hrp0086p1-p633 | Growth P1 | ESPE2016

Evaluation of Prepubertal Patients with Suspected Neurosecretory Dysfunction of Growth Hormone Secretion: Diagnostic Steps and Treatment Response

Sydlik Carmen , Weiszenbacher Claudia , Pozza Susanne Bechtold-Dalla , Schmidt Heinrich

Background and aims: Existence and diagnostic procedures of neurosecretory dysfunction (NSD) are still a matter of debate. The aim of the study was to analyse prediagnostic data of short-statured children with pathologic and normal spontaneous GH-secretion and to evaluate the effect of GH-therapy in NSD-patients.Methods: Of 90 children aged 3–16 years, in whom 12-hour night profiles for GH-secretion were performed (unicentric), in 49 NSD was diagnos...

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...

hrp0095p2-228 | Pituitary, Neuroendocrinology and Puberty | ESPE2022

Infection with SARS-CoV-2 may alter the half-life of desmopressin (DDAVP) in children with central diabetes insipidus

Dubinski Ilja , Bechtold-Dalla Pozza Susanne , Schmidt Heinrich

Background: Central diabetes insipidus (CDI) is characterised by a central deficiency of arginine vasopressin (AVP) with polyuria and polydipsia. The etiology is heterogeneous. The treatment of choice is the oral or nasal application of DDAVP (synthetic analogue of AVP). CDI in the context of coronavirus disease 2019 (COVID19) has been reported in an individual case.Case report: We present a 9-year old male with CDI of u...

hrp0089p1-p037 | Bone, Growth Plate & 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...

hrp0086p1-p141 | Bone & Mineral Metabolism P1 | ESPE2016

Off-Label Use of the Aromatase Inhibitor Letrozole in Pubertal Boys to Improve Final Height: Laboratory, Auxological and Bone Age Data

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

Background and aims: The aromatase inhibitor letrozole is used to improve final height in boys with predicted short stature by delaying bone age (BA) maturation due to suppression of estradiol levels. There are few data regarding its effects, especially when used as single medication.Methods: Ten pubertal boys with predicted low final height treated with letrozole 2.5 mg/d p.o. (no other medication) for up to 24 months were analysed for auxological, labo...

hrp0084p2-227 | Bone | ESPE2015

Longitudinal Bone Development in Patients with Classical Congenital Adrenal Hyperplasia: Data Using Peripheral Quantitative Computed Tomography

Bechtold Susanne , Duman Leyla , Weissenbacher Claudia , Roeb Julia , Pozza Robert Dalla , Schmidt Heinrich

Background and aims: Glucocorticoid treatment may influence bone and muscle development in patients with congenital adrenal hyperplasia (CAH). This study evaluated bone mineral density (BMD), bone geometry and muscle mass longitudinally throughout childhood.Methods: 18 patients (ten males, eight females) with classical CAH were included. BMD, bone geometry and muscle mass were measured using peripheral quantitative computed tomography (pQCT) in prepubert...

hrp0084p2-453 | Growth | ESPE2015

Cross-Sectional and Longitudinal Follow-Up of Changes in Glucose Metabolism in Prepubertal GH-Treated SGA-Patients: Results of an Unicentric Study

Sydlik Carmen , Bechtold Susanne , Weissenbacher Claudia , Roeb Julia , Buckl Matthias , Putzker Stefanie , Schmidt Heinrich

Background: Several studies show impaired glucose tolerance at a certain number of years after start of growth hormone in SGA-children.Objective and hypotheses: To perform a longitudinal and cross-sectional evaluation of the oGTTs (glucose-insulin-pairs) in prepubertal GH-treated SGA-children and to evaluate if ISI and HOMA are effective surrogates for glucose tolerance and of prognostic value (0–1 year data).Method: In 81 pre...

hrp0084p3-660 | Bone | ESPE2015

4 Years Follow-Up for 25OHD and iPTH in Vitamin D Substituted Patients with Diabetes Mellitus 1: An Unicentric Prospective Study

Weissenbacher Claudia , Roeb Julia , Sydlik Carmen , Pozza Susanne Bechtold Dalla , Marx Michael , Schmidt Heinrich

Background: Vitamin D deficiency/insufficiency seems to occur frequently in children and teenagers but it is a matter of debate if limits (<20 ng/ml; <30 ng/ml) are correct. Besides its effect in bone metabolism Vit-D is also supposed to have a positive influence in diabetes mellitus 1 (DM1).Objective and hypotheses: To study 25OHD and iPTH values in a group of patients (pts) (n=57) with DM1 without Vit-D substitution (2011), with 1000 I...

hrp0084p3-741 | Diabetes | ESPE2015

Low fT3 Syndrome due to Metabolic Acidosis/Ketoacidosis in Type 1 Diabetes Mellitus (Type 1 DM)

Demikhova Irina , Pozza Susanne Bechtold Dalla , Weissenbacher Claudia , Sydlik Carmen , Roeb Julia , Schmidt Heinrich

Background: Type 1 DM is an autoimmune disease, characterized by destruction of the insulin-producing beta-cells in the islets of Langerhans. The absolute insulin deficiency leads to metabolic imbalance with hyperglycaemia, acidosis and proneness to ketosis. This acute disturbance can change thyroid hormone metabolism.Objective and hypotheses: To examine the influence of metabolic acidosis/ketoacidosis in type 1 DM on thyroid hormone levels.<p class=...

hrp0094fc1.1 | Adrenal | ESPE2021

Salivary profiles of 11 oxygenated androgens follow a diurnal rhythm in patients with congenital adrenal hyperplasia

Nowotny Hanna F. , Auer Matthias K. , Lottspeich Christian , Schmidt Heinrich , Dubinski Ilja , Bidlingmaier Martin , Adaway Jo , Hawley James , Keevil Brian , Reisch Nicole ,

Background: Routine biochemical assessment in patients with congenital adrenal hyperplasia (CAH) includes measurement of serum 17 hydroxyprogesterone (17OHP), androstenedione (A4) and testosterone (T) and their metabolites in urine. Several studies have also described 11 oxygenated 19 carbon (11oxC19) steroids as a clinically relevant androgenetic source and highlighted their potential as markers for evaluation of adrenal androgen excess in patients with 21-hy...