1 Department of Pediatrics, Johns Hopkins University, and The Johns Hopkins, Hospital
Since the advent of cortisone for treatment of congenital adrenal hyperplasia, eight patients have died, representing a mortality of approximately 8%. These cases are presented, with an analysis of the findings in an attempt to explain the cause of death. In three of these cases errors in management were considered as factors in the terminal illness. One child died of an unrelated disorder. The remaining four died under circumstances that allow no ready explanation for their death. The possible mechanisms in these patients are discussed, including a poorly understood relationship of disturbance in the central nervous system to disorders of adrenal metabolism.