HETEROZYGOSITY FOR LONG-CHAIN 3-HYDROXYACYL-COENZYME-A DEHYDROGENASE-DEFICIENCY AND DETERIORATION IN LIVER-FUNCTION IN A NEWBORN-INFANT INFECTED WITH HUMAN-IMMUNODEFICIENCY-VIRUS

A child with perinatally acquired human immunodeficiency virus infecti on had rapidly progressive hepatic dysfunction, as had her older sibli ng who died, Urinary organic acid studies revealed 3-hydroxydicarboxyl ic aciduria, and cultured skin fibroblasts had reduced activity of 3-h ydroxyl-coenzyme A dehydrogenase. The introduction of a low fat diet r esulted in marked improvement in clinical status and reversal of the l iver disease, This case illustrates the necessity of metabolic evaluat ion in patients with liver dysfunction, even when other causes of live r dysfunction are present.