hrp0084p3-1068 | Hypo | ESPE2015

Pancreatic Hormones in Children with Hyperinsulinaemic Hypoglycaemia

Guemes Maria , Gilbert Clare , Morgan Kate , Rahman Sofia , Hussain Khalid

Background: In congenital hyperinsulinism (CHI) there is dysregulation of insulin secretion that leads to hypoglycaemia. It is possible that other pancreatic hormones are also dysregulated in CHI.Objectives: i) To verify the utility of Luminex Multiplex to determine pancreatic hormones in the paediatric age. ii) To investigate the response of pancreatic hormones (insulin, C-peptide, glucagon, amylin and PP) to a fast in children with CHI due to different...

hrp0092p1-205 | Fetal, Neonatal Endocrinology and Metabolism (to include Hypoglycaemia) (1) | ESPE2019

Central Hypoventilation Syndrome and Hyperinsulinameic Hypoglycaemia

Dastamani Antonia , Yau Daphne , Gilbert Clare , Morgan Kate , Banerjee Indie , Shah Pratik

Objectives: Congenital central hypoventilation syndrome (CCHS) is a rare autosomal dominant condition due to mutations in the transcription factor PHOX2B. It is characterized by alveolar hypoventilation with symptoms of autonomic nervous system dysfunction and both hyperglycaemia as well as hyperinsulinaemic hypoglycaemia (HH) have been reported. Although the mechanism is unclear, autonomic dysfunction may underlie this dysregulation of glucose homeos...

hrp0089fc7.2 | Fetal, Neonatal Endocrinology and Metabolism | ESPE2018

Enteroinsular Hormone Responses During Fasting, Oral glucose Tolerance Test and Mixed Meal in Children with Hyperinsulinaemic Hypoglycaemia

Guemes Maria , Rahman Sofia , Morgan Kate , Gilbert Clare , Shah Pratik , Hussain Khalid

Objective(s): To understand how plasma concentrations of pancreatic (glucagon, amylin, pancreatic polypeptide (PP), insulin) and gut hormones ‘incretins’ (Glucagon-like peptide 1 (GLP-1) and Glucose dependent insulinotropic peptide (GIP)) change in relation to fasting and feeding (different types of nutrients) in healthy and hyperinsulinaemic hypoglycaemia (HH) children of different ages.Methods: Plasma pancreatic and incretin hormone concentra...

hrp0086p1-p556 | Perinatal Endocrinology P1 | ESPE2016

Pancreatic Glucagon Secretion is Severely Impaired and Somatostatin Secretion Unchanged in Patients with Hyperinsulinaemic Hypoglycaemia

Shah Pratik , Rahman Sofia , Gilbert Clare , Morgan Kate , Hinchey Louise , Bech Paul , Amin Rakesh , Hussain Khalid

Background: Hyperinsulinaemic hypoglycaemia (HH) is a common cause of hypoglycaemia in children. Glucagon is an important counter-regulatory hormone and the role of somatostatin is not known in children with HH.Objective and hypotheses: To understand the roles of glucagon and somatostatin in children with HH.Method: Children admitted for management of HH in our hospital were included in the study. Plasma insulin, glucagon and somat...

hrp0086p1-p562 | Perinatal Endocrinology P1 | ESPE2016

Effectiveness of Calcium Channel Blocker Nifedipine in Children with Hyperinsulinaemic Hypoglycaemia Due to Genetically Proven Mutations in the ABCC8/KCNJ11/GCK Genes

Guemes Maria , Shah Pratik , Silvera Shavel , Morgan Kate , Gilbert Clare , Hinchey Louise , Hussain Khalid

Background: Several previous publications have documented the usefulness of Nifedipine for treating hyperinsulinaemic hypoglycaemia (HI). These reports include transient and persistent forms of HI, with and without known genetics, used in monotherapy or in combination with other drugs, and demonstrate various outcomes.Objective and hypotheses: To systematically trial Nifedipine in children with known HI mutations and diazoxide unresponsive, assessing gly...

hrp0082p2-d3-482 | Hypoglycaemia | ESPE2014

Feeding Issues in Children With Congenital Hyperinsulinism

Gilbert Clare , Morgan Kate , Hinchey Louise , Margetts Rebecca , Forsyth Alex , Ryan Martina , Shah Pratik , Hussain Khalid

Background: Congenital hyperinsulinism (CHI) is the most common cause of persistent hypoglycaemia in the neonatal period, characterized by unregulated insulin secretion by pancreatic β-cells. In addition to medical therapy, frequent feeding to prevent hypoglycaemia is one of the most important aspects in the management of CHI.Objective and hypotheses: To identify the number of patients with CHI who have associated feeding difficulties and determine ...

hrp0094p1-65 | Diabetes B | ESPE2021

Efficacy of Use of Continuous Glucose Monitoring System in patients with Congenital Hyperinsulinism

Tropeano Yesica , Purushothaman Preetha , Gilbert Clare , Morgan Kate , Doodson Louise , Dastamani Antonia ,

Background: Hypoglycaemic episodes in patients with Congenital Hyperinsulinism (CHI) are not always detected due to the intermittent measurement of blood glucose concentrations, particularly the asymptomatic episodes. Detecting and treating these episodes is important for prevention of hypoglycaemic brain injury. Although the Continuous Glucose Monitoring System (CGMS) is widely used in adults and children with diabetes, the use in patients with CHI remains li...

hrp0089fc7.1 | Fetal, Neonatal Endocrinology and Metabolism | ESPE2018

Expression and Localisation of Insulin, Glucagon, Amylin, Pancreatic Polypeptide and PDX-1 in Pancreatic Tissue of Children with Congenital Hyperinsulinism

Guemes Maria , Rahman Sofia , Solanky Nita , Gilbert Clare , Morgan Kate , Shah Pratik , Hussain Khalid

Backgound: There is insufficient knowledge about the characterisation of insulin, glucagon, amylin, pancreatic polypeptide (PP) and Pancreas/Duodenum Homeobox Protein 1 (PDX-1) in pancreatic tissue of children with diffuse (DCHI) and focal (FCHI) congenital hyperinsulinism (CHI).Objective(s): To understand the expression profile and localisation of insulin, glucagon, amylin, PP and PDX-1 in pancreatic tissue of children with DCHI and FCHI.<p class="a...

hrp0086rfc10.4 | Perinatal Endocrinology | ESPE2016

Pharmacokinetics of Intravenous Glucagon in Children with Hyperinsulinaemic Hypoglycaemia

Shah Pratik , Rahman Sofia , Gilbert Clare , Morgan Kate , Hinchey Louise , Bech Paul , Amin Rakesh , Hussain Khalid

Background: Hyperinsulinaemic hypoglycaemia (HH) is one of the common causes of hypoglycaemia in infants and children. It can cause severe brain injury in children if not treated promptly. Diazoxide is first-line treatment for HH. Glucagon infusion is used in the management of children with HH. However it is unclear what dose of glucagon should be used in children.Objective and hypotheses: To evaluate the efficacy, safety and pharmacokinetics of intraven...

hrp0086p1-p566 | Perinatal Endocrinology P1 | ESPE2016

Challenging Management of Costello Syndrome with Severe Congenital Hyperinsulinaemic Hypoglycaemia

Guemes Maria , Shah Pratik , Hinchey Louise , Gilbert Clare , Morgan Kate , Silvera Shavel , Hussain Khalid

Background: Costello syndrome may be associated with Hyperinsulinaemic Hypoglycaemia (HI), but this is usually a mild medically-responsive form.Objective and hypotheses: To describe the clinical characteristics, biochemical findings and challenging management of a case of Costello syndrome with severe HI.Method: Review of the patient’s medical records.Results: Male, born to non-consanguineous healthy par...