hrp0092fc8.2 | Pituitary, Neuroendocrinology and Puberty Session 1 | ESPE2019

Analysis of Hypothalamic Metabolic Circuits after Normalization of Body Weight in Mice That Had Been Obese Due to High Fat Diet Intake

Guerra-Cantera Santiago , Frago Laura , Ros Purificación , Canelles Sandra , Díaz Francisca , Freire-Regatillo Alejandra , Torrecilla-Parra Marta Marta , Argente Jesús , Chowen Julie A.

The obesity epidemic continues to be a dramatic problem in the developed world despite attempts to curtail its rise. Reducing energy intake and/or increasing energy expenditure can result in weight loss; however, if one returns to their poor lifestyle habits the previous weight is not only recuperated, but often surpassed.We hypothesized that although a normal body weight may be achieved, the hypothalamic circuits controlling appetite and energy expendit...

hrp0092p1-402 | Pituitary, Neuroendocrinology and Puberty (2) | ESPE2019

Evaluation of Brain Mri Lesions in 381 Girls with Central Precocious Puberty

Helvacioglu Didem , Guran Tulay , Kirkgoz Tarik , Atay Zeynep , Abali Zehra Yavas , Eltan Mehmet , Kaygusuz Sare Betul , Seven Tuba , Gurpinar Busra , Turan Serap , Bereket Abdullah

Central precocious puberty (CPP) in girls is a diagnosis increasingly made by the Pediatric Endocrinologists worldwide. Although it is most frequently of idiopathic origin, magnetic resonance imaging (MRI) of the brain is recommended to rule out organic lesions causing CPP. However, controversy exists regarding the age limits for routinely performing MRI in girls with CPP.Objective: To evaluate the outcome of brain MRI in girls diagnosed...

hrp0092p2-179 | Growth and Syndromes (to include Turner Syndrome) | ESPE2019

Unusual Case of Patient with Klinefelter Syndrome with Shox Deletion Born to the Mother with Leri-weill Dyschondrosteosis

Krnic Nevena , Huljev Frkovic Sanda , Dumic Kubat Katja , Braovac Duje , Uroic Anita Spehar

Introduction: Klinefelter syndrome (KS) describes the phenotype of the most common sex chromosome abnormality in humans. About 80% of KS patients have 47,XXY karyotype, while rest of the patients can have mosaicism or other numeric or structural sex chromosome abnormalities. Tall stature is one of the hallmarks of KS and it is thought to be due to supernumerary X chromosome leading to SHOX gene overdosage. Deletion of SHOX gene, on the contrary, has been r...

hrp0092p3-312 | Late Breaking Abstracts | ESPE2019

Emotional Status Instability and Body Mass Index as Predictive Markers for Dopamine System Dysfunction Evaluation in Pubertal Age Children

Viazava Liudmila , Solntsava Anzhalika , Zaytseva Elena

Study aim was to create a prognostic algorithm of dopamine system dysfunction in pubertal age children, based on emotional instability markers, depending on body mass index and pubertal periods.Materials and Methods: Study subjects comprised 120 children (11.6 – 17.9 y/o, the 2nd – 5th Tanner stages) from Belarusian population between 2015 and 2017. Emotional status instability sings were obtained using th...

hrp0089p1-p157 | GH & IGFs P1 | ESPE2018

EasypodTM Connect Observational Study: The Italian Experience

Loche* Sandro , Centonze Chiara

Objective: The Easypod Connect Observational Study (ECOS) is a prospective long-term observational study aimed at evaluating the level of adherence in patients receiving growth hormone via the easypod device. ECOS started in 2010 in 24 countries. The easypod auto-injector device enables accurate records of patients’ adherence to recombinant human growth hormone (r-hGH) to be collected, providing real-world data for evaluation. We present three years prospective adherence ...

hrp0086p1-p558 | Perinatal Endocrinology P1 | ESPE2016

Cerebral Outcome of Children with Congenital Hyperinsulism

Rasmussen Annett Helleskov , Melikian Maria , Portner Fani , Larsen Anna-marie , Scherderkina Inna , Globa E , Filipsen Karen , Brusgaard Klaus , Christesen Henrik

Background: Congenital hyperinsulinism (CHI) is a rare condition characterized by unregulated secretion of insulin from pancreatic islet β cells. The primary treatment goal is to obtain normoglycemia, since hypoglycemia in the neonatal period can have severe impact on cerebral development.Objective and hypotheses: To assess the cerebral function in children with CHI at follow up.Method: From an international cohort, 40 childre...

hrp0082p2-d3-613 | Turner Syndrome | ESPE2014

Nationwide Study of Turner Syndrome During Childhood in Turkey: Evaluation of Associated Problems

Yesilkaya Ediz , Darendeliler Feyza , Bereket Abdullah , Bas Firdevs , Poyrazoglu Sukran , Aydin Banu Kucukemre , Darcan Sukran , Dundar Bumin , Buyukinan Muammer , Kara Cengiz , Sari Erkan , Adal Erdal , Akinci Aysehan , Atabek Mehmet Emre , Demirel Fatma , Celik Nurullah , Ozkan Behzat , Ozhan Bayram , Cinaz Peyami , Pediatric Endocrinology Turner Study Group

Background: Turner syndrome is one of the most common chromosomal disorders and is seen in 1:2500 female live births. The disease manifests with various clinical features and can be classified according to karyotype as monosomy, mosaicism, numeric, and structural abnormalities.Objective and hypotheses: Patients with Turner syndrome have complicated with various manifestations congenital or acquired. In this study we aimed to emphasise the importance of a...

hrp0084p1-93 | Growth | ESPE2015

Severe IGF1 Deficiency and Multi-Organ Autoimmune Disease Associated with Novel Germline STAT3 Mutations

Scaglia Paula , Keselman Ana , Gutierrez Mariana , Domene Sabina , Blanco Miguel , Sanguinetti Nora , Bezrodnik Liliana , Di Giovanni Daniela , Caldirola Maria Soledad , Martucci Lucia , Karabatas Liliana , Kumar Ashish , Jones Nana-Hawa , Hwa Vivian , Revale Santiago , Vazquez Martin , Jasper Hector , Domene Horacio

Background: Primary IGF1 deficiency can result from molecular defects in genes encoding for the GHR, IGF1, STAT5b and ALS. Heterozygous, activating mutations in the STAT3 gene have been recently described in children with severe growth failure associated with a spectrum of early-onset autoimmune disease.Case presentation: We report the molecular diagnosis in two unrelated patients with severe growth failure and IGF1 deficiency: P1, a 3.6 year ol...

hrp0084p2-356 | Fat | ESPE2015

Physical and Metabolic Evolution of Obese Children and Adolescents after the Attainment of Intense Weight Reduction

Martos-Moreno Gabriel A , Martinez-Villanueva Julian , Gonzalez-Leal Rocio , Argente Jesus

Background: Despite the lack of drugs, conservative management of childhood obesity allows for considerable weight reduction.Objective and hypotheses: i) To evaluate anthropometric and metabolic changes in obese children after intense weight loss. ii) To analyze the influence of the amount of weight loss and the time spent to attain it on the observed changes. iii) To investigate BMI evolution during the first 3 years after weight reduction.<p class=...

hrp0094p1-101 | Adrenal B | ESPE2021

The management of adrenal cell carcinoma in a single tertiary centre: 25 year experience

Goff Nicole , Hughes Claire , Katugampola Harshini , Mushtaq Imran , Hindmarsh Peter , Peters Catherine , Brain Caroline , Jorgensen Mette , Dattani Mehul ,

Background: Adrenal cortical carcinoma (ACC) in children is rare and aggressive, with the mainstay of treatment being surgical resection, although there have been recent improvements in outcomes with chemotherapy. Further characterisation of the presenting features and biochemical markers are needed to support earlier diagnosis. Refractory hypertension related to high cortisol concentrations prior to surgery, and post-operative decrease in cortisol can be chal...