male sudden cardiac arrest survivors had intronic variant (rs11720524) in SCN5A gene. None of female survivors of sudden cardiac arrest had SCN5A gene mutations despite similar frequencies of intronic variants between males and females in 55 normal controls. Common intronic variant in KCNQ1 gene is associated with sudden cardiac arrest caused by idiopathic ventricular tachyarrhythmia in Koreans.

  16. Idiopathic multiple aneurysm of external carotid artery

    Directory of Open Access Journals (Sweden)

    Saravanan Balachandran

    2014-01-01

    Full Text Available Aneurysms of external carotid artery are rare. Treatments for these are undertaken for the prevention of complications like hemorrhage or rupture, and embolism. We present a 71-year-old male with idiopathic multiple aneurysm for the past 34 years on conservative management and regular follow up for the past 4 years. This case was discussed for the rarity of idiopathic multiple aneurysm of the external carotid artery and the need for individualized treatment protocol to be followed as in this case, only watchful observation considering the age and patient compliance. In this world of evolving surgical techniques and newer treatment modalities, conservative treatment still has a role to play. Primary care physicians at the community level have a major role in following these patients and referring them as and when the need arises.

  17. Giant scrotal elephantiasis: an idiopathic case.

    Science.gov (United States)

    Dianzani, C; Gaspardini, F; Persichetti, P; Brunetti, B; Pizzuti, A; Margiotti, K; Degener, A M

    2010-01-01

    Scrotal elephantiasis is very rare disease in industrialized countries, where it is mainly due to surgery, irradiation or malignancies. It can be defined as idiopathic only when the possible congenital, infectious and compressive causes are excluded. We report a case of massive scrotal lymphoedema in an adult Caucasian patient, in Italy. He presented an extremely voluminous scrotal mass measuring 50 x 47 x 13 cm (weight 18 kg), which extended below his knees, invalidating all his daily activities. The patient was hospitalized in order to undergo to surgical treatment. Although genetic causes were searched and the possible role of infectious agents and compressive factors was evaluated, no etiology was ascertained. Histopathologic examination showed non-specific chronic inflammation, confirming the diagnosis of idiopathic elephantiasis. One year after surgical treatment, the patient is healthy without recurrence signs.

  18. Impact of juvenile idiopathic arthritis on schooling

    Directory of Open Access Journals (Sweden)

    Bouaddi Ilham

    2013-01-01

    Full Text Available Abstract Background Juvenile idiopathic arthritis (JIA is the most common arthropathy of childhood. Different diseases affect school attendance to varying degrees. The aim of this study was to assess the impact of juvenile idiopathic arthritis (JIA on Moroccan children’s schooling. Methods Thirty-three children with JIA were included in this study, having been previously diagnosed according to the classification criteria of the International League of Associations for Rheumatology (ILAR. Seventy-four healthy children were recruited to serve as controls. Data was obtained for all children on their school level, educational performance, and attendance. The rate of absenteeism due to health complications was noted. Results All healthy children were able to attend school (p Conclusions Our study suggested that the schooling of children with JIA was negatively impacted due to the disorder. More studies, with a larger sample of children, are needed to confirm our findings.

  19. US Evaluation of Juvenile Idiopathic Arthritis and Osteoarticular Infection.

    Science.gov (United States)

    Nguyen, Jie C; Lee, Kenneth S; Thapa, Mahesh M; Rosas, Humberto G

    2017-01-01

    Juvenile idiopathic arthritis (JIA) and osteoarticular infection can cause nonspecific articular and periarticular complaints in children. Although contrast material-enhanced magnetic resonance imaging is the reference standard imaging modality, musculoskeletal ultrasonography (US) is emerging as an important adjunct imaging modality that can provide valuable information relatively quickly without use of radiation or the need for sedation. However, diagnostic accuracy requires a systemic approach, familiarity with various US techniques, and an understanding of maturation-related changes. Specifically, the use of dynamic, Doppler, and/or multifocal US assessments can help confirm sites of disease, monitor therapy response, and guide interventions. In patients with JIA, ongoing synovial inflammation can lead to articular and periarticular changes, including synovitis, tenosynovitis, cartilage damage, bone changes, and enthesopathy. Although these findings can manifest in adult patients with rheumatoid arthritis, important differences and pitfalls exist because of the unique changes associated with an immature and maturing skeleton. In patients who are clinically suspected of having osteoarticular infection, the inability of US to evaluate the bone marrow decreases its sensitivity. Therefore, the US findings should be interpreted with caution because juxtacortical inflammation is suggestive, but neither sensitive nor specific, for underlying osteomyelitis. Similarly, the absence of a joint effusion makes septic arthritis extremely unlikely but not impossible. US findings of JIA and osteoarticular infection often overlap. Although certain clinical scenarios, laboratory findings, and imaging appearances can favor one diagnosis over the other, fluid analysis may still be required for definitive diagnosis and optimal treatment. US is the preferred modality for fluid aspiration and administering intra-articular corticosteroid therapy. © RSNA, 2017.

  20. Epithelial to mesenchymal transition-related proteins ZEB1, β-catenin, and β-tubulin-III in idiopathic pulmonary fibrosis.

    Science.gov (United States)

    Chilosi, Marco; Caliò, Anna; Rossi, Andrea; Gilioli, Eliana; Pedica, Federica; Montagna, Licia; Pedron, Serena; Confalonieri, Marco; Doglioni, Claudio; Ziesche, Rolf; Grubinger, Markus; Mikulits, Wolfgang; Poletti, Venerino

    2017-01-01

    Epithelial to mesenchymal transition has been suggested as a relevant contributor to pulmonary fibrosis, but how and where this complex process is triggered in idiopathic pulmonary fibrosis is not fully understood. Beta-tubulin-III (Tubβ3), ZEB1, and β-catenin are partially under the negative control of miR-200, a family of micro-RNAs playing a major role in epithelial to mesenchymal transition, that are reduced in experimental lung fibrosis and idiopathic pulmonary fibrosis. We wonder whether in situ expression of these proteins is increased in idiopathic pulmonary fibrosis, to better understand the significance of miR-200 feedback loop and epithelial to mesenchymal transition. We investigated the immunohistochemical and immunofluorescent expression and precise location of ZEB1, Tubβ3, and β-catenin in tissue samples from 34 idiopathic pulmonary fibrosis cases and 21 controls (5 normal lungs and 16 other interstitial lung diseases). In 100% idiopathic pulmonary fibrosis samples, the three proteins were concurrently expressed in fibroblastic foci, as well in damaged epithelial cells overlying these lesions and in pericytes within neo-angiogenesis areas. These results were also confirmed by immunofluorescence assay. In controls the abnormal expression of the three proteins was absent or limited. This is the first study that relates concurrent expression of Tubβ3, ZEB1, and β-catenin to abnormal epithelial and myofibroblast differentiation in idiopathic pulmonary fibrosis, providing indirect but robust evidence of miR-200 deregulation and epithelial to mesenchymal transition activation in idiopathic pulmonary fibrosis. The abnormal expression and localization of these proteins in bronchiolar fibro-proliferative lesions are unique for idiopathic pulmonary fibrosis, and might represent a disease-specific marker in challenging lung biopsies.

  1. Suspected primary immune deficiency in a Donge de Bordeaux dog : short communication

    Directory of Open Access Journals (Sweden)

    R.G. Lobetti

    2002-07-01

    Full Text Available A young Donge de Bordeaux dog was presented with chronic intermittent antibiotic responsive gastrointestinal and respiratory disease. Further evaluation showed bacterial lymphadenitis, bacterial tracheitis, normal white cell and differential cell counts, hypogammaglobulinaemia, and the absence of B-lymphocytes but the presence of T-lymphocytes in the lymphoid tissue stained with lymphocyte markers. As the dog came from a narrow genetic base, with related dogs showing similar clinical signs, possible B-cell congenital immune deficiency was suspected.

  2. Do We Need Exercise Tests to Detect Gas Exchange Impairment in Fibrotic Idiopathic Interstitial Pneumonias?

    Directory of Open Access Journals (Sweden)

    Benoit Wallaert

    2012-01-01

    Full Text Available In patients with fibrotic idiopathic interstitial pneumonia (f-IIP, the diffusing capacity for carbon monoxide (DLCO has been used to predict abnormal gas exchange in the lung. However, abnormal values for arterial blood gases during exercise are likely to be the most sensitive manifestations of lung disease. The aim of this study was to compare DLCO, resting PaO2, P(A-aO2 at cardiopulmonary exercise testing peak, and oxygen desaturation during a 6-min walk test (6MWT. Results were obtained in 121 patients with idiopathic pulmonary fibrosis (IPF, n=88 and fibrotic nonspecific interstitial pneumonias (NSIP, n=33. All but 3 patients (97.5% had low DLCO values (35 mmHg and 100 (83% demonstrated significant oxygen desaturation during 6MWT (>4%. Interestingly 27 patients had low DLCO and normal P(A-aO2, peak and/or no desaturation during the 6MWT. The 3 patients with normal DLCO also had normal PaO2, normal P(A-aO2, peak, and normal oxygen saturation during 6MWT. Our results demonstrate that in fibrotic IIP, DLCO better defines impairment of pulmonary gas exchange than resting PaO2, exercise P(A-aO2, peak, or 6MWT SpO2.

  3. An Update on Idiopathic Intracranial Hypertension

    OpenAIRE

    Thurtell, Matthew J.; Bruce, Beau B.; Newman, Nancy J.; Biousse, Valérie

    2010-01-01

    Idiopathic intracranial hypertension (IIH) is a condition of unknown etiology often encountered in neurologic practice. It produces non-localizing symptoms and signs of raised intracranial pressure and, when left untreated, can result in severe irreversible visual loss. It most commonly occurs in obese women of childbearing age, but it can also occur in children, men, non-obese adults, and older adults. While it is frequently associated with obesity, it can be associated with other conditions...

  4. Juvenile idiopathic arthritis-associated uveitis

    OpenAIRE

    Clarke, Sarah; Sen, Ethan; Ramanan, Athimalaipet

    2016-01-01

    Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease of childhood, with JIA-associated uveitis its most common extra-articular manifestation. JIA-associated uveitis is a potentially sight-threatening condition and thus carries a considerable risk of morbidity. The aetiology of the condition is autoimmune in nature with the predominant involvement of CD4(+) T cells. However, the underlying pathogenic mechanisms remain unclear, particularly regarding interplay between geneti...

  5. A case of idiopathic interstitial pneumonia

    OpenAIRE

    豊見山, 寛; 兼島, 洋; 下地, 克佳; 金城, 勇徳; 中富, 昌夫; 小張, 一峰; 松井, 克明; Tomiyama, Hiroshi; Kaneshima, Hiroshi; Shimoji, Katsuyoshi; Kinjo, Yutoku; Nakatomi, Masao; Kobari, Kazumine; Matsui, Katsuaki; 琉球大学医学部第一内科

    1982-01-01

    A case of idiopathic interstitial pneumonia was reported. A 53-year-old man was admitted to our hospital with his complaint of shortness of breath. On physical examination clubbing of the fingers was noticed and velcro rales was heard on the bilateral lower back. On laboratory data no remarkable finding was revealed. Chest roentgenogram showed diffuse reticulonodular shadow and small ring shadow of the bilateral lower lung fields. On pulmonary function test decline of VC and DLco was revealed...

  6. Idiopathic granulomatous hypophysitis: clinical and imaging features

    Energy Technology Data Exchange (ETDEWEB)

    Vasile, M. [Service de Radiologie, Hopital Saint-Antoine, 75 - Paris (France); Marsot-Dupuch, K. [Service de Radiologie, Hopital Saint-Antoine, 75 - Paris (France); Kujas, M. [Service d`Histologie Embryologie Cytogenetique, Hopital Pitie-Salpetriere, 75 - Paris (France); Brunereau, L. [Service de Radiologie, Hopital Saint-Antoine, 75 - Paris (France); Bouchard, P. [Service d`Histologie Embryologie Cytogenetique, Hopital Pitie-Salpetriere, 75 - Paris (France); Comoy, J. [Service de Neurochirurgie, Hopital Kremlin Bicetre, 94 (France); Tubiana, J.M. [Service de Radiologie, Hopital Saint-Antoine, 75 - Paris (France)

    1997-01-01

    Idiopathic pituitary granuloma is a rare disorder similar to lymphocytic adenohypophysitis. Few cases have been reported. We report a new histologically case proven with MRI. The patterns of clinical and radiological presentation and the management of this disorder are discussed. MRI findings suggestive of this condition include an intensely enhancing pituitary mass, associated with dural enhancement. Steroid therapy may be suggested avoiding unnecessary surgery. (orig.)

  7. Evaluation of the pituitary gland in idiopathic hypogonadotropic hypogonadism

    Energy Technology Data Exchange (ETDEWEB)

    Jayasundar, R.; Raghunathan, P. [All India Inst. of Medical Sciences, New Delhi (India). Dept. of NMR; Ammini, A.C. [All India Inst. of Medical Sciences, New Delhi (India). Dept. of Endocrinology and Metabolism; Gupta, R. [All India Inst. of Medical Sciences, New Delhi (India). Dept. of Radiodiagnosis

    1999-01-01

    Purpose: Evaluation of the pituitary gland has been carried out in idiopathic hypogonadotropic hypogonadism (IHH) to test the potential of MR imaging in differentiating IHH patients from normals. Material and Methods: Thirty-seven patients (aged 18-30 years), and 20 volunteers (aged 20-30 years) were studied by T1-weighted MR imaging. Length (L{sub A} and L{sub P}), height (H), width (W), area (A{sub A} and A{sub P}), and volume (V{sub 1A}, V{sub 1P}, V{sub 2A}, V{sub 2P}) of the pituitary gland were determined. (Subscripts P and A refer to measurements made with and without the posterior lobe, respectively.) V{sub 1} and V{sub 2} were estimated using two different methods. Results: in the control group, L{sub P}, W and V{sub 2A} exhibited significant differences between female and male volunteers. While W was the only parameter that did not show significant difference between normals and patients (in both men and women), all other parameters except L{sub P}, H and A{sub P} showed statistically significant differences between normals and IHH patients in both males and females. While L{sub P} was significantly different between normals and patients (men), H and A{sub P} were significantly different between normals and patients only in the female group. Conclusion: Correlation analysis between various parameters has shown that L{sub A} can be used for evaluating the pituitary in both the male and female IHH patients. (orig.)

  8. Ultrasonographic features of normal lower ureters

    International Nuclear Information System (INIS)

    Kim, Young Soon; Bae, M. Y.; Park, K. J.; Jeon, H. S.; Lee, J. H.

    1990-01-01

    Although ultrasonographic evaluation of the normal ureters is difficult due to bowel gas, the lower segment of the normal ureters can be visualized using the urinary bladder as an acoustic window. Authors prospetively performed ultrasonography with the standard suprapubic technique and analyzed the ultrasonographic features of normal lower ureters in 79 cases(77%). Length of visualized segment of the distal ureter ranged frp, 1.5cm to 7.2 cm and the visualized segment did not exceed 3.9mm in maximum diameter. Knowledge of sonographic features of the normal lower ureters can be helpful in the evaluation of pathologic or suspected pathologic conditions of the lower ureters

  9. Pulmonary function in children with idiopathic scoliosis

    Directory of Open Access Journals (Sweden)

    Tsiligiannis Theofanis

    2012-03-01

    Full Text Available Abstract Idiopathic scoliosis, a common disorder of lateral displacement and rotation of vertebral bodies during periods of rapid somatic growth, has many effects on respiratory function. Scoliosis results in a restrictive lung disease with a multifactorial decrease in lung volumes, displaces the intrathoracic organs, impedes on the movement of ribs and affects the mechanics of the respiratory muscles. Scoliosis decreases the chest wall as well as the lung compliance and results in increased work of breathing at rest, during exercise and sleep. Pulmonary hypertension and respiratory failure may develop in severe disease. In this review the epidemiological and anatomical aspects of idiopathic scoliosis are noted, the pathophysiology and effects of idiopathic scoliosis on respiratory function are described, the pulmonary function testing including lung volumes, respiratory flow rates and airway resistance, chest wall movements, regional ventilation and perfusion, blood gases, response to exercise and sleep studies are presented. Preoperative pulmonary function testing required, as well as the effects of various surgical approaches on respiratory function are also discussed.

  10. Neurodevelopment in preschool idiopathic toe-walkers.

    Science.gov (United States)

    Martín-Casas, P; Ballestero-Pérez, R; Meneses-Monroy, A; Beneit-Montesinos, J V; Atín-Arratibel, M A; Portellano-Pérez, J A

    2017-09-01

    Idiopathic toe walking, a differential diagnosis for neurological and orthopaedic disorders, has been associated with neurodevelopmental alterations. Neurodevelopmental assessment at early ages using specific tests may improve management and follow-up of these patients. The aim of our study is to analyse the neurodevelopmental characteristics of preschool idiopathic toe-walkers (ITW) by comparing them to a control group. Our descriptive cross-sectional study compared possible risk factors, neurodevelopmental characteristics, and scores on the Child Neuropsychological Maturity Questionnaire (CUMANIN) between a group of 56 ITWs aged 3 to 6 and a control group including 40 children. The proportion of males was significantly higher in the ITW group (P=.008). The percentage of patients with a family history (P=.000) and biological risk factors during the perinatal period (P=.032) was also higher in this group. According to the parents' reports, motor coordination in ITWs was significantly poorer (59%; P=.009). ITWs scored significantly lower on CUMANIN subscales of psychomotricity (=0,001) and memory (P=.001), as well as in verbal development (P=.000), non-verbal development (P=.026), and overall development (P=.004). Foot preference was less marked in the ITW group (P=.047). The neurodevelopmental characteristics of our sample suggest that idiopathic toe walking is a marker of neurodevelopmental impairment. However, further studies are necessary to confirm these findings. Copyright © 2016 Sociedad Española de Neurología. Publicado por Elsevier España, S.L.U. All rights reserved.

  11. Interventional Radiologic Treatment for Idiopathic Portal Hypertension

    International Nuclear Information System (INIS)

    Hirota, Shozo; Ichikawa, Satoshi; Matsumoto, Shinichi; Motohara, Tomofumi; Fukuda, Tetsuya; Yoshikawa, Takeshi

    1999-01-01

    Purpose: To evaluate the usefulness of interventional radiological treatment for idiopathic portal hypertension. Methods: Between 1995 and 1998, we performed an interventional radiological treatment in five patients with idiopathic portal hypertension, four of whom had refused surgery and one of whom had undergone surgery. Three patients with gastroesophageal varices (GEV) were treated by partial splenic embolization (PSE), one patient with esophageal varices (EV) and massive ascites by transjugular intrahepatic portosytemic shunt (TIPS) and PSE, and one patient with GEV by percutaneous transhepatic obliteration (PTO). Midterm results were analyzed in terms of the effect on esophageal and/or gastric varices. Results: In one woman with severe GEV who underwent three sessions of PSE, there was endoscopic confirmation that the GEV had disappeared. In one man his EV shrunk markedly after two sessions of PSE. In two patients slight reduction of the EV was obtained with one application of PSE combined with endoscopic variceal ligation therapy. PTO for GV in one patient resulted in good control of the varices. All patients have survived for 16-42 months since the first interventional treatment, and varices are well controlled. Conclusion: Interventional radiological treatment is effective for patients with idiopathic portal hypertension, whether or not they have undergone surgery

  12. Depressed mood, positive affect, and heart rate variability in patients with suspected coronary artery disease.

    Science.gov (United States)

    Bhattacharyya, Mimi R; Whitehead, Daisy L; Rakhit, Roby; Steptoe, Andrew

    2008-11-01

    To test associations between heart rate variability (HRV), depressed mood, and positive affect in patients with suspected coronary artery disease (CAD). Depression is associated with impaired HRV post acute cardiac events, but evidence in patients with stable coronary artery disease (CAD) is inconsistent. Seventy-six patients (52 men, 24 women; mean age = 61.1 years) being investigated for suspected CAD on the basis of symptomatology and positive noninvasive tests, completed 24-hour electrocardiograms. The Beck Depression Inventory (BDI) was administered, and positive and depressed affect was measured over the study period with the Day Reconstruction Method (DRM). A total of 46 (60.5%) patients were later found to have definite CAD. HRV was analyzed, using spectral analysis. Typical diurnal profiles of HRV were observed, with greater normalized high frequency (HF) and lower normalized low frequency (LF) power in the night compared with the day. BDI depression scores were not consistently associated with HRV. But positive affect was associated with greater normalized HF power (p = .039) and reduced normalized LF power (p = .007) independently of age, gender, medication with beta blockers, CAD status, body mass index, smoking, and habitual physical activity level. In patients with definite CAD, depressed affect assessed using the DRM was associated with reduced normalized HF power and heightened normalized LF power (p = .007) independently of covariates. Relationships between depression and HRV in patients with CAD may depend on affective experience over the monitoring period. Enhanced parasympathetic cardiac control may be a process through which positive affect protects against cardiovascular disease.

  13. Lineup composition, suspect position, and the sequential lineup advantage.

    Science.gov (United States)

    Carlson, Curt A; Gronlund, Scott D; Clark, Steven E

    2008-06-01

    N. M. Steblay, J. Dysart, S. Fulero, and R. C. L. Lindsay (2001) argued that sequential lineups reduce the likelihood of mistaken eyewitness identification. Experiment 1 replicated the design of R. C. L. Lindsay and G. L. Wells (1985), the first study to show the sequential lineup advantage. However, the innocent suspect was chosen at a lower rate in the simultaneous lineup, and no sequential lineup advantage was found. This led the authors to hypothesize that protection from a sequential lineup might emerge only when an innocent suspect stands out from the other lineup members. In Experiment 2, participants viewed a simultaneous or sequential lineup with either the guilty suspect or 1 of 3 innocent suspects. Lineup fairness was varied to influence the degree to which a suspect stood out. A sequential lineup advantage was found only for the unfair lineups. Additional analyses of suspect position in the sequential lineups showed an increase in the diagnosticity of suspect identifications as the suspect was placed later in the sequential lineup. These results suggest that the sequential lineup advantage is dependent on lineup composition and suspect position. (c) 2008 APA, all rights reserved

  14. Malware Normalization

    OpenAIRE

    Christodorescu, Mihai; Kinder, Johannes; Jha, Somesh; Katzenbeisser, Stefan; Veith, Helmut

    2005-01-01

    Malware is code designed for a malicious purpose, such as obtaining root privilege on a host. A malware detector identifies malware and thus prevents it from adversely affecting a host. In order to evade detection by malware detectors, malware writers use various obfuscation techniques to transform their malware. There is strong evidence that commercial malware detectors are susceptible to these evasion tactics. In this paper, we describe the design and implementation of a malware normalizer ...

  15. Platelet antibody in prolonged remission of childhood idiopathic thrombocytopenic purpura

    International Nuclear Information System (INIS)

    Ware, R.; Kinney, T.R.; Rosse, W.

    1985-01-01

    Evaluations were performed in 20 patients with childhood idiopathic thrombocytopenic purpura (ITP) who remained in remission longer than 12 months. The mean duration of follow-up from diagnosis was 39 months (range 17 to 87 months). Eleven patients (four girls) in group 1 had an acute course of ITP, defined as platelet count greater than 150 X 10(9)/L within 6 months of diagnosis. Nine patients (five girls) in group 2 had a chronic course, defined as platelet count less than 150 X 10(9)/L for greater than or equal to 1 year or requiring splenectomy in an attempt to control hemorrhagic symptoms. Platelet count and serum (indirect) platelet-associated IgG (PAIgG) levels were normal in all 20 patients at follow-up. Both direct and indirect PAIgG levels were measured using a 125 I-monoclonal anti-IgG antiglobulin assay. All had normal direct PAIgG levels, except for one patient in group 1 who had a borderline elevated value of 1209 molecules per platelet. These data suggest that the prevalence of elevated platelet antibodies is low during sustained remission without medication in patients with a history of childhood ITP. These data may be relevant for pregnant women with a history of childhood ITP, with regard to the risk of delivering an infant with thrombocytopenia secondary to transplacental passage of maternal platelet antibody

  16. MRI of the facial nerve in idiopathic facial palsy

    International Nuclear Information System (INIS)

    Saatci, I.; Sahintuerk, F.; Sennaroglu, L.; Boyvat, F.; Guersel, B.; Besim, A.

    1996-01-01

    The purpose of this prospective study was to define the enhancement pattern of the facial nerve in idiopathic facial paralysis (Bell's palsy) on magnetic resonance (MR) imaging with routine doses of gadolinium-DTPA (0.1 mmol/kg). Using 0.5 T imager, 24 patients were examined with a mean interval time of 13.7 days between the onset of symptoms and the MR examination. Contralateral asymptomatic facial nerves constituted the control group and five of the normal facial nerves (20.8%) showed enhancement confined to the geniculate ganglion. Hence, contrast enhancement limited to the geniculate ganglion in the abnormal facial nerve (3 of 24) was referred to a equivocal. Not encountered in any of the normal facial nerves, enhancement of other segments alone or associated with geniculate ganglion enhancement was considered to be abnormal and noted in 70.8% of the symptomatic facial nerves. The most frequently enhancing segments were the geniculate ganglion and the distal intracanalicular segment. (orig.)

  17. MRI of the facial nerve in idiopathic facial palsy

    Energy Technology Data Exchange (ETDEWEB)

    Saatci, I. [Dept. of Radiology, Hacettepe Univ., Hospital Sihhiye, Ankara (Turkey); Sahintuerk, F. [Dept. of Radiology, Hacettepe Univ., Hospital Sihhiye, Ankara (Turkey); Sennaroglu, L. [Dept. of Otolaryngology, Head and Neck Surgery, Hacettepe Univ., Hospital Sihhiye, Ankara (Turkey); Boyvat, F. [Dept. of Radiology, Hacettepe Univ., Hospital Sihhiye, Ankara (Turkey); Guersel, B. [Dept. of Otolaryngology, Head and Neck Surgery, Hacettepe Univ., Hospital Sihhiye, Ankara (Turkey); Besim, A. [Dept. of Radiology, Hacettepe Univ., Hospital Sihhiye, Ankara (Turkey)

    1996-10-01

    The purpose of this prospective study was to define the enhancement pattern of the facial nerve in idiopathic facial paralysis (Bell`s palsy) on magnetic resonance (MR) imaging with routine doses of gadolinium-DTPA (0.1 mmol/kg). Using 0.5 T imager, 24 patients were examined with a mean interval time of 13.7 days between the onset of symptoms and the MR examination. Contralateral asymptomatic facial nerves constituted the control group and five of the normal facial nerves (20.8%) showed enhancement confined to the geniculate ganglion. Hence, contrast enhancement limited to the geniculate ganglion in the abnormal facial nerve (3 of 24) was referred to a equivocal. Not encountered in any of the normal facial nerves, enhancement of other segments alone or associated with geniculate ganglion enhancement was considered to be abnormal and noted in 70.8% of the symptomatic facial nerves. The most frequently enhancing segments were the geniculate ganglion and the distal intracanalicular segment. (orig.)

  18. Ghrelin plasma levels in patients with idiopathic short stature.

    Science.gov (United States)

    Iñiguez, Germán; Román, Rossana; Youlton, Ronald; Cassorla, Fernando; Mericq, Verónica

    2011-02-01

    Novel molecular insights have suggested that ghrelin may be involved in the pathogenesis of some forms of short stature. Recently, growth hormone secretagogue receptor (GHSR) mutations that segregate with short stature have been reported. To study plasma ghrelin levels in prepubertal patients with idiopathic short stature (ISS). Fasting total plasma ghrelin levels (radioimmunoassay) in 41 prepubertal patients with ISS (18 females, age 7.9 ± 0.5 years) compared with 42 age- and sex-matched controls (27 females, age 8.0 ± 0.3 years) with normal height. In a subset of 28 patients, the ghrelin receptor was sequenced. ISS patients exhibited a higher level of ghrelin (1,458 ± 137 vs. 935 ± 55 pg/ml, p ghrelin levels greater than +2 SDS compared to controls. These patients did not differ in height, BMI or IGF-I SDS compared to ISS patients with ghrelin levels within the normal range. Molecular analysis of GHSR did not show any mutations, but showed some polymorphisms. These results suggest that in ISS patients, short stature does not appear to be frequently caused by abnormalities in ghrelin signaling. Copyright © 2010 S. Karger AG, Basel.

  19. Audiovestibular function in patients with juvenile idiopathic arthritis (JIA).

    Science.gov (United States)

    El-Gharib, Amani Mohamed; El-Barbary, Amal Mohamad; Aboelhawa, Marwa Ahmed; Elkholy, Radwa Mostafa

    2016-10-01

    It was found that JIR children had potential sensory neural hearing loss and vestibular affection. Therefore, this study recommends: early complete audiologic evaluation of JIA child followed by regular follow-up, including TOAEs, extended high-frequency audiometry, and VNG. This follow-up is important for preliminary diagnosis and management in order to prevent the negative impact of hearing loss on a child's life. The aim of this study was to assess hearing in children with Juvenile idiopathic arthritis (JIA) and compare them with a healthy control group. In addition to conventional audiometry, extended high-frequency audiometry and Transient otoacoustic emission (TOAEs) were used. This study also tried to investigate the vestibular function in JIR children by videonystagmography (VNG). The study group comprised of 28 children with JIR and 28 healthy children. All subjects were examined audiologically using basic audiological evaluation, high-frequency audiometry, TOAEs, and VNG. Children with JIR had apparent normal peripheral hearing in conventional audiometry; sub-clinical sensory neural hearing loss was detected. This sub-clinical hearing loss appeared in statistically significant difference between them and normal in high-frequency audiometry and TOAEs. VNG test results showed affected tracking and second tests.

  20. Relating gastric scintigraphy and symptoms to motility capsule transit and pressure findings in suspected gastroparesis.

    Science.gov (United States)

    Hasler, W L; May, K P; Wilson, L A; Van Natta, M; Parkman, H P; Pasricha, P J; Koch, K L; Abell, T L; McCallum, R W; Nguyen, L A; Snape, W J; Sarosiek, I; Clarke, J O; Farrugia, G; Calles-Escandon, J; Grover, M; Tonascia, J; Lee, L A; Miriel, L; Hamilton, F A

    2018-02-01

    Wireless motility capsule (WMC) findings are incompletely defined in suspected gastroparesis. We aimed to characterize regional WMC transit and contractility in relation to scintigraphy, etiology, and symptoms in patients undergoing gastric emptying testing. A total of 209 patients with gastroparesis symptoms at NIDDK Gastroparesis Consortium centers underwent gastric scintigraphy and WMCs on separate days to measure regional transit and contractility. Validated questionnaires quantified symptoms. Solid scintigraphy and liquid scintigraphy were delayed in 68.8% and 34.8% of patients; WMC gastric emptying times (GET) were delayed in 40.3% and showed 52.8% agreement with scintigraphy; 15.5% and 33.5% had delayed small bowel (SBTT) and colon transit (CTT) times. Transit was delayed in ≥2 regions in 23.3%. Rapid transit was rarely observed. Diabetics had slower GET but more rapid SBTT versus idiopathics (P ≤ .02). GET delays related to greater scintigraphic retention, slower SBTT, and fewer gastric contractions (P ≤ .04). Overall gastroparesis symptoms and nausea/vomiting, early satiety/fullness, bloating/distention, and upper abdominal pain subscores showed no relation to WMC transit. Upper and lower abdominal pain scores (P ≤ .03) were greater with increased colon contractions. Constipation correlated with slower CTT and higher colon contractions (P = .03). Diarrhea scores were higher with delayed SBTT and CTT (P ≤ .04). Wireless motility capsules define gastric emptying delays similar but not identical to scintigraphy that are more severe in diabetics and relate to reduced gastric contractility. Extragastric transit delays occur in >40% with suspected gastroparesis. Gastroparesis symptoms show little association with WMC profiles, although lower symptoms relate to small bowel or colon abnormalities. © 2017 John Wiley & Sons Ltd.

  1. STATUS GIZI DAN STATUS IODIUM PADA BALITA DENGAN SUSPECT DOWN SYNDROME

    Directory of Open Access Journals (Sweden)

    Hastin Dyah K.

    2012-10-01

    Full Text Available Background: Nutritional status is one of the factor that determines the human resources and quality of life. Toddlers are one of vulnerable groups for nutritional problems. One of the nutrition problem in Indonesia is Iodine deficiency disorder. Clinical manifestations are not only endemic goiter and cretins, but also abortion, stillbirth, and congenital abnormalities. Congenital abnormalities are commonly found in areas ofendemic iodine deficiency disorder such as Down Syndrome. Objectives: This study aims to determine the iodine nutrition status of children under jive with Down Syndrome Suspect in endemic areas ofiodine deficiency disorder. Method: This research is descriptive study with cross sectional design. The study was conducted in Magelang district, which is endemic iodine deficiency disorder. Total sample of30 under jive years old with Down Syndrome Suspect. Result: The result showed that the nutritional status of respondents based on the weight/age index was 50% had good nutritional status. According to height/age index as much as 70% are short and very short. Based on weight/height index was 73,3% with normal nutritional status. At least 33% of children with Down Syndrome Suspect accompanied hypothyroidism.  Keywords: Suspect Down Syndrome, Nutritional Status, Iodine Status

  2. Optimisation of the MR protocol in pregnant women with suspected acute appendicitis

    Energy Technology Data Exchange (ETDEWEB)

    Shin, Ilah; Chung, Yong Eun; An, Chansik; Kim, Honsoul; Lim, Joon Seok; Kim, Myeong-Jin [Yonsei University College of Medicine, Department of Radiology, Research Institute of Radiological Science, Severance Hospital, Seoul (Korea, Republic of); Lee, Hye Sun [Yonsei University College of Medicine, Department of Research Affairs, Biostatistics Collaboration Unit, Seoul (Korea, Republic of)

    2018-02-15

    To investigate the optimal magnetic resonance (MR) imaging protocol in pregnant women suspected of having acute appendicitis. One hundred and forty-six pregnant women with suspected appendicitis were included. MR images were reviewed by two radiologists in three separate sessions. In session 1, only axial single-shot turbo spin echo (SSH-TSE) T2-weighted images (WI) were included with other routine sequences. In sessions 2 and 3, coronal and sagittal T2WI were sequentially added. The visibility of the appendix and diagnostic confidence of appendicitis were evaluated in each session using a 5-point grading scale. If diseases other than appendicitis were suspected, specific diagnosis with a 5-point confidence scale was recorded. Diagnostic performance for appendicitis and other diseases were evaluated. Twenty-five patients (17.1%) were diagnosed with appendicitis. Among the patients with normal appendix, 28 were diagnosed with other disease. Diagnostic performance including sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and area under the curve values for diagnosing appendicitis and other diseases showed no significant difference among sets for both reviewers (p>0.05). Diagnostic performance of MR in pregnant patients with suspected appendicitis can be preserved with omission of sagittal or both coronal and sagittal SSH-T2WI. (orig.)

  3. Unexpected MRI findings in clinically suspected Legg-Calve-Perthes disease

    Energy Technology Data Exchange (ETDEWEB)

    Lobert, Philip F.; Dillman, Jonathan R.; Strouse, Peter J.; Hernandez, Ramiro J. [University of Michigan Health System, Department of Radiology, Section of Pediatric Radiology, C.S. Mott Children' s Hospital/F3503, Ann Arbor, MI (United States)

    2011-03-15

    In the setting of clinically suspected Legg-Calve-Perthes (LCP) disease and negative/equivocal radiographs, contrast-enhanced MRI can be performed to confirm the diagnosis. To determine the frequency of unexpected causes of hip pain as identified by MRI in children with clinically suspected LCP disease and negative/equivocal radiographs. All pediatric contrast-enhanced MRI examinations of the pelvis and hips performed between January 2000 and February 2009 to evaluate for possible LCP disease in the setting of negative/equivocal radiographs were identified. MRI examinations performed to evaluate for secondary avascular necrosis were excluded. Imaging reports were retrospectively reviewed for unexpected clinically important causes of hip pain. Thirty-six pediatric patients underwent contrast-enhanced MRI examinations for clinically suspected LCP disease in the setting of negative/equivocal radiographs. Twenty-two (61%) imaging studies were normal, while four (11%) imaging studies demonstrated findings consistent with LCP disease. Ten (28%) imaging studies revealed unexpected clinically important causes of hip pain, including nonspecific unilateral joint effusion and synovitis (n = 7, juvenile chronic arthritis was eventually diagnosed in 3 patients), sacral fracture (n = 1), apophyseal injury (n = 1), and femoral head subluxation (n = 1). MRI frequently reveals unexpected clinically important causes of hip pain in children with suspected LCP disease and negative/equivocal radiographs. (orig.)

  4. Optimisation of the MR protocol in pregnant women with suspected acute appendicitis

    International Nuclear Information System (INIS)

    Shin, Ilah; Chung, Yong Eun; An, Chansik; Kim, Honsoul; Lim, Joon Seok; Kim, Myeong-Jin; Lee, Hye Sun

    2018-01-01

    To investigate the optimal magnetic resonance (MR) imaging protocol in pregnant women suspected of having acute appendicitis. One hundred and forty-six pregnant women with suspected appendicitis were included. MR images were reviewed by two radiologists in three separate sessions. In session 1, only axial single-shot turbo spin echo (SSH-TSE) T2-weighted images (WI) were included with other routine sequences. In sessions 2 and 3, coronal and sagittal T2WI were sequentially added. The visibility of the appendix and diagnostic confidence of appendicitis were evaluated in each session using a 5-point grading scale. If diseases other than appendicitis were suspected, specific diagnosis with a 5-point confidence scale was recorded. Diagnostic performance for appendicitis and other diseases were evaluated. Twenty-five patients (17.1%) were diagnosed with appendicitis. Among the patients with normal appendix, 28 were diagnosed with other disease. Diagnostic performance including sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and area under the curve values for diagnosing appendicitis and other diseases showed no significant difference among sets for both reviewers (p>0.05). Diagnostic performance of MR in pregnant patients with suspected appendicitis can be preserved with omission of sagittal or both coronal and sagittal SSH-T2WI. (orig.)

  5. Improving the diagnostic performance of lung scintigraphy in suspected pulmonary embolic disease

    International Nuclear Information System (INIS)

    Gleeson, F.V.; Turner, S.; Scarsbrook, A.F.

    2006-01-01

    Aim: to determine the effectiveness of a new imaging algorithm in the investigation of suspected pulmonary embolism (PE). Materials and methods: A new imaging algorithm for suspected PE was introduced following the installation of a multisection computed tomography (CT) machine at our institution. Before its installation, patients with suspected PE were evaluated with ventilation/perfusion (V/Q) scintigraphy. Subsequently, patients were triaged according to chest radiography (CR) and respiratory history to either lung scintigraphy or CT pulmonary angiography (CTPA). Patients with a normal CR and no history of lung disease were evaluated using perfusion (Q) scintigraphy [ventilation (V) scintigraphy was no longer performed]. Patients with an abnormal CR, asthma or chronic lung disease were evaluated using CTPA. All V/Q images in a continuous 3-year period before the introduction of the new imaging algorithm and all Q images performed in a 3-year period after its introduction were retrospectively reviewed. Imaging reports were categorized into normal, non-diagnostic (low or intermediate probability) or high probability for PE. Patients in the later group who subsequently underwent CTPA, were also reviewed. Results: After the policy change the percentage of normal scintigrams significantly increased (39 to 60%; p < 0.001). There was a non-significant increase in the percentage of high probability scintigrams (15 to 18%; p = 0.716). Overall the diagnostic yield of lung scintigraphy improved significantly (54 to 78%; p < 0.001). Conclusion: the diagnostic performance of lung scintigraphy can be improved by careful triage of patients to either Q scintigraphy or CTPA based on clinical history and CR findings. Q scintigraphy remains a valuable diagnostic test in the investigation of suspected PE in carefully selected patients

  6. Growth retardation due to idiopathic growth hormone deficiencies: MR findings in 24 patients

    International Nuclear Information System (INIS)

    Ochi, M.; Morikawa, M.; Yoshimoto, M.; Kinoshita, E.; Hayashi, K.

    1992-01-01

    In this study we evaluated the pituitary-hypothalamic abnormalities of ''idiopathic growth hormone (GH) deficiency'' as demonstrated by MR imaging. Twenty-four patients were examined with a 1.5-T unit using spin echo T-1 weighted images. The patients were divided into two groups according to MR findings: those with ectopic posterior pituitary glands (12 patients), and those with normal posterior pituitary glands (12 patients). Ten patients in the former group and four in the latter group had small anterior pituitary glands. All patients in the former group but only four in the latter group had severe GH deficiencies. Multiple hormone deficiencies were found in eight patients in the former group, but in only two in the latter group. It is speculated that perinatal abnormalities can cause posterior pituitary ectopia and that there is a close correlation between breech delivery and the male disadvantage of posterior pituitary ectopia. Half of our patients with ''idiopathic GH deficiency'' had ectopic posterior pituitaries. GH deficiency with posterior pituitary ectopia should no longer be considered idiopathic because organic lesions can now be identified during life. (orig./GD)

  7. Familial idiopathic pulmonary fibrosis. Evidence of lung inflammation in unaffected family members

    International Nuclear Information System (INIS)

    Bitterman, P.B.; Rennard, S.I.; Keogh, B.A.; Wewers, M.D.; Adelberg, S.; Crystal, R.G.

    1986-01-01

    We evaluated 17 clinically unaffected members of three families with an autosomal dominant form of idiopathic pulmonary fibrosis for evidence of alveolar inflammation. Each person in the study was examined by gallium-67 scanning for a general estimate of pulmonary inflammation, and by bronchoalveolar lavage for characterization of the types of recovered cells and their state of activation. Eight of the 17 subjects had evidence of alveolar inflammation on the lavage studies. Supporting data included increased numbers of neutrophils and activated macrophages that released one or more neutrophil chemoattractants, and growth factors for lung fibroblasts--findings similar to those observed in patients with overt idiopathic pulmonary fibrosis. Four of these eight also had a positive gallium scan; in all the other clinically unaffected subjects the scan was normal. During a follow-up of two to four years in seven of the eight subjects who had evidence of inflammation, no clinical evidence of pulmonary fibrosis has appeared. These results indicate that alveolar inflammation occurs in approximately half the clinically unaffected family members at risk of inheriting autosomal dominant idiopathic pulmonary fibrosis. Whether these persons with evidence of pulmonary inflammation but no fibrosis will proceed to have clinically evident pulmonary fibrosis is not yet known

  8. MRI assessment of tenosynovitis in children with juvenile idiopathic arthritis: inter- and intra-observer variability

    International Nuclear Information System (INIS)

    Lambot, Karen; Brunelle, Francis; Boavida, Peter; Damasio, Maria Beatrice; Tanturri de Horatio, Laura; Barbuti, Domenico; Desgranges, Marie; Bader-Meunier, Brigitte; Quartier, Pierre; Malattia, Clara; Bracaglia, Claudia; Ording Mueller, Lil-Sofie; Elie, Caroline; Rosendahl, Karen

    2013-01-01

    There is sparse knowledge about grading tenosynovitis using MRI. The purpose of this study was to assess the reliability of a tenosynovitis MRI scoring system in juvenile idiopathic arthritis. Children with juvenile idiopathic arthritis and wrist involvement were enrolled in two paediatric centres, from October 2006 to January 2010. The extensor (compartments II, IV and VI) and flexor tendons were assessed for the presence of tenosynovitis on T1-weighted postcontrast fat-saturated MR images and were scored from 0 (normal) to 2 (moderate to severe) by two observers independently. Intra- and interobserver agreement was assessed. Ninety children (age range: 5-18.5 years) were included, of whom 34 had tenosynovitis involving extensors and 28 had tenosynovitis involving flexors. A total of 360 tendon areas were analysed, of which 114 had tenosynovitis (86/270 extensors and 28/90 flexors). Intra-reader 1 agreement was excellent for the extensors (k = 0.82-0.91) and for the flexors (k = 0.85); intra-reader 2 agreement was moderate to good for the extensors (k = 0.51-0.72) and good for the flexors (k = 0.64). Inter-reader agreement was good for the extensors (k = 0.69-0.73) and moderate for the flexors (k = 0.49). The proposed MRI scoring system for the assessment of wrist tenosynovitis in juvenile idiopathic arthritis appears feasible with an observer agreement sufficient for clinical use. (orig.)

  9. Effect of animal and vegetable protein intake on oxalate excretion in idiopathic calcium stone disease.

    Science.gov (United States)

    Marangella, M; Bianco, O; Martini, C; Petrarulo, M; Vitale, C; Linari, F

    1989-04-01

    Oxalate excretion was measured in healthy subjects and idiopathic calcium stone-formers on dietary regimens which differed in the type and amount of protein allowed; 24-h urine collections were obtained from 41 practising vegetarians and 40 normal persons on a free, mixed, "mediterranean" diet. Twenty idiopathic calcium stone-formers were also studied while on two low calcium, low oxalate diets which differed in that animal protein was high in one and restricted in the other. Vegetarians had higher urinary oxalate levels than controls and although the calcium levels were markedly lower, urinary saturation with calcium/oxalate was significantly higher. This mild hypercalciuria was interpreted as being secondary to both a higher intake and increased fractional intestinal absorption of oxalate. Changing calcium stone-formers from a high to a low animal protein intake produced a significant decrease in calcium excretion but there was no variation in urinary oxalate. As a result, the decrease in calcium oxalate saturation was only marginal and not significant. It was concluded that dietary animal protein has a minimal effect on oxalate excretion. Mild hyperoxaluria of idiopathic calcium stone disease is likely to be intestinal in origin. Calcium stone-formers should be advised to avoid an excess of animal protein but the risks of a vegetable-rich diet should also be borne in mind.

  10. CSF Hypocretin-1 Levels and Clinical Profiles in Narcolepsy and Idiopathic CNS Hypersomnia in Norway

    Science.gov (United States)

    Heier, Mona Skard; Evsiukova, Tatiana; Vilming, Steinar; Gjerstad, Michaela D.; Schrader, Harald; Gautvik, Kaare

    2007-01-01

    Objective: To evaluate the relationship between CSF hypocretin-1 levels and clinical profiles in narcolepsy and CNS hypersomnia in Norwegian patients. Method: CSF hypocretin-1 was measured by a sensitive radioimmunoassay in 47 patients with narcolepsy with cataplexy, 7 with narcolepsy without cataplexy, 10 with idiopathic CNS hypersomnia, and a control group. Results: Low hypocretin-1 values were found in 72% of the HLA DQB1*0602 positive patients with narcolepsy and cataplexy. Patients with low CSF hypocretin-1 levels reported more extensive muscular involvement during cataplectic attacks than patients with normal levels. Hypnagogic hallucinations and sleep paralysis occurred more frequently in patients with cataplexy than in the other patient groups, but with no correlation to hypocretin-1 levels. Conclusion: About three quarters of the HLA DQB1*0602 positive patients with narcolepsy and cataplexy had low CSF hypocretin-1 values, and appear to form a distinct clinical entity. Narcolepsy without cataplexy could not be distinguished from idiopathic CNS hypersomnia by clinical symptoms or biochemical findings. Citation: Heier MS; Evsiukova T; Vilming S; Gjerstad MD; Schrader H; Gautvik K. CSF hypocretin-1 levels and clinical profiles in narcolepsy and idiopathic CNS hypersomnia in norway. SLEEP 2007;30(8):969-973. PMID:17702265

  11. MRI assessment of tenosynovitis in children with juvenile idiopathic arthritis: inter- and intra-observer variability

    Energy Technology Data Exchange (ETDEWEB)

    Lambot, Karen; Brunelle, Francis [Hopital Necker-Enfants Malades, Department of Paediatric Radiology, Paris (France); Boavida, Peter [Great Ormond Street Hospital, Department of Radiology, London (United Kingdom); Damasio, Maria Beatrice [Ospedale Pediatrico Gaslini, Department of Radiology, Genoa (Italy); Tanturri de Horatio, Laura; Barbuti, Domenico [Ospedale Pediatrico Bambino Gesu, Department of Radiology, Rome (Italy); Desgranges, Marie; Bader-Meunier, Brigitte; Quartier, Pierre [Hopital Necker-Enfants Malades, Department of Paediatric Immunology, Hematology and Rheumatology, APHP French Reference Center ' ' Arthrites juveniles' ' , Paris (France); Malattia, Clara [University of Genoa, Department of Paediatrics, Genoa (Italy); Bracaglia, Claudia [Ospedale Pediatrico Bambino Gesu, Department of Paediatrics, Rome (Italy); Ording Mueller, Lil-Sofie [Great Ormond Street Hospital, Department of Radiology, London (United Kingdom); University Hospital of North Norway, Department of Radiology, Tromsoe (Norway); Elie, Caroline [Paris Descartes University, Department of Biostatistics, Hopital Necker-Enfants Malades, Paris (France); Rosendahl, Karen [Great Ormond Street Hospital, Department of Radiology, London (United Kingdom); Haukeland University Hospital, Department of Radiology, Bergen (Norway)

    2013-07-15

    There is sparse knowledge about grading tenosynovitis using MRI. The purpose of this study was to assess the reliability of a tenosynovitis MRI scoring system in juvenile idiopathic arthritis. Children with juvenile idiopathic arthritis and wrist involvement were enrolled in two paediatric centres, from October 2006 to January 2010. The extensor (compartments II, IV and VI) and flexor tendons were assessed for the presence of tenosynovitis on T1-weighted postcontrast fat-saturated MR images and were scored from 0 (normal) to 2 (moderate to severe) by two observers independently. Intra- and interobserver agreement was assessed. Ninety children (age range: 5-18.5 years) were included, of whom 34 had tenosynovitis involving extensors and 28 had tenosynovitis involving flexors. A total of 360 tendon areas were analysed, of which 114 had tenosynovitis (86/270 extensors and 28/90 flexors). Intra-reader 1 agreement was excellent for the extensors (k = 0.82-0.91) and for the flexors (k = 0.85); intra-reader 2 agreement was moderate to good for the extensors (k = 0.51-0.72) and good for the flexors (k = 0.64). Inter-reader agreement was good for the extensors (k = 0.69-0.73) and moderate for the flexors (k = 0.49). The proposed MRI scoring system for the assessment of wrist tenosynovitis in juvenile idiopathic arthritis appears feasible with an observer agreement sufficient for clinical use. (orig.)

  12. Idiopathic avascular necrosis of the femoral heads in five members of a Moroccan family.

    Science.gov (United States)

    Sekkat, Jihane; Rachidi, Ouafaa; Janani, Saadia; Mkinsi, Ouafaa

    2012-10-01

    Avascular necrosis (AVN) is idiopathic in about 40% of cases. The pathophysiology of avascular necrosis remains incompletely elucidated. Here, we report a case that underlines the role for inherited factors in AVN of the femoral heads. Idiopathic AVN of the femoral heads occurred in five members of the same family (a woman, her two paternal aunts, her male paternal cousin and her female paternal cousin) at a mean age of 42.4 years (range, 33-58 years). Standard pelvic radiographs showed Arlet and Ficat stage 4 AVN in three patients and stage 3 in two patients. None of the patients had a history of glucocorticoid therapy, alcohol abuse, or trauma. All five patients underwent investigations for a cause, including blood cell counts, a lipid profile, coagulation tests, testing for antinuclear antibodies, hemoglobin electrophoresis, ultrasonography of the abdomen, and standard radiographs of the long limb bones. The results were normal or negative, ruling out known hereditary causes of AVN such as sickle cell anemia and Gaucher disease. Many cases of familial AVN of the femoral head have been described in patients with sickle cell anemia or Gaucher disease. However, only five families with idiopathic familial AVN of the femoral heads have been reported (three in the US and two in Taiwan). All the patients in these families had isolated bilateral AVN of the femoral heads without AVN at other sites. Copyright © 2012. Published by Elsevier SAS.

  13. 111indium-antimyosin immunoscintigraphy in suspected myocarditis

    International Nuclear Information System (INIS)

    Krause, T.; Schuemichen, C.; Joseph, A.; Moser, E.; Zeiher, A.

    1991-01-01

    111 Indium-monoclonal antimyosin scans were carried out in 21 patients with suspected myocarditis, confirmed by reduced ejection volume, pericardial effusion and clinical follow up in 12 patients. Coronary heart disease was excluded angiographically in all cases. Quantitative evaluation of myocardial 111 In-antimyosin accumulation 48 hours after injection showed a pathological uptake in 10/12 patients with increased heart/lung ratios (Q 48 >1,58). Ratios were also elevated in 2 patients with cardiomyopathy, 2 suffering from vasculitis and 1 with dermatomyositis. Four patients without proven cardiac disease had normal ratios (Q 48 ≤1,58). Examination after 24 hours was of limited value, depending on the residual blood pool activity. Visual analysis of the scans showed a high interobserver variation despite a positive correlation with quantitative analysis (48 h p.i.: r=0,72; p 111 In-antimyosin scan as a screening method prior to myocardial biopsy. However, scintigraphy cannot definitely elucidate the cause of myocardial damage. Therefore, myocardial biopsy is still recommended after positive antimyosin scans. (orig.) [de

  14. [Analysis on the role of Sirius combined topography and tomography system in screening for suspect keratoconus].

    Science.gov (United States)

    Zhang, Y; Chen, Y G; Yang, H Y; Xia, Y J; Zhao, R

    2018-01-11

    Objective: To evaluate the role of Sirius combined topography and tomography system in screening for suspect keratoconus among the corneal refractive surgery candidates. Methods: Retrospective case series study. Eight hundred and sixteen consecutive ametropic patients (1 632 eyes) who underwent routine examinations before corneal refractive surgery at Peking University Third Hospital from January 2016 to September 2016 were reviewed. All the cases were analyzed with Sirius combined topography and tomography system. Fifty-nine eyes of 37 patients, aged (28.9±7.4) years, classified as suspect keratoconus by the system were enrolled in the suspect group, including 25 females (40 eyes) and 12 males (19 eyes). A random eye of the first 59 patients, aged (27.1±6.4)years, whose both eyes were classified as normal by Sirius system were enrolled in the control group, including 38 females and 21 males. The corneal anterior surface, posterior surface and minimum thickness data of the suspect group were analyzed and then compared with the control group. The classified results were further verified by Pentacam system. Independent-samples t test and Mann-Whitney U test were applied to analyze the normal distribution and non-normal distribution data respectively. Results: The medians of anterior surface and posterior surface symmetry index and Baiocchi Calossi Versaci index of anterior surface and posterior surface of the suspect group were 0.84, 0.22, 0.58 and 0.51 D, and that of the control group were 0.05, 0.04, 0.09 and 0.06 D, and the differences were of statistical significance ( Z=- 18.764, -8.351, -12.248, -10.709, Ptopography and tomography and its integral automatic classification system is capable to screen out the suspect keratoconus simply and effectively. These indices maybe arranged in descending order in terms of the sensitivity as corneal posterior surface, followed by corneal anterior surface and then corneal thinnest point thickness. (Chin J Ophthalmol, 2018

  15. Normal accidents

    International Nuclear Information System (INIS)

    Perrow, C.

    1989-01-01

    The author has chosen numerous concrete examples to illustrate the hazardousness inherent in high-risk technologies. Starting with the TMI reactor accident in 1979, he shows that it is not only the nuclear energy sector that bears the risk of 'normal accidents', but also quite a number of other technologies and industrial sectors, or research fields. The author refers to the petrochemical industry, shipping, air traffic, large dams, mining activities, and genetic engineering, showing that due to the complexity of the systems and their manifold, rapidly interacting processes, accidents happen that cannot be thoroughly calculated, and hence are unavoidable. (orig./HP) [de

  16. Idiopathic gastric perforation in an asplenic infant | Olsen | African ...

    African Journals Online (AJOL)

    Introduction: The cause of idiopathic gastric perforations in neonates remains unknown. Perforations of the abdominal oesophagus, stomach and duodenum in infants and children are the rarest type of intestinal perforations. There are 21 reported cases of an idiopathic gastric rupture in nonneonates. Case Report: A ...

  17. The clinical profile of idiopathic Parkinson's disease in a South ...

    African Journals Online (AJOL)

    The clinical profile of idiopathic Parkinson's disease in a South African hospital complex - the influence of ethnicity and gender. Marcelle Smith, Girish Modi. Abstract. Background Idiopathic Parkinson's Disease (IPD) has not been well studied in Black African populations. Data on the demographics, phenotype differences ...

  18. Is idiopathic recurrent pancreatitis attributed to small stones?

    OpenAIRE

    Chow, Wai-Keung; Peng, Yen-Chun

    2013-01-01

    Idiopathic recurrent pancreatitis remains a clinical challenge. Intraductal ultrasonography in the management of idiopathic recurrent pancreatitis may be a new strategy for undetermined causes after initial diagnostic approaches, including endoscopic retrograde cholangio-pancreatography (ERCP). However, no definite cause after ERCP should be defined under optimal settings and with experienced technique.

  19. Perceived health status in self-reported adolescent idiopathic scoliosis

    DEFF Research Database (Denmark)

    Andersen, Mikkel Ø; Thomsen, Karsten; Kyvik, Kirsten O

    2010-01-01

    A questionnaire-based identification of adolescent idiopathic scoliosis (AIS) patients and measure of Short Form-12 (SF-12) in a big twin-cohort.......A questionnaire-based identification of adolescent idiopathic scoliosis (AIS) patients and measure of Short Form-12 (SF-12) in a big twin-cohort....

  20. The clinical course of patients with suspected pulmonary embolism

    NARCIS (Netherlands)

    van Beek, E. J.; Kuijer, P. M.; Büller, H. R.; Brandjes, D. P.; Bossuyt, P. M.; ten Cate, J. W.

    1997-01-01

    BACKGROUND: The outcome of patients with suspected pulmonary embolism is known to a limited extent only. OBJECTIVE: To address this limited knowledge in a cohort in whom pulmonary embolism was proved or ruled out. METHODS: Consecutive patients with clinically suspected pulmonary embolism underwent

  1. 48 CFR 403.303 - Reporting suspected antitrust violations.

    Science.gov (United States)

    2010-10-01

    ... Antitrust Violations 403.303 Reporting suspected antitrust violations. Contracting officers shall report the circumstances of suspected violations of antitrust laws to the Office of Inspector General in accordance with... antitrust violations. 403.303 Section 403.303 Federal Acquisition Regulations System DEPARTMENT OF...

  2. 48 CFR 1403.303 - Reporting suspected antitrust violations.

    Science.gov (United States)

    2010-10-01

    ... Antitrust Violations 1403.303 Reporting suspected antitrust violations. (a) Reports on suspected violations of antitrust laws as required by FAR 3.303 shall be prepared by the CO, reviewed by the SOL, and... antitrust violations. 1403.303 Section 1403.303 Federal Acquisition Regulations System DEPARTMENT OF THE...

  3. Detection of Rabies antigen in brains of suspected Rabid dogs ...

    African Journals Online (AJOL)

    Objective: To detect the presence of rabies antigen in brains of suspected rabid dogs. Materials and Methods: Ninety six (96) brain specimens from suspected rabid dogs were examined for the presence of rabies antigen using Seller's staining technique and enzyme immunoassay. Results: The two techniques were both ...

  4. Selecting foils for identification lineups: matching suspects or descriptions?

    Science.gov (United States)

    Tunnicliff, J L; Clark, S E

    2000-04-01

    Two experiments directly compare two methods of selecting foils for identification lineups. The suspect-matched method selects foils based on their match to the suspect, whereas the description-matched method selects foils based on their match to the witness's description of the perpetrator. Theoretical analyses and previous results predict an advantage for description-matched lineups both in terms of correctly identifying the perpetrator and minimizing false identification of innocent suspects. The advantage for description-matched lineups should be particularly pronounced if the foils selected in suspect-matched lineups are too similar to the suspect. In Experiment 1, the lineups were created by trained police officers, and in Experiment 2, the lineups were constructed by undergraduate college students. The results of both experiments showed higher suspect-to-foil similarity for suspect-matched lineups than for description-matched lineups. However, neither experiment showed a difference in correct or false identification rates. Both experiments did, however, show that there may be an advantage for suspect-matched lineups in terms of no-pick and rejection responses. From these results, the endorsement of one method over the other seems premature.

  5. Arthroscintigraphy in suspected rotator cuff rupture

    International Nuclear Information System (INIS)

    Gratz, S.; Behr, T.; Becker, W.; Koester, G.; Vosshenrich, R.; Grabbe, E.

    1998-01-01

    Aim: In order to evaluate the diagnostic efficiency of arthroscintigraphy in suspected rotator cuff ruptures this new imaging procedure was performed 20 times in 17 patients with clinical signs of a rotator cuff lesion. The scintigraphic results were compared with sonography (n=20), contrast arthrography (n=20) and arthroscopy (n=10) of the shoulder joint. Methods: After performing a standard bone scintigraphy with intravenous application of 300 MBq 99m-Tc-methylene diphosphonate (MDP) for landmarking of the shoulder region arthroscintigraphy was performed after an intraarticular injection of 99m-Tc microcolloid (ALBU-RES 400 μCi/5 ml). The application was performed either in direct combination with contrast arthrography (n=10) or ultrasound conducted mixed with a local anesthetic (n=10). Findings at arthroscopical surgery (n=10) were used as the gold standard. Results: In case of complete rotator cuff rupture (n=5), arthroscintigraphy and radiographic arthrography were identical in 5/5. In one patient with advanced degenerative alterations of the shoulder joint radiographic arthrography incorrectly showed a complete rupture which was not seen by arthroscintigraphy and endoscopy. In 3 patients with incomplete rupture, 2/3 results were consistant. A difference was seen in one patient with a rotator cuff, that has been already revised in the past and that suffered of capsulitis and calcification. Conclusion: Arthroscinitgraphy is a sensitive technique for detection of rotator cuff ruptures. Because of the lower viscosity of the active compound, small ruptures can be easily detected, offering additional value over radiographic arthrography and ultrasound, especially for evaluation of incomplete cuff ruptures. (orig.) [de

  6. Angiopoietin-2 polymorphism in women with idiopathic recurrent miscarriage.

    Science.gov (United States)

    Pietrowski, Detlef; Tempfer, Clemens; Bettendorf, Hertha; Bürkle, Bernd; Nagele, Fritz; Unfried, Gertrud; Keck, Christoph

    2003-10-01

    To investigate the relationship between idiopathic recurrent miscarriage and a polymorphism of the gene encoding for angiopoietin-2 (ANGPT2), an autochthonous modulator of angiogenesis during pregnancy. Prospective case control study. Academic research institution. One hundred thirty-one women with a history of three or more consecutive pregnancy losses before 20 weeks' gestation, and 125 healthy, postmenopausal controls with at least two live births and no history of pregnancy loss. Peripheral venous puncture. Polymerase chain reaction and restriction fragment length polymorphism analysis were performed to identify the different ANGPT2 alleles. No association between mutant (mt) allele and the occurrence of idiopathic recurrent miscarriage was found. Between women with primary and secondary idiopathic recurrent miscarriage, no statistically significant differences with respect to allele frequencies were observed. This is the first report on the ANGPT2 gene polymorphism in women with idiopathic recurrent miscarriage, demonstrating that the investigated polymorphism is not associated with idiopathic recurrent miscarriage in a white population.

  7. Acute exacerbation of idiopathic pulmonary fibrosis triggered by Aspergillus empyema

    Directory of Open Access Journals (Sweden)

    Atsushi Suzuki

    Full Text Available Acute exacerbation (AE is a severe and life-threatening complication of idiopathic pulmonary fibrosis (IPF. In 2016, the definition and diagnostic criteria for AE-IPF were updated by an international working group. The new definition includes any acute, clinically significant respiratory deterioration (both idiopathic and triggered events characterized by evidence of new widespread alveolar abnormality in patients with IPF. There are no currently proven beneficial management strategies for idiopathic and triggered AE-IPF. This is the first report describing AE-IPF triggered by Aspergillus empyema, which was improved by a combination of corticosteroid, systemic antifungal therapy, local antifungal therapy, and additional pharmacological therapies. Future research may reveal optimal strategies for both idiopathic and triggered AE-IPF. Keywords: Idiopathic pulmonary fibrosis, Acute exacerbation, AE-IPF, Triggered AE, Aspergillus infection

  8. Seasonal variation among tuberculosis suspects in four countries

    DEFF Research Database (Denmark)

    Mabaera, Biggie; Naranbat, Nymadawa; Katamba, Achilles

    2009-01-01

    The objective of the study was to analyze monthly trends across a calendar year in tuberculosis suspects and sputum smear-positive cases based on nationally representative samples of tuberculosis laboratory registers from Moldova, Mongolia, Uganda and Zimbabwe. Out of the 47 140 suspects registered...... in the tuberculosis laboratory registers, 13.4% (6312) were cases. The proportion varied from country to country, Moldova having the lowest (9%) and Uganda the highest (21%). From the monthly proportion of suspects and cases among total suspects and cases, seasonal variations were most marked in Mongolia which, among...... attendance to diagnostic laboratory services, evidenced by the contrasting findings of Mongolia (extreme continental northern climate) compared to Uganda (equatorial climate). A combination of external and possibly endogenous factors seems to determine whether tuberculosis suspects and cases present...

  9. Idiopathic paraproteinemia. III. Increased frequency of paraproteinemia in thymectomized aging C57BL/KaLwRij and CBA/BrARij mice

    NARCIS (Netherlands)

    Radl, J.; Glopper, E. de; Berg, P. van den; Zwieten, M.J. van

    1980-01-01

    The influence of thymectomy on the appearance of idiopathic paraproteinemia (IP) during aging was investigated in mice of the C57BL/KaLwRij and CBA/BrARij strains, which under normal conditions develop IP in high and low frequency, respectively. Compared with sham-thymectomized mice, C57BL mice

  10. Asymmetry of the Vertebral Body and Pedicles in the True Transverse Plane in Adolescent Idiopathic Scoliosis : A CT-Based Study

    NARCIS (Netherlands)

    Brink, Rob C.; Schlösser, Tom P C; Colo, Dino; Vincken, Koen L.; van Stralen, Marijn; Hui, Steve C N; Chu, Winnie C W; Cheng, Jack C Y; Castelein, RM

    2017-01-01

    Study Design Cross-sectional. Objectives To quantify the asymmetry of the vertebral bodies and pedicles in the true transverse plane in adolescent idiopathic scoliosis (AIS) and to compare this with normal anatomy. Summary of background data There is an ongoing debate about the existence and

  11. Segmental torso masses in adolescent idiopathic scoliosis.

    Science.gov (United States)

    Keenan, Bethany E; Izatt, Maree T; Askin, Geoffrey N; Labrom, Robert D; Pettet, Graeme J; Pearcy, Mark J; Adam, Clayton J

    2014-08-01

    Adolescent idiopathic scoliosis is the most common type of spinal deformity whose aetiology remains unclear. Studies suggest that gravitational forces in the standing position play an important role in scoliosis progression, therefore anthropometric data is required to develop biomechanical models of the deformity. Few studies have analysed the trunk by vertebral level and none have performed investigations of the scoliotic trunk. The aim of this study was to determine the centroid, thickness, volume and estimated mass, for sections of the scoliotic trunk. Existing low-dose CT scans were used to estimate vertebral level-by-level torso masses for 20 female adolescent idiopathic scoliosis patients. ImageJ processing software was used to analyse the CT images and enable estimation of the segmental torso mass corresponding to each vertebral level. The patients' mean age was 15.0 (SD 2.7) years with mean major Cobb angle of 52 (SD 5.9)° and mean patient weight of 58.2 (SD 11.6) kg. The magnitude of torso segment mass corresponding to each vertebral level increased by 150% from 0.6kg at T1 to 1.5kg at L5. Similarly, segmental thickness from T1-L5 increased inferiorly from a mean 18.5 (SD 2.2) mm at T1 to 32.8 (SD 3.4) mm at L5. The mean total trunk mass, as a percentage of total body mass, was 27.8 (SD 0.5) % which was close to values reported in previous literature. This study provides new anthropometric reference data on segmental (vertebral level-by-level) torso mass in adolescent idiopathic scoliosis patients, useful for biomechanical models of scoliosis progression and treatment. Copyright © 2014 Elsevier Ltd. All rights reserved.

  12. Usefulness of the Negative Multidetector CT Angiography in Patients with Suspected Pulmonary Embolism

    International Nuclear Information System (INIS)

    Holguin Holguin, Alfonso Jose; Lombana, Milton; Collazos, Giovanny; Bravo, Hector; Abella Calle, Jose

    2012-01-01

    Objective: The aim of the study is to evaluate alternative findings in pulmonary CT angiography using MDCT reported as negative in patients with suspected pulmonary embolism. Material and method: Cross sectional study with 178 patients. We recognized other findings reported in pulmonary CT angiography using MDCT whose result was negative. Subsequently these findings were classified into six different categories of clinical relevance and their association with clinical scenario was analyzed. Results: from November 2004 to June 2007 we requested 231 pulmonary CT angiography using MDCT in patients with suspected pulmonary embolism. 217 patients were included in the final analysis. Of these, 39 (185) were positive for pulmonary embolism. The 178 remaining negative studies, 8.43% were normal, 53.37% were classified as category A, 20.79% category B, 14.04% category C; category D 1.12% and 2.25% were not interpretable. The most frequent diagnoses were pneumonia, interstitial lung disease and cardiogenic pulmonary edema. It was less likely to find a normal report whether the study was requested in the hospital department in emergency patients (OR = 0.38 95% 0.16 to 0.88, p = 0.0007). No differences were found in other categories or between patients younger or older than 65 years (p = 0.436). Conclusion: Pulmonary CT angiography as first line imaging tool in patients with suspected pulmonary embolism is useful, offering alternative diagnoses in hospitalized and emergency room patients, regardless of their age and clinical findings.

  13. Coronary 64-slice CT angiography predicts outcome in patients with known or suspected coronary artery disease

    Energy Technology Data Exchange (ETDEWEB)

    Gaemperli, Oliver; Valenta, Ines; Schepis, Tiziano [University Hospital Zurich NUK C 32, Cardiovascular Center, Zurich (Switzerland); Husmann, Lars; Scheffel, Hans; Desbiolles, Lotus; Leschka, Sebastian; Alkadhi, Hatem [University Hospital Zurich, Institute of Diagnostic Radiology, Zurich (Switzerland); Kaufmann, Philipp A. [University Hospital Zurich NUK C 32, Cardiovascular Center, Zurich (Switzerland); University of Zurich, Zurich Center for Integrative Human Physiology (ZIHP), Zurich (Switzerland)

    2008-06-15

    The aim of this study was to assess the prognostic value of 64-slice CT angiography (CTA) in patients with known or suspected coronary artery disease (CAD). Sixty-four-slice coronary CTA was performed in 220 patients [mean age 63 {+-} 11 years, 77 (35%) female] with known or suspected CAD. CTA images were analyzed with regard to the presence and number of coronary lesions. Patients were followed-up for the occurrence of the following clinical endpoints: death, nonfatal myocardial infarction, unstable angina, and coronary revascularization. During a mean follow-up of 14 {+-} 4 months, 59 patients (27%) reached at least one of the predefined clinical endpoints. Patients with abnormal coronary arteries on CTA (i.e., presence of coronary plaques) had a 1st-year event rate of 34%, whereas in patients with normal coronary arteries no events occurred (event rate, 0%, p < 0.001). Similarly, obstructive lesions ({>=}50% luminal narrowing) on CTA were associated with a high first-year event rate (59%) compared to patients without stenoses (3%, p < 0.001). The presence of obstructive lesions was a significant independent predictor of an adverse cardiac outcome. Sixty-four-slice CTA predicts cardiac events in patients with known or suspected CAD. Conversely, patients with normal coronary arteries on CTA have an excellent mid-term prognosis. (orig.)

  14. Reconstructing Normality

    DEFF Research Database (Denmark)

    Gildberg, Frederik Alkier; Bradley, Stephen K.; Fristed, Peter Billeskov

    2012-01-01

    Forensic psychiatry is an area of priority for the Danish Government. As the field expands, this calls for increased knowledge about mental health nursing practice, as this is part of the forensic psychiatry treatment offered. However, only sparse research exists in this area. The aim of this study...... was to investigate the characteristics of forensic mental health nursing staff interaction with forensic mental health inpatients and to explore how staff give meaning to these interactions. The project included 32 forensic mental health staff members, with over 307 hours of participant observations, 48 informal....... The intention is to establish a trusting relationship to form behaviour and perceptual-corrective care, which is characterized by staff's endeavours to change, halt, or support the patient's behaviour or perception in relation to staff's perception of normality. The intention is to support and teach the patient...

  15. Pursuing Normality

    DEFF Research Database (Denmark)

    Madsen, Louise Sofia; Handberg, Charlotte

    2018-01-01

    implying an influence on whether to participate in cancer survivorship care programs. Because of "pursuing normality," 8 of 9 participants opted out of cancer survivorship care programming due to prospects of "being cured" and perceptions of cancer survivorship care as "a continuation of the disease......BACKGROUND: The present study explored the reflections on cancer survivorship care of lymphoma survivors in active treatment. Lymphoma survivors have survivorship care needs, yet their participation in cancer survivorship care programs is still reported as low. OBJECTIVE: The aim of this study...... was to understand the reflections on cancer survivorship care of lymphoma survivors to aid the future planning of cancer survivorship care and overcome barriers to participation. METHODS: Data were generated in a hematological ward during 4 months of ethnographic fieldwork, including participant observation and 46...

  16. Idiopathic interstitial pneumonias: imaging-pathology correlation

    International Nuclear Information System (INIS)

    Ellis, Stephen M.; Hansell, David M.

    2002-01-01

    The terminology related to idiopathic interstitial pneumonia (IIP) remains confusing and in some cases wholly inaccurate. In addition, a greater understanding of the correlation between high-resolution computed tomography (HRCT) appearances and the corresponding histopathological changes found in the interstitial pneumonias has resulted in a crucial role for HRCT in the investigation of IIPs. The role of the radiologist is becoming increasingly important with a strong emphasis on establishing a diagnosis without resorting to lung biopsy. We aim to clarify the current classification of the IIPs highlighting their clinical, pathological and imaging characteristics in order to assist the radiologist in performing their increasingly important diagnostic role. (orig.)

  17. Autonomic symptoms in idiopathic REM behavior disorder

    DEFF Research Database (Denmark)

    Ferini-Strambi, Luigi; Oertel, Wolfgang; Dauvilliers, Yves

    2014-01-01

    Patients with idiopathic REM sleep behavior disorder (iRBD) are at very high risk of developing neurodegenerative synucleinopathies, which are disorders with prominent autonomic dysfunction. Several studies have documented autonomic dysfunction in iRBD, but large-scale assessment of autonomic...... symptoms has never been systematically performed. Patients with polysomnography-confirmed iRBD (318 cases) and controls (137 healthy volunteers and 181 sleep center controls with sleep diagnoses other than RBD) were recruited from 13 neurological centers in 10 countries from 2008 to 2011. A validated scale...

  18. Radioaerosol lung scintigraphy in idiopathic scolios

    International Nuclear Information System (INIS)

    Maini, C.L.; Giordano, A.; Santucci, B.; Aulisa, L.; Pistelli, R.; Fuso, L.

    1988-01-01

    The study of respiratory fuctions is of key importance for the clinical evaluation of patients with idiopathic scoliosis. Such study has been traditionally based on classical pulmonary function tests and arterial hemogasanalysis. However, neither procedure gives any information on the topographical distribution of abnormalities, and both might be suboptimal as far as sensitivity is concerned. The preliminary results obtained with radioaerosol lung scintigraphy in 11 patients with scoliosis are here presented. They lead to the conclusion that radioaerosol lung scintigraphy, besides being an useful adjunct to more traditional diagnostic procedures, can yield unique information on the localization of convective ventilation derangements induced by the dynamic abnormalities of the rib cage

  19. Surgical repair of idiopathic scrotal elephantiasis.

    Science.gov (United States)

    Zacharakis, Evangelos; Dudderidge, Tim; Zacharakis, Emmanouil; Ioannidis, Evangelos

    2008-02-01

    Scrotal lymphedema (scrotal elephantiasis) is uncommon outside of filariasis endemic regions. We present a case of a 65-year-old with idiopathic lymphedema of the scrotum and functional impairment of the penis. The patient underwent surgical excision of the edematous subcutaneous tissues and plastic reconstruction of his penis and scrotum. Three years later, the patient showed no signs of local recurrence, had complete restoration of urinary and sexual function and was extremely satisfied with the result. Surgical management was an effective strategy in the management of scrotal lymphedema in this case.

  20. [Idiopathic scrotal elephantiasis. A case report].

    Science.gov (United States)

    López-Caballero, Ignacio; Sánchez-Ruvalcaba, Itzel; Sánchez-Martinez, Luis Carlos; Hernández-Ordoñez, Octavio; Gómez-Lara, Miguel; Flores-Carrillo, Víctor

    2014-01-01

    Penoscrotal lymphedema (scrotal elephantiasis) is a condition that has been described in areas in which filariasis is endemic. This paper presents a case of a 45-year-old man with idiopathic lymphedema isolated to the scrotum. After acquired causes of lymphedema were ruled out, the patient was treated with scrotectomy and penoscrotal reconstruction. Currently, the patient is receiving follow-up care without evidence of recurrence. Penoscrotal lymphedema may cause symptoms of weakness, immobility and emotional disturbance. Surgery procedure provides a satisfactory cosmetical and functional outcome.

  1. Phototesting In The Idiopathic Photodermatoses Among Indians

    Directory of Open Access Journals (Sweden)

    Bejoy P

    1998-01-01

    Full Text Available 50 patients with idiopathic photodermatoses were phototested using the solar simulator, with broad spectrum wavebands of the whole spectrum (WS and the whole spectrum â€" ultraviolet B (WS- UVB to determine the minimal erythema dose. Cases diagnosed as solar urticaria were further tested with visible light. Among the photodermatoses seen in this part of the country polymorphic light eruption (PLE was the commonest followed by chronic actinic dermatitis (CAD and solar urticaria. The UVB wavelengths appeared to be more responsible than UVA in PLE and CAD while the UVA and visible wavelengths were responsible for solar urticaria.

  2. Dystonia Associated with Idiopathic Slow Orthostatic Tremor

    Directory of Open Access Journals (Sweden)

    Christopher Kobylecki

    2016-02-01

    Full Text Available Background: We aimed to characterize the clinical and electrophysiological features of patients with slow orthostatic tremor.Case Report: The clinical and neurophysiological data of patients referred for lower limb tremor on standing were reviewed. Patients with symptomatic or primary orthostatic tremor were excluded. Eight patients were identified with idiopathic slow 4–8 Hz orthostatic tremor, which was associated with tremor and dystonia in cervical and upper limb musculature. Coherence analysis in two patients showed findings different to those seen in primary orthostatic tremor.Discussion: Slow orthostatic tremor may be associated with dystonia and dystonic tremor.

  3. Idiopathic Pulmonary Fibrosis: Diagnosis and Clinical Manifestations

    Science.gov (United States)

    Nakamura, Yutaro; Suda, Takafumi

    2015-01-01

    Idiopathic pulmonary fibrosis (IPF) is a parenchymal lung disease characterized by progressive interstitial fibrosis. The clinical course of IPF can be unpredictable and may be punctuated by acute exacerbations. Although much progress is being made in unraveling the mechanisms underlying IPF, effective therapy for improving survival remains elusive. Longitudinal disease profiling, especially in terms of clinical manifestations in a large cohort of patients, should lead to proper management of the patients and development of new treatments for IPF. Appropriate multidisciplinary assessment in ongoing registries is required to achieve this. This review summarizes the current status of the diagnosis and clinical manifestations of IPF. PMID:27625576

  4. Idiopathic incapacitates in infancy and childhood

    International Nuclear Information System (INIS)

    Crankson, Stanley J.; Al-Rabeeh, Abdulla A.; Fischer, James D.; Al-Jaddan, Saud A.

    2003-01-01

    Idiopathic intussusception is an important cause of abdominal pain, bleeding per rectum and intestinal obstruction in infancy and childhood. The main aim of this study was to undertake a retrospective review of all chidren who presented with idiopathic intussusception over a 17-year period. The medical record of children who presented idiopathic intussusception from January 1984 to December 2000 at King Fahad National Guard Hospital ,Riyadh, Kingdom of Saudi Arabia were reviewed. The data obtained included age, sex clincal presentation, diagonstic investigations, mode of treatment, length of hospital stay and results. Thirty-three chidren (21 male, 12female) presented with 37 episodes of intussusception.Their mean age was 8.4 months (range 5 hours to 36 months ). Clinical features included rectal bleeding (81%), vomiting (78%), abdominal colic/pain (65%) and abdominal mass (62%) .All cases were ileocolic intussusception with no leading point. Barium enema was attempted in 36 cases with success in 20 (56%). Laparotomy was required in 16 csaes, manual reduction being successful in 11(30%) and 6 (16%) had bowel resection. At surgery, after attempted Barium reduction, 9(56%) cases had the intussusception already reduced to cecum. Seventy percent of the cases presented within 24 hours of onset of the symptoms.The 4 recurrences in 3 children had successful enema reduction. There was no mortality but 3 operative cases of this type required late surgery for adhesive intestinal obstruction including one requiring bowel resection. Idiopathic indussusception commonly presents as an ileo-colic type but is uncommon in our institution. The clinical features are classical, rectal bleeding being the most common. The majority presented within 24 hours of onset of symptoms and Barium enema reduction was sucessfull in 20 out of 36 cases in which it was attempted . Since most intussusception were already in the cecum at surgey after failed enema reduction could be considerd in stable

  5. Cognitive functions, electroencephalographic and diffusion tensor imaging changes in children with active idiopathic epilepsy.

    Science.gov (United States)

    A Yassine, Imane; M Eldeeb, Waleed; A Gad, Khaled; A Ashour, Yossri; A Yassine, Inas; O Hosny, Ahmed

    2018-07-01

    Neurocognitive impairment represents one of the most common comorbidities occurring in children with idiopathic epilepsy. Diagnosis of the idiopathic form of epilepsy requires the absence of any macrostructural abnormality in the conventional MRI. Though changes can be seen at the microstructural level imaged using advanced techniques such as the Diffusion Tensor Imaging (DTI). The aim of this work is to study the correlation between the microstructural white matter DTI findings, the electroencephalographic changes and the cognitive dysfunction in children with active idiopathic epilepsy. A comparative cross-sectional study, included 60 children with epilepsy based on the Stanford-Binet 5th Edition Scores was conducted. Patients were equally assigned to normal cognitive function or cognitive dysfunction groups. The history of the epileptic condition was gathered via personal interviews. All patients underwent brain Electroencephalography (EEG) and DTI, which was analyzed using FSL. The Fractional Anisotropy (FA) was significantly higher whereas the Mean Diffusivity (MD) was significantly lower in the normal cognitive function group than in the cognitive dysfunction group. This altered microstructure was related to the degree of the cognitive performance of the studied children with epilepsy. The microstructural alterations of the neural fibers in children with epilepsy and cognitive dysfunction were significantly related to the younger age of onset of epilepsy, the poor control of the clinical seizures, and the use of multiple antiepileptic medications. Children with epilepsy and normal cognitive functions differ in white matter integrity, measured using DTI, compared with children with cognitive dysfunction. These changes have important cognitive consequences. Copyright © 2018 Elsevier Inc. All rights reserved.

  6. Natural evolution from idiopathic photosensitive occipital lobe epilepsy to idiopathic generalized epilepsy in an untreated young patient.

    Science.gov (United States)

    Bonini, Francesca; Egeo, Gabriella; Fattouch, Jinan; Fanella, Martina; Morano, Alessandra; Giallonardo, Anna Teresa; di Bonaventura, Carlo

    2014-04-01

    Idiopathic photosensitive occipital lobe epilepsy (IPOE) is an idiopathic localization-related epilepsy characterized by age-related onset, specific mode of precipitation, occipital photic-induced seizures--frequently consisting of visual symptoms--and good prognosis. This uncommon epilepsy, which usually starts in childhood or adolescence, has rarely been observed in families in which idiopathic generalized epilepsy also affects other members. We describe a nuclear family in which the proband showed electro-clinical features of idiopathic photosensitive occipital lobe epilepsy in childhood, which subsequently evolved into absences and a single generalized tonico-clonic seizure in early adolescence. His mother had features suggestive of juvenile myoclonic epilepsy. This case illustrates a continuum between focal and generalized entities in the spectrum of the so-called idiopathic (genetically determined) epileptic syndromes. Copyright © 2013 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

  7. Idiopathic pulmonary fibrosis: current understanding of the pathogenesis and the status of treatment.

    Science.gov (United States)

    Khalil, Nasreen; O'Connor, Robert

    2004-07-20

    Idiopathic pulmonary fibrosis (IPF) is a progressive and lethal pulmonary fibrotic lung disease. The diagnostic histological changes are called usual interstitial pneumonia and are characterized by histological temporal heterogeneity, whereby normal lung tissue is interspersed with interstitial fibrosis, honeycomb cysts and fibroblast foci. Pulmonary functions show restricted volumes and capacities, preserved flows and evidence of decreased gas exchange. High-resolution computed axial tomography demonstrates evidence of fibrosis and lung remodelling such as honeycomb cysts and traction bronchiectasis. There is no known effective treatment for IPF, but lung transplantation improves survival.

  8. Nonsustained Repetitive Upper Septal Idiopathic Fascicular Left Ventricular Tachycardia: Rare Type of VT

    Directory of Open Access Journals (Sweden)

    Gokhan Aksan

    2016-05-01

    Full Text Available Upper septal fascicular ventricular tachycardia is a very rare form of idiopathic fascicular ventricular tachycardia. Upper septal fascicular tachycardia uses the posterior fascicle as the anterograde limb and the septal fascicle as the retrograde limb. When evaluating the electrocardiography for this form of tachycardia, the presence of narrow QRS morphology and normal axis may be misinterpreted as supraventricular tachycardia. Here, we report a very rare subtype of fascicular tachycardia that originates more proximally in the His-Purkinje system at the base of the heart.

  9. Iodine-123 metaiodobenzylguanidine imaging of the heart in idiopathic congestive cardiomyopathy and cardiac transplants

    International Nuclear Information System (INIS)

    Glowniak, J.V.; Turner, F.E.; Gray, L.L.; Palac, R.T.; Lagunas-Solar, M.C.; Woodward, W.R.

    1989-01-01

    Iodine-123 metaiodobenzylguanidine ([ 123 I]MIBG) is a norepinephrine analog which can be used to image the sympathetic innervation of the heart. In this study, cardiac imaging with [ 123 I]MIBG was performed in patients with idiopathic congestive cardiomyopathy and compared to normal controls. Initial uptake, half-time of tracer within the heart, and heart to lung ratios were all significantly reduced in patients compared to normals. Uptake in lungs, liver, salivary glands, and spleen was similar in controls and patients with cardiomyopathy indicating that decreased MIBG uptake was not a generalized abnormality in these patients. Iodine-123 MIBG imaging was also performed in cardiac transplant patients to determine cardiac nonneuronal uptake. Uptake in transplants was less than 10% of normals in the first 2 hr and nearly undetectable after 16 hr. The decreased uptake of MIBG suggests cardiac sympathetic nerve dysfunction while the rapid washout of MIBG from the heart suggests increased cardiac sympathetic nerve activity in idiopathic congestive cardiomyopathy

  10. Prevalence of vulvovaginitis and relation to physical findings in girls assessed for suspected child sexual abuse.

    Science.gov (United States)

    Rahman, Gisel; Ocampo, Dolores; Rubinstein, Anahí; Risso, Paula

    2015-10-01

    The presence of sexually transmitted infections (STIs) in patients with suspected sexual abuse is uncommon in the field of pediatrics. To establish the prevalence of anogenital findings and their relation to the presence of STIs in girls referred for suspected child sexual abuse. Retrospective study conducted between January 1st, 2003 and December 31st, 2013. Physical findings and detection of STIs in girls with suspected child sexual abuse were analyzed. One thousand thirty-four patients were included. Their median age was 7.9 years old. Anogenital findings were classified as class I (normal):38.4%, class II (nonspecific):38.1%, class III (specific):19.9% and class IV (definitive):3.6%. STIs were observed in 42 patients (4.1%). A relation was established between STIs and the classification of physical findings: 10 (class II: 9; class III: 1) Neisseria gonorrhoeae, 17 (class I: 2; class II: 8; class III: 7) Chlamydia trachomatis, 15 (class I: 2; class II: 10; class III: 3) Trichomonas vaginalis. Statistically significant differences for Trichomonas vaginalis (p= 0.01) and Neisseria gonorrhoeae (p < 0.0001) were observed, with predominance of nonspecific clinical signs. Both nonspecific and specific findings were similarly observed for Chlamydia trachomatis (p= 0.03). Most cases of girls with suspected child sexual abuse had normal or nonspecific anogenital findings. The prevalence of STIs in these girls is low. Trichomonas vaginalis and Neisseria gonorrhoeae were related to nonspecific findings, while both nonspecific and specific findings were observed for Chlamydia trachomatis.

  11. Idiopathic Noncirrhotic Portal Hypertension: An Appraisal

    Science.gov (United States)

    Lee, Hwajeong; Rehman, Aseeb Ur; Fiel, M. Isabel

    2016-01-01

    Idiopathic noncirrhotic portal hypertension is a poorly defined clinical condition of unknown etiology. Patients present with signs and symptoms of portal hypertension without evidence of cirrhosis. The disease course appears to be indolent and benign with an overall better outcome than cirrhosis, as long as the complications of portal hypertension are properly managed. This condition has been recognized in different parts of the world in diverse ethnic groups with variable risk factors, resulting in numerous terminologies and lack of standardized diagnostic criteria. Therefore, although the diagnosis of idiopathic noncirrhotic portal hypertension requires clinical exclusion of other conditions that can cause portal hypertension and histopathologic confirmation, this entity is under-recognized clinically as well as pathologically. Recent studies have demonstrated that variable histopathologic entities with different terms likely represent a histologic spectrum of a single entity of which obliterative portal venopathy might be an underlying pathogenesis. This perception calls for standardization of the nomenclature and formulation of widely accepted diagnostic criteria, which will facilitate easier recognition of this disorder and will highlight awareness of this entity. PMID:26563701

  12. Idiopathic constipation: A challenging but manageable problem.

    Science.gov (United States)

    Bischoff, Andrea; Brisighelli, Giulia; Dickie, Belinda; Frischer, Jason; Levitt, Marc A; Peña, Alberto

    2017-10-10

    A protocol to treat idiopathic constipation is presented. A contrast enema is performed in every patient and, when indicated, patients are initially submitted to a "clean out" protocol. All patients are started on a Senna-based laxative. The initial dosage is empirically determined and adjusted daily, during a one week period, based on history and abdominal radiographs, until the amount of Senna that empties the colon is reached. The management is considered successful when patients empty their colon daily and stop soiling. If the laxatives dose provokes abdominal cramping, distension, and vomiting, without producing bowel movements, patients are considered nonmanageable. From 2005 to 2012, 215 patients were treated. 121 (56%) were males. The average age was 8.2years (range: 1-20). 160 patients (74%) presented encopresis. 67 patients (32%) needed a clean out. After one week, 181 patients (84%) achieved successful management, with an average Senna dose of 67mg (range: 5-175mg). In 34 patients (16%) the treatment was unsuccessful: 19 were nonmanageable, 3 noncompliant, and 12 continued soiling. At a later follow-up (median: 329days) the success rate for 174 patients was 81%. We designed a successful protocol to manage idiopathic constipation. The key points are clean out before starting laxatives, individual adjustments of laxative, and radiological monitoring of colonic emptying. Level IV. Copyright © 2017 Elsevier Inc. All rights reserved.

  13. Idiopathic sclerosing encapsulating peritonitis (or abdominal cocoon

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    Legakis Nikolaos

    2006-02-01

    Full Text Available Abstract Background Idiopathic sclerosing encapsulating peritonitis (or abdominal cocoon is a rare cause of small bowel obstruction, especially in adult population. Diagnosis is usually incidental at laparotomy. We discuss one such rare case, outlining the fact that an intra-operative surprise diagnosis could have been facilitated by previous investigations. Case presentation A 56 year-old man presented in A&E department with small bowel ileus. He had a history of 6 similar episodes of small bowel obstruction in the past 4 years, which resolved with conservative treatment. Pre-operative work-up did not reveal any specific etiology. At laparotomy, a fibrous capsule was revealed, in which small bowel loops were encased, with the presence of interloop adhesions. A diagnosis of abdominal cocoon was established and extensive adhesiolysis was performed. The patient had an uneventful recovery and follow-up. Conclusion Idiopathic sclerosing encapsulating peritonitis, although rare, may be the cause of a common surgical emergency such as small bowel ileus, especially in cases with attacks of non-strangulating obstruction in the same individual. A high index of clinical suspicion may be generated by the recurrent character of small bowel ileus combined with relevant imaging findings and lack of other plausible etiologies. Clinicians must rigorously pursue a preoperative diagnosis, as it may prevent a "surprise" upon laparotomy and result in proper management.

  14. Idiopathic sclerosing encapsulating peritonitis (or abdominal cocoon).

    Science.gov (United States)

    Serafimidis, Costas; Katsarolis, Ioannis; Vernadakis, Spyros; Rallis, George; Giannopoulos, George; Legakis, Nikolaos; Peros, George

    2006-02-13

    Idiopathic sclerosing encapsulating peritonitis (or abdominal cocoon) is a rare cause of small bowel obstruction, especially in adult population. Diagnosis is usually incidental at laparotomy. We discuss one such rare case, outlining the fact that an intra-operative surprise diagnosis could have been facilitated by previous investigations. A 56 year-old man presented in A&E department with small bowel ileus. He had a history of 6 similar episodes of small bowel obstruction in the past 4 years, which resolved with conservative treatment. Pre-operative work-up did not reveal any specific etiology. At laparotomy, a fibrous capsule was revealed, in which small bowel loops were encased, with the presence of interloop adhesions. A diagnosis of abdominal cocoon was established and extensive adhesiolysis was performed. The patient had an uneventful recovery and follow-up. Idiopathic sclerosing encapsulating peritonitis, although rare, may be the cause of a common surgical emergency such as small bowel ileus, especially in cases with attacks of non-strangulating obstruction in the same individual. A high index of clinical suspicion may be generated by the recurrent character of small bowel ileus combined with relevant imaging findings and lack of other plausible etiologies. Clinicians must rigorously pursue a preoperative diagnosis, as it may prevent a "surprise" upon laparotomy and result in proper management.

  15. [Biologic therapy in idiopathic inflammatory myopathy].

    Science.gov (United States)

    Selva-O'Callaghan, Albert; Ramos Casals, Manel; Grau Junyent, Josep M

    2014-09-15

    The aim of this article is to study the evidence-based knowledge related to the use of biological therapies in patients diagnosed with idiopathic inflammatory myopathy (dermatomyositis, polymyositis and inclusion body myositis). In this review the leading published studies related to the use of biological therapy in patients with myositis are analysed; mainly those with high methodological standards, that means randomized and controlled studies. Methodological drawbacks due to the rarity and heterogeneity of these complex diseases are also addressed. Up to now is not possible to ascertain the biologics as a recommended therapy in patients with myositis, at least based in the current evidence-based knowledge, although it can not be neglected as a therapeutic option in some clinical situations, taking into account the scarce of effective treatments in those patients, especially in refractory myositis. Future studies probably will help to better define the role of biological therapies in patients with idiopathic inflammatory myopathy. Copyright © 2013 Elsevier España, S.L.U. All rights reserved.

  16. Idiopathic Harlequin syndrome – case report

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    Marcelina Grochowiec

    2015-09-01

    Full Text Available Introduction. Harlequin syndrome is a very rare neurological condition characterized by redness and excessive sweating of one half of the face in response to exercise and emotions. In most cases this disorder is not life-threatening. Objective. To present diagnostic difficulties of Harlequin syndrome in dermatological practice. Case report. We present a case of a 30-year-old man with redness and excessive sweating of the right half of the face as a result of exercise that was observed during the diagnosis of chronic urticaria at the Department of Dermatology. The patient was examined ophthalmologically and neurologically, had a CT scan of the head, and the Minor test performed. Idiopathic Harlequin syndrome was diagnosed based on case history and workup results. Conclusions . Harlequin syndrome occurs most often in the form of an idiopathic condition, but neurologic and ophthalmologic assessment should be performed since some diseases, such as brainstem infarction and schwannoma of the upper chest, may initially appear as Harlequin syndrome.

  17. Intravenous immunoglobulin therapy leading to dramatic improvement in a patient with systemic juvenile idiopathic arthritis and severe pericarditis resistant to steroid pulse therapy.

    Science.gov (United States)

    Aizawa-Yashiro, Tomomi; Oki, Eishin; Tsuruga, Kazushi; Nakahata, Tohru; Ito, Etsuro; Tanaka, Hiroshi

    2012-05-01

    A 7-year-old Japanese boy with a 4-month history of systemic juvenile idiopathic arthritis (s-JIA) experienced disease flare with spiking fever, exanthema and arthralgia. He then developed progressive dyspnea due to severe pericarditis, and proinflammatory hypercytokinemia was suspected. Methylprednisolone pulse therapy was ineffective and echocardiography showed massive pericardial effusion had persisted. Alternatively, subsequent intravenous immunoglobulin (IVIG) therapy resulted in dramatic resolution of the pericardial effusion, and his general condition significantly improved within a few days. This case report may lend further support the use of IVIG for selected patients with s-JIA and severe pericarditis.

  18. The child with suspected hearing loss

    Directory of Open Access Journals (Sweden)

    Rajan P

    2013-10-01

    Full Text Available An 8-year-old girl was noticed by her parents to be less attentive and she would respond only after being called several times. She had just recovered from an upper respiratory tract infection two weeks before. The parents brought her to see a primary care physician. The patient had no other complaints, and the rest of the history was unremarkable. Physical examination was normal except for the otoscopic findings shown below (Figure 1 Tuning fork tests indicated conductive deafness.

  19. A simple MRI protocol in patients with clinically suspected appendicitis: results in 138 patients and effect on outcome of appendectomy

    International Nuclear Information System (INIS)

    Cobben, Lodewijk; Groot, Ingrid; Kingma, Lucas; Coerkamp, Emile; Puylaert, Julien; Blickman, Johan

    2009-01-01

    To establish the value of breathhold magnetic resonance imaging (MRI) in the diagnosis of acute appendicitis. Over a 14-month period, 138 patients clinically suspected of having appendicitis were evaluated prospectively with MRI and comprised the study group. Fast turbo spin-echo breathhold T1, T2 and T2 fat suppression sequences were used in coronal and axial planes. The imaging results were recorded separately and subsequently correlated with clinical, radiological and histopathological follow-up. The effect of imaging strategies in patients suspected of appendicitis on hospital resources was calculated. Sixty-two of the 138 patients had a histopathologically proven appendicitis. MRI determined appendicitis in 63 patients, with one examination being false positive. The resulting sensitivity and specificity were 100% and 99%, respectively. MRI showed an alternative diagnosis in 41 of the 75 remaining patients. In 22 of the remaining 34 patients, a normal appendix was depicted with MRI. In two patients, where MRI showed no appendicitis, an alternative diagnosis or normal appendix, an unnecessary appendectomy was performed. The overall effect of using MRI in patients suspected of appendicitis on the use of hospital resources could have been a net saving between EUR 55,746 and EUR 72,534. MRI has a high accuracy in detecting and excluding appendicitis, an alternative diagnosis or showing the normal appendix, and can be a valuable and cost-effective tool in the workup of patients clinically suspected of having appendicitis. (orig.)

  20. A simple MRI protocol in patients with clinically suspected appendicitis: results in 138 patients and effect on outcome of appendectomy

    Energy Technology Data Exchange (ETDEWEB)

    Cobben, Lodewijk [Medisch Centrum Haaglanden, Department of Radiology, Leidschendam (Netherlands); Groot, Ingrid [Medisch Centrum Haaglanden, Department of Surgery, Leidschendam (Netherlands); Kingma, Lucas; Coerkamp, Emile; Puylaert, Julien [Medisch Centrum Haaglanden, Department of Radiology, Den Haag (Netherlands); Blickman, Johan [Universitair Medisch Centrum St Radboud, Department of Radiology, Geert Grooteplein 10, GA, Nijmegen (Netherlands)

    2009-05-15

    To establish the value of breathhold magnetic resonance imaging (MRI) in the diagnosis of acute appendicitis. Over a 14-month period, 138 patients clinically suspected of having appendicitis were evaluated prospectively with MRI and comprised the study group. Fast turbo spin-echo breathhold T1, T2 and T2 fat suppression sequences were used in coronal and axial planes. The imaging results were recorded separately and subsequently correlated with clinical, radiological and histopathological follow-up. The effect of imaging strategies in patients suspected of appendicitis on hospital resources was calculated. Sixty-two of the 138 patients had a histopathologically proven appendicitis. MRI determined appendicitis in 63 patients, with one examination being false positive. The resulting sensitivity and specificity were 100% and 99%, respectively. MRI showed an alternative diagnosis in 41 of the 75 remaining patients. In 22 of the remaining 34 patients, a normal appendix was depicted with MRI. In two patients, where MRI showed no appendicitis, an alternative diagnosis or normal appendix, an unnecessary appendectomy was performed. The overall effect of using MRI in patients suspected of appendicitis on the use of hospital resources could have been a net saving between EUR 55,746 and EUR 72,534. MRI has a high accuracy in detecting and excluding appendicitis, an alternative diagnosis or showing the normal appendix, and can be a valuable and cost-effective tool in the workup of patients clinically suspected of having appendicitis. (orig.)

  1. Quantitative electromyographic characteristics of idiopathic unilateral vocal fold paralysis.

    Science.gov (United States)

    Chang, Wei-Han; Fang, Tuan-Jen; Li, Hsueh-Yu; Jaw, Fu-Shan; Wong, Alice M K; Pei, Yu-Cheng

    2016-11-01

    Unilateral vocal fold paralysis with no preceding causes is diagnosed as idiopathic unilateral vocal fold paralysis. However, comprehensive guidelines for evaluating the defining characteristics of idiopathic unilateral vocal fold paralysis are still lacking. In the present study, we hypothesized that idiopathic unilateral vocal fold paralysis may have different clinical and neurologic characteristics from unilateral vocal fold paralysis caused by surgical trauma. Retrospective, case series study. Patients with unilateral vocal fold paralysis were evaluated using quantitative laryngeal electromyography, videolaryngostroboscopy, voice acoustic analysis, the Voice Outcome Survey, and the Short Form-36 Health Survey quality-of-life questionnaire. Patients with idiopathic and iatrogenic vocal fold paralysis were compared. A total of 124 patients were recruited. Of those, 17 with no definite identified causes after evaluation and follow-up were assigned to the idiopathic group. The remaining 107 patients with surgery-induced vocal fold paralysis were assigned to the iatrogenic group. Patients in the idiopathic group had higher recruitment of the thyroarytenoid-lateral cricoarytenoid muscle complex and better quality of life compared with the iatrogenic group. Idiopathic unilateral vocal fold paralysis has a distinct clinical presentation, with relatively minor denervation changes in the involved laryngeal muscles, and less impact on quality of life compared with iatrogenic vocal fold paralysis. 4. Laryngoscope, 126:E362-E368, 2016. © 2016 The American Laryngological, Rhinological and Otological Society, Inc.

  2. Evidence for cognitive vestibular integration impairment in idiopathic scoliosis patients

    Directory of Open Access Journals (Sweden)

    Mercier Pierre

    2009-08-01

    Full Text Available Abstract Background Adolescent idiopathic scoliosis is characterized by a three-dimensional deviation of the vertebral column and its etiopathogenesis is unknown. Various factors cause idiopathic scoliosis, and among these a prominent role has been attributed to the vestibular system. While the deficits in sensorimotor transformations have been documented in idiopathic scoliosis patients, little attention has been devoted to their capacity to integrate vestibular information for cognitive processing for space perception. Seated idiopathic scoliosis patients and control subjects experienced rotations of different directions and amplitudes in the dark and produced saccades that would reproduce their perceived spatial characteristics of the rotations (vestibular condition. We also controlled for possible alteration of the oculomotor and vestibular systems by measuring the subject's accuracy in producing saccades towards memorized peripheral targets in absence of body rotation and the gain of their vestibulo-ocular reflex. Results Compared to healthy controls, the idiopathic scoliosis patients underestimated the amplitude of their rotations. Moreover, the results revealed that idiopathic scoliosis patients produced accurate saccades to memorized peripheral targets in absence of body rotation and that their vestibulo-ocular reflex gain did not differ from that of control participants. Conclusion Overall, results of the present study demonstrate that idiopathic scoliosis patients have an alteration in cognitive integration of vestibular signals. It is possible that severe spine deformity developed partly due to impaired vestibular information travelling from the cerebellum to the vestibular cortical network or alteration in the cortical mechanisms processing the vestibular signals.

  3. TAFRO syndrome: New subtype of idiopathic multicentric Castleman disease

    Directory of Open Access Journals (Sweden)

    Gordan Srkalovic

    2017-05-01

    Full Text Available Castleman disease (CD describes a group of three rare and poorly understood lymphoproliferative disorders that have heterogeneous clinical symptoms and common lymph node histopathological features. Unicentric CD (UCD involves a single region of enlarged nodes. Multicentric CD (MCD involves multiple regions of enlarged lymph nodes, constitutional symptoms, and organ dysfunction due to a cytokine storm often including interleukin 6. MCD is further divided into Human Herpes Virus-8 (HHV-8-associated MCD, which occurs in immunocompromised individuals, and HHV-8-negative/idiopathic MCD (iMCD. Recently, iMCD has been further sub-divided into patients with TAFRO syndrome, which involves thrombocytopenia (T, anasarca (A, fevers (F, reticulin myelofibrosis (R, organomegaly (O, and normal or only slightly elevated immunoglobulin levels, and those who do not have TAFRO syndrome. Non-TAFRO iMCD patients typically have thrombocytosis, less severe fluid accumulation, and hypergammaglobulinemia. iMCD patients with TAFRO syndrome may have a worse prognosis, but more research is needed.

  4. IDIOPATHIC INTRACRANIAL HYPERTENSION IN A WOMAN WITH SCHIZOPHRENIA

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    Ivan N. Dimitrov

    2012-02-01

    Full Text Available Idiopathic intracranial hypertension (IIH or benign intracranial hypertension is a neurological syndrome characterized by elevated intracranial pressure. This uncommon disorder occurs primarily in obese women aged 10 to 50 years, sometimes in association with endocrine and metabolic dysfunction, with systemic diseases or when treated with multiple medications. We describe a case of IIH in a 43-year-old woman with schizophrenia treated with risperidone, demonstrating a typical clinical picture of benign intracranial hypertension. For the 5 years of treatment with risperidone she put on 35 kg in total (BMI> 35; for the last 2-3 months she began to complain of visual obscurations, nausea with vomiting. Ophthalmoscopy revealed bilateral asymmetric papilledema (OD>OS. Magnetic resonance imaging was normal, intracranial pressure was elevated IIH was diagnosed. Risperidone was discontinued and replaced with Seroquel 200 mg daily. Treatment with furosemide and mannitol 10 % was initiated. Papilledema resolved completely over the next 2 months. The patient was followed-up for four years after risperidone withdrawal. Weight loss of 28 kg was noted for four years. There were no relapses of headache, nausea, visual obscuration. Ophthalmologic examination revealed no papilledema.We suggest that prolonged use of antipsychotics, such as risperidone, should require proper surveillance for possible development of IIH and routine ophthalmologic examinations should be performed.

  5. A Network Investigation on Idiopathic Hypogonadotropic Hypogonadism in China

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    Weiwei Zhao

    2013-01-01

    Full Text Available Idiopathic hypogonadotropic hypogonadism (IHH is a rare condition in which puberty does not take place naturally. We aimed to develop and follow an internet-based cohort and to improve our understanding of the disease. We established an internet-based questionnaire survey. A total of 74 male IHH patients were recruited from the Chinese largest IHH network social group. The clinical symptoms before treatment mainly included small testis, underdeveloped secondary sexual characteristics, and sexual dysfunction. After treatment, the penis length, testicular volume, external genital organ development, pubic hair, beard, laryngeal prominence, erection, and spermatorrhea were improved significantly (P<0.001. 18.9% of the patients completed fertility; however, more than half of the patients still complained of poor happiness and low physical strength. In addition, improvements in penis and pubic hair development, testosterone normalization and the physical strength in IHH patients who received gonadotropin and androgen replacement therapy were better than in those who received single gonadotropin therapy (P<0.05 for all. In conclusion, disease-specific network investigation can be used as an alternative method of medical research for rare diseases. The results of our cross-sectional study showed the effectiveness of hormone replacement therapy for IHH and implied that gonadotropin and androgen replacement therapy may be superior to gonadotropin treatment alone.

  6. Abdominal and lower back pain in pediatric idiopathic stabbing headache.

    Science.gov (United States)

    Kakisaka, Yosuke; Ohara, Tomoichiro; Hino-Fukuyo, Naomi; Uematsu, Mitsugu; Kure, Shigeo

    2014-01-01

    Idiopathic stabbing headache (ISH) is a primary headache syndrome characterized by transient, sharp, stabbing pains located in the first division of the trigeminal nerve. Reports of pediatric ISH are rare, and extracephalic pain in pediatric ISH is extremely rare. Here we report the case of a 7-year-old male patient suffering from frequent, short, stabbing headache, which was occasionally associated with abdominal and lower back pain. Various investigations were normal. He was diagnosed with ISH, and valproic acid was administered to relieve his headache and accompanying symptoms. Our case demonstrates that abdominal and lower back pain may occur in pediatric ISH. This case may provide new evidence linking ISH and migraine by showing that extracephalic symptoms accompanying ISH are similar to those of migraine. We hypothesize that the mechanism underlying the headache and abdominal and lower back pain associated with ISH may be similar to that of a migraine headache. Accumulating additional cases by asking specific questions regarding the presence of the unusual symptoms presented in our case may help to establish a detailed clinical profile of these unfamiliar and peculiar symptoms in the pediatric ISH population.

  7. Bone scintigraphy in idiopathic aseptic femoral head necrosis (IAFHN)

    Energy Technology Data Exchange (ETDEWEB)

    Dodig, D; Ugarkovic, B; Orlic, D

    1983-01-01

    Idiopathic aseptic femoral head necrosis (IAFHN) is a disease of unknown aetiology most commonly affecting adults, producing functional impairment of the hip joint and immobility. Characteristic macroscopic, microscopic and radiologic features include: (1) changes in joint cartilage, (2) subchondral necrotic area, (3) changes in fibrous tissue, and (4) osteosclerosis and vascular proliferation. The diagnosis is based on clinical and X-ray examinations. More recently scintigraphy has been used in diagnosing IAFHN. It is a very sensitive, but non-specific method. Nineteen patients aged 25-57 years were included in our study. Scintigrams were divided in four groups: (1) normal, (2) uniformly increased activity in the femoral head, (3) non-uniformly increased activity in the femoral head, and (4) a photon deficient area in the femoral head. A comparison of scintigraphy and radiological examination suggests that a photon deficient area corresponds with stage III of radiological classification, non-uniformly increased activity with stage II, and uniformly increased activity with stage I. These results indicate that scintigraphy can be used for staging of disease.

  8. Kallman syndrome versus idiopathic hypogonadotropic hypogonadism at MR imaging

    Energy Technology Data Exchange (ETDEWEB)

    Vogl, T.J.; Stemmler, J.; Bergman, C.; Balzer, J.O.; Felix, R. [Free Univ. of Berlin (Germany); Heye, B.; Schopohl, J.; Danek, A. [Univ. of Munich (Germany)

    1994-04-01

    To identify morphologic differences between Kallman syndrome (KS) and idiopathic hypogonadotropic hypogonadism (IHH) and establish a role for magnetic resonance (MR) imaging in these disorders. Twenty-eight patients were compared with 10 eugonal male volunteers. Eighteen patients had KS (hypogonadotropic hypogonadism with anosmia) and 10 had IHH. All participants underwent hormone analysis, a sniff-bottle smell test, and gadolinium-enhanced MR imaging. Changes in the hypothalamic-hypophyseal region and the rhinencephalon were evaluated. MR imaging revealed intracranial morphologic changes in all patients on plain T1-weighted sections. Seventeen patients with KS demonstrated aplasia of an olfactory bulb; one olfactory sulcus was absent in six, rudimentary in four, and normal in eight. Olfactory bulbs were present in all 10 IHH patients and three showed one slightly hypoplastic bulb. Ten patients with KS and three with IHH showed an enlarged paranasal sinus system. Further MR findings were similar. MR imaging demonstrates abnormalities of the rhinencephalon present in KS patients and occasionally absent in IHH patients. 18 refs., 10 figs., 1 tab.

  9. Genomic profiles of lung cancer associated with idiopathic pulmonary fibrosis.

    Science.gov (United States)

    Hwang, Ji An; Kim, Deokhoon; Chun, Sung-Min; Bae, SooHyun; Song, Joon Seon; Kim, Mi Young; Koo, Hyun Jung; Song, Jin Woo; Kim, Woo Sung; Lee, Jae Cheol; Kim, Hyeong Ryul; Choi, Chang-Min; Jang, Se Jin

    2018-01-01

    Little is known about the pathogenesis or molecular profiles of idiopathic pulmonary fibrosis-associated lung cancer (IPF-LC). This study was performed to investigate the genomic profiles of IPF-LC and to explore the possibility of defining potential therapeutic targets in IPF-LC. We assessed genomic profiles of IPF-LC by using targeted exome sequencing (OncoPanel version 2) in 35 matched tumour/normal pairs surgically resected between 2004 and 2014. Germline and somatic variant calling was performed with GATK HaplotypeCaller and MuTect with GATK SomaticIndelocator, respectively. Copy number analysis was conducted with CNVkit, with focal events determined by Genomic Identification of Significant Targets in Cancer 2.0, and pathway analysis (KEGG) with DAVID. Germline mutations in TERT (rs2736100, n = 33) and CDKN1A (rs2395655, n = 27) associated with idiopathic pulmonary fibrosis risk were detected in most samples. A total of 410 somatic mutations were identified, with an average of 11.7 per tumour, including 69 synonymous, 177 missense, 17 nonsense, 1 nonstop and 11 splice-site mutations, and 135 small coding indels. Spectra of the somatic mutations revealed predominant C > T transitions despite an extensive smoking history in most patients, suggesting a potential association between APOBEC-related mutagenesis and the development of IPF-LC. TP53 (22/35, 62.9%) and BRAF (6/35, 17.1%) were found to be significantly mutated in IPF-LC. Recurrent focal amplifications in three chromosomal loci (3q26.33, 7q31.2, and 12q14.3) and 9p21.3 deletion were identified, and genes associated with the JAK-STAT signalling pathway were significantly amplified in IPF-LC (P = 0.012). This study demonstrates that IPF-LC is genetically characterized by the presence of somatic mutations reflecting a variety of environmental exposures on the background of specific germline mutations, and is associated with potentially targetable alterations such as BRAF mutations. Copyright © 2017

  10. Single photon emission computed tomography in children with idiopathic seizures

    International Nuclear Information System (INIS)

    Hara, Masafumi; Takahashi, Mutsumasa; Kojima, Akihiro; Shimomura, Osamu; Kinoshita, Rumi; Tomiguchi, Seiji; Taku, Keiichi; Miike, Teruhisa

    1991-01-01

    Single photon emission computed tomography (SPECT) with N-isoprophyl-p [ 123 I]-iodoamphetamine (IMP), X-ray computed tomography (X-CT), and magnetic resonance imaging (MRI) were performed in 20 children with idiopathic seizures. In children with idiopathic seizures, SPECT could detect the abnormal sites at the highest rate (45%) compared with CT (10%) and MRI (12%), but the abnormal sites on SPECT correlated poorly with the foci on electroencephalograph (EEG). Idiopathic epilepsy with hypoperfusion on SPECT was refractory to treatment and was frequently associated with mental and/or developmental retardation. Perfusion defects on SPECT scans probably affect the development and maturation of the brain in children. (author)

  11. Antibodies to myelin oligodendrocyte glycoprotein in idiopathic optic neuritis.

    Science.gov (United States)

    Nakajima, Hideki; Motomura, Masakatsu; Tanaka, Keiko; Fujikawa, Azusa; Nakata, Ruka; Maeda, Yasuhiro; Shima, Tomoaki; Mukaino, Akihiro; Yoshimura, Shunsuke; Miyazaki, Teiichiro; Shiraishi, Hirokazu; Kawakami, Atsushi; Tsujino, Akira

    2015-04-02

    To investigate the differences of clinical features, cerebrospinal fluid (CSF), MRI findings and response to steroid therapies between patients with optic neuritis (ON) who have myelin oligodendrocyte glycoprotein (MOG) antibodies and those who have seronegative ON. We recruited participants in the department of neurology and ophthalmology in our hospital in Japan. We retrospectively evaluated the clinical features and response to steroid therapies of patients with ON. Sera from patients were tested for antibodies to MOG and aquaporin-4 (AQP4) with a cell-based assay. Between April 2009 and March 2014, we enrolled serial 57 patients with ON (27 males, 30 females; age range 16-84 years) who ophthalmologists had diagnosed as having or suspected to have ON with acute visual impairment and declined critical flicker frequency, abnormal findings of brain MRI, optical coherence tomography and fluorescein fundus angiography at their onset or recurrence. We excluded those patients who fulfilled the diagnostic criteria of neuromyelitis optica (NMO)/NMO spectrum disorders (NMOSD), MS McDonald's criteria, and so on. Finally we defined 29 patients with idiopathic ON (14 males, 15 females, age range 16-84 years). 27.6% (8/29) were positive for MOG antibodies and 3.4% (1/29) were positive for AQP4. Among the eight patients with MOG antibodies, five had optic pain (p=0.001) and three had prodromal infection (p=0.179). Three of the eight MOG-positive patients showed significantly high CSF levels of myelin basic protein (p=0.021) and none were positive for oligoclonal band in CSF. On MRIs, seven MOG-positive patients showed high signal intensity on optic nerve, three had a cerebral lesion and one had a spinal cord lesion. Seven of the eight MOG-positive patients had a good response to steroid therapy. Although not proving primary pathogenicity of anti-MOG antibodies, the present results indicate that the measurement of MOG antibodies is useful in diagnosing and treating ON

  12. Idiopath=ic Granulomatous Lobular Mastitis Masquerading as a Breast Tumor: A Case Report

    OpenAIRE

    Raman R, Thulasi; Manimaran, D

    2016-01-01

    Introduction Idiopathic granulomatous lobular mastitis (IGLM) is an inflammatory disease of the breast with an obscure etiology. It occurs mainly in women of reproductive age, and the lesion mimics carcinoma of the breast both clinically and radiologically Case Presentation We present the case of a 29-year-old female who visited our hospital in Kancheepuram, Tamil Nadu, with a 4 ? 3 cm lump in the upper outer quadrant of her left breast. The clinical and radiological findings were indicative ...

  13. Using Internet Artifacts to Profile a Child Pornography Suspect

    OpenAIRE

    Marcus K. Rogers; Kathryn C. Seigfried-Spellar

    2014-01-01

    Digital evidence plays a crucial role in child pornography investigations. However, in the following case study, the authors argue that the behavioral analysis or “profiling” of digital evidence can also play a vital role in child pornography investigations. The following case study assessed the Internet Browsing History (Internet Explorer Bookmarks, Mozilla Bookmarks, and Mozilla History) from a suspected child pornography user’s computer. The suspect in this case claimed to be conducting an...

  14. Talking heads : interviewing suspects from a cultural perspective

    OpenAIRE

    Beune, K.

    2009-01-01

    Although the literature on the interviewing of suspects has increased over the past decade, research on the use and effectiveness of police strategies and their boundary conditions is very rare. The present dissertation aims to fill this void by identifying behaviors that appeal to and persuade suspects to talk (i.e., influencing behavior), focusing on the effects of such behaviors and their dependency on cultural context (low-context vs. high-context). In doing so, we depart from a theoretic...

  15. Interviewing strategically to elicit admissions from guilty suspects.

    Science.gov (United States)

    Tekin, Serra; Granhag, Pär Anders; Strömwall, Leif; Giolla, Erik Mac; Vrij, Aldert; Hartwig, Maria

    2015-06-01

    In this article we introduce a novel interviewing tactic to elicit admissions from guilty suspects. By influencing the suspects' perception of the amount of evidence the interviewer holds against them, we aimed to shift the suspects' counterinterrogation strategies from less to more forthcoming. The proposed tactic (SUE-Confrontation) is a development of the Strategic Use of Evidence (SUE) framework and aims to affect the suspects' perception by confronting them with statement-evidence inconsistencies. Participants (N = 90) were asked to perform several mock criminal tasks before being interviewed using 1 of 3 interview techniques: (a) SUE-Confrontation, (b) Early Disclosure of Evidence, or (c) No Disclosure of Evidence. As predicted, the SUE-Confrontation interview generated more statement-evidence inconsistencies from suspects than the Early Disclosure interview. Importantly, suspects in the SUE-Confrontation condition (vs. Early and No disclosure conditions) admitted more self-incriminating information and also perceived the interviewer to have had more information about the critical phase of the crime (the phase where the interviewer lacked evidence). The findings show the adaptability of the SUE-technique and how it may be used as a tool for eliciting admissions. (c) 2015 APA, all rights reserved).

  16. Chronic Idiopathic Neutrophilia in Two Twins

    Directory of Open Access Journals (Sweden)

    Roberto Miniero

    2014-01-01

    Full Text Available Neutrophilia in adults refers to an alteration in the total number of blood neutrophils that is in excess of about 7500 cells/μL. This definition is restrictive in childhood as neutrophil count is age-dependent. Chronic Idiopathic Neutrophilia (CIN refers to a condition that persists for many years in individuals who appear otherwise healthy. CIN is rarely mentioned in scientific literature and in academic books of hematology; only few words are dedicated to this topic. We report a case study of two twins with CIN followed from the first year of life to 24 years of age. To the best of our knowledge this is the first case report of two twins with CIN followed through a long period of time. We believe that our observation may contribute to better understand and characterize this hematologic abnormality.

  17. Radiographic manifestations of diffuse idiopathic skeletal hyperostosis

    International Nuclear Information System (INIS)

    Ono, Minoru; Kudo, Sho; Russell, W.J.

    1984-09-01

    A relatively high frequency of hyperostosis in various body sites was found in 240 subjects with ''diffuse idiopathic skeletal hyperostosis'' (DISH) in the thoracic spine over an average observation period of 11 years. The onset and initial appearance of DISH in the thoracic spine were identified in 69 cases. The age at onset ranged from 40 to 65 years, and the initial sites involved were usually T9-10 and T10-11. As criteria in differentiating DISH from spondylosis deformans the hyperostoses anterior to the vertebral body itself appeared to be more important than the ''bridges'' anterior to the intervertebral spaces. Although it was difficult to differentiate the early appearance of DISH from spondylosis deformans in some cases, these long-term observations suggest that they are different entities. (author)

  18. Imaging findings in idiopathic pelvic fibrosis

    International Nuclear Information System (INIS)

    Wiesner, W.; Bongartz, G.; Stoffel, F.

    2001-01-01

    Two patients presented with ureteric obstruction, and voiding symptoms and constipation, respectively, and were examined by means of intravenous urography and computed tomography. One patient was additionally examined by means of MR tomography. After CT (performed in both patients) and MRT (performed in one patient) had shown a diffuse, contrast-enhancing, infiltrating process in the small pelvis with infiltration of adjacent organs and vessels, surgical biopsy proved the diagnosis of idopathic pelvic fibrosis. Extension of retroperitoneal fibrosis below the pelvic rim is very rare. Clinical symptoms of pelvic fibrosis are variable and imaging findings may lead to a broad list of differential diagnoses. We present two patients with idiopathic pelvic fibrosis and discuss radiological findings and differential diagnoses of this rare disease. (orig.)

  19. Cognitive function in idiopathic intracranial hypertension

    DEFF Research Database (Denmark)

    Yri, Hanne Maria; Fagerlund, Birgitte; Forchhammer, Hysse Birgitte

    2014-01-01

    -up. At the time of testing, none of the patients took medication potentially affecting cognitive function. Controls were 31 healthy age-matched and sex-matched volunteers from the local community. OUTCOME MEASURES: Executive function, working memory, visuospatial memory, processing speed, attention and reaction......OBJECTIVE: To explore the extent and nature of cognitive deficits in patients with idiopathic intracranial hypertension (IIH) at the time of diagnosis and after 3 months of treatment. DESIGN: Prospective case-control study. SETTING: Neurological department, ophthalmological department...... time assessed by a comprehensive neuropsychological test battery consisting of validated computerised (Cambridge neuropsychological test automated battery) and paper-and-pencil tests. RESULTS: Patients with IIH performed significantly worse than controls in four of six cognitive domains (p≤0...

  20. Diffuse Idiopathic Skeletal Hyperosteosis: A Review

    Directory of Open Access Journals (Sweden)

    Sevgi İkbali Afşar

    2015-12-01

    Full Text Available Diffuse idiopathic skeletal hyperosteosis (DISH is also known as Forestier disease and is a systemic non-inflammatory disorder seen more commonly in males and elderly. It is characterized by calcification of the anterior longitudinal ligament of the vertebral column and various extraspinal ligaments. It is usually asymptomatic while the most common symptoms are spinal pain, limited range of spinal motion and dysphagia due to esophagus compression. The etiopathogenesis is not clear. It is commonly seen together with diabetes mellitus, obesity, hyperinsulinemia, hypertension and lipid and purine metabolism disorders, indicating an association with metabolic disorders. Recent studies have emphasized that the pathological calcification of the anterior longitudinal ligament plays a role in the pathophysiology. The aim of this study was to summarize new pathogenetic, clinical and therapeutic insights of this disease, based on published literature.

  1. Malignant Hyperthermia and Idiopathic HyperCKemia

    Directory of Open Access Journals (Sweden)

    Pashtoon Murtaza Kasi

    2011-01-01

    Full Text Available Malignant hyperthermia (MH is a rare but life-threatening condition that is more frequently encountered and discussed within the anesthesia literature. Here we through a case specifically discuss the susceptibility of individuals and/or families with asymptomatic unexplained elevations of creatine kinase (CK, also frequently referred to as hyperCKemia or idiopathic hyperCKemia (IHCK in recent reports. The clinical implications would be to underscore the importance of this as a susceptibility to developing MH and highlight the importance of genetic susceptibility testing in such cases. Anesthesiologists and critical care intensivists as well as primary care physicians should keep this in mind when seeing patients with asymptomatic hyperCKemia and potentially inform them about the possibility of developing MH if exposed to triggering agents. Genetic susceptibility testing should be considered if available and family members should also receive nontriggering agents when undergoing anesthesia and wear Medic Alert tags.

  2. Dysphagia due to Diffuse Idiopathic Skeletal Hyperostosis

    Directory of Open Access Journals (Sweden)

    Masafumi Ohki

    2012-01-01

    Full Text Available Diffuse idiopathic skeletal hyperostosis (DISH is usually asymptomatic. However, rarely, it causes dysphagia, hoarseness, dyspnea, snoring, stridor, and laryngeal edema. Herein, we present a patient with DISH causing dysphagia. A 70-year-old man presented with a 4-month history of sore throat, dysphagia, and foreign body sensation. Flexible laryngoscopy revealed a leftward-protruding posterior wall in the hypopharynx. Computed tomography and magnetic resonance imaging revealed a bony mass pushing, anteriorly, on the posterior hypopharyngeal wall. Ossification included an osseous bridge involving 5 contiguous vertebral bodies. Dysphagia due to DISH was diagnosed. His symptoms were relieved by conservative therapy using anti-inflammatory drugs. However, if conservative therapy fails and symptoms are severe, surgical treatments must be considered.

  3. Imaging findings in idiopathic pelvic fibrosis

    Energy Technology Data Exchange (ETDEWEB)

    Wiesner, W.; Bongartz, G. [Inst. of Diagnostic Radiology University Hospital Basel (Switzerland); Stoffel, F. [Inst. of Urology, University Hospital Basel (Switzerland)

    2001-04-01

    Two patients presented with ureteric obstruction, and voiding symptoms and constipation, respectively, and were examined by means of intravenous urography and computed tomography. One patient was additionally examined by means of MR tomography. After CT (performed in both patients) and MRT (performed in one patient) had shown a diffuse, contrast-enhancing, infiltrating process in the small pelvis with infiltration of adjacent organs and vessels, surgical biopsy proved the diagnosis of idopathic pelvic fibrosis. Extension of retroperitoneal fibrosis below the pelvic rim is very rare. Clinical symptoms of pelvic fibrosis are variable and imaging findings may lead to a broad list of differential diagnoses. We present two patients with idiopathic pelvic fibrosis and discuss radiological findings and differential diagnoses of this rare disease. (orig.)

  4. Proton MR spectroscopy in idiopathic spasmodic torticollis

    Energy Technology Data Exchange (ETDEWEB)

    Federico, F.; Lucivero, V.; Simone, I.L.; Defazio, G.; De Salvia, R.; Mezzapesa, D.M.; Petruzzellis, M.; Tortorella, C.; Livrea, P. [Dept. of Neurology and Psychiatry, Bari (Italy)

    2001-07-01

    Single-voxel proton magnetic resonance spectroscopy ({sup 1}H-MRS), localised to the basal ganglia, was used to determine changes in metabolite levels in idiopathic spasmodic torticollis (IST). We examined nine patients and 13 healthy subjects. The mean values ({+-} SD) of peak area ratios were: IST: N-acetyl-aspartate (NAA)/choline-containing compounds (Cho) 1.79 {+-} 0.39, NAA/creatine and phosphocreatine compounds (Cr) 1.61 {+-} 0.38, Cho/Cr 0.91 {+-} 0.19; controls: NAA/Cho 2.07 {+-} 0.35, NAA/Cr 1.82 {+-} 0.31, Cho/Cr 0.89 {+-} 0.12. Statistical analysis showed that NAA/Cho and NAA/Cr were significantly lower in patients than in controls (P = 0.0304 and 0.0431, respectively). These results indicate a reduction in NAA, and suggest striatal involvement in the pathogenesis IST. (orig.)

  5. Managing comorbidities in idiopathic pulmonary fibrosis

    Science.gov (United States)

    Fulton, Blair G; Ryerson, Christopher J

    2015-01-01

    Major risk factors for idiopathic pulmonary fibrosis (IPF) include older age and a history of smoking, which predispose to several pulmonary and extra-pulmonary diseases. IPF can be associated with additional comorbidities through other mechanisms as either a cause or a consequence of these diseases. We review the literature regarding the management of common pulmonary and extra-pulmonary comorbidities, including chronic obstructive pulmonary disease, lung cancer, pulmonary hypertension, venous thromboembolism, sleep-disordered breathing, gastroesophageal reflux disease, coronary artery disease, depression and anxiety, and deconditioning. Recent studies have provided some guidance on the management of these diseases in IPF; however, most treatment recommendations are extrapolated from studies of non-IPF patients. Additional studies are required to more accurately determine the clinical features of these comorbidities in patients with IPF and to evaluate conventional treatments and management strategies that are beneficial in non-IPF populations. PMID:26451121

  6. Lung Fibroblasts, Aging, and Idiopathic Pulmonary Fibrosis.

    Science.gov (United States)

    Pardo, Annie; Selman, Moisés

    2016-12-01

    Idiopathic pulmonary fibrosis (IPF) is an aging-associated, progressive, and irreversible lung disease of unknown etiology, elusive pathogenesis, and very limited therapeutic options. The hallmarks of IPF are aberrant activation of alveolar epithelial cells and accumulation of fibroblasts and myofibroblasts along with excessive production of extracellular matrix. The linkage of aging with this disorder is uncertain, but a number of changes associated with aging, including telomere attrition, cell senescence, and mitochondrial dysfunction, have been revealed in IPF lungs. Also, aging seems to confer a profibrotic phenotype upon fibroblasts and to increase the severity of the fibrogenic response in non-IPF fibrotic lung disorders. Better knowledge of the pathophysiological mechanisms linking aging to IPF will advance understanding of its pathogenesis and may provide new therapeutic windows to treatment of this devastating disease.

  7. Molecular biomarkers in idiopathic pulmonary fibrosis

    Science.gov (United States)

    Ley, Brett; Brown, Kevin K.

    2014-01-01

    Molecular biomarkers are highly desired in idiopathic pulmonary fibrosis (IPF), where they hold the potential to elucidate underlying disease mechanisms, accelerated drug development, and advance clinical management. Currently, there are no molecular biomarkers in widespread clinical use for IPF, and the search for potential markers remains in its infancy. Proposed core mechanisms in the pathogenesis of IPF for which candidate markers have been offered include alveolar epithelial cell dysfunction, immune dysregulation, and fibrogenesis. Useful markers reflect important pathological pathways, are practically and accurately measured, have undergone extensive validation, and are an improvement upon the current approach for their intended use. The successful development of useful molecular biomarkers is a central challenge for the future of translational research in IPF and will require collaborative efforts among those parties invested in advancing the care of patients with IPF. PMID:25260757

  8. Neocortical gamma oscillations in idiopathic generalized epilepsy

    DEFF Research Database (Denmark)

    Benedek, Krisztina; Berenyi, Antal; Gombkoto, Peter

    2016-01-01

    Objective: Absence seizures in patients with idiopathic generalized epilepsy (IGE) may in part be explained by a decrease in phasic GABAA (type-A c-aminobutyric acid) receptor function, but the mechanisms are only partly understood. Here we studied the relation between ictal and interictal spike......-wave discharges (SWDs) and electroencephalography (EEG) gamma oscillatory activity (30-60 Hz) in patients with IGE. Methods: EEG recordings were obtained of 14 children with IGE (mean age, 8.5 +/- 5 years) and 14 age-and sex-matched controls. Time-frequency analysis of each seizure and seizure-free control epochs...... was performed and cross-coherences of neocortical gamma oscillations were calculated to describe interictal and ictal characteristics of generalized seizures. Results: SWDs were characterized with an abrupt increase of oscillatory activity of 34 and 13-60 Hz, peaking at 3-4 and 30-60 Hz, and with a simultaneous...

  9. Idiopathic interstitial pneumonias: radiologic-pathologic correlation

    International Nuclear Information System (INIS)

    Yoon, Young Cheol; Suh, Gee Young; Han, Joung Ho; Lee, Kyung Soo

    2002-01-01

    Idiopathic interstitial pneumonias are at present classified as one of four types: usual, nonspecific, acute, or desquamative. The acute form has the worst prognosis, followed by the usual and the nonspecific form; it is in desquamative cases that prognosis is best. At high-resolution CT, usual interstitial pneumonia, the most frequent type, manifests as patchy subpleural areas of ground-glass attenuation, irregular linear opacity, and honeycombing, which the nonspecific type, the second most frequent, appears as subpleural patchy areas of ground-glass attenuation with associated areas of irregular linear opacity. Acute interstitial pneumonia demonstrates extensive bilateral airspace consolidation and patchy or diffuse bilateral areas of ground-glass attenuation in middle and lower lung zones

  10. The lived experience with idiopathic pulmonary fibrosis

    DEFF Research Database (Denmark)

    Overgaard, Dorthe; Kaldan, Gudrun; Marsaa, Kristoffer

    2016-01-01

    caregivers. Both patients and family caregivers face an altered life as the disease progresses. The aim of our study was to increase knowledge of life with IPF for patients and family caregivers.This study had a qualitative descriptive design using in-depth dyadic interviews with IPF patients (n=25......) and family caregivers (n=24). We used the five-step analysis from the framework method and analysed the data on three levels: the patient, the family caregivers and couple level.The following six themes emerged as the main results: information and disclosure, reactional dyssynchrony, perpetual vigilance......The disease course in idiopathic pulmonary fibrosis (IPF) is variable, but patients experience a progressive decline in lung function and increased symptom burden leading to death. Little is known about the patients' experience and their needs during the disease course or about the burden on family...

  11. Idiopathic intracranial hypertension, hormones, and 11β-hydroxysteroid dehydrogenases

    DEFF Research Database (Denmark)

    Markey, Keira A; Uldall, Maria; Botfield, Hannah

    2016-01-01

    Idiopathic intracranial hypertension (IIH) results in raised intracranial pressure (ICP) leading to papilledema, visual dysfunction, and headaches. Obese females of reproductive age are predominantly affected, but the underlying pathological mechanisms behind IIH remain unknown. This review provi...

  12. Diffuse idiopathic skeletal hyperostosis: case report and literature ...

    African Journals Online (AJOL)

    Diffuse idiopathic skeletal hyperostosis: case report and literature review. ... NSAIDs, local applications and physiotherapy, might also prove to be useful in patients with ... He had no history of peripheral joint involvement, fevers, cough, bowel ...

  13. Clinical characteristics and outcomes of familial and idiopathic ...

    African Journals Online (AJOL)

    Clinical characteristics and outcomes of familial and idiopathic dilated cardiomyopathy in Cape Town: A comparative study of 120 cases followed up over 14 years. NBA Ntusi, M Badri, F Gumedze, A Wonkam, BM Mayosi ...

  14. A Case Study on Idiopathic Orbital Pseudotumor: Surgery and ...

    African Journals Online (AJOL)

    one year revealed a gradual improvement in the vision of all the patients. The VA of the right eye for all the patients ... Hunt syndrome, pituitary histiocytosis, idiopathic meningitis ... computer tomography (CT) and magnetic resonance imaging ...

  15. Idiopathic Pulmonary Calcification and Ossification in an Elderly ...

    African Journals Online (AJOL)

    Histology of tissue from autopsy showed intraparenchymal pulmonary calcification and ossification with marrow elements. Idiopathic pulmonary calcification and ossification is rare. At autopsy, she was also found to have had bilateral subarachnoid haemorrhage (SAH), a diagnosis missed during clinical evaluation.

  16. Idiopathic Hypoparathyroidism Mimicking Ankylosing Spondylitis: A Case Report

    Directory of Open Access Journals (Sweden)

    Ozge Illeez Memetoglu

    2016-01-01

    Full Text Available Idiopathic hypoparathyroidism, inadequate secretion of parathyroid hormone of unknown etiology, may mimic ankylosing spondylitis both clinically and radiologically. Spinal complaints may be the first sign of any endocrinological disorder.

  17. Computed tomography in the assessment of idiopathic spontaneous pneumothorax

    International Nuclear Information System (INIS)

    Kim, Sang Jin; Lee, Doo Yun; Kim, Hyung Jung

    1991-01-01

    It is well known that idiopathic spontaneous pneumothorax is caused by rupture of the subpleural bleb and presents difficulty in exact detection and localization of the bleb with plain chest X - ray alone. The authors performed chest CT scans for accurate diagnosis of bleb that would act as a guide for optimal management of idiopathic spontaneous pneumothorax patients in order to prevent recurrent pneumothorax. We could detect blebs in 93 % (26/28) of the patients with idiopathic spontaneous pneumothorax, and 68 % (19/ 28) of the patient had bilateral blebs. Sensitivity was 0.63, and false negative was 37% (37/100) of the blebs, and 51% (19/37) of these 37 false negative cases were ruptured blebs. Only 7 % (2/28) of the patients had a single bleb. The authors concluded that CT is a useful method of study for optimal management of idiopathic spontaneous pneumothorax patients

  18. Patellofemoral morphometry in patients with idiopathic patellofemoral pain syndrome

    Energy Technology Data Exchange (ETDEWEB)

    Mar Carrion Martin, Maria del [Department of Rehabilitation, Hospital of Traumatology (Ciudad Sanitaria Virgen de las Nieves), Carretera de Jaen SN, 18014 Granada (Spain); Ruiz Santiago, Fernando, E-mail: ferruizsan@terra.e [Department of Radiology, Hospital of Traumatology (Ciudad Sanitaria Virgen de las Nieves), Carretera de Jaen SN, 18014 Granada (Spain); Pozuelo Calvo, Rocio [Department of Rehabilitation, Hospital of Traumatology (Ciudad Sanitaria Virgen de las Nieves), Carretera de Jaen SN, 18014 Granada (Spain); Guzman Alvarez, Luis [Department of Radiology, Hospital of Traumatology (Ciudad Sanitaria Virgen de las Nieves), Carretera de Jaen SN, 18014 Granada (Spain)

    2010-07-15

    Purpose: To compare clinical and computed tomography (CT) measures in extension, 20{sup o} and 30{sup o} of flexion of symptomatic knees of patient with idiopathic patellofemoral pain syndrome with the contra lateral asymptomatic knee. Materials and methods: Knees of 52 consecutive patients with idiopathic patellofemoral pain were studied with CT. In 28 patients this condition was unilateral and asymptomatic knee was used as control; 76 knees were symptomatic. Results: In patients with idiopathic patellofemoral pain we found a greater Q angle and internal condylar facet width in symptomatic knees with regard to asymptomatic knees. Conclusion: Greater Q angle and medial condylar facet can lead to overpressure on the medial knee compartment during maneuvers that increase contact between patella and medial condylar facet, such as knee flexion and squatting, contributing to development of idiopathic patellofemoral pain.

  19. Guidelines for the medical treatment of idiopathic pulmonary fibrosis.

    Science.gov (United States)

    Xaubet, Antoni; Molina-Molina, María; Acosta, Orlando; Bollo, Elena; Castillo, Diego; Fernández-Fabrellas, Estrella; Rodríguez-Portal, José Antonio; Valenzuela, Claudia; Ancochea, Julio

    2017-05-01

    Idiopathic pulmonary fibrosis is defined as chronic fibrosing interstitial pneumonia limited to the lung, with poor prognosis. The incidence has been rising in recent years probably due to improved diagnostic methods and increased life expectancy. In 2013, the SEPAR guidelines for the diagnosis and treatment for idiopathic pulmonary fibrosis were published. Since then, clinical trials and meta-analyses have shown strong scientific evidence for the use of pirfenidone and nintedanib in the treatment of idiopathic pulmonary fibrosis. In 2015, the international consensus of 2011 was updated and new therapeutic recommendations were established, prompting us to update our recommendation for the medical treatment of idiopathic pulmonary fibrosis accordingly. Diagnostic aspects and non-pharmacological treatment will not be discussed as no relevant developments have emerged since the 2013 guidelines. Copyright © 2017 SEPAR. Publicado por Elsevier España, S.L.U. All rights reserved.

  20. Structural white matter abnormalities in patients with idiopathic dystonia

    NARCIS (Netherlands)

    Bonilha, Leonardo; de Vries, Paulien M.; Vincent, Diana J.; Rorden, Chris; Morgan, Paul S.; Hurd, Mark W.; Besenski, Nada; Bergmann, Kenneth J.; Hinson, Vanessa K.

    2007-01-01

    We investigated whether structural white matter abnormalities, in the form of disruption of axonal coherence and integrity as measured with diffusion tensor imaging (DTI), constitute an underlying pathological mechanism of idiopathic dystonia (ID), independent of genotype status. We studied seven

  1. The pattern of juvenile idiopathic arthritis; a retrospective Egyptian ...

    African Journals Online (AJOL)

    Ehab

    subtype specially at rural areas which differed from Western and Gulf countries pattern. ... version 18) and the following statistical measures ..... the results of Abdwani et al27 at 2015 (an Omani ... Ravelli A, Martini A. Juvenile idiopathic arthritis.

  2. Patellofemoral morphometry in patients with idiopathic patellofemoral pain syndrome

    International Nuclear Information System (INIS)

    Mar Carrion Martin, Maria del; Ruiz Santiago, Fernando; Pozuelo Calvo, Rocio; Guzman Alvarez, Luis

    2010-01-01

    Purpose: To compare clinical and computed tomography (CT) measures in extension, 20 o and 30 o of flexion of symptomatic knees of patient with idiopathic patellofemoral pain syndrome with the contra lateral asymptomatic knee. Materials and methods: Knees of 52 consecutive patients with idiopathic patellofemoral pain were studied with CT. In 28 patients this condition was unilateral and asymptomatic knee was used as control; 76 knees were symptomatic. Results: In patients with idiopathic patellofemoral pain we found a greater Q angle and internal condylar facet width in symptomatic knees with regard to asymptomatic knees. Conclusion: Greater Q angle and medial condylar facet can lead to overpressure on the medial knee compartment during maneuvers that increase contact between patella and medial condylar facet, such as knee flexion and squatting, contributing to development of idiopathic patellofemoral pain.

  3. Evaluation of classification systems for nonspecific idiopathic orbital inflammation

    NARCIS (Netherlands)

    Bijlsma, Ward R.; van 't Hullenaar, Fleur C.; Mourits, Maarten P.; Kalmann, Rachel

    2012-01-01

    To systematically analyze existing classification systems for idiopathic orbital inflammation (IOI) and propose and test a new best practice classification system. A systematic literature search was conducted to find all studies that described and applied a classification system for IOI.

  4. Idiopathic Syringomyelia in a Military Helicopter Pilot.

    Science.gov (United States)

    Schiemer, Anthony

    2017-10-01

    A syrinx is a fluid-filled cavity within the spinal cord. They can lead to a variety of symptoms, including limb weakness and back pain. Incidental finding of syringomyelia provides a challenge for clinicians due to the wide variety of possible symptoms. In military aviation, neurological findings in pilots can result in extensive investigation that can lead to potentially invasive management. Conversely, the potential for chronic progression of a spinal syrinx and subsequent neurological deterioration makes early identification critical. Ultimately, the discovery of a lesion may have implications for flying status and operational capability. A 25-yr-old man working as a navy Seahawk helicopter pilot presented with episodes of right arm paraesthesia and pain between the scapulae. On at least one occasion, these symptoms woke him at night. Upon magnetic resonance imaging, dilatation of the central canal in a syrinx-like pattern in the lower cervical region was noted. Neurology review suggested the finding was persistent and unlikely to be responsible for his symptoms. No surgical input was recommended. His symptoms were attributed to mild cervical spondylosis, which resolved with ongoing physiotherapy, and he was returned to flying status. This case highlights several issues involved with the incidental finding of a syringomyelia. Surgical intervention has been known to worsen symptoms. Conversely, studies have identified minimal radiological progression in cases of idiopathic syringomyelia, with fewer individuals displaying neurological deterioration. For aircrew, potentially unnecessary neurosurgical intervention poses risks to a flying career and overall operational capability.Schiemer A. Idiopathic syringomyelia in a military helicopter pilot. Aerosp Med Hum Perform. 2017; 88(10):962-965.

  5. Surgical treatment analysis of idiopathic esophageal achalasia.

    Science.gov (United States)

    Aquino, José Luis Braga de; Said, Marcelo Manzano; Pereira, Douglas Rizzanti; Amaral, Paula Casals do; Lima, Juliana Carolina Alves; Leandro-Merhi, Vânia Aparecida

    2015-01-01

    Idiopathic esophageal achalasia is an inflammatory disease of unknown origin, characterized by aperistalsis of the esophageal body and failure of the lower esophageal sphincter in response to swallowing, with consequent dysphagia. To demonstrate the results of surgical therapy in these patients, evaluating the occurred local and systemic complications. Were studied retrospectively 32 patients, 22 of whom presented non-advanced stage of the disease (Stage I/II) and 10 with advanced disease (Stage III/IV). All of them had the clinical conditions to be submitted to surgery. The diagnoses were done by clinical, endoscopic, cardiological, radiological and esophageal manometry analysis. Pre-surgical evaluation was done with a questionnaire based on the most predisposing factors in the development of the disease and the surgical indication was based on the stage of the disease. The patients with non-advanced stages were submitted to cardiomyotomy with fundoplication, wherein in the post-surgical early assessment, only one (4,4%) presented pulmonary infection, but had a good outcome. In patients with advanced disease, seven were submitted to esophageal mucosectomy preserving the muscular layer, wherein one patient (14,2%) presented dehiscence of gastric cervical esophagus anastomosis as well as pulmonary infection; all of these complications were resolved with proper specific treatment; the other three patients with advanced stage were submitted to transmediastinal esophagectomy; two of them presented hydropneumothorax with good evolution, and one of them also presented fistula of the cervical esophagogastric anastomosis, but with spontaneous healing after conservative treatment and nutritional support. The two patients with fistula of the cervical anastomosis progressed to stenosis, with good results after endoscopic dilations. In the medium and long term assessment done in 23 patients, all of them reported improvement in life quality, with return to swallowing. The

  6. Idiopathic Bilateral External Jugular Vein Thrombosis.

    Science.gov (United States)

    Hindi, Zakaria; Fadhel, Ehab

    2015-08-20

    Vein thrombosis is mainly determined by 3 factors, which constitute a triad called Virchow's triad: hypercoagulability, stasis, and endothelial injury. Venous thrombosis commonly occurs in the lower extremities since most of the blood resides there and flows against gravity. The veins of the lower extremities are dependent on intact valves and fully functional leg muscles. However, in case of valvular incompetency or muscular weakness, thrombosis and blood stasis will occur as a result. In contrast, the veins of the neck, specially the jugulars, have distensible walls which allow flexibility during respiration. In addition, the blood directly flows downward towards the heart. Nevertheless, many case reports mentioned the thrombosis of internal jugular veins and external jugular veins with identified risk factors. Jugular vein thrombosis has previously been associated in the literature with a variety of medical conditions, including malignancy. This report is of a case of idiopathic bilateral external jugular vein thrombosis in a 21 year-old male construction worker of Southeast Asian origin with no previous medical history who presented with bilateral facial puffiness of gradual onset over 1 month. Doppler ultrasound and computed tomography were used in the diagnosis. Further work-up showed no evidence of infection or neoplasia. The patient was eventually discharged on warfarin. The patient was assessed after 6 months and his symptoms had resolved completely. Bilateral idiopathic external jugular veins thrombosis is extremely rare and can be an indicator of early malignancy or hidden infection. While previous reports in the literature have associated jugular vein thrombosis with malignancy, the present case shows that external jugular vein thrombosis can also be found in persons without malignancy.

  7. Anticoagulation period in idiopathic venous thromboembolism

    International Nuclear Information System (INIS)

    Farraj, Rami S.

    2004-01-01

    The period of anticoagulation of a first episode of idiopathic venous thromboembolism has been 6 months. It is unclear if such patients would benefit from longer treatment, as there appears to be an increased risk of recurrence after anticoagulation is stopped. In a randomized prospective study of 64 patients admitted to King Hussein Medical city, Amman, Jordan, who developed a first episode of venous thromboembolism, 32 patients were given warfarin for 24-months, while 32 patients stopped anticoagulation after completion of 6-months of therapy. Our goal was to determine the effects of extended anticoagulation on rates of recurrence of symptomatic venous thromboembolism and bleeding. The patients were followed for 12-months after stopping anticoagulation. After 24-months, 7 of the 32 patients (21%) who had standard anticoagulation for 6-months had a recurrent episode of thromboembolism compared to one of the 32 patients who received anticoagulation for 24 months (3%). Extended warfarin therapy for 24-months has resulted in an absolute risk reduction of 0.1% (p<0.05). This translates into 8 patients having to be treated for 24-months to avoid one recurrence without increasing the risk of major bleeding. Two patients in each group (6%) had major nonfatal bleeding, all 4 bleeding episodes occurring within the first 3-months of anticoagulation. After 36-months of follow up, the recurrence rate of extended warfarin therapy was only 3 patients (9%), which is a 43% relative reduction in recurrence of thromboembolism compared to standard therapy for 6-months. Patients with first episodes of idiopathic venous thromboembolism have an increased risk of recurrent venous thromboembolism and should be treated with oral anticoagulants for longer than 6-months, probably 24-months. (author)

  8. Microcatheter Embolization of Intractable Idiopathic Epistaxis

    International Nuclear Information System (INIS)

    Leppaenen, Martti; Seppaenen, Seppo; Laranne, Jussi; Kuoppala, Katriina

    1999-01-01

    Purpose: To assess the efficacy and safety of microcatheter embolization in the treatment of intractable idiopathic epistaxis. Methods: Thirty-seven patients underwent microcatheter embolization in 1991-1998. We evaluated retrospectively the technical and clinical outcome, the number of complications, the duration of embolization in each case, and the number of blood transfusions needed. All embolizations were done with biplane digital subtraction angiography (DSA) equipment. The procedure was carried out under local anesthesia using transfemoral catheterization, except in one case where the translumbar route was used. Tracker 18 or 10 microcatheters were advanced as far as possible to the distal branches of the sphenopalatine artery. Polyvinyl alcohol (PVA) particles were used for embolization in most cases, while platinum coils or a combination of these two materials were occasionally used. The primary outcome was always assessed immediately by angiography. Follow-up data were obtained from patient records, by interviewing patients on the telephone or by postal questionnaires when necessary. The mean follow-up time was 21 months. Results: The embolization was technically successful in all 37 cases. A curative outcome was achieved in 33 cases (89%). The mean duration of the procedure was 110 min. Four patients (8%) had mild transient complications, but no severe or persistent complications were encountered. Twenty-three patients needed a blood transfusion. Slight rebleeding occurred in three patients during the follow-up; all responded to conservative treatment. One patient suffered two episodes of rebleeding within 2 months after primary embolization. Re-embolizations successfully stopped the bleeding. Conclusion: Embolization is the primary invasive modality for treating intractable idiopathic epistaxis. It proved both safe and effective over a relatively long follow-up

  9. Intravoxel incoherent motion magnetic resonance imaging of the knee joint in children with juvenile idiopathic arthritis

    Energy Technology Data Exchange (ETDEWEB)

    Hilbert, Fabian; Sauer, Alexander; Koestler, Herbert [University Hospital Wuerzburg, Department of Diagnostic and Interventional Radiology, Wuerzburg (Germany); Holl-Wieden, Annette [University Hospital Wuerzburg, Department of Paediatrics, Wuerzburg (Germany); Neubauer, Henning [University Hospital Wuerzburg, Department of Diagnostic and Interventional Radiology, Wuerzburg (Germany); University Hospital Ulm, Department of Diagnostic and Interventional Radiology, Ulm (Germany)

    2017-05-15

    MRI of synovitis relies on use of a gadolinium-based contrast agent. Diffusion-weighted MRI (DWI) visualises thickened synovium but is of limited use in the presence of joint effusion. To investigate the feasibility and diagnostic accuracy of diffusion-weighted MRI with intravoxel incoherent motion (IVIM) for diagnosing synovitis in the knee joint of children with juvenile idiopathic arthritis. Twelve consecutive children with confirmed or suspected juvenile idiopathic arthritis (10 girls, median age 11 years) underwent MRI with contrast-enhanced T1-weighted imaging and DWI at 1.5 T. Read-out segmented multi-shot DWI was acquired at b values of 0 s/mm{sup 2}, 200 s/mm{sup 2}, 400 s/mm{sup 2} and 800 s/mm{sup 2}. We calculated the IVIM parameters perfusion fraction (f) and tissue diffusion coefficient (D). Diffusion-weighted images at b=800 s/mm{sup 2}, f parameter maps and post-contrast T1-weighted images were retrospectively assessed by two independent readers for synovitis using the Juvenile Arthritis MRI Scoring system. Seven (58%) children showed synovial hypertrophy on contrast-enhanced imaging. Diagnostic ratings for synovitis on DWI and on f maps were fully consistent with contrast-enhanced imaging, the diagnostic reference. Two children had equivocal low-confidence assessments on DWI. Median f was 6.7±2.0% for synovitis, 2.1±1.2% for effusion, 5.0±1.0% for muscle and 10.6±5.7% for popliteal lymph nodes. Diagnostic confidence was higher based on f maps in three (25%) children and lower in one child (8%), as compared to DWI. DWI with IVIM reliably visualises synovitis of the knee joint. Perfusion fraction maps differentiate thickened synovium from joint effusion and hence increase diagnostic confidence. (orig.)

  10. Cost-appropriateness of whole body vs limited bone imaging for suspected focal sports injuries

    International Nuclear Information System (INIS)

    Nagle, C.E.

    1986-01-01

    Bone imaging has been recognized as a useful diagnostic tool in detecting the presence of focal musculoskeletal injury when radiographs are normal. A retrospective review of bone images in a small number of amateur athletes indicates that secondary injuries were commonly detected at sites different from the site of musculoskeletal pain being evaluated for injury. While a larger study will be necessary to confirm the data, this review suggests that it is medically justified and cost-appropriate to perform imaging of the entire skeleton as opposed to imaging limited to the anatomic site of pain and suspected injury

  11. [Clinical application of proton magnetic resonance spectroscopy in children with idiopathic epilepsy].

    Science.gov (United States)

    Shao, Xiao-Li; Zhou, Zhong-Shu; Hong, Wen

    2010-06-01

    This study examined the biochemical metabolism by proton magnetic resonance spectroscopy ('H-MRS) in order to explore the value of 'H-MRS in idiopathic epilepsy in children. Thirty-three children with idiopathic epilepsy (14 cases with history of febrile seizures and 19 cases without) and six normal controls experienced MRI of the skull and brain and single-voxel 'H-MRS examinations of the hippocampi-temporal lobe. The signal intensities of N-acetylaspartate (NAA), eatine+phosphocreatine (Cr), choline-containing compounds (Cho) and lactate (Lac) and the ratios of NAA/ (Cho+Cr) and Lac/Cr were compared between the patients and normal controls. MRI examination showed that only one child with epilepsy had myelin dysplasia. 'H-MRS examination showed that the ratio of NAA/ (Cho+Cr) in the epilepsy group was lower than that in the control group (0.64+/-0.07 vs 0.73+/-0.05; Pepilepsy and the control groups. 'H-MRS may provide early information on brain injury sensitively and non-invasively in children with epilepsy. It may be used for diagnosis and prognosis evaluation of epilepsy.

  12. Repressive coping and alexithymia in idiopathic environmental intolerance

    DEFF Research Database (Denmark)

    Skovbjerg, Sine; Zachariae, Robert; Rasmussen, Alice

    2010-01-01

    To examine if the non-expression of negative emotions (i.e., repressive coping) and differences in the ability to process and regulate emotions (i.e., alexithymia) is associated with idiopathic environmental intolerance (IEI).......To examine if the non-expression of negative emotions (i.e., repressive coping) and differences in the ability to process and regulate emotions (i.e., alexithymia) is associated with idiopathic environmental intolerance (IEI)....

  13. A CASE OF IDIOPATHIC PULMONARY ARTERIAL HYPERTENSION IN MALE

    OpenAIRE

    Poongavanam Paranthaman; Ramani Bala Subra Manian; Thenrajan Balaji; Jayakrishnan Jayakumar; Govindaraj Ranjani

    2016-01-01

    Primary Pulmonary Hypertension is a rare disease occurring in 1-2 per million population. It is 2-4 times more common in female. Idiopathic or primary pulmonary hypertension is defined as a disorder with no identifiable cause in which resting mean pulmonary artery pressure in adults is above 25 mmHg and 30 mmHg with exercise. Idiopathic or primary pulmonary hypertension is diagnosed after ruling out all the possible secondary causes of pulmonary hypertension. We are presenting a ...

  14. MUSCLE DISORDERS IN ADOLESCENT IDIOPATHIC SCOLIOSIS: LITERATURE REVIEW

    OpenAIRE

    Débora Pinheiro Lédio Alves; Barbara de Araújo

    2016-01-01

    ABSTRACT Scoliosis is characterized by three-dimensional changes of the spine and is estimated to be present in 4% of the population worldwide. The most common form is the adolescent idiopathic. The purpose of this study is to identify the major muscle abnormalities found in patients with adolescent idiopathic scoliosis through a literature review. We conducted an electronic search of the national databases PubMed, Lilacs, PEDro, and EMBASE using the keywords "scoliosis", "biomechanics", "exe...

  15. CELSR2 is a candidate susceptibility gene in idiopathic scoliosis

    DEFF Research Database (Denmark)

    Einarsdottir, Elisabet; Grauers, Anna; Wang, Jingwen

    2017-01-01

    and the US. No association was found to variants in CELSR1 or CELSR3. Our findings suggest a rare variant in CELSR2 as causative for idiopathic scoliosis in a family with dominant segregation and further highlight common variation in CELSR2 in general susceptibility to idiopathic scoliosis in the Swedish......-Danish population. Both variants are located in the highly conserved GAIN protein domain, which is necessary for the auto-proteolysis of CELSR2, suggesting its functional importance....

  16. Idiopathic Non-traumatic Facial Nerve Palsy (Bell’s Palsy) in Neonates; An Atypical Age and Management Dilemma

    Science.gov (United States)

    Khair, Abdulhafeez M.; Ibrahim, Khalid

    2018-01-01

    Idiopathic (Bell’s) palsy is the commonest cause of unilateral facial paralysis in children. Although being idiopathic by definition, possible infectious, inflammatory, and ischemic triggers have been suggested. Bell’s palsy is thought to be responsible for up to three-fourths of cases of acute unilateral facial paralysis worldwide. The diagnosis has to be reached after other causes of acute peripheral palsy have been excluded. However, it is rarely described in neonates and young infants. Steroids may have some role in treatment, but antiviral therapies have doubtful evidence of benefit. Prognosis is good, though residual dysfunction is occasionally encountered. We report the case of a two-week-old neonate with no prior illnesses who presented with acute left facial palsy. Clinical findings and normal brain imaging were consistent with the diagnosis of Bell’s palsy. The patient had a good response to oral steroids. PMID:29468002

  17. Idiopathic Non-traumatic Facial Nerve Palsy (Bell’s Palsy in Neonates; An Atypical Age and Management Dilemma

    Directory of Open Access Journals (Sweden)

    Abdulhafeez M. Khair

    2018-01-01

    Full Text Available Idiopathic (Bell’s palsy is the commonest cause of unilateral facial paralysis in children. Although being idiopathic by definition, possible infectious, inflammatory, and ischemic triggers have been suggested. Bell’s palsy is thought to be responsible for up to three-fourths of cases of acute unilateral facial paralysis worldwide. The diagnosis has to be reached after other causes of acute peripheral palsy have been excluded. However, it is rarely described in neonates and young infants. Steroids may have some role in treatment, but antiviral therapies have doubtful evidence of benefit. Prognosis is good, though residual dysfunction is occasionally encountered. We report the case of a two-week-old neonate with no prior illnesses who presented with acute left facial palsy. Clinical findings and normal brain imaging were consistent with the diagnosis of Bell’s palsy. The patient had a good response to oral steroids.

  18. Sleepiness in Idiopathic REM Sleep Behavior Disorder and Parkinson Disease.

    Science.gov (United States)

    Arnulf, Isabelle; Neutel, Dulce; Herlin, Bastien; Golmard, Jean-Louis; Leu-Semenescu, Smaranda; Cochen de Cock, Valérie; Vidailhet, Marie

    2015-10-01

    To determine whether patients with idiopathic and symptomatic RBD were sleepier than controls, and if sleepiness in idiopathic RBD predicted earlier conversion to Parkinson disease. The Epworth Sleepiness Scale (ESS) and its determinants were compared at the time of a video-polysomnography for an RBD diagnosis in patients with idiopathic RBD, in patients with Parkinson disease, and in controls. Whether sleepiness at time of RBD diagnosis predicted an earlier conversion to neurodegenerative diseases was retrospectively analyzed in the followed-up patients. The 75 patients with idiopathic RBD were sleepier (ESS: 7.8 ± 4.6) at the time of RBD diagnosis than 74 age- and sex-matched controls (ESS: 5.0 ± 3.6, P sleep measures. Among the 69 patients with idiopathic RBD who were followed up for a median 3 years (1-15 years), 16 (23.2%) developed parkinsonism (n = 6), dementia (n = 6), dementia plus parkinsonism (n = 2), and multiple system atrophy (n = 2). An ESS greater than 8 at time of RBD diagnosis predicted a shorter time to phenoconversion to parkinsonism and dementia, from RBD onset, and from RBD diagnosis (when adjusted for age and time between RBD onset and diagnosis). Sleepiness is associated with idiopathic REM sleep behavior disorder and predicts more rapid conversion to parkinsonism and dementia, suggesting it is an early marker of neuronal loss in brainstem arousal systems. © 2015 Associated Professional Sleep Societies, LLC.

  19. Obesity is a significant susceptibility factor for idiopathic AA amyloidosis.

    Science.gov (United States)

    Blank, Norbert; Hegenbart, Ute; Dietrich, Sascha; Brune, Maik; Beimler, Jörg; Röcken, Christoph; Müller-Tidow, Carsten; Lorenz, Hanns-Martin; Schönland, Stefan O

    2018-03-01

    To investigate obesity as susceptibility factor in patients with idiopathic AA amyloidosis. Clinical, biochemical and genetic data were obtained from 146 patients with AA amyloidosis. Control groups comprised 40 patients with long-standing inflammatory diseases without AA amyloidosis and 56 controls without any inflammatory disease. Patients with AA amyloidosis had either familial Mediterranean fever (FMF) or long-standing rheumatic diseases as underlying inflammatory disease (n = 111, median age 46 years). However, in a significant proportion of patients with AA amyloidosis no primary disease was identified (idiopathic AA; n = 37, median age 60 years). Patients with idiopathic AA amyloidosis were more obese and older than patients with AA amyloidosis secondary to FMF or rheumatic diseases. Serum leptin levels correlated with the body mass index (BMI) in all types of AA amyloidosis. Elevated leptin levels of more than 30 µg/l were detected in 18% of FMF/rheumatic + AA amyloidosis and in 40% of patients with idiopathic AA amyloidosis (p = .018). Finally, the SAA1 polymorphism was confirmed as a susceptibility factor for AA amyloidosis irrespective of the type of the disease. Obesity, age and the SAA1 polymorphism are susceptibility factors for idiopathic AA amyloidosis. Recent advances in treatment of FMF and rheumatic disorders will decrease the incidence of AA amyloidosis due to these diseases. Idiopathic AA, however, might be an emerging problem in the ageing and increasingly obese population.

  20. Idiopathic renal hematuria in a dog; the usefulness of a method of partial occlusion of the renal artery.

    Science.gov (United States)

    Mishina, M; Watanabe, T; Yugeta, N; Maeda, H; Fujii, K; Wakao, Y; Takahashi, M; Yamamura, H

    1997-04-01

    Exploratory laparotomy was performed on a dog suspected of having idiopathic renal hematuria. Two catheters were inserted into the bilateral ureters, and hematuria from the left kidney was confirmed. The blood flow was occluded in the ventral and dorsal rami of the left renal artery in order to localize the site of hemorrhage. As hematuria disappeared when the dorsal ramus was occluded, the site of renal hematuria was localized to the area dominated by the dorsal ramus of the renal artery. As a result of ligating the dorsal ramus of the left renal artery in this dog, renal hematuria subsided, and the dog has shown a favorable course, to date, one year after surgery.

  1. CT analysis of 40 patients with chronic type of idiopathic interstitial pneumonia from an air-polluted area

    International Nuclear Information System (INIS)

    Katagiri, Shiro; Ohshima, Kazunori; Fukushima, Kazuo; Kawabata, Yoshinori.

    1995-01-01

    We analyzed CT scans of 40 patients with chronic type of idiopathic interstitial pneumonia (IIP) who had admitted to our hospital in Kawasaki. All patients were diagnosed because of the presence of honeycombing. The characteristic findings were centrilobular emphysema and bulla. In addition to such common features of IIP multiple centrilobular nodular shadows were seen in about 60% of the patients. Considering that all patients had lived for more than 30 years in Kawasaki when air-pollution had been badly high in this area and that the majority of the patients (36 of 50) had been smokers we suspected that multiple nodules on CT might be due to long-term exposure to particular dusts including asbestos and that centrilobular emphysema might be due to smoking. (author)

  2. Disk abnormality coexists with any degree of synovial and osseous abnormality in the temporomandibular joints of children with juvenile idiopathic arthritis

    International Nuclear Information System (INIS)

    Kirkhus, Eva; Smith, Hans-Joergen; Arvidsson, Linda Z.; Larheim, Tore A.; Flatoe, Berit; Hetlevik, Siri O.

    2016-01-01

    MRI manifestation of temporomandibular joint arthritis is frequently reported in children with juvenile idiopathic arthritis. However, little attention has been paid to temporomandibular joint disk abnormalities. To assess combinations of MRI findings in the symptomatic temporomandibular joint in children with juvenile idiopathic arthritis with focus on disk abnormalities. This was a retrospective study of 46 patients with juvenile idiopathic arthritis, mean age 12 years (range: 5-17 years). Mean disease duration was 70 months (standard deviation: 61 months). MR images of 92 temporomandibular joints were scored for thickness of abnormally enhancing synovium (synovitis), joint effusion, bone marrow oedema, abnormal bone shape, bone erosion and disk abnormalities. The 92 temporomandibular joints were categorized as A: No synovitis and normal bone shape (30/92; 33%), B: Synovitis and normal bone shape (14/92: 15%), C: Synovitis and abnormal bone shape (38/92; 41%) and D: No synovitis but abnormal bone shape (10/92; 11%). Thirty-six of the 46 patients (78%) had synovitis and 33/46 (72%) had abnormal bone shape, most frequently in combination (30/46; 65%). Disk abnormalities (flat disk, fragmented disk, adherent disk and displaced disk) were found in 29/46 patients (63%). Disk abnormalities were found in all categories of juvenile idiopathic arthritis involved temporomandibular joints (B: 8/14 [57%]; C: 25/38 [66%] and D: 7/10 [70%]). Disk displacement was found in half of the joints (7/14) in category B. Synovitis was most pronounced in this category. Disk abnormalities were frequent. Disk displacement also occurred in joints with early temporomandibular joint arthritis, i.e., with normal bone shape. Other disk abnormalities were found in joints with bone abnormalities. Attention should be paid to disk abnormalities both in early and long-standing temporomandibular joint arthritis in children with juvenile idiopathic arthritis. (orig.)

  3. Disk abnormality coexists with any degree of synovial and osseous abnormality in the temporomandibular joints of children with juvenile idiopathic arthritis

    Energy Technology Data Exchange (ETDEWEB)

    Kirkhus, Eva; Smith, Hans-Joergen [Oslo University Hospital, Rikshospitalet, Department of Radiology and Nuclear Medicine, Oslo (Norway); University of Oslo, Institute of Clinical Medicine, Oslo (Norway); Arvidsson, Linda Z.; Larheim, Tore A. [University of Oslo, Department of Maxillofacial Radiology, Institute of Clinical Dentistry, Oslo (Norway); Flatoe, Berit; Hetlevik, Siri O. [Oslo University Hospital, Rikshospitalet, Department of Rheumatology, Oslo (Norway); University of Oslo, Institute of Clinical Medicine, Oslo (Norway)

    2016-03-15

    MRI manifestation of temporomandibular joint arthritis is frequently reported in children with juvenile idiopathic arthritis. However, little attention has been paid to temporomandibular joint disk abnormalities. To assess combinations of MRI findings in the symptomatic temporomandibular joint in children with juvenile idiopathic arthritis with focus on disk abnormalities. This was a retrospective study of 46 patients with juvenile idiopathic arthritis, mean age 12 years (range: 5-17 years). Mean disease duration was 70 months (standard deviation: 61 months). MR images of 92 temporomandibular joints were scored for thickness of abnormally enhancing synovium (synovitis), joint effusion, bone marrow oedema, abnormal bone shape, bone erosion and disk abnormalities. The 92 temporomandibular joints were categorized as A: No synovitis and normal bone shape (30/92; 33%), B: Synovitis and normal bone shape (14/92: 15%), C: Synovitis and abnormal bone shape (38/92; 41%) and D: No synovitis but abnormal bone shape (10/92; 11%). Thirty-six of the 46 patients (78%) had synovitis and 33/46 (72%) had abnormal bone shape, most frequently in combination (30/46; 65%). Disk abnormalities (flat disk, fragmented disk, adherent disk and displaced disk) were found in 29/46 patients (63%). Disk abnormalities were found in all categories of juvenile idiopathic arthritis involved temporomandibular joints (B: 8/14 [57%]; C: 25/38 [66%] and D: 7/10 [70%]). Disk displacement was found in half of the joints (7/14) in category B. Synovitis was most pronounced in this category. Disk abnormalities were frequent. Disk displacement also occurred in joints with early temporomandibular joint arthritis, i.e., with normal bone shape. Other disk abnormalities were found in joints with bone abnormalities. Attention should be paid to disk abnormalities both in early and long-standing temporomandibular joint arthritis in children with juvenile idiopathic arthritis. (orig.)

  4. Low density in liver of idiopathic portal hypertension. A computed tomographic observation with possible diagnostic significance

    Energy Technology Data Exchange (ETDEWEB)

    Ishito, Hiroyuki

    1988-01-01

    In order to evaluate the diagnostic value of low density in liver on computed tomography (CT), CT scans of 11 patients with idiopathic portal hypertension (IPH) were compared with those from 22 cirrhotic patients, two patients with scarred liver and 16 normal subjects. Low densities on plain CT scans in patients with IPH were distinctly different from those observed in normal liver. Some of the low densities had irregular shape with unclear margin and were scattered near the liver surface, and others had vessel-like structures with unclear margin and extended as far as near the liver surface. Ten of the 11 patients with IPH had low densities mentioned above, while none of the 22 cirrhotic patients had such low densities. The present results suggest that the presence of low densities in liver on plain CT scan is clinically beneficial in diagnosis of IPH.

  5. Use of sodium chromate Cr51 in diagnosing childhood idiopathic pulmonary hemosiderosis

    International Nuclear Information System (INIS)

    Kurzweil, P.R.; Miller, D.R.; Freeman, J.E.; Reiman, R.E.; Mayer, K.

    1984-01-01

    The diagnosis of idiopathic pulmonary hemosiderosis (IPH) may be elusive. A 6-year-old boy had microcytic hypochromic anemia and a hemolytic component. Hemosiderin-laden macrophages were not found in the gastric aspirate. He had no pulmonary signs or symptoms. Extensive hematologic and roentgenologic investigations failed to reveal the cause of the anemia. Quantitative serial scintigraphic scanning showed significant (35%) pulmonary sequestration of autologous erythrocytes labeled with sodium chromate Cr51. The half-life of the RBCs was moderately decreased (half-life, 15 days; normal, 25 to 35 days). An open-lung biopsy spece Cr51. The half-life of the RBCs was moderately decreased (half-life, 15 days; normal, 25 to 35 days). An open-lung biopsy specimen confirmed the diagnosis of IPH. A diagnosis of IPH should be considered when children have iron deficiency anemia and pulmonary signs or symptoms. Organ sequestration studies may be helpful in equivocal cases

  6. Contributions of positron emission tomography to elucidating the pathogenesis of idiopathic Parkinsonism and dopa responsive dystonia

    International Nuclear Information System (INIS)

    Calne, D.B.; Fuente-Fernandez, R. de la; Kishore, A.

    1996-01-01

    The metabolic mapping of brain activity. using PET, confirms the conventional wisdom of neurophysiology. In studies of pathophysiology, PET has yielded evidence that has generated new hypotheses. Progression of the lesion detectable with fluorodopa, in human subjects exposed to MPTP, raises the possibility of a transient environmental event being a cause of progressive neurodegeneration. Studies with fluorodopa in Idiopathic Parkinsonism indicate that the rate of loss of neurons is faster initially, and then tends to approach the normal age-related decline. In dopa responsive dystonia, the finding of normal fluorodopa PET led to the prediction that the lesion would be functional rather than anatomical; this has been confirmed by the identification of a defect in dopamine synthesis in this disorder. Finally, new PET ligands show promise for future studies designed to unravel the pathogenesis of diseases involving the basal ganglia. (author)

  7. Further characterization of computed tomographic and clinical features for staging and prognosis of idiopathic pulmonary fibrosis in West Highland white terriers.

    Science.gov (United States)

    Thierry, Florence; Handel, Ian; Hammond, Gawain; King, Lesley G; Corcoran, Brendan M; Schwarz, Tobias

    2017-07-01

    Idiopathic pulmonary fibrosis is an interstitial lung disease of unknown etiology resulting in progressive interstitial fibrosis, with a known predilection in West Highland white terriers. In humans, computed tomography (CT) is a standard method for providing diagnostic and prognostic information, and plays a major role in the idiopathic pulmonary fibrosis staging process. Objectives of this retrospective, analytical, cross-sectional study were to establish descriptive criteria for reporting CT findings and test correlations among CT, clinical findings and survival time in West Highland white terriers with idiopathic pulmonary fibrosis. Inclusion criteria for affected West Highland white terriers were a diagnosis of idiopathic pulmonary fibrosis and available CT, bronchoscopy, bronchoalveolar lavage, echocardiography, and routine blood analysis findings. Clinically normal West Highland white terriers were recruited for the control group. Survival times were recorded for affected dogs. The main CT lung pattern and clinical data were blindly and separately graded as mild, moderate, or severe. Twenty-one West Highland white terriers with idiopathic pulmonary fibrosis and 11 control West Highland white terriers were included. The severity of pulmonary CT findings was positively correlated with severity of clinical signs (ρ = 0.48, P = 0.029) and negatively associated with survival time after diagnosis (ρ = -0.56, P = 0.025). Affected dogs had higher lung attenuation (median: -563 Hounsfield Units (HU)) than control dogs (median: -761 HU), (P idiopathic pulmonary fibrosis in West Highland white terriers and providing prognostic information for owners. © 2017 The Authors. Veterinary Radiology & Ultrasound published by Wiley Periodicals, Inc. on behalf of American College of Veterinary Radiology.

  8. V/P SPECT as a diagnostic tool for pregnant women with suspected pulmonary embolism

    Energy Technology Data Exchange (ETDEWEB)

    Bajc, Marika; Olsson, Berit; Joegi, Jonas [Skaane University Hospital and Lund University, Clinical Physiology and Nuclear Medicine, Lund (Sweden); Gottsaeter, Anders [Skaane University Hospital, Vascular Diseases, Malmoe (Sweden); Hindorf, Cecilia [Skaane University Hospital, Radiation Physics, Lund (Sweden)

    2015-07-15

    The purpose of the study was to assess the prevalence of pulmonary embolism (PE) and other lung diseases among pregnant women with suspected PE and to calculate the radiation exposure to patient and fetus in this population. As a secondary aim, we evaluated the negative predictive value of a normal ventilation/perfusion single photon emission computed tomography (V/P SPECT) examination in pregnancy. We studied all 127 pregnant women who had suspected PE and had undergone V/P SPECT at our institution in the course of a 5-year period. Radiation exposure to patient and fetus and the negative predictive value of a normal V/P SPECT examination were also measured. V/P SPECT identified PE in 11 women (9 %). Moreover, in 15 women (12 %) the examination revealed pneumonia (in 2 cases in addition to PE) and in 1 woman signs of airway obstruction were revealed. Among the 116/127 women (91 %) where PE was ruled out by V/P SPECT, none was diagnosed subsequently with PE or deep venous thrombosis (DVT) during the same pregnancy or puerperal period. For P SPECT, the calculated fetal absorbed dose was < 0.6 mGy,and the calculated breast absorbed dose 0.6 mGy. For V SPECT, the calculated fetal absorbed dose was < 0.014 mGy and the breast absorbed dose 0.25 mGy. The prevalence of PE was low (9 %) among pregnant women with suspected disease. Pneumonia was diagnosed in 12 % of patients. The negative predictive value of V/P SPECT was high, and the radiation exposure from V/P SPECT was low both for fetus and patient. (orig.)

  9. PARAMETERS FOR THE EVALUATION OF CERVICAL SAGITTAL BALANCE IN IDIOPATHIC SCOLIOSIS

    Directory of Open Access Journals (Sweden)

    MAURICIO COELHO LIMA

    Full Text Available ABSTRACT Objective: There are no values defined as standard in the literature for the parameters of assessment of cervical sagittal balance in patients with idiopathic scoliosis. This study describes the sagittal cervical parameters in patients with idiopathic scoliosis. Methods: Study carried out in a tertiary public hospital in patients with adolescent idiopathic scoliosis, through the evaluation of panoramic radiographs in lateral view. The Cobb method was used to evaluate cervical lordosis from C2 to C7, distance from the center of gravity (COG of the skull to C7, measurement of T1 slope, thoracic inlet angle (TIA, neck tilt, and plumb line from C7 to S1 (SVA C7-S1. A statistical analysis was performed, to demonstrate the relationship between the alignment of the thoracic spine in the sagittal plane and the cervical sagittal balance of patients with scoliosis. Results: Thirty-four patients were female (69.4% and 15 male (30.6%. The mean values for COG-C7 were 0.71 mm (median 0.8 mm/standard deviation [SD]= 0.51 mm. For Cobb C2-C7, the mean was -11.7° (median -10°/SD= 20.4°. The mean slope of T1 was 23.5° (median 25°/SD= 9.5°. The mean cervical version was 58.8° (median 60°/DP= 15.4°. The mean TIA was 81.8° (median 85°/SD= 16.7°. The mean plumb line C7-S1 was -0.28 (-0.3/SD= 1.0. Conclusion: The analysis of the results showed that the mean values for the cervical lordosis are lower than the values described as normal in the literature, suggesting a loss of sagittal cervical balance in these patients.

  10. Esophageal striated muscle contractions in patients with Chagas' disease and idiopathic achalasia

    Directory of Open Access Journals (Sweden)

    R.O. Dantas

    2002-06-01

    Full Text Available Chagas' disease causes degeneration and reduction of the number of intrinsic neurons of the esophageal myenteric plexus, with consequent absent or partial lower esophageal sphincter relaxation and loss of peristalsis in the esophageal body. The impairment of esophageal motility is seen mainly in the distal smooth muscle region. There is no study about esophageal striated muscle contractions in the disease. In 81 patients with heartburn (44 with esophagitis taken as controls, 51 patients with Chagas' disease (21 with esophageal dilatation and 18 patients with idiopathic achalasia (11 with esophageal dilatation we studied the amplitude, duration and area under the curve of esophageal proximal contractions. Using the manometric method and a continuous perfusion system we measured the esophageal striated muscle contractions 2 to 3 cm below the upper esophageal sphincter after swallows of a 5-ml bolus of water. There was no significant difference in striated muscle contractions between patients with heartburn and esophagitis and patients with heartburn without esophagitis. There was also no significant difference between patients with heartburn younger or older than 50 years or between men and women or in esophageal striated muscle contractions between patients with heartburn and Chagas' disease. The esophageal proximal amplitude of contractions was lower in patients with idiopathic achalasia than in patients with heartburn. In patients with Chagas' disease there was no significant difference between patients with esophageal dilatation and patients with normal esophageal diameter. Esophageal striated muscle contractions in patients with Chagas' disease have the same amplitude and duration as seen in patients with heartburn. Patients with idiopathic achalasia have a lower amplitude of contraction than patients with heartburn.

  11. Global alteration in gene expression profiles of deciduas from women with idiopathic recurrent pregnancy loss.

    Science.gov (United States)

    Krieg, S A; Fan, X; Hong, Y; Sang, Q-X; Giaccia, A; Westphal, L M; Lathi, R B; Krieg, A J; Nayak, N R

    2012-09-01

    Recurrent pregnancy loss (RPL) occurs in ∼5% of women. However, the etiology is still poorly understood. Defects in decidualization of the endometrium during early pregnancy contribute to several pregnancy complications, such as pre-eclampsia and intrauterine growth restriction (IUGR), and are believed to be important in the pathogenesis of idiopathic RPL. We performed microarray analysis to identify gene expression alterations in the deciduas of idiopathic RPL patients. Control patients had one antecedent term delivery, but were undergoing dilation and curettage for current aneuploid miscarriage. Gene expression differences were evaluated using both pathway and gene ontology (GO) analysis. Selected genes were validated using quantitative reverse transcription-polymerase chain reaction (qRT-PCR). A total of 155 genes were found to be significantly dysregulated in the deciduas of RPL patients (>2-fold change, P genes up-regulated and 133 genes down-regulated. GO analysis linked a large percentage of genes to discrete biological functions, including immune response (23%), cell signaling (18%) and cell invasion (17.1%), and pathway analysis revealed consistent changes in both the interleukin 1 (IL-1) and IL-8 pathways. All genes in the IL-8 pathway were up-regulated while genes in the IL-1 pathway were down-regulated. Although both pathways can promote inflammation, IL-1 pathway activity is important for normal implantation. Additionally, genes known to be critical for degradation of the extracellular matrix, including matrix metalloproteinase 26 and serine peptidase inhibitor Kazal-type 1, were also highly up-regulated. In this first microarray approach to decidual gene expression in RPL patients, our data suggest that dysregulation of genes associated with cell invasion and immunity may contribute significantly to idiopathic recurrent miscarriage.

  12. Relieving idiopathic dental pain without drugs

    Directory of Open Access Journals (Sweden)

    Haryono Utomo

    2011-06-01

    Full Text Available Background: Teeth are commonly obvious source of orofacial pain. Sometimes the pain source is undetectable, thus called as idiopathic dental pain. Since dentist wants to alleviate or eliminate the pains with every effort in their mind, a lot of drugs could be prescribed. Moreover, it is make sense that endodontic treatment or even tooth extraction will be done. Unfortunately, endodontic treatment may also initiate neuropathic tooth pain that is caused by nerve extirpation, thus worsen the pain. Therefore, another cause of dental pain such as referred pain, periodontal disease, or stress which related to psychoneuroimmunology should be considered. In order to prevent from unnecessary drugs or invasive treatment such as root canal treatment and extraction, correct diagnosis and preliminary non-invasive therapies should be done. Purpose: This review elucidates several therapies that could be done by dentists for relieving idiopathic dental pain which includes massage, the “assisted drainage” therapy, modulation of psychoneuroimmunologic status and dietary omega-3. Reviews: Understanding the basic pathogenesis of pain may help in elucidating the effects of non-drug pain therapy such as muscle massage, the “assisted drainage” therapy, omega-3 and psychological stress relieving. These measures are accounted for eliminating referred pain, reducing proinflammatory mediators and relieving unwanted stress reactions consecutively. Psychological stress increases proinflammatory cytokines and thus lowered pain threshold. Conclusion: As an individual treatment, this non-drug therapy is useful in relieving idiopathic dental pain; nevertheless, if they work together the result could be more superior.Latar belakang: Gigi adalah suatu penyebab umum dari nyeri orofasial. Kadang kala penyebab nyeri tidak dapat ditemukan, sehingga disebut sebagai nyeri gigi idiopatik. Karena dokter gigi berupaya untuk mengurangi atau menghilangkan nyeri dengan segala cara

  13. The use of psychoactive prescription drugs among DUI suspects.

    Science.gov (United States)

    Karjalainen, Karoliina; Haukka, Jari; Lintonen, Tomi; Joukamaa, Matti; Lillsunde, Pirjo

    2015-10-01

    The study seeks to increase understanding of the use of psychoactive prescription drugs among persons suspected of driving under the influence (DUI). We studied whether the use of prescribed psychoactive medication was associated with DUI, and examined the difference in the use of prescription drugs between DUI recidivists and those arrested only once. In this register-based study, persons suspected of DUI (n=29470) were drawn from the Register of DUI suspects, and an age- and gender-matched reference population (n=30043) was drawn from the Finnish general population. Data on prescription drug use was obtained by linkage to the National Prescription Register. The associations of DUI arrest and use of psychoactive prescription drugs in different DUI groups (findings for alcohol only, prescription drugs, prescription drugs and alcohol, illicit drugs) were estimated by using mixed-effect logistic regression. The use of psychoactive prescription drugs and DUI appeared to be strongly associated, with DUI suspects significantly more likely to use psychoactive prescription drugs compared to the reference population. Gender differences existed, with the use of benzodiazepines being more common among female DUI suspects. Moreover, DUI recidivists were more likely to use psychoactive prescription drugs compared to those arrested only once. In addition to alcohol and/or illicit drug use, a significant proportion of DUI suspects were using psychoactive prescription drugs. When prescribing psychoactive medication, especially benzodiazepines, physicians are challenged to screen for possible substance use problems and also to monitor for patients' alcohol or illicit drug use while being medicated. Copyright © 2015 Elsevier Ireland Ltd. All rights reserved.

  14. Book Review: Placing the Suspect behind the Keyboard: Using Digital Forensics and Investigative Techniques to Identify Cybercrime Suspects

    Directory of Open Access Journals (Sweden)

    Thomas Nash

    2013-06-01

    Full Text Available Shavers, B. (2013. Placing the Suspect behind the Keyboard: Using Digital Forensics and Investigative Techniques to Identify Cybercrime Suspects. Waltham, MA: Elsevier, 290 pages, ISBN-978-1-59749-985-9, US$51.56. Includes bibliographical references and index.Reviewed by Detective Corporal Thomas Nash (tnash@bpdvt.org, Burlington Vermont Police Department, Internet Crime against Children Task Force. Adjunct Instructor, Champlain College, Burlington VT.In this must read for any aspiring novice cybercrime investigator as well as the seasoned professional computer guru alike, Brett Shaver takes the reader into the ever changing and dynamic world of Cybercrime investigation.  Shaver, an experienced criminal investigator, lays out the details and intricacies of a computer related crime investigation in a clear and concise manner in his new easy to read publication, Placing the Suspect behind the Keyboard. Using Digital Forensics and Investigative techniques to Identify Cybercrime Suspects. Shaver takes the reader from start to finish through each step of the investigative process in well organized and easy to follow sections, with real case file examples to reach the ultimate goal of any investigation: identifying the suspect and proving their guilt in the crime. Do not be fooled by the title. This excellent, easily accessible reference is beneficial to both criminal as well as civil investigations and should be in every investigator’s library regardless of their respective criminal or civil investigative responsibilities.(see PDF for full review

  15. Idiopathic urethritis in children: Classification and treatment with steroids.

    Science.gov (United States)

    Jayakumar, Sivasankar; Pringle, Kirsty; Ninan, George K

    2014-07-01

    Idiopathic urethritis [IU] in children is of unknown etiology and treatment options are limited. We propose a classification for IU based on cystourethroscopy findings and symptoms (Grade 1 - 4) and report our experience with use of topical and oral steroids in IU. Retrospective data collection of all male children (0-16 years) diagnosed with IU over a period of 8 years between 2005 and 2012 at our institution. Data was collected on patient demographics, laboratory and radiological investigations, cystourethroscopy findings, management and outcomes. A total of 19 male children were diagnosed with IU. The median age of the patients was 13(7-16) years. Presenting symptoms included dysuria in 12; hematuria in 9; loin pain in 6; and scrotal pain in 2 patients. Both patients with scrotal pain had previous left scrotal exploration that revealed epididymitis. Serum C-reactive protein and Full blood count was tested in 15 patients and was within normal limits in all of them. Cystourethroscopy revealed urethritis of grade-I in 2; grade-II in 11; and grade-III in 3 patients. There were 3 patients with systemic symptoms from extra-urethral extension of inflammation (grade-IV). Mean follow up was 18.9(1-74) months. All patients had steroid instillation at the time of cystourethroscopy. Three patients with IU grade IV required oral steroids (prednisolone) in view of exacerbation of symptoms and signs despite steroid instillation. Complete resolution of symptoms and signs occurred in 18(94.7%) patients. Significant improvement in symptoms and signs was noted in 1(5.3%) patient who is still undergoing treatment. IU in male children can be successfully managed with steroid instillation, especially in grade I and II. Grade III, will need steroid instillation but treatment of scarring and stricture will necessitate longer duration of treatment. In children with IU and extra-urethral symptoms (grade IV), oral steroids may be required.

  16. Idiopathic urethritis in children: Classification and treatment with steroids

    Directory of Open Access Journals (Sweden)

    Sivasankar Jayakumar

    2014-01-01

    Full Text Available Background: Idiopathic urethritis [IU] in children is of unknown etiology and treatment options are limited. We propose a classification for IU based on cystourethroscopy findings and symptoms (Grade 1 - 4 and report our experience with use of topical and oral steroids in IU. Materials and Methods: Retrospective data collection of all male children (0-16 years diagnosed with IU over a period of 8 years between 2005 and 2012 at our institution. Data was collected on patient demographics, laboratory and radiological investigations, cystourethroscopy findings, management and outcomes. Results: A total of 19 male children were diagnosed with IU. The median age of the patients was 13(7-16 years. Presenting symptoms included dysuria in 12; hematuria in 9; loin pain in 6; and scrotal pain in 2 patients. Both patients with scrotal pain had previous left scrotal exploration that revealed epididymitis. Serum C-reactive protein and Full blood count was tested in 15 patients and was within normal limits in all of them. Cystourethroscopy revealed urethritis of grade-I in 2; grade-II in 11; and grade-III in 3 patients. There were 3 patients with systemic symptoms from extra-urethral extension of inflammation (grade-IV. Mean follow up was 18.9(1-74 months. All patients had steroid instillation at the time of cystourethroscopy. Three patients with IU grade IV required oral steroids (prednisolone in view of exacerbation of symptoms and signs despite steroid instillation. Complete resolution of symptoms and signs occurred in 18(94.7% patients. Significant improvement in symptoms and signs was noted in 1(5.3% patient who is still undergoing treatment. Conclusions: IU in male children can be successfully managed with steroid instillation, especially in grade I and II. Grade III, will need steroid instillation but treatment of scarring and stricture will necessitate longer duration of treatment. In children with IU and extra-urethral symptoms (grade IV, oral

  17. Use of the Universal Clamp in adolescent idiopathic scoliosis.

    Science.gov (United States)

    Sales de Gauzy, Jérôme; Jouve, Jean-Luc; Ilharreborde, Brice; Blondel, Benjamin; Accadbled, Franck; Mazda, Keyvan

    2014-07-01

    The Universal Clamp (UC) equipped with a soft sublaminar band is a relatively new thoracic anchor that can be used in hybrid constructs. A dedicated reduction tool that applies traction to the sublaminar band permits gentle translation of the thoracic curve to the pre-contoured fusion rods, which have been previously anchored distally by pedicle screws and proximally by hooks in a claw configuration. A literature review concerning the use of UC in adolescent idiopathic Scoliosis was performed. The results confirm the efficacy with the UC hybrid constructs, the good correction (71-66%) of the main thoracic curve and low loss of correction at 2-year follow-up (3-4%). The thoracic curve correction achieved and maintained in the AIS patients appears to be substantially higher than that reported for all hook and hook hybrid techniques. Regardless of the deformity correcting method employed with all-screw constructs, the Cobb angle correction achieved is equivalent to that achieved with UC hybrid devices. Among the present AIS patients who had normal values of thoracic kyphosis pre-operatively, there was almost no change in average thoracic kyphosis at the latest follow-up. However, among the patients with low pre-operative values of T5-T12 kyphosis, the average T5-T12 kyphosis improved. Hybrid construct with UC is safe, with reduced operative time and blood loss. While achieving deformity correction in the coronal and axial planes equivalent to the best-reported results of all screw or previous hybrid constructs, the UC hybrid technique appears to provide superior correction in the sagittal plane. The excellent outcome is maintained at the 2-year follow-up.

  18. Homocysteine plasma levels in patients suspected coronary artery disease: Relation to myocardial perfusion image

    International Nuclear Information System (INIS)

    Yao, Z.Y.; He, Q.; Qu, W.

    2002-01-01

    Purpose: Although there is considerable epidemiologic evidence for a relationship between plasma homocysteine (Hcy) levels and coronary artery disease (CAD), not all studies, especially prospective ones have shown such a relationship. The purpose of this study was to investigate a possible association between Hcy plasma levels and myocardial perfusion defect by SPECT in patients suspected CAD. Methods and Materials: A cohort of 238 patients suspected CAD (age: 60.65±10.43, male to female: 172: 66) was examined for Hcy, tetrahydrofolic acid (FH4), vitamine B12 and coronary angiography (CAG). Furthermore, 42 patients also underwent 99m Tc-MIBI myocardial perfusion images (MPI) to assess the myocardial perfusion. Results: There were 69 patients with normal CAG and 63, 60, 42 and 4 patients with 1 vessel, two vessel, 3 vessel and left main coronary stenosis. The plasma Hcy of this group was significantly increased, p 0.05. In patients with >=3 segments myocardial perfusion defect, 10 of them had normal Hcy, and 7 with hyperhomocysteinemia, in patients with 0.05). Conclusion: Our data may indicate that hyperhomocysteinemia represents an independent risk factor in patients with high possibility of CAD rather than a mark of myocardial ischemia or coronary stenosis

  19. Molecular Identification of Zoonotic Tissue-Invasive Tapeworm Larvae Other than Taenia solium in Suspected Human Cysticercosis Cases.

    Science.gov (United States)

    Tappe, Dennis; Berkholz, Jörg; Mahlke, Uwe; Lobeck, Hartmut; Nagel, Thomas; Haeupler, Alexandra; Muntau, Birgit; Racz, Paul; Poppert, Sven

    2016-01-01

    Rarely, zoonotic Taenia species other than Taenia solium cause human cysticercosis. The larval stages are morphologically often indistinguishable. We therefore investigated 12 samples of suspected human cysticercosis cases at the molecular level and surprisingly identified one Taenia crassiceps and one Taenia serialis (coenurosis) infection, which were caused by tapeworm larvae normally infecting rodents and sheep via eggs released from foxes and dogs. Copyright © 2015, American Society for Microbiology. All Rights Reserved.

  20. Development of additional pituitary hormone deficiencies in pediatric patients originally diagnosed with idiopathic isolated GH deficiency

    NARCIS (Netherlands)

    W.F. Blum (Werner); C.L. Deal (Cheri Lynn); A.G. Zimmermann (Alan); E.P. Shavrikova (Elena); C.J. Child (Christopher); C.A. Quigley (Charmian); S.L.S. Drop (Stenvert); G. Cutler (Gordon); R.G. Rosenfeld (Ron)

    2014-01-01

    textabstractObjective: We assessed the characteristics of children initially diagnosed with idiopathic isolated GH deficiency (IGHD) who later developed additional (multiple) pituitary hormone deficiencies (MPHD). Design: Data were analyzed for 5805 pediatric patients with idiopathic IGHD, who were

  1. Bone marrow stroma in idiopathic myelofibrosis and other haematological diseases. An immunohistochemical study

    DEFF Research Database (Denmark)

    Lisse, I; Hasselbalch, H; Junker, P

    1991-01-01

    Bone marrow stroma was investigated immunohistochemically in 31 patients with haematological diseases, mainly idiopathic myelofibrosis (n = 8) and related chronic myeloproliferative disorders (n = 14). The bone marrow from patients with idiopathic myelofibrosis and some CML patients showed marked...

  2. Comparing new treatments for idiopathic pulmonary fibrosis--a network meta-analysis.

    LENUS (Irish Health Repository)

    Loveman, Emma

    2015-01-01

    The treatment landscape for idiopathic pulmonary fibrosis, a devastating lung disease, is changing. To investigate the effectiveness of treatments for idiopathic pulmonary fibrosis we undertook a systematic review, network meta-analysis and indirect comparison.

  3. Body image in patients with adolescent idiopathic scoliosis: validation of the Body Image Disturbance Questionnaire--Scoliosis Version.

    Science.gov (United States)

    Auerbach, Joshua D; Lonner, Baron S; Crerand, Canice E; Shah, Suken A; Flynn, John M; Bastrom, Tracey; Penn, Phedra; Ahn, Jennifer; Toombs, Courtney; Bharucha, Neil; Bowe, Whitney P; Newton, Peter O

    2014-04-16

    Appearance concerns in individuals with adolescent idiopathic scoliosis can result in impairment in daily functioning, or body image disturbance. The Body Image Disturbance Questionnaire (BIDQ) is a self-reported, seven-question instrument that measures body image disturbance in general populations; no studies have specifically examined body image disturbance in those with adolescent idiopathic scoliosis. This study aimed to validate a modified version of the BIDQ in a population with adolescent idiopathic scoliosis and to establish discriminant validity by comparing responses of operatively and nonoperatively treated patients with those of normal controls. In the first phase, a multicenter study of forty-nine patients (mean age, fourteen years; thirty-seven female) with adolescent idiopathic scoliosis was performed to validate the BIDQ-Scoliosis version (BIDQ-S). Participants completed the BIDQ-S, Scoliosis Research Society (SRS)-22, Children's Depression Index (CDI), and Body Esteem Scale for Adolescents and Adults (BESAA) questionnaires. Descriptive statistics and Pearson correlation coefficients were calculated. In the second phase, ninety-eight patients with adolescent idiopathic scoliosis (mean age, 15.7 years; seventy-five female) matched by age and sex with ninety-eight healthy adolescents were enrolled into a single-center study to evaluate the discriminant validity of the BIDQ-S. Subjects completed the BIDQ-S and a demographic form before treatment. Independent-sample t tests and Pearson correlation coefficients were calculated. The BIDQ-S was internally consistent (Cronbach alpha = 0.82), and corrected item total correlations ranged from 0.47 to 0.67. The BIDQ-S was significantly correlated with each domain of the SRS-22 and the total score (r = -0.50 to -0.72, p ≤ 0.001), with the CDI (r = 0.31, p = 0.03), and with the BESAA (r = 0.60, p image disturbance compared with healthy controls. To our knowledge, this user-friendly instrument is the first to

  4. Prucalopride: A Review in Chronic Idiopathic Constipation.

    Science.gov (United States)

    Garnock-Jones, Karly P

    2016-01-01

    Prucalopride (Resolor®), a highly selective serotonin 5-HT4 receptor agonist, is indicated in the European Economic Area for the treatment of adults with chronic idiopathic constipation (CIC) in whom laxatives have failed to provide adequate relief. This article reviews the pharmacological properties of prucalopride and its clinical efficacy and tolerability in patients with CIC. In five well-designed, 12-week trials in patients with CIC, oral prucalopride 2 mg/day was significantly more effective than placebo at improving bowel function, including the number of bowel movements and a range of other constipation symptoms, as well as health-related quality of life and patient satisfaction; however, no significant differences in bowel function measures were observed between prucalopride and placebo in a 24-week trial. Oral PEG-3350 + electrolytes reconstituted powder was found to be noninferior but not superior to prucalopride according to primary endpoint data from a 4-week, controlled-environment trial. Prucalopride was generally well tolerated in clinical trials; the most common adverse events were headache, diarrhoea, nausea and abdominal pain. No cardiovascular safety issues have arisen with prucalopride treatment. Although further long-term and comparative data would be beneficial, prucalopride provides an additional treatment option for patients with CIC.

  5. [Juvenile idiopathic arthritis and oral health].

    Science.gov (United States)

    Kobus, Agnieszka; Kierklo, Anna; Sielicka, Danuta; Szajda, Sławomir Dariusz

    2016-05-04

    Juvenile idiopathic arthritis (JIA) is the most common autoimmune inflammatory disease of connective tissue in children. It is characterized by progressive joint destruction which causes preserved changes in the musculoskeletal system. The literature describes fully clinical symptoms and radiological images in different subtypes of JIA. However, there is still a limited number of studies reporting on the medical condition of the oral cavity of ill children. JIA can affect hard and soft tissues of the oral cavity by: the general condition of the child's health, arthritis of the upper limbs, as the result of the pharmacotherapy, changes in secretion and composition of saliva, inflammation of the temporomandibular joint and facial deformity. The study summarizes the available literature on the condition of the teeth and periodontal and oral hygiene in the course of JIA. The presence of diverse factors that modify the oral cavity, such as facial growth, functioning of salivary glands, or the supervision and care provided by adults, prevents clear identification if JIA leads to severe dental caries and periodontal disease. Despite conflicting results in studies concerning the clinical oral status, individuals with JIA require special attention regarding disease prevention and maintenance of oral health.

  6. Juvenile idiopathic arthritis-associated uveitis.

    Science.gov (United States)

    Clarke, Sarah L N; Sen, Ethan S; Ramanan, Athimalaipet V

    2016-04-27

    Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease of childhood, with JIA-associated uveitis its most common extra-articular manifestation. JIA-associated uveitis is a potentially sight-threatening condition and thus carries a considerable risk of morbidity. The aetiology of the condition is autoimmune in nature with the predominant involvement of CD4(+) T cells. However, the underlying pathogenic mechanisms remain unclear, particularly regarding interplay between genetic and environmental factors. JIA-associated uveitis comes in several forms, but the most common presentation is of the chronic anterior uveitis type. This condition is usually asymptomatic and thus screening for JIA-associated uveitis in at-risk patients is paramount. Early detection and treatment aims to stop inflammation and prevent the development of complications leading to visual loss, which can occur due to both active disease and burden of disease treatment. Visually disabling complications of JIA-associated uveitis include cataracts, glaucoma, band keratopathy and macular oedema. There is a growing body of evidence for the early introduction of systemic immunosuppressive therapies in order to reduce topical and systemic glucocorticoid use. This includes more traditional treatments, such as methotrexate, as well as newer biological therapies. This review highlights the epidemiology of JIA-associated uveitis, the underlying pathogenesis and how affected patients may present. The current guidelines and criteria for screening, diagnosis and monitoring are discussed along with approaches to management.

  7. Uveitis associated with juvenile idiopathic arthritis.

    Science.gov (United States)

    Sen, Ethan S; Dick, Andrew D; Ramanan, Athimalaipet V

    2015-06-01

    Uveitis is a potentially sight-threatening complication of juvenile idiopathic arthritis (JIA). JIA-associated uveitis is recognized to have an autoimmune aetiology characterized by activation of CD4(+) T cells, but the underlying mechanisms might overlap with those of autoinflammatory conditions involving activation of innate immunity. As no animal model recapitulates all the features of JIA-associated uveitis, questions remain regarding its pathogenesis. The most common form of JIA-associated uveitis is chronic anterior uveitis, which is usually asymptomatic initially. Effective screening is, therefore, essential to detect early disease and commence treatment before the development of visually disabling complications, such as cataracts, glaucoma, band keratopathy and cystoid macular oedema. Complications can result from uncontrolled intraocular inflammation as well as from its treatment, particularly prolonged use of high-dose topical corticosteroids. Accumulating evidence supports the early introduction of systemic immunosuppressive drugs, such as methotrexate, as steroid-sparing agents. Prospective randomized controlled trials of TNF inhibitors and other biologic therapies are underway or planned. Future research should aim to identify biomarkers to predict which children are at high risk of developing JIA-associated uveitis or have a poor prognosis. Such biomarkers could help to ensure that patients receive earlier interventions and more-potent therapy, with the ultimate aim of reducing loss of vision and ocular morbidity.

  8. Gastaut type idiopathic childhood occipital epilepsy.

    Science.gov (United States)

    Ferrari-Marinho, Taissa; Macedo, Eugenia Fialho; Costa Neves, Rafael Scarpa; Costa, Lívia Vianez; Tudesco, Ivanda S S; Carvalho, Kelly C; Carrete, Henrique; Caboclo, Luis Otavio; Yacubian, Elza Marcia; Hamad, Ana Paula

    2013-03-01

    Gastaut type idiopathic childhood occipital epilepsy is an uncommon epileptic syndrome characterised by frequent seizures, most commonly presenting as elementary visual hallucinations or blindness. Other occipital (non-visual) symptoms may also occur. Interictal EEG typically shows occipital paroxysms, often with fixation-off sensitivity. Ictal EEG is usually characterised by interruption by paroxysms and sudden appearance of low-voltage, occipital, fast rhythm and/or spikes. Despite well described clinical and EEG patterns, to our knowledge, there are very few reports in the literature with video-EEG recording of either seizure semiology or fixation-off phenomena. We present a video-EEG recording of a 12-year-old girl with Gastaut type epilepsy, illustrating the interictal and ictal aspects of this syndrome. Our aim was to demonstrate the clinical and neurophysiological pattern of a typical seizure of Gastaut type epilepsy, as well as the fixation-off phenomena, in order to further clarify the typical presentation of this syndrome. [Published with video sequences].

  9. Idiopathic Granulomatous Mastitis Associated with Erythema Nodosum

    Directory of Open Access Journals (Sweden)

    Tuğba Özlem Kalaycı

    2016-04-01

    Full Text Available Background: Idiopathic granulomatous mastitis (IGM is an uncommon benign chronic inflammatory breast disease, and erythema nodosum (EN is an extremely rare systemic manifestation of IGM. Here, we report a rare case of IGM accompanied by EN. Case Report: A 32-year-old patient was admitted to our clinic with a history of a tender mass in the right breast. On physical examination, the right breast contained a hard, tender mass in the lower half with in-drawing of the nipple. She had florid EN affecting both legs. She was evaluated with mammography, ultrasound, power Doppler ultrasound, non-enhancing magnetic resonance imaging (MRI, dynamic contrast-enhanced MRI, fine needle aspiration biopsy (FNAB and excisional biopsy. Time-intensity curves showed a type II pattern on dynamic contrast-enhanced MRI, which has an intermediate probability for malignancy. The FNAB reported a benign cytology suggestive of a granulomatous inflammation, which was also supported by the histopathological findings. A partial mastectomy was performed following medical treatment. There was no recurrence at 1-year follow-up. Conclusion: IGM should be considered in the differential diagnosis of EN. Although histopathological examination remains the only method for the definite diagnosis of IGM, MRI can be helpful in the diagnosis or differentiation of benign lesions from malignant ones.

  10. Two Sisters with Idiopathic Pulmonary Hemosiderosis

    Directory of Open Access Journals (Sweden)

    Mehmet Gencer

    2007-01-01

    Full Text Available Idiopathic pulmonary hemosiderosis (IPH is a rare cause of diffuse alveolar hemorrhage with unknown etiology. In the present report, the presentations of two sisters are described: one sister had IPH, eosinophilia and a high serum immunoglobulin E (IgE level; and the other had IPH, pneumothorax, eosinophilia and a high serum IgE level. Both cases had quite unusual presentations. The first patient was 23 years of age, and had suffered from dry cough and progressive dyspnea for four years. Her hemoglobin level was 60 g/L, total serum IgE level was 900 U/mL and eosinophilia was 9%. Her chest radiography revealed diffuse infiltration. She died due to respiratory failure. The second patient was 18 years of age. She had also suffered from dry cough and gradually increasing dyspnea for two years. She had partial pneumothorax in the right lung and diffuse infiltration in other pulmonary fields on chest radiography. Her hemoglobin level was 99 g/L, total serum IgE level was 1200 U/mL and eosinophilia was 8%. IPH was diagnosed by open lung biopsy. All these findings suggested that familial or allergic factors, as well as immunological factors, might have contributed to the etiology of IPH.

  11. Outcomes of Noninvasively Treated Idiopathic Toe Walkers.

    Science.gov (United States)

    Radtke, Kerstin; Karch, Nataliia; Goede, Fabian; Vaske, Bernhard; von Lewinski, Gabriela; Noll, Yvonne; Thren, Anneke

    2018-03-01

    Idiopathic toe walking (ITW) causes a common problem in pediatric orthopaedics. In the literature, numerous treatment options have been reported, but consensus about the management of ITW is still missing. The aim of the current study was to evaluate conservative treatment with pyramidal insoles. A total of 193 patients underwent conservative treatment between January 2010 and June 2013. Mean age at the beginning of the treatment was 7.75 ± 0.23 years (range 2.0-17.0 years). For all patients, demographic data, comorbid diseases, passive range of motion (ROM), persistent toe walking, and performed operations were retrospectively evaluated. Following operative treatment was defined failure. Eight (4.15%) patients underwent Achilles tendon lengthening operation after mean therapy time of 2.72 years (range 0.1-7.0 years), 174 cases were treated successfully (90.16%). In 50 cases, toe walking suspended completely after mean therapy time of 2.83 years. In cases of failure, patients were older at diagnosis and at the beginning of the treatment. Mean passive ROM increased over the time. In cases of failure, ROM decreased from the first to the second examination. Conservative treatment of ITW using pyramidal insoles can be effective. Ankle dorsiflexion significantly improved in the patients who were successfully treated. Therapy should start early. A decrease of ROM under therapy should lead to critical revisal of individual therapy. Therapeutic, level IV: Case series.

  12. The knee in diffuse idiopathic skeletal hyperostosis

    International Nuclear Information System (INIS)

    Orzincolo, C.; Scutellari, P.N.; Aiello, N.; Trotta, F.

    1987-01-01

    Diffuse idiopathic skeletal hyperostosis (DISH) is a disorder characterized by bone proliferation of spinal and extraspinal structures. Frequently, extraspinal manifestations may occur before the involvement of the spine. These alterations are so common and characteristc that they should be included in diagnostic criteria for DISH. The patella is one of the most commonly involved sites. The knee of 48 patients affected by DISH, according to Resnick's diagnostic criteria, were subjected to systematic, radiographic and xerographic studies. The most characteristic radiographic patterns are: thickening of the anterior margin of the patella (81.1%); ossifying enthesopathy of supero-anterior margin of patella (68.7%); periosteal new bone formation on the tibial insertion of the cruciateligaments (47.8%); presence of fabella (41%); presence of megafabella (22.9%), which may come near the posterior profile of the femur. Changes are usually symmetrical in DISH: entheseal abnormalities mostly involve the antero-superior margin of the patella, whereas in osteoarthritis, osteophytes are found on the postero-superior margin. In the lateral view enthesophytes at the bone attachment of the cruciate ligaments are oriented inside the joint space; on the contrary, osteophytes are always oriented outside the joint space. Radiographic manifestations of the fabella are similar to those observed in osteoarthritis, and so they cannot be utilized in the differential diagnosis of these diseases

  13. Blood Biomarkers in Idiopathic Pulmonary Fibrosis.

    Science.gov (United States)

    Guiot, Julien; Moermans, Catherine; Henket, Monique; Corhay, Jean-Louis; Louis, Renaud

    2017-06-01

    Idiopathic pulmonary fibrosis (IPF) is a progressive and lethal lung disease of unknown origin whose incidence has been increasing over the latest decade partly as a consequence of population ageing. New anti-fibrotic therapy including pirfenidone and nintedanib have now proven efficacy in slowing down the disease. Nevertheless, diagnosis and follow-up of IPF remain challenging. This review examines the recent literature on potentially useful blood molecular and cellular biomarkers in IPF. Most of the proposed biomarkers belong to chemokines (IL-8, CCL18), proteases (MMP-1 and MMP-7), and growth factors (IGBPs) families. Circulating T cells and fibrocytes have also gained recent interest in that respect. Up to now, though several interesting candidates are profiling there has not been a single biomarker, which proved to be specific of the disease and predictive of the evolution (decline of pulmonary function test values, risk of acute exacerbation or mortality). Large scale multicentric studies are eagerly needed to confirm the utility of these biomarkers.

  14. The natural history of adolescent idiopathic scoliosis

    Directory of Open Access Journals (Sweden)

    Wong Hee-Kit

    2010-01-01

    Full Text Available There have been great advances in the conservative and surgical treatment for adolescent idiopathic scoliosis in the last few decades. The challenge for the physician is the decision for the optimal time to institute therapy for the individual child. This makes an understanding of the natural history and risk factors for curve progression of significant importance. Reported rates of curve progression vary from 1.6% for skeletally mature children with a small curve magnitude to 68% for skeletally immature children with larger curve magnitudes. Although the patient′s age at presentation, the Risser sign, the patient′s menarchal status and the magnitude of the curve have been described as risk factors for curve progression, there is evidence that the absolute curve magnitude at presentation may be most predictive of progression in the long term. A curve magnitude of 25º at presentation may be predictive of a greater risk of curve progression. Advances in research may unlock novel predictive factors, which are based on the underlying pathogenesis of this disorder.

  15. PROFILEing idiopathic pulmonary fibrosis: rethinking biomarker discovery

    Directory of Open Access Journals (Sweden)

    Toby M. Maher

    2013-06-01

    Full Text Available Despite major advances in the understanding of the pathogenesis of idiopathic pulmonary fibrosis (IPF, diagnosis and management of the condition continue to pose significant challenges. Clinical management of IPF remains unsatisfactory due to limited availability of effective drug therapies, a lack of accurate indicators of disease progression, and an absence of simple short-term measures of therapeutic response. The identification of more accurate predictors of prognosis and survival in IPF would facilitate counseling of patients and their families, aid communication among clinicians, and would guide optimal timing of referral for transplantation. Improvements in molecular techniques have led to the identification of new disease pathways and a more targeted approach to the development of novel anti-fibrotic agents. However, despite an increased interest in biomarkers of IPF disease progression there are a lack of measures that can be used in early phase clinical trials. Careful longitudinal phenotyping of individuals with IPF together with the application of novel omics-based technology should provide important insights into disease pathogenesis and should address some of the major issues holding back drug development in IPF. The PROFILE (Prospective Observation of Fibrosis in the Lung Clinical Endpoints study is a currently enrolling, prospective cohort study designed to tackle these issues.

  16. A Rare Case of Idiopathic Plastic Bronchitis

    Directory of Open Access Journals (Sweden)

    Mohammed Raoufi

    2017-01-01

    Full Text Available Plastic bronchitis is a rare disorder characterized by formation of large, branching bronchial casts, which are often expectorated. We present an interesting case of a 35-year-old woman who presented for evaluation of a chronic cough productive of voluminous secretions. Clinical and radiological examination confirmed a total left lung atelectasis without any pathological mediastinal node. Flexible bronchoscopy demonstrated tenacious, thick, and sticky whitish secretions blocking the left stem bronchus. This material was extracted, and inspection demonstrated a bronchial cast, whose pathological analysis revealed necrotic epithelial cells, some eosinophils, and Charcot-Leyden crystals. Two days after bronchoscopy, the patient rejected more bronchial casts, and dyspnea improved. Control of chest x-ray revealed complete left lung aeration and the diagnosis of idiopathic plastic bronchitis was obtained. This article shows the interest in clinical practice to evoke the diagnosis of plastic bronchitis in front of a productive chronic cough. Our case illustrates a rare clinical presentation represented by an atelectasis of an entire lung.

  17. Idiopathic inflammatory myopathies and the lung

    Directory of Open Access Journals (Sweden)

    Jean-Christophe Lega

    2015-06-01

    Full Text Available Idiopathic inflammatory myositis (IIM is a group of rare connective tissue diseases (CTDs characterised by muscular and extramuscular signs, in which lung involvement is a challenging issue. Interstitial lung disease (ILD is the hallmark of pulmonary involvement in IIM, and causes morbidity and mortality, resulting in an estimated excess mortality of 50% in some series. Except for inclusion body myositis, these extrapulmonary disorders are associated with the general and visceral involvement frequently found in other CTDs including fever, Raynaud's phenomenon, arthralgia, nonspecific cutaneous modifications and ILD, for which the prevalence is estimated to be up to 65%. Substantial heterogeneity exists within the spectrum of IIMs, and each condition is associated with various frequencies and subtypes of pulmonary involvement. This heterogeneity is partly related to the presence of various autoantibodies encompassing anti-synthetase, anti-MDA5 and anti-PM/Scl. ILD is present in all subsets of IIM including juvenile myositis, but is more frequent in dermatomyositis and overlap myositis. IIM can also be associated with other presentations of respiratory involvement, namely pulmonary arterial hypertension, pleural disease, infections, drug-induced toxicity, malignancy and respiratory muscle weakness. Here, we critically review the current knowledge about adult and juvenile myositis-associated lung disease with a detailed description of therapeutics for chronic and rapidly progressive ILD.

  18. Right thoracic curvature in the normal spine

    Directory of Open Access Journals (Sweden)

    Masuda Keigo

    2011-01-01

    Full Text Available Abstract Background Trunk asymmetry and vertebral rotation, at times observed in the normal spine, resemble the characteristics of adolescent idiopathic scoliosis (AIS. Right thoracic curvature has also been reported in the normal spine. If it is determined that the features of right thoracic side curvature in the normal spine are the same as those observed in AIS, these findings might provide a basis for elucidating the etiology of this condition. For this reason, we investigated right thoracic curvature in the normal spine. Methods For normal spinal measurements, 1,200 patients who underwent a posteroanterior chest radiographs were evaluated. These consisted of 400 children (ages 4-9, 400 adolescents (ages 10-19 and 400 adults (ages 20-29, with each group comprised of both genders. The exclusion criteria were obvious chest and spinal diseases. As side curvature is minimal in normal spines and the range at which curvature is measured is difficult to ascertain, first the typical curvature range in scoliosis patients was determined and then the Cobb angle in normal spines was measured using the same range as the scoliosis curve, from T5 to T12. Right thoracic curvature was given a positive value. The curve pattern was organized in each collective three groups: neutral (from -1 degree to 1 degree, right (> +1 degree, and left ( Results In child group, Cobb angle in left was 120, in neutral was 125 and in right was 155. In adolescent group, Cobb angle in left was 70, in neutral was 114 and in right was 216. In adult group, Cobb angle in left was 46, in neutral was 102 and in right was 252. The curvature pattern shifts to the right side in the adolescent group (p Conclusions Based on standing chest radiographic measurements, a right thoracic curvature was observed in normal spines after adolescence.

  19. Idiopathic diaphragmatic paralysis: Bell's palsy of the diaphragm?

    Science.gov (United States)

    Crausman, Robert S; Summerhill, Eleanor M; McCool, F Dennis

    2009-01-01

    Idiopathic diaphragm paralysis is probably more common and responsible for more morbidity than generally appreciated. Bell's palsy, or idiopathic paralysis of the seventh cranial nerve, may be seen as an analogous condition. The roles of zoster sine herpete and herpes simplex have increasingly been recognized in Bell's palsy, and there are some data to suggest that antiviral therapy is a useful adjunct to steroid therapy. Thus, we postulated that antiviral therapy might have a positive impact on the course of acute idiopathic diaphragm paralysis which is likely related to viral infection. Three consecutive patients with subacute onset of symptomatic idiopathic hemidiaphragm paralysis were empirically treated with valacyclovir, 1,000 mg twice daily for 1 week. Prior to therapy, diaphragmatic function was assessed via pulmonary function testing and two-dimensional B-mode ultrasound, with testing repeated 1 month later. Diaphragmatic function pre- and post-treatment was compared to that of a historical control group of 16 untreated patients. All three subjects demonstrated ultrasound recovery of diaphragm function 4-6 weeks following treatment with valacyclovir. This recovery was accompanied by improvements in maximum inspiratory pressure (PI(max)) and vital capacity (VC). In contrast, in the untreated cohort, diaphragm recovery occurred in only 11 subjects, taking an average of 14.9 +/- 6.1 months (mean +/- SD). The results of this small, preliminary study suggest that antiviral therapy with valacyclovir may be helpful in the treatment of idiopathic diaphragm paralysis induced by a viral infection.

  20. Treatment of the idiopathic scoliosis with brace and physiotherapy.

    Science.gov (United States)

    Hundozi-Hysenaj, Hajrije; Dallku, Iliriana Boshnjaku; Murtezani, Ardiana; Rrecaj, Shkurte

    2009-01-01

    Scoliosis is a three-dimensional deformation of the spine with a lateral curvature or deviation greater than 10 degrees and associated with vertebral rotation. Many conservative treatments are available for adolescents with idiopathic scoliosis, but the evidence for their effectiveness is still questioned. The objective of this study was to define the effectiveness of braces and individual physiotherapy for the comprehensive treatment of idiopathic scoliosis in adolescents. A retrospective study of 57 children with idiopathic thoracic dextroscoliosis with the magnitude of the thoracic curve between 20 degrees-35 degrees, treated in Orthopedic and Physiatrist Clinic as well as National Ortho-prosthetic Center within University Clinical Center of Kosova in Prishtina, during the period of 2003-2006. Inclusion of kinesitherapy in the comprehensive management of idiopathic scoliosis varied in the improvement of the muscle strength (satisfied and moderate) in almost 80% of the children while the correction of the curve was small in approximately 42.1% of cases. For children with idiopathic scoliosis, who require braces, an exercise program helps chest mobility, muscle strength, proper breathing flexibility in the spine, correct posture and keeps muscles in tone so that the transition period after brace removal is easier.

  1. Intraspinal anomalies in early-onset idiopathic scoliosis.

    Science.gov (United States)

    Pereira, E A C; Oxenham, M; Lam, K S

    2017-06-01

    In the United Kingdom, lower incidences of intraspinal abnormalities in patients with early onset idiopathic scoliosis have been observed than in studies in other countries. We aimed to determine the rates of these abnormalities in United Kingdom patients diagnosed with idiopathic scoliosis before the age of 11 years. This retrospective study of patients attending an urban scoliosis clinic identified 71 patients satisfying a criteria of: clinical diagnosis of idiopathic scoliosis; age of onset ten years and 11 months or less; MRI screening for intraspinal abnormalities. United Kingdom census data combined with patient referral data was used to calculate incidence. Mean age at diagnosis was six years with 39 right-sided and 32 left-sided curves. Four patients (5.6%) were found to have intraspinal abnormalities on MRI. These consisted of: two combined Arnold-Chiari type 1 malformations with syrinx; one syrinx with a low lying conus; and one isolated syrinx. Overall annual incidence of early onset idiopathic scoliosis was one out of 182 000 (0.0006%). This study reports the lowest rates to date of intraspinal anomalies in patients with early onset idiopathic scoliosis, adding to knowledge regarding current incidences of these abnormalities as well as any geographical variation in the nature of the disease. Cite this article: Bone Joint J 2017;99-B:829-33. ©2017 The British Editorial Society of Bone & Joint Surgery.

  2. Normal Pressure Hydrocephalus (NPH)

    Science.gov (United States)

    ... local chapter Join our online community Normal Pressure Hydrocephalus (NPH) Normal pressure hydrocephalus is a brain disorder ... Symptoms Diagnosis Causes & risks Treatments About Normal Pressure Hydrocephalus Normal pressure hydrocephalus occurs when excess cerebrospinal fluid ...

  3. Direct repair of spondylolysis presenting after correction of adolescent idiopathic scoliosis.

    Science.gov (United States)

    Koptan, Wael M T; ElMiligui, Yasser H; ElSharkawi, Mohammad M

    2011-02-01

    Although spondylolysis is found in 6% of idiopathic scoliosis patients, very little was reported on management of pars defects in this group. These patients with painful spondylolysis are most eligible for direct repair of the defect rather than lumbosacral fusion in an attempt to save motion segments. The aim of this work was to analyze the clinical and radiologic outcome of pars repair in a group of adolescents who presented after surgical correction of their idiopathic scoliosis. A prospective nonrandomized study. Ten consecutive patients with spondylolysis presenting after an average of 3 months (range, 2-7 months) from correction of their idiopathic scoliosis with low back pain not responding to conservative therapy and interfering with everyday activities. The mean age at operation was 16 years (range, 14-19 years). Total blood loss, operative time, and hospital stay were recorded. Clinical outcome was assessed by the Oswestry Disability Index (ODI), visual analog scale (VAS), and Scoliosis Research Society (SRS)-22 questionnaire. Fusion of the pars interarticularis was assessed using plain, lateral, and oblique radiographs and a computed tomography (CT) scan. The surgical technique consisted of thorough debridement of the defect, impacting the gap created with a tricortical iliac crest graft, and rigid fixation by either pedicle screws and a V-shaped rod (five patients) or a cable-screw construct (five patients). Patients were followed up for an average of 4.5 years (range, 2-7 years). Nine patients had a good-to-excellent result, returned to normal everyday life, and participated in sports when desired. The mean ODI, VAS, and SRS total scores were 11 (range, 0-34), 1.1 (range, 0-2), and 92 (range, 61-108), respectively. Follow-up radiographs and CT scans revealed healing of all defects in nine cases, no signs of disc degeneration in any, and no implant-related complications. The results of direct repair of spondylolysis in idiopathic scoliosis patients were

  4. Cytogenetic abnormalities and their prognostic significance in idiopathic myelofibrosis: a study of 106 cases.

    Science.gov (United States)

    Reilly, J T; Snowden, J A; Spearing, R L; Fitzgerald, P M; Jones, N; Watmore, A; Potter, A

    1997-07-01

    The prognostic significance of cytogenetic abnormalities was determined in 106 patients with well-characterized idiopathic myelofibrosis who were successfully karyotyped at diagnosis. 35% of the cases exhibited a clonal abnormality (37/106), whereas 65% (69/106) had a normal karyotype. Three characteristic defects, namely del(13q) (nine cases), del(20q) (eight cases) and partial trisomy 1q (seven cases), were present in 64.8% (24/37) of patients with clonal abnormalities. Kaplan-Meier plots and log rank analysis demonstrated an abnormal karyotype to be an adverse prognostic variable (P 10.3 x 10(9)/l; P=0.06) were also associated with a shorter survival. In contrast, sex, spleen and liver size, and percentage blast cells were not found to be significant. Multivariate analysis, using Cox's regression, revealed karyotype, haemoglobin concentration, platelet and leucocyte counts to retain their unfavourable prognostic significance. A simple and useful schema for predicting survival in idiopathic myelofibrosis has been produced by combining age, haemoglobin concentration and karyotype with median survival times varying from 180 months (good-risk group) to 16 months (poor-risk group).

  5. Idiopathic Pulmonary Fibrosis: A Genetic Disease That Involves Mucociliary Dysfunction of the Peripheral Airways

    Science.gov (United States)

    Evans, Christopher M.; Fingerlin, Tasha E.; Schwarz, Marvin I.; Lynch, David; Kurche, Jonathan; Warg, Laura; Yang, Ivana V.; Schwartz, David A.

    2016-01-01

    Idiopathic pulmonary fibrosis (IPF) is an incurable complex genetic disorder that is associated with sequence changes in 7 genes (MUC5B, TERT, TERC, RTEL1, PARN, SFTPC, and SFTPA2) and with variants in at least 11 novel loci. We have previously found that 1) a common gain-of-function promoter variant in MUC5B rs35705950 is the strongest risk factor (genetic and otherwise), accounting for 30-35% of the risk of developing IPF, a disease that was previously considered idiopathic; 2) the MUC5B promoter variant can potentially be used to identify individuals with preclinical pulmonary fibrosis and is predictive of radiologic progression of preclinical pulmonary fibrosis; and 3) MUC5B may be involved in the pathogenesis of pulmonary fibrosis with MUC5B message and protein expressed in bronchiolo-alveolar epithelia of IPF and the characteristic IPF honeycomb cysts. Based on these considerations, we hypothesize that excessive production of MUC5B either enhances injury due to reduced mucociliary clearance or impedes repair consequent to disruption of normal regenerative mechanisms in the distal lung. In aggregate, these novel considerations should have broad impact, resulting in specific etiologic targets, early detection of disease, and novel biologic pathways for use in the design of future intervention, prevention, and mechanistic studies of IPF. PMID:27630174

  6. Idiopathic Pulmonary Fibrosis: The Association between the Adaptive Multiple Features Method and Fibrosis Outcomes.

    Science.gov (United States)

    Salisbury, Margaret L; Lynch, David A; van Beek, Edwin J R; Kazerooni, Ella A; Guo, Junfeng; Xia, Meng; Murray, Susan; Anstrom, Kevin J; Yow, Eric; Martinez, Fernando J; Hoffman, Eric A; Flaherty, Kevin R

    2017-04-01

    Adaptive multiple features method (AMFM) lung texture analysis software recognizes high-resolution computed tomography (HRCT) patterns. To evaluate AMFM and visual quantification of HRCT patterns and their relationship with disease progression in idiopathic pulmonary fibrosis. Patients with idiopathic pulmonary fibrosis in a clinical trial of prednisone, azathioprine, and N-acetylcysteine underwent HRCT at study start and finish. Proportion of lung occupied by ground glass, ground glass-reticular (GGR), honeycombing, emphysema, and normal lung densities were measured by AMFM and three radiologists, documenting baseline disease extent and postbaseline change. Disease progression includes composite mortality, hospitalization, and 10% FVC decline. Agreement between visual and AMFM measurements was moderate for GGR (Pearson's correlation r = 0.60, P fibrosis (as measured by GGR densities) is independently associated with elevated hazard for disease progression. Postbaseline change in AMFM-measured and visually measured GGR densities are modestly correlated with change in FVC. AMFM-measured fibrosis is an automated adjunct to existing prognostic markers and may allow for study enrichment with subjects at increased disease progression risk.

  7. An ROC study detecting ability of idiopathic pulmonary fibrosis using digital radiography

    International Nuclear Information System (INIS)

    Chung, Eun Chul; Im, Jung Gi; Han, Man Chung; Kim, Jong Hyo

    1991-01-01

    One potential advantage of the digital radiography system is its ability to enhance image quality by various types of processing. Digital unsharp masking is one of the simplest and most useful forms of enhancing processes. The efficacy of unsharp masking in radiological diagnosis has not been investigated thoroughly. To evaluate the effects of unsharp masking in film-digital chest images, 3 observers were shown 150 test radiographs. These test radiographs consisted of 50 unprocessed images (25 normals and 25 patients with idiopathic pulmonary fibrosis with honey combing) and their 100 processed images by using 450 and 15-sized masks respectively. An ROC analysis of these data suggests that unsharp masking is more effective in detecting idiopathic pulmonary fibrosis than unprocessed image (ρ < 0.05), and so it may improve diagnostic accuracy for interstitial fibrosis. In addition, the smaller mask size (15) is more effective than the larger one (mask size 45) (ρ < 0.05). By using this analytic approach, an optimal parameter in digital chest radiography may be investigated in many other forms of pulmonary disease such as pulmonary nodule or mediastinal mass

  8. An ROC study detecting ability of idiopathic pulmonary fibrosis using digital radiography

    Energy Technology Data Exchange (ETDEWEB)

    Chung, Eun Chul; Im, Jung Gi; Han, Man Chung; Kim, Jong Hyo [College of Medicine, Ewha Womens University, Seoul (Korea, Republic of)

    1991-03-15

    One potential advantage of the digital radiography system is its ability to enhance image quality by various types of processing. Digital unsharp masking is one of the simplest and most useful forms of enhancing processes. The efficacy of unsharp masking in radiological diagnosis has not been investigated thoroughly. To evaluate the effects of unsharp masking in film-digital chest images, 3 observers were shown 150 test radiographs. These test radiographs consisted of 50 unprocessed images (25 normals and 25 patients with idiopathic pulmonary fibrosis with honey combing) and their 100 processed images by using 450 and 15-sized masks respectively. An ROC analysis of these data suggests that unsharp masking is more effective in detecting idiopathic pulmonary fibrosis than unprocessed image ({rho} < 0.05), and so it may improve diagnostic accuracy for interstitial fibrosis. In addition, the smaller mask size (15) is more effective than the larger one (mask size 45) ({rho} < 0.05). By using this analytic approach, an optimal parameter in digital chest radiography may be investigated in many other forms of pulmonary disease such as pulmonary nodule or mediastinal mass.

  9. A Study of SPINK 1 Mutation and Other Clinical Correlates in Idiopathic Chronic Pancreatitis

    OpenAIRE

    Shiran Shetty; Venkatakrishnan Leelakrishnan; Krishnaveni; S Ramalingam; Seethalakshmi

    2016-01-01

    Chronic pancreatitis is labelled as idiopathic when no identifiable factors are found. The identifications of genetic mutations associated with pancreatitis have provided opportunities for identifying patients at risk for idiopathic pancreatitis. The aim of this study was to study the demographic, clinical profile and assess the prevalence of genetic mutation (SPINK 1) in idiopathic chronic pancreatitis.

  10. Differential Diagnosis of Children with Suspected Childhood Apraxia of Speech

    Science.gov (United States)

    Murray, Elizabeth; McCabe, Patricia; Heard, Robert; Ballard, Kirrie J.

    2015-01-01

    Purpose: The gold standard for diagnosing childhood apraxia of speech (CAS) is expert judgment of perceptual features. The aim of this study was to identify a set of objective measures that differentiate CAS from other speech disorders. Method: Seventy-two children (4-12 years of age) diagnosed with suspected CAS by community speech-language…

  11. Use of budesonide Turbuhaler in young children suspected of asthma

    DEFF Research Database (Denmark)

    Bisgaard, H; Pedersen, S; Nikander, K

    1994-01-01

    The question addressed in this study was the ability of young children to use a dry-powder inhaler, Turbuhaler. One hundred and sixty five children suspected of asthma, equally distributed in one year age-groups from 6 months to 8 yrs, inhaled from a Pulmicort Turbuhaler, 200 micrograms budesonide...

  12. Spatial epidemiology of suspected clinical leptospirosis in Sri Lanka.

    Science.gov (United States)

    Robertson, C; Nelson, T A; Stephen, C

    2012-04-01

    Leptospirosis is one of the most widespread zoonoses in the world. A large outbreak of suspected human leptospirosis began in Sri Lanka during 2008. This study investigated spatial variables associated with suspected leptospirosis risk during endemic and outbreak periods. Data were obtained for monthly numbers of reported cases of suspected clinical leptospirosis for 2005-2009 for all of Sri Lanka. Space-time scan statistics were combined with regression modelling to test associations during endemic and outbreak periods. The cross-correlation function was used to test association between rainfall and leptospirosis at four locations. During the endemic period (2005-2007), leptospirosis risk was positively associated with shorter average distance to rivers and with higher percentage of agriculture made up of farms <0·20 hectares. Temporal correlation analysis of suspected leptospirosis cases and rainfall revealed a 2-month lag in rainfall-case association during the baseline period. Outbreak locations in 2008 were characterized by shorter distance to rivers and higher population density. The analysis suggests the possibility of household transmission in densely populated semi-urban villages as a defining characteristic of the outbreak. The role of rainfall in the outbreak remains to be investigated, although analysis here suggests a more complex relationship than simple correlation.

  13. PMS2 Involvement in Patients Suspected of Lynch Syndrome

    NARCIS (Netherlands)

    Niessen, Renee C.; Kleibeuker, Jan H.; Westers, Helga; Jager, Paul O. J.; Rozeveld, Dennie; Bos, Krista K.; Boersma-van Ek, Wytske; Hollema, Harry; Sijmons, Rolf H.; Hofstra, Robert M. W.

    It is well-established that germline mutations in the mismatch repair genes MLH1, MSH2, and MSH6 cause Lynch syndrome. However, mutations in these three genes do not account for all Lynch syndrome (suspected) families. Recently, it was shown that germline mutations in another mismatch repair gene,

  14. Correlates and Suspected Causes of Obesity in Children

    Science.gov (United States)

    Crothers, Laura M.; Kehle, Thomas J.; Bray, Melissa A.; Theodore, Lea A.

    2009-01-01

    The correlates and suspected causes of the intractable condition obesity are complex and involve environmental and heritable, psychological and physical variables. Overall, the factors associated with and possible causes of it are not clearly understood. Although there exists some ambiguity in the research regarding the degree of happiness in…

  15. 48 CFR 3.303 - Reporting suspected antitrust violations.

    Science.gov (United States)

    2010-10-01

    ... suspect. Paragraph (c) below identifies behavior patterns that are often associated with antitrust... offers; (2) A sudden change from competitive bidding to identical bidding; (3) Simultaneous price... turn in sequence as low bidder, or so that certain competitors bid low only on some sizes of contracts...

  16. Is extended biopsy protocol justified in all patients with suspected ...

    African Journals Online (AJOL)

    Objective: To determine the significance of an extended 10-core transrectal biopsy protocol in different categories of patients with suspected prostate cancer using digital guidance. Materials and Methods: We studied 125 men who were being evaluated for prostate cancer. They all had an extended 10-core digitally guided ...

  17. A suspected case of Addison's disease in cattle.

    Science.gov (United States)

    Lambacher, Bianca; Wittek, Thomas

    2015-09-01

    A 4.75-year old Simmental cow was presented with symptoms of colic and ileus. The clinical signs and blood analysis resulted in the diagnosis of suspected primary hypoadrenocorticism (Addison's disease). Although Addison's disease has been frequently described in other domestic mammals, to our knowledge, this disease has not previously been reported in cattle.

  18. Talking heads : interviewing suspects from a cultural perspective

    NARCIS (Netherlands)

    Beune, K.

    2009-01-01

    Although the literature on the interviewing of suspects has increased over the past decade, research on the use and effectiveness of police strategies and their boundary conditions is very rare. The present dissertation aims to fill this void by identifying behaviors that appeal to and persuade

  19. 48 CFR 903.303 - Reporting suspected antitrust violations.

    Science.gov (United States)

    2010-10-01

    ... as described in FAR 3.301, and antitrust law violations as described in FAR 3.303, evidenced in bids... antitrust violations. 903.303 Section 903.303 Federal Acquisition Regulations System DEPARTMENT OF ENERGY GENERAL IMPROPER BUSINESS PRACTICES AND PERSONAL CONFLICTS OF INTEREST Reports of Suspected Antitrust...

  20. Suspected infection in afebrile patients : Are they septic?

    NARCIS (Netherlands)

    Martins, Fernanda de Souza; Guedes, Gisele Giuliane; Santos, Thiago Martins; de Carvalho-Filho, Marco A

    We prospectively evaluated afebrile patients admitted to an emergency department (ED), with suspected infection and only tachycardia or tachypnea.The white blood cell count (WBC) was obtained, and patients were considered septic if leukocyte count was >12,000 μL-1 or <4000 μL-1 or with >10% of band