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58th Annual ESPE

Vienna, Austria
19 Sep 2019 - 21 Sep 2019

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The theme of this year’s meeting is Variety and Variation in Paediatric Endocrinology. Join us in Vienna to explore the diversity we encounter in our discipline and the care that we have to exercise when using the term “normality”. Whilst gaining an update on the latest treatments, clinical best practice and cutting edge research in the field of paediatric endocrinology.

Poster Category 1

Pituitary, Neuroendocrinology and Puberty (2)

hrp0092p1-394 | Pituitary, Neuroendocrinology and Puberty (2) | ESPE2019

Next Generation Sequencing in GnRH Deficient Patients with Congenital Hypogonadotrophic Hypogonadism: Novel Findings in KAL1, SRA1, WDR11, FGFR1, CHD7 and PROP1 Genes

Neocleous Vassos , Fanis Pavlos , Toumba Meropi , Cinarli Feride , Schiza Melpo , Stylianou Charilaos , Tanteles George A , Oulas Anastasios , Spyrou George M , Skordis Nicos , Phylactou Leonidas A

Abstract: Objective: Congenital hypogonadotropic hypogonadism (CHH) is a rare genetic disease caused by GnRH deficiency and characterized by absent or incomplete puberty with infertility. The identification of the genetic cause in this group of patients through the use of next generation sequencing (NGS) can assist to the clinical management.Methods: Seven GnRH deficient nonrelated Cypriot probands were ...

hrp0092p1-395 | Pituitary, Neuroendocrinology and Puberty (2) | ESPE2019

Presentation and Diagnosis of Childhood Onset Combined Pituitary Hormone Deficiency: A Single Center Experience from Over 30 Years.

Hietamäki Johanna , Miettinen Päivi J. , Hero Matti , Tarkkanen Annika , Raivio Taneli

Background: We describe the incidence, etiologies and clinical features of combined pituitary hormone deficiency (CPHD) in pediatric patients from a single tertiary center.Methods: The cohort comprised of patients with CPHD, treated in the Helsinki University Hospital between 1985 and 2018. They were identified through an ICD-9/ICD-10 code search, and the clinical data were recorded from the patient charts.<p class="...

hrp0092p1-396 | Pituitary, Neuroendocrinology and Puberty (2) | ESPE2019

The Relationship Between Precocious Puberty and Premature Thelarche withSerum Irisin Levels

Kutlu Esra , Özgen Ilker Tolga , Bulut Huri , Temur Hafize Otçu , Torun Emel , Cesur Yasar

Background and Aims: Irisin is a newly discovered adipomyokine that occurs by the cleaved from a transmembrane protein called "Fibronectintype III domain containing 5" (FNDC5).Previously, there was a change in irisin levels in different prepubertal and pubertal stages.Although the mechanisms that trigger the onset of puberty cannot be explained yet, there is a hypothesis that peripheral adipose tissue and adipokines secreted there may induce the initia...

hrp0092p1-397 | Pituitary, Neuroendocrinology and Puberty (2) | ESPE2019

Questioning the Value of Brain MRIs in the Evaluation of Children with Isolated Growth Hormone Deficiency

Oren Asaf , Singer Dana , Rachmiel Mariana , Hamiel Uri , Shiran Shelly , Ben-Sira Liat , Schachter-Davidov Anita , Eyal Ori

Background: Isolated growth hormone deficiency (IGHD) is a relatively common disorder. Current diagnostic protocols require a brain MRI of the hypothalamus and the hypophysis after establishment of the diagnosis, with the aim of identifying structural defects and specifically rule out an underlying space-occupying lesion. An MRI scan is costly and requires general anesthesia in young children. Data on the contribution of brain MRI in diagnosing children with I...

hrp0092p1-398 | Pituitary, Neuroendocrinology and Puberty (2) | ESPE2019

Postoperative Quality of Life in Children and Adolescents with Craniopharyngioma – Results of the Prospective Multicenter Trial KRANIOPHARYNGEOM 2007

Eveslage Maria , Calaminus Gabriele , Warmuth-Metz Monika , Kortmann Rolf-Dieter , Pohl Fabian , Timmermann Beate , Schuhmann Martin , Flitsch Jörg , Faldum Andreas , Müller Hermann L.

Background: Craniopharyngioma is an embryonic tumor of low-grade malignancy. Children and adolescents with this diagnosis are analyzed concerning quality of life (QoL) and (progression-free) survival within the project KRANIOPHARYNGEOM 2007.Methods: The prospective, multi-center project consists of a randomized, unblinded substudy with adaptive design and an observational study. The randomized substudy for incompletely r...

hrp0092p1-399 | Pituitary, Neuroendocrinology and Puberty (2) | ESPE2019

Pubertal Events, Reproductive and Growth Hormones and Predictive Factors in Healthy Girls with Transient Thelarche.

Soto Julio , Pereira Ana , Busch Alexander , Almstrup Kristian , Corvalan Camila , Juul Anders , Mericq Veronica

Context: Transient thelarche (TT) corresponds to the appearance, regression and subsequent reappearance of the breast bud in girls. Only a single study about its frequency and progression is available (Lindhardt Johansen JCEM 2017).Objective and Hypotheses: To determine whether girls with TT girls (group 1) compared to girls without TT (group 2) show differences in i) timing/sequence of pubertal events ii) pattern of rep...

hrp0092p1-400 | Pituitary, Neuroendocrinology and Puberty (2) | ESPE2019

Plasma Copeptin Distribution in the Pediatric Age: A Useful Diagnostic Tool for AVP-Related Disorders

Tuli Gerdi , Tessaris Daniele , Buganza Raffaele , Matarazzo Patrizia , De Sanctis Luisa

Introduction: Copeptin is a stable AVP surrogate, secreted in equimolar relationship, who has been proposed for the diagnosis of AVP-related hypo and hypernatremic disorders, i.e. the syndrome of inappropriate ADH secretions (SIADH), the cerebral/renal salt wasting syndrome (C/RSW) and diabetes insipidus (DI). Few data exist about the normal ranges for plasma copeptin levels in the pediatric age, reported between 2.4-8.6 pmol/L. The aim of this study is to rep...

hrp0092p1-401 | Pituitary, Neuroendocrinology and Puberty (2) | ESPE2019

Management and Treatment Outcome of Childhood-Onset Craniopharyngioma (CP) in Italy: Multicentre Collection of 117 Cases

Zucchini Stefano , Fantini Jacopo , Mazzatenta Diego , Pozzobon Gabriella , Partenope Cristina , Pedicelli Stefania , Ubertini Graziamaria , Parpagnoli Maria , Genitori Lorenzo , Menardi Rachele , Driul Daniela , Matarazzo Patrizia , Tuli Gerdi , Guzzetti Chiara , Iughetti Lorenzo , Aversa Tommaso , Di Mase Raffaella , Rutigliano Irene , Iezzi Maria Laura , Cherubini Valentino , Grandone Anna , Cassio Alessandra

In Italy, treatment of children with CP is not centralized. We collected data of 117 patients (pts) (M/F 56/41) with CP diagnosed after 01/01/2000, followed-up in 14 centres of paediatric endocrinology belonging to the I.S.P.E.D. Five centres provided data on more than 10 pts (range 12-19), while the remaining on 1-9. 46 pts were diagnosed between 2000-2010 and 71 afterwards. Follow-up was 7.5±4.1 yrs.Results. Histology was adamanti...

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...

hrp0092p1-403 | Pituitary, Neuroendocrinology and Puberty (2) | ESPE2019

Xanthomatous Hypophysitis: A Rare Case in a Paediatric Patient

Jeanne Wong Sze Lyn , Selveindran Nalini , Hong Janet , Zain Fuziah

Background: Hypophysitis is a rare inflammatory condition of the pituitary that can mimic a neoplastic lesion. Histopathology subtypes include lymphocytic, granulomatous, xanthomatous, plasmacytic or a mixed picture. Among these, xanthomatous hypophysitis (XH) is the least common with an unknown aetiology. Unlike lymphocytic hypophysitis, which is believed to be autoimmune in origin, XH is rarely reported to be associated with other autoimmune diseases and res...

hrp0092p1-404 | Pituitary, Neuroendocrinology and Puberty (2) | ESPE2019

Improvement of Final Height in Idiopathic Central Precocious Puberty is Associated with Delay of Bone Maturation with GnRH Agonist Therapy Under the Age of 7 Years

Vuralli Dogus , Gonc E. Nazli , Ozon Z. Alev , Kandemir Nurgun , Alikasifoglu Ayfer

Background: GnRHa therapy is shown to be beneficial in increasing final height when started before 6 years of age in girls with idiopathic CPP(iCPP). However controversial data exist in improvement of final height whose pubertal signs appear after age of 6.Aim: To investigate effect of age of onset of GnRHa therapy on final height n girls with iCPP, and to evaluate factors affecting height gain.<st...

hrp0092p1-405 | Pituitary, Neuroendocrinology and Puberty (2) | ESPE2019

Metabolic Changes in Children Treated for Medulloblastoma

Kalinin Alexey , Strebkova Natalia , Vasyukova Olga , Okorokov Pavel , Zheludkova Olga

Abstract: The development of endocrine disorders after complex treatment of medulloblastoma is out of doubt. Much less attention is paid to the study of metabolic changes in the outcome of treatment. In our clinic, we examined 63 patients (40 males/23 females) after the complex therapy of medulloblastoma (surgery, craniospinal radiation therapy and chemotherapy). Patients had a median age (range) of 11.3 (5.5÷17.9) years. They were treated for medulloblas...

hrp0092p1-406 | Pituitary, Neuroendocrinology and Puberty (2) | ESPE2019

Tolvaptan for Management of Intractable Salt and Water Imbalance in a Case with Suprasellar Tumor after Surgery

Yamaguchi Tomoe , Terashita Shintaro , Kinjo Kenichi , Fujisawa Yusuke , Yoshii Keisuke , Naiki Yasuhiro , Horikawa Reiko

Background: It is sometimes difficult to diagnose and manage fluid and electrolyte imbalance after surgery for hypothalamic/pituitary tumors. We present a pediatric case of severe SIADH successfully treated with tolvaptan after suprasellar tumor resection.Case: The case was 8-year-old girl with growth failure. She was found to have suprasellar tumor on CT scan when she accidentally fell down and hit her head. MRI suggest...

hrp0092p1-407 | Pituitary, Neuroendocrinology and Puberty (2) | ESPE2019

No Association Between Serum Level of NPTX 1 and MKRN3 in Central Precocious Puberty

Rim Jeong Hwal , Tae Hwang Il

Abstract: Background: Makorin ring finger protein 3 (MKRN3) is most common genetic cause of central precocious puberty (CPP) and associated with the initiation of puberty. Although its actual function is veiled. Recent study reported MKRN3 interacted with and suppressed neural pentraxin-1 precursor (NPTX1) activity via polyubiquitination during early puberty in mice.Objective: The aim of this study was t...

hrp0092p1-408 | Pituitary, Neuroendocrinology and Puberty (2) | ESPE2019

Training in Pubertal Assessment – First Step to the Observational Pilot Study PROSPEL (Premier Observatoire des Stades Pubertaires en Libéral)

Jesuran-Perelroizen Monique , Puel Olivier , Mazzarino Johan

Introduction: Recent publications report an earlier age of onset of pubertal changes in the US and Europe. Referrals to pediatric-endocrinologist for early puberty are increasing. Precocious puberty diagnosis is often delayed, especially in boys. Improvement in pubertal assessment (age at onset of puberty) by pediatricians and general practitioners is needed to participate in the PROSPEL study and to improve management of pubertal abnormalities.<p class="a...