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PEDIATRICS Vol. 107 No. 3 March 2001, pp. 476-479

Neurologic Outcomes of 90 Neonates and Infants With Persistent Hyperinsulinemic Hypoglycemia

Received Jan 20, 2000; accepted Jul 24, 2000.

Francesca Menni*, Pascale de Lonlay*, Caroline Sevin*, Guy Touati*, Catherine Peigné*, Valérie Barbier*, Claire Nihoul-FékétéDagger , Jean-Marie Saudubray*, and Jean-Jacques Robert*

From the * Fédération de Pédiatrie, Diabète-Métabolisme-Neurologie and Dagger  Clinique Chirurgicale Infantile, Centre Hospitalier Universitaire Necker-Enfants Malades, Paris, France.

Objective.  To evaluate the neurologic outcomes of neonates and infants suffering from persistent hyperinsulinemic hypoglycemia of infancy (PHHI).

Methods.  The neurologic development of 90 PHHI patients was studied retrospectively. Sixty-three patients were treated surgically and 27 were treated medically. Fifty-four patients were neonates, of whom 8 were treated medically and 46 were operated on (19 for a focal adenomatous hyperplasia and 27 for diffuse hyperinsulinism). Thirty-six patients had infancy-onset hyperinsulinism, of whom 19 were treated medically and 17 underwent pancreatectomy (10 patients for a focal adenomatous hyperplasia and 7 for diffuse hyperinsulinism).

Results.  Severe psychomotor retardation was found in 7 patients, 6 with neonatal-onset PHHI. Intermediate psychomotor disability existed in 12 patients; epilepsy existed in 16. Neonatal-onset was the main risk factor for severe retardation or epilepsy. Medically treated patients were less severely affected than those treated by surgery, and there was no difference between the diffuse and focal forms of hyperinsulinism.

Conclusion.  Neonatal hyperinsulinemic hypoglycemia is still a severe disease with an important risk to rapidly develop severe mental retardation and epilepsy.  Key words:  persistent hyperinsulinemic hypoglycemia of infancy, psychomotor retardation, epilepsy, pancreatectomy.




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