sildenafil-citrate and Beckwith-Wiedemann-Syndrome

Diazoxide, a drug used to treat hyperinsulinemic hypoglycemia (HH), is associated with pulmonary hypertension (PH), as reported by the US Food and Drug Administration. However, no report has detailed the association between diazoxide dose and PH development. We report a case of an infant with HH, subsequently complicated by diazoxide-induced PH. When diazoxide was introduced, PH did not appear initially, but it developed during increased dosing. We monitored PH via regular echocardiography examinations. PH gradually improved with tapering of the diazoxide dose and disappeared after drug discontinuation. Our case suggests a diazoxide dose threshold might induce PH. Therefore, close echocardiography examinations should accompany diazoxide treatment.

Topics: Atrial Natriuretic Factor; Beckwith-Wiedemann Syndrome; Cardiac Catheterization; Congenital Hyperinsulinism; Deprescriptions; Diazoxide; Diuretics; Dose-Response Relationship, Drug; Echocardiography; Electrocardiography; Humans; Hypertension, Pulmonary; Infant; Infant, Newborn; Male; Natriuretic Peptide, Brain; Sildenafil Citrate; Vasodilator Agents