THE ROLE OF THE ADRENAL CORTEX IN SOMATOSEXUAL DISTURBANCES IN INFANTS AND CHILDREN: A CLINICO-PATHOLOGIC ANALYSIS*

Abstract

The effect of cortical hyperfunction depends on the type of hormone excess (androgen or estrogen), age, and sex. It may (but need not) be associated with hyperplasia or neoplasms, benign or malignant, unilateral or bilateral. During intrauterine life it may be a factor in causing pseudohermaproditism in which true sex may not be recognized except by laparotomy and biopsy of gonads. In male infants or boys, androgen excess produces the adreno-genital (corticosexual) syndrome (Child Hercules, precocious puberty, adrenal dwarfism); in females the somatosexual pattern of males is approximated (tomboy, virilism). In both sexes, bone maturation is accelerated and urinary 17-ketosteroids increased, and hirsutism is evident. The aim of therapy is to halt the process and remove its cause; this can be accomplished by adequate clinical studies and partial resection of enlarged adrenal or removal of adrenal neoplasm. Preponderance of granular cells resembling those of the reticular one was a usual finding. In Cushing''s disease somatic alteration is mainly due to excess corticosteroids, apparently elaborated by the fascicular zone, and sections revealed preponderance of swollen foam cells. The authors present 20 cases: 11 pseudo-hermaphrodites (3 females, sex in doubt; 4 females, raised as males; 2 males, sex in doubt; 2 males raised as females); 4 females with masculinization; 4 males with precocious puberty; and 1 male with Cushing''s syndrome.