Linked Life Data

6198903

Source:http://linkedlifedata.com/resource/pubmed/id/6198903

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
2
pubmed:dateCreated
1984-3-16
pubmed:abstractText
Cholesterol ester storage disease is a rare, inherited metabolic disorder of lipid associated with acid cholesteryl ester hydrolase deficiency. Thus far, 15 cases have been reported in the world literature. Reported here is the autopsy study of the oldest patient with this disease. The lipid storage occurred in the forms of birefringent needle-shaped crystals limited to hepatocytes and non-birefringent autofluorescent granules accumulated within the foam cells of the hepatic portal triads, duodenum, and ovaries. The cholesterol content of the liver was 16 times normal, primarily caused by increased cholesterol ester. Only trace cholesteryl ester hydrolase activity was demonstrated in the liver. An additional unique finding in our case was the presence of mesenteric lipodystrophy. Whether these two rare disorders observed in our patient represent unrelated conditions or have an etiologic association remains unknown.
pubmed:grant
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
AIM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Feb
pubmed:issn
0002-9173
pubmed:author
pubmed:issnType
Print
pubmed:volume
81
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
263-9
pubmed:dateRevised
2007-11-15
pubmed:meshHeading
pubmed:year
1984
pubmed:articleTitle
Cholesterol ester storage disease and mesenteric lipodystrophy.
pubmed:publicationType
Journal Article, Research Support, U.S. Gov't, P.H.S., Case Reports