Sayuri Koizumi, Ikuma Musha, Koichi Toda, Hiroshi Kawana, Chikahiko Numakura, Katsuhiko Tabata, Takamasa Mizumoto, Taisuke Nabeshima, Takuro Kojima, Toshiki Kobayashi, Takaya Hoashi, Toru Kikuchi
Saitama Medical University Hospital.
Japan
Clinical Pediatric Endocrinology
Clin Pediatr Endocrinol 2025; 34: 188-192
DOI: 10.1297/cpe.2024-0096
Abstract
Congenital hyperinsulinism (CHI) is characterized by hypoglycemia caused by excessive insulin secretion. CHI is classified into two types: transient CHI, which resolves within 3-4 mo of birth, and persistent CHI, which persists beyond this period. Diazoxide, the first-line treatment for CHI, may cause pulmonary hypertension (PH) as a side effect. Here, we report the case of a 2-mo-old girl with CHI and an atrial septal defect who initially responded well to diazoxide but developed dose-dependent PH. Diazoxide was discontinued, and treatment was switched to octreotide, glycogen storage disease milk, and glucagon. However, maintaining stable blood glucose levels remained challenging. Surgical intervention is typically required when medical management is ineffective; however, such procedures are limited to specialized facilities. Additionally, pancreatic resection carries a high risk of postoperative diabetes. To enable the safe reintroduction of diazoxide, we surgically closed the atrial septal defect with a left-to-right shunt and combined diazoxide therapy with anti-PH medication. This approach successfully controlled PH and achieved good glycemic control.
Category
Class I. Drug-induced and Toxin-induced Pulmonary Hypertension
Medical Therapy. Efficacy or Lack of Efficacy
Age Focus: Pediatric Pulmonary Vascular Disease
Fresh or Filed Publication: Fresh (PHresh). Less than 1-2 years since publication
Article Access
Free PDF File or Full Text Article Available Through PubMed or DOI: Yes