Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:dateCreated
1995-12-14
pubmed:abstractText
Secondary porphyrinopathias were investigated in hereditary hyperbilirubinemias of the types Dubin-Johnson syndrome (DJS), Rotor's syndrome (RS), Gilbert's syndrome (GS) and compared with the findings in alcohol-induced cholestasis. The determination of urinary coproporphyrin excretion including its isomer I and III relation allows to diagnose and differentiate DJS from the other two forms of hereditary hyperbilirubinemias. An isomer I proportion of > 80% (normal < 32%) in association with a normal coproporphyrin excretion is pathognomonic for DJS. An increased coproporphyrinuria with about 70% isomer I is found in RS; a normal urinary coproporphyrin excretion combined with an isomer I increase of about 50-70% indicates the frequent GS. Alcohol-related cholestasis reveals a distinct pathologic coproporphyrinuria and elevated isomer I proportions between 37-67%. Due to the overlapping of isomer alterations in DJS-gene carriers, patients with GS and patients with exogenous toxically-induced cholestasis, additional diagnostic criteria are required to clarify the porphyrinopathia in its clinical context.
pubmed:language
ger
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Feb
pubmed:issn
0044-2771
pubmed:author
pubmed:issnType
Print
pubmed:volume
31 Suppl 2
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
111-3
pubmed:dateRevised
2009-11-11
pubmed:meshHeading
pubmed:year
1993
pubmed:articleTitle
[Diagnostic porphyrinopathies in hereditary hyperbilirubinemia].
pubmed:affiliation
Abteilung für Gastroenterologie und Stoffwechsel, Universitätsklinik Marburg.
pubmed:publicationType
Journal Article, English Abstract, Research Support, Non-U.S. Gov't