Malignant hypertension in congenital adrenal hyperplasia due to 11 beta-hydroxylase deficiency

Two brothers, aged 20 and 12, with the 11 beta-hydroxylase deficient form of congenital adrenal hyperplasia, are presented. They had refused treatment for the previous four years until the younger was admitted with malignant hypertension, partial blindness and evidence of myocardial damage. The elder was therefore admitted for assessment and found to have less severe hypertension. Gas liquid chromatographic analysis of the steroids in urine from each revealed a possible difference in the enzyme defect between the two brothers--the younger having a complete, while the elder had only a partial block. Family studies have confirmed no association with the HLA locus.