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Review
. 1999 Mar;134(3):352-4.
doi: 10.1016/s0022-3476(99)70463-4.

Respiratory chain complex III [correction of complex] in deficiency with pruritus: a novel vitamin responsive clinical feature

D Mowat  1 , D M Kirby, K R Kamath, A Kan, D R Thorburn, J Christodoulou
Affiliations
Review

Respiratory chain complex III [correction of complex] in deficiency with pruritus: a novel vitamin responsive clinical feature

D Mowat et al. J Pediatr. 1999 Mar.

Erratum in

  • J Pediatr 1999 Sep;135(3):402

Abstract

We report a child with an isolated complex III respiratory chain deficiency and global developmental delay who had severe pruritus with elevated plasma bile acid levels. A liver biopsy showed micronodular cirrhosis, and enzymologic evaluation demonstrated an isolated complex III deficiency in both liver and muscle. His pruritus improved and serum bile acid levels decreased after treatment with menadione and vitamin C.

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