hrp0092p1-393 | Growth and Syndromes (to include Turner Syndrome) (2) | ESPE2019

Evaluation of Body Composition and Resting Metabolic Rate in Children with Growth Hormone Deficiency

Masueva Madina , Okorokov Pavel , Pankratova Maria , Yusipovich Alexander , Shiryaeva Tatiana

Background/Aims: It is known that growth hormone regulated metabolic processes, including lipid metabolism and the amount of adipose tissue. The purpose was to study metabolic rates at rest in children with growth hormone deficiency (GHD) and their relationship with lipid and hormone levels.Objective: To analyse the body composition and resting metabolic rate (RMR) among prepubertal children with GHD<p class="abstext...

hrp0089p3-p153 | Fat, Metabolism and Obesity P3 | ESPE2018

Resting Metabolic Rate and the Development of Metabolic Disorders in Obese Children

Okorokov Pavel , Vasyukova Olga , Shiryaeva Tatiana , Peterkova Vanentina

Decreased resting metabolic rate (RMR) is a risk factor for the development and progression of obesity. Childhood obesity is accompanied by the development of metabolic disorders, which often persist in adults. The relationship between the rate of basal metabolism and development of childhood obesity complications is not well understood.Objective and hypotheses: Measure resting metabolic rate in obese children and assess the pronouncement of metabolic di...

hrp0089p2-p236 | GH &amp; IGFs P2 | ESPE2018

Artificial Neural Networks for Prediction Final Height in Children with growth Hormone Deficiency

Gavrilova Anna , Nagaeva Elena , Rebrova Olga , Shiryaeva Tatiana , Peterkova Valentina

Background: Mathematical models predicting final height (FH) and its standard deviation score (SDS) in children with growth hormone deficiency is an important tool for clinicians to manage treatment process. Previously developed models do not have enough accuracy or are not good enough for practical use.Objective and hypotheses: We used four binary and seven continuous predictors available at the time of diagnosis and start of therapy and developed multi...

hrp0089p3-p207 | GH &amp; IGFs P3 | ESPE2018

Erythropoietin and Granulocyte Macrophage Colony Stimulating Factor Levels in Growth Hormone Deficient Children after 1 Year of Growth Hormone Therapy

Pankratova Maria , Vorontsova Maria , Yusipovich Alexander , Shiryaeva Tatiana , Peterkova Valentina

Background/Aims: An increase in growth rate in children suffering from growth hormone deficiency (GHD) subjected to recombinant growth hormone treatment (rGHT) was shown to be accompanied by acceleration of metabolic processes that may stimulate hematopoiesis. Therefore, the aim of the present study was to examine the effects of one year rGHT on erythropoietin (EPO) and Granulocyte Macrophage Colony Stimulating Factor (GM-CSF) levels in GHD children.Meth...

hrp0086p2-p647 | Growth P2 | ESPE2016

Final Adult Height (FAH) in Patients with PROR-1 Gene Mutations during GH Long-Term Therapy

Anna Gavrilova , Elena Nagaeva , Tatiana Shiryaeva , Valentina Petekova , Ivan Dedov

Background: PROP-1 gene mutations are responsible for most of the cases of multiple pituitary hormone deficiencies (MPHD).Objective and hypotheses: We performed to evaluate the final adult height (FAH) in a group of patients with a PROP-1 gene mutations. Twenty-five patients (11 males) with a PROP-1 gene mutation, not treated before, were recruited. All the patients had been treated with a fixed rhGH dose (0.033 mg/kg per day) for 10.5 years (7.0–11...

hrp0095p1-197 | Thyroid | ESPE2022

A familial case of PAX8 gene variant with incomplete penetrance

Shreder Ekaterina , Vadina Tatiana , Solodovnikova Ekaterina , Shiryaeva Tatyana , Konuhova Marina , Dmitrieva Maria , Bogdanov Viktor , Nagaeva Elena , Bezlepkina Olga

Background: The PAX8 gene (Paired box gene 8) is located on the long arm of chromosome 2 (2q12–q14), contains 12 exons and encodes a similarly named PAX8 protein. This protein is a transcription factor which in the thyroid gland is essential for the follicular cells formation and takes part in the expression of the thyroid-specific genes (TG, TPO, and SLC5A5). Variants in the gene have been previously associated with autosomal dominant thyroid dysgenesis...

hrp0095p1-316 | Growth and Syndromes | ESPE2022

Curve matching to predict future growth in patients receiving recombinant human growth hormone: an interpretable and explainable method using big data

van Dommelen Paula , Arnaud Lilian , Koledova Ekaterina

Background: Prediction models demonstrate potential in predicting growth in patients receiving recombinant human growth hormone (r-hGH) for growth disorders. However, considerable information from patients is needed to calculate a predicted growth curve. The curve matching technique only requires height data. This technique identifies growth curves that are similar (matched) among real-world patients within a database. The growth curves of these ‘matched...

hrp0092p1-220 | GH and IGFs (1) | ESPE2019

Real-World Data From Electronic Monitoring of Adherence to Growth Hormone Treatment in Children with Growth Disorders: A Descriptive Analysis

Koledova Ekaterina , Tornincasa Vincenzo , van Dommelen Paula

Poor adherence to long-term growth hormone (GH) treatment can lead to suboptimal clinical outcomes. The easypod™ connect eHealth platform enables healthcare professionals to obtain an accurate picture of real-world adherence by allowing patients to transmit adherence data to a database. Our aims were to assess adherence to r-hGH (Saizen, Merck KGaA, Darmstadt, Germany) treatment with the easypod connect platform in children from treatment start to 48 months and to investi...

hrp0097fc11.6 | GH and IGFs | ESPE2023

Longitudinal analysis of the risk of brain tumour recurrence or progression in relation to the timing of commencement of growth hormone replacement therapy.

Paula Gonzalez-Mereles Ana , Gan Hoong-Wei

Introduction: Growth hormone deficiency (GHD) is the most frequent endocrine deficit in childhood survivors of brain tumours. However, there is insufficient evidence to guide the timing of growth hormone replacement (GHR). At Great Ormond Street Hospital timing is based on clinical need rather than in relation to oncological treatment. Therefore, sufficient variability in GHR timing is available to analyze its effect on tumour progression and recurrence.<p...

hrp0089p1-p202 | Pituitary, Neuroendocrinology and Puberty P1 | ESPE2018

Long-acting Octeotride Treatment in Children with Neurofibromatosis Type 1 - Optic Pathway Tumors and Growth Hormone Excess

Guiraldo Paula Ximena Molina , Hernandez Hector Salvador , Bartomeu Joan Prat , Paula Casano Sancho

Introduction: Growth hormone excess (GHE) in children with neurofibromatosis type 1 (NF-1) has been reported in some sporadic cases. Whether GHE stimulates progressive optic pathway glioma (OPG) growth is of concern. The prevalence of GHE in NF-1 has not been described and the scheme treatment has not been well characterized.Objective: To describe in children with NF-1/OPG and GH excess the treatment regimen and long term response to long- acting octeotr...