hrp0097p1-484 | Fetal, Neonatal Endocrinology and Metabolism | ESPE2023

Relationships between birth body weight<10.th centile (SGA) and insulin-like growth factor-ii / insulin-like growth factor binding protein-3 ratio in the not-life threatened newborn: relevance of birth chest circumference / birth body weight ratio and oxygen supplementation

Terzi Cesare , F. Blum Werner , Magnani Cristiana , Tridenti Gabriele , Cerioli Andrea , Riani Marco , Chesi Elena , Luigi De Angelis Gian , Bernasconi Sergio , Virdis Raffaele , Banchini Giacomo

Direct relationships of estimated birth brain weight(BRW) to birth body weight (BW) ratios (BBR) and of BW<=10.th centile for GA(SGA) with blood serum Insulin-like Growth Factor-II(IG2) to blood serum Insulin-like Growth Factor Binding Protein-3 (IB3) ratios (IG2/IB3R), and inverse relations between BW-SDS and birth chest circumference(CC) / BW ratio (i.e., CC through BW; CC/BWR) have been detected by our group in the human newborn(NWB). We evaluated the possibility that CC...

hrp0082fc13.6 | Thyroid | ESPE2014

Abnormal Thyroid Hormone Metabolism in Patients with THRA Mutations due to Impaired Expression of the Type 3 Deiodinase

van Gucht Anja , van Mullem Alies , Horn Sigrun , Meima Marcel , Forrest Douglas , van Heerebeek Ramona , Visser Edward , Heuer Heike , Peeters Robin , Visser Theo

Background: Patients with a mutation in the thyroid hormone (TH) receptor TRα1 are characterized by growth retardation, delayed bone development, mild cognitive defects and constipation. They also have abnormal TH levels: low FT4, high T3, and low rT3 levels, suggesting an altered peripheral TH metabolism by deiodinases. The type 3 deiodinase (D3) inactivates TH by catalyzing the degradation of T3. D3 is importantly expressed in...

hrp0094p2-452 | Thyroid | ESPE2021

Complications after pediatric thyroidectomy: lymph node dissection is a risk factor for permanent hypocalcemia

van Rooijen Jesse J. , Paul van Trotsenburg A.S. , Zwaveling-Soonawala Nitash , Nieveen van Dijkum Els J.M. , Engelsman Anton F. , Derikx Joep P.M. , Mooij Christiaan F.

Background: Thyroidectomy is a definitive treatment option in some benign thyroid disorders and the definitive treatment option for thyroid cancer. As postoperative mortality is extremely rare data on postoperative complications and long-term health consequences are important.Objective: To evaluate the frequencies of short- and long-term complications, and their risk factors in pediatric patients (0-18 years) who underwent a thyroidectomy in a tertiary c...

hrp0097rfc13.3 | Pituitary, neuroendocrinology and puberty 2 | ESPE2023

Treatment and outcome of the Dutch Childhood Craniopharyngioma Cohort study; first results after centralization of care

van Schaik Jiska , Schouten-van Meeteren Netteke , Vos-Kerkhof Evelien , Janssens Geert , Porro Giorgio , Fiocco Marta , Bakker Boudewijn , Tissing Wim , Hoving Eelco , van Santen Hanneke

Introduction: Childhood craniopharyngioma (cCP) has excellent survival, but quality of life may be severely hampered by hypothalamic dysfunction. We aimed to evaluate treatment and hypothalamic outcomes of a Dutch cCP cohort, and evaluate the effect of centralization of care.Methods: A retrospective cohort study was performed, including cCP patients diagnosed between 2004-2021. Treatment characteristics and hypothalamic ...

hrp0095p1-185 | Sex Differentiation, Gonads and Gynaecology, and Sex Endocrinology | ESPE2022

Puberty induction in boys with CHARGE syndrome and hypogonadism: experiences of patients, parents and doctors

Dijk Dieuwerke , Bocca Gianni , Ranchor Adelita , Van Ravenswaaij-Arts Conny

CHARGE syndrome is a rare and complex disorder with an incidence of 1:15000 to 1:17000 live births. It is associated with a wide range of issues, including, but not limited to, coloboma of the eye, congenital heart disease, atresia of the choanae, retardation of growth and development, hypogonadotropic hypogonadism, ear abnormalities and hearing loss. Hypogonadotropic hypogonadism is present in 60-88% of individuals with CHARGE syndrome. In these patients, hormone replacement ...

hrp0092p1-221 | GH and IGFs (1) | ESPE2019

Individual Patterns of Objectively Measured Adherence to Growth Hormone Treatment and its Effect on Growth in Prepubertal Children with Growth Hormone Deficiency

van Dommelen Paula , Wit Jan M , Koledova Ekaterina

The easypod™ electromechanical injection in combination with the easypod Connect platform electronically records and transmits, in real time, accurate, objective records of the date, time and dose injected for patients receiving Growth Hormone (GH) for growth disorders, limiting the risk of misreporting and allowing physicians to accurately monitor patient behavior.The aim was to study individual patterns of adherence from start treatment up to 24 m...

hrp0089p3-p007 | Adrenals and HPA Axis P3 | ESPE2018

Refractory Cyclical Cushing’s Disease - a Case of Multiple Pituitary Micro-adenomas in a Three Year Old Girl after 8 Years Follow up

Robinson Elizabeth , Poonam Poonam Dharmaraj , Heyningen Carl van

Case presentation: A 3 years 10 months old British white girl presented with rapid weight gain of 11 kg over 4 months, hirsutism, central obesity, moon face, buffalo hump and hypertension.Investigations: Plasma cortisol, IGF-1 and ACTH levels were elevated. The 9am plasma cortisol was 1035 nmol/l (140–500) with simultaneous plasma ACTH 13 pmol/l (1–11). Plasma cortisol and ACTH levels responded to both dexamethasone suppression and CRH stimulat...

hrp0086rfc9.7 | Pathophysiology of Disorders of Insulin Secretion | ESPE2016

Chronotype and Type 2 Diabetes Risk in Preadolescents

Dumin Magdalena , O'Sullivan Katie , Van Cauter Eve , Koren Dorit

Background: An individual’s chronotype, or preference in the timing of sleep or food intake, may have metabolic implications. Late chronotype has been associated with higher body mass index (BMI) and hemoglobin A1c (HbA1c) in adults and greater BMI, portion sizes, and lower HDL cholesterol levels in adolescents.Objective and hypothesis: To examine associations between chronotype and risk factors for type 2 diabetes in children ages 10–13 years....

hrp0082p2-d1-518 | Pituitary | ESPE2014

Goliath, a Variant of DAVID Syndrome?

Hasselmann Caroline , Samuels Mark E , Van Vliet Guy

Background: DAVID syndrome (deficit in anterior pituitary function and variable immune deficiency) (J Clin Endocrinol Metab 97 E121, 2012) can be caused by NFKB2 mutations (Am J Hum Genet 93 13, 2013). All patients have an orthotopic posterior pituitary (PP) and most only ACTH deficiency.Objective and Hypothesis: To describe a girl with common variable immunodeficiency (CVID), ectopic PP (EPP) ...

hrp0084p1-119 | Puberty | ESPE2015

Lipid Profiles in Gender Dysphoric Adolescents Treated with GnRH Agonists Alone and in Combination with Cross-Sex Hormones

Schagen Sebastian , Delemarre-van de Waal Henriette , Hannema Sabine

Background: In gender dysphoric adolescents GnRH agonists can be used to suppress pubertal development of the natal sex. Subsequently cross sex hormones can be given to induce pubertal development of the experienced gender. Only few data are available on the safety of this treatment. Lipid levels are known to increase during puberty and pubertal suppression may alter this increase. In gender dysphoric male-to female (MtF) adults oestrogens has been shown to result in a more fa...