Neurologic outcomes of 90 neonates and infants with persistent hyperinsulinemic hypoglycemia.
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.


CONCLUSIONS
Neonatal hyperinsulinemic hypoglycemia is still a severe disease with an important risk to rapidly develop severe mental retardation and epilepsy.
Menni
F
F
Fédération de Pédiatrie, Diabète-Métabolisme-Neurologie, Centre Hospitalier Universitaire Necker-Enfants Malades, Paris, France.
de Lonlay
P
P
Sevin
C
C
Touati
G
G
Peigné
C
C
Barbier
V
V
Nihoul-Fékété
C
C
Saudubray
J M
JM
Robert
J J
JJ
D017668:Age of Onset; D002648:Child; D002675:Child, Preschool; D002658:Developmental Disabilities / Q000175:diagnosis / Q000209:etiology*; D003981:Diazoxide / Q000627:therapeutic use; D004827:Epilepsy / Q000209:etiology*; D006801:Humans; D006946:Hyperinsulinism / Q000150:complications* / Q000628:therapy; D007003:Hypoglycemia / Q000150:complications* / Q000628:therapy; D007004:Hypoglycemic Agents / Q000627:therapeutic use; D007223:Infant; D007231:Infant, Newborn; D009460:Neurologic Examination; D010180:Pancreatectomy; D011596:Psychomotor Disorders / Q000209:etiology*; D012189:Retrospective Studies
D007004:Hypoglycemic Agents; D003981:Diazoxide