[Congenital adrenocortical hyperplasia].
The authors report about 3 cases of the congenital adreno-genital syndrome in first-born children with a high weight at birth (3900, 3600, and 4200 g) who perished in early infancy. One child, who was considered to be a boy, died of septic postoperative complications; during the operation the uterus, ovaries, tubes, phenomena of pseudofemale hemaphroditis were observed. The considerably enlarged adrenals were gyral, goffered and their cortex consisted of cells of the fetal zone. Two other boys, who perished at the age of 3 1/2 and 2 1/2 months, were sick from the birth. In the clinical picture there prevailed anorexia, dehydration, regurgitation, periodic vomiting, convulsions, clonic spasms, Hyponatrema, hyperkalemia, sudden arrest of the heart, rose no suspicion in pediatricians with respect to the salt-losing form of the adreno-genital syndrome. Post-mortem examination revealed congenital hyperplasia of the adrenals, whose cortex consisted of the fetal zone cells.
D000311:Adrenal Glands / Q000473:pathology*; D000312:Adrenal Hyperplasia, Congenital / Q000175:diagnosis / Q000473:pathology*; D006801:Humans; D008297:Male
O vrozhdennoĭ adrenokortikal'noĭ giperplazii nadpochechnikov