       Document 0344
 DOCN  M9610344
 TI    Heterozygosity for long-chain 3-hydroxyacyl-coenzyme A dehydrogenase
       deficiency and deterioration in liver function in a newborn infant
       infected with human immunodeficiency virus.
 DT    9601
 AU    Hicks P; Bennett MJ; Squires J; Ramilo O; Department of Pediatrics,
       University of Texas Southwestern; Medical Center at Dallas 75235, USA.
 SO    J Pediatr. 1995 Oct;127(4):599-602. Unique Identifier : AIDSLINE
       MED/96006279
 AB    A child with perinatally acquired human immunodeficiency virus infection
       had rapidly progressive hepatic dysfunction, as had her older sibling
       who died. Urinary organic acid studies revealed 3-hydroxydicarboxylic
       aciduria, and cultured skin fibroblasts had reduced activity of
       3-hydroxy-coenzyme A dehydrogenase. The introduction of a low fat diet
       resulted in marked improvement in clinical status and reversal of the
       liver disease. This case illustrates the necessity of metabolic
       evaluation in patients with liver dysfunction, even when other causes of
       liver dysfunction are present.
 DE    *Acquired Immunodeficiency Syndrome  Acyl Coenzyme A/*METABOLISM
       Alanine Aminotransferase/METABOLISM  *Heterozygote  Human  HIV
       Seropositivity/COMPLICATIONS/*METABOLISM/*PHYSIOPATHOLOGY  Infant,
       Newborn  Liver/ENZYMOLOGY/*METABOLISM/*PHYSIOPATHOLOGY  Liver
       Diseases/DIAGNOSIS/ETIOLOGY/*PHYSIOPATHOLOGY  Liver Function Tests
       Mothers  JOURNAL ARTICLE

       SOURCE: National Library of Medicine.  NOTICE: This material may be
       protected by Copyright Law (Title 17, U.S.Code).

