Document Detail


Pulmonary haemorrhage and cardiac dysfunction in a neonate with medium-chain acyl-CoA dehydrogenase (MCAD) deficiency.
MedLine Citation:
PMID:  15858970     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
We report on a favourable case of MCAD deficiency (homozygous 985A > G) that presented as lethargy, poor feeding, pulmonary haemorrhage and cardiac arrest without hypoglycaemia. The cessation of intralipid and the commencement of carnitine supplementation were associated with a rapid clinical improvement. CONCLUSION: Mild carnitine depletion and secondary impairment of long-chain fatty acid metabolism may have contributed to post-asphyxial myocardial dysfunction and ventricular arrhythmias. Metabolic disorders must be kept in mind as a differential diagnosis in acutely ill infants, but it must also be emphasized that carnitine therapy is not uniformly effective in all MCAD patients.
Authors:
K Maclean; V S Rasiah; E P E Kirk; K Carpenter; S Cooper; K Lui; J Oei
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Acta paediatrica (Oslo, Norway : 1992)     Volume:  94     ISSN:  0803-5253     ISO Abbreviation:  Acta Paediatr.     Publication Date:  2005 Jan 
Date Detail:
Created Date:  2005-04-29     Completed Date:  2005-06-07     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9205968     Medline TA:  Acta Paediatr     Country:  Norway    
Other Details:
Languages:  eng     Pagination:  114-6     Citation Subset:  IM    
Affiliation:
Department of Medical Genetics, Sydney Children's Hospital, Sydney, Australia.
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MeSH Terms
Descriptor/Qualifier:
Acyl-CoA Dehydrogenase / deficiency*
Heart Arrest / etiology*
Hemorrhage / etiology*
Humans
Infant, Newborn
Lung Diseases / etiology*
Male
Chemical
Reg. No./Substance:
EC 1.3.99.3/Acyl-CoA Dehydrogenase

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


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