Document Detail

Essential fatty acid deficiency mimicking porphyria cutanea tarda in a patient with chronic cholestasis.
MedLine Citation:
PMID:  2303977     Owner:  NLM     Status:  MEDLINE    
Essential fatty acid deficiency was documented in a 3-year-old boy with chronic cholestasis secondary to paucity of intrahepatic bile ducts (Alagille's syndrome). Dietary management had consisted almost exclusively of a proprietary formula with over 80% of the fat as medium-chain triglycerides. The bullous lesions involved mostly sun-exposed areas and were diagnosed initially as being compatible with acquired porphyria cutanea tarda. Improvement followed correction of the fatty acid abnormalities with a polyunsaturated fat supplement administered orally. We postulate that the association of fatty acid deficiency and abnormal vitamin E status contributed to skin damage, possibly involving photosensitizing compounds poorly cleared by the markedly cholestatic liver.
J Levy; A DeFelice; G Lepage
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of pediatric gastroenterology and nutrition     Volume:  10     ISSN:  0277-2116     ISO Abbreviation:  J. Pediatr. Gastroenterol. Nutr.     Publication Date:  1990 Feb 
Date Detail:
Created Date:  1990-03-29     Completed Date:  1990-03-29     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  8211545     Medline TA:  J Pediatr Gastroenterol Nutr     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  242-5     Citation Subset:  IM    
Department of Pediatrics, Columbia University College of Physicians and Surgeons, New York, New York.
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MeSH Terms
Child, Preschool
Cholestasis / complications*,  diagnosis,  drug therapy
Chronic Disease
Diagnostic Errors
Fatty Acids, Essential / deficiency*,  therapeutic use
Porphyrias / diagnosis*
Skin Diseases / diagnosis*
Vitamin E / therapeutic use
Vitamin E Deficiency / complications*,  drug therapy
Reg. No./Substance:
0/Fatty Acids, Essential; 1406-18-4/Vitamin E

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

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