نتایج جستجو برای: mucopolysaccharidosis

تعداد نتایج: 2370  

2014
Paulina Krawiec Elżbieta Pac-Kożuchowska Beata Mełges Agnieszka Mroczkowska-Juchkiewicz Stanisław Skomra Agnieszka Pawłowska-Kamieniak Katarzyna Kominek

UNLABELLED ᅟ: Mucopolysaccharidosis type III (MPS III; Sanfilippo syndrome) is a metabolic disorder characterized by the deficiency of a lysosomal enzyme catalyzing the catabolic pathway of heparan sulphate. MPS III presents with progressive mental deterioration, speech delay and behavioural problems with subtle somatic features, which can often lead to misdiagnosis with idiopathic developmenta...

2015
Elizabeth Ashby Deborah Eastwood

PURPOSE To describe knee alignment in children of different ages with severe mucopolysaccharidosis (MPS) I and II and the outcome of treatment with guided growth in a patient subgroup. METHODS This is a retrospective observational study of 58 knees in 29 children with severe MPS I and II. Long-leg standing radiographs were evaluated to determine mechanical axis deviation, mechanical lateral d...

Journal: :Orphanet Journal of Rare Diseases 2008
David Moore Martin J Connock Ed Wraith Christine Lavery

BACKGROUND Mucopolysaccharidosis type I (MPS I) is a rare lysosomal storage disease subdivided into three phenotypes of increasing severity: Scheie, Hurler-Scheie and Hurler. To gauge the effectiveness of treatments and to determine the load likely to fall on health-care systems, it is necessary to understand the prevalence and natural progression of the disease especially with regard to life-e...

Journal: :Molecular Genetics and Metabolism Reports 2016

Journal: :Archives of Disease in Childhood 1976

Journal: :International Journal of Endocrinology and Metabolic Disorders 2015

Journal: :Journal of Inherited Metabolic Disease 2020

Journal: :Journal of Clinical Investigation 1993

Journal: :Journal of Orthopaedic Surgery and Research 2021

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