[Latent porphyria cutanea tarda].
A total of 142 subjects have been examined; of these 49 healthy relatives of patients with manifest porphyria cutanea tarda (PCT) (group 1), 48 subjects with melanodermal skin changes characteristic of PCT abd with anamnesis aggravated for alcoholism (group 2), and 45 patients with chronic liver diseases (group 3). None of the examinees has developed photosensitization symptoms. The findings have been compared to the results of examinations of 24 normal subjects and of 145 patients with manifest PCT. Minimal abnormalities of porphyrin metabolism have been detected in 43 subjects (30.2%). In group 1 subjects these abnormalities presented as increased levels of uroporphyrin and fecal coproporphyrin, in Groups 2 and 3 as secondary coproporphyrinuria and a symptomatic rise of fecal protoporphyrin level. Latent PCT has been diagnosed in 18 patients (12.7%). In latent PCT the total porphyrin excretion with the urine has been 10-fold lower than in manifest PCT, not exceeding 1000 nmol/day; in has been associated with a relative elevation of uroporphyrin level (up to 42-65% of the total porphyrin content). Increased coproporphyrin concentrations have been recorded, with coproporphyrin share making up over 60% of the total amount. It is possible that the minimal shifts of porphyrin metabolism anticipate the development of the biochemical syndrome of latent PCT. The author suggests criteria for the early diagnosis of the latent forms of the disease. He considers that the examinees should be referred to a group at risk of developing manifest PCT.
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BN
Krivosheev
D000293:Adolescent; D000328:Adult; D000368:Aged; D000437:Alcoholism / Q000378:metabolism; D005243:Feces / Q000032:analysis; D005260:Female; D006801:Humans; D008107:Liver Diseases / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D011164:Porphyrias / Q000175:diagnosis* / Q000235:genetics / Q000378:metabolism; D011166:Porphyrins / Q000032:analysis; D012871:Skin Diseases / Q000175:diagnosis* / Q000235:genetics / Q000378:metabolism; D012880:Skin Pigmentation
D011166:Porphyrins
Latentnaia pozdniaia kozhnaia porfiriia.