Abstract

ORTEGA-VARGAS, Adrián J. et al. Persistent congenital hyperinsulinemic hypoglucemia, case report. Perinatol. Reprod. Hum. [online]. 2021, vol.35, n.1, pp.31-40.  Epub June 06, 2022. ISSN 2524-1710.  https://doi.org/10.24875/per.19000052.

The placenta and liver are responsible for the metabolism of carbohydrates. The glucose is fundamental for brain metabolism. Hypoglycaemia is defined as values < 47 mg/dl. Hypoglycaemia that persists for more than 7 days is attributed to metabolic or endocrine problems and requires glucose flow > 12 GKM to reach normoglycemia. Persistent congenital hyperinsulinemic hypoglycemia (PCHH) is uncommon (1:50,000 live births) is the most common cause of persistent hypoglycemia secondary to inadequate insulin secretion, can significantly affect neurodevelopment. There is a diffuse and a focal form, with identical clinical manifestations, but with different pathological mechanisms. The medical treatment is diazoxide and ocreotide. In 95% of cases there is no response to medical treatment, requiring subtotal pancreatectomy. Ocreotide and nifedipine were used. Positron emission computed tomography (PET/CT 18F-DOPA) found an increase in pancreatic insulin capacity, a pancreactectomy was performed. He was discharged without complications and in pediatric follow-up without neurological alterations.

Keywords : Persistent congenital hyperinsulinemic hypoglycemia; Subtotal pancreatectomy; PET/CT 18F-DOPA.