Abstract
Congenital adrenocortical hyperplasia (CAH) is an inborn error of metabolism produced by a genetically transmitted enzymatic defect in the functioning of the adrenal cortices of males and females. Enzymatic defects induce varying degrees of insufficiency of cortisol and aldosterone and excessive amounts of androgen and adrenocorticotropin. The incidence of CAH is estimated to be 1:15,000. Clinical manifestations include virilization, sexual ambiguity, hyponatremia, hyperkalemia, and/or hypertension. If the salt-losing form of CAH is undetected at birth, acute adrenal crisis may develop at 10 to 14 days of age with sometimes fatal results. Nursing interventions based on the Neuman Health Care Systems Model are discussed to assist individuals and families affected by CAH. A case study of a virilized female infant with the salt-losing form of CAH, Zl-hydroxylase deficiency, is presented to illustrate clinical aspects. Health-care professionals are encouraged not to overlook the emotional and psychological needs of families affected by CAH or other congenital anomalies. 0 1986 by Grune & Stratton, Inc.
Published Version
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