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Urinary Coproporphyrin isomers in Rotor's syndrome: A study in eight families

✍ Scribed by Yoshitaka Shimizu; Hiroshi Naruto; Setsuko Ida; Masateru Kohakura

Publisher
John Wiley and Sons
Year
1981
Tongue
English
Weight
391 KB
Volume
1
Category
Article
ISSN
0270-9139

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✦ Synopsis

Urinary coproporphyrin isomers were measured in 17 patients with Rotor's syndrome, 65 phenotypically normal relatives of 14 patients from eight families, and 21 normal subjects. Coproporphyrin I was elevated in Rotor's syndrome (p < 0.001) and, to a lesser degree, in phenotypically normal parents (p < 0.005), children, and siblings (p < 0.025) as compared to normal controls. Coproporphyrin 111 in patients, parents, and children did not differ from that in normal controls. Genetic analysis was consistent with transmission of Rotor's syndrome as an autosomal recessive trait with respect to urinary excretion of coproporphyrin I. After i.v. injection of 8-aminolevulinic acid, urinary coproporphyrin I increased to a similar extent in Rotor's syndrome and in normal

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