GH & IGF

hrp0084p2-392 | GH & IGF | ESPE2015

Unaltered Ratio of Circulating Levels of GH Isoforms after Administration of Different GH Provocative Tests in a Population of Short Stature Children

Rigamonti Antonello , Meazza Cristina , Bidlingmaier Martin , Muratore Valentina , Martina Luparia , Bini Silvia , Pagani Sara , Cella Silvano , Bozzola Mauro

Background: Human GH is a heterogeneous protein hormone consisting of several isoforms, the most abundant being 22 kDa- and 20 kDa-GH. The availability of analytical methods to measure these GH isoforms might represent a valuable diagnostic tool to investigate GH secretion in short stature.Objective and hypotheses: Aim of the present study was to measure circulating levels of 22 kDa- and 20 kDa-GH in children with different diagnosis of short stature suc...

0 shares

ePoster

hrp0084p2-393 | GH & IGF | ESPE2015

Study of IGF1 Receptor Gene in Small for Gestational Age Patients with Short Stature Treated with RHGH

Ferrer Marta , de Arriba Antonio , de Zabarte Jose Miguel Martinez , Barrio Eva , Labarta Jose Ignacio

Background: IGF1 is essential for pre and postnatal growth. Mutations in IGF1 receptor (IGF1R) gene have been described in patients with intrauterine growth retardation and other anomalies.Objective and hypotheses: To study IGF1R gene in small for gestational age (SGA) patients with short stature and correlate the results with clinical presentation and response to rhGH treatment.Method: Longitudinal retrospective study of 69 SGA pa...

0 shares

ePoster

hrp0084p2-394 | GH & IGF | ESPE2015

Gene Expression Profiles in GH Deficient Children Relate Peak GH Levels to Circadian Clock, Chromatin Remodelling, and WNT Signalling Pathways

Murray Philip , Stevens Adam , DeLeonibus Chiara , Koledova Ekaterina , Chatelain Pierre , Clayton Peter

Background: GH deficiency (GHD) is classically defined on the basis of a cut-off applied to the peak GH level during stimulation tests; a process with recognised limitations. Identifying the functional role of genes whose expression is associated with pGH may help with our understanding and classification of GHD.Objective and hypotheses: Identify patterns of gene expression (GE) related to pGH and to describe the function, and regulation of these genes.<...

0 shares

ePoster

hrp0084p2-395 | GH & IGF | ESPE2015

Growth and GH in Kabuki Syndrome

Schott Dina , Gerver Willem-Jan , Zimmermann Luc , Stumpel Constance

Background: Kabuki syndrome (KS; OMIM 147920) is a congenital anomaly/intellectual disability syndrome caused by a mutation in the KMT2D or KDM6A gene. Children with KS have a spectrum of clinical features, but one of the key features in KS patients is postnatal growth retardation. GH deficiency has been reported in some children with KS, but no structural research is done in this field.Objective and hypotheses: We studied the growth ho...

0 shares

ePoster

hrp0084p2-396 | GH & IGF | ESPE2015

...

0 shares

hrp0084p2-397 | GH & IGF | ESPE2015

Silver Russell syndrome: A Cause of Partial IGF1 Resistance?

Dufourg Marie-Noelle , Perin Laurence , Houang Muriel , Daubard Marie-Laure , Brioude Frederic , Bouc Yves Le , Netchine Irene

Background: Silver-Russell syndrome (SRS) is characterized by intrauterine and postnatal growth retardation, relative macrocephaly at birth, prominent forehead, severe feeding difficulties and body asymmetry. In around 50%, it is secondary to hypomethylation at the IGF2/H19 imprinted locus on 11p15 (11p15 LOM), and in 10% to a maternal disomy of chromosome 7 (mUPD7). Mechanisms of postnatal growth failure in SRS are not well understood.Objective and hypo...

0 shares

ePoster

hrp0084p2-398 | GH & IGF | ESPE2015

The In vitro Functional Analysis of Gene Promoter Region Single Nucleotide Polymorphisms Associated with GH Response in Children with GH Deficiency

De Leonibus Chiara , Murray Philip , Hanson Dan , Stevens Adam , Clayton Peter

Background: Response to GH treatment has been associated with single nucleotide polymorphisms (SNPs) within the promoter region of growth-related genes including GRB10 (rs1024531 (A/G, allele A increased response)), IGFBP3 (rs3110697 (G/A, G increased response)), CYP19A1 (rs10459592 (T/C, T increased response)) and SOS1 (rs2888586 (G/C, C increased response)). SOS1 is a positive regulator of GH signalling (MAPK pathway); the aromatase CYP19A1 promotes oestrogen synthesis to im...

0 shares

ePoster

hrp0084p2-399 | GH & IGF | ESPE2015

Is Retesting in GH Deficient Children Really Useful?

Pagani Sara , Meazza Cristina , Gertosio Chiara , Donati Chaira , Scavuzzo Francesca , Pilotta Alba , Buzi Fabio , Larizza Daniela , Bozzola Mauro

Background: Patients with childhood-onset GH deficiency (GHD) are usually retested in late adolescence or young adulthood, after achievement of final height, to verify whether they need to continue GH treatment. Most of the patients found to have idiopathic GHD when tested as children have normal GH responses when retested in the early or late adolescence. Indeed, the 2007 Consensus guidelines for the diagnosis and treatment of GHD adults recommended that idiopathic GHD patien...

0 shares

ePoster

hrp0084p2-400 | GH & IGF | ESPE2015

Regulation of IGF1R mRNA Expression by GnRH Agonist may be Involved in the Decrease of Height Velocity During Central Precocious Puberty Therapy

De Paula Mariana Sarti , Ricco Rafaela , Custodio Rodrigo , Milani Soraya , Atique Patricia , Moreira Ayrton , Antonini Sonir , Liberatorejr Raphael , Martinelli jr Carlos

Background: Growth spurt is a major event in central precocious puberty (CPP). GnRH analogue (GnRHa) therapy inhibits gonadal axis and decreases height velocity. However, serum IGF1 and IGFBP-3 remain high as before therapy. Reports on IGF type 1 receptor (IGF1R) in CPP are yet unavailable.Aim: To study IGF1R mRNA expression in girls with CPP before and during GnRHa therapy.Methods: 34 girls with CPP were studied. Sixteen of them (...

0 shares

ePoster

hrp0084p2-401 | GH & IGF | ESPE2015

GRB10 Knockdown in Zebrafish is Associated with Decreased Weight-to-length Ratio without Alterations in AKT and ERK Activity: A Model to Study Human Growth Regulation

De Leonibus Chiara , Broadbent Jack , Murray Philip , Whitehead Joseph , Hurlstone Adam , Shiels Holly , Clayton Peter , Stevens Adam

Background: In humans GRB10 negatively regulates GH and IGF1 signaling predominantly via altering phosphorylation of PI3K/mTOR/AKT and MEK/ERK pathways which relate to both growth and metabolic function. We have previously shown that Grb10 knockdown in Zebrafish results in overgrowth with an increase in length and head size. However the impact on weight in relation to length has not been assessed.Objective: To develop a model t...

0 shares

ePoster

hrp0084p2-402 | GH & IGF | ESPE2015

The Involvement of the Epidermal Growth Factor Receptor in the Successful GH Signalling and the Role of p21 in the Negative Regulation of the GH/GHR and EGF/EGFR Pathways, in GH Transduction Defect

Kostopoulou Eirini , Gil Andrea Paola Rojas , Karvela Alexia , Spiliotis Bessie Eugenia

Background: GH transduction defect (GHTD) is characterised by severe short stature and impaired STAT3 phosphorylation, which is overcome by simultaneous induction of GHTD fibroblasts with 200 ng/ml GH and short interference mRNA CIS (GH200/siRNA) or with 1 000 ng/ml GH (GH1000) and is clinically expressed with catch-up growth after rhGH treatment.Objective and hypotheses: The involvement of epidermal growth factor receptor (EGFR) in the succe...

0 shares

ePoster

hrp0084p2-403 | GH & IGF | ESPE2015

Homozygous Carriers of a Novel IGFALS Mutation are 1.5 SD Shorter than Heterozygous Relatives and Tend to have Lower Bone Mineral Density

Isik Emregul , van Doorn Jaap , Demirbilek Huseyin , Losekoot Monique , Wit Jan-Maarten

Background: There are limited data on differences in height, bone mineral density (BMD) and pubertal delay between homozygous and heterozygous carriers of IGFALS defects.Objective and hypotheses: To describe clinical and laboratory features and BMD of homozygous and heterozygous carriers of a novel IGFALS mutation in a large Kurdish family.Method: Index cases were two first degree cousins presenting with short stature, low IGF1, ve...

0 shares

ePoster

hrp0084p2-404 | GH & IGF | ESPE2015

The Role of β-TrCP, an E3 Ubiquitin Ligase, in the Signalling of the GH and Epidermal Growth Factor Pathways in Growth Hormone Transduction Defect

Kostopoulou Eirini , Gil Andrea Paola Rojas , Karvela Alexia , Spiliotis Bessie Eugenia

Background: epidermal growth factor (EGF) stimulates cell growth and differentiation through its receptor EGFR. Cross-talking between the GH and EGF signaling pathways is important for normal cellular development. GH transduction defect (GHTD), a clinical disorder characterized by impaired STAT3 phosphorylation due to excessive GHR degradation, is caused by over-expression of the E3 ubiquitin ligase, CIS.Induction of GHTD fibroblasts with 200 ng/ml hGH (GH200) and silencing mR...

0 shares

ePoster

hrp0084p2-405 | GH & IGF | ESPE2015

SGA Short Stature Bearing with a Novel Nonsense Mutation (p.W1249X) in the IGF1R Gene

Fujimoto Masanobu , Kawashima Yuki , Hamajima Takashi , Miyahara Naoki , Nishimura Rei , Hanaki Keiichi , Kanzaki Susumu

Background: The type I IGF1R plays a role in intrauterine and postnatal growth. Heterozygous IGF1R mutations have been identified in over 20 families. Some of them are linked to the etiology of short stature in previous studies. In addition, we previously reported that a heterozygous nonsense mutation (p.Q1250X) of the IGF1R gene led to decrease IGF1R protein expression through endoplasmic reticulum-associated protein degradation (ERAD) mechanism, resulted in...

0 shares

ePoster

hrp0084p2-406 | GH & IGF | ESPE2015

Metabolic Health in Short Children Born Small for Gestational Age Treated with GH and GnRHa: Results of a Randomised, Dose-response Trial

van der Steen M , Lem A J , van der Kaay D C M , Waarde W M Bakker-van , van der Hulst F J P C M , Neijens F S , Noordam C , Odink R J , Oostdijk W , Schroor E J , Sulkers E J , Westerlaken C , Hokken-Koelega A C S

Background: Previously we showed that 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 (GnRHa) and even more so with a double GH dose. GnRHa treatment is thought to have negative effects on body composition and blood pressure. Long-term effects and GH-dose effects on metabolic health in children treate...

0 shares

ePoster

hrp0084p2-407 | GH & IGF | ESPE2015

A Phase 2, 6-Month, Randomised, Active-Controlled, Safety and Efficacy Study of TransCon hGH Compared to Daily Human GH in Children with GH Deficiency

Chatelain Pierre , Malievsky Oleg , Radziuk Klaudziya , Senatorova Ganna , Beckert Michael

Background: TransCon human GH (hGH) is a long-acting prodrug of recombinant hGH that releases fully active unmodified hGH. TransCon hGH was shown in phase 1 studies in healthy volunteers and a phase 2 study in adults with hGH deficiency to: i) be safe and well tolerated, ii) provide dose-dependent hGH levels, with same peak level and exposure compared to daily GH, iii) be suitable for a once-weekly dosing regimen, and iv) induce an IGF1 response within the normal range. This o...

0 shares

ePoster

hrp0084p2-408 | GH & IGF | ESPE2015

Does Skeletal Disproportion in Children with Idiopathic Short Stature Influence Response to GH Therapy?

Cutfield Wayne S , Lindberg Anders , Hofman Paul , Derraik Jose , Geffner Mitchell E , Camacho-Hubner Cecilia

Background: Children with ISS have an array of causes that lead to short stature and/or poor growth velocity. Genetic causes of short stature, notably SHOX mutations, can be associated with subtle skeletal disproportion with shorter limbs, manifesting as increased sitting-to-standing height ratios or SDS.Objective and hypothesis: Children with ISS and skeletal disproportions have a diminished growth response to GH treatment compared to chi...

0 shares

ePoster

hrp0084p2-409 | GH & IGF | ESPE2015

The Influence of GH Therapy on Chemerin Concentration, Body Mass and Selected Parameters of Carbohydrate Metabolism in Prepubertal Non-Obese Children with GH Deficiency

Rudzka-Kocjan Agnieszka , Szalecki Mieczyslaw

Background: Chemerin is an adipocytokine which plays a great role in metabolism of carbohydrates. Chemerin concentration correlates positively with body mass (BM). GH deficiency (GHD) is associated with excess of abdominal fat tissue also in patients with normal BMI.Objective and hypotheses: To estimate the chemerin concentration and its correlation with BM and carbohydrate metabolism in non-obese, prepubertal children with isolated GHD before (GHD untre...

0 shares

ePoster

hrp0084p2-410 | GH & IGF | ESPE2015

Response to GH Treatment in the Very Young with GH Deficiency

Cetinkaya Semra , Poyrazoglu Sukran , Aycan Zehra , Siklar Zeynep , Berberoglu Merih , Atay Zeynep , Bereket Abdullah , Ercan Oya , Mengen Eda , Demirel Fatma , Darcan Sukran , Darendeliler Feyza

Aim: Data on response to GH treatment in the very young children with GH deficiency is scarce. The aim of this study was to evaluate the growth response in such children in a national multicentre study and to analyse the factors affecting the growth response.Materials and methods: In this study, we retrospectively evaluated the files of GH deficiency patients who had started GH treatment between 03 years of age who were being followed in 14 differe...

0 shares

ePoster

hrp0084p2-411 | GH & IGF | ESPE2015

Estimation of Adipsin, Omentin and Vaspin Concentration in Prepubertal Children with GH Deficiency before and after 6 Months of GH Treatment

Rudzka-Kocjan Agnieszka , Szalecki Mieczyslaw

Background: GH deficiency (GHD) is usually associated with excess of abdominal fat tissue and increased risk of developing cardiovascular diseases. Adipose tissue produces different adipocytokines that could explain the relationship between excess of fat tissue and increased metabolic risk. Adipsin, omentin and vaspin are adipocytokines which are still not well examined.Objective and hypotheses: To estimate the effect of 6 months GH therapy on serum conc...

0 shares

ePoster

hrp0084p2-412 | GH & IGF | ESPE2015

Final Height and Safety Outcomes in GH-Treated Children Born Small for Gestational Age: Experience from the Prospective GeNeSIS Observational Study

Child Chris , Quigley Charmian , Zimmermann Alan , Deal Cheri , Ross Judith , Schonau Eckhard , Blum Werner

Background: GH treatment in children born small for gestational age (SGA) has both short- and long-term growth-promoting effects and is approved in Europe and the USA (recommended dosages 0.250.47 mg/kg per week).Objective and hypotheses: To assess final height (FH) and safety outcomes in SGA patients receiving GH treatment in routine clinical practice using data from GeNeSIS.Method: 1208 GH-treated SGA patients were enrolled...

0 shares

ePoster

hrp0084p2-413 | GH & IGF | ESPE2015

Results up to January 2015 from PATRO Children, a Multi-Centre, Non-Interventional Study of the Long-Term Safety and Efficacy of Omnitrope^® in Children Requiring GH Treatment

Pfaffle Roland , Kanumakala Shankar , Hoybye Charlotte , Kristrom Berit , Schuck Ellen , Zabransky Markus , Battelino Tadej , Colle Michel

Background: PATRO Children is an international, open, longitudinal, non-interventional study designed to evaluate the long-term safety and efficacy of Omnitrope®, a biosimilar recombinant human GH (rhGH).Objective and hypotheses: Long-term safety of Omnitrope® is the primary objective of PATRO Children (particularly the diabetogenic potential of GH in short children born small for gestational age, the risk of malignancies,...

0 shares

ePoster

hrp0084p2-414 | GH & IGF | ESPE2015

GH Treatment Prevents Hypoxia-Induced Decrease of GH and IGF1 Plasma Concentrations in Neonatal Mice

Jung Susan , Richter-Kraus Mandy , Brackmann Florian , Dorr Helmut G , Trollmann Regina

Background: Hypoxia represents a main risk factor for acquired brain injuries and neurological disabilities in premature and full-term infants. Stabilization of hypoxia-inducible transcription factors (HIF) is one of the most important adaptive mechanism modulating the degree of hypoxic-ischemic brain lesions due to cellular adaptation to reduced oxygen concentrations. Additionally, neuro-protective effects of GH and GH-induced neurotrophic factors are evident as shown by prev...

0 shares

hrp0084p2-415 | GH & IGF | ESPE2015

The Pharmacokinetics and Pharmacodynamics of TV-1106, a Once Weekly GH Supplement: Results from a Phase 2 Study of TV-1106 in Adults with GH Deficiency

Cohen-Barak Orit , Christiansen Jens Sendahl , Bidlingmaier Martin , Brown Kurt , Sakov Anat , Anscheutz Gaya , Butler Kathleen

Background: TV-1106 (Teva Pharmaceuticals Ltd) is a genetically fused recombinant GH (rhGH) and human serum albumin in development as a once weekly treatment of GH deficiency (GHD) in children and adults. TV-1106 has an extended duration of action compared to daily rhGH treatment, reducing the frequency of injections.Objective and hypotheses: The pharmacokinetics and pharmacodynamics of TV-1106 were evaluated in phase 2 study using non-compartmental anal...

0 shares

ePoster

hrp0084p2-416 | GH & IGF | ESPE2015

Effects of GH Treatment on the Heart in Children with GH Deficiency

Gomez-Guzman Elena , Canete Mara Dolores , Valle-Jimenez Miguel , Caballero-Villarraso Javier , Canete Ramon

Introduction: GH deficiency (GHD) in adults is associated with impaired cardiac function, contributing to increased mortality due to cardiovascular disease. Research has shown that adults with GHD have high levels of brain natriuretic peptide as a measure of cardiac function. GH replacement therapy can improve cardiac function and lower BNP levels. The scarce research carried out in children appears to show that GHD is linked to decreased left-ventricle size and reduced left-v...

0 shares

hrp0084p2-417 | GH & IGF | ESPE2015

GH Therapy in Skeletal Dysplasias: Final Height Data

Scarano Emanuela , Procopio Matteo , Tamburrino Federica , Perri Annamaria , Bettocchi Ilaria , Vestrucci Benedetta , Rollo Alessandra , Balsamo Antonio , Mazzanti Laura

Background: Skeletal dysplasias are characterised by bone-cartilage involvement and impairment of growth and body proportions. Reports of the benefits of GH treatment are difficult to evaluate for the small number of subjects, short period of treatment, few final height (FH) data in groups with and without GH deficiency (GHD).Objective and hypotheses: The aim of our study was to assess FH and body proportions in pts with skeletal dysplasia and GHD.<p...

0 shares

ePoster

hrp0084p2-418 | GH & IGF | ESPE2015

Random Forest Classification Predicts Response to Recombinant GH in GH Deficient Children Using Baseline Clinical Parameters and Genetic Markers

Stevens Adam , Murray Philip , Wojcik Jerome , Raelson John , Koledova Ekaterina , Chatelain Pierre , Clayton Peter

Background: Prediction of response to recombinant GH (r-GH) is currently based on regression modelling. This approach generates a prediction equation which can be applied to data from an individual child. However this method can underestimate the effect of inter-dependent variables. Random forest classification (RFC) is an alternative prediction method based on decision trees that is not sensitive to the relationships between variables.Objective and hypo...

0 shares

ePoster

hrp0084p2-419 | GH & IGF | ESPE2015

Safety Evaluation of Long-Term Recombinant GH Treatment in Childhood: Interim Analysis of the NordiNet^® International Outcome Study (IOS)

Savendahl Lars , Pournara Effie , Rohrer Tilman R , Pedersen Birgitte Tonnes , Saha Marja-Terttu , Blankenstein Oliver

Background: Long-term safety data are reported for paediatric patients treated with recombinant GH (GH; Norditropin®, Novo Nordisk A/S) at the treating physicians discretion and enrolled in the observational NordiNet® International Outcome Study (IOS) (NCT00960128).Objective and hypotheses: To evaluate incidence rates (IR) (events/1 000 patient-years) of adverse drug reactions (ADR), serious adverse events (SAE), and...

0 shares

ePoster

hrp0084p2-420 | GH & IGF | ESPE2015

Hypoglycaemic Adverse Events Reported in Children Enrolled in the European Increlex^® Growth Forum Database in Europe (5-Year Interim Data)

Woelfle Joachim , Polak Michel , Bang Peter , Houchard Aude , Sert Caroline

Background: The post-authorisation registry, European Increlex® (mecasermin (rDNA origin) injection) Growth European Increlex Growth Forum Database (EU-IGFD), initiated in December 2008, collects safety and efficacy data in children receiving Increlex® for growth failure. Hypoglycaemia has been reported as a common adverse event (AE) during any IGF1 replacement therapy in randomised clinical trials, and is therefore of interest in real-life sett...

0 shares

ePoster

hrp0084p2-421 | GH & IGF | ESPE2015

Application of Neural Networks for Final Height Prediction Based on Pre-Treatment Data in Children with GH Deficiency Treated with GH

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

Background: Prediction of the effectiveness of GH therapy in children with short stature is an important issue. Artificial neural networks (ANN) seem to be promising tool for this purpose, not requiring any assumption on functions linking independent and dependent variables.Objective and hypotheses: The aim of the study was to compare ANN models of GH therapy effectiveness, based on the data available at therapy onset with multiple linear regression (MLR...

0 shares

ePoster

hrp0084p2-422 | GH & IGF | ESPE2015

Physical Performance and Right Ventricular Function in Children with GH Deficiency before and after 12 Months-GH Replacement Therapy

Barbieri Flavia , Capalbo Donatella , Lucci Rosa , Vitelli Alessandra , Esposito Andrea , Di Pietro Elisa , Rapacciuolo Antonio , Vigorito Carlo , Salerno Mariacarolina

Background: Several metabolic abnormalities, including unfavourable lipid profile, impaired cardiac performance, muscle strength and endurance capacity have been documented in GH Deficiency (GHD) adults. Alterations in cardiac morphology and left ventricular function and early markers of cardiovascular risk have been also detected in GHD children; however, no studies have so far investigated the effects of GHD on physical performance and right ventricular (RV) function in chil...

0 shares

hrp0084p2-423 | GH & IGF | ESPE2015

The Accuracy of Bioelectrical Impedance Analysis to Detect the Body Composition Changes in Adolescents with Severe GHD During Transition

Ziegler Julian , Schweizer Roland , Binder Gerhard

Background: Male adolescents with severe GH deficiency show both loss of lean body mass (LBM) and gain of fat free mass (FFM) when off GH treatment. We recently showed that determining gain of FFM and loss of LBM by dual-energy x-ray absorption (DXA) is helpful in the diagnosis of severe GHD during transition as these body composition changes are correlated to the GH-peak of the arginine-GHRH-re-test.Objective and hypotheses: We wanted to explore if the ...

0 shares

ePoster

hrp0084p2-424 | GH & IGF | ESPE2015

Timing of GH Peak in Provocation Tests is Important in Predicting the Effectiveness of Treatment with rhGH in Prepubertal Children with GHD

Georeli Irene , Triantafyllou Panagiota , Slavakis Aris , Christoforidis Athanasios

Background: Peak GH level during stimulation tests (STs) stands as an important parameter in growth prediction models and recently it was shown that timing of the peak value in glucagon stimulation test (GST) may be an important indicator of growth hormone deficiency (GHD).Objective and hypotheses: To detect a possible relationship between timing of the peak value of GH during STs and the effectiveness of treatment with rhGH in children with idiopathic G...

0 shares

ePoster

hrp0084p2-425 | GH & IGF | ESPE2015

Comparison of Baseline Parameters and Response to GH Treatment in 125 Children with Short Stature with Eight Different Diagnosis

Ioimo Irene , Mussa Alessandro , Vannelli Silvia , Feyles Francesca , Einaudi Silvia , Matarazzo Patrizia

Background: Response to rhGH treatment is variable among GH deficiency (GHD), GH insensitivity and several intermediate conditions.Aims and objectives: To compare baseline parameters and response to rhGH treatment in eight diagnostic categories of patients with short stature.Methods: We selected 125 prepubertal children presenting at least 2 years of rhGH treatment (mean 5.29 years, range 215.6 years), hormonal and clinic par...

0 shares

hrp0084p2-426 | GH & IGF | ESPE2015

The Acid-Labile Subunit Dose Matters? Response to Human GH Treatment in Patients with Acid-Labile Subunit Deficiency

Bechtold Susanne , Roeb Julia , Weissenbacher Claudia , Sydlik Carmen , Schmidt Heinrich

Background: In patients with acid-labile subunit (ALS) deficiency, the inability to build ternary complexes results in a marked reduction of circulating total IGF1. Height reduction by heterozygosity is about 1 SD in comparison to wild type. In homozygosity or compound heterozygosity a height loss of −2 to −2.5 SD occurs. This is suggestive of a gene-dose effect. How does treatment with human GH influence height development in relation...

0 shares

ePoster

hrp0084p2-427 | GH & IGF | ESPE2015

rhGH Replacement Therapy Ameliorates Body Composition Substantially but has No Effect in the Quality of Life in Adolescents with GH deficiency – A Cross-Sectional Study

Stefanaki Charikleia , Paltoglou George , Bacopoulou Flora , Boschiero Dario , Chrousos George P

Background: GH is fundamental in skeletal growth during puberty, however detailed studies of body composition analyses in adolescents with GH deficiency on GH therapy are scarce. Moreover, there are few studies on the psychologic aspects of hrGH therapy in this population.Objective and hypotheses: In this case-control study, we investigated differences in body composition based on bio-impedance measurements between adolescents with idiopathic GH deficien...

0 shares

ePoster

hrp0084p2-428 | GH & IGF | ESPE2015

Evaluation of the Effect of GH Treatment on Insulin Resistance and Cardiovascular Tissue

Saygili Seha Kamil , Kocaaga Mehmet , Kaya Gamze , Sukur Mine , Ozer Pelin , Bas Firdevs , Bundak Ruveyde , Darendeliler Feyza

Background: GH treatment may cause insulin resistance, which is associated with metabolic syndrome and co-morbidities.Objective and hypotheses: The aim of our study was to evaluate GH deficiency (GHD) patients on GH treatment for hyperlipidemia, insulin resistance and carotid intima media thickness (CIMT) and left ventricular global longitudinal strain (GLS) and assess cardiovascular tissue level effects of insulin sensitivity.Meth...

0 shares

ePoster

hrp0084p2-429 | GH & IGF | ESPE2015

Effectiveness of Recombinant IGF1 Treatment in a Patient with Isolated GH IA Deficit Producer of Anti-GH Antibodies

Porcar Ines , Oriola Josep , Kratzsch Jurgen , Escribano Joaquin , Feliu Albert

Background: GH deficiency type IA represents the most serious form of isolated deficit GH (IDGH). Its transmitted as an autosomal recessive pattern and in most cases there is a homozygous deletion of the GH1 gene. Good initial response to treatment is characteristic, although often could appear antibodies against recombinant GH.Case presentation: We report a case of a 5-year-old Pakistanist boy evaluated for severe growth failure (heigh <8.07 <...

0 shares

ePoster

hrp0084p2-430 | GH & IGF | ESPE2015

Analysis of CD133+CD45+ Hematopoietic Progenitor/Stem Cells and CD133+/CD45- Very Small Embryonic-Like Stem Cells in Children with GH Deficiency Subjected to GH Therapy

Bossowski A , Singh P , Grubczak K , Radzikowska U , Sawicka B , Miklasz P , Dabrowska M , Bossowska A , Moniuszko M

Background: GH deficiency (GHD) is an endocrine condition, caused by problems arising in the pituitary gland that does not produce sufficient quantities of GH. GHD is treated by replacing GH with one daily injections. Recent studies suggested that GH could be involved in regulation of certain stem cell subset potential and function. However, the exact effects of GH therapy on biology of stem cells in paediatric patients were not studied in detail.Methods...

0 shares

ePoster

hrp0084p2-431 | GH & IGF | ESPE2015

Effectiveness of rhIGF1 Treatment in a Girl with Leprechaunism

Kamrath Clemens , Boettcher Claudia , Wudy Stefan

Background: Infants with severe insulin resistance syndrome show failure to thrive.Objective and hypotheses: Effect of rhIGF1 treatment on growth in a patient with severe insulin resistance syndrome.Method: Case report.Results: The patient is a 4-years-old Caucasian girl of unrelated healthy parents. She was born after a 40 weeks gestation as a small for gestational age infant with a birth weight of 1970 g. A...

0 shares

ePoster

ESPE Abstracts

54th Annual ESPE

GH &amp; IGF

BiosciAbstracts