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Nephrol Dial Transplant (2000) 15: 1232-1234
© 2000 European Renal Association-European Dialysis and Transplant Association

Case Reports

Very-long-chain acyl-coenzyme A dehydrogenase deficiency— a new cause of myoglobinuric acute renal failure

Andrew P. Cairns1, Paula M. O'Donoghue1, Victor H. Patterson2 and J. Henry Brown1,

1 The Renal Unit, Antrim Area Hospital, and 2 The Department of Neurology, Belfast City Hospital, Northern Ireland

Keywords: acute renal failure; acylcarnitines; myoglobinuria; rhabdomyolysis; very-long-chain acyl coenzyme A dehydrogenase deficiency



   Introduction
 
Skeletal muscle uses two principal sources of energy: fatty acids and glucose. Fatty acids are particularly important when fasting, or during prolonged exercise. Very-long-chain acyl-CoA dehydrogenase (VLCAD) is an enzyme catalysing the dehydrogenation of long-chain fatty acids in the first step of mitochondrial fatty acid oxidation [1]. VLCAD deficiency normally presents in infancy or early childhood with cardiomyopathy or episodes of hypoglycaemia [1].

We describe the case of a man with VLCAD deficiency who presented for the first time at the age of 41 with muscle pains, dark urine, and renal failure. We believe this to be only the second published case of VLCAD deficiency presenting in adult life as acute renal failure requiring dialysis.



   Case
 
A 41-year-old man was referred complaining of . . . [Full Text of this Article]



   Discussion
 


   Acknowledgments
 


   Notes
 


   References
 

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