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[Acute intermittent porphyria associated with hyperaldosteronism and inappropriate antidiuretic hormone secretion syndrome].

作者信息

Frère T, Roy-Peaud F, Ripault M P, Dumas P, Silvain C, Pourrat O, Beauchant M

机构信息

Service d'Hépato-Gastroentérologie et d'Assistance Nutritive, CHU La Milétrie, Poitiers.

出版信息

Gastroenterol Clin Biol. 1998 Aug-Sep;22(8-9):727-31.

PMID:9823563
Abstract

Acute intermittent porphyria, the most commun acute porphyria in France, is an autosomal dominant disorder of heme biosynthesis. The basic biochemical defect is reduced activity of the enzyme porphobilinogen deaminase. Clinical evolution is characterized by acute attacks, with a severe prognosis due to acute abdominal pain and risk of neurological complications, induced by drug intake, infection, alcohol intake or unknown factors. We report the case of a patient with an inappropriate antidiuretic secretion syndrome and secondary hyperaldosteronism associated with acute intermittent porphyria and polyradiculoneuritis syndrome. This syndrome was found to be induced a delayed reaction to thiopental. A favorable response was achieved with heme-arginate treatment.

摘要

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