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...

hrp0082p2-d2-433 | Growth Hormone (1) | ESPE2014

Comparison of Measured Lean Body Mass (LBMDXA) and Estimated LBMBIA in Children with GH Deficiency

Schweizer Roland , Ziegler Julian , Frank Laura , Binder Gerhard

Background: Long standing GH deficiency (GHD) causes loss of muscle mass. DXA enables the measurement of LBMDXA but is accompanied with a potentially harmful X-ray exposition. BIA measures the electric resistance of the body which correlates with LBM. A comparison of both methods in children with GHD has not been performed yet.Aim: Calculation of a regression formula for LBM using resistance and anthropometry based on LBMDXA in chil...

hrp0097rfc13.2 | Pituitary, neuroendocrinology and puberty 2 | ESPE2023

Arginine-stimulated copeptin is independent of GH secretion status

Binder Gerhard , Weber Karin , Peter Andreas , Schweizer Roland

Background: Copeptin is secreted in isomolar amounts along with arginine vasopressin peptide from the posterior pituitary. Its stability makes it a perfect candidate for the endocrine approach in the diagnosis of AVP deficiency. Arginine-stimulated copeptin is a possible alternative for the water deprivation test. We wondered whether basal and stimulated copeptin secretion is related to growth hormone secretion status or independent of it.<p class="abstext...

hrp0089p3-p310 | Pituitary, Neuroendocrinology and Puberty P3 | ESPE2018

Poland’s Syndrome and Hypogonadotropic Hypogonadism

Stancampiano Marianna Rita , Meroni Silvia Laura Carla , Lascio Alessandra Di , Gianninoto Moira , Russo Gianni

Poland’s syndrome is characterised by unilateral absence or hypoplasia of the pectoralis muscle, associated with the ipsilateral malformation of the hand. The syndrome is usually sporadic and occurs in about 1:32.000 live births. Poland’s syndrome has been described associated with other abnormalities, including renal aplasia or hypoplasia, hemivertebra, Klippel-Feil syndrome and Moebius’ syndrome. In literature are reported six cases of Moebius syndrome associa...

hrp0095t7 | Section | ESPE2022

Efficacy and safety of once-weekly somatrogon in pediatric subjects with growth hormone deficiency: lack of impact of anti-drug antibodies

Deal Cheri , Silverman Lawrence , Korth-Bradley Joan , Roland Carl , Taylor Carrie , Cara Jose , Wajnrajch Michael

Objectives: Somatrogon, a long-acting recombinant human growth hormone (GH) consisting of the amino acid sequence of human GH (hGH) and three copies of the carboxy-terminal peptide (CTP) of human chorionic gonadotropin, is approved by the EMA for treatment of children with GH deficiency (GHD). In this phase 3 study, children with GHD received either somatrogon or Genotropin. The impact of testing positive for anti-drug antibodies to somatrogon (ADA+) on the ef...

hrp0092fc6.5 | Bone, Growth Plate and Mineral Metabolism Session 2 | ESPE2019

No Change in Bone Density During 6 Months Off GH in Adolescents with Severe GHD at Near-Adult Height

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

Objective: Re-testing of childhood-onset GHD needs discontinuation of GH treatment at near-adult height. We recently reported significant changes of body composition as a consequence of severe GHD during this time period.Aim: Does a 6 month interruption of GH treatment decrease bone quality significantly in patients with severe GHD of adolescence?Patients and Methods: In 90 patient...

hrp0089rfc15.6 | Growth and syndromes | ESPE2018

Latest Results from PATRO Children, a Multi-Centre, Non-Interventional Study of the Long-Term Safety and Efficacy of Omnitrope® in Children Requiring Growth Hormone Treatment

Kanumakala Shankar , Pfaffle Roland , Hoybye Charlotte , Kristrom Berit , Battelino Tadej , Zabransky Markus , Zouater Hichem

Objectives: PATRO Children is a non-interventional, international, longitudinal study of the long-term safety of a recombinant human growth hormone (rhGH; Omnitrope®, Sandoz). In particular, the study will assess the diabetogenic potential of rhGH and the risk of malignancies. The long-term efficacy is a secondary objective of the study.Methods: The study population includes infants, children and adolescents receiving Omnitrope®...

hrp0089p1-p037 | Bone, Growth Plate &amp; Mineral Metabolism P1 | ESPE2018

Hypercalcaemia after Treatment with Denosumab in Children: Bisphosphonates as an Option for Therapy and/or Prevention?

Sydlik Carmen , Weissenbacher Claudia , Roeb Julia , Roland Durr Hans , Bechtold-Dalla Pozza Susanne , Schmidt Heinrich

Background: Pharmacologic options for treatment of osteolytic diseases especially in children are limited. Although not licensed for use, denosumab, a fully humanized antibody to RANKL, is used in children and shows good effects. Among others, one indication are giant cell tumors of the bone. Yet, there are reports of severe hypercalcemia after stop of denosumab, an adverse effect which is rarely seen in adults.Case reports: Four patients, aged 6, 13 and...

hrp0089p1-p153 | GH &amp; IGFs P1 | ESPE2018

Testing the Performance of a Preexisting Growth Prediction Model in a Cohort of Prepubertal Patients Born Small for Gestational Age (SGA) Receiving GH Treatment in PATRO Children

Land Christof , Pfaffle Roland , Schwab Karl Otfried , Sommer Heide , Partsch Carl Joachim

Aim and background: Growth hormone (GH) treatment of short children born SGA and its effects on growth varies greatly between treated individuals. In the present study we tested the performance of a preexisting growth prediction model (Ranke et al.; JCE&M 2003 pp. 125–131) to estimate 1st-year height velocity (HV) in a german subcohort of prepubertal children born SGA treated with GH (Omnitrope).Methods: 190 treatment-naïve prepu...

hrp0089p2-p398 | Thyroid P2 | ESPE2018

Diagnosis and Clinical Course of Amiodarone Induced Hyperthyroidism in Three Adolescent Patients

Gesing Julia , Hoppmann Julia , Gebauer Roman , Pfaffle Roland , Bertsche Astrid , Kiess Wieland

Amiodarone induced hyperthyroidism is a known side effect of amiodarone treatment. In the pediatric population long-term amiodarone treatment is rarely indicated because of its severe side effects including thyroid function impairment and therefore treatment is restricted to therapy resistant arrhythmias. In the literature scarce data is available on management and therapy of amiodarone induced thyroid dysfunction at a young age. We present three adolescent patients developin...