Document Detail


Liver lipids in Letterer-Siwe disease.
MedLine Citation:
PMID:  7449795     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
A case of Letterer-Siwe disease in a thirteen-month-old girl with markedly swollen gums, an enlarged liver, and seborrhoic skin eruptions is reported. She died from pyopneumothorax in spite of treatment with prednisolone and vinblastine. Analysis of hepatic lipids showed that there was remarkable increase in free fatty acid, which is not seen in normal human liver. Cholesterol ester was also accumulated in the liver tissue. However, the concentrations of phospholipids and glycerides were low and lysophospholipid was found. It is assumed that there was increased intracellular lipolysis in the proliferating reticuloendothelial cells.
Authors:
H Miyata; R Murata; K Arita; K Yoshioka; T Yamamoto; H Suzuki
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  European journal of pediatrics     Volume:  135     ISSN:  0340-6199     ISO Abbreviation:  Eur. J. Pediatr.     Publication Date:  1980 Oct 
Date Detail:
Created Date:  1981-03-24     Completed Date:  1981-03-24     Revised Date:  2005-11-17    
Medline Journal Info:
Nlm Unique ID:  7603873     Medline TA:  Eur J Pediatr     Country:  GERMANY, WEST    
Other Details:
Languages:  eng     Pagination:  91-5     Citation Subset:  IM    
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MeSH Terms
Descriptor/Qualifier:
Cholesterol Esters / analysis
Female
Glycerides / analysis
Histiocytosis, Langerhans-Cell / pathology*
Humans
Infant
Lipids / analysis*
Liver / analysis*
Phospholipids / analysis
Skin / pathology
Chemical
Reg. No./Substance:
0/Cholesterol Esters; 0/Glycerides; 0/Lipids; 0/Phospholipids

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


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