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April 1983

Persistent Leucine Sensitivity Following Partial Pancreatectomy and Diazoxide Treatment: Therapeutic Response

Author Affiliations

From the Division of Pediatric Endocrinology (Drs Zuniga and Golden) and the Nutrition Service (Mss Graham and Bradvica), University of California Irvine Medical Center, Orange; the Division of Endocrinology and Metabolism, St Louis Children's Hospital (Drs Sargeant, Santiago, and White); and the Edward Mallinckrodt Department of Pediatrics, Washington University, St Louis (Drs Sargeant, Santiago, and White). Dr Golden is now with the James Whitcomb Riley Hospital for Children, Indianapolis.

Am J Dis Child. 1983;137(4):393-395. doi:10.1001/archpedi.1983.02140300071020

• Hyperinsulinemic hypoglycemia secondary to nesidioblastosis persisted in a 4-year-old girl despite a 95% pancreatectomy and high-dose diazoxide treatment. Current literature suggests that dietary treatment, specifically protein restriction, of patients who are receiving diazoxide is ineffective and that further surgical pancreatectomy is required. However, in this patient, marked leucine sensitivity was demonstrated and a subsequent reduction in leucine intake eliminated hypoglycemia and the need for total or nearly total pancreatectomy. Therefore, dietary alteration should be considered in some patients with hyperinsulinism as an adjunct to other medical treatment.

(Am J Dis Child 1983;137:393-395)