Orthopedic Pathology in Children with Mucopolysaccharidosis Type I
Mucopolysaccharidosis type I is inherited in an autosomal recessive manner and results from the defective activity of the enzyme alpha-L-iduronidase, which leads to the accumulation of glycosaminoglycans (mainly heparan and dermatan sulfate) in the lysosomes and further multiple organ dysfunction. T...
Main Authors: | , , , , , , , , , |
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Format: | Article |
Language: | English |
Published: |
"Paediatrician" Publishers LLC
2016-12-01
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Series: | Вопросы современной педиатрии |
Subjects: | |
Online Access: | https://vsp.spr-journal.ru/jour/article/view/1693 |