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Pediatrics Metabolic disorders 3cdcb8a3

Only male are affected in

A
Scheie's syndrome
B
Hunter's syndrome
C
Hurler's syndrome
D
Gaucher's disease
High-Yield Explanation
Hunter's syndrome belongs to Type II mucopolysaccharidoses in which mucopolysaccharides accumulate in the lysosomes causing disorganization of the cell structure & function.This syndrome is characterised by mild mental retardation;marked skeletal changes like thickening of the skull,marked deformity of sella tursica,broad spatula like ribs,beak shaped veebrae(around L1 veebra) and proximal tapering of metacarpals-these abnormalities are referred to as dysostosis multiplex;coarse facies & hepatosplenomegaly. Reference:Essential pediatrics-Ghai,8th edition,page no: 660,661.

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