Terminal chronic kidney disease with arterial hypotension in a patient with classic congenital adrenal hyperplasia

Abstract

Congenital adrenal hyperplasia (CAH) is a rare genetic disorder which is caused by autosomal recessive mutations in genes, encoding enzymes involved in the process of glycoand/ or the mineralocorticoid synthesis. It is most common 21-hydroxylase deficiency. Renal disease in patients with CAH is not common but is usually associated with hypertension. Here we present the case of hypotensive terminal chronic renal diseases, which required the support of hemodialysis in patients with noncompliance CAH intermediation with a loss of salt. Also, we analyzed the influence of hemodialysis treatment on biochemical indicators of quality of CAH treatment.