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54th Annual ESPE

Barcelona, Spain
01 Oct 2015 - 03 Oct 2015

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Barcelona, Spain; 1-3 October 2015 Further information

Poster Category 3


hrp0084p3-916 | GH & IGF | ESPE2015

Vitamin D Deficiency can Modulate GH/IGF1 Axis in GH Deficient Children

Witkowska-Sedek Ewelina , Kucharska Anna , Majcher Anna , Pyrzak Beata

Background: According to the latest studies vitamin D has an effect on the production and/or secretion of IGF1 in the liver, but the exact mechanism regulating these relationships has not been thoroughly explained.Objective and hypotheses: Evaluation of the relationship between 25(OH)D and IGF1 levels in the serum of children with GH deficiency (GHD).Method: The study group consisted of 76 GH deficient children qualified for GH the...

hrp0084p3-917 | GH & IGF | ESPE2015

Serum IGFI Concentration and Growth During Infancy Correlate to Polyunsaturated Fatty Acid Pattern

Dahlgren Jovanna , Kjellberg Emma , Strandvik Birgitta , Roswall Josefine

Background: IGF1 is important for fetal and infant growth and is influenced by nutrition. In young pigs, docosahexaenoic acid (DHA)-enriched food is associated with higher IGF1 levels but studies in human infants are lacking.Aims and objectives: To assess levels of polyunsaturated fatty acids (PUFA) relate to IGF1, birth size and growth during infancy.Methods: The setting was a population-based longitudinal cohort comprising 126 fu...

hrp0084p3-918 | GH & IGF | ESPE2015

Are Short Children with Low GH Secretion Metabolically Different from Children of Normal Height?

Tidblad Anders , Ekstrom Klas , Ritzen Martin , Marcus Claude

Background: Severe GH deficiency (GHD) leads to several metabolic effects in the body ranging from abnormal body composition to biochemical disturbances such as high insulin sensitivity. However, less is known regarding these parameters in children with a milder deficiency in GH secretion.Objective and hypotheses: To analyse if short children with a relatively low GH secretion differ metabolically from healthy children of normal height.<p class="abst...

hrp0084p3-919 | GH &amp; IGF | ESPE2015

Familial Short Stature Associated to Terminal Microdeletion of 15q26.3: Variable Phenotype not Involving the IGF1 Receptor Gene

Lucaccioni Laura , Madeo Simona F , Stanghellini Ilaria , Bruzzi Patrizia , Predieri Barbara , Iughetti Lorenzo , Percesepe Antonio

Background: Terminal deletions of chromosome 15q are associated with different degrees of pre- and post-natal growth failure, dysmorphic features, functional impairments and congenital anomalies. Although monosomies of 15q26 do not represent a classical contiguous gene syndrome, candidate genes for selected features have been identified. Short stature is referred to deletions of the IGF1-R gene, located on 15q26.3. We demonstrate evidence of phenotype comparable with 15q26 mon...

hrp0084p3-920 | GH &amp; IGF | ESPE2015

Is the Insulin Secretion in Pancreatic Beta Cells Related with IGF-1/IGFBP-1 Axis in Korean Children?

Kim Min Sun , Lee Dae-Yeol

Background: The IGF system is involved in the development of metabolic and cardiovascular disease. This study aimed to investigate the association of insulin-like growth factor-1 (IGF1), IGF-binding protein-1 (IGFBP1) and IGFBP3 with insulin resistance and type 2 diabetes in children.Methods: We included 36 children aged 10 to 16 years without known diabetes, medication, chronic disease. They were classified into three groups according to the results of ...

hrp0084p3-921 | GH &amp; IGF | ESPE2015

Severe Isolated Growth Hormone Deficiency and Myopathy in Two Brothers with RNPC3 Mutation

Gucev Zoran , Polenakovic Momir , Tasic Velibor , LeBouc Yves , Klammt Jurgen , Pfaeffle Roland , Filipovska Aleksandra

Background: We present two brothers with very distinct phenotype, possibly constituting a novel clinical and genetic entity. The common phenotype included red hair, obesity, myopathy, severe IGHD and, growth without growth hormone.Case presentation: At the age of 28 months the older brother had a height of 68 cm (−9 SD), his bone age was 6 months. The younger brother had a height of 62 cm at the age of 20 months (−5 SD), bone age 10 months. T...

hrp0084p3-922 | GH &amp; IGF | ESPE2015

Gigantism Secondary to Growth Hormone Secreting Pituitary Macrodenoma

Sng Andrew , Loke Kah Yin

Background: Pituitary gigantism is an extremely rare disorder characterised by GH excess that occurs before fusion of the epiphyseal growth plates.Case summary: A 13 years 6 month old Chinese boy presented to the paediatric endocrine outpatient clinic with tall stature. He was noted by his parents to have a continued growth spurt since 9 years old, and he required new shoes and clothes every school term. He had no headaches or visual disturbances. There ...

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...

hrp0084p3-924 | GH &amp; IGF | ESPE2015

Severe Isolated Growth Hormone Deficiency and Myopathy in Two Brothers With RNPC3 Mutation

Gucev Zoran , Polenakovic Momir , Tasic Velibor , LeBouc Yves , Amselem Serge , Klammt Jurgen , Pfaeffle Roland , Filipovska Aleksandra

Background: We present two brothers with very distinct phenotype, possibly constituting a novel clinical and genetic entity. The common phenotype included red hair, obesity, myopathy, severe IGHD and, growth without growth hormone.Case presentation: At the age of 28 months the older brother had a height of 68 cm (−9 SD), his bone age was 6 months. The younger brother had a height of 62 cm at the age of 20 months (−5 SD), bone age 10 months. T...

hrp0084p3-925 | GH &amp; IGF | ESPE2015

Influence of the -202 A/C IGFBP3 Promoter Polymorphism on Individual Variation in Body Height in Korean Girls

Yang Seung , Shim Young Suk , Kang Min Jae , Oh Yeon Joung , Hwang Il Tae

Background: The most common single nucleotide polymorphism in the IGFBP3 promoter region occurs at position -202. This polymorphic variation occurs frequently and may influence GH responsiveness and somatic growth.Objective and hypotheses: This study aimed to assess the effects of IGFBP3 promoter polymorphism on growth in children.Method: Restriction fragment length polymorphism (RFLP)-based genotyping of the -202 single nucleotide...

hrp0084p3-926 | GH &amp; IGF | ESPE2015

Usefulness of Priming with Gonadal Steroids Prior to GH Stimulation with Clonidine in the Evaluation of the GH Status of Short Children

Rakhimova Gulnara , Gilyazetdinov Kamil

Aim: To determine the usefulness of priming with gonadal steroids prior to GH stimulation with clonidine in the evaluation of the GH status of short children.Method: 21 males were studied, with a mean chronological age of 13.2±1.5 years (range 11–16 years), mean bone age 11.0±1.4 years, Tanner stage 1–2, with height 135.8±7.4 cm (Ht-SDS −2.8±0.5), and an inadequate response to an initial GH stimulation test with clonid...

hrp0084p3-927 | GH &amp; IGF | ESPE2015

Comparison of Two IGF1 Assays in Patients Treated with GH

Martinez Maria , Murillo Marta , Granada M Luisa , Aldea Marta , Guerrini Estefania , Bel Joan

Background: IGF1 measurements are used to diagnose and monitoring GH related disorders. GH dose is titrated against IGF1 concentrations which should be kept within the age-and sex-related normal range. However, IGF1 results vary widely depending on the immunoassay used. International guidelines advise to report IGF1 results as S.D. scores from an assay-specific age-related reference population.Objective and hypotheses: Our objective was to...

hrp0084p3-928 | GH &amp; IGF | ESPE2015

Acute Effects of a Training Session on IGF1 and IGFBP3 Concentrations in Brazilian Jiu-Jitsu Fighters

Filho Hugo Tourinho , Puggina Enrico , Moraes Camila , Oliveira L , Martinelli Jr Carlos

Introduction: The relationship between sports intensity and growth has become a concern in teenagers. Changes in the GH/IGF1 axis have been studied as biomarkers for training intensity in adolescents; however, reports on the effects of physical effort on GH and IGF1 levels are discordant and studies on GH and IGF1 responses in combat sports are scarce.Aim: To investigate the effects of a Jiu-Jitsu training session on serum IGF1 and IGFBP3 concentrations....

hrp0084p3-929 | GH &amp; IGF | ESPE2015

Biochemical Profiles Differentials by SGA Children Catch Up

Diez-Lopez Ignacio , Sarasua-Miranda Ainhoa , del Hoyo-Moracho Marta , de Segura Raquel Gomez , Perez-Campos Dorleta , Mararulla-Arenaza Maria Teresa , Rodriguez-Rivera Victor Manuel

Background: SGA cohort study in analytical and metabolic variables at 3 and 12 months of age with somatométrica situation.Material and methods: Live births in singleton pregnancies in our hospital during 2012–2014, PEG were studied according EG and weight/height (Spanish Tablas 2008). Tours are conducted at 0, 3, 6, 9, 12 months, with measurements of weight, height and perimeter. Blood samples are obtained at least fasting discussed 4h and exce...

hrp0084p3-930 | GH &amp; IGF | ESPE2015

IGF1 Deficiency: An Important Differential Diagnosis in Severe Growth Failure and Its Excellent Response to rhIGF1 Replacement Therapy

Giri Dinesh , Storr Helen , Savage Martin O , Ramakrishnan Renuka

Background: IGF1 is the key effector peptide in the control of normal growth. IGF1 deficiency in the presence of normal GH is associated with growth failure. This may be caused by primary defects in the GH-IGF1 axis or by conditions such as malnutrition or chronic inflammation. Severe primary IGF1 deficiency (height <−3 S.D., serum IGF1 <2.5th centile, GH normal) is an European Medicines Agency (EMA) licensed indication for rhIGF1 therapy. We repor...

hrp0084p3-931 | GH &amp; IGF | ESPE2015

5-Year Response to GH in Children with Noonan Syndrome and GH Deficiency: Our Experience and Review of the Literature

Meazza Cristina , Zavras Niki , Pilotta Alba , Gertosio Chiara , Pagani Sara , Tinelli Carmine , Bozzola Mauro

Background: Noonan syndrome (NS) is an autosomal dominant disorder characterized by specific features including short stature, distinctive facial dysmorphic features, congenital heart defects, hypertrophic cardiomyopathy, skeletal anomalies and webbing of the neck. Molecular screening has shown that the majority of individuals with NS have a mutation in the PTPN11 gene. Noonan syndrome children may show an impaired GH/IGF axis. Moreover, recombinant human GH (rhGH) has been sh...

hrp0084p3-932 | GH &amp; IGF | ESPE2015

Modification of Cardiovascular Risk Factors in Children Treated with GH

Llop Maria Escola , Gelonch Raquel Monne , Cabases Montse Olona , Navarro Angels Vilanova , Canhoto Diana Sofia de Melo , Yuste Encarnacion Torruz

Background: The administration of GH on paediatric patients to optimize the longitudinal growth, can modify some cardiovascular risk factors due to their effects on metabolism.Objective and hypotheses: Evaluation of the effect of GH on total cholesterol blood levels (TCBL), fasting blood glucose concentration (FBGC), blood pressure (BP) and BMI, comparing these variables before starting treatment, after a year and at the end. And it also assesses whether...

hrp0084p3-933 | GH &amp; IGF | ESPE2015

Adult Height in Children Born Small for Gestational Age and Treated with GH: Data from the French KIGS Database

Polak Michel , Czernichow Paul

Background: Treatment with GH in children born small for gestational age (SGA) increases height velocity (HV) but data on adult height (AH) are scarce.Objective and hypotheses: To report AH in a group of SGA children treated with GH.Method: This is a post-marketing longitudinal analysis of SGA children treated with GH and included in France in KIGS. Selection criteria were children followed at least one year and having reached AH b...

hrp0084p3-934 | GH &amp; IGF | ESPE2015

Psychosocial Functioning and Self-Perception of Children and Adolescents Treated with GH

Drosatou Chrysoula , Vlachopapadopoulou Elpis-Athina , Karachaliou Feneli , Prodromidis Apostolos , Michalakos Stefanos , Tsoumakas Konstantinos

Background: Coping with a chronic medical condition requiring prolonged treatment may have an effect on psychological adaptation and self-esteem of patients.Objective and hypotheses: To identify key factors that influence self-perception and well-being in children and adolescents on GH therapy.Method: A prospective study with the use of validated questionnaires SPP (the Greek version). The patient cohort included 272 Greek children...

hrp0084p3-935 | GH &amp; IGF | ESPE2015

Do IGF1 Generation Test Results Predict 1st-Year Growth Response to GH Treatment in Idiopathic Short Stature?

Masoom Mohammad Ajmal , Ercan Oya , Bucak Feride Tahmiscioglu , Ozcabi Bahar Taskin , Evliyaoglu Olcay

Background: It is well-known that human GH (hGH) treatment increases growth rate in idiopathic short stature (ISS) in the short term which might predict the overall height gain. However,the fact that ISS might involve a heterogeneous group of individuals with individual benefits from hGH treatment makes the decision to treat or not to treat difficult.Objective and hypotheses: The aim of this study was to investigate retrospectively whether an IGF1 genera...

hrp0084p3-936 | GH &amp; IGF | ESPE2015

Adherence to GH Treatment: Impact of Actual Height, Treatment Duration, and Puberty

Rothermel Juliane , Scheite Karl , Nazari Nadine , Hauffa Berthold , Reinehr Thomas

Background: Adherence to GH treatment is a challenge.Objective and hypotheses: We analysed the impact of treatment duration, treatment success, treatment indication, age, gender, pubertal stage, and height on treatment adherence (TA) to optimise treatment success.Method: Based on the easypod autoinjector used in the Saizen-online prospective, multicenter, open-label, noninterventional study we analyzed TA in 6 months periods. TA wa...

hrp0084p3-937 | GH &amp; IGF | ESPE2015

The Blood Oxidant System and Insulin Resistance in Girls with Turner Syndrome after 1 Year of GH Therapy

Pankratova Maria , Faassen Maria , Shiryaeva Tatyana , Nagaeva Elena , Peterkova Valentina , Baizhumanov Adil , Yusipovich Alexander , Cherkashin Alexander , Maksimov Georgy

Background: The effects of recombinant GH therapy on development of oxidative stress and insulin resistance in girls with Turner syndrome (TS) were observed.Objective and hypotheses: The aim of this study is to examine the longitudinal relationships of oxidative stress markers with the development of insulin resistance during GH treatment in girls with TS.Method: Ten prepubertal girls (aged 12–14 years; median 13.0 years) with...

hrp0084p3-938 | GH &amp; IGF | ESPE2015

An Open-Label Phase 2 Dose-Finding Study Comparing Three Different Doses of Weekly TV-1106 and Daily Recombinant Human GH (Genotropin®) in Treatment-Naive, Pre-Pubertal, GH-Deficient Children

Rosenfeld Ron G , Wit Jan M , Malievsky Oleg , Bolshova Elena , Brown Kurt , Sakov Anat , Anscheutz Gaya , Bassan Merav , Butler Kathleen

Background: TV-1106 (Teva Pharmaceuticals) is a genetically fused human GH and human serum albumin, in development for treatment of GH deficiency (GHD). TV-1106 has an extended duration of action compared to daily GH treatment and thus it is believed that treatment with TV-1106 can reduce the frequency of injections and improve compliance and quality of life for those requiring growth hormone replacement therapy. The efficacy and safety data from adult studies with TV-1106 sup...

hrp0084p3-939 | GH &amp; IGF | ESPE2015

Increasing Lean Body Mass, Phase Angle, and Total Body Water But Decreasing Body Fat Among Short-statured Children Born Small-for-Gestational Age on GH Treatment

Volkl Thomas M K , Stumpf Isabel , Dorr Helmuth-G

Background: There is a small proportion of children born SGA without postnatal catch-up growth who are presented with persistent short stature, low BMI, and decreased lean body mass (LBM). Data on body composition are rarely reported in the literature. Our study addresses the question whether human recombinant GH treatment could affect body composition in these SGA children or not.Design: We included 58 SGA children (n=20 females) with SGA (birt...

hrp0084p3-940 | GH &amp; IGF | ESPE2015

GH Dosing Patterns in Children with Isolated GH Deficiency and Multiple Pituitary Hormone Deficiency Enrolled in the NordiNet® International Outcome Study

Snajderova Marta , Pournara Effie , Pedersen Birgitte Tonnes , Blankenstein Oliver

Background: Long-term monitoring of GH treatment in children is very important.Objective and hypotheses: To describe patterns of GH dosing in clinical practice in children with isolated GH deficiency (IGHD) and multiple pituitary hormone deficiency (MPHD).Method: We analysed 7 years of GH treatment data from NordiNet® International Outcome Study (IOS) (NCT00960128), an observational study evaluating the long-term effectiveness...

hrp0084p3-941 | GH &amp; IGF | ESPE2015

Decrease of Small Dense LDL and Lipoprotein-Associated Phospholipase A2 due to Human GH Treatment in Short Children with GH Deficiency and Small for Gestational Age Status

Krebs Andreas , Kratzin Thomas , Doerfer Jurgen , Winkler Karl , Wurm Michael , van der Werf-Grohmann Natascha , Krause Alexandra , Schwab Karl Otfried

Objectives: GH deficiency (GHD) and small for gestational age (SGA) status are associated with cardiovascular risks. We therefore investigated antiatherogenic effects of GH.Methods: Subfractions of LDL and HDL, lipoprotein-associated phospholipase A2 (Lp-PLA2), and high-sensitivity C-reactive protein (hsCRP) were measured at baseline, after 8 and 52 weeks of GH treatment in 51 short children born SGA (n=33) or with GHD (n=18).<p cla...

hrp0084p3-942 | GH &amp; IGF | ESPE2015

Long-Term Insulin Sensitivity and β-Cell Function in Short Children Born Small for Gestational Age Treated with GH and GnRHa: Results of a Randomised, Dose-response Trial

van der Steen Manouk , Lem Annemieke J , van der Kaay Danielle C M , Hokken-Koelega Anita C S

Background: Pubertal children born small for gestational age (SGA) with a poor adult height (AH) expectation can benefit from treatment with GH 1 mg/m2 per day (~0.033 mg/kg per day) in combination with 2 years of GnRH analogue (2 years GnRHa) and even more so with 2 mg/m2 per day (~0.067 mg/kg per day). Concerns haven been raised about the effects of GH and GnRHa on insulin sensitivity on the long-term.Objective and hypotheses: To ...

hrp0084p3-943 | GH &amp; IGF | ESPE2015

Influence of the Application of the POI Score on the Results of GH Therapy in Prader-Willi

Salvatoni Alessandro , Bocchini Sarah , Crino Antonino , Di Candia Stefania , Grugni Graziano , Iughetti Lorenzo , Nespoli Luigi , Nosetti Luana , Padoan Giovanni , Pilotta Alba , Piran Marzia , Russotto Valeria Spica

Background: According to international guidelines Prader-Willi children during GH treatment must be closely monitored by polysomnography, ENT evaluation and IGF1 levels.Objective and hypotheses: The study aims to determine whether the modulation of GH therapy in children and adolescents with Prader-Willi Syndrome with a specific decisional score (POI score; Salvatoni A., Horm Res Paediatr. 2012) changes and to what extent the results of the therapy.<...

hrp0084p3-944 | GH &amp; IGF | ESPE2015

The Impact of GH Therapy in Noonan Syndrome Children with Identified Mutations in RAS/MAPK Pathway

Malaquias Alexsandra , Moraes Michelle , Funari Mariana , Pereira Alexandre , Bertola Debora , Jorge Alexander

Objective: To evaluate the response to recombinant human GH (rhGH) treatment in NS children with short stature and previously identified mutations in the RAS/MAPK pathway genes.Methods: 23 patients with NS (17 males; 19 PTPN11, 3 RAF1 e 1 SHOC2) were daily treated with rhGH (mean rhGH dose of 47 μg/kg per day). The main outcome measures were 1st year growth velocity, change in height SDS (Noonan syndrome specifi...

hrp0084p3-945 | GH &amp; IGF | ESPE2015

As Great Intra as Interindividual Variability in Uptake of s.c. GH Injections in Longitudinally Followed GH Treated Children

Lundberg Elena , Kristrom Berit , Andersson Bjorn , Rosberg Sten , Albertsson-Wikland Kerstin

Background: The variation in uptake of daily sc GH-injections is hardly known.Objective and hypotheses: There is a considerable variability in uptake of s.c. GH-injections both within and between children.Method: 65 children used (Genotropin® pen 4/16, needle 12 mm), dose 0.08–0.14 mU/kg per day within trials: TRN 87–010; 88–080; 88–177; followed yearly two–eight times 1992–1999 (n=214). ...

hrp0084p3-946 | GH &amp; IGF | ESPE2015

The Correlation between the Increase in IGF1 and the Growth Improvement Induced by GH Treatment in Short Children Born Small for Gestational Age

Hattori Atsushi , Eto Junya , Kawano Atsuko , Miyako Kenichi

Background: IGF1 is an important marker of GH treatment and is used to titrate the GH dose.Objective and hypotheses: We report the correlation between the GH treatment induced increase in IGF1 and growth improvement in short children born small for gestational age (SGA).Method: We recruited 13 pre-pubertal SGA children (eight boys, five girls) who received GH treatment. Eight continued treatment for >2 years. We retrospectively...

hrp0084p3-947 | GH &amp; IGF | ESPE2015

Medical and Biochemical Effects of Intervention Program in Patients with Poor Adherence to rhGH Treatment

Martinez Maria , Murillo Marta , Guerrini Estefania , Granada Maria Luisa , Bel Joan

Background: Optimising adherence to treatment in paediatric patients is important, since non-adherence may lead to incorrect interpretation of growth results and in the treatment course. It has been found that injection frequency is correlated with growth response and final height among children treated with rhGH.Objective and hypotheses: The main aim of this study was to evaluate IGF1 levels and growth velocity before and after a medical intervention in...

hrp0084p3-948 | GH &amp; IGF | ESPE2015

Favourable GH Treatment Response in a Young Boy with Achondroplasia

Krstevska-Konstantinova Marina , Slaveska Nevenka

Background: Achondroplasia is a skeletal dysplasia being the most common cause of rhizomelic dwarfism.Case presentation: We present a 10 years old boy who was first diagnosed prenatally. He had a mutation c1138G>A in the gene FGFR3 in a heterozygotic constellation. His IGF1 levels and IGFBP3 were normal. Two stimulation tests for GH were performed with normal levels of the hormone. His psychomotor development was adequate for his age except for speec...

hrp0084p3-949 | GH &amp; IGF | ESPE2015

Plexiform Neurofibroma and Demielinisant Lesions in a Patient with GH Deficiency Treated with rGH

Cima Luminita-Nicoleta , Albu Alice , Fica Simona , Barbu Carmen Gabriela

Case report: A 13-year old boy treated with rGH for short stature due to isolated GH deficiency was submitted in our clinic in June 2014 for regular follow-up. From his medical history we note that he was diagnosed with GH deficiency in 2009 (−4.5 S.D.) and started treatment with rGH 0.035 mg/kgc per day since February 2009. The physical exam showed H=149.9 cm (−0.74 S.D.), 39 kg, Tanner P3G3 and a subcutaneous left paravertebral tumou...

hrp0084p3-950 | GH &amp; IGF | ESPE2015

Growth, Development and Puberty of Patients with Congenital Multiple Pituitary Hormone Deficiencies

Haim-Pinhas Hadar , Kauli Rivka , Laron Zvi

Background: The congenital form of congenital multiple pituitary hormone deficiencies (cMPHD) is rarely differentiated from the acquired type. cMPHD is due to impaired production of several pituitary hormones, caused by mutations in the pituitary transcription factors genes: PROP1, POU1F1 (PIT1), HESX1, LHX3, LHX4. It includes short stature, delayed puberty, cognitive impairment, obesity and metabolic abnormalities.Objective and hypotheses: To evaluate i...

hrp0084p3-951 | GH &amp; IGF | ESPE2015

Predictors of Response to rhGH Treatment in 125 Children with Short Stature of Various Aetiologies

Ioimo Irene , Mussa Alessandro , Vannelli Silvia , Verna Francesca , Borraccino Alberto , Matarazzo Patrizia

Background: Response to rhGH treatment is extremely variable in pediatric growth disorders; predictors of the response are not yet clearly determined, due to disomogeneity of studied cohorts.Aims and objectives: To investigate the correlation between clinical parameters and height gain after the 1st year of rhGH treatment and at the last visit in eight different aetiologies of short stature, with the aim of identifying predictors of response to rhGH trea...

hrp0084p3-952 | GH &amp; IGF | ESPE2015

Linear Regression Model of Final Height Prediction Based on Pre-Treatment Data in Children with GH Deficiency Treated with GH

Hilczer Maciej , Smyczynska Joanna , Smyczynska Urszula , Stawerska Renata , Lewinski Andrzej

Background: Prediction of GH therapy effectiveness in children with short stature is an important issue in paediatric endocrinology.Objective and hypotheses: The aim of the study was to create a linear regression model of GH therapy effectiveness, based on the data available before treatment.Method: Retrospective analysis comprised the data of 150 short children (101 boys), diagnosed with isolated GH deficiency, who were treated wi...

hrp0084p3-953 | GH &amp; IGF | ESPE2015

Vitamin D Levels and not Vitamin A are Correlated with Height Velocity in Children with GH Deficiency Who are Under GH Treatment

Xatzipsalti Maria , Polychroni Ioulia , Vazeou Andriani , Maravelia Vasiliki , Papadimitriou Eirini , Stamogiannou Lela

Background: It has been suggested that Vitamin A intake may affect height velocity in children with GH deficiency (GHD) who were under GH replacement (GHR).Objective and hypotheses: Aim of the study was to evaluate vitamin A levels in GHD children under GHR.Method: Vitamin A levels were measured in 38 children (23 males, mean age 10.8 (S.D. 3.3) years) with GHD, after mean duration of GH treatment of 3.1 (S.D....

hrp0084p3-954 | GH &amp; IGF | ESPE2015

Long-Term Effects of GH Replacement Therapy on Hematopoiesis in GH Deficient Children

Esposito Andrea , De Martino Lucia , Barbieri Flavia , Rezzuto Martina , Improda Nicola , Cerbone Manuela , Capalbo Donatella , Salerno Mariacarolina

Background: Among their metabolic effects, GH and its mediator IGF1 have been reported to influence hematopoiesis. Indeed, GH/IGF1 axis promotes erythropoiesis and GH deficiency (GHD) has been associated with a normochromic and normocytic anemia both in adults and in children. In contrast, in vivo data on the effects of GH/IGF1 axis on leukocytes and platelets are scanty.Objective and hypotheses: To evaluate the effects of 4-years GH replacement...

hrp0084p3-955 | GH &amp; IGF | ESPE2015

Thyroid Function in Children Treated with rhGH for GH Deficiency

Triantafyllou Panagiota , Georeli Irene , Dimitriadou Meropi , Maliahova Olga , Daflos Anreas , Christoforidis Athanasios

Background: The relation between thyroid function and treatment with recombinant human GH (rhGH) has been the subject of many studies which indicate a decrease of fT4 levels and a compensatory TSH increase at rhGH therapy onset. On the other hand, we have identified a number of patients with documented primary hypothyroidism (either on treatment with L-thyroxine or not) before the onset of rhGH treatment.Objective and hypotheses...

hrp0084p3-956 | GH &amp; IGF | ESPE2015

A 5-year Follow-up of Adults, with Childhood-Onset GH Deficiency, Treated with GENOTONORM® in France

Touraine Philippe , Borson-Chazot Francoise , Delemer Brigitte , Brue Thierry

Background: Young adult patients with childhood-onset GH deficiency (GHD) whose GH replacement therapy (GHRT) is discontinued exhibit negative metabolic and physiological effects, reversible through GHRT.Objective and hypotheses: To report the characteristics and 5-year GHRT in adults with childhood-onset GHD.Method: Analysis of the subgroup of adults with childhood-onset GHD included between March 2003 and October 2006 in KIMS. In...

hrp0084p3-957 | GH &amp; IGF | ESPE2015

Somatotropic Pituitary Insufficiency in Kearns-Sayre Syndrome – The Clinical Picture, Genetic Diagnosis and Efficacy of rhGH Therapy

Rojek Aleksandra , Niedziela Marek

Background: Kearns-Sayre syndrome (KSS, OMIM #530000) is a rare disease belonging to a heterogeneous group of mitochondrial cytopathies. KSS is caused by deletions and/or duplications in the mitochondrial DNA, which lead to the dysfunction of the respiratory chain and to disorders in tissues with a high energy demands (muscle, nervous system).Case presentation: The girl was admitted to the hospital at the age of 13 years with the suspision of KSS. Progre...

hrp0084p3-958 | GH &amp; IGF | ESPE2015

The Correlation between the Increase in IGF1 24 h after the First Injection of GH and the Improved Growth

Miyako Kenichi , Mushimoto Yuichi , Kawano Atsuko

Background: IGF1 is a biomarker of GH, and is often used to titrate the dose of GH therapy. However, IGF1 production is regulated by not only GH but also other factors.Objective and hypotheses: We investigated whether the increase in IGF1 at several time points after the commencement of GH therapy could be a predictive factor for the improved growth.Method: We studied 45 pre-pubertal patients with GH deficiency (GHD) that had conti...

hrp0084p3-959 | GH &amp; IGF | ESPE2015

Late Diagnosis of a Type II/III Mucolipidoses Treated with GH Replacement Therapy

Crumpei Iulia , Belceanu Alina , Armasu Ioana , Braha Elena , Rusu Cristina , Manolachie Adina , George Zmau , Preda Cristina , Vulpoi Carmen

Background: Mucolipidoses II/III (ML) are rare autosomal recessive lysosomal storage disorders (incidence: 1/325 000 live births). They have overlapping clinical phenotypes with mucopolysaccharidosis disorders and include growth retardation, facial dysmorphism, skeletal abnormalities, respiratory and heart diseases, hepatosplenomegaly and abdominal hernias. There is no specific treatment and the management has been limited to supportive care.Case present...

hrp0084p3-960 | GH &amp; IGF | ESPE2015

Does Applying Regular Questionnaire to Patients on GH Increase the Compliance?

Genens Mikayir , Poyrazoglu Sukran , Sukur Mine , Bas Firdevs , Bundak Ruveyde , Darendeliler Feyza

Background: Compliance of patients on GH treatment is very important for the success of the treatment. In a multıcentre study done in Turkey we had shown that compliance decreased at the end of 1st year of treatment which had an adverse effect on growth rate and IGF1 levels.Objective and hypotheses: To evaluate whether doing regular questionnaire to patients on GH has an effect on compliance at long term.Method: A questionnair...

hrp0084p3-961 | GH &amp; IGF | ESPE2015

Characterisation of Children Born Small for Gestational Age within the Australian Indications for GH (GH) Therapy: An OZGROW Analysis

Hughes Ian , Harris Mark , Cotterill Andrew

Background: Small for gestational age (SGA) without subsequent catch up growth is an indication for GH treatment in Europe, the US, and Korea but not in Australia. However, many SGA are likely to be included under the ‘short stature and slow growth’ (SSSG) indication. It is unknown to what extent children born SGA are included in the Australian indications or how they differ from non-SGA patients within each indication and gender.Objective and ...

hrp0084p3-962 | GH &amp; IGF | ESPE2015

Effectiveness of rhGH Treatment in a Boy with Nephrogenic Diabetes Insipidus

Kamrath Clemens , Wudy Stefan

Background: The majority of children with primary nephrogenic diabetes insipidus grow below the third centile.Objective and hypotheses: Effect of rhGH treatment on growth in a patient with primary nephrogenic diabetes insipidus.Results: The patient is an 11-years and 2 month old Caucasian boy of unrelated healthy parents. At the age of 7 years and 9 month he was admitted to our hospital for evaluation of polydipsia and polyuria. Hi...

hrp0084p3-963 | GH &amp; IGF | ESPE2015

The Easypod™ Connect Observational Study: Comparison of Results from Interim Analyses

Davies Peter , Nicolino Marc , Norgren Svante , Stoyanov George , Koledova Ekaterina , VanderMeulen John

Background: The Easypod Connect Observational Study (ECOS) observational study follows children with GHD, SGA and Turner syndrome receiving r-hGH therapy for up to 5 years, with interim analyses each year. The easypod electromechanical auto-injector device enables accurate, real-world digital records of patients’ adherence to rhGH to be collected for evaluation.Objective and hypotheses: The primary objective of ECOS is to evaluate the level of adher...

hrp0084p3-964 | GH &amp; IGF | ESPE2015

Evaluation of the Facility of Use of a New GH Administration Device – Study DAGH2014

Rivero-Martin Maria Jose , Ontanon-Nasarre Ana , Alcazar-Villar Maria Jose , Carrasco-Torrents America , Andres-Rosado Ana , Montes-Bentura David

Background: One of the limiting factors in adherence to GH therapy, is satisfaction with the administration device used.Objective and hypotheses: With the emergence of a new biosmiliar GH (BGH) administration device, we will assess the simplicity, ease of use, management and pain perceived by the patients.Method: Prospective through survey caregivers and children treated with BGH at least for 6 months. Signed informed consent was r...

hrp0084p3-965 | GH &amp; IGF | ESPE2015

Effect of Human Growth Hormone on Growth Rate of Short Stature Children with Low Birth Weight

Saffari Fatemeh , Hassani Hoda , Esmailzadehha Neda , Javadi Amir

Background: If children with intrauterine growth retardation (IUGR) are stunt after birth, they will not have the desired height. Short stature is not fatal but affects personality and social and physical development of children.Objective and hypotheses: The aim of this study was to determine the effect of human GH on growth rate of short stature children with history of low birth weight.Method: This study was conducted on 148 chil...

hrp0084p3-966 | GH &amp; IGF | ESPE2015

GH Treatment and First Year Response: A Retrospective Study

Moniz Catarina , Vasconcelos Carlos , Limbert Clotilde , Saraiva Catarina

Background: GH treatment is proven to increase adult final height in some pathology and first year response is assumed as an index of treatment’s effectiveness.Objective and hypotheses: Identify the prevalence of each indication in children treated with GH in our hospital and evaluate the first year response to treatment.Method: We retrospectively analysed the files of 30 patients followed for short stature and on GH treatment...

hrp0084p3-967 | GH &amp; IGF | ESPE2015

Usefulness of Reevaluation of Growth Hormone Secretion During Puberty

Cavarzere Paolo , Ramaroli Diego , Lauriola Silvana , Morandi Grazia , Gaudino Rossella , Antoniazzi Franco

Background: Endogenous GH secretion physiologically increases during puberty. In particular, a correlation between GH levels and pubertal stages can be stated. Therefore, it is possible that some patients with childhood-onset GH deficiency (GHD) at puberty normalize their GH secretion. Finally, there are not so far assessed potential predictors of persistent GHD in patients during puberty.Objective and hypotheses: Our study aims evaluating the normalisat...

hrp0084p3-968 | GH &amp; IGF | ESPE2015

Bone Age Maturation in Prader-Willi Syndrome on GH Treatment is Accelerated in Pre-Pubertal Age without Affecting Final Height

Eiholzer Urs , Obwegeser Carla , Witassek Fabienne , Meinhardt Udo

Background: In children with Prader-Willi Syndrome (PWS) on Growth Hormone treatment (GHT) bone age (BA) acceleration is often observed. Little is known on reasons and consequences.Objective and hypotheses: To quantify BA acceleration in pre-pubertal PWS children on GHT and to investigate how BA correlates with weight gain and age at onset of GHT. To assess how final height depends on pre-pubertal bone maturation, weight gain and age at onset of GHT....

hrp0084p3-969 | GH &amp; IGF | ESPE2015

Evaluating First Year Response and Final Height to Growth Hormone Treatment in Growth Hormone Deficiency Based on Peak GH Levels on Testing

Abali Saygin , Bas Serpil , Akbarzade Azad , Atay Zeynep , Haliloglu Belma , Guran Tulay , Turan Serap , Bereket Abdullah

Background: Diagnosis of GH deficiency (GHD) is a complicated issue especially in isolated GH deficiency. Auxological evaluation, IGFI, IGFBP3 levels and GH response to provocative testing are all considered in the diagnosis. However, cut-off values for GH levels at stimulation tests are controversial. We aimed to evaluate the response to rhGH treatment in patients with different GH peak levels in stimulation test.Objective: We aimed to evaluate the resp...

hrp0084p3-970 | GH &amp; IGF | ESPE2015

Growth Hormone Therapy in Children: Predictive Factors and Short-Term and Long-Term Response Criteria in an Italian Cohort

Partenope Cristina , Pruccoli Giulia , Damia Chiara Maria , Ferrarello Maria Piera , Garbetta Gisella , Osimani Sara , Weber Giovanna , Pozzobon Gabriella

Background: The correct diagnosis of growth hormone deficiency (GHD) and the definition of growth response in the management of growth hormone (GH)-treated children is controversial.Objective and hypotheses: To evaluate: i) short-term and long-term efficacy of GH treatment; ii) various criteria commonly used to define poor response to GH therapy and compare them in the same cohort of GHD patients.Method: Our study includes 94 child...

hrp0084p3-971 | GH &amp; IGF | ESPE2015

Patients with Childhood Onset Growth Hormone Deficiency Treated with rhGH – Reevaluation in the Transition Period between Childhood and Adulthood – Preliminary Study

Procopiuc Camelia , Caragheorgheopol Andra , Gherlan Iuliana , Brehar Andreea , Padure Adriana , Dumitrascu Andra , Costache Mariana , Ardeleanu Ioana , Dumitrescu Crisina

Background: More than two thirds of teenagers with childhood-onset GH deficiency (CO-GHD) documented normal GH response when retested at final height.Objective and hypotheses: To identify potential predictors for persistent GHD after reaching final height under rhGH with a particular accent on children with isolated GHD (IGHD).Method: Prospective study: reevaluation CO-GHD in the transition period; cohort of 27 CO-GHD patient who r...

hrp0084p3-972 | GH &amp; IGF | ESPE2015

Time Trends in Baseline Characteristics (2006–2014) in Short Children with Growth Hormone Deficiency (GHD), Born Small for Gestational Age (SGA) and with Ullrich-Turner Syndrome (TS) Enrolled in Nordinet® International Outcomes Study (IOS) in Germany and Czech Republic

Dorr Helmuth Gunther , Bramswig Jurgen , Meckes-Ferber Stefanie , Pournara Effie , Pedersen Birgitte Tonnes , Snajderova Marta

Background: Early diagnosis of growth disorders and initiation of GH therapy at a younger age improves clinical outcomes.Aims and objectives: To analyse time trends in baseline parameters at GH treatment start (2006 – 2014) in short children with GHD, SGA and TS from Germany and Czech Republic enrolled in NordiNet® IOS (NCT00960128).Method: Baseline data (chronological age, height, weight, BMI, GH dose) from pa...

hrp0084p3-973 | GH &amp; IGF | ESPE2015

A Patient with an 13q Deletion Syndrome, Important Growth Delay and Somatotropine Insufficiency Undergoing Growth Hormone Therapy–Case Report.

Wolaniecka-Deahan Kinga , Szalecki Mieczyslaw , Chrzanowska Krystyna

Background: Growth deficiency is a common symptom of many genetic syndromes. 13q deletion is a very rare genetic syndrome described in almost 200 cases. Growth reduction is a constant symptom along with mental retardation, congenital defects varying according to the deleted region of chromosome 13.Objective and hypotheses: The aim of this paper was to present the case of a 12 year old patient with an interstitial deletion 13q (22.3–31.1), important ...

hrp0084p3-974 | GH &amp; IGF | ESPE2015

Linear Growth in a Child with Ellis Van Creveld Syndrome: Positive Effect of Growth Hormone Therapy

Soliman Ashraf , Alyafei Fawzia

Background: Ellis van Creveld syndrome (EVC) (chondroectodermal dysplasia) is a rare, multisystem disorder. It probably affects only around 1 in every 150,000 individuals. It is characterized by a long, narrow trunk and shortened arms and legs; extra fingers (postaxial polydactyly), and abnormalities of the oral region and teeth. In infants, non-bony manifestations, particularly congenital heart defects, may be health or life-threatening.Case presentatio...

hrp0084p3-975 | GH &amp; IGF | ESPE2015

Factors Effecting Response to Growth Hormone Treatment in Children with Turner Syndrome

Bas Serpil , Abali Saygin , Atay Zeynep , Haliloglu Belma , Gurbanov Ziya , Guran Tulay , Bereket Abdullah , Turan Serap

Background: Short stature is the most common presenting symptom in Turner Syndrome (TS). GH treatment helps alleviating short stature in TS, although response to treatment varies significantly.Objective and hypotheses: We aimed to evaluate the response to GH treatment and factors affecting this response in children with TS.Method: Forty-nine patients with TS diagnosed by cytogenetic analysis and who had been followed minimum of 1-y...

hrp0084p3-976 | GH &amp; IGF | ESPE2015

Thyroid Function in Children with Prader-Willi Syndrome, the First 12 Months of GH Therapy

Musthaffa Yassmin M , Hughes Ian P , Harris Mark , Leong Gary

Background: Normal thyroid function is necessary for the optimal growth promoting effects of GH. Changes in the hypothalamic-pituitary-thyroid (HPT) axis following GH have been reported in subjects initially thought to be euthyroid. A specific group of patients, children with Prader-Willi syndrome (PWS), are thought to have a ‘vulnerable’ HPT axis.Objective and hypotheses: To evaluate the impact of paediatric GH therapy on thyroid hormone statu...

hrp0084p3-977 | GH &amp; IGF | ESPE2015

Congenital Hypopituitarism and Severe Developmental Delay Associated with Homozygous POU1F1 Mutation

Melikian Maria , Tiulpakov Anatoly , Gavrilova Anna

Background: Mutations in POU1F1 is a rare cause of combined pituitary hormone deficiency, which commonly includes GH, TSH and prolactin deficiencies and characterised by hypoplastic anterior pituitary.Objective and hypotheses: To present a case of severe short stature and developmental delay 1.5 years old girl, who was admitted to our hospital because of short stature.Method: Hypopituitarism panel’ genes were sequence...

hrp0084p3-978 | GH &amp; IGF | ESPE2015

GH Therapy in Lery-Weill Syndrome: Report of Three Cases

Guidoni Elisa , Franceschini Michela , Mucaria Cristina , Scarinci Renato , Municchi Giovanna

Background: SHOX deficiency is a frequent cause of short stature. GH therapy has been approved for growth promotion in individuals with SHOX mutations by FDA and EMEA.Case report: Three patients with Leri-Weill syndrome (LWS) started GH therapy at different chronological ages (CA). Patient 1 started GH+LHRHa at the CA of 13.4 years, height 148.4 cm (−1.5 S.D.), Tanner stage 4, bone age (BA) 13 years. Target height (TH) 160.5 cm (&#87...

hrp0084p3-979 | GH &amp; IGF | ESPE2015

The Assessment of Quality of Life and New Technologies for Therapeutic Monitoring in a Cohort of Paediatric Patients Treated with GH

Pruccoli Giulia , Partenope Cristina , Ferrarello Maria Piera , Damia Chiara Maria , Osimani Sara , Garbetta Gisella , Weber Giovanna , Pozzobon Gabriella

Background: Short stature may represent a significant psychosocial problem. The rationale for GH treatment has traditionally rested upon the clinical improvement in terms of growth and well-being. Great importance is also associated with the adherence to the therapy.Objective and hypotheses: We have investigated the benefits obtained by GH treatment, the factors that positively influence growth, the differences between ‘objective’ and ‘sub...

hrp0084p3-980 | GH &amp; IGF | ESPE2015

Final Height in Patients with Isolated GH Deficiency and Multiple Pituitary Hormone Deficiencies, Treated with GH

Bashnina Elena , Berseneva Olga , Fogt Sergey

Background: To date a lot of data on the efficacy of GH treatment of children with short stature was accumulated. GH is the major but not exclusive endocrine regulator of linear growth. Influence of multiple pituitary hormone deficiencies on the final growth remains poorly understood.Aim: To compare the results of treatment with GH in patients with isolated GH deficiency and multiple pituitary hormone deficiencies.Methods: 15 patie...

hrp0084p3-981 | GH &amp; IGF | ESPE2015

Current Practice in Diagnosis and Treatment of GH Deficiency in Childhood: A Survey from Turkey

Poyrazoglu Sukran , Akcay Teoman , Atabek Mehmet Emre , Bideci Aysun , Bircan Iffet , Bober Ece , Can Sule , Darcan Sukran , Ersoy Betul , Guven Ayla , Kara Cengiz , Keskin Mehmet , Kurtoglu Selim , Ozbek Mehmet Nuri , Ozgen Tolga , Siklar Zeynep , Simsek Enver , Turan Serap , Yuksel Bilgin , Darendeliler Feyza

Background: Diagnosis and treatment of GH deficiency(GHD) in children are different between countries, and even among centres in the same country.Objective and hypotheses: To evaluate current practices in diagnosis and treatment of GHD in the process of preparing the new consensus on GHD by Turkish Society for Pediatric Endocrinology and Diabetes.Method: A questionnaire was sent out to all paediatric endocrinology centres.<p cl...

hrp0084p3-982 | GH &amp; IGF | ESPE2015

Experience of the Use of Genetically Engineered GH ‘Rastan’ by Children in Clinical Practice

Kulagina Elena , Savelyeva Elena , Chistousova Galina

Objective: To evaluate the efficacy of the drug ‘Rastan’, (manufacturer JSC ‘Pharmstandard-Ufa Vita’), in children with GH deficiency at the beginning of treatment at different ages.Materials and methods: We examined two groups of patients with GH deficiency who receive the drug for 3 years. In group 1, nine boys and two girls with age at debut of therapy 5.88±1.35 (from 3.3 to 7.1 years); in the 2nd group of ten boys and two gir...

hrp0084p3-983 | GH &amp; IGF | ESPE2015

GH Therapy in Kuwait: First Report on Characteristics and Response in Treated Children

Al-Abdulrazzaq Dalia , Al-Basari Iman

Background: Recombinant GH (rGH) treatment is approved in many countries for treatment of short stature in a number of childhood diagnoses. rGH was first introduced in Kuwait in the 1990s. Since its introduction, there has been no reported data on the clinical profile of treated children. There is a huge gap in knowledge of use and response to Paediatric rGH therapy in Kuwait and the region.Objective and hypotheses: The objective of this study is to repo...