[Clinical features and X-ray manifestations of mucopolysaccharidosis-II-B].

Abstract

Mucopolysaccharidosis-II (MPS-II) and MPS-I are both characterized by gargoylist dwarfism. MPS-II is of X-linked recessive heredity. Five cases of MPS-II-B in our series were followed up for ten years. Corneal opacity and dysmnesia were not found in all of them. Although MPS-II possessed many radiologic similarities to MPS-I, the former presented certain characteristic features such as butterfish-like vertebra, and small triangular malformation of carpal bones and tarsal scaphoid etc.