Beckwith-Wiedemann syndrome [John B. Beckwith, American pathologist, b. 1933] , a hereditary disorder of unknown cause associated with neonatal hypoglycemia and hyperinsulinism. Formerly called EMG syndrome. ▪ OBSERVATIONS: Clinical manifestations include unusual growth in both height and weight until approximately the age of 8, a large tongue, and an umbilical hernia. There is enlargement of internal organs, with hyperplasia of the kidney and pancreas and extreme enlargement of the cells of the adrenal cortex. ▪ INTERVENTIONS: The management of blood sugar levels is critical. Subtotal pancreatectomy is often necessary in cases of beta cell hyperplasia, nesidioblastosis, or beta cell tumor of the pancreas. Surgical repair of abdominal defects is often necessary. ▪ PATIENT CARE CONSIDERATIONS: Children who survive infancy generally do well. Monitoring for signs of tumors is important.
