Anales de pediatria (Barcelona, Spain : 2003) · 2006
[Congenital hyperinsulinism. Review of 22 cases].
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Guerrero-Fernández J, González Casado I, Espinoza Colindres L, Gracia Bouthelier R
Аннотация
INTRODUCTION: 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.
MATERIAL AND 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.
OBJECTIVE: To review the presentation, management and outcome of patients with CHI attended at our hospital.
MATERIAL AND 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.