Cutaneous hepatic porphyria and oestrogens.
نویسندگان
چکیده
منابع مشابه
Symptomatic Cutaneous Hepatic Porphyria and from Rats with Porphyria
1. Three tetracarboxylate porphyrins, apart from coproporphyrin, were isolated from the faeces of patients with symptomatic cutaneous hepatic porphyria and of rats with porphyria caused by hexachlorobenzene poisoning. The porphyrins were not present in the urine of the porphyric rats or in the faeces of control rats. 2. Two of the porphyrins were identified as tris-(2-carboxyethyl)-carboxymethy...
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The inborn errors of heme biosynthesis, the porphyrias, are 8 genetically distinct metabolic disorders that can be classified as "acute hepatic," "hepatic cutaneous," and "erythropoietic cutaneous" diseases. Recent advances in understanding their pathogenesis and molecular genetic heterogeneity have led to improved diagnosis and treatment. These advances include DNA-based diagnoses for all the ...
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Porphyrias are groups of heterogeneous metabolic disorders that result from derangement of the heme biosynthesis. There are 8 distinct subtypes each corresponds to a distinct enzymatic step in this pathway. There are generally classified as erythoid, hepatic and hepatic cutaneous disorders. Several mutations have been identified that result in these metabolic abnormalities. This group of disord...
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The porphyrias are uncommon, compSex, and fascinating metabolic conditions, caused by deficiencies in the activities ofthe enzymes ofthe heme biosynthetic pathway. ISvo cardinal symptoms of the porphyrias are cutaneous photosensitivity and neurologic disturbances. Molecular analysis of gene defects has shown that there are multiple and heterogeneous mutations in each porphyria. Patients with sy...
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ورودعنوان ژورنال:
- British medical journal
دوره 1 5485 شماره
صفحات -
تاریخ انتشار 1966