hrp0089p1-p194 | Pituitary, Neuroendocrinology and Puberty P1 | ESPE2018

A 7-Year Update Report of a National, Interdisciplinary Endeavour to Improve Outcomes for Children and Young People Under 19 Years of Age with Hypothalamic Pituitary Axis Tumours (HPAT) Using Multi-site Video Conferencing

Freund Katja , Malhorta Neha , Dastamani Antonia , Dorward Neil , Aquilina Kristian , Chang Yen-Ching , Mankad Kshitij , Pettorini Benedetta , Blair J Jo , Kamaly Ian , Clayton Peter E , Hargrave Darren , Korbonits Marta , Spoudeas Helen A

Background: Paediatric HPAT, though generally benign, incurs significant neuro-endocrine morbidity. Their management is unclear and the paediatric neuro-oncology or adult pituitary forum at which they are discussed lack pituitary or age-specific expertise respectively. The UK National HPAT Interest Group has pioneered a monthly, multi-site, interdisciplinary, video conferencing decision-making forum, to garner necessary experience and evidence of outcomes to assist worldwide r...

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...

hrp0084p1-134 | Turner & Puberty | ESPE2015

Co-Existing Variants of FOXE1 and BMP15 Genes in Young Females with Primary Ovarian Insufficiency: Evidence of Digenic Inheritance

Settas Nikolaos , Michala Lina , Deligeoroglou Efthimios , Chrysis Dionisios , Pampanos Andreas , Theochari Eleni , Valla Olympia , Sofocleous Christalena , Sofia Kitsiou , Fryssira Helen , Kanavakis Emmanuel , Dacou-Voutetakis Catherine , Chrousos George P , Voutetakis Antonis

Background: FOXE1 gene variants containing alterations in the alanine tract length may confer susceptibility to primary ovarian insufficiency (POI). BMP15 gene variants have also been related to POI.Objective and hypotheses: To evaluate the contribution of FOXE1 and BMP15 variants in the pathogenesis of POI and to investigate the hypothesis of digenic inheritance in this disorder.Method: FOXE1 and BMP...

hrp0094p1-25 | Diabetes A | ESPE2021

Longitudinal Auxological Recovery and Reduced Neurodevelopmental Problems in Hyperinsulinaemic Hypoglycaemia

Worth Chris , Hashmi Laila Al , Yau Daphne , Salomon-Estebanez Maria , Perez-Ruiz Diego , Hall Caroline , O’Shea Elaine , Pimlott Helen , Foster Peter , Flanagan Sarah , Cosgrove Karen , Dunne Mark , Banerjee Indraneel ,

Background: Hypoglycaemia due to congenital hyperinsulinism (CHI) is the commonest cause of severe, recurrent hypoglycaemia in childhood. Previous follow up studies have focused on neurodevelopmental status which is noted to be delayed in as many as 48% of cases. There has been less emphasis on other long-term outcomes in patients with CHI. Given the requirement for high volume carbohydrate in most patients, there are concerns regarding the adverse effects on ...

hrp0086rfc1.6 | Adrenals | ESPE2016

Pediatric Patients with Congenital Adrenal Hyperplasia have Unfavorable Changes in their Cardiovascular Risk Profile

Mooij Christiaan F. , van Herwaarden Antonius E. , Roeleveld Nel , de Korte Chris L. , Kapusta Livia , Claahsen - van der Grinten Hedi L.

Background: Patients with congenital adrenal hyperplasia (CAH) are at risk of developing an unfavorable cardiovascular risk (CVR) profile. Data on the CVR profile in pediatric CAH patients are scarce.Objective and hypotheses: To evaluate the CVR profile of pediatric CAH patients.Method: A cross-sectional study in CAH patients (8–16 years) was performed (n=27). Blood was taken to evaluate several circulating CVR marker...

hrp0095rfc9.6 | Pituitary, Neuroendocrinology and Puberty | ESPE2022

Craniopharyngiomas diagnosed as incidentalomas - Results of KRANIOPHARYNGEOM 2007

Boekhoff Svenja , Bison Brigitte , Eveslage Maria , Sowithayasakul Panjarat , Beckhaus Julia , Friedrich Carsten , L. Müller Hermann

Purpose: Childhood-onset craniopharyngiomas (CP) are diagnosed due to clinical symptoms (symCP) or incidentally (incCP). In childhood-onset CP, studies on history before CP diagnosis have revealed symptoms and complaints related to CP, which were documented in CP patients’ records with a median duration of 5 months prior to CP diagnosis. We investigated clinical manifestations and outcome in incCPs and symCPs.Methods:</stro...

hrp0095p1-355 | Pituitary, Neuroendocrinology and Puberty | ESPE2022

Serum Ghrelin and Neuropeptide Y Concentrations and Their Relationship with Gonadotropins and Anthropometric Parameters in Girls with Idiopathic CEntral Precocious Puberty, Premature Thelarche and Premature Adrenarche

İzel Bizbirlik Zeynep , Kilic Sümeyra , Angin Ahmet , ERSOY Betül

Orexigenic molecules such as ghrelin and neuropeptide Y (NPY) can contribute puberty as directly or indirectly. Our aim is to investigate the changes in the levels of orexigenic peptides ghrelin and neuropeptide Y at the onset of puberty in girls with idiopathic central precocious puberty (ICPP), premature thelarche (PT) and premature adrenarche (PA), and to determine their relationship with gonadotropins and anthropometric parameters.Subjects an...

hrp0095p1-548 | Pituitary, Neuroendocrinology and Puberty | ESPE2022

Legal degree of disability in childhood-craniopharyngioma survivors during long-term follow-up: Results of the HIT-ENDO study

Boekhoff Svenja , Eveslage Maria , Beckhaus Julia , Friedrich Carsten , L. Müller Hermann

Background: Cranioparyngiomas are rare low-grade embryonic malformational tumors of the sellar/parasellar region. The prognosis after diagnosis during childood and adolescence is influenced by endocrine and hypothalamic long-term sequelae. A legal status of the degree of disability (GdB), according to the German Social Code Book V that is worthy of support provides financial means for psychosocial rehabilitation and participation of craniopharyngioma survivors...

hrp0095p2-265 | Sex Differentiation, Gonads and Gynaecology, and Sex Endocrinology | ESPE2022

Gynecomastia and Short Height;45,X[2]/ 46,X, +mar[28] karyotype

Derya Buluş Ayşe , Dünya Betül , Yaşartekin Yüksel , Cevdet Ceylan Ahmet

Introduction: Physiological pubertal gynecomastia is common in adolescents aged 13-14 years. Most physiological pubertal gynecomastia regresses spontaneously within 1-2 years. In studies: If it lasts more than 2 years, if prepubertal has started, endocrine evaluation is recommended. Sex differentiation disorders or genital abnormalities such as increased aromatase activity, adrenal/testicular neoplasia, partial androgen insensitivity syndrome and Klinefelter s...

hrp0092rfc4.6 | Fat Metabolism and Obesity Session | ESPE2019

Appetite Suppressing Effects of Glucoregulatory Peptides Devoid of Nausea

Doyle Robert P. , Elfers Clinton T. , Milliken Brandon T. , Sweet Ian , Roth Christian L.

Few treatments for type 2 diabetes (T2D) and obesity achieve meaningful long-term weight-loss and are often accompanied by nausea and vomiting. Thus, there is a critical need for a new generation of obesity medications that provide glycemic control with enhanced hypophagic response without nausea. Our group has developed and tested two new monomeric chimeric peptides against a novel target for obesity treatment concomitant with T2D in the form of dual agonism of the anorectic ...