Diabetes In A Nonpancreatectomized Child With Nesidioblastosis
- Bassam S. Bin-Abbas, MD and
- Abdullah A. Al-Ashwal, MD
- From the Department of Pediatrics, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia
- Address correspondence to Bassam Saleh Bin-Abbas, MD Consultant, Section of Pediatric Endocrinology Department of Pediatrics, MBC 58 King Faisal Specialist Hospital and Research Center, P.O. Box 3354 Riyadh 11211 Saudi Arabia. E-mail: benabbas{at}kfshrc.edu.sa
Persistent hyperinsulinemic hypoglycemia of infancy (PHHI) (Online Mendelian Inheritance in Man [OMIM] 256450), formerly known as nesidioblastosis, is a glucose metabolism disorder characterized by profound hypoglycemia and inappropriate secretion of insulin (1). Affected children run the risk of severe neurological damage unless immediate and adequate steps are taken (2). Treatment with diazoxide and/or somatostatin analogue is the first line of therapy. However, it not always effective, especially in familial cases, which may necessitate an alternative intervention such as pancreatectomy (3).
Several studies have suggested that partial pancreatectomy endangers future islet cell function (4,5). The incidence of diabetes increases with age and correlates with the extent of surgical resection (6,7). However, there was no report of occurrence of overt diabetes in medically treated patients (8). In this report, we described an adolescent female with neonatal nesidioblastosis who developed diabetes after medical treatment with diazoxide/octreotide. To our knowledge, this is the first nesidioblasosis case subject who developed diabetes following medical therapy.
A 14-year-old Saudi female presented with severe persistent hypoglycemia during the first few …
