Combined defects of muscle phosphofructokinase and AMP deaminase in a child with myoglobinuria.

Bruno, C; Minetti, C; Shanske, S; Morreale, G; Bado, M; Cordone, G; DiMauro, S · Neurology · 1998

case_report · Level V

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Abstract

A 14-year-old boy with exercise-related myalgia and cramps had several episodes of myoglobinuria since early childhood. An episode at 2 years of age caused acute renal failure. Histochemical and biochemical analysis of muscle showed a combined defect of phosphofructokinase (PFK) and adenosine monophosphate (AMP) deaminase. DNA analysis showed that the patient was homozygous for a G-to-C substitution at codon 39 of the PFK gene (previously described in an Italian patient) and for the common mutation found in AMP deaminase deficiency.

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