Heterozygosity for long-chain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency and deterioration in liver function in a newborn infant infected with human immunodeficiency virus

Hicks, Patricia J.; Bennett, Michael J.; Squires, Janet; Ramilo, Octavio

doi:10.1016/s0022-3476(95)70122-2

Cited by 4 publications

(1 citation statement)

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“…This infant had concurrent HIV infection, so that it was difficult to define the etiology of the liver disease, although the introduction of a low-fat diet resulted in a marked improvement in the child's clinical status. However, the molecular basis for LCHAD deficiency was not defined (Hicks et al, 1995). The homozygous fetus is unlikely to produce intermediates of fatty acid oxidation because glucose is the main energy source for the fetus, and fetal fatty acid oxidation is low (Herrera and Amusquivar, 2000).…”

Section: Pregnancy and Fatty Acid Oxidation Defectsmentioning

confidence: 99%

Long-Chain L-3-Hydroxyacyl-Coenzyme A Dehydrogenase Deficiency: A Molecular and Biochemical Review

Rakheja

Bennett

Rogers

2002

Laboratory Investigation

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SUMMARY:Since the first report of long-chain L-3-hydroxyacyl-coenzyme A dehydrogenase deficiency a little more than a decade ago, its phenotypic and genotypic heterogeneity in individuals homozygous for the enzyme defect has become more and more evident. Even more interesting is its association with pregnancy-specific disorders, including preeclampsia, HELLP syndrome (hemolysis, elevated liver enzymes, low platelets), hyperemesis gravidarum, acute fatty liver of pregnancy, and maternal floor infarct of the placenta. In this review we discuss the biochemical and molecular basis, clinical features, diagnosis, and management of long-chain L-3-hydroxyacyl-coenzyme A dehydrogenase deficiency. (Lab Invest 2002, 82:815-824).

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Section: Pregnancy and Fatty Acid Oxidation Defectsmentioning

confidence: 99%