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October 1976

Hypokalemic Crisis Simulating Intestinal Obstruction in a 4-Year-Old Girl: A Consequence of 17α-Hydroxylase Deficiency

Author Affiliations

From the Department of Pediatrics, Ramathibodi Hospital Medical School, Mahidol University, Bangkok, Thailand.

Am J Dis Child. 1976;130(10):1143-1145. doi:10.1001/archpedi.1976.02120110105016

• A 4-year-old girl had abdominal distention, muscular weakness, renal tubular dysfunction, and hypertension associated with hypokalemic metabolic alkalosis. There were no clinical symptoms of cortisol deficiency, but there was excessive deoxycorticosterone and corticosterone production. Basal plasma aldosterone levels were undetectable; however, adrenocorticotropic hormone (ACTH) stimulation brought plasma aldosterone levels up to normal. The urinary pregnanediol, tetrahydro-deoxycorticosterone (THDOC), and tetrahydrocorticosterone (THB) concentrations were elevated. Stimulation of ACTH failed to increase urinary 17-ketosteroid, 17-hydroxycorticosteroid, or plasma cortisol levels significantly, while urinary THDOC, THB, and plasma corticosterone concentrations were further elevated. The elevated plasma corticosteroid intermediates were suppressed by dexamethasone administration. When physiologic doses of dexamethasone were administered, the hypertension, electrolyte imbalance, and abnormal corticosteroid secretion were all corrected. The studies indicated a partial 17α-hydroxylase defect in this patient.

(Am J Dis Child 130:1143-1145, 1976)