Congenital Virilizing Adrenal Hyperplasia

Abstract

In the past several years, great strides have been made in the elucidation of certain inborn defects in steroid metabolism. This has led to a better understanding of the pathophysiology of congenital virilizing adrenal hyperplasia and has given us a more rational approach to the treatment of this disorder.The purpose of this discussion is to review some of the newer knowledge of steroidal biogenesis, to point out the different enzymatic deficiencies that have been found in various forms of the adrenogenital syndrome, to summarize the salient clinical features, and to discuss the rationale of therapy.Physiological Considerations Adrenal Physiology in the Newborn.\p=m-\ It is helpful to be familiar with the physiology of the adrenal gland in the newborn infant in order to understand the changes that take place in congenital adrenal hyperplasia. At birth the newly born baby is endowed with an extremely large adrenal gland which is 0.29% of his total body