THE EFFECTS OF ADRENOCORTICOTROPIC HORMONE AND CORTISONE IN THE ADRENOGENITAL SYNDROME ASSOCIATED WITH CONGENITAL ADRENAL HYPERPLASIA: AN ATTEMPT TO EXPLAIN AND CORRECT ITS DISORDERED HORMONAL PATTERN 12

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Abstract
Four courses of ACTH were admd. to three female patients with the adrenogenital syndrome resulting from congenital adrenal hyperplasia. The dosage ranged from .71 to 3.33 mg. per day per kg. body weight, the duration from 9 to 12 days. Studies included urinary 17-ketosteroids, reducing steroids, glucose, and chloride, balances of N, P, Ca, K, Mg, and Na, and measurements of circulating eosinophils, serum P, Ca, K, Mg, Cl, CO2 and glucose. ACTH produced a rise in urinary 17-ketosteroids in three of the four courses, but none of its other usual metabolic effects. With the highest dosage of ACTH there was actual N retention. Cortisone acetate was admd. to two of these patients on the same regimen. The dosages were 1.90 and 2.22 mg. per day per kg. body weight, the duration 12 days. Cortisone produced a fall to normal and even below normal in the elevated urinary 17-ketosteroid excretion, and a negative N balance. The latter is exceptional at the dosages employed. ACTH (2.05 mg. per kg. per day) and cortisone (2.32 mg. per kg. per day) were admd. together to one of these patients for eight days. The resulting rise in urinary 17-ketosteroids was essentially the same as that produced by ACTH alone. Cortisone acetate is being admd. continuously to two of these patients at dosages of 100-200 mg. a week. At this dosage the urinary 17-ketosteroids remain normal and the circulating eosinophils are not depressed. One patient is having monthly periods with cramps. The above findings suggest that this syndrome involves a primary defect of the adrenal cortex, and a secondary increase of pituitary ACTH production. In all patients the eegs. showed asymmetry, elevation of voltages, and abnormal slow waves.