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54th Annual ESPE

Barcelona, Spain
01 Oct 2015 - 03 Oct 2015

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Barcelona, Spain; 1-3 October 2015 Further information

Free Communications

Beta cell disorders

hrp0084fc9.1 | Beta cell disorders | ESPE2015

Islet δ-Cells Contribute to the Pathobiology of Atypical Congenital Hyperinsulinism

Han Bing , Bourke Siobahn , Mohammad Zainab , Craigie Ross , Skae Mars , Cheeseman Edmund , Banerjee Indi , Cosgrove Karen , Dunne Mark

Background: Atypical forms of congenital hyperinsulinism in infancy (CHI-A) represent a novel subgroup of patients who present later in the neonatal period; have poor responses to medical intervention; an unremarkable histopathology and no known genetic cause of disease.Objective and hypotheses: To compare the expression profiles of insulin and somatostatin in islets from patients with CHI-A, diffuse CHI (CHI-D) and age-matched control tissue.<p clas...

hrp0084fc9.2 | Beta cell disorders | ESPE2015

A Novel Source of Mesenchymal Stem Cells Lines from the Human Neonatal Pancreas of Patients with Congenital Hyperinsulinism in Infancy

Kellaway Sophie , Mosinska Karolina , Han Bing , Mohammad Zainab , Rigby Lindsey , Skae Mars , Padidela Raja , Banerjee Indi , Cosgrove Karen , Dunne Mark

Background: Congenital hyperinsulinism in infancy (CHI) is a neonatal disorder of uncontrolled insulin release leading to profound hypoglycaemia. In addition to defects in pancreatic β-cell function, we have recently demonstrated that the CHI pancreas is highly proliferative, with rates of proliferation up to 14-fold higher than in age-matched controls.Objective and hypotheses: As patients require pancreatectomy to alleviate hypoglycaemia, our aim w...

hrp0084fc9.3 | Beta cell disorders | ESPE2015

Failure to Terminate Cell Proliferation Contributes to the Pathobiology of Congenital Hyperinsulinism in Infancy

Han Bing , Mohammad Zainab , Rigby Lindsey , Craigie Ross , Skae Mars , Padidela Raja , Cheesman Edmund , Cosgrove Karen , Banerjee Indi , Dunne Mark

Background: Diffuse congenital hyperinsulinism in infancy (CHI-D) mainly arises from mutations in KATP channel genes. In addition, there are also several reports of increased cell proliferation in CHI-D. We hypothesised that the higher rates of proliferation in CHI-D are as a consequence of failure to terminate proliferation in the neonatal period.Objective and hypotheses: To test this we examined the proliferative index (PI) of CHI-D tissue a...

hrp0084fc9.4 | Beta cell disorders | ESPE2015

Novel Molecular Mechanisms of Congenital Hyperinsulinism due to Autosomal Dominant Mutations in ABCC8

Nessa Azizun , Aziz Qadeer , Thomas Alison , Harmer Stephen , Flanagan Sarah , Ellard Sian , Kapoor Ritika , Tinker Andrew , Hussain Khalid

Background: Dominant mutations in ABCC8 can cause congenital hyperinsulinism (CHI), which is characterised by unregulated insulin secretion.Objective and hypotheses: To understand the molecular basis of medically unresponsive CHI due to dominant ABCC8 mutations.Method: We investigated ten patients with diazoxide unresponsive CHI who required a near total pancreatectomy. DNA sequencing revealed seven dominant heter...

hrp0084fc9.5 | Beta cell disorders | ESPE2015

The Use of Glucagon for Management of Severe-Persistent Hypoglycaemia in Patients with Congenital Hyperinsulinism

Thornton Paul , Truong Lisa , Kinzel John , Empting Susann , Mohnike Klaus , Banerjee Indi

Background: Severe-persistent hypoglycaemia (SPH) in congenital hyperinsulinism (HI) can cause blindness and brain damage. First line treatment with diazoxide treatment can cause significant side effects, including fluid retention. Off-label use of i.v. reconstituted glucagon is also used but little safety and efficacy data have been reported.Objective and hypotheses: To evaluate the use of i.v. glucagon infusion for management of SPH in HI.<p class=...

hrp0084fc9.6 | Beta cell disorders | ESPE2015

Pharmacokinetics of a New Suspension of Glibenclamide for Use in Young Patients and Infants with Neonatal Diabetes

Beltrand Jacques , Busiah Kanetee , Berdugo Marianne , Treluyer Jean-Marc , Elie Caroline , Polak Michel

Background: Sulfonylurea therapy allows a better metabolic control than insulin in patients with neonatal diabetes secondary to mutation in potassium channel. Its galenic form (tablets) is not suitable for children, as the dosage can’t be easily modulated and as it induces large pharmacokinetics (PK) variations when administer to young children.Objective and hypotheses: To measure relative biodisponibility of a new galenic form of glibenclamide and ...