Revista médica de Chile
versión impresa ISSN 0034-9887
HERNANDEZ C, M Isabel; HODGSON B, M Isabel y CATTANI O, Andreina. Persistent neonatal hyperinsulinism: Report of two cases . Rev. méd. Chile [online]. 2004, vol.132, n.8, pp. 995-1000. ISSN 0034-9887. doi: 10.4067/S0034-98872004000800013.
Persistent neonatal hyperinsulinism is the most common cause of refractory hypoglycemia during the first year of life. Inadequate insulin secretion is associated to mutations of four different genes, that can be diagnosed to orient patient management. We report two patients: a female newborn that presented a hypoglycemia of 16 mg/dl two hours after birth, was subjected to a subtotal pancreatectomy that did not correct hypoglycemia, requiring a total pancreatectomy. Pathological study of the pancreas showed a focal adenomatous hyperplasia. At the present time, she is three years of age and maintains euglycemia with fractionated feeding. A male newborn that had seizures at 28 hours of life and a hypoglycemia of 15 mg/dl was detected. He was also subjected to a subtotal pancreatectomy, that did not correct hypoglycemia and had to be extended to a total pancreatectomy. At the present time, he is 3 years and 11 months of age and has a normal psychomotor development (Rev Méd Chile 2004; 132: 995-1000)
Palabras clave : Endogenous hyperinsulism; Hyperinsulism; Hypoglicemia.