PEDIATRICS Vol. 10 No. 4 October 1952, pp. 397-413
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FURTHER STUDIES ON THE TREATMENT OF CONGENITAL ADRENAL HYPERPLASIA WITH CORTISONE

IV. Effect of Cortisone and Compound B in Infants with Disturbed Electrolyte Metabolism

JOHN F. CRIGLER JR. M.D.1, SAMUEL H. SILVERMAN M.D.1, and LAWSON WILKINS M.D.1

1 The Department of Pediatrics, Johns Hopkins University School of Medicine and the Harriet Lane Home of the Johns Hopkins Hospital, Baltimore.

Three cases of congenital adrenal hyperplasia with disturbed electrolyte metabolism, followed for 14 to 20 months, have been reported in detail.

The effects of cortisone (compound E) and corticosterone (compound B) on the suppression of the abnormal adrenals and on the electrolyte disturbance have been discussed. Cortisone produces more marked suppression of the adrenal overactivity/mg. (as measured by the urinary excretion of 17-KS), but less sodium retention than corticosterone. Both steroids, however, significantly improve the electrolyte abnormality.

The possible mechanism of action of cortisone on the electrolyte metabolism is discussed.

Suggestions concerning the therapeutic management of these patients are offered. The use of adequate NaCl without DCA in the initial treatment is emphasized, since suppression of the adrenal with cortisone seems to alter materially DCA requirement. The final combination of therapy, however, must be decided in each patient individually.

Submitted on April 11, 1952