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Pediatrics Metabolic Disorders 61de955d

Abnormalities of copper metabolism is implicated in the pathogenesis of all except?

A
Cardiomyopathy
B
Indian childhood cirrhosis
C
Menkes Disease
D
Wilson Disease
High-Yield Explanation
Ans. A. CardiomyopathyCopper is a constituent of several Metalloenzymes required for oxidative metabolism. Ceruloplasmin, a glycoprotein that contains eight copper atoms per molecule, accounts for 95% of the ion in blood. The richest sources are meats, liver, seafood, nuts and seeds. Primary deficiency is infrequent. Secondary deficiency may develop in malabsorption syndromes, liver disease, peritoneal dialysis and other conditions causing excessive copper loss. Features include microcytic, hypochromic anemia unresponsive to iron therapy, neutropenia and osteoporosis. Skeletal lesions include periosteal elevation, changes in metaphyses of long bones, and rarely Sub metaphyseal fractures., flaring of the anterior ribs and spontaneous fractures of the ribs.

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