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46th Meeting of the British Society for Paediatric Endocrinology and Diabetes

Birmingham, UK
07 Nov 2018 - 09 Nov 2018

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Birmingham, UK - 7-9 November 2018

Oral Communications

Oral Communications 4

ea0058oc4.1 | Oral Communications 4 | BSPED2018

Hydrocortisone granules in capsules for opening: phase 3 trial in children with adrenal insufficiency and long-term safety data

Lewis Alexander , Neumann Uta , Wiegand Susanna , Krude Heiko , Digweed Dina , Voet Bernard , Ross Richard , Davies Madhu , Blankenstein Oliver

Introduction: Children with adrenal insufficiency requiring hydrocortisone rely on compounded adult medication. This study aimed to evaluate the absorption, palatability and safety of Alkindi® (hydrocortisone granules in capsules for opening).Methods: The phase 3 study was an open-label, single-dose study in a total of 24 children (aged 0–6 years) with adrenal insufficiency. Fasted children were given a single dose of Alkindi&#1...

ea0058oc4.2 | Oral Communications 4 | BSPED2018

Gene expression signatures in children with growth hormone deficiency (GHD) and Turner syndrome (TS) predict response to growth hormone

Clayton Peter , Stevens Adam , Murray Philip , Garner Terence

Background: Recombinant human growth hormone (r-hGH) is the primary therapeutic agent for disorders of growth including growth hormone deficiency (GHD) and Turner syndrome (TS). There is a high cost associated with treatment and existing methods to predict response (and hence alter management) can only account for 40–60% of the variance.Methods: GHD (n=71) and TS patients (n=43) were recruited as part of a study (PREDICT) on the lo...

ea0058oc4.3 | Oral Communications 4 | BSPED2018

Recommendations for management of paediatric phaeochromocytoma/paraganglioma (PCC/PGL): On behalf of the UK Paediatric PCC/PGL Guideline Development Group

Katugampola Harshini , Harrison Barney , Quek Samuel , Yadav Prateek , Spoudeas Helen , Marks Stephen

Background: Phaeochromocytoma/paraganglioma (PCC/PGL) are rare in children and young people (CYP) under 19 years of age. National registry data reveal an annual incidence between 0.2 and 0.3 per million in 5–9 and 10–14 year age groups respectively. Almost all result from a genetic predisposition and can present a significant management challenge.Aims: We aimed to provide the first interdisciplinary management guidelines using the AGREEII frame...

ea0058oc4.4 | Oral Communications 4 | BSPED2018

Identification and characterisation of a small-molecule ACTH receptor/Melanocortin-2-receptor antagonist

Chan Li , Hussain Mashal , Forfar Rachel , Khurana Puneet , Cook Jennifer , Lewis Steve , McIver Ed , Jerman Jeff , Taylor Debra , Clark Adrian

The overproduction of ACTH, in conditions such as Congenital Adrenal Hyperplasia (CAH) leads to significant morbidity. Current treatment with glucocorticoids does not adequately suppress plasma ACTH, resulting in excess adrenal androgen production. At present, there is no effective medical treatment that would directly block ACTH action. Such a therapy, especially one that can be orally administered, would be of great clinical value allowing a ‘block and replace’ tre...

ea0058oc4.5 | Oral Communications 4 | BSPED2018

Delayed or Absent? – use of next generation sequencing diagnostic tools in a UK puberty cohort

Howard Sasha R , Cabrera Claudia P , Barnes Michael R , Dunkel Leo

Objectives: Several different pathogenic mechanisms may converge on a final common pathway to produce the phenotype of delayed pubertal timing. Abnormal pubertal timing affects >4% of adolescents and is associated with adverse health outcomes. Up to 80% of variation in the timing of pubertal onset is genetically determined. Self-limited delayed puberty (DP) segregates in an autosomal dominant pattern, but in the majority the neuroendocrine pathophysiology and genetic regul...

ea0058oc4.6 | Oral Communications 4 | BSPED2018

Implementation of a novel non-invasive test for monitoring control in individuals with congenital adrenal hyperplasia

Bacila Irina , Acerini Carlo L , Krone Ruth E , Patel Leena , Alvi Sabah , Randel Tabitha , Gevers Evelin F , Dattani Mehul , Cheetham Timothy , Ryan Fiona , Crowne Elizabeth , Davies Justin , Faisal Ahmed , Kyriakou Andreas , Adaway Jo , Schiffer Lina , Keevil Brian , Krone Nils

Introduction: Monitoring of hormonal control represents a key part in the management of congenital adrenal hyperplasia (CAH). It remains suboptimal and relies on frequent blood tests, which are traumatising in children and young persons (CYP). Recent evidence suggests a crucial role of adrenal-derived 11-oxygenatedC19 androgens in the pathogenesis of CAH. Therefore, we aimed to establish a non-invasive test for monitoring of adrenal-specific androgens in CAH.<p class="abst...

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...

ea0058oc4.8 | Oral Communications 4 | BSPED2018

Prolactinoma in Childhood and Adolescence – outcomes relating to the size of tumour

Arya Ved Bhushan , Kapoor Ritika , Hulse Tony , Ajzensztejn Michal , Kalitsi Jennifer , Kalogirou Nicolas , Bodi Istvan , Thomas Nick , Hampton Tim , Aylwin Simon , Buchanan Charles R

Objective: To describe the clinical presentation, management and treatment outcomes of prolactinomas diagnosed in childhood and adolescence in a consecutive series.Design and Methods: A retrospective review of medical records of patients with prolactinoma less than 20 years at diagnosis, referred to a tertiary paediatric endocrine service between 1996 and 2018.Results: Twenty-three patients (14 females) were identified; median age ...