Cushing's Syndrome in Childhood

Of 8 children with Cushing's syndrome, 7 had tumours and 1 bilateral adrenal hyperplasia. All had hypertension; 5 cases also showed virilization; only 3 had short stature. All excreted raised urinary 17KS and 17OHCS. Pyelography showed displaced kidneys on the affected side in 5 cases. Early postoperative complications occurred in 4 cases. 2 patients developed adrenal crisis. In 1 patient the blood pressure rose to very high levels but subsequently fell to normal. The fourth patient developed severe hypertension and acute pancreatitis and died. One patient died from pneumonia 3½ years after operation. A scheme for postoperative replacement therapy with cortisol is given.