Hyperinsulinemic Hypoglycemia in Beckwith-Wiedemann Syndrome due to Defects in the Function of Pancreatic β-Cell Adenosine Triphosphate-Sensitive Potassium Channels | Zendy

Khalid Hussain | Zendy; Karen E. Cosgrove | Zendy; Ruth M. Shepherd | Zendy; Anita Luharia | Zendy; V. V. Smith | Zendy; Sameer Kassem | Zendy; John W Gregory | Zendy; Asipu Sivaprasadarao | Zendy; Henrik Thybo Christesen | Zendy; Bendt Brock Jacobsen | Zendy; Klaus Brusgaard | Zendy; Benjamin Gläser | Zendy; E. A. Maher | Zendy; Keith Lindley | Zendy; Peter C. Hindmarsh | Zendy; Mehul Dattani | Zendy; Mark J. Dunne | Zendy

Open Access

Hyperinsulinemic Hypoglycemia in Beckwith-Wiedemann Syndrome due to Defects in the Function of Pancreatic β-Cell Adenosine Triphosphate-Sensitive Potassium Channels

Author(s) -

Khalid Hussain,

Karen E. Cosgrove,

Ruth M. Shepherd,

Anita Luharia,

V. V. Smith,

Sameer Kassem,

John W Gregory,

Asipu Sivaprasadarao,

Henrik Thybo Christesen,

Bendt Brock Jacobsen,

Klaus Brusgaard,

Benjamin Gläser,

E. A. Maher,

Keith Lindley,

Peter C. Hindmarsh,

Mehul Dattani,

Mark J. Dunne

Publication year - 2005

Publication title -

the journal of clinical endocrinology and metabolism

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 2.206

H-Index - 353

eISSN - 1945-7197

pISSN - 0021-972X

DOI - 10.1210/jc.2005-0158

Subject(s) - hyperinsulinemic hypoglycemia , congenital hyperinsulinism , hyperinsulinism , medicine , hypoglycemia , endocrinology , uniparental disomy , beckwith–wiedemann syndrome , sulfonylurea receptor , insulin , biology , diabetes mellitus , insulin resistance , genetics , chromosome , karyotype , glibenclamide , gene , gene expression , dna methylation

Beckwith-Wiedemann syndrome (BWS) is a congenital overgrowth syndrome that is clinically and genetically heterogeneous. Hyperinsulinemic hypoglycemia occurs in about 50% of children with BWS and, in the majority of infants, it resolves spontaneously. However, in a small group of patients the hypoglycemia can be persistent and may require pancreatectomy. The mechanism of persistent hyperinsulinemic hypoglycemia in this group of patients is unclear.

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