hrp0084p2-514 | Pituitary | ESPE2015

Hydrocephalus and Hypothalamic Involvement in Paediatric Patients with Craniopharyngioma or Cysts of Rathke’s Pouch: Impact on Long-term Prognosis

Daubenbuchel Anna M M , Hoffmann Anika , Gebhardt Ursel , Warmuth-Metz Monika , Sterkenburg Anthe S , Muller Hermann L

Background: Paediatric patients with sellar masses such as craniopharyngioma (CP) or cyst of Rathke’s pouch (CRP) frequently suffer disease- and treatment-related sequelae.Objective and hypotheses: We analysed the impact and prognostic relevance of initial hydrocephalus (HY) and hypothalamic involvement (HI) on long-term survival and functional capacity (FC) in children with CP or CRP.Method: Using retrospective analysis of pa...

hrp0084p3-820 | Endocrine Oncology | ESPE2015

AIP Polymorphism in Familiar Isolated Pituitary Adenomas: Case Report

Viale Maria Lorena , Hernandez Claudia L , Rampi Maria Gabriela , Serra Maria Pia , Figueroa Veronica , Day Patricia Fainstein , Brunetto Oscar H

Background: Familiar isolated pituitary adenomas (FIPA) encompasses the familiar occurrence of isolated pituitary adenomas outside the setting of syndromic conditions such as MEN1 and Carney’s complex, and comprise about 2–3% of pituitary adenomas. About 20% of FIPA have mutations in the aryl hydrocarbon receptor interacting protein gene (AIP), usually associated with a worse outcome.Objective and hypotheses: Evaluate the presence of AIP gene m...

hrp0094fc8.4 | Neuroendocrinology | ESPE2021

Cerebral infarction in childhood-onset craniopharyngioma patients: results of KRANIOPHARYNGEOM 2007

Boekhoff Svenja , Bison Brigitte , Genzel Daniela , Eveslage Maria , Otte Anna , Friedrich Carsten , Flitsch Jorg , Muller Hermann L. ,

Background: Cerebral infarction (CI) is a known vascular complication following treatment of suprasellar tumors. Risk factors for CI, incidence rate, and long-term prognosis are unknown for patients with childhood-onset craniopharyngioma (CP).Methods: MRI of 244 CP patients, recruited between 2007 and 2019 in KRANIOPHARYNGEOM 2007, were reviewed for CI. Risk factors for CI and outcome after CI were analyzed.<p class=...

hrp0094p1-110 | Adrenal B | ESPE2021

Current Management Of Acute Adrenal Insufficiency Related Adverse Events In Children- Results Of An International Survey Of Specialist Centres

Ali Salma R. , Bryce Jillian , Krone Nils P. , Grinten eHedi L. Claahsen-van der , Ahmed S. Faisal ,

Background: >There is a wide variation in the reported rate of acute adrenal insufficiency (AI) related adverse events (sick day episodes and adrenal crises) between centres.Objective: Evaluate the level of consensus on the criteria that should be considered ‘essential’ for defining and managing adverse events associated with acute AI in children.Methods: Three groups of ...

hrp0094p1-179 | Growth Hormone and IGFs B | ESPE2021

Novel dominant negative GH receptor variants provide important insights into GH receptor physiology

Andrews Afiya , Cottrell Emily , Maharaj Avinaash , Ladha Tasneem , Williams Jack , Metherell Louise A , McCormick Peter J , Storr Helen L ,

Background: Growth hormone insensitivity (GHI) encompasses normal/elevated growth hormone (GH), low IGF-I levels and growth restriction. Non-classical/mild-moderate GHI is an emerging entity which is poorly characterised, and, in many subjects, the underlying cause is unclear. Heterozygous dominant negative (DN) variants located in the intracellular/transmembrane domain of the GH receptor (GHR) cause a ‘non-classical’ GHI phenotype.<p class="abst...

hrp0097p1-11 | Adrenals and HPA Axis | ESPE2023

Girls with Premature Adrenarche but not SGA Reach Their Target Height

Bingöl Aydın Dilek , Tin Oğuzhan , Turan Hande , Bayramoğlu Elvan , Ercan Oya , Evliyaoğlu Olcay

Introduction:Premature adrenarche (PA) is the most common cause of premature pubic and axillary hair growth before the age of 8 in girls. At the time of diagnosis, the height and bone age of the cases with premature adrenarche were determined to be higher than their peers. The long-term effects of advanced bone age on the final length of the cases are controversial. In this study, it is aimed to share the long-term follow-up data of the cases diagnosed with pr...

hrp0097p2-42 | Adrenals and HPA Axis | ESPE2023

Exploring the Experiences of Parents of Children with Congenital Adrenal Hyperplasia: a study in Developing Country

Utari Agustini , Dewi Ariani Mahayu , Galih Panunggal Damianus , L Claahsen-van Der Grinten Hedi , MH Faradz Sultana , Ediati Annastasia

Keywords: Congenital Adrenal Hyperplasia, Parent’s experience, Focus Group DiscussionBackground: Congenital Adrenal Hyperplasia (CAH) leads to many unseen social burdens for parents, including ambiguous genitalia (in girls), lifelong use of medication, including stress dosing, social and psychological pressure, and stigmatization. This study aimed to investigate various lived experiences of parents caring for their...

hrp0094p2-309 | Growth and syndromes (to include Turner syndrome) | ESPE2021

Early-onset growth hormone treatment in Prader-Willi syndrome attenuates the risk of transition to severe obesity

Kodytkova Aneta , El Lababidi Eva , Čermakova Ivana , Černa Jana , Čižek Jindřich , Dvořakova Marcela , Kalvachova Božena , Magnova Olga , Neumann David , Novotna Dana , Obermannova Barbora , Plašilova Ivana , Pomahačova Renata , Průhova Štěpanka , Strnadel Jiři , Škvor Jaroslav , Šnajderova Marta , Šumnik Zdeněk , Zapletalova Jiřina , Kusalova Kateřina , Šilar Jiři , Kolouškova Stanislava , Zemkova Daniela , Lebl Jan ,

Background: Following a phase of feeding difficulties and underweight in early life, children with Prader-Willi syndrome (PWS) develop hyperphagia and a tendency towards severe obesity. Growth hormone therapy (GH) has been approved in PWS to compensate their growth failure but may exert also additional effects on muscle component and body composition as well.Objectives: We aimed to test if an early initiation of GH thera...

hrp0097p1-300 | GH and IGFs | ESPE2023

Assessment of the rhGH treatment compliance in children with growth hormone deficiency.

Scheuring Dorian , Walczak Mieczysław , Nowak Katarzyna , Dragan Wojciech , Starzyk Jerzy , Wędrychowicz Anna , Kapusta Alicja , Bossowski Artur , Sawicka Beata , Gawlik Aneta , Błaszczyk Ewa , Gieburowska Joanna , Beń-Skowronek Iwona , Trwoga Aleksandra , Sokół Martyna , Kołtowska-Häggström Maria , Kolasa-Kicińska Marzena , Łupińska Anna , Stawerska Renata , Lewiński Andrzej , Dudek Adam , Mazur Artur , Zachurzok Agnieszka , Mierzwa Magdalena , Wikiera Beata , Pyrżak Beata , Witkowska-Sędek Ewelina , Witkowska-Krawczak Ewa , Szewczak-Matan Bogumiła , Kędzia Andrzej , Moszczyńska Elżbieta , Kot Karolina , Birkholz-Walerzak Dorota , Myśliwiec Małgorzata , Petriczko Elżbieta

Aim: To identify factors affecting compliance to treatment with recombinant growth hormone (rhGH) in children with growth hormone deficiency (GHD).Study population and Methods: The following data were collected during standard visits in 8 endocrine clinics in Poland: medical history, auxological measurements, laboratory tests and the numbers of empty and full rhGH ampoules dispensed and returned by the patients. The obse...

hrp0084p3-892 | Fat | ESPE2015

The Triglyceride-to-HDL Cholesterol Ratio is Associated with Insulin Resistance in Obese Boys But Not in Obese Girls

Massa Guy , Bervoets Liene , Massa Jonas , Zeevaert Renate

Background: Children and adolescents with obesity often have insulin resistance (IR) and are at increased risk to develop coronary heart disease (CHD) in adulthood. The triglyceride to HDL-cholesterol (TG/HDL-C) ratio reflects small, dense low-density lipoprotein (LDL), an atherogenic lipoprotein particle that strongly predicts CHD.Objective and hypotheses: To evaluate the TG/HDL-C ratio in obese children and adolescents and study the relationship with a...