PEDIATRICS Vol. 46 No. 2 August 1970, pp. 217-229
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CUSHING'S SYNDROME IN INFANCY

Michel G. Gilbert M.D.1 and William W. Cleveland M.D.1

1 Department of Pediatric Surgery, Variety Children's Hospital, Miami, Florida, and the Department of Pediatrics, University of Miami School of Medicine, Miami, Florida

Cushing's syndrome in infancy is usually due to adrenal tumor which is often malignant. Three cases are described in which the disorder was due to adenoma and surgical cure was achieved. The clinical picture is strongly suggestive if not diagnostic. Its hallmark is generalized obesity with typical facies; also present are hypertension and signs of virilization. Striae were not found in our patients. Increased excretion of both 17-hydroxysteroids and 17-ketosteroids is characteristic; this excretion was not suppressed by dexamethasone in two of these patients in whom it was tested. Intravenous pyelogram was distinctly abnormal in all cases and indicated the presence and location of the tumor. The diagnosis can be made efficiently and surgical treatment can be promptly instituted with reasonable expectation of cure. Careful attention must be paid to preoperative and postoperative administration of adrenal steroids.

Submitted on October 24, 1969
Accepted on February 23, 1970




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