[Acute intermittent porphyria associated with hyperaldosteronism and inappropriate antidiuretic hormone secretion syndrome].
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.
Frère
T
T
Service d'Hépato-Gastroentérologie et d'Assistance Nutritive, CHU La Milétrie, Poitiers.
Roy-Peaud
F
F
Ripault
M P
MP
Dumas
P
P
Silvain
C
C
Pourrat
O
O
Beauchant
M
M
D000328:Adult; D006579:Heterozygote; D006801:Humans; D006929:Hyperaldosteronism / Q000150:complications* / Q000235:genetics; D007177:Inappropriate ADH Syndrome / Q000150:complications* / Q000235:genetics; D008297:Male; D010375:Pedigree; D017118:Porphyria, Acute Intermittent / Q000150:complications* / Q000235:genetics
Porphyrie aiguë intermittente associée à un hyperaldostéronisme et à un syndrome de sécrétion inappropriée d'hormone anti-diurétique.