hrp0095p2-30 | Bone, Growth Plate and Mineral Metabolism | ESPE2022

Long QT revealing hypoparathyroidism in a B thalassemic major

Khensal Sabrina , Bouhelassa Amina , Benmohammed Karima , Nouri Nassim

Introduction: Beta thalassemia major (BTM), a hereditary haemoglobinopathy due to a deficiency in the synthesis of beta-globin, leads in the homozygous state to severe anemia requiring regular transfusions associated with chelator treatment. The endocrine complications encountered in BTM, secondary to martial overload by direct damage to the glandular parenchyma, continue to be observed in our consultations, although early and well-managed chelation can reduce...

hrp0097fc10.1 | Fetal, neonatal endocrinology and metabolism (to include hypoglycaemia) & Multisystem endocrine disorders | ESPE2023

Dasiglucagon safety in paediatric participants with CHI

Meissner Thomas , D. De León Diva , Thornton Paul , Zangen David , Mohnike Klaus , Andersen Marie , Bøge Eva , Birch Sune , Ivkovic Jelena , Banerjee Indi

Background: Congenital hyperinsulinism (CHI) is a rare disorder, which causes persistent and severe hypoglycaemia in infants and children. CHI can be treated with glucagon, but long-term use is challenging owing to its instability in aqueous solution. Dasiglucagon, a stable glucagon analogue designed for long-term use as a subcutaneous continuous infusion, is in clinical development. Here, we present dasiglucagon safety results in participants treated for up t...

hrp0098fc6.5 | Fat, Metabolism and Obesity 1 | ESPE2024

First cross sectional analysis of the eclip registry

von Schnurbein Julia , Nagel Gabriele , Akinci Baris , Broekma Marjoleine , Csajbok Eva , Rosaria D'Apice Maria , Gambineri Alessandra , Heldt Kathrin , Kleinendorst Lotte , Lattanzi Giovanna , Miehle Konstanze , Prodami Flavia , Santini Ferruccio , Santos Silva Ermelinda , B Savage David , Scherer Thomas , Sbraccia Paolo , Sorkina Ekaterina , Štotl Iztok , Vantyghem Marie-Christine , Vigouroux Corinne , Vorona Elena , Araújo-Vilar David , Wabitsch Martin , Vatier Camille , Ceccarini Giovanni

Background: Lipodystrophy syndromes comprise a group of extremely rare and heterogeneous diseases characterized by a selective loss of adipose tissue in the absence of nutritional deprivation or catabolic state. Because of the rarity of each lipodystrophy sub-form, research in this area requires international co-operation. Therefore, in 2016, the European Consortium of Lipodystrophies (ECLip) decided to create a registry for patients affected by lipodystrophy....

hrp0095p1-151 | Pituitary, Neuroendocrinology and Puberty | ESPE2022

The effect of a GnRH analogue injection on the circulating levels of kisspeptin-1 in girls with suspected central precocious puberty

Rodanaki Maria , Rask Eva , Lodefalk Maria

Introduction: Kisspeptin stimulates the gonadotropin releasing hormone (GnRH) neurons in hypothalamus initiating puberty. However, it is not known whether GnRH inhibits kisspeptin secretion by negative feedback and whether there are any associations between circulating levels of kisspeptin and other hormones, like ghrelin, important for the onset of puberty.Methods: Thirteen girls with suspected central precocious pubert...

hrp0089p2-p311 | Pituitary, Neuroendocrinology and Puberty P2 | ESPE2018

Incidence of Delayed Puberty in Adolescents. A Population-Based Study in a County in Central Sweden

Rodanaki Maria , Rask Eva , Lodefalk Maria

Introduction: Delayed puberty is defined as the absence of physical signs of puberty by the age of 14 years in boys and 13 years in girls. According to this definition, the prevalence of delayed puberty would be 2%, if the ages of pubertal onset were normally distributed in the population. However, the prevalence or incidence of delayed puberty has not been described before, as far as we know. Our aim was to study the incidence of delayed puberty in central Sweden.<p class...

hrp0094p1-86 | Pituitary A | ESPE2021

Adding a protease inhibitor to sampling tubes increases the acylated ghrelin and decreases the desacylated ghrelin levels in girls.

Rodanaki Maria , Rask Eva , Lodefalk Maria ,

Introduction: Ghrelin is a growth hormone-releasing acylated peptide stimulating the appetite, mainly produced in the stomach, and with an important role in pubertal development (1). Two ghrelin forms have been described, acylated (AG) and desacylated (DAG), but it is debated whether DAG is an active hormone or a degradation product of AG (2). Our aim was to evaluate the effects of adding the protease inhibitor 4-(2-aminoethyl) benzenesufonyl fluoride hydrochl...

hrp0092p1-258 | Pituitary, Neuroendocrinology and Puberty (1) | ESPE2019

Novel Pubertal References for Girls Using Ultrasound to Stage Breast Development. The Bergen Growth Study 2

Bruserud Ingvid S. , Roelants Mathieu , Oehme Ninnie HB , Madsen André , Eide Geir Egil , Rosendahl Karen , Júlíusson Pétur B.

Background: Using ultrasound to examine the glandular breast tissue is a promising method for staging pubertal breast development. However, breast ultrasound for this purpose has not been explored in a large sample of healthy girls in different developmental stages, and normative data have been unavailable.Objective: To present novel pubertal references for breast ultrasound stages, Tanner breast (B) and pubic hair (PH) ...

hrp0097p2-197 | Pituitary, Neuroendocrinology and Puberty | ESPE2023

Precocious thelarche and menarche in 5 years old girl – McCune - Albright syndrome – our experience.

Vitariusova Eva , Kostalova Ludmila , Pribilincova Zuzana , Niznanska Zuzana , Wallenfelsova Eva , Podracka Ludmila

Introduction: McCune - Albright syndrome is rare genetic disorder characterized by periphereal precocious puberty, fibrous bone dysplasia and cafe au lait skin spots.Case: Almost 5 years old girl was sent to Endocrinological department due to precocious thelarche and menarche. At the time of first examination, patient had breast M 3 and large cafe au lait macule on the right tight, no pubic and axilar hair was present, n...

hrp0084p3-1157 | Puberty | ESPE2015

Assessment of Endocrine Function in Egyptian Adolescent B-Thalassemia Major Patients

Dayem Soha Abd El , Kader Mona Abd El

Objective: To evaluate the endocrine function among Egyptian adolescent thalassemic patients.Patients and methods: 54 patients and 28 age-matched normal controls was included. Anthropometric measurements, clinical pubertal assessment were done for all patients. Oral glucose tolerance test (OGTT) was done to all patients and controls with measuring serum insulin level at 0.120 minutes. Insulin sensitivity and release index were calculated. 32 patients had...

hrp0084p3-1179 | Thyroid | ESPE2015

Plasma Visfatin Level and Its Association with Apolipoproteins A1 and B in Hypothyroid Children

Dehkordi Elham Hashemi , Rostampour Noushin , Kasiri Karamali , Moafi Mohammad , Aramesh Mohammad Esmail , Hashemipour Mahin

Background and aim: Hypothyroidism could be accountable for cardiovascular diseases; hence, necessity of novel biomarkers being capable to predict patientÂ’s status is indispensable. The aim of this study was to appraise alteration of plasma visfatin levels (as a newly discovered proteins) and its association with lipid profiles of hypothyroid patients.Materials and methods: In this cross-sectional, descriptive, and analytical study, 30 children bein...