Management of the Adult with Congenital Adrenal Hyperplasia

Management of the Adult with Congenital Adrenal Hyperplasia

Congenital adrenal hyperplasia (CAH), most commonly due to 21-hydroxylase deficiency (21OHD), has been studied by pediatric endocrinologists for decades. Advances in the care of these patients have enabled many of these children to reach adulthood. In contrast to the course and management of the disease in childhood, little is known about CAH in adults. In many patients, the proclivity to salt-...

Adult congenital adrenal hyperplasia.

CONGENITAL adrenal hyperplasia is a rare disease of adults. Probably the oldest recorded case in the literature is of a 57-year-old woman (Logan and McMillan, 1964) who presented as an abdominal emergency. Most of the published reports and standard references are concerned with the biochemical data and there is little suggestion of a characteristic clinical picture. The following case is presen...

Congenital adrenal hyperplasia: diagnosis, evaluation, and management.

1. Describe the pathophysiology of congenital adrenal hyperplasia (CAH). 2. Characterize the signs and symptoms of CAH. 3. Describe the appropriate laboratory evaluation of CAH. 4. Know that CAH can be diagnosed prenatally. 5. Recognize adrenal insufficiency by laboratory and clinical evaluation. 6. Anticipate and plan treatment for both acute adrenal crisis and long-term therapy for a patient ...

Congenital adrenal hyperplasia: management during critical illness.

BACKGROUND Little is known of the optimal dose and administration schedule of hydrocortisone in critically ill patients with congenital adrenal hyperplasia (CAH) caused by 21-hydroxylase deficiency. AIM To determine plasma cortisol concentrations after intravenous administration of hydrocortisone in children with CAH and to relate these to plasma cortisol concentrations achieved by endogenous...

Editorial: Management of Females with Congenital Adrenal Hyperplasia