[Congenital hyperinsulinism. Review of 22 cases].
BACKGROUND
Congenital hyperinsulinism (CHI) is the most common cause of recurrent episodes of hypoglycemia in early childhood and consists of a group of distinct genetic disorders causing dysregulation of insulin secretion.


OBJECTIVE
To review the presentation, management and outcome of patients with CHI attended at our hospital.


METHODS
A retrospective review of all patients diagnosed with CHI between 1982 and 2004 was performed. Data were collected on age, gender, clinical presentation, medical and surgical management, and complications.


RESULTS
Twenty-two patients were identified. Notable features were early symptom onset in 80 %, pancreatectomy in 72 %, and neurological sequels in 28 % (abnormal neurodevelopment in 22 % and epilepsy in 13 %).


CONCLUSIONS
The presentation, management and outcome in our patients were similar to those in other series, indicating the need for early diagnosis and treatment to avoid neurological sequels.
Guerrero-Fernández
J
J
Servicio de Endocrinología Pediátrica, Hospital Infantil La Paz, Madrid, España.
González Casado
I
I
Espinoza Colindres
L
L
Gracia Bouthelier
R
R
D044903:Congenital Hyperinsulinism / Q000175:diagnosis / Q000628:therapy; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male