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.
A four-year-old girl experienced persistent hypoglycemia shortly after birth and was diagnosed with congenital hyperinsulinism due to genetic mutations in the ABCC8 gene.
She did not respond to typical treatments like oral diazoxide but showed improvement with subcutaneous somatostatin injections.
At age three and a half, her treatment was changed to a long-acting somatostatin analogue, leading to better blood sugar control, normal growth, and the ability to stop tube feeding.
Department of Pediatric Endocrinology and Inherited Metabolic Diseases, National Children's Medical Center, Children's Hospital of Fudan University, Shanghai, 201102, China.
Octreotide serves as a second-line treatment for congenital hyperinsulinism (CHI) patients who do not respond to diazoxide or surgery, showing a 93% success rate in managing blood glucose levels.
A study analyzing 122 CHI patients indicated common side effects, including gastrointestinal issues and transient hyperglycemia, with a higher rate of adverse events linked to increased octreotide doses.
The findings suggest that using intermediate doses of octreotide may reduce the risk of adverse effects, highlighting the need for careful patient monitoring during treatment, particularly at higher dosage levels.
Diazoxide (DZX) is the first-line treatment for hyperinsulinemic hypoglycemia (HH), but about 40%-50% of HH cases have unknown genetic causes.
A case study reports a neonate experiencing DZX hypersensitivity linked to a novel mutation inherited from the mother, requiring a very low DZX dosage for effective treatment.
Close monitoring is essential when starting DZX to prevent complications like diabetic ketoacidosis and hyperglycemic hyperosmolar state.
Introduction: Hyperinsulinemic hypoglycaemia (HH) is characterised by unregulated insulin secretion, leading to persistent non-ketotic hypoglycaemia with a lack of alternate fuel that induces a severe risk for brain damage and neurodevelopmental abnormalities. Octreotide, a somatostatin analogue, has been effectively administered as subcutaneous injections or depot preparations in diazoxide-unresponsive HH.
Methods: Children and infants with HH receiving short-acting octreotide injections were included.
Diazoxide is the only medication approved by the United States Food and Drug Administration for the treatment of hyperinsulinism-induced hypoglycemia. Overdose is infrequently reported. This case describes a preterm four-week-old male who was prescribed diazoxide and chlorothiazide for perinatal stress-induced hyperinsulinism.
