hrp0097fc11.6 | GH and IGFs | ESPE2023

Longitudinal analysis of the risk of brain tumour recurrence or progression in relation to the timing of commencement of growth hormone replacement therapy.

Paula Gonzalez-Mereles Ana , Gan Hoong-Wei

Introduction: Growth hormone deficiency (GHD) is the most frequent endocrine deficit in childhood survivors of brain tumours. However, there is insufficient evidence to guide the timing of growth hormone replacement (GHR). At Great Ormond Street Hospital timing is based on clinical need rather than in relation to oncological treatment. Therefore, sufficient variability in GHR timing is available to analyze its effect on tumour progression and recurrence.<p...

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...

hrp0089p3-p022 | Adrenals and HPA Axis P3 | ESPE2018

Identification of X-linked Adrenoleukodystrophy in Boys Presenting with Adrenal Insufficiency in the Absence of Adrenal Antibodies

Ryalls Michael , Gan Hoong-Wei , Biedenkapp Joe , Davison James

Adrenoleukodystrophy (ALD) is an X-linked, metabolic disorder caused by genetic deficiency of peroxisomal ALD protein resulting in accumulation of very-long chain fatty acids (VLCFA) primarily in the adrenal cortex and central nervous system. Approximately 35–40% of boys with ALD develop cerebral ALD (CALD), which causes rapidly progressive cerebral demyelination, loss of neurologic function, and death. Disease progress can be halted by allogeneic hematopoietic cell trans...

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...

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...

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.<...

hrp0084p1-157 | Miscelleaneous | ESPE2015

How do Adolescent Minors Banking Sperm Before Cancer Therapy Subsequently use the Fertility Service? A Post Banking Re-Evaluation

Pigot James , Michaelidou Maria , Williamson Elizabeth , Webb Alison , Gan Hoong-Wei , Spoudeas Helen

Background: Gonadotoxic cancer therapy may cause adult male infertility. We previously reported that, of 2/3rds of underage males agreeing to pre-treatment sperm cryopreservation before cancer treatment, 2/3rds succeeded.Objective and hypotheses: i) To evaluate how many patients banking sperm successfully (GP1) returned for post treatment re-evaluation compared with those who attempted but failed (GP2). ii) To compare intergroup survival and fertility ra...

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...

hrp0086rfc3.5 | Pituitary | ESPE2016

Prospective Dynamic Evaluation of Hypothalamo-Pituitary Function in 30 Cases of Paediatric Craniopharyngioma, by Hypothalamic Injury and Treatment; A Single Centre Series

Guzzetti Chiara , Losa Laura , Improda Nicola , Pang Gloria , Phatarakijnirund Voraluck , Gan Hoong-Wei , Hayward Richard , Aquilina Kristian , Ederies Ash , Spoudeas Helen A.

Background: Craniopharyngiomas are the commonest pituitary tumours of childhood. Though benign histologically, their localisation and invasive tendency can cause significant neuroendocrine morbidity and late mortality.Objective and hypotheses: To prospectively determine risk factors for neuroendocrine morbidity by longitudinal survival analysis.Method: All children with craniopharyngioma newly presenting to our quaternary centre be...

hrp0094p1-13 | Bone A | ESPE2021

Characterisation and phenotype-genotype associations of a large cohort of patients with pseudohypoparathyroidism type 1A and 1B

Prentice Philippa , Wilson Louise , Gevers Evelien , Buck Jackie , Raine Joseph , Rangasami Jayanti , McGloin Helen , Peters Catherine , Amin Rakesh , Gan Hoong-Wei , Brain Caroline , Dattani Mehul , Allgrove Jeremy ,

We characterised the phenotype of PHP patients at two UK tertiary care centres and investigated phenotype-genotype correlations.Method: Retrospective review of case notes for patients with PHP at two UK tertiary care centres.Results: 55 patients, from 41 kindreds, were identified; 32 with PHP1a, 23 with PHP1b. The PHP1a cohort (56% female, 69% White), currently aged 16.7+/-10.6 years, presented at ...