hrp0095rfc10.5 | GH and IGFs | ESPE2022

Evaluation of health-related quality of life & problem behavior in adults born small for gestational age treated with growth hormone during childhood in comparison with untreated controls

Dorrepaal Demi , Wesley Goedegebuure , der Steen Manouk van , Anita Hokken-Koelega

Background: Being born small for gestational age (SGA) has been associated with a reduction in health-related quality of life (HRQoL) and more problem behaviour. Growth hormone (GH) treatment, which leads to a significant improvement in adult height (AH), improves HRQoL and decreases problem behavior in SGA children. However, such results are very limited in SGA adults who were treated with GH during childhood.Objective:...

hrp0092rfc8.6 | Pituitary, Neuroendocrinology and Puberty Session 1 | ESPE2019

Growth, Pubertal Course and Long-Term Outcome of 46,XY Boys Born with Atypical Genitalia and Low Birthweight

Tack Lloyd , Straaten Saskia van der , Cools Martine , consortium On behalf of the I-DSD

Introduction: Boys born small for gestational age (SGA) often have undermasculinized genitalia. Little is known about the pubertal development and gonadal function on a longer-term in this specific group of males.Aims: To determine the (pubertal) development and long-term urological and endocrine outcome of undermasculinized boys born SGA compared to undervirilized boys born appropriate for gestational age (AGA).<p c...

hrp0092rfc12.1 | Growth and Syndromes (to include Turner syndrome) | ESPE2019

Karyotyping of oocytes, granulosa cells and stromal cells in the ovarian tissue from patients with Turner syndrome: a pilot study

Peek Ron , Schleedoorn Myra , Beerendonk Catharina , van der Velden Janielle , Braat Didi , Smeets Dominique , Fleischer Kathrin

Summary Answer: Most primordial/primary oocytes were found to have a 46,XX karyotype. Chromosome patterns of the ovarian cells were different from that observed in other tissues.Background: TS is a chromosomal condition associated with partial or complete absence of one of the two X-chromosomes. Females with TS have a limited reproductive lifespan due to an accelerated loss of germ cells. It has been hypothesized that vi...

hrp0089p2-p035 | Adrenals and HPA Axis P2 | ESPE2018

Pseudopubertas Praecox in a 4 Year Old Boy with Bilateral Atypical Adrenocortical Adenomas

Brichta Corinna , Wurm Michael , Hodde Franka , van der Werf-Grohmann Natascha , Schwab Karl Otfried

Introduction: Adrenocortical tumors are very rare in children, with a prevalence of just 0.3 cases/million/year. Autonomic hormone production by adrenal cortical tumors may cause peripheral precocious puberty.Case report: A 4-year-old boy was presented by his parents because of pubertal behavior with aggressive features and a significant increase in the size of the penis. The parents also noticed a strong growth spurt and sweat odor. The boy had a good g...

hrp0089p2-p225 | GH &amp; IGFs P2 | ESPE2018

Health-Related Quality of Life and Psychosocial Functioning in Young Adults Born SGA after GH/GnRHa Treatment

Goedegebuure Wesley , van der Steen Manouk , de With Justine , Hokken-Koelega Anita

Background: Being born small for gestational age (SGA) has a negative effect on health-related quality of life (HRQoL) and self-perception. This might be more negatively influenced by postponement of puberty using additional gonadotropin-releasing hormone analogue (GnRHa) treatment.Methods: 154 adolescents born SGA participating in a large Dutch growth hormone (GH) trial (75 with 2 years of GnRHa-treatment) completed the TNO-AZL Adults Quality of Life qu...

hrp0086fc8.5 | Growth: Clinical | ESPE2016

Longitudinal Study on Body Composition, Insulin Sensitivity and β-cell Function in SGA Adults from Stop of Long-term GH Treatment until 5 Years after Stop

van der Steen Manouk , Kerkhof Gerthe F. , Hokken-Koelega Anita C.S.

Background: GH treatment results in a decrease in fat mass (FM) and insulin sensitivity (Si), and an increase in lean body mass (LBM). Only limited data are available on the longitudinal changes after discontinuation of GH treatment in SGA adults, aged 21 years.Objective and hypotheses: To assess longitudinal changes in body composition (BC) and glucose homeostasis after stop of GH treatment in SGA adults.Method: 197 previously GH-...

hrp0086p1-p3 | Adrenal P1 | ESPE2016

Gender-Specific Differences in Hypothalamus–Pituitary–Adrenal Axis Activity in Children: A Meta-Analysis

van der Voorn Bibian , Hollanders Jonneke , Ket Johannes , Rotteveel Joost , Finken Martijn

Background: Differences in hypothalamus–pituitary–adrenal (HPA-)axis functioning have been proposed to underlie gender-specific cardiovascular and neurocognitive disease susceptibility.Objective and hypotheses: We conducted a systematic review and meta-analysis to test the hypothesis that gender-specific differences in HPA-axis activity are already present in childhood.Method: We searched two electronic databases (PubMed ...

hrp0086p1-p565 | Perinatal Endocrinology P1 | ESPE2016

Different Long-term Neurodevelopmental Outcomes in Very Preterm Versus Very-low-birth-weight Infants

Hollanders Jonneke J , Schaefer Nina , van der Pal Sylvia M , Rotteveel Joost , Finken Martijn J J

Background: Birth weight (BW) is often used as a proxy for gestational age (GA) by studies on preterm birth. Recent data indicate that the terms very-low-birth-weight (VLBW; BW <1500 g) and very preterm (VP; GA <32 weeks) birth are not equivalent with regard to perinatal outcomes and postnatal growth up until final height. It is unknown whether the differences between these terms could be extended to long-term neurodevelopmental outcomes.Objectiv...

hrp0082p1-d1-144 | Growth | ESPE2014

Genetics of Growth Failure in Small for Gestational Age Children

Keppler Romy , van der Werft-Grohmann Natascha , Schwab Karl Otfried , Lausch Ekkehart

Background: Small for gestational age (SGA), defined as ≤−2.0 SDS birth length or weight, is a condition seen in up to 3% of all newborn. Most SGA children catch up height in postnatal life. In a significant number (~10%), however, height remains below the third centile. Recombinant GH therapy is indicated when growth failure continues to 4 years of age. The pathophysiological basis of SGA is complex: monogenic disorders and/or fetal programming by environmental fa...

hrp0082p2-d1-418 | Growth Hormone | ESPE2014

Response to GH Treatment in Patients with Silver Russell Syndrome

Smeets C C J , Renes J S , van der Steen M , Hokken-Koelega A C S

Background: Silver Russell syndrome (SRS) is characterized by low birth weight, severe postnatal short stature and distinctive facies. In ~50% of patients, (epi)genetic alterations can be detected (~40% hypomethylation of H19 on chromosome 11, ~10% maternal uniparental disomy (UPD) 7). As SRS patients are usually born small for gestational age (SGA), they are treated with GH to improve height. However, data on long-term effects of GH treatment in SRS patients are very limited....