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Decreased hepatic uroporphyrinogen decarboxylase activity in porphyria cutanea tarda.
N Engl J Med 1982; 306(13):766-9NEJM

Abstract

To test whether reduced hepatic uroporphyrinogen decarboxylase activity is a specific and intrinsic defect in porphyria cutanea tarda, we measured enzymatic activity in the livers of 17 patients with porphyria cutanea tarda, 12 "normal" control patients without liver disease, and 41 patients with other forms of porphyria, alcoholic liver disease, hemochromatosis, or chronic hepatitis. Enzyme activity in all the patients with porphyria cutanea tarda was lower than in the patients without this disease, except for one patient with alcohol-induced fatty liver. Reduction of hepatic iron stores by phlebotomy did not alter the enzymatic activity in porphyria cutanea tarda. We conclude that reduced hepatic uroporphyrinogen decarboxylase activity is a specific and intrinsic hepatic defect in porphyria cutanea tarda, but modulation of uroporphyrinogen synthesis by extrinsic factors is required for the full biochemical expression of the disease.

Authors

No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, U.S. Gov't, P.H.S.

Language

eng

PubMed ID

7062951

Citation

Felsher, B F., et al. "Decreased Hepatic Uroporphyrinogen Decarboxylase Activity in Porphyria Cutanea Tarda." The New England Journal of Medicine, vol. 306, no. 13, 1982, pp. 766-9.
Felsher BF, Carpio NM, Engleking DW, et al. Decreased hepatic uroporphyrinogen decarboxylase activity in porphyria cutanea tarda. N Engl J Med. 1982;306(13):766-9.
Felsher, B. F., Carpio, N. M., Engleking, D. W., & Nunn, A. T. (1982). Decreased hepatic uroporphyrinogen decarboxylase activity in porphyria cutanea tarda. The New England Journal of Medicine, 306(13), pp. 766-9.
Felsher BF, et al. Decreased Hepatic Uroporphyrinogen Decarboxylase Activity in Porphyria Cutanea Tarda. N Engl J Med. 1982 Apr 1;306(13):766-9. PubMed PMID: 7062951.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Decreased hepatic uroporphyrinogen decarboxylase activity in porphyria cutanea tarda. AU - Felsher,B F, AU - Carpio,N M, AU - Engleking,D W, AU - Nunn,A T, PY - 1982/4/1/pubmed PY - 1982/4/1/medline PY - 1982/4/1/entrez SP - 766 EP - 9 JF - The New England journal of medicine JO - N. Engl. J. Med. VL - 306 IS - 13 N2 - To test whether reduced hepatic uroporphyrinogen decarboxylase activity is a specific and intrinsic defect in porphyria cutanea tarda, we measured enzymatic activity in the livers of 17 patients with porphyria cutanea tarda, 12 "normal" control patients without liver disease, and 41 patients with other forms of porphyria, alcoholic liver disease, hemochromatosis, or chronic hepatitis. Enzyme activity in all the patients with porphyria cutanea tarda was lower than in the patients without this disease, except for one patient with alcohol-induced fatty liver. Reduction of hepatic iron stores by phlebotomy did not alter the enzymatic activity in porphyria cutanea tarda. We conclude that reduced hepatic uroporphyrinogen decarboxylase activity is a specific and intrinsic hepatic defect in porphyria cutanea tarda, but modulation of uroporphyrinogen synthesis by extrinsic factors is required for the full biochemical expression of the disease. SN - 0028-4793 UR - https://www.unboundmedicine.com/medline/citation/7062951/Decreased_hepatic_uroporphyrinogen_decarboxylase_activity_in_porphyria_cutanea_tarda_ L2 - http://www.nejm.org/doi/full/10.1056/NEJM198204013061302?url_ver=Z39.88-2003&rfr_id=ori:rid:crossref.org&rfr_dat=cr_pub=pubmed DB - PRIME DP - Unbound Medicine ER -