Decreased Hepatic Uroporphyrinogen Decarboxylase Activity in Porphyria Cutanea Tarda
- 1 April 1982
- journal article
- research article
- Published by Massachusetts Medical Society in New England Journal of Medicine
- Vol. 306 (13), 766-769
- https://doi.org/10.1056/nejm198204013061302
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. (N Engl J Med. 1982; 306: 766–9.)This publication has 21 references indexed in Scilit:
- Red-Cell Uroporphyrinogen Decarboxylase Activity in Porphyria Cutanea Tarda and in Other Forms of PorphyriaNew England Journal of Medicine, 1978
- Decreased Activity of Hepatic Uroporphyrinogen Decarboxylase in Sporadic Porphyria Cutanea TardaNew England Journal of Medicine, 1978
- An inherited enzymatic defect in porphyria cutanea tarda: decreased uroporphyrinogen decarboxylase activity.JCI Insight, 1976
- The role of iron in the pathogenesis of porphyria cutanea tarda. II. Inhibition of uroporphyrinogen decarboxylase.JCI Insight, 1975
- HEPATIC δ-AMINOLÆVULINIC ACID SYNTHETASE ACTIVITY IN PORPHYRIA CUTANEA TARDAThe Lancet, 1972
- THE EFFECT OF REPLENISHMENT OF IRON STORES AFTER PHLEBOTOMY THERAPY IN PORPHYRIA CUTANEA TARDAActa Medica Scandinavica, 1971
- Porphyrin Metabolism in Experimental Hepatic Siderosis in the RatBritish Journal of Haematology, 1970
- Effect of Ethanol on Liver δ‐Aminolaevulinate Synthetase Activity and Urinary Porphyrin Excretion in Symptomatic PorphyriaBritish Journal of Haematology, 1969
- Hepatic δ-Aminolaevulinic Acid Synthetase Activity in Symptomatic PorphyriaBritish Journal of Haematology, 1968
- EFFECT OF CHLOROQUINE ON HEPATIC UROPORPHYRIN METABOLISM IN PATIENTS WITH PORPHYRIA CUTANEA TARDA1,2Medicine, 1966