Porphyrins in Dubin-Johnson Syndrome
- 12 April 1973
- journal article
- Published by Massachusetts Medical Society in New England Journal of Medicine
- Vol. 288 (15) , 795-796
- https://doi.org/10.1056/nejm197304122881518
Abstract
To the Editor: I have some questions and comments about the article by Wolkoff et al. entitled "Inheritance of the Dubin-Johnson Syndrome" (N Engl J Med 288:113, 1973). The authors, and others previously, have observed both decreased urinary excretion of coproporphyrin III and increased excretion of coproporphyrin I in the Dubin-Johnson syndrome, yielding an abnormally high ratio of I-isomer excretion. The "obligate heterozygotes" do not conform to this pattern in that decreased III-isomer excretion is not associated with increased I-isomer excretion to yield only a 7 per cent increase in percentage of coproporphyrin I excretion vs. controls. Although the authors . . .Keywords
This publication has 6 references indexed in Scilit:
- Uroporphyrinogen III Cosynthetase Activity in the Fox Squirrel ( Sciurus niger )Science, 1971
- Abnormal Excretion of the Isomers of Urinary Coproporphyrin by Patients with Dubin—Johnson Syndrome in IsraelClinical Science, 1971
- Uroporphyrinogen III cosynthetase activity in fibroblasts from patients with congenital erythropoietic porphyriaBiochemical Genetics, 1970
- Uroporphyrinogen III cosynthetase in asymptomatic carriers of congenital erythropoietic porphyriaBiochemical Genetics, 1970
- Heme Biosynthesis in Intermittent Acute Porphyria: Decreased Hepatic Conversion of Porphobilinogen to Porphyrins and Increased Delta Aminolevulinic Acid Synthetase ActivityProceedings of the National Academy of Sciences, 1970
- UROPORPHYRINOGEN III COSYNTHETASE IN HUMAN CONGENITAL ERYTHROPOIETIC PORPHYRIAProceedings of the National Academy of Sciences, 1969