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Title:
Hereditary uroporphyrinogen-decarboxylase deficiency predisposing porphyria cutanea tarda (chronic hepatic porphyria) in females after oral contraceptive medication | Archives of Dermatological Research
Description:
Porphyria cutanea tarda (PCT) was diagnosed in 27 women aged 23β48 years (mean, 35 years) who had been under oral-hormonal-contraceptive medication for 1β18 years, in 3 women under substitutional estrogen treatment in the menopause, and in 2 men aged 65 and 76 years after estrogen treatment of prostatic carcinoma. In all patients, total urinary porphyrin excretion was elevated, with an average uro-and heptacarboxyporphyrin predominance of 88%, thus proving PCT. On the patients, 84% showed a significant decrease of erythrocyte uroporphyrinogen-decarboxylase (UD; EC 4.1.1.37) activity to β50% of control levels suggesting a hereditary predisposition for the development of a chronic hepatic porphyria. Estrogens and alcohol are capable of reducing hepatic UD activity. Women with hereditary red cell UD deficiency may be regarded as predisposed to PCT when under estrogen intake, especially in combination with the potentiating influence of alcohol and chronic liver disease. Normal erythrocyte UD values in patients with additive alcohol consumption may implicate a stronger inhibitory effect for alcohol on UD, suggesting a merely toxic form of chronic hepatic porphyria.
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porphyria, google, scholar, cutanea, tarda, hepatic, doss, uroporphyrinogen, decarboxylase, article, chronic, hereditary, activity, med, dermatol, clin, privacy, cookies, content, research, uroporphyrinogendecarboxylase, estrogen, alcohol, liver, access, induced, arch, publish, search, deficiency, years, patients, porphyrin, clinical, defect, chem, porphyrins, wochenschr, data, information, log, journal, oral, medication, sixeldietrich, pct, women, erythrocyte, predisposition, estrogens,
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headline:Hereditary uroporphyrinogen-decarboxylase deficiency predisposing porphyria cutanea tarda (chronic hepatic porphyria) in females after oral contraceptive medication
description:Porphyria cutanea tarda (PCT) was diagnosed in 27 women aged 23β48 years (mean, 35 years) who had been under oral-hormonal-contraceptive medication for 1β18 years, in 3 women under substitutional estrogen treatment in the menopause, and in 2 men aged 65 and 76 years after estrogen treatment of prostatic carcinoma. In all patients, total urinary porphyrin excretion was elevated, with an average uro-and heptacarboxyporphyrin predominance of 88%, thus proving PCT. On the patients, 84% showed a significant decrease of erythrocyte uroporphyrinogen-decarboxylase (UD; EC 4.1.1.37) activity to β50% of control levels suggesting a hereditary predisposition for the development of a chronic hepatic porphyria. Estrogens and alcohol are capable of reducing hepatic UD activity. Women with hereditary red cell UD deficiency may be regarded as predisposed to PCT when under estrogen intake, especially in combination with the potentiating influence of alcohol and chronic liver disease. Normal erythrocyte UD values in patients with additive alcohol consumption may implicate a stronger inhibitory effect for alcohol on UD, suggesting a merely toxic form of chronic hepatic porphyria.
datePublished:
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keywords:
Porphyria cutanea tarda
Chronic hepatic porphyria
Oral contraceptives
Estrogens
Uroporphyrinogen-decarboxylase defect
Hereditary predisposition
Dermatology
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headline:Hereditary uroporphyrinogen-decarboxylase deficiency predisposing porphyria cutanea tarda (chronic hepatic porphyria) in females after oral contraceptive medication
description:Porphyria cutanea tarda (PCT) was diagnosed in 27 women aged 23β48 years (mean, 35 years) who had been under oral-hormonal-contraceptive medication for 1β18 years, in 3 women under substitutional estrogen treatment in the menopause, and in 2 men aged 65 and 76 years after estrogen treatment of prostatic carcinoma. In all patients, total urinary porphyrin excretion was elevated, with an average uro-and heptacarboxyporphyrin predominance of 88%, thus proving PCT. On the patients, 84% showed a significant decrease of erythrocyte uroporphyrinogen-decarboxylase (UD; EC 4.1.1.37) activity to β50% of control levels suggesting a hereditary predisposition for the development of a chronic hepatic porphyria. Estrogens and alcohol are capable of reducing hepatic UD activity. Women with hereditary red cell UD deficiency may be regarded as predisposed to PCT when under estrogen intake, especially in combination with the potentiating influence of alcohol and chronic liver disease. Normal erythrocyte UD values in patients with additive alcohol consumption may implicate a stronger inhibitory effect for alcohol on UD, suggesting a merely toxic form of chronic hepatic porphyria.
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Chronic hepatic porphyria
Oral contraceptives
Estrogens
Uroporphyrinogen-decarboxylase defect
Hereditary predisposition
Dermatology
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