hrp0084p2-408 | GH & IGF | ESPE2015

Does Skeletal Disproportion in Children with Idiopathic Short Stature Influence Response to GH Therapy?

Cutfield Wayne S , Lindberg Anders , Hofman Paul , Derraik Jose , Geffner Mitchell E , Camacho-Hubner Cecilia

Background: Children with ISS have an array of causes that lead to short stature and/or poor growth velocity. Genetic causes of short stature, notably SHOX mutations, can be associated with subtle skeletal disproportion with shorter limbs, manifesting as increased sitting-to-standing height ratios or SDS.Objective and hypothesis: Children with ISS and skeletal disproportions have a diminished growth response to GH treatment compared to chi...

hrp0084p2-437 | Gonads | ESPE2015

Implementation of a High Sensitive LC-MS/MS Method for Measurement of Oestradiol, Oestrone and Oestriol

Kulle Alexandra , Reinehr Thomas , Simic-Schleicher Gunter , Roscheswsky Jule , Welzel Maik , Holterhus Paul-Martin

Background: Specific measurement of low serum/plasma concentrations of 17β-oestradiol (E2) is important in pre-pubertal and pubertal children in routine paediatric endocrinology. The role of oestrone (E1) and oestriol (E3) is not as comprehensively well understood in different pediatric endocrine disease states. We describe a method for high sensitivity analysis of estradiol (E2), oestrone (E1) and oestriol (E3) using LC-MS/MS.Objective and hypothes...

hrp0084p3-588 | Adrenals | ESPE2015

Case Report: Hypothyroidism and Acth-Deficiency Caused by TBX 19 Mutation Coincidence or Pathogenetic Correlation?

Schulz Esther , Akkurt Halit Ilker , Muhlhausen Chris , Santer Rene , Welzel Maik , Holterhus Paul Martin

Background: Congenital isolated ACTH-deficiency is a rare disorder characterized by low plasma ACTH and cortisol levels and normal secretion of other pituitary hormones. TBX19 is a t-box transcription factor with a specific role in the differentiation of corticotroph cells. TPIT gene mutations can be found in early onset isolated ACTH deficiency.Case report: We report on a 2; 6 year old girl, offspring from consanguineous parents from Turkey, born at 40 ...

hrp0084p3-832 | Fat | ESPE2015

The French Experience in Bariatric Surgery ‘Laparoscopic Adjustable Gastric Banding’ in Adolescence

Dabbas Myriam , Khen-Dunlop Naziha , De Filippo Jean Paul , Bougneres Pierre , Goulet Olivier , Revillon Yann

Background: Because the success rate of lifestyle interventions is modest in severely obese adolescents, surgical treatments are now proposed. Laparoscopic adjustable gastric banding (LAGB) represents an attractive treatment with minimal morbidity and reversible procedure.Method: All adolescents were included in prospective longitudinal assessment and entirely managed by a pediatric team. Surgery was proposed only after at least 1-year lifestyle interven...

hrp0094fc9.3 | Growth Hormone and IGFs | ESPE2021

Once-weekly somapacitan versus daily growth hormone in growth hormone deficiency: 3 year efficacy and safety results from REAL 3, a randomised controlled phase 2 trial

Savendahl Lars , Battelino Tadej , Rasmussen Michael Hojby , Saenger Paul , Horikawa Reiko ,

Growth hormone (GH) replacement therapy currently requires daily subcutaneous injections. Somapacitan is a long-acting GH-derivative in development for once-weekly use in children with GH deficiency (GHD). A phase 2, multinational, randomised, open-label, controlled trial (NCT02616562) investigated the efficacy and safety of somapacitan compared with daily GH (Norditropin®). GH-treatment-naïve prepubertal children with GHD received 0.04 (n = 16), 0.08 (n...

hrp0094p1-9 | Adrenal A | ESPE2021

Backdoor Pathway hormones and 11-oxygenated Androgens are elevated in Patients with 21-hydroxylase deficiency

Kulle Alexandra , Lamprecht Tabea , Pinto Francisca , Wulf Kristina , Hornig Nadine , Reinehr Thomas , Holterhus Paul-Martin ,

Background: Patients with 21-Hydroxylase deficiency (21OHD) present increased levels of cytochrome P450 21-hydroxylase substrates, like progesterone and 17α-hydroxyprogesterone. Previous studies could show that these hormones are involved in the production of androgens via the backdoor pathway. As a second source of androgens, 11-oxyandrogens derived from the human adrenal glands are recognized as being major androgens. Specifically, 11-oxyandrogens ...

hrp0094p1-91 | Thyroid A | ESPE2021

The effect of preoperative calcitriol prophylaxis on post-thyroidectomy hypocalcaemia in children

Vendrig Lisanne , Mooij Christiaan , Derikx Joep , Trotsenburg Paul van , Zwaveling-Soonawala Nitash ,

Background: Transient or permanent hypoparathyroidism is one of the most well-known complications of total thyroidectomy and may lead to symptomatic hypocalcaemia. Treatment of post-thyroidectomy hypocalcaemia usually consists of postoperative calcium and/or vitamin D supplementation. In 2013 we implemented prophylactic pre-thyroidectomy calcitriol supplementation for all children undergoing total thyroidectomy at Amsterdam UMC. The objective of this study was...

hrp0094p1-147 | Sex Endocrinology and Gonads B | ESPE2021

A pre-analytical challenge to determine estradiol in children: A monovette systematically causing increased estradiol-concentrations in LC-MS/MS analysis

Lamprecht Tabea , Kleber Michaela , Rothermel Juliane , Holterhus Paul-Martin , Kulle Alexandra ,

Introduction: In children, estradiol (E2) concentrations are 1000-times lower in comparison to their precursors, the androgens. Depending on sex, age, and pathology, plasma concentrations vary in a broad range. The sensitive and specific determination of E2 is a particular challenge in pediatric endocrinology. Here we report a female patient aged 12 6/12 years and Tanner stage B1 with a presumed diagnosis of ovarian insufficiency. She showed a very high E2 con...

hrp0094p2-446 | Thyroid | ESPE2021

The efficacy and short- and long-term side effects of radioactive iodine treatment in pediatric Graves’ disease: a systematic review

Lutterman Sarah , Zwaveling-Soonawala Nitash , Verberne Hein , Verburg Frederik , van Trotsenburg Paul , Mooij Christiaan

Background: Graves’s disease (GD) is the most common cause of hyperthyroidism. Maximal 30% of pediatric GD patients achieve remission with anti-thyroid drugs. The majority of patients therefore require definitive treatment. Both thyroidectomy and radioactive iodine (RAI) are often used as definitive treatment for GD. However, data on efficacy, and short- and long-term side effects of RAI treatment for pediatric GD are relatively scarce.Methods: A ...

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...