نتایج جستجو برای: npc2

تعداد نتایج: 199  

2010
Misbaudeen Abdul-Hammed Bernadette Breiden Matthew A. Adebayo Jonathan O. Babalola Günter Schwarzmann Konrad Sandhoff

2016
Béranger Lueza Federico Rotolo Julia Bonastre Jean-Pierre Pignon Stefan Michiels

BACKGROUND The difference in restricted mean survival time ([Formula: see text]), the area between two survival curves up to time horizon [Formula: see text], is often used in cost-effectiveness analyses to estimate the treatment effect in randomized controlled trials. A challenge in individual patient data (IPD) meta-analyses is to account for the trial effect. We aimed at comparing different ...

2014
Helena Jahnova Lenka Dvorakova Hana Vlaskova Helena Hulkova Helena Poupetova Martin Hrebicek Pavel Jesina

BACKGROUND Niemann-Pick disease type C (NPC) is a rare, fatal neurovisceral disorder with autosomal recessive inheritance, and featuring striking clinical variability dependent on the age at onset of neurological symptoms. We report data from a large cohort of 56 Czech patients with NPC diagnosed over a period of 37 years. METHODS An observational, retrospective analysis of historic and curre...

Journal: :Brain : a journal of neurology 2007
Mathieu Sévin Gaëtan Lesca Nicole Baumann Gilles Millat Olivier Lyon-Caen Marie T Vanier Frédéric Sedel

Niemann-Pick disease type C (NPC) is a fatal neurovisceral lipid storage disease of autosomal inheritance resulting from mutations in either the NPC1 (95% of families) or NPC2 gene. The encoded proteins appear to be involved in lysosomal/late endosomal transport of cholesterol, glycolipids and other molecules but their exact function is still unknown. The clinical spectrum of the disease ranges...

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