title: [Congenital hyperinsulinism. Review of 22 cases].
abstract: 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.
authors.affiliation: Servicio de Endocrinología Pediátrica, Hospital Infantil La Paz, Madrid, España.
authors.forename: J
authors.initials: J
authors.lastname: Guerrero-Fernández
authors.forename: I
authors.initials: I
authors.lastname: González Casado
authors.forename: L
authors.initials: L
authors.lastname: Espinoza Colindres
authors.forename: R
authors.initials: R
authors.lastname: Gracia Bouthelier
mesh_terms: D044903:Congenital Hyperinsulinism / Q000175:diagnosis / Q000628:therapy; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male