Searchable abstracts of presentations at key conferences in endocrinology

ea0039ep83 | Miscellaneous/other | BSPED2015

Determination of pancreatic hormones in children with different forms of hyperinsulinaemic hypoglycaemia

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

Introduction: In congenital hyperinsulinism (CHI) hypoglycaemia results from a dysregulation of insulin secretion. We hypothesised that other pancreatic hormones may also be dysregulated in this condition.Objectives: To proof the applicability of Luminex Multiplex method to measure pancreatic hormones (insulin, C-peptide, glucagon, amylin and PP) in the paediatric age. To elucidate the fasting response of these hormones in children with different forms o...

ea0051p035 | Miscellaneous/other | BSPED2017

Post-prandial hyerinsulinaemic hypoglycaemia post-esophageal surgery in children

Malhotra Neha , Dastamani Antonia , Guemes Maria , Gilbert Clare , Ress Clare , Dattani Mehul , Shah Pratik

Introduction: Post-prandial hyerinsulinaemic hypoglycaemia (PPHH) or dumping is a recognized complication of various gastric surgeries. There are very few paediatric case reports to confirm PPHH post esophageal repair. We here report two cases who presented with dumping syndrome after a variable time period post esophageal atresia repair and response to medications.Case 1: A 6 month old female diagnosed with Wolf-Hirschhorn syndrome, born at 38+3 weeks b...

ea0033oc3.4 | Oral Communications 3 | BSPED2013

Successful Use of Long Acting Octreotide in Treatment of Congenital Hyperinsulinism

Shah Pratik , Gilbert Clare , Morgan Kate , Hinchey Louise , Senniappan Senthil , Arya Ved , Levy Hannah , Hussain Khalid

Introduction/Aim: Congenital hyperinsulinism (CHI) is a cause of severe hypoglycaemia in infancy. Treatment of diazoxide unresponsive patients includes the use of somatostatin analogues (octreotide given either as four s.c. injections daily or via a pump). We aimed to evaluate the use of a long acting somatostatin analogue (Lanreotide) in children with CHI, switching them from daily oral diazoxide or s.c. Octreotide injections to 4 weekly Lanreotide injections.<p class="ab...

ea0033p23 | (1) | BSPED2013

Can we prevent hypoglycaemic brain injuries in term babies with no risk factors of Hyperinsulinaemic Hypoglycaemia?

Gilbert Clare , Morgan Kate , Hinchey Louise , Shah Pratik , Kumaran Anitha , Hussain Khalid

Introduction: Hyperinsulinaemic hypoglycemia (HH) represents the most common cause of hyperinsulinism in neonates, often termed as congenital hyperinsulinism of infancy (CHI). CHI is characterised by inappropriate raised insulin secretion from the pancreatic β-cells in relation to blood glucose concentration. Insulin suppresses NEFA and BOHB production. Neurological damage is a known risk associated with hyperinsulinaemic hypoglycaemia (HH).Aim: To ...

ea0045oc6.2 | Oral Communications 6- Endocrine | BSPED2016

Systematic trial of Nifedipine in children with Hyperinsulinaemic Hypoglycaemia due to mutations in the ABCC8 gene

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

Introduction: Several previous case reports have described the use of the calcium-blocker Nifedipine for the treatment of hyperinsulinaemic hypoglycaemia (HH). These cases are a collection of transient/permanent forms of HH, with known/unknown genetics, where Nifedipine has been used either as monotherapy or in combination with other medications. There have been no previous reports of any systematic trial of Nifedipine use in patients with HH due to mutations in the ABCC8<...

ea0095p136 | Miscellaneous/other 2 | BSPED2023

Aortic valve disease in two females with congenital hyperinsulinism due to activating GCK mutation

Alhusaini Fatemah , Dastamani Antonia , Gilbert Clare , Flanagan Sarah , Alhomaidah Doha , Morgan Kate

Introduction: Activating mutations of Glucokinase (GCK) gene are described as a rare genetic aetiology of Congenital Hyperinsulinism (CHI), which can cause variable disease severity. However, cardiac anomalies such as aortic valve disease have not been reported as a feature of this genetic form of CHI. We describe two patients diagnosed with GCK-CHI and aortic valve disease.Case 1: A twelve-month-old female presented at ...

ea0033p24 | (1) | BSPED2013

Normoammonaemic Protein Sensitive Hyperinsulinaemic Hypoglycaemia: ? A novel syndrome

Arya Ved Bhushan , Heslegrave Amanda , Shah Pratik , Gilbert Clare , Morgan Kate , Hinchey Louise , Flanagan Sarah E. , Ellard Sian , Hussain Khalid

Introduction: Hyperinsulinaemic hypoglycaemia (HH), characterized by unregulated insulin secretion from pancreatic β-cells, is an important cause of hypoglycaemia in children. Mutations in the KATP channel genes (ABCC8/KCNJ11) are the most common cause of congenital HH. The second common cause, hyperinsulinism hyperammonaemia (HIHA) syndrome caused by mutations in GLUD1 gene, is associated with elevated serum ammonia and protein sensitivity. W...

ea0058oc4.7 | Oral Communications 4 | BSPED2018

Clinical outcomes of focal congenital hyperinsulinism – a UK perspective

Dastamani Antonia , Yau Daphne , Gilbert Clare , Morgan Kate , O'Shea Elaine , Pimlott Helen , DeCoppi Paolo , Craigie Ross , Flanagan Sarah , Houghton Jayne , Senniappan Senthil , Didi Mohammed , Banerjee Indi , Shah Pratik

Background: The focal type of Congenital Hyperinsulinism (CHI) is characterized by a cluster of abnormal insulin over-secreting β-cells within a restricted area of the pancreas. Early identification and intervention of the focal lesion is critical in CHI management, preventing both acute and chronic complications.Objective: The purpose of this study is to review outcomes of treatment response in focal CHI.Design: Retr...

ea0033p20 | (1) | BSPED2013

18F-DOPA PET and enhanced CT imaging for congenital hyperinsulinism: Our experience of using oral sedation

Shah Pratik , Senniappan Senthil , du Preez Marguerite , Endozo Raymond , Townsend Caroline , Gilbert Clare , Morgan Kate , Hinchey Louise , Pierro Agostino , Biassoni Lorenzo , Olsen Oystein , Bomanji Jamshed , Hussain Khalid

Introduction: Congenital hyperinsulinism (CHI) is the most common cause of persistent hypoglycaemia in infants and children. Histologically there are two subgroups, diffuse and focal. Fluorine-18-L dihydroxyphenylalanine positron emission tomography (18F-DOPA PET/CT) helps to differentiate focal from diffuse CHI.Objective and hypotheses: To evaluate the feasibility of using 18F-DOPA PET/CT for the diagnosis of focal or diffuse CHI u...

ea0039p4 | (1) | BSPED2015

Use of long acting somatostatin analogue (Lanreotide) in CHI – its pharmacokinetics and long-term follow-up study

Shah Pratik , Rahman Sofia , McElroy Sharon , Gilbert Clare , Morgan Kate , Hinchey Louise , Button Roberta , Margetts Rebecca , Senniappan Senthil , Guemes Maria , Levy Hannah , Morte Marti Carles , Celma Lezcano Carles , Amin Rakesh , Hussain Khalid

Background: CHI is a cause of severe hypoglycaemia in children. Diazoxide (KATP channel agonist) is used as first-line treatment but is known to cause severe hypertrichosis and reduced appetite in children. Diazoxide unresponsive CHI us treated with daily octreotide subcutaneous injections (3–4 times/day).Objective and hypotheses: To evaluate the efficacy, safety and pharmacokinetics of long acting Somatostatin analogue (Lanreotide) thera...