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February 24, 1999

HELLP Syndrome

Author Affiliations

Margaret A.WinkerMD, Deputy EditorIndividualAuthorPhil B.FontanarosaMD, Interim CoeditorIndividualAuthor

JAMA. 1999;281(8):703-705. doi:10-1001/pubs.JAMA-ISSN-0098-7484-281-8-jbk0224

To the Editor: In the Grand Rounds on the HELLP syndrome, Dr Stone1 discusses the risks of the HELLP syndrome to the infant both before and after birth. However, he missed the opportunity to remind readers that a specific inborn error of metabolism of fatty acid degradation can occur in the fetus and may be associated with the HELLP syndrome in the mother. The defect is that of autosomal recessive long-chain 3-hydroxyacyl coenzyme A dehydrogenase deficiency (LCHAD). Neither the frequency of this defect nor the true incidence of the association between the 2 disorders is known. The HELLP syndrome is common, whereas LCHAD deficiency appears to be rare. Wilcken et al2 reported 6 such cases, and others have been reported since.3,4 The mothers are not affected with the biochemical defect but are, of course, obligate carriers of the LCHAD deficiency and their carnitine status is not known.

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