hrp0082p2-d2-466 | Growth (1) | ESPE2014

Normal Growth in Aromatase Excess Syndrome by Pharmacological Inhibition of Aromatase Activity

Deubzer Beate , Binder Gerhard

Introduction: In the rare aromatase excess syndrome aromatase turnover from androgens to estrogens is constitutively increased. Affected males show signs of hyperestrogenism such as feminization (particularly gynecomastia), hypogonadism, and short adult height due to early epiphyseal closure.Case report: Here, we report the different statural growth of two first degree cousins affected by aromatase excess syndrome. Both carried the same heterozygote dele...

hrp0086p1-p829 | Syndromes: Mechanisms and Management P1 | ESPE2016

Premature Adrenarche in Silver-Russell Syndrome: A Longitudinal Study

Ferrand Nawfel , Schweizer Roland , Binder Gerhard

Background: Silver-Russell syndrome (SRS) is reported to be associated with early adrenarche, but substantial studies are lacking.Objective and hypotheses: We aimed to determine the median age at onset of adrenarche, the prevalence of premature adrenarche as well as its causes and consequences.Method: Currently we have collected longitudinal data from 40 children with SRS seen during the last 20 years in our centre. The patients fu...

hrp0082p2-d3-393 | Fat Metabolism & Obesity (2) | ESPE2014

Comparison of FMDXA and FMBIA in Obese Adolescents

Ziegler Julian , Schweizer Roland , Binder Gerhard

Background: Determining fat mass (FM) using methods of body composition analysis is useful in diagnosis and treatment of obese adolescents who undergo life style intervention. The use of dual-energy X-ray absorption (DXA) is time-consuming, potentially harmful and expensive. Alternative methods for accurately estimating FM are needed.Objective and hypotheses: We evaluated single-frequency arm-to-leg bioelectrical impedance analysis at 50 kHz (sf-BIA) in ...

hrp0084p1-79 | Growth Hormone | ESPE2015

Decrease of Jumping Power in Adolescents with Severe GHD After Stop of GH-Therapy

Schweizer Roland , Ziegler Julian , Binder Gerhard

Background: Recently we demonstrated that male adolescents with severe GHD (sGHD) had a significant decrease of lean body mass and increase in fat mass after stop of GH-therapy. The functional consequence of this observation is unknown.Objective and hypotheses: The aim was to study the changes in parameters of jumping mechanography in adolescents with GHD in the transition period (end of growth) after stop of GH-therapy.Patients an...

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 ...

hrp0092p1-231 | Growth and Syndromes (to include Turner Syndrome) (1) | ESPE2019

Height and Weight Dynamics in Preschool Boys with Constitutional Delay of Growth and Puberty

Reinehr Thomas , Hoffmann Elisa , Rothermel Juliane , Lehrian Thersia , Binder Gerhard

Background: Constitutional delay of growth and puberty (CDGP) is one of the most frequent norm variants in children presenting with short stature. Knowing the height, growth, and weight pattern of CDGP in the first years of life is important to distinguish CDGP from growth hormone deficiency (GHD) or other diseases.Methods: We studied height and weight in the first 5 years of life in 54 boys with CDGP including measureme...

hrp0092p1-380 | Growth and Syndromes (to include Turner Syndrome) (2) | ESPE2019

A New Model of Adult Height Prediction Validated in Boys with Constitutional Delay of Growth and Puberty

Reinehr Thomas , Hoffmann Elisa , Rothermel Juliane , Lehrian Theresia , Binder Gerhard

Background: For children with retarded bone ages such as in constitutional delay of growth and puberty (CDGP) there are no specific methods to predict adult height based on bone age. Widely used methods such as Bayley-Pinneau (BP) tend to overestimate adult height in CDGP. Therefore, we aimed to develop a specific adult height prediction model for boys in pubertal age with retarded bone age >1 year.Methods</...

hrp0089p2-p327 | Pituitary, Neuroendocrinology and Puberty P2 | ESPE2018

Changes of Body Composition of Male Adolescents with GH Deficiency are Diagnostic During Transition

Binder Gerhard , Becker Bettina , Bauer Jana Leonie , Schweizer Roland

Background: Restarting rhGH treatment in adolescents with childhood-onset GH deficiency (CO-GHD) is usually based on the GH re-test, IGF-1, additional pituitary hormone deficiencies and pituitary gland morphology, but not on body composition. Short-term changes of body composition in adolescents with CO-GHD when off rhGH may contribute to the identification of those in need of continuation of treatment.Study design: In this prospective single-centre stud...

hrp0086p1-p595 | Growth P1 | ESPE2016

The Diagnostic Value of IGF-II, IGF-I and IGFBP-3 in Silver–Russell Syndrome

Binder Gerhard , Eggermann Thomas , Weber Karin , Schweizer Roland

Background: Recently we described a family with several members having intrauterine and postnatal growth failure as well as signs of Silver–Russell syndrome (SRS) who carried a heterozygote nonsense mutation of IGF2. The patients had low IGF-II serum levels, but normal IGF-I serum levels.Objective and hypotheses: We aimed to estimate the diagnostic value of the IGF-II, IGF-I and IGFBP-3 measurements in the assessment of children with SRS.<p clas...

hrp0082fc12.3 | Obesity | ESPE2014

Increased Resting Energy Expenditure in Girls with Turner Syndrome

Binder Gerhard , Frank Laura , Blumenstock Gunnar , Schweizer Roland

Background: There is no knowledge of the energy metabolism in the presence of X chromosome aneuploidy or structural aberrations. Recently, an abnormal muscle metabolism was observed in girls with Turner syndrome (TS).Objective and Hypotheses: Resting energy expenditure was prospectively estimated by indirect spirometry in 92 short prepubertal girls at the start of GH therapy.Method: The diagnoses were TS (n=23), GH deficie...