Go Debre-Fibiger syndrome (adrenal hyperplasia)

Debre-Fibiger syndrome

Debre-Fibiger syndrome (synonymous with hyperplasia of the adrenal cortex with virilization and tyunyene release of mineral-corticoids). The disease is caused by stimulation of the adrenal cortex by the pituitary gland, which leads to bilateral hyperplasia or to the development of a tumor of the adrenal cortex, may be artificially caused by the use of adrenocorticotropic hormone or an overdose of glycocorticoids. With the disease there is an excess production of corticosteroids. Sometimes, against the background of an increase in the production of one of the hormones, a decrease in the production of others is observed, which suggests that the adrenal cortex is dysfunctional.

The syndrome appears as a combination of sexual disorders with the syndrome of "salt loss". The clinical picture is determined by the age at which hyperfunction of the gland occurs. If the over-excretion of androgens with increased secretion of 17-ketosteroids prevails, then premature puberty occurs in boys, if it develops in girls in the first half of intrauterine development, then pseudohermaphroditism develops, but if development occurs in the second half of intrauterine development or after birth, then girls develop progressive virilization.

The syndrome may be the result of congenital abnormal metabolism, in which there is a violation of the conversion of progesterone into hydrocortisone by hydrolysis of C17, Cu and C21. In this regard, there is an unusually high excretion of pregnandiol and pregnantiriol. These hormones give three clinical forms: a) due to insufficient hydrolysis at the 21st carbon atom; b) with prolonged loss of sodium and with hyperkalemia and c) associated with the production of “hormone-removing hormone”.