Linked Life Data

6614036

Source:http://linkedlifedata.com/resource/pubmed/id/6614036

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
3
pubmed:dateCreated
1983-10-8
pubmed:abstractText
Tangier disease is a rare, autosomal recessive condition characterized by cholesterol ester deposition in reticuloendothelial cells, abnormal chylomicron remnants, decreased low-density lipoprotein levels, and a marked deficiency of high-density lipoproteins. Apolipoprotein A-I, a major protein constituent of chylomicrons and high-density lipoproteins, has been shown to be structurally and metabolically abnormal in this disease (apolipoprotein A-ITangier). A 63-year-old Tangier homozygous man is described, who underwent splenectomy because of thrombocytopenia and splenomegaly. Subsequently, a large orange mass developed at the base of the mesentery, with several smaller omental masses and thickening of the entire omentum due to infiltration with lipid-laden macrophages. Splenectomy appears to predispose to such deposition, since such masses have not been observed in other Tangier homozygotes. The spleen appears to play a significant role in the removal of abnormal lipoproteins in Tangier homozygotes; therefore, splenectomy may be contraindicated in Tangier disease.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
AIM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Sep
pubmed:issn
0002-9343
pubmed:author
pubmed:issnType
Print
pubmed:volume
75
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
521-6
pubmed:dateRevised
2006-11-15
pubmed:meshHeading
pubmed:year
1983
pubmed:articleTitle
Massive omental reticuloendothelial cell lipid uptake in Tangier disease after splenectomy.
pubmed:publicationType
Journal Article, Case Reports