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Porphyria cutanea tarda.
Semin Liver Dis. 1998; 18(1):67-75.SL

Abstract

Porphyria cutanea tarda (PCT) is a skin disease that results from decreased activity of uroporphyrinogen decarboxylase (UROD). About 80% of patients have the sporadic (type I) form in which UROD deficiency is restricted to the liver. Others have familial (type II) PCT in which mutations in the UROD gene are inherited in an autosomal dominant pattern with low clinical penetrance. PCT may also follow exposure to porphyrogenic chemicals. Clinically overt PCT (types I and II) is provoked by liver cell injury, particularly when associated with alcohol abuse, hepatitis C infection, or estrogens. Hepatic iron overload is common, depletion of iron stores produces remission, and their replenishment leads to relapse. In PCT, hepatic UROD is inactivated by a process targeted at its catalytic site, which is iron-dependent, requires a heme precursor, and may be accelerated by induction of cytochrome P450s. Susceptibility to develop PCT in response to common causes of liver injury may be determined by co-inheritance of genes that regulate components of this inactivation process.

Authors+Show Affiliations

Department of Medical Biochemistry, University of Wales College of Medicine, Cardiff, UK.

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

9516680

Citation

Elder, G H.. "Porphyria Cutanea Tarda." Seminars in Liver Disease, vol. 18, no. 1, 1998, pp. 67-75.
Elder GH. Porphyria cutanea tarda. Semin Liver Dis. 1998;18(1):67-75.
Elder, G. H. (1998). Porphyria cutanea tarda. Seminars in Liver Disease, 18(1), 67-75.
Elder GH. Porphyria Cutanea Tarda. Semin Liver Dis. 1998;18(1):67-75. PubMed PMID: 9516680.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Porphyria cutanea tarda. A1 - Elder,G H, PY - 1998/3/28/pubmed PY - 1998/3/28/medline PY - 1998/3/28/entrez SP - 67 EP - 75 JF - Seminars in liver disease JO - Semin. Liver Dis. VL - 18 IS - 1 N2 - Porphyria cutanea tarda (PCT) is a skin disease that results from decreased activity of uroporphyrinogen decarboxylase (UROD). About 80% of patients have the sporadic (type I) form in which UROD deficiency is restricted to the liver. Others have familial (type II) PCT in which mutations in the UROD gene are inherited in an autosomal dominant pattern with low clinical penetrance. PCT may also follow exposure to porphyrogenic chemicals. Clinically overt PCT (types I and II) is provoked by liver cell injury, particularly when associated with alcohol abuse, hepatitis C infection, or estrogens. Hepatic iron overload is common, depletion of iron stores produces remission, and their replenishment leads to relapse. In PCT, hepatic UROD is inactivated by a process targeted at its catalytic site, which is iron-dependent, requires a heme precursor, and may be accelerated by induction of cytochrome P450s. Susceptibility to develop PCT in response to common causes of liver injury may be determined by co-inheritance of genes that regulate components of this inactivation process. SN - 0272-8087 UR - https://www.unboundmedicine.com/medline/citation/9516680/Porphyria_cutanea_tarda_ L2 - http://www.thieme-connect.com/DOI/DOI?10.1055/s-2007-1007142 DB - PRIME DP - Unbound Medicine ER -
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