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[Hepatitis C, hemochromatosis and porphyria cutanea tarda].
Dtsch Med Wochenschr. 2006 Mar 31; 131(13):691-5.DM

Abstract

Porphyria cutanea tarda (PCT) is characterized by decreased activity of the enzyme uroporphyrinogen decarboxylase (URO-D) and the accumulation of uro- and heptaporphyrins in the liver. Apart from increased alcohol exposure and certain drugs, PCT is associated with antibodies to the hepatitis C virus (HCV), with its prevalence increasing from Northern (8-10%) to Southern Europe (71 to 91%). Chronic HCV-infection is thus considered to be a major trigger for PCT and PCT is said to be an important extrahepatic manifestation of HCV-infection in predisposed individuals. Iron overload is common in PCT. Iron is an inhibitory co-factor of URO-D activity in hepatocytes. Accordingly, in support of the critical role of iron, the clinical efficacy of iron removal is coupled to an improvement of hepatic URO-D activities. Up to two thirds of Saxon patients with PCT carry the classical hemochromatosis (HFE) mutations (C282Y and/or H63D). HFE genotyping can help to further classify patients with PCT and associated hemochromatosis. Simple or compound heterozygosity of HFE mutations does not affect the therapeutic response to chloroquine in PCT. Since Patients carrying homozygous mutations (C282Y/C282Y) with hemochromatosis and PCT do not respond to chloroquine, phlebotomy should be first-line treatment to remove toxic iron.

Authors+Show Affiliations

Sächsisches Porphyriezentrum, Medizinische Klinik II (Gastroenterologie, Hepatologie, Diabetes und Stoffwechsel, Endokrinologie, Infektiologie und Reisemedizin, Onkologie und Internistische Intensivmedizin), Klinikum Chemnitz.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

English Abstract
Journal Article
Review

Language

ger

PubMed ID

16555178

Citation

Teubner, A, et al. "[Hepatitis C, Hemochromatosis and Porphyria Cutanea Tarda]." Deutsche Medizinische Wochenschrift (1946), vol. 131, no. 13, 2006, pp. 691-5.
Teubner A, Richter M, Schuppan D, et al. [Hepatitis C, hemochromatosis and porphyria cutanea tarda]. Dtsch Med Wochenschr. 2006;131(13):691-5.
Teubner, A., Richter, M., Schuppan, D., Köstler, E., & Stölzel, U. (2006). [Hepatitis C, hemochromatosis and porphyria cutanea tarda]. Deutsche Medizinische Wochenschrift (1946), 131(13), 691-5.
Teubner A, et al. [Hepatitis C, Hemochromatosis and Porphyria Cutanea Tarda]. Dtsch Med Wochenschr. 2006 Mar 31;131(13):691-5. PubMed PMID: 16555178.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [Hepatitis C, hemochromatosis and porphyria cutanea tarda]. AU - Teubner,A, AU - Richter,M, AU - Schuppan,D, AU - Köstler,E, AU - Stölzel,U, PY - 2006/3/24/pubmed PY - 2006/6/22/medline PY - 2006/3/24/entrez SP - 691 EP - 5 JF - Deutsche medizinische Wochenschrift (1946) JO - Dtsch Med Wochenschr VL - 131 IS - 13 N2 - Porphyria cutanea tarda (PCT) is characterized by decreased activity of the enzyme uroporphyrinogen decarboxylase (URO-D) and the accumulation of uro- and heptaporphyrins in the liver. Apart from increased alcohol exposure and certain drugs, PCT is associated with antibodies to the hepatitis C virus (HCV), with its prevalence increasing from Northern (8-10%) to Southern Europe (71 to 91%). Chronic HCV-infection is thus considered to be a major trigger for PCT and PCT is said to be an important extrahepatic manifestation of HCV-infection in predisposed individuals. Iron overload is common in PCT. Iron is an inhibitory co-factor of URO-D activity in hepatocytes. Accordingly, in support of the critical role of iron, the clinical efficacy of iron removal is coupled to an improvement of hepatic URO-D activities. Up to two thirds of Saxon patients with PCT carry the classical hemochromatosis (HFE) mutations (C282Y and/or H63D). HFE genotyping can help to further classify patients with PCT and associated hemochromatosis. Simple or compound heterozygosity of HFE mutations does not affect the therapeutic response to chloroquine in PCT. Since Patients carrying homozygous mutations (C282Y/C282Y) with hemochromatosis and PCT do not respond to chloroquine, phlebotomy should be first-line treatment to remove toxic iron. SN - 0012-0472 UR - https://www.unboundmedicine.com/medline/citation/16555178/[Hepatitis_C_hemochromatosis_and_porphyria_cutanea_tarda]_ L2 - http://www.thieme-connect.com/DOI/DOI?10.1055/s-2006-933718 DB - PRIME DP - Unbound Medicine ER -