hrp0095p1-354 | Pituitary, Neuroendocrinology and Puberty | ESPE2022

Single centre experience of using 6 monthly GnRH analogues in the paediatric population

Apperley Louise , Dharmaraj Poonam , Blair Joanne , Ramakrishnan Renuka , Das Urmi , Didi Mohammed , Laing Peter , Yung Zoe , Cassidy Kelly , Blundell Pauline , Jarvis Charlotte , Parkinson Jennifer , Senniappan Senthil

Background: Mounting evidence points to an association between increased glucocorticoid (GC) action and weight gain. However, the response to GCs is not only determined by GC serum concentrations, but also by individual differences in tissue-specific sensitivity, influenced by genetic and acquired (e.g. disease-related) factors. The extent to which differences in GC sensitivity may influence development of (abdominal) obesity, or vice versa, is poorly understo...

hrp0092p1-102 | Pituitary, Neuroendocrinology and Puberty | ESPE2019

Clinical Presentation, Management, and the Outcomes of Pituitary Adenomas in Children

Sethi Aashish , Didi Mohammed , Dharmraj Poonam , Ramakrishnan Renuka , Senniappan Senthil , Das Urmi , Avula Shivaram , Sinha Ajay , Mallucci Conor , Daousi Christina , Gilkes Catherine , Thorp Nicola , Blair Joanne

Introduction: Pituitary adenoma (PA) in childhood is a rare disease, accounting for 3 % of all intracranial paediatric neoplasm, and between 3 to 6% of all PA. There are only few large studies describing paediatric pituitary adenoma and even fewer studies with long-term outcome.Methods: In this retrospective study, clinical, biochemical and radiological parameters and outcome of paediatric patients (<16 years...

hrp0086rfc1.7 | Adrenals | ESPE2016

The Recovery of Adrenal Function in Children with Chronic Asthma Assessed by Low Dose Short Synacthen Test (LDSST)

Gangadharan Arundoss , McCoy Paul , McGuigan Michael , Didi Mohammed , Das Urmi , Dharmaraj Poonam , Senniappan Senthil , Ramakrishnan Renuka , Yung Zoe , Hatchard Lynn , Blundell Pauline , Stirrup Kelly , Blair Joanne

Background: Hypothalamic-Pituitary–Adrenal (HPA) axis suppression is common during inhaled corticosteroid (ICS) treatment of asthma, and deaths due to adrenal crisis are described. Little is known about the optimal treatment or recovery of ICS induced HPA axis suppression.Aims: To describe the treatment and outcomes of children with ICS induced HPA suppression diagnosed on the low dose short Synacthen test (LDSST).Method: The ...

hrp0097fc11.1 | GH and IGFs | ESPE2023

GH replacement therapy with once-weekly somapacitan in children with GH deficiency is effective and well-tolerated: 2-year results from REAL4

Miller Bradley , Blair Joanne , Højby Rasmussen Michael , Maniatis Aristides , Mori Jun , Böttcher Volker , Bang Rikke , Polak Michel , Horikawa Reiko

Daily subcutaneous (s.c.) injections of growth hormone (GH) to treat GH deficiency (GHD) in children is burdensome for both patients and caregivers. Somapacitan (Novo Nordisk) is a long-acting reversible albumin-binding human GH derivative in development for once-weekly s.c. administration in children with GHD, and aims to overcome the treatment burden of daily injections. REAL4 is a multi-national, randomised, open labelled phase 3 trial with a 52-week main phase followed by ...

hrp0097fc14.5 | Late Breaking | ESPE2023

Predicting Average IGF-I Concentration for Once-Weekly Somapacitan in Children with Growth Hormone Deficiency

F. Backeljauw Philippe , Kamal Lyauk Yassine , C. Blair Joanne , S. Miller Bradley , Mori Jun , Højby Rasmussen Michael , Juul Kildemoes Rasmus

Growth hormone (GH) stimulates insulin like growth factor I (IGF I) release. IGF-I is the standard biomarker for monitoring GH effects during treatment and to achieve optimal long-term safety, and to a limited extent, monitor efficacy in children with GH deficiency (GHD). The IGF I profile during treatment with a long acting GH (LAGH), such as once weekly somapacitan (Novo Nordisk), differs from the daily GH profile by exhibiting larger peaks and troughs over the dosing interv...

hrp0084p2-256 | Diabetes | ESPE2015

Exposure to Phthalates and Phenols in Relation to Gestational Blood Glucose Homeostasis

Fisher Benjamin G. , Frederiksen Hanne , Andersson Anna-Maria , Juul Anders , Thankamony Ajay , Ong Ken K. , Dunger David B. , Hughes Ieuan A. , Acerini Carlo L.

Background: Endocrine disrupting chemicals (EDCs), such as phthalates and bisphenol A (BPA), have been associated with insulin resistance (IR) and type 2 diabetes (T2D) in non-pregnant adults. By contrast, recent pilot studies of pregnant women found negative associations between phthalates and blood glucose, and a lack of association with BPA. No studies have examined gestational IR or secretion in relation to EDC exposure.Objective: To confirm these re...

hrp0095p1-574 | Sex Differentiation, Gonads and Gynaecology, and Sex Endocrinology | ESPE2022

Phenotype-genotype characteristics of SRD5A2 with variation in gender choice among affected individuals: A single centre experience

Kaninde Abhidhamma , Baranowski Elizabeth , Igbowke Rebecca , McCarthy Liam , Chandran Harish , Vandermerwe Elmarie , Fulton Piers , Godber Caroline , Smart Helen , Curtis Joanne , Cole Trevor , Gleeson Helena , Latthe Pallavi , Kirk Jeremy , Idkowiak Jan , Mohamed Zainaba

Background: 5α-reductase type 2 deficiency (SRD5A2) is a rare cause of 46,XY DSD. Consensus guidelines on sex of rearing assignment at birth favours male gender. Typically undervirilised genitalia at birth virilise variably at puberty, posing gender identity challenges.Aim: We describe relevant data on clinical phenotype, hormonal and molecular workup and gender preference in patients with SRD5A2 deficiency from a ...

hrp0084p3-698 | Diabetes | ESPE2015

Adherence to Diabetic Ketoacidosis Management Protocol: a Paediatric Centre Experience

Cloutier Daphne , Barbe Joanie , Bouchard Isabelle , Gagne Julie , Bourdages Macha

Background: Paediatric diabetic ketoacidosis (DKA) management should be regulated by specific protocols. Following the Canadian Diabetes Association recommendations, our paediatric tertiary care hospital was provided with such a protocol in 2009.Objective and hypotheses: Assess the proportion of DKA episodes that are marked by non-adherence to our DKA management protocol (DKAp). We suspected a moderate non-adherence rate.Method: We...

hrp0095p1-381 | Sex Differentiation, Gonads and Gynaecology, and Sex Endocrinology | ESPE2022

Gynaecomastia And Its Management In Partial Androgen Insensitivity Syndrome (PAIS)

Patjamontri Supitcha , Lucas-Herald Angela , Bryce Jillian , Cools Martine , Gianni Russo , Globa Evgenia , Zelinska Natalia , Guerra-Junior Gil , Holterhus Paul-Martin , A Hughes Ieuan , Tadokoro-Cuccaro Rieko , Nordenstrom Anna , Rita Stancampiano Marianna , Weintrob Naomi , van den Akker Erica , Hiort Olaf , Hoffman Paul , Juul Anders , Nimali Seneviratne Sumudu , Faisal Ahmed S.

Introduction: In adolescents and men with PAIS, gynaecomastia has been reported in the majority but its management remains challenging.Objectives: To assess the current management of gynaecomastia as well as clinical characteristics in male PAIS.Materials and Methods: Retrospective review in the I-DSD registry of 46, XY male PAIS who were over the age of 10 years.<p class="abst...

hrp0084p1-58 | DSD | ESPE2015

Long-term Endocrine Outcome in Men with Partial Androgen Insensitivity Syndrome

Lucas-Herald Angela K , Ahmed S Faisal , Bertelloni Silvano , Juul Anders , Bryce Jillian , Jiang Jipu , Rodie Martina , Johansen Marie L , Hiort Olaf , Holterhus Paul-Martin , Cools Martine , Desloovere An , Weintrob Naomi , Hannema Sabine E , Guran Tulay , Darendeliler Feyzad , Nordenstrom Anna , Hughes Ieuan

Background: Partial Androgen insensitivity syndrome (PAIS) is a rare condition which is associated with a variable phenotype. To date, there are limited data reporting long-term endocrine outcome for this condition.Aims: To determine the outcomes and clinical characteristics for 46, XY males with PAIS, using information from the International DSD (I-DSD) Registry and its clinical users.Methods: The I-DSD Registry and its users were...