Persistent neonatal hyperinsulinism. Analysis of the differential diagnosis. Report of two cases

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 pr...

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Bibliographic Details
Published in:Revista medíca de Chile 2004-08, Vol.132 (8), p.995
Main Authors: Hernández, M Isabel, Hodgson, M Isabel, Cattani, Andreina
Format: Article
Language:Spanish
Subjects:
Congenital Hyperinsulinism - diagnosis
Congenital Hyperinsulinism - surgery
Diagnosis, Differential
Female
Humans
Hyperplasia - diagnosis
Infant, Newborn
Male
Pancreas - pathology
Pancreatectomy
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Summary: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 bad 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.
ISSN:0034-9887