Search results for "hunt"
showing 10 items of 286 documents
I06 Dysphagia In Huntington`s Disease: A Multicenter Study
2014
Background Dysphagia is a common complication of neurogenerative disorders, and a leading cause of death due to aspiration pneumonia. Little is known about the frequency and the characteristics of dysphagia in HD. Well-balanced strategies for treatment and prevention of dysphagia in HD are lacking. Objetives To determine the prevalence of dysphagia in HD. Methods National, multicenter, observational, cross-sectional Study (Spanish EHDN). Dysphagia was assessed using the questionnaire EAT-10 (eating assessment tool), caregiver burden using the SumaCare, functional capacity using the TFC score, disease severity using the Unified HD Rating Scale (UHDRS), psyquiatric status (PBA-s), nutritional…
Orthopedic manifestations in patients with mucopolysaccharidosis type II (Hunter syndrome) enrolled in the Hunter Outcome Survey.
2010
Mucopolysaccharidosis type II (MPS II or Hunter syndrome) is a rare, inherited disorder caused by deficiency of the lysosomal enzyme iduronate-2-sulfatase. As a result of this deficiency, glycosaminoglycans accumulate in lysosomes in many tissues, leading to progressive multisystemic disease. The cardiopulmonary and neurological problems associated with MPS II have received considerable attention. Orthopedic manifestations are common but not as well characterized. This study aimed to characterize the prevalence and severity of orthopedic manifestations of MPS II and to determine the relationship of these signs and symptoms with cardiovascular, pulmonary and central nervous system involvemen…
Right posterior diaphragmatic hernia (Bochdalek) with liver involvement and alteration of hepatic outflow in adult: a case report
2016
Introduction Posterior right diaphragmatic hernia is rare in newborn patients but when present, is accompanied by high mortality. Pulmonary hypoplasia seems to be the main cause of death but the presence of liver involvement remains one of the reasons for poor prognosis even when intrauterine surgery is performed. Case Description In this article, we will present a rare case that was diagnosed by chance in a 65-year old adult presenting with an adenocarcinoma of the rectosigmoid junction and a right Bochdalek hernia with liver herniation and modification of the hepatic vein outflow with a natural right to left shunt. Discussion Diaphragmatic repair was performed on the patient with a mash a…
Urolithiasis Following Portacaval Shunt in Rats
1987
A reliable technique for a portacaval anastomosis (PCA) in rats was first published by Lee and Fisher 1961. Since then the biological and biochemical consequences have been studied extensively in numerous investigations. The occurrence of a urolithiasis following PCA was first reported by Herz et al. 1972. Disturbances of uric acid metabolism that were caused by the shunt were thought to be responsible. They should lead to an increased production of endogenous uric acid and thus to hyperuricosuria. Investigations that were published since then mainly focused on chemical stone analysis (Bichler et al. 1974; Rasenack et al. 1977; Wallace et al. 1984).
Imaging of Ventriculoperitoneal Shunt Complications: Comparison of Whole Body Low-Dose Computed Tomography and Radiographic Shunt Series.
2016
OBJECTIVE To determine diagnostic value and radiation exposure of low-dose computed tomography (LD-CT) compared to radiographic shunt series (SS) for the detection of ventriculoperitoneal (VP) shunt complications. METHODS Fourteen VP shunts were implanted in 7 swine cadavers. Mechanical complications were induced in 50% of VP shunts. Low-dose CT (80 kVp, 10 mAs, Pitch = 1.5) and SS were acquired. Dose area product (DAP) and effective doses for SS and LD-CT were collected. Scoring of diagnostic confidence and blinded readings of SS and CT data were performed. RESULTS The sensitivity of LD-CT was high (0.97; 95% confidence interval, 0.91-1.00) with excellent interobserver agreement (κ = 0.88)…
Video-assisted two-stage basilic vein transposition for creation of brachio-basilic arteriovenous fistulae
2012
We report our experience in a mixed minimally-in- vasive technique for the two-stage transposition of basilic vein on a small series of eight patients. The operative tech- nique consisted of a modified endoscopic (1) approach for the two-stage transposition (2). The intervention was performed under Brachial Plexus Block (3).
The use of a new kind of low profile retractor for arteriovenous fistula procedure simplifies and speeds up the intervention
2009
Purpose The aim of this study was to demonstrate the effectiveness of a new kind of disposable surgical retractor in arteriovenous fistula (AVF) procedures in order to achieve an easier, faster and safer surgical intervention. Methods Between January and June 2008, 22 AVF procedures were performed using the 3PAWS ReeTrakttrade; (Insightra Inc. - Irvine, Ca., USA) a self-retaining, low profile retractor. An equivalent patient sample, in which an AVF was performed using conventional retraction devices, was considered for comparison of the intra- and post-operative results. Results In all of the 22 AVF procedures performed, the ReeTrakt™ system has simplified the performance of the surgical te…
Development of Bladder Carcinoma Following Portacaval Shunt in Rats
1987
Portacaval anastomosis (PCA) was first introduced by Eck (1877) and was investigated in a number of different animals. Lee et al. (1961; 1974) published a reliable method with low mortality in rats. The occurrence of urolithiasis in rats following portacaval anastomosis happened to be noticed by chance. It was published by Herz et al. (1972a, 1972b, 1973), who defined the portacaval shunt rat as an experimental model. Heine et al. (1979) described dysplastic and neoplastic changes of the bladder mucosa following portacaval anastomosis. His findings were confirmed by Duy et al. (1981); Grun et al. (1982), and Dubuisson et al. (1984). However, Wallace et al. (1984) found papillary hyperplasia…
Long-term, open-labeled extension study of idursulfase in the treatment of Hunter syndrome.
2011
Purpose: This study evaluated the safety and effectiveness of long-term enzyme replacement therapy with idursulfase (recombinant human iduronate-2-sulfatase) in patients with Hunter syndrome. Methods: All 94 patients who completed a 53-week double-blinded study of idursulfase enrolled in this open-labeled extension study and received intravenous idursulfase at a dose of 0.5 mg/kg weekly for 2 years, and clinical outcomes and safety were assessed. Results: No change in percent predicted forced vital capacity was seen, but absolute forced vital capacity demonstrated sustained improvement and was increased 25.1% at the end of the study. Statistically significant increases in 6-minute walking t…