Document Detail


Acute fatty liver of pregnancy and neonatal long-chain 3-hydroxyacyl-coenzyme A dehydrogenase (LCHAD) deficiency.
MedLine Citation:
PMID:  18408953     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Here we report a 7-month-old girl with long-chain 3-hydroxyacyl-coenzyme A dehydrogenase (LCHAD) deficiency with hypoketotic hypoglycemia; the mother had a history of acute fatty liver in a previous pregnancy leading to fetal death at 34 weeks of gestation. The misense mutation 1528G > C was detected in both alleles in the proband and in one allele in both parents. We emphasize that screening for fatty acid oxidation disorders and specifically LCHAD deficiency should be performed in newborns from mothers with hepatic complications during pregnancy such as acute fatty liver of pregnancy or severe or recurrent HELLP syndrome.
Authors:
Carolina Gutiérrez Junquera; Elena Balmaseda; Esther Gil; Andrés Martínez; Moisés Sorli; Isabel Cuartero; Begoña Merinero; Magdalena Ugarte
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Publication Detail:
Type:  Case Reports; Journal Article     Date:  2008-04-12
Journal Detail:
Title:  European journal of pediatrics     Volume:  168     ISSN:  1432-1076     ISO Abbreviation:  Eur. J. Pediatr.     Publication Date:  2009 Jan 
Date Detail:
Created Date:  2008-11-26     Completed Date:  2009-04-28     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7603873     Medline TA:  Eur J Pediatr     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  103-6     Citation Subset:  IM    
Affiliation:
Servicio de Pediatría, Complejo Hospitalario Universitario de Albacete, Universidad de Castilla La-Mancha, Albacete, Spain. cgutierrezj@telefonica.net
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MeSH Terms
Descriptor/Qualifier:
3-Hydroxyacyl CoA Dehydrogenases / deficiency*,  genetics*
Acute Disease
Alleles
Blood Transfusion
Cardiomyopathies / ultrasonography
Fatty Liver / diagnosis*
Female
Gene Expression / genetics
Humans
Infant
Metabolism, Inborn Errors / diagnosis*,  genetics,  therapy
Pregnancy
Pregnancy Complications
Chemical
Reg. No./Substance:
EC 1.1.1.35/3-Hydroxyacyl CoA Dehydrogenases

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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