Relevance of urinary coproporphyrin isomers in hereditary hyperbilirubinemias.
Clin Biochem
; 22(3): 221-2, 1989 Jun.
Article
en En
| MEDLINE
| ID: mdl-2736773
ABSTRACT
Porphyrin metabolism is impaired in Dubin-Johnson syndrome (DJS), Rotor's syndrome (RS), and Gilbert's syndrome (GS). Urinary coproporphyrin (CP) isomer I is increased in these hereditary hyperbilirubinemias to different degrees in DJS to 85%, in RS to 70%, and in GS to 50% in the homozygous state (p less than 0.001 compared to controls with isomer I of 27%). Intermediate isomer proportions were found in heterozygote carriers of DJS. An overlapping distribution of the isomer I/III ratio is observed in DJS and RS carriers, homozygous subjects with GS, and individuals suffering from alcohol-related intrahepatic cholestasis. The diagnosis of DJS and RS can be based mainly on porphyrin analysis, but the detection of carriers (heterozygotes) requires additional criteria to distinguish them from patients with intrahepatic cholestasis of a different etiology.
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Banco de datos:
MEDLINE
Asunto principal:
Porfirinas
/
Coproporfirinas
/
Hiperbilirrubinemia Hereditaria
Tipo de estudio:
Diagnostic_studies
Límite:
Humans
Idioma:
En
Año:
1989
Tipo del documento:
Article