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rdf:type | |
lifeskim:mentions | |
pubmed:dateCreated |
1995-12-14
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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.
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pubmed:language |
ger
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pubmed:journal | |
pubmed:citationSubset |
IM
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pubmed:chemical | |
pubmed:status |
MEDLINE
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pubmed:month |
Feb
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pubmed:issn |
0044-2771
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pubmed:author | |
pubmed:issnType |
Print
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pubmed:volume |
31 Suppl 2
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
111-3
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pubmed:dateRevised |
2009-11-11
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pubmed:meshHeading |
pubmed-meshheading:7483689-Adolescent,
pubmed-meshheading:7483689-Adult,
pubmed-meshheading:7483689-Child,
pubmed-meshheading:7483689-Child, Preschool,
pubmed-meshheading:7483689-Coproporphyrins,
pubmed-meshheading:7483689-Female,
pubmed-meshheading:7483689-Heterozygote Detection,
pubmed-meshheading:7483689-Humans,
pubmed-meshheading:7483689-Hyperbilirubinemia, Hereditary,
pubmed-meshheading:7483689-Male,
pubmed-meshheading:7483689-Pedigree,
pubmed-meshheading:7483689-Phenotype,
pubmed-meshheading:7483689-Porphyrias,
pubmed-meshheading:7483689-Syndrome
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pubmed:year |
1993
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pubmed:articleTitle |
[Diagnostic porphyrinopathies in hereditary hyperbilirubinemia].
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pubmed:affiliation |
Abteilung für Gastroenterologie und Stoffwechsel, Universitätsklinik Marburg.
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pubmed:publicationType |
Journal Article,
English Abstract,
Research Support, Non-U.S. Gov't
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