The authors report the cases of two patients with neonatal onset of hypoketotic hypoglycemia with hyperinsulinism and a poor response to diazoxide. Pancreatic venous sampling showed a diffuse pancreatic hyperplasia in one patient and a focal lesion in the other. The second patient, diagnosed after a significant delay, suffered severe long-term neurological sequelae, despite having more limited hyperplasia; the brain MRI also showed severe pathological changes in cortex and white matter, predominantly in the parieto-occipital region. Early and accurate diagnosis is critical in these patients in whom hypoglycemia, is compounded by a lack of the ketone bodies which represent a vital alternative source of energy for the central nervous system.

Different neurologic outcomes in two patients with neonatal hyperinsulinemic hypoglycemia

Filippi L.;
1996

Abstract

The authors report the cases of two patients with neonatal onset of hypoketotic hypoglycemia with hyperinsulinism and a poor response to diazoxide. Pancreatic venous sampling showed a diffuse pancreatic hyperplasia in one patient and a focal lesion in the other. The second patient, diagnosed after a significant delay, suffered severe long-term neurological sequelae, despite having more limited hyperplasia; the brain MRI also showed severe pathological changes in cortex and white matter, predominantly in the parieto-occipital region. Early and accurate diagnosis is critical in these patients in whom hypoglycemia, is compounded by a lack of the ketone bodies which represent a vital alternative source of energy for the central nervous system.
Zammarchi, E.; Filippi, L.; Fonda, C.; Benedetti, P. A.; Pistone, D.; Donati, M. A.
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11568/1072153
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