hrp0089p2-p313 | Pituitary, Neuroendocrinology and Puberty P2 | ESPE2018

Neuroendocrine Consequences of Hypothalamic Hamartoma and their Imaging (MRI) and Surgery Correlates

Corredor Beatriz , Caredda Elisabetta , Ederies Ash , Tisdall Martin , Cross Helen , Spoudeas Helen A.

Background: Hypothalamic hamartomas(HH) are rare heterotopic congenital malformations causing central precocious puberty(CPP) and/or resistant epilepsy whose natural history is unknown.Aim: To describe clinical and imaging features, and the risk of developing endocrine deficits, particularly after surgery.Method: Retrospective case note and imaging review of all HH diagnosed by MRI between 30.08.1991 and 24.11.17, analysed by initi...

hrp0086p1-p738 | Pituitary and Neuroendocrinology P1 | ESPE2016

Oxytocin Deficiency is Associated with Hyperphagia and Weight Gain in Hypothalamic and Common Obesity: A First-in-Humans Proof-of-Concept Study

Gan Hoong-Wei , Leeson Clare , Aitkenhead Helen , Spoudeas Helen , Martinez-Barbera Juan Pedro , Dattani Mehul

Background: Hypothalamic obesity (HyOb) is a rare form of treatment-resistant morbid obesity associated with congenital or acquired hypothalamic damage. Its pathophysiology has been attributed to hyperphagia and hyperinsulinaemia. The wider roles of oxytocin (OXT) in regulating appetite and weight have recently emerged in animal and human studies, but there is no human evidence that hypo-oxytocinaemia contributes to weight gain.Hypothesis: Hypo-oxytocina...

hrp0084p2-339 | Fat | ESPE2015

Hypothalamic Obesity, Hyperphagia, and Hyperinsulinaemia: Time for a Paradigm Shift in Assumptions?

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

Background: Hypothalamic obesity (HyOb) is a syndrome of inexorable, treatment-resistant obesity seen after congenital (e.g. septo-optic dysplasia (SOD)) or acquired (e.g. tumour-related) hypothalamic damage, often co-existing with hyperphagia, panhypopituitarism, autism, sleep, and temperature dysregulation. Its pathophysiology is poorly understood but hyperphagia and excess caloric intake may be less important than previously thought.Objective and hypo...

hrp0089fc9.4 | Pituitary, neuroendocrinology and Puberty 1 | ESPE2018

Neuroendocrine Morbidity after Paediatric Craniopharyngioma: a Longitudinal Single Centre Analysis of 93 Patients Over 30 Years

Dehalvi Shiraz , Guzzetti Chiara , Gan Hoong-Wei , Spoudeas Helen

Context: Craniopharyngiomas are rare, suprasellar tumours with excellent 5-year survival rates of 95%. Despite their benign histology, their tendency to invade vital nearby optic, hypothalamopituitary and vascular structures, complicates resection whilst potentially causing secondary life limiting morbidity, panhypopituitarism and premature mortality. Since 1997, conservative hypothalamic-sparing surgery and radiation to the residual tumour has replaced aggressive resection at...

hrp0089p2-p058 | Bone, Growth Plate & Mineral Metabolism P2 | ESPE2018

Validation of an Automated Method (BoneXpert) for the Determination of Bone Age in Paediatric Endocrinology – A Single Centre Experience

Calder Alistair , Dastamani Antonia , Spoudeas Helen , Dattani Mehul

Background: The BoneXpert method is an automated determination of bone age, which employs deformable models of each bone to locate the bones, and extracts the component of the bone appearance related to maturity in a holistic, statistical manner. The BoneXpert has been validated on normal children and children with diagnoses typical of pediatric endocrinology. Multiple clinical studies suggested that BoneXpert has adequate accuracy, precision, and efficiency to be clinically u...

hrp0089p2-p277 | Growth & Syndromes P2 | ESPE2018

Pulling the Brakes – ‘Catch Down Growth’: A Phenomenon for Achieving Mid-parental Height Centile After Acquired, All-cause, Brain Injury

Kraus Fabian B T , Hindmarsh Peter C , Spoudeas Helen A

Introduction: Of any pituitary dysfunction following brain injury, growth hormone (GH) deficiency (GHD) is the most prevalent. The cut-point for defining GHD has been placed at 7 ng/mL representing optimum test performance. We hypothesised this cut-off may be set too low for genetically taller children with acquired brain injury, notably brain tumours, who demonstrate severe growth failure but repeatedly fail to meet diagnostic thresholds for GH replacement until several centi...

hrp0086rfc3.2 | Pituitary | ESPE2016

Subfertility After Chemotherapy in PNET Tumours: 34 year Experience from a Single Centre (1980–2013)

Serra-Caetano Joana , Pandalai Soumya , Phipps Kim , Spoudeas Helen Alexandra

Background: PanEuropean(SIOP) trials of treatment for childhood brain cancers (medulloblastomas/PNET) showed a 5% survival advantage with ‘sandwich’ gonadotoxic chemotherapy (CT) over surgical excision, neuraxial radiation and tumour boost (RT) alone. But this was tempered by a reduced quality of survival at 7 years.Objective: To assess the long term prevalence of subfertility after CT with/without neuraxial radiation.<p class="a...

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...