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Thursday, February 3, 2011

KINKY HAIR (MENKES) DISEASE

Kinky hair disease, a sex- linked recessive disorder of copper metabolism, is characterized by poor weight gain, proneness to infection and , latter, hair becoming sparse and brittle, and myoclonic seizures.


X-ray shows scurvy- like picture in ling bones. Serum copper and ceruplasmin levels are reduced. Despite parenteral copper therapy, gross cerebral and arterial changes prove fatal in infancy per se.

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