hrp0097p1-324 | Growth and Syndromes | ESPE2023

PROGRES, a multi-country, non-interventional, prospective study of patients receiving human growth hormone treatment under routine clinical care: Study update

Geffner Mitchell , Maniatis Aristides , Ibañez Lourdes , La Torre Daria , Huang Carol , Darendeliler Feyza , Dattani Mehul , Maghnie Mohamad , Phillip Moshe , Horikawa Reiko , Gomez Roy , Viswanathan Shilpa , Carlsson Martin , Wajnrajch Michael

Objectives: Children with growth hormone deficiency (GHD) are usually treated with once-daily injections of recombinant human growth hormone (rhGH). Somatrogon is a long-acting rhGH (LAGH) approved in the EU and other countries for once-weekly treatment of children with short stature. The Pfizer Registry of Outcomes in Growth hormone RESearch (PROGRES) study will assess the long-term safety and effectiveness of once-weekly somatrogon and once-daily rhGH prepar...

hrp0089p1-p001 | Adrenals and HPA Axis P1 | ESPE2018

Evaluation of Long Term Metabolic Effects After Prenatal Dexamethasone Treatment in the Context of CAH – the Swedish Cohort

Wallensteen Lena , Karlsson Leif , Messina Valeria , Nordenstrom Anna , Lajic Svetlana

Background: Prenatal dexamethasone (DEX) treatment is in many countries offered to the pregnant woman, at risk of having a child with classic congenital adrenal hyperplasia (CAH), to reduce virilization in an affected female fetus. The treatment is effective in reducing virilization but may give long lasting effects on somatic and cognitive health. Here, we explore the potential effect on metabolism in children and adults not having CAH and exposed to DEX during the first trim...

hrp0089p1-p003 | Adrenals and HPA Axis P1 | ESPE2018

Cognition in Children with Congenital Adrenal Hyperplasia

Messina Valeria , Karlsson Leif , Hirvikoski Tatja , Nordenstrom Anna , Lajic Svetlana

Background: Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency is one of the most common autosomal recessive disorders, affecting around 1/10 000 newborns worldwide. Postnatally, patients with classic CAH are treated with life-long glucocorticoid (GC) replacement therapy, such as hydrocortisone or prednisolone, and in the more severe cases also with mineralocorticoids. A negative impact of GCs on human cognition such as memory deficits have been reported bot...

hrp0086p1-p37 | Adrenal P1 | ESPE2016

DNA Methylation Signatures Associated with Prenatal Dexamethasone Treatment

Karlsson Leif , Barbaro Michela , Gomez-Cabrero David , Lajic Svetlana

Background: Prenatal treatment with dexamethasone (DEX) has been used since the mid 80’s to minimize virilisation of girls with congenital adrenal hyperplasia. Long-term data on treatment safety and health outcome are still limited. It has been shown in animal models that prenatal dexamethasone treatment affects DNA methylation signatures as well as metabolism and behavior. We have previously shown that DEX affects working memory in children who were treated with DEX duri...

hrp0082p3-d1-884 | Perinatal and Neonatal Endocrinology | ESPE2014

Gender Differences in Sex Steroids and IGF1 at Birth and at 5 Years of Age

Allvin Kerstin , Karlsson Ann-Katrine , Ankarberg-Lindgren Carina , Dahlgren Jovanna

Background: Gender differences in sex steroids and IGF1 are well known from pubertal years into adulthood. Few studies report data from pre-school years.Objective and hypotheses: To study gender specific changes in sex steroids and IGF1 at birth and at 5 years of age and correlate these with auxological measurements. There are gender differences in IGF1 levels due to differences in sex steroids already at birth and during pre-school years.<p class="a...

hrp0097t6 | Section | ESPE2023

Granulosa cell tumors in girls: Preliminary results of a meta-analysis of new and published cases

Kiss Eszter , Wessmann Sandra , W Carlson Joseph , Lundberg Elena , Stenmarker Margaretha , Bobeck Johan , Lodefalk Maria

Background: Granulosa cell tumors (GCT) originate from sex cord/stromal tissue in the gonad. They are typically located in an ovary, but extra-gonadal localisation exists. These tumors are extremely rare in children and no systematic review has been published. The objective of this systematic review is to examine the following questions: What is the clinical picture of girls with a GCT? How are these patients treated and what is their prognosis?<p class="a...

hrp0095rfc5.4 | Adrenals and HPA Axis | ESPE2022

Increased resting-state functional connectivity in the medial orbitofrontal cortex of patients with autoimmune Addison's disease

van't Westeinde Annelies , Padilla Nelly , Bensing Sophie , Lajic Svetlana

Background: Patients with autoimmune Addison’s disease (AAD) lack production of glucocorticoids (GCs), mineralocorticoids (MCs) and androgens from the adrenal gland, due to autoimmune destruction of its cortex. Patients require replacement of GCs and MCs for the rest of their lives. In some cases, testosterone is supplemented in females. Brain function is sensitive to fluctuations in cortisol and may therefore be affected in AAD due to long-term disturba...

hrp0089p1-p006 | Adrenals and HPA Axis P1 | ESPE2018

Altered DNA Methylation in Peripheral T-cells from Patients with Congenital Adrenal Hyperplasia

Karlsson Leif , Barbaro Michela , Ewing Ewoud , Gomez-Cabrero David , Lajic Svetlana

Background: Patients with Congenital Adrenal Hyperplasia (CAH) are at risk of several co-morbidities, such as impaired cognitive functions, short stature and adverse effects on metabolism. The causes of these effects are suboptimal glucocorticoid replacement therapy, adrenal crises and prenatal glucocorticoid exposure. However, there are no data available to this day how these factors are affecting epigenomic programs.Objective and hypot...

hrp0086p1-p18 | Adrenal P1 | ESPE2016

Adult Individuals with Classic Congenital Adrenal Hyperplasia Exhibit Deficits in Executive Functions

Karlsson Leif , Zimmermann Marius , Wallensteen Lena , Barbaro Michela , Nordenstrom Anna , Hirvikoski Tatja , Lajic Svetlana

Background: Individuals with classic congenital adrenal hyperplasia (CAH) are treated postnatally with glucocorticoids. Earlier research with animals and other disorders with excess GC exposure implicate that GCs can influence memory. Deficits in working memory can be seen already during childhood in children with classic CAH.Objective: We tested the hypothesis that adult individuals with classic CAH show impaired cognitive functions.<p class="abstex...

hrp0084p3-586 | Adrenals | ESPE2015

Longitudinal Changes During Prepubertal Years in Visceral Fat and Steroid Hormones – SGA vs AGA Children

Ankarberg-Lindgren Carina , Andersson Mats X , Karlsson Ann-Katrine , Dahlgren Jovanna

Background: Small for gestational age (SGA) children have reportedly increased DHEAS levels during prepubertal years. However, steroid hormones have not been followed longitudinally in a healthy population compared to body composition.Aims and objectives: To evaluate steroid hormone patterns in prepubertal children correlated to visceral fat measures.Methods: Body composition was investigated with magnetic resonance (MR) of truncal...