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43rd Meeting of the British Society for Paediatric Endocrinology and Diabetes



ea0039ep79 | Miscellaneous/other | BSPED2015

The not so sweet truth of paediatric hypoglycaemia

Ramsden Louise , Wright Katherine , Natarajan Anuja

Introduction: Paediatric hypoglycaemia is a relatively common medical emergency. In order to allow identification of the underlying cause, investigations need to be performed urgently prior to treatment being given. If done correctly this can save the need for future investigations. A ‘hyposcreen’ costs ~£450 so careful consideration is needed to ensure correct patient selection, as inadequate investigations have further cost and patient safety implications....

ea0039ep80 | Miscellaneous/other | BSPED2015

Volumetric changes in the hippocampus and relationship to memory indices in children with hyperinsulinaemic hypoglycaemia and ketotic hypoglycaemia

Kumaran Anitha , Bullock Jemima , Kapoor Ritika , Chong Kling , Gadian David , Vargha-Khadem Faraneh , Hussain Khalid

Background: Children with hyperinsulinaemic hypoglycaemia (HH) are at a high risk of brain injury, while children with ketotic hypoglycaemia (KH) are believed to be neurologically normal. Hippocampus is known to be susceptible to hypoglycaemia, and is one of the key structures in the memory system. Our objective was to ascertain if children with HH sustain greater hippocampal injury and memory deficits in comparison to children with KH.Methods: Twenty on...

ea0039ep81 | Miscellaneous/other | BSPED2015

Pilot study on the utility and acceptability of video animation as a delivery method for educational materials for families and carers of patients with congenital hyperinsulinism in infancy

Rodgers Elizabeth , Rigby Lindsey , Dunne Mark , Banerjee Indraneel , Cosgrove Karen

Introduction: To prevent neurological damage caused by congenital hyperinsulinism (CHI), hypoglycaemia must be avoided and treated promptly. Education of parents, carers and families of patients with CHI (PCFs) about the causes and consequences of CHI may help to reduce severity of hypoglycaemia due to earlier correction of blood glucose levels. We aimed to determine whether video animations could be used to improve understanding of CHI among PCFs, and the acceptability of thi...

ea0039ep82 | Miscellaneous/other | BSPED2015

Development of a feasible intervention to support communication with young people

Callery Peter , Downing Jenny , Gleeson Helena , Clayton Peter , Davis Julian , Dimitri Paul , Wales Jerry , Young Bridget

Background: Many young people have inadequate follow up because they lose contact with adult care following transfer from paediatrics. There is a need to adapt communication interventions to help young people to determine and enact their preferred involvement in consultations with health professionals.Objective: To develop interventions to support communication with young people in endocrine care and to assess the feasibility of implementation in routine...

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...

ea0039ep84 | Miscellaneous/other | BSPED2015

Congenital hyperinsulinism due to SUR1 (ABCC8) mutation in newborn twins: improvement of clinical outcome after eight years follow-up

Ersoy Betul , Tansug Nermin , Genc Abdulkadir , Kizilay Deniz , Kiremitci Semiha , Ayhan Semin , Lonlay Pascale D E

Introduction: Congenital hyperinsulinism (CHI), is the most frequent cause of persistent hypoglycemia in infancy. Mutations in the ABCC8 gene are responsible for 40–50% of CHI cases. Its management can be extremely complicated. The main goal of the treatment is to maintain normoglycemia, since hypoglycemia during infancy can have severe neurological consequences. Herein, we report 8 year follow up of twin patients who were diagnosed with CHI at neonatal period due to SUR1...

ea0039ep85 | Miscellaneous/other | BSPED2015

Digenic mutation resulting in a rare form of diazoxide responsive congenital hyperinsulinism

Giri Dinesh , Flanagan Sarah E , Ellard Sian , Didi Mohammed , Senniappan Senthil

Introduction: Congenital hyperinsulinism (CHI) results from unregulated insulin secretion from pancreatic β-cells, which leads to persistent hypoglycaemia. Mutations in nine different genes are reported and phenotypic variability exists both within and between the genetic subgroups. Variable penetrance has been described in some families with the same mutation; for example HNF4A mutations cause neonatal hypoglycaemia and/or maturity onset diabetes of the young (M...

ea0039ep86 | Miscellaneous/other | BSPED2015

The use of glucagon in the treatment of hypoglycaemia due to congenital hyperinsulinism

Jadawji Chandni , Estebanez Maria , Padidela Raja , Bowden Louise , Rigby Lindsey , Kinzell John , Cosgrove Karen , Dunne Mark , Banerjee Indraneel

Background: Congenital hyperinsulinism (CHI) can cause severe hypoglycaemia with consequent adverse neurodevelopment. Continuous glucagon infusion (CGI) through intravenous and subcutaneous routes has been utilised to achieve glycaemic stability, but the efficacy has not been reported systematically in a CHI cohort.Aim: We aimed to investigate the efficacy and safety profile of CGI in the management of hypoglycaemia due to CHI.Meth...

ea0039ep87 | Miscellaneous/other | BSPED2015

An incidental finding of an abdominopelvic macrocystic lymphangioma in a girl with Turners syndrome

Bhandari Jasjit Kaur , Nathwani Nisha

Background: Cystic lymphangiomata are rare benign tumours of childhood resulting from an abnormal development of the lymphatic system, most of which occur in the head and axillary region, referred to as cystic hygromas. Lymphangioma arising in the abdomen are particularly rare and the symptoms are variable. They usually affect boys and can be associated with specific genetic abnormalities, most notably Turners syndrome.Case: We report the case of a 14-ye...

ea0039ep88 | Miscellaneous/other | BSPED2015

Usefulness of bedside ketone testing in the evaluation of children with hypoglycaemia

Alsaffar Hussain , Phanse Supriya , Collingwood Catherine , Didi Mohammed , Senniappan Senthil

Introduction: Bedside blood ketone measurement has often been used in the management of diabetic ketoacidosis. However there is no available data on its reliability in the evaluation of hypoglycaemia in children. We aimed to assess the reliability of bedside ketones (beta-hydroxybutyrate (BHB)) in the evaluation of hypoglycaemia in children.Methods: We collected data on 20 children who had paired measurement of bedside and lab BHB at the end of a control...

ea0039ep89 | Miscellaneous/other | BSPED2015

Isolated postprandial hyperinsulinaemic hypoglycaemia in children

Guemes Maria , Melikyan Maria , Senniappan Senthil , Hussain Khalid

Introduction: Isolated postprandial hyperinsulinaemic hypoglycaemia (PPHH) in the paediatric age has been exceptionally reported in the literature.Objective: To describe the clinical and biochemical characteristics as well as the management of a cohort of children with isolated PPHH followed at a single tertiary paediatric centre.Subjects and methods: Six children (three males) were collected. The clinical characteristics, diagnosi...

ea0039ep90 | Miscellaneous/other | BSPED2015

Case of raised creatinine in a newborn with congenital hyperinsulinism: diazoxide induced acute kidney injury

Godinho Felvira , Lewin Rebecca , Park Julie , Losa Ignatius

Background: Congenital hyperinsulinism (CHI) is the result of unregulated insulin secretion from the pancreatic β-cells leading to severe hypoglycaemia.1 Diazoxide is effective in virtually all forms of CHI except in those due to recessive (and some dominant) inactivating mutations in ABCC8 and KCNJ11 and in patients with focal CHI.2 We report a case of CHI with acute kidney injury secondary to diazoxide.Case ...