hrp0092fc9.4 | Fetal, Neonatal Endocrinology and Metabolism (to include Hypoglycaemia) | ESPE2019

Prenatal Environment and Genetic Background Influence Urinary Steroid Excretion in Monozygotic Twins with Intra-Twin Birth-Weight Differences

Schulte Sandra , Woelfle Joachim , Schreiner Felix , Kasner Charlotte , Gruenewald Mathias , Hartmann Michaela F. , Wudy Stefan A. , Bartmann Peter , Gohlke Bettina

Background: Low birth-weight and unfavourable intrauterine conditions are associated with a subsequent impact on the endocrine system. Many studies reported hyperandrogenaemia and precocious adrenarche in children born small for gestational age (SGA). However, little information is available on steroid profiles in these subjects.Objective and Hypotheses: We followed genetically identical twins with intra-twin birth-weigh...

hrp0086fc10.6 | Perinatal Endocrinology | ESPE2016

Developmental Programming of Somatic Growth, Behavior and the Endocannabinoid System (ECS) by Variation of Early Postnatal Nutrition in a Cross-Fostering Mouse Model

Schreiner Felix , Ackermann Merle , Michalik Michael , Rother Eva , Bilkei-Gorzo Andras , Racz Ildiko , Dotsch Jorg , Zimmer Andreas , Woelfle Joachim

Background: Nutrient deprivation during early development has been associated with the predisposition to metabolic disorders in numerous epidemiological studies. Experiments with rodents also indicate a developmental plasticity of neuropsychological characteristics following alterations of early postnatal nutrition.Objective: Considering its interaction with metabolism and regulation of appetite and behavior, the endocannabinoid system (ECS) may represen...

hrp0097p1-506 | Growth and Syndromes | ESPE2023

Characteristics, effectiveness and safety data for patients with growth failure treated with recombinant IGF-1 and achieving adult or near-adult height: results from the Increlex® Global Registry

Bang Peter , Ramón Krauel Marta , Maghnie Mohamad , Woelfle Joachim , Sert Caroline , Perrot Valérie , Pennestri Daniele , Polak Michel

Background: severe primary insulin-like growth factor-1 deficiency (SPIGFD) is a rare growth disorder. Recombinant human insulin-like growth factor (IGF-1) (rhIGF-1; Increlex® [mecasermin]) replacement therapy is EU and US-approved for treating growth failure due to SPIGFD. The long-term therapeutic objective of rhIGF-1 treatment in SPIGFD is to improve adult height (AH). Objective: to describe the characteristics, safety and effectiveness data ...

hrp0097p1-198 | Thyroid | ESPE2023

Graves’ disease in children with Down syndrome

Nurcan Cebeci Ayse , Schempp Vera , Reinauer Christina , Gohlke Bettina , Marx Michaela , Dörr Helmuth-Günther , Woelfle Joachim

Introduction: Thyroid abnormalities such as subclinical or overt hypothyroidism are common in children with Down syndrome (DS) with ranges from 4% to 19.5%, whereas Graves´ disease (GD) is extremely rare (ranges 0.6%-0.9 %).Aims: To evaluate clinical features, course, and treatment of GD in children with DS.Patients and Methods: Among 161 children with GD, diagnosed between 1...

hrp0095fc5.5 | Adrenals and HPA Axis | ESPE2022

Influence of prenatal environment and genetic background on glucocorticoid steroid metabolism in monozygotic twins with intra-twin birthweight-differences

Schulte Sandra , Schreiner Felix , Plamper Michaela , Kasner Charlotte , Gruenewald Mathias , Bartmann Bartmann Peter , Fimmers Rolf , Hartmann Michaela F. , Wudy Stefan A. , Woelfle Joachim , Gohlke Bettina

Preliminary results:Objective: Although low birthweight (bw) and unfavourable intrauterine conditions have been associated with metabolic sequelae in later life, little is known about their impact on steroid metabolism. We studied genetically identical twins with intra-twin bw-differences from birth to adolescence to analyse the long-term impact of bw on steroid metabolism.Methods:...

hrp0089p2-p004 | Adrenals and HPA Axis P2 | ESPE2018

Hydrocortisone (HC) Dose in Children with Congenital Adrenal Hyperplasia (CAH)

Hoyer-Kuhn Heike , Huebner Angela , Richter-Unruh Annette , Oeverink Rudolf , Bettendorf Markus , Rohrer Tilman , Kapelari Klaus , Roehl Friedrich-Wilhelm , Holl Reinhard , Woelfle Joachim , on behalf of the German CAH registry

Background: Recommendations for initial and maintenance dose of HC in CAH differ widely. However, treatment of CAH in young children is complicated by the lack of a suitable low-dose HC preparations.Methods: The German Society for Paediatric Endocrinology and Diabetology (DGKED) initiated a registry for patients with classical CAH (German CAH registry). Anonymized data are transferred for central analysis, including a validation step and a benchmarking r...

hrp0097rfc6.4 | Pituitary, neuroendocrinology and puberty 1 | ESPE2023

Earlier Occurrence of Puberty and Pubertal Hair Development in Boys and Girls - Insights from the DPV Initiative Data

Reschke Felix , Gohlke Bettina , Lanzinger Stefanie , Boettcher Claudia , Gemulla Gita , Thiele-Schmitz Susanne , Dunstheimer Desiree , van den Boom Louise , Joachim Woelfle , Reinhard Holl

Introduction: Pubertal onset is signaled by thelarche (in girls) and gonadarche (in boys) and indicates the beginning of the hypothalamic-pituitary-gonadal axis activity. Thus, the onset of pubic hair development usually coincides with adrenarche. Girls worldwide are experiencing earlier puberty, but it is uncertain if this trend affects boys, or if it applies to children with type 1 diabetes (T1D).Aim: The aim of this s...

hrp0097p1-388 | Thyroid | ESPE2023

Neutropenia in paediatric Graves disease patients occurs more often under Carbimazole than Methimazole

Schempp Vera , Nurcan Cebeci Ayse , Reinauer Christina , Woelfle Joachim , Dörr Helmuth-Günther , Roosen Marie-Thérèse , Gohlke Jonas , Gohlke Bettina

Introduction: Neutropenia is known as a rare adverse event of anti-thyroid drug treatment (ATD) but has also been reported as pre-treatment neutropenia in Graves’ disease (GD). Studies on paediatric patients are rare. To the best of our knowledge, there are no data comparing the effects of methimazole (MMI) and carbimazole (CBZ) treatment in children on the absolute neutrophil count (ANC).Methods: We analyzed retro...

hrp0092p3-258 | Thyroid | ESPE2019

The Challenge to Treat Neonatal Autoimmune Hyperthyroidism in a Small Preterm

Lignitz Sarah , Coors Detlef , Pohlenz Joachim

Background: The prevalence of hyperthyroidism in pregnancy is about 0.2%, mostly due to Graves disease. Neonatal autoimmune hyperthyroidism caused by the transplacental passage of stimulatory thyrotropin receptor antibodies (TRAB) of the IgG class is a rare disorder. It occurs in only 2% of the neonates of mothers with Graves disease, is transient and associated with high morbidity and mortality rates up to 25%. Antithyroid drugs are the treatment ...

hrp0095p1-24 | Bone, Growth Plate and Mineral Metabolism | ESPE2022

SIADH as presenting feature of foramen magnum stenosis in a 2-month-old infant with achondroplasia (ACH): case report and discussion on management of infants with ACH in order to reduce the risk of serious complications

Nurcan Cebeci Ayse , Hebert Steven , Reutter Heiko , Wölfle Joachim

Background and Aim: Achondroplasia is the most common skeletal dysplasia and is associated with serious complications such as foramen magnum stenosis (FMS). Current guidelines in Germany recommend polysomnography at 2-3 months and cranial imaging at 6-9 months of life in achondroplasia, whereas American Academy of Pediatrics recommends to evaluate every infant with achondroplasia for cranio-cervical junction risks via neurologic examination, polysomnography an...