publication . Article . 2006

Diagnosis and management of congenital hyperinsulinism: a case report

Wilma Oostdijk; Boudewijn Bakker;
Open Access
  • Published: 30 Oct 2006 Journal: European Journal of Endocrinology, volume 155, pages S153-S155 (issn: 0804-4643, eissn: 1479-683X, Copyright policy)
  • Publisher: Bioscientifica
Abstract
Congenital hyperinsulinism (CHI) is a common cause of persistent hypoglycaemia in neonates, with an estimated incidence of one in 50 000 live births (1). The molecular basis for CHI can be established in approximately 50% of the cases and is categorised as channelopathies – defects in the ATP-sensitive potassium (KATP) channels that regulate insulin secretion, and metabolopathies – defects in metabolic pathways altering b-cell function (2). Channelopathies form the largest group and can be further sub-divided into focal and diffuse forms. In contrast to metabolopathies, most channelopathies do not respond to pharmacological treatment and require surgical removal...
Subjects
free text keywords: Endocrinology, Diabetes and Metabolism, Endocrinology, General Medicine, Pharmacological treatment, Surgical removal, Hyperinsulinemia, medicine.disease, medicine, Insulin secretion, medicine.medical_specialty, Congenital hyperinsulinism, Background information, Pancreatectomy, medicine.medical_treatment, Internal medicine, business.industry, business
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