PEDIATRICS Vol. 52 No. 3 September 1973, pp. 372-381
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BECKWITH'S SYNDROME WITH EXTREME ORGAN HYPERPLASIA

Thomas F. Roe M.D.1, Ann K. Kershnar M.D.1, Jordan J. Weitzman M.D.1, and Luis Salinas Madrigal M.D.1

1 Divisions of Endocrinology and Metabolism, Pediatric Surgery and the Department of Pathology of Childrens Hospital of Los Angeles, and the Department of Pediatrics, Surgery and Pathology of the University of Southern California School of Medicine

A large newborn infant with hemihypertrophy, omphalocele, hepatomegaly, left upper quadrant abdominal mass, and zoster-like skin rash had severe hypoglycemia at 4 hours of age. Serum immunoreactive insulin levels were markedly elevated. Hypoglycemia was not controlled by vigorous medical therapy but blood glucose levels returned toward normal following subtotal excision of the markedly hyperplastic pancreas at 24 days of age. At 4frac12 months of age, a right upper quadrant abdominal mass was noted and urinary adrenal steroid levels were elevated. The right adrenal gland was found to be markedly hyperplastic and it was removed; the left adrenal gland was slightly hyperplastic. Between the ages of 5 and 8 months the umbilical stump enlarged progressively forming a large pedunculated tumor which was removed. This patient presents an unusually severe example of Beckwith's syndrome with both prenatal and postnatal organ overgrowth, severe hypoglycemia and hyperinsulinism.

Submitted on January 26, 1973
Accepted on April 9, 1973




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