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Medicine Disorder of Metabolism & Connective Tissue 08e1a6ed

Most common cause of death in menke's disease is-

A
Cardiac rupture
B
Diaphragmatic paralysis
C
Laryngospasm
D
Cyano-methemoglobinemia
High-Yield Explanation
Ans. is 'a' i.e., Cardiac rupture o Children with this disease often die within 5 years because of dissecting aneurysms or cardiac rupture.Menkes kinky hair syndromeo An X-1 inked metabolic disturbance of copper metabolism characterized by# Mental retardation, hypocupremia, and decreased circulating ceruloplasmin.o Caused by mutations in the copper-transporting A TP7A gene.o Children with this disease often die within 5 years because of dissecting aneurysms or cardiac rupture.

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