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...

hrp0094fc9.2 | Growth Hormone and IGFs | ESPE2021

A simple model with height and age at start of treatment with recombinant human growth hormone can accurately predict future growth in children with growth disorders

van Dommelen Paula , Arnaud Lilian , Masne Quentin Le , Koledova Ekaterina ,

Background: A growth prediction model would not only allow patients with growth disorders the opportunity to see the expected effect of their recombinant human growth hormone (r hGH) treatment, but also support healthcare professionals to individualise treatment to optimise growth outcomes.Aim: To develop a growth prediction model in children with growth disorders.Patients and Methods: Height and c...

hrp0086p1-p26 | Adrenal P1 | ESPE2016

Sex-Specific Differences in Hypothalamus-Pituitary-Adrenal Axis Activity in Newborns with Very Low Birth Weight

van der Voorn Bibian , de Waard Marita , Rotteveel Joost , Hartmann Michaela , van Goudoever Johannes , Lafeber Harrie , Wudy Stefan , Finken Martijn

Background: Male preterm infants are at increased risk of neonatal mortality when compared to their female counterparts. The mechanisms explaining this male disadvantage are not fully elucidated yet.Objective and hypotheses: To compare glucocorticoid metabolite excretion in urine obtained at day 10 between male and female infants born with a very low birth weight (VLBW; i.e. <1500 g). We hypothesized that male preterm infants have impaired adrenocort...

hrp0092p1-228 | Growth and Syndromes (to include Turner Syndrome) (1) | ESPE2019

Broadening of the Phenotypic Spectrum of Coats Plus Syndrome: A Patient Presenting with Extreme Short Stature as a Hallmark Feature

Riquelme Joel , Mericq Veronica , Pena Fernanda , Boogaard Merel W. , van Dijk Tessa , van Duyvenvoorde Hermine A. , Wit Jan-Maarten , Losekoot Monique

Background and Aims: Coats plus syndrome (MIM # 612199) is a highly pleiotropic disorder particularly affecting brain, eye, bone and gastrointestinal tract. We describe the phenotype of a patient with severe growth failure where whole exome sequencing (WES) revealed compound heterozygosity for two mutations in the CTC1 gene.Patient and Methods: The patient, the fourth child of healthy non-consanguineous parents, was born...

hrp0094p1-134 | Growth Hormone and IGFs A | ESPE2021

Starting recombinant human growth hormone treatment at an early age improves adherence and catch-up growth in patients with growth disorders, and highlights the importance of the new guideline on referral of short children to paediatric care

van Dommelen Paula , Zoonen Renate van , Vlasblom Eline , Wit Jan M. , Beltman Maaike , Arnaud Lilian , Koledova Ekaterina ,

Background: The new preventive child health care guideline for referral of short/tall children facilitates early detection of growth disorders. Understanding the impact of early treatment initiation on recombinant human growth hormone (rhGH) adherence and catch-up growth can support the use of the guideline.Aim: To evaluate the impact of age at rhGH treatment start on adherence (≥85% vs <85% of prescribed doses...

hrp0084p3-923 | GH &amp; IGF | ESPE2015

IGFI and Relation to Growth in Infancy and Early Childhood in Very-Low-Birth-Weight Infants and Term Appropriate for Gestational Age Infants

Jong Miranda de , Cranendonk Anneke , van Weissenbruch Mirjam M.

Background: IGFI regulates early postnatal growth of preterm infants and also plays an important role in growth in childhood. Very-low-birth-weight (VLBW) infants are at risk for reduced growth in early childhood.Objective and hypotheses: To compare IGFI and the correlation to growth parameters in infancy and early childhood in VLBW and term appropriate for gestational age (AGA) infants.Method: We included 41 VLBW children and 64 t...

hrp0092p1-10 | Adrenals and HPA Axis | ESPE2019

Global Practice of Glucocorticoid and Mineralocorticoid Treatment in Children and Adults with Congenital Adrenal Hyperplasia – Insights from the I-CAH Registry

Bacila Irina-Alexandra , Blankenstein Oliver , Neumann Uta , van der Grinten Hedi L Claahsen , Krone Ruth , Acerini Carlo , Bachega Tania SS , Miranda Mirella C , Mendonca Berenice , Birkebaek Niels H , Cools Martine , Milenkovic Tatjana , Bonfig Walter , Tomlinson Jeremy , Elsedfy Heba , Balsamo Antonio , Hannema Sabine , Higham Claire , Atapattu Navoda , Lichiardopol Corina , Guran Tulay , Abali Zehra , Mohnike Klaus , Finken Martijn JJ , Vieites Ana , Darendeliler Feyza , Guven Ayla , Korbonits Marta , de Vrie Liat , Costa Eduardo , Einaudi Silvia , van der Kamp Hetty , Iotova Violeta , Ross Richard , Faisal Ahmed S , Krone Nils P

Introduction: Despite existing guidelines there is no unified approach to glucocorticoid and mineralocorticoid replacement in congenital adrenal hyperplasia (CAH). Consequently, treatment varies in adults and children as well as across countries.Objective: We used data from the I-CAH Registry to identify geographical and temporal variations in the treatment with glucocorticoids and mineralocorticoids of children and adul...

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...

hrp0086p1-p462 | Fat Metabolism and Obesity P1 | ESPE2016

Determinants of Advanced Bone Age in Childhood Obesity

Jan de Groot Cornelis , van den Berg Adriaan , Ballieux Bart , Kroon Herman , Rings Edmond , Maarten Wit Jan , van den Akker Erica

Background: Childhood obesity is associated with advanced bone age (BA), leading to an altered growth pattern. Previous results of studies suggest that androgens, estrogens, sex hormone binding globulin (SHBG) and insulin are responsible for this phenomenon, but results are contradictory and might be biased by confounders.Objective and hypotheses: To investigate the independent effects of estrogens, androgens, SHBG and insulin parameters on BA advancemen...

hrp0092fc13.2 | Adrenals and HP Axis | ESPE2019

Sexual Dimorphism in Cortisol Production and Metabolism Throughout Pubertal Development: A Longitudinal Study

van Keulen Britt , Dolan Conor , van der Voorn Bibian , Andrew Ruth , Walker Brian , Boomsma Dorret , Hulshoff-Pol Hilleke , Rotteveel Joost , Finken Martijn

Background: Sex differences in disease susceptibility might be explained by a sexual dimorphism in hypothalamic-pituitary-adrenal axis activity, which has been postulated to emerge during puberty. The aim of this study is to assess the contribution of pubertal development to sexual dimorphism in cortisol production and metabolism.Methods: Participants, born between 1995 and 1996, were enrolled from the population-based N...