Perinatology.  2022 Mar;33(1):37-42. 10.14734/PN.2022.33.1.37.

Diazoxide-Associated Pulmonary Hyperten sion in an Infant with Beck with-Wiedemann Syndrome: A Case Report

• Ryu JH
• Kang D
• Lee J
• Ko JM
• Jung YH
• Kim JH
• Choi CW

• KMID: 2527755
• DOI: http://doi.org/10.14734/PN.2022.33.1.37

Abstract

Diazoxide, an ATP-sensitive potassium channel opener, has been used as 1st line treatment for hyperinsulinemic hypoglycemia. As congenital hyperinsulinism is the most common cause of hyperinsulinemic hypoglycemia, diazoxide is more often used in neonates and infants than in adults.In 2015, the United States Food and Drug Administration issued a safety alert cautioning about pulmonary hypertension (PH) in children treated with diazoxide. Fluid overload prior to diazoxide administration and the presence of congenital heart disease have been known as risk factors for PH developing after diazoxide administration in children. Herein, we report a case of an infant with congenital hyperinsulinism associated with Beckwith-Wiedemann syndrome which developed PH after diazoxide administration. This is the first report of diazoxide-associated PH in Korea, which emphasizes the need for special awareness on this life-threatening side effect of diazoxide treatment in children.

Keyword

Diazoxide; Congenital hyperinsulinism; Beckwith-Wiedemann syndrome; Pulmonary hypertension