hrp0094p2-6 | Adrenals and HPA Axis | ESPE2021

Single centre experience of Hydrocortisone Granules (Alkindi) in children under 6 years of age with Adrenal Insufficiency

Snow Claire , Owen Catherine ,

Introduction: Children with Adrenal insufficiency (AI) are usually managed with glucocorticoid replacement as hydrocortisone. Treatment options in younger patients include administering an aliquot of 10mg hydrocortisone tablets dissolved in water. However, this is time consuming and may be inaccurate. We describe our experience of using Alkindi (www.diurnal.co.uk), the first hydrocortisone preparation licensed for children with AI.<stron...

hrp0097p1-363 | Pituitary, Neuroendocrinology and Puberty | ESPE2023

Xanthomatous hypophysitis: A rare cause of paediatric hypopituitarism

Bendor-Samuel Owen , Statchard Rebecca , Daskas Nikolaos

Primary Xanthomatous Hypophysitis (XH) is the rarest histological subtype of hypophysitis. Here we describe the case of a young 9-year-old girl diagnosed with this condition. The patient presented with a four-month history of an intermittent temporal-frontal headache that became gradually worse and constant for three days prior to admission. She did not have clinical signs or symptoms suggestive of increased intracranial pressure but had mild ataxia. On admission she was febri...

hrp0095rfc5.1 | Adrenals and HPA Axis | ESPE2022

Single centre use of Hydrocortisone granules (Alkindi) in children with Adrenal Insufficiency: the patient experience.

O'Sullivan Jacqueline , Snow Claire , Wood Claire , Owen Catherine

Standard management of Adrenal Insufficiency (AI) in children is with hydrocortisone (HC). Under or over-treating impacts on growth and quality of life. Until recently, treatment involved dissolving 10mg HC tablets in water; this is time consuming and may give inaccurate doses. We have offered HC granules (Alkindi) to all new patients ≤6-years since January 2019 and 22 patients who were currently dissolving HC tablets. We have reviewed our patients’ experience with Al...

hrp0084p3-1134 | Puberty | ESPE2015

Paraphilic Compulsion Secondary to Dopamine Replacement Therapy and Successful Treatment with GnRH Analogues

Brewka Anna , Owen Tamsin , Lin Jean-Pierre , Ajzensztejn Michal

Background: Hypersexualized behaviour in the paediatric population is a rare phenomenon. The aetiology of paraphilia is not completely understood, but some studies suggest imbalance of the dopamine serotonin system. Paraphilia has also been described as a side-effect of treatment with monoaminooxidase inhibitors (MAOI) and dopamine agonists. Most of the currently used pharmacologic treatments of the paraphilias have serotonin and testosterone/dihydrotestosterone as their targe...

hrp0089p1-p030 | Bone, Growth Plate &amp; Mineral Metabolism P1 | ESPE2018

Bone Biochemistry in Children with Fractures Presenting with Suspected Non-accidental Injury

Forbes Owen , McNeilly Jane , McDevitt Helen , Houston James , Ahmed S. Faisal , Mason Avril

Introduction: Fractures have been recorded in up to one third of children who have suffered from physical abuse. The British Paediatric and Adolescent Bone Group (BPABG) position statement on vitamin D states that the level of 25 hydroxyvitamin D is not relevant to causation of fractures unless there is radiological or biochemical evidence of rickets. Clinicians are often asked in the court setting about the relevance of abnormal serum investigations in children who have fract...

hrp0097ha1 | An integrated roadmap of human fetal adrenal gland development | ESPE2023

An integrated roadmap of human fetal adrenal gland development

del Valle Ignacio , Young Matthew , Kildisiute Gerda , Ogunbiyi Olumide , Buonocore Federica , Simcock Ian , Khabirova Eleonora , Crespo Berta , Moreno Nadjeda , Brooks Tony , Niola Paola , Swarbrick Katherine , Suntharalingham Jenifer , McGlacken-Byrne Sinead , Arthurs Owen , Behjati Sam , Achermann John

Introduction: The human adrenal gland originates from the adrenogonadal primordium at around 4 weeks post conception (wpc) and undergoes marked developmental changes throughout the first half of pregnancy. Several key aspects of adrenal maturation are well-established, such as the formation of a large inner fetal zone (FZ) and synthesis of dehydroepiandrosterone, but many other processes contributing to adrenal gland development and function in humans are stil...

hrp0097fc7.2 | Sex differentiation, gonads and gynaecology or sex endocrinology | ESPE2023

Mapping the transcriptomic landscape of early human fetal ovary development through a clinically-focused lens

M McGlacken-Byrne Sinead , Del Torres Ignacio , P Suntharalingham Jenifer , Buonocore Federica , HDBR ICH , Crespo Berta , Moreno Nadjeda , C Simcock Ian , Arthurs Owen , Xenakis Theodoros , Niola Paola , Brooks Tony , T Dattani Mehul , C Achermann John

Introduction: Ovary development was once considered a largely passive process. RNA sequencing (RNAseq) approaches have allowed us to begin to characterise ovary development in previously unparalleled detail, revealing the process to be complex and, still, little understood. A challenge is synthesising and using these data to advance our understanding of clinical disease. Through a clinically-focused lens, we elucidate novel aspects of the transcriptional lands...