hrp0089p1-p195 | Pituitary, Neuroendocrinology and Puberty P1 | ESPE2018

Long Term Reversibility of Presumed ACTH Deficiency (ACTHd) in Children and Young People (CYP) with Intracranial Germ Cell Tumours (IGCT)

Pieri Kyriaki , Michaelidou Maria , Chatoo Zaynab , Holloway Ross , Dastamani Antonia , Spoudeas Helen A

Introduction: ACTHd is life-threatening and difficult to differentiate from ACTH suppression (ACTHs) especially in CYP receiving perioperative corticosteroids. In our experience, this is always the most robust anterior pituitary hormone to brain injury, whilst GH deficiency (GHd) is the first and LH/FSHd and TSHd intermediate in hierarchy. We previously showed HPA axis recovery at 3.08 (2.38–10.33) years after cortiscosteroid therapy for ACTHd in 13.6% of 44 CYP with cran...

hrp0089p1-p197 | Pituitary, Neuroendocrinology and Puberty P1 | ESPE2018

A Single Centre Experience of Managing a Series of Childhood Macro/Giant-Prolactinoma

Dastamani Antonia , Bulwer Chloe , Ederies Adhraf , Jeelani Owase , Fersht Naomi , Aquilina Kristian , Korbonits Marta , Spoudeas Helen

Introduction: Childhood prolactinomas often occur as aggressive macro (1–4 cm) or giant (>4 cm) tumours, with little consensus regarding timing of optimal therapies.Aim: To highlight the phenotype and treatment outcome of childhood macroprolactinomas.Subjects and methods: Case-note review of 10 (five male) children (<18 years) (presenting to our centre between 2009 and 2017 with hyperprolactinaemia due to macro/giant-p...

hrp0089p1-p198 | Pituitary, Neuroendocrinology and Puberty P1 | ESPE2018

A National UK Guideline for Managing Pituitary Adenomas in Children and Young People Under 19 Years Developed According to the AGREE II Framework

Blair Jo , Korbonits Marta , Ronaldson Amy , Dang Mary N , Spoudeas Helen

Pituitary adenomas are usually benign tumours arising from the hormone-secreting cells of the anterior pituitary gland. These adenomas can result in excess hormone secretion and the development of characteristic syndromes, such as Cushing’s disease, acromegaly and hyperprolactinaemia, and/or mass effects on surrounding vital structures causing for example visual disturbances and pituitary hormone deficiencies. In children and young people under 19 years (CYP), the managem...

hrp0089p3-p287 | Pituitary, Neuroendocrinology and Puberty P3 | ESPE2018

Invasive Macroprolactinoma with Cabergoline Induced Cerebrospinal Fluid Rhinorrhoea in Childhood

Dastamani Antonia , Ederies Ashraf , Aquilina Kristian , Dorward Neil , Korbonits Marta , Spoudeas Helen

Background: Nonsurgical development of nasal cerebrospinal fluid (CSF) leaks may occur in the setting of pituitary adenomas, especially following a favorable response of invasive prolactinomas to initiation of Dopamine Agonist (DA) therapy, but this has not previously described in children.Case description: A girl of Srilankan origin, aged 13.8 years, whose parents spoke no English, presented with headaches and secondary amenorrhea. Pituitary MRI reveale...

hrp0089p3-p418 | Pituitary, Neuroendocrinology and Puberty P3 | ESPE2018

Clinical and Endocrinological Manifestations of Partial Ectopic Posterior Pituitary: A New Imaging Entity

Ybarra Marina , Hafiz Rawan , Robinson Marie-Eve , Oettingen Julia von , Bui Helen , Saint-Martin Christine

Objective: To describe six cases of possible partial ectopic posterior pituitary gland (PEPP) seen on head magnetic resonance imaging (MRI) and their associated clinical and endocrinological manifestations.Methods: This is a single-center case series, from a tertiary public university health center in Montreal, Canada. Cases of children with possible PEPP were selected prospectively from 2005 to 2017, based on head MRI findings. Medical history, exam fi...

hrp0086rfc3.1 | Pituitary | ESPE2016

Endocrinopathy in Childhood Intracranial Germ Cell Tumours is Predicted by Disease Location not Treatment: 30 year Experience from a Single Tertiary Centre

Serra-Caetano Joana , Dimitrakopoulou Eftychia , Ederies Ash , Phipps Kim , Spoudeas Helen Alexandra

Background: Childhood pineal and/or suprasellar intracranial germ cell tumours (IGCT) are highly curable (>90%) with neuraxial radiation alone; international (SIOP) trials have aimed to decrease late radiation-induced neuroendocrine morbidity by substituting chemotherapy. However, without longitudinal study, disease and treatment contributions to long term outcomes remain unknown.Objective: To define tumour and treatment factors implicated in neuroen...

hrp0086p1-p200 | Diabetes P1 | ESPE2016

Driving Paediatric Diabetes Care Forward in the UK: Improvements in Outcomes in the North West Following National Initiatives

Moore Helen S , Carson Margot E , Maiden Jonathan , Ng Sze May

Background: Type 1 diabetes mellitus (T1DM) continues to pose serious health risks with devastating long-term complications. UK management and control of T1DM in children and young people (CYP) remains amongst the poorest in Europe and significant variations in diabetes health outcomes are evident. In 2012–13 a Best Practice Tariff (BPT) for paediatric diabetes care was introduced and a National Peer Review Quality Assurance programme (DQuINS) developed. Both were develop...

hrp0086p2-p516 | Fat Metabolism and Obesity P2 | ESPE2016

4G Polymorphism of Plasminogen Activator Inhibitor-1 (PAI-1), PAI-1 Plasma Levels, and Lipid Profiles in Overweight/Obese Children and Adolescents

Karabouta Zacharoula , Makedou Areti , Papandreou Dimitrios , Argiriou Anagnostis , Xanthopoulou Helen , Rousso Israel , Athanassiadou-Piperopoulou Fani

Background: Studies have shown that PAI-1 4G polymorphism is related to increased plasma PAI-1 levels, obesity, dyslipidaemia and cardiovascular disease (CVD) in adults. Few studies have explored that relationship in overweight/obese (Ow/Ob) children/adolescents.Objective and hypotheses: We investigated the relation between plasma PAI-1 levels, PAI-1 4g polymorphisms and lipid profiles in Ow/Ob children/ adolescents compared with healthy normal ...

hrp0086p2-p656 | Growth P2 | ESPE2016

Improved Growth Outcomes with Jet Delivery of Growth Hormone in Children are Maintained Over Long-Term Treatment

Michaelidou Maria , Knight Alastair D , Whitten Sue , Bajaj Priti , Spoudeas Helen A

Background: We previously reported in a national cohort, that adherence to subcutaneous Growth Hormone (GH) treatment in children is better with jet delivery compared with needle devices (Spoudeas et al. Patient Prefer Adherence. 2014;8:1255–1263). We also separately reported that adherent children showed significantly improved height outcomes at one year.Objective and hypotheses: To examine the potential influence of adherence and demograp...

hrp0082fc5.6 | Neuroendocrinology | ESPE2014

The Diencephalic Syndrome of Emaciation in Infantile Hypothalamochiasmatic Low-Grade Gliomas : a Retrospective Case–Control Study of Diagnostic Parameters and Long-Term Outcomes Over 30 Years of Follow-Up

Gan Hoong-Wei , Meijer Lisethe , Gullick Georgina , Senbanjo Margaret , Walker David , Spoudeas Helen

Background: Diencephalic syndrome (DS) is a rare disorder of severe emaciation classically associated with infantile Hypothalamochiasmatic low-grade gliomas (HCLGGs) and GH excess. However, diagnostic criteria remain undefined and published literature includes non-specific tumour-related cachexia. In a large optic pathway LGG cohort (n=166), we have previously demonstrated that DS independently predicts multiple tumour progressions and severe endocrine morbidity.<...