hrp0082fc1.6 | Adrenal | ESPE2014

Molecular Characterization of Testicular Adrenal Rest Tumours in Congenital Adrenal Hyperplasia; Lesions with both Adrenocortical and Leydig Cell Features

Smeets Evelien , Span Paul , van Herwaarden Antonius , Wevers Ron , Sweep Fred , Claahsen-van der Grinten Hedi

Background: Characterization of testicular adrenal rest tumours (TART) are one of the major long-term complications in congenital adrenal hyperplasia (CAH) patients. Although several adrenal-like properties have been assigned to these benign lesions, the exact etiology has not been established yet.Objective and Hypotheses: The aim of this study was to analyze several (steroidogenic) characteristics of TART tissue which may be classified as adrenal cortex...

hrp0082p1-d2-253 | Thyroid (1) | ESPE2014

Maternal Hypothyroxinemia in Early Pregnancy is Associated with Poorer Arithmetic Performance in a School Test in Offspring at Age 5 Years

Noten Anna , Loomans Eva , Vrijkotte Tanja , van Trotsenburg Paul , van Eijsden Manon , Rotteveel Joost , Finken Martijn

Background: Subtle impairments in the thyroid function of pregnant women are associated with poorer scores on mental developmental scales in their children at age 2–3 years, and with reduced performance in a simple reaction time test at 5–6 years. However, associations with school performance estimates have never been studied.Objective and hypotheses: We aimed to assess the effect of normal variation in the maternal thyroid function during earl...

hrp0082p2-d1-267 | Adrenals & HP Axis | ESPE2014

An adolescent with Hypertension Caused by Primary Hyperaldosteronism due to KCNJ5 Mutation

van der Linde Annelieke , Deinum Jaap , Konijnenberg Yvette , Keijzer-Veen Mandy , Claahsen-van der Grinten Hedi

Background: Primary aldosteronism (PA) is a rare form of secondary hypertension. In adults PA is often caused by unilateral adrenal adenoma which can be cured by unilateral adrenalectomy. However, in young patients hereditary causes of PA have to be considered with bilaterally affected adrenal glands.Objective and hypotheses: We report on an adolescent with PA due to a recently described KCNJ5 mutation and want to point out the importance of performing m...

hrp0084p2-192 | Adrenals | ESPE2015

Baseline Adrenal Function by Measurement of Long-term Cortisol Levels in Scalp Hair of Asthmatic Children Using Inhaled Corticosteroids Equals Healthy Controls

Smit Maria , van Leer Ed , Noppe Gerard , de Rijke Yolanda , Hoekx Jurriaan , van den Akker Erica

Background: Inhalation corticosteroids (ICS) as treatment for asthma can interfere with the hypothalamic-pituitary-adrenal axis and could lead to hypocortisolism. The frequency of this systemic side effect and the correlation to ICS usage is still unclear. Given that the cortisol excretion is pulsatile and determined by acute stress and circadian rhythms, the usefulness of tests based on momentary measures, such as saliva or serum are therefore limited. Cortisol levels measure...

hrp0084p3-705 | Diabetes | ESPE2015

Treatment of Dyslipidemia in Children and Adolescents with Diabetes Mellitus Type 1

Borgerink M M H , van Albada M A , Venema G , Bakker-van Waarde W M

Background: Cardiovascular disease (CVD) is the major cause of mortality in diabetes mellitus type 1 (T1DM). Dyslipidemia will increase this risk. Several guidelines have been published, how to treat dyslipidemia in T1DM, yet some studies have shown that the number of patients who are treated according to these guidelines is low.Objective and hypotheses: To investigate the frequency of dyslipidemia in children and adolescents with T1DM and if they are tr...

hrp0094p2-448 | Thyroid | ESPE2021

Methimazole-induced remission rates in pediatric graves’ disease: A systematic review

van Lieshout Jelmer , Mooij Christiaan , van Trotsenburg Paul , Zwaveling-Soonawala Nitash

Objective: Comparison of studies on remission rates in pediatric Graves’ disease is complicated by lack of uniformity in treatment protocols, remission definition, and follow-up duration. We performed a systematic review on remission rates in pediatric Graves’ disease and attempted to create uniformity by recalculating remission rates based on an intention-to-treat analysis. Results: Of 1,890 articles, 29 articles consist...

hrp0094p2-21 | Adrenals and HPA Axis | ESPE2021

The reliability of Salivary Cortisol compared to Serum Cortisol for diagnosing adrenal insufficiency in the gold standard ACTH stimulation test in children

Ciancia Silvia , van den Berg Sjoerd A.A. , van den Akker Erica L.T. ,

Introduction: The gold standard for the diagnosis of adrenal insufficiency (AI) is the ACTH stimulation test. According to the guidelines approved by the European Endocrine Society, the ACTH stimulation test should be performed with the administration of the standard dose of i.v. Synachten® 250 µg for adults and children aged ≥ 2 years (infants should receive 15 µg/kg, children ≤ 2 years 125 µg). In many clinical settings a Syna...

hrp0097rfc13.5 | Pituitary, neuroendocrinology and puberty 2 | ESPE2023

Growth hormone and TSH deficiency after [177Lu]Lu-DOTATATE therapy for pediatric neuroblastoma; description of a first case.

van Wessel Karen , de Keizer Bart , Dierselhuis Miranda , van Santen Hanneke

Background: Neuroblastoma (NBL) is the most common extra cranial solid tumor in children. Endocrine adverse effects after treatment for NBL have been reported of which mainly caused by treatment with 131meta-iodobenzylguanidine ([131I]MIBG) or with alkylating agents and may consist of thyroid disorders, gonadal insufficiency or short stature. Due to recent developments patients are increasingly treated with [177Lu]Lu-DOTATATE peptide receptor radionuclide ther...

hrp0097p1-318 | Growth and Syndromes | ESPE2023

15-year experience with the IGF1 generation test in the Netherlands

Kruijsen Anne , de Groote Kirsten , Punt Lauren , van Trotsenburg Paul , Pijnenburg-Kleizen Karijn , Bocco Gianni , Berkenbosch Lizanne , van Setten Petra , Claahsen - van der Grinten Hedi , van der Kaay Danielle , Schott Nina , van Tellingen Vera , van Mill Edgar , van der Heyden Josine , Brandsma Annelies , Hendriks Yvonne , Losekoot Monique , van Duyvenvoorde Hermine , Hokken-Koelega Anita , Renes Judith , Maarten Wit Jan , de Bruin Christiaan , Joustra Sjoerd

Introduction: Among children with short stature, some show persistent IGF-I levels <-2.0 SDS despite a normal growth hormone (GH) response in a stimulation test. This may be caused by conditions that could benefit from recombinant human GH (rhGH) therapy (e.g. GH neurosecretory dysfunction, bioinactive GH, partial GH insensitivity). Therefore, the IGF-I generation test (IGFIGT) was implemented in 2006 using a national, standardized protocol. Children with a...

hrp0097p1-356 | Pituitary, Neuroendocrinology and Puberty | ESPE2023

Acquired Hypothalamic dysfunction in childhood: “What do patients need?“ – an Endo-ERN survey

van Roessel I.M.A.A. , de Graaf J.P. , Biermasz N.R. , Charmandari E. , van Santen H.M.

Objective: Hypothalamic dysfunction is a rare condition and can be encountered in patients who have been diagnosed or treated for a suprasellar brain tumor. Due to its rarity, signs and symptoms of hypothalamic dysfunction may be difficult to recognize, leading to delayed diagnosis of the suprasellar brain tumor or to difficulties in finding the health care expertise for hypothalamic dysfunction after tumor treatment. To improve care and outcome of patients wi...