Adrenocortical hyperplasia in childhood.

Adrenocortical hyperplasia is uncommon in childhood. It occurs as the adrenogenital syndrome (congenital virilism in girls) which has now been shown to be due to a congenital defect in the synthesis of cortisol by the adrenal cortex. In the United States its incidence is once in every 58,000 live births (Grumbach, 1956) and it has a recessive mode of inheritance. The disease is more easily diagnosed in females than in males and this is probably the reason for the higher observed incidence in girl babies. Cushing's syndrome is a still more uncommon result of adrenocortical hyperplasia in children. Before 1949 this had not been described in a child under the age of 10 years (Wilkins, 1950) but in 1949 Chute, Robinson and Donohue described it in an 8-year-old girl. Twenty-two other cases of Cushing's syndrome under the age of 10 years have been reported and all were due to a tumour. One of the cases now to be described is a boy with Cushing's syndrome which had its onset at the age of 6 years; the other is a girl aged 21 years with congenital virilism. Both cases illustrate some problems in the pathogenesis, diagnosis and treatment of adrenocortical hyperplasia in childhood.