Syndrome De Schwartz-Bartter Au Cours D'une Porphyrie Aiguë Intermittente

Abstract

SummaryA case of acute intermittent porphyria with marked hyponatraemia (109 mEq/1) and hypochloraemia (72 mEq/1) is presented. In the absence of adrenocortical insufficiency, chronic nephritis and digestive disorders with Na loss, the presence of a Schwartz-Bartter syndrome or inappropriate secretion of antidiuretic hormone is suspected. This hypothesis is confirmed be repeated acute water loading tests and by the fact that, during a recrudescence of the porphyria, a high fluid intake reproduces the fall in plasma Na and CI concentrations while these disorders arc corrected by water restriction. The authors review features of the Schwartz-Banter's syndrome and its relations with porphyrias.This case is interesting lor two other reasons. The administration of guancthidine has been accompanied by the disappearance of the arterial hypertension which had occured during the porphyria attack. The severe crisis with quadri-plegia, paralysis of several cranial nerves, respiratory palsy, convulsions and coma, has...