Development of Pulmonary Hypertension in an Infant Treated With Diazoxide for Hyperinsulinism, a Case Report and Literature Review

摘要

Congenital hyperinsulinism (CHI) is heterogeneous disorder in the neonatal period characterized by inappropriate insulin secretion in the presence of hypoglycemia. Known etiologies include inactivating mutations of the KATP channel. Its management can be extremely complicated, and may involve medical therapy and surgery. We describe a full term baby male at one month-old without dysmorphic features or congenital anomalies, the baby was presented with asymptomatic hypoglycemia by routine screening at the age of 1.5 hour of life, he was treated with diazoxide for prolonged neonatal hyperinsulinism. Tolerance of diazoxide is usually excellent, but several adverse effects of this drug have been described. During diazoxide therapy he presented with tachycardia and low oxygen saturation, the baby became dyspnoeic with fluid retention and facial edema. Echocardiography showed moderate pulmonary hypertension. Diazoxide was withdrawn, his clinical status and pulmonary hypertension dramatically improved and returned to baseline.