hrp0084p3-1131 | Puberty | ESPE2015

Growth Outcomes in Childhood Craniopharyngioma: A Longitudinal Assessment of 21 Cases at a Single Centre

Michaelidou Maria , Losa Laura , Ederies Ashraf , Spoudeas Helen

Background: Craniopharyngiomas are rare suprasellar tumours with good survival but high endocrine morbidity. The commonest endocrinopathy is GHd which can precede diagnosis. Later obesity (BMI >+2SDS) may be treatment or tumour related.Objective and hypotheses: To evaluate endocrinopathy, height, weight and BMI outcomes after a conservative surgical treatment strategy with pre-treatment pituitary provocation tests for children diagnosed between 2009 ...

hrp0094p2-372 | Pituitary, neuroendocrinology and puberty | ESPE2021

The impact of the Covid-19 pandemic on the UK National Hypothalamic-pituitary Axis Tumour (HPAT) multidisciplinary meeting

Bosch i Ara Laura , Spoudeas Helen , Wei Gan Hoong ,

Background: The HPAT multi-disciplinary meeting (MDT) was set up in 2011 with the aim to improve collaboration with other hospitals treating children with rare paediatric suprasellar brain tumours. It is a monthly remote meeting conducted virtually.Objective: To evaluate the impact of COVID19 pandemic by comparing the number of meetings, cases (news/previously discussed), diagnosis, attendees, specialties, centres and ou...

hrp0089fc14.2 | Multisystem Endocrine Disorders | ESPE2018

National UK Guidelines for the Clinical Assessment, Diagnosis, Treatment and Follow-up of Children and Young People (CYP) Under 19 Years of Age with Phaeochromocytoma (PCC) and Paraganglioma (PGL) – On Behalf of the UK Paediatric Phaeochromocytoma and Paraganglioma Guideline Development Group (GDG)

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

Background: PCC and PGL are rare in CYP. National children’s registry data reveal an annual incidence of 0.2 and 0.3 per million in 5–9 and 10–14 year age groups respectively. Almost all result from a genetic predisposition, can present with non-specific symptoms, and represent a significant management challenge.Aims: We aimed to provide the first interdisciplinary national management guidelines using the AGREEII framework for CYP with con...

hrp0089p1-p106 | Fat, Metabolism and Obesity P1 | ESPE2018

Towards a Greater Understanding of the Pathophysiology of Obesity: Hypothalamic Obesity as a Model of Dysregulation of Appetite and Metabolic Homeostasis

Gan Hoong-Wei , Leeson Clare , Aitkenhead Helen , Farooqi Sadaf , Spoudeas Helen , Dattani Mehul

Introduction: Hypothalamic obesity (HyOb) is a rare form of treatment-resistant morbid obesity associated with congenital or acquired hypothalamic damage. Its pathophysiology is incompletely understood, with weight gain being attributed to hyperphagia and hyperinsulinaemia. We sought to compare the physiology of various plasma appetite-regulating hormones in HyOb and ‘simple’ obesity (Ob) to improve our understanding of both forms of obesity and identify novel therap...

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...

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...

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...