hrp0094p1-174 | Growth Hormone and IGFs B | ESPE2021

High incidence and male predominance of transient form of isolated growth hormone (GH) deficiency in children. What is the optimal time for GH therapy withdrawal and retesting?

Smyczyńska Joanna , Hilczer Maciej , Lewiński Andrzej , Smyczyńska Urszula , Stawerska Renata ,

Introduction: Normal growth hormone (GH) secretion after the attainment of final height (FH) is observed in the majority of GH-treated children with GH deficiency (GHD), however it is unclear when such normalization of GH secretion occurs.The Aim: Of the study was to assess the incidence of transient GHD in retesting with respect to pediatric criteria in the patients with isolated GHD diagnosed in childhood, depending on the moment of GH...

hrp0097rfc11.5 | GH and IGFs | ESPE2023

Influence of sirtuin 1 and fibroblast growth factor 21 levels on IGF-1 concentration in children with short stature of different etiology

Fedorczak Anna , Stawerska Renata , Lewiński Andrzej

Introduction: Short stature is one of the most common reasons for children presenting to an endocrinologist. In normal conditions, growth hormone (GH) stimulates the IGF-1 production in hepatocytes via the STAT5 signaling pathway. Approximately 40% of children diagnosed with idiopathic short stature (ISS), i.e. with normal GH secretion, have a reduced IGF-1 level for unknown reasons. Recently, it has been described that there are certain factors that reduce IG...

hrp0097p1-329 | Growth and Syndromes | ESPE2023

Long-term (9-year, 8-year and 3.5-year) follow-up of treatment with rhGH in three patients with Noonan syndrome due to PTPN11 mutation and confirmed growth hormone deficiency.

Kowalik Dorota , Stawerska Renata , Lewiński Andrzej

Introduction: Noonan syndrome (NS) is relatively common genetic disorder caused by mutations in the PTPN11 (50%), SOS1 (10-13%), and RAF1 (3-17%) genes responsible for disturbances in the activation of the RAS/MAPK signaling pathway. NS is characterized by facial dysmorphic features (90%), congenital cardiac disturbances and short stature (<3c) - the average final adult height is 162.5 cm for male and 153 cm for female. In some, but not all of the NS patien...

hrp0092p1-77 | GH and IGFs | ESPE2019

Severe IGF-I Deficiency in Children with Normal Growth Hormone (GH) Secretion and Excluded GH Insensitivity – is it Really Idiopathic Short Stature?

Joanna Smyczynska , Urszula Smyczynska , Andrzej Lewinski , Maciej Hilczer

According to current recommendations, children with height SDS <-3.0, normal growth hormone (GH) peak in stimulation tests (stimGH) and severe IGF-I deficiency (IGFD) may be diagnosed with primary IGFD and treated with recombinant IGF-I. The need for direct confirmation of GH insensitivity is a matter of discussion. On the other hand, children born small for gestational age (SGA) with no catch-up growth are qualified to GH therapy despite normal GH secretion. The fact that...

hrp0089p2-p235 | GH &amp; IGFs P2 | ESPE2018

Hormonal Predictors of Growth Hormone Therapy Effectiveness in Children with Short Stature – Evidence from Neural Prediction Model for Final Height

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

Prediction of growth hormone (GH) therapy effectiveness in children with short stature is an important issue for optimizing its course. Recently, our research group has published prediction models derived with neural networks. The main predictors of final height (FH) in our model were: patientÂ’s height SDS at therapy onset, pre-treatment change of height SDS (HSDS V0) and pre-treatment IGF-I and IGFBP-3 secretion but not the results of GH stimulation tests; the...

hrp0089p2-p237 | GH &amp; IGFs P2 | ESPE2018

Predictors of Poor Response to Growth Hormone Therapy in Children with Short Stature – Evidence from Neural Prediction Model for Final Height

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

Prediction of poor response to growth hormone (GH) therapy in children with short stature is an important issue for personalized approach to treatment. Recently, our research group has published prediction models derived with neural networks. The main predictors of final height (FH) in our model were: patientÂ’s height SDS at therapy onset (H0SDS) and pre-treatment IGF-I and IGFBP-3 concentrations but not the results of GH stimulation tests; pre-treatment growth...

hrp0086p1-p626 | Growth P1 | ESPE2016

Significance of IGF-I Generation Test in Diagnosing Primary and Non-Primary IGF-I Deficiency – Clinical Considerations

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

Background: The diagnosis of severe primary IGF-I deficiency (IGFD) in children with normal growth hormone (GH) peak in stimulation tests (stimGH) should be confirmed by IGF-I increase <15.0 ng/ml during generation test (IGF-GT), however the significance of IGF-GT has been questioned by some researchers.Objective and hypotheses: Evaluation of the significance of IGF-GT in children with normal stim GH and IGFD, with respect to efficacy of growth-promo...

hrp0082p2-d2-429 | Growth Hormone (1) | ESPE2014

What Should be the Diagnosis and Management of Short Children with IGF1 Deficiency, Responding to GH Administration Despite Normal GH Secretion?

Smyczynska Joanna , Stawerska Renata , Lewinski Andrzej , Hilczer Maciej

Background: GH deficiency (GHD) is currently defined as secondary IGF1 deficiency (IGFD). In the patients with normal GH secretion and IGFD, significant increase of IGF1 during generation test excludes primary IGFD, however is not an approved indication for GH therapy.Objective and hypotheses: The aim of the study was to assess GH therapy effectiveness in children with IGFD, responding to short-term GH administration despite normal GH peak after falling ...

hrp0082p3-d2-834 | Growth (1) | ESPE2014

Effectiveness of GH Therapy in Children with Short Stature and Decreased GH Peak in Stimulating Tests is Independent from GH Secretion After Falling Asleep

Hilczer Maciej , Smyczynska Joanna , Stawerska Renata , Lewinski Andrzej

Background: GH deficiency (GHD) is routinely diagnosed on the basis of decreased GH peak in two stimulating tests (GHST). In Poland, few years ago, an assessment of nocturnal GH secretion after falling asleep (noctGH) has been introduced as a screening test in diagnosing GHD.Objective and hypotheses: The aim of the study was to assess GH therapy effectiveness in children with decreased GHST with respect to noctGH.Method: Retrospect...

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...