[21-hydroxylase deficit revealed by hyperkalemia in the absence of renal failure in an insulin-dependent diabetic (author's transl)].
The authors report a case of severe hyperkalemia (7 mmol/l) in an insulin-dependent diabetic in the absence of renal failure. This hyperkalemia was due to hypoaldosteronism caused by inadequate hormone biosynthesis in the absence of 21-hydroxylase. Replacement therapy allowed normalization of clinical and laboratory parameters. The various causes of hyperkalemia in the absence of renal failure in insulin-dependent diabetes are discussed, notably the renin deficiency hypoaldosteronism syndrome. This case study is remarkable for a number of reasons, including the very limited degree of virilization, the occurrence of a pregnancy, and the degree of salt excretion. This is a new observation in the diabetic, and is apparently a coincidental association since deficits in 21-hydroxylase are not usually associated with insulin-dependent diabetes.
D000312:Adrenal Hyperplasia, Congenital; D000328:Adult; D000450:Aldosterone / Q000172:deficiency; D003922:Diabetes Mellitus, Type 1 / Q000150:complications* / Q000201:enzymology; D005260:Female; D006801:Humans; D006947:Hyperkalemia / Q000209:etiology* / Q000503:physiopathology; D013250:Steroid Hydroxylases / Q000172:deficiency*
D000450:Aldosterone; D013250:Steroid Hydroxylases
Déficit en 21 hydroxylase révélé par une hyperkaliémie sans insuffisance rénale chez une diabétique insulino-dépendante.