hrp0086p1-p814 | Syndromes: Mechanisms and Management P1 | ESPE2016

Changes to Thyroid Function (TF) Following Treatment with Growth Hormone (GH) Therapy in Children with Prader-Willi Syndrome (PWS)

Musthaffa Yassmin , Scheermeyer Elly , Hughes Ian , Harris Mark , Crock Patricia , Leong Gary

Background: Normal TF is necessary for optimal growth. Changes in the hypothalamic-pituitary-thyroid (HPT) axis following GH therapy are reported. GH therapy has been suggested to centrally inhibit TSH production as well as peripherally increasing T4 to T3 conversion which increases negative feedback on TSH production. Hypothalamic dysfunction is a feature of PWS, therefore these patients may be at risk of developing central hypothyroidism associated with GH therapy.<p cla...

hrp0095p1-99 | GH and IGFs | ESPE2022

Survey to Investigate Adherence to Growth Hormone Treatment in Children: The Impact of COVID-19 Pandemic

Eren Erdal , Cetinkaya Semra , Denkboy Ongen Yasemin , Tercan Ummuhan , Darcan Sukran , Turan Hande , Aydin Murat , Yavuz Yilmaz Fatma , Kilci Fatih , Sever Eklioglu Beray , Hatipoglu Nihal , Yuksek Acinikli Kubra , Orbak Zerrin , Camtosun Emine , Savas Erdeve Senay , Arslan Emrullah , Ercan, Feyza Darendeliler Oya

Introduction: Treatment adherence is crucial for the success of Growth Hormone (GH) therapy. Non-adherence rates have varied over a wide range from 5% to 80% in the literature. Several factors may have an impact on treatment adherence. Besides, with the COVID-19 pandemic that affected the whole world, there were problems with the hospital admission and routine controls of the patients who used GH treatment.Objective: The...

hrp0094p1-2 | Adrenal A | ESPE2021

Adrenocortical hormone profiles do not predict the molecular etiology in non-CAH primary adrenal insufficiency

Seven Menevse Tuba , Demirkol Yasemin Kendir , Tosun Busra Gurpinar , Bayramoglu Elvan , Yildiz Melek , Acar Sezer , Karaca Seda Erisen , Orbak Zerrin , Onder Asan , Sobu Elif , Anik Ahmet , Atay Zeynep , Bugrul Fuat , Demir Korcan , Dogan Durmus , Emeksiz Hamdi Cihan , Kirmizibekmez Heves , Murat Nurhan Ozcan , Yaman Akan , Turan Serap , Bereket Abdullah , Guran Tulay ,

Background: Primary adrenal insufficiency other than congenital adrenal hyperplasia (non-CAH PAI) is very uncommon in children but associated with a variety of molecular defects. Biosynthesis of adrenocortical hormones is reduced although the relation of steroid profiles with underlying molecular etiology is not yet studied.Objective: Investigation of clinical and steroid hormone profiles of a multicenter cohort of child...

hrp0095p1-469 | Fat, Metabolism and Obesity | ESPE2022

Triglyceride-glucose-alanine aminotransferase index is associated with the severity of transient elastography in pediatric non-alcoholic steatohepatitis

Seo Yoon Jong , Tae Hwang Il , Hee Yi Kyung

Background: The triglyceride glucose (TyG) index is significantly associated with the presence of nonalcoholic fatty liver disease (NAFLD) and is a reliable insulin resistance (IR) surrogate marker. Alanine aminotransferase (ALT) has been commonly used to reflect liver inflammation and liver damage in patients with chronic liver diseases, including NAFLD. Transient elastography (TE) uses pulsed echo ultrasound to measure the hardness of the liver, showing exce...

hrp0097p2-268 | Late Breaking | ESPE2023

Clinical characteristics, molecular genetics analysis results and long-term follow-up of a large cohort of congenital hyperinsulinism from Turkey: A nationwide cross-sectional study

Demirbilek Huseyin , Nuri Ozbek M , Yıldız Melek , LA Houghton Jayne , Onal Hasan , Gurbuz Fatih , Cetinkaya Semra , Cayir Atilla , Denkboy-Ongen Yasemin , Parlak Mesut , Gurpınar Tosun Busra , Mert Erbas Ibrahim , Akinci Aysehan , Okdemir Deniz , Anik Ahmet , Direk-Trabzon Gul , Acar Sezer , Yildirim Ruken , Celebi-Bitkin Eda , Turan Hande , Aydin Murat , Keskin Mehmet , Kilinc Suna , Curek Yusuf , Turan Ihsan , Mengen Eda , Odabas Sevinc , Akin Onur , Ozalkak Servan , Vuralli Dogus , Atalay Ilknur , Uçar Ahmet , Yuksel Bilgin , Ellard Sian , Hussain Khalid , E Flanagan Sarah

Objective: Congenital Hyperinsulinism(CHI) is a clinically, genetically, and histologically heterogeneous disease. Turkey is a county with highly prevalent cases of severe CHI due to the high rate of consanguinity and recessively inherited KATP gene mutations. We herein evaluated the clinical characteristics, molecular genetic analysis, and follow-up of a large nationwide cohort of CHI from Turkey.Patients and method: Th...

hrp0095p1-264 | Fat, Metabolism and Obesity | ESPE2022

Triglyceride–glucose–alanine aminotransferase index as a novel and superior predictor for non-alcoholic fatty liver disease in children and adolescents

Seo Yoon Jong , Gyeol Kim Bit , Tae Hwang Il

Background: Insulin resistance, non-alcoholic fatty liver disease (NAFLD), and metabolic syndrome (MetS) are strongly correlated with each other. Triglyceride and glucose (TyG) index, a simple and useful insulin resistance surrogate marker, has recently been reported to be superior in predicting NAFLD in adults.Objects: We evaluated the usefulness of TyG–alanine aminotransferase (ALT) for detecting NAFLD and compar...

hrp0089p3-p243 | Growth &amp; Syndromes P3 | ESPE2018

Increased Serum Activity of Liver Aminotransferases in Young Patients with Turner Syndrome

Wojcik Malgorzata , Ruszala Anna , Janus Dominika , Sztefko Krystyna , Starzyk Jerzy B.

Background: Liver tests abnormalities are common in adult patients with Turner Syndrome (TS). The data regarding liver tests in children and adolescents with TS remain lacking.Design and patients: A cross-sectional review of liver function of 100 girls with TS (age range 4–16, the mean BMI SDS 0.63 [−1,86 −6,78]); 56 receiving rhGH therapy (9 obese, 47 normal weight), and 44 receiving rhGH therapy and estrogen or estrogen/progesterone ho...

hrp0086rfc4.5 | Pathophysiology of Obesity | ESPE2016

Steroid Metabolomic Signature of Liver Disease in Childhood Obesity

Gawlik Aneta , Shmoish Michael , Hartmann Michaela F. , Malecka-Tendera Ewa , Wudy Stefan A. , Hochberg Ze'ev

Background: Analysis of steroids by gas chromatography-mass spectrometry (GC-MS) defines a subject’s steroidal fingerprint. Here, we compare the steroidal fingerprints of obese children with or without liver disease to identify the ‘steroid metabolomic signature’ of childhood non-alcoholic fatty liver disease.Methods: Urinary samples of 85 children (43 girls) age 8.5–18.0 with obesity (BMI >97%) were quantified for 31 steroid meta...

hrp0097p1-69 | Fat, Metabolism and Obesity | ESPE2023

Difference of nafld frequency between younger and older children with obesity

Stanikova Daniela , Vitariusova Eva , Kosnacova Jana , Cierna Iveta , Stanik Juraj

Background: Non-alcoholic Fatty Liver Disease (NAFLD) is a frequent complication of obesity in both adults and children and there is an alarming increase in prevalence of both. Studying of risk factors for NAFLD in children and adolescents might help to select vulnerable groups to start an early intervention.Objectives: The aim of our study was to detect the prevalence of NAFLD in pediatric obese patients (0-18.9 years, ...

hrp0097p1-200 | Adrenals and HPA Axis | ESPE2023

Evaluation of chromatin remodeling factors ATRX and DAXX and telomeres in pediatric adrenocortical tumors.

F Stecchini Monica , Carolina Bueno Ana , R de Campos Mateus , Marrero-Gutiérrez Junier , A Cardinalli Izilda , Junqueira Tais , A Scridelli Carlos , AF Molina Carlos , Tucci Silvio , B Coeli-Lacchini Fernanda , C Moreira Ayrton , S Ramalho Fernando , NZ Ramalho Leandra , R Brandalise Silvia , A Yunes Jose , Chahud Fernando , ZN Vêncio Ricardo , de Castro Margaret , R Antonini Sonir

Background: Impairment of the chromatin remodeling factors ATRX and DAXX and telomeres abnormality play a role in cancer biology, influencing the clinical outcomes. However, their roles in adrenal tumorigenesis require broader investigation.Aim: To evaluate ATRX and DAXX genotype and expression, telomere length, and the alternative lengthening of telomeres (ALT), as well as their clinical significance, in primary adrenoc...