hrp0082p2-d3-494 | Endocrine Oncology | ESPE2014

Three Year Experience of a National Interdisciplinary Initiative to Improve Outcomes for Children with Hypothalamic Pituitary Axis Tumours (HPATs) Using Multi-site Videoconferencing for Decision Making on Behalf of the UK HPAT Interest Group

Perelberg Daniel , Morillon Paul , Ederies Ash , Aquilina Kristan , Dorward Neil , Michalski Anton , Hargrave Darren , Chang Yen-Ching , Bozorgi Nillofar , James Samantha , Korbonits Marta , Drake William , Akker Scott , Mallucci Connor , Pizer Barry , Blair Jo , Kamaly Ian , Clayton Peter , Spoudeas Helen

Background: Childhood tumours of the hypothalamic pituitary axis (HPATs) are very rare and hence any single centre experience is limited. Without evidence-based guidance, treatment is individualised on a case basis. Survival rates are high, but at the expense of significant morbidity. Centralised care or wider multi-professional consultation may improve neuroendocrine and visual outcomes.Objective and hypotheses: i) To facilitate multi-professional dialo...

hrp0086p2-p773 | Pituitary and Neuroendocrinology P2 | ESPE2016

Primary Thirst Defect is a Rare But Important Complication Following Surgery for Hypothalamic Hamartoma and Intractable Epilepsy

Giri Dinesh , Blair Jo , Das Urmi , Dharmaraj Poonam , Senniappan Senthil , Malluci Connor , Benedetta Pettorini , Pizer Barry , Burns Sasha , Didi Mohammed

Background: Diabetes insipidus (DI) is a well-recognized post neuro surgical complication arising after hypothalamic-pituitary surgery. DI occurring in the post-operative period can be transient happening within 24–48 hour of surgery, secondary to trauma to the connections between the magnocellular bodies and the nerve terminals in the posterior pituitary, or to axonal shock from disturbances in the vascular supply to the pituitary stalk and posterior pituitary.<p cla...

hrp0092p1-259 | Pituitary, Neuroendocrinology and Puberty (1) | ESPE2019

Disruption of Hypothalamic Regulation of Appetite Associated with Proton Beam Therapy

Sethi Aashish , Didi Mohammed , Mallucci Conor , Thorp Nicola , Hayden James , Pizer Barry , Blair Joanne

Introduction: Proton beam therapy (PBT) is being used increasingly for craniopharyngioma, as it is perceived to be a major benefit. There are some limited data relating to endocrine dysfunction following PBT, but very limited data on hypothalamic disturbance. Here we report two patients who presented with hypothalamic disruption immediately following PBT for craniopharyngioma.Case 1: A 12 year old girl presented with sei...

hrp0082p2-d3-493 | Endocrine Oncology | ESPE2014

Role of Metformin in the Treatment of Hypothalamic Obesity

Gangadharan Arundoss , Didi Mohammed , Pizer Barry , Howell Lisa , Hayden James , Mallucci Conor , Pettorini Benedetta , Blair Joanne C

Background: Disruption of homeostatic functioning of the hypothalamic centres results in hyperphagia, autonomic imbalance, reduction of energy expenditure, and hyperinsulinemia. A syndrome of rapid, unrelenting weight gain is often observed in patients with structural lesions of the hypothalamus. Hypothalamic obesity syndrome (HOS) is often refractory to standard dietary and lifestyle interventions. It has been reported that metformin induces anorectic effects via an increase ...

hrp0094fc6.5 | Bone and Mineral Metabolism | ESPE2021

What is the natural history of cervicomedullary compression without signal change in infants with achondroplasia? Investigating foramen magnum stenosis evolution.

Harvey Charlotte , Brett Connor , Cocca Alessandra , D’Arco Felice , Irving Melita , Thompson Dominic , Cheung Moira ,

Background: Foramen magnum stenosis (FMS) is a common, serious complication of achondroplasia in infancy. The Achondroplasia Foramen Magnum Score (AFMS; 1-4) was developed to facilitate early detection of stenosis and inform appropriate neurosurgical management. All infants have a baseline screening MRI, of which approximately half of cases demonstrate AFMS3 (craniovertebral junction (CVJ) narrowing with flattening of the cervical cord) or AFMS4 (CVJ narrowing...

hrp0092p1-102 | Pituitary, Neuroendocrinology and Puberty | ESPE2019

Clinical Presentation, Management, and the Outcomes of Pituitary Adenomas in Children

Sethi Aashish , Didi Mohammed , Dharmraj Poonam , Ramakrishnan Renuka , Senniappan Senthil , Das Urmi , Avula Shivaram , Sinha Ajay , Mallucci Conor , Daousi Christina , Gilkes Catherine , Thorp Nicola , Blair Joanne

Introduction: Pituitary adenoma (PA) in childhood is a rare disease, accounting for 3 % of all intracranial paediatric neoplasm, and between 3 to 6% of all PA. There are only few large studies describing paediatric pituitary adenoma and even fewer studies with long-term outcome.Methods: In this retrospective study, clinical, biochemical and radiological parameters and outcome of paediatric patients (<16 years...

hrp0092rfc11.1 | Pituitary, Neuroendocrinology and Puberty Session 2 | ESPE2019

National United Kingdom Evidence- and Consensus-Based Guidelines for the Investigation, Treatment and Long-Term Follow-Up of Paediatric Craniopharyngioma

Gan Hoong-Wei , Morillon Paul , Albanese Assunta , Aquilina Kristian , Barkas Konstantinos , Chandler Chris , Chang Yen-Ch'ng , Daousi Christina , Drimtzias Evangelos , Farndon Sarah , Jacques Tom , Korbonits Marta , Kuczynski Adam , Limond Jennifer , Robinson Louise , Simmons Ian , Thomas Nick , Thomas Sophie , Thorpe Nicola , Vargha-Khadem Faraneh , Warren Daniel , Zebian Bassel , Gamble Ashley , Wilne Sophie , Harrison Barney , Spoudeas Helen , Mallucci Conor

Aims: Although rare, craniopharyngiomas are the commonest suprasellar tumour in childhood. Despite high overall survival, children and young people <19 years with craniopharyngiomas are at risk of multiple relapses and long-term tumour- and treatment-related neuroendocrine, cognitive and visual morbidity. A recent international survey highlighted the considerable variation in management strategies employed for these tumours, and the lack of any evidence- a...

hrp0094p2-208 | Fat, metabolism and obesity | ESPE2021

OSAS in childhood obesity is a more frequent and earlier complication than expected

Deiana Giuseppina , Maltoni Giulio , Zucchini Stefano , Gallucci Marcella , Levi della Vida Francesca , Barberi Carolina , Pession Andrea , Cassio Alessandra ,

Introduction: Childhood obesity is associated with several complications, involving many systems. The prevalence of respiratory problems, such as obstructive sleep apnea syndrome (OSAS), is higher in obese children and adolescents. In fact, OSAS affects 13–59% of obese children and the severity is strongly associated with weight excess. Although overnight pulse oximetry (PO) can be used for diagnosing OSAS, a complete night polysomnography (PSG), which re...