Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
1
pubmed:dateCreated
1986-1-15
pubmed:abstractText
Protoporphyria was diagnosed in a 56-yr-old man based upon a typical clinical and family history, marked increases in erythrocyte and fecal protoporphyrin concentrations, and a marked decrease (21% of normal) in activity of hepatic heme synthase. Routine tests of liver function and histology were normal, except for a slight increase in bromsulphalein retention (9% at 45 min). Liver chemistries remained normal for 8 more years, but deteriorated rapidly when the patient was 63 yr old, with cholestasis precipitated by injury due to excess intake of ethanol. This, in turn, led to a defect in hepatic protoporphyrin excretion and to further worsening of liver injury due to porphyrin deposition. Our patient represents the 21st and oldest patient thus far reported to have died of liver failure complicating protoporphyria.
pubmed:grant
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
AIM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Jan
pubmed:issn
0016-5085
pubmed:author
pubmed:issnType
Print
pubmed:volume
90
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
191-201
pubmed:dateRevised
2007-11-14
pubmed:meshHeading
pubmed:year
1986
pubmed:articleTitle
Fatal liver failure in protoporphyria. Synergism between ethanol excess and the genetic defect.
pubmed:publicationType
Journal Article, Research Support, U.S. Gov't, P.H.S., Research Support, U.S. Gov't, Non-P.H.S., Case Reports