hrp0098fc7.6 | GH and IGFs | ESPE2024

Growth, IGF-1 and IGFBP-3 Responses to Oral LUM-201 in OraGrowtH210 and OraGrowtH212 Trials in Pediatric Growth Hormone Deficiency (PGHD) over 12 to 24 Months on Treatment

Petriczko Elzbieta , Cassorla Fernando , Investigator Group OraGrowtH210 , Research Team OraGrowtH212 , Bruchey Aleksandra , Smith Christopher , L. Brincks Erik , C. McKew John , O. Thorner Michael , “Duke” Pitukcheewanont Pisit

Background: LUM-201, a potent long-acting oral GH secretagogue (GHS), acts on the GHS Receptor-1a to induce GH secretion. The best candidates for this investigative oral treatment are pre-pubertal children with moderate GHD (standard stimulation testing peak GH between ≥3 <10ng/ml) that respond positively to the LUM-201 Predictive Enrichment Marker (PEM) test (Bright et al JES, 2021). PEM positive responders have basal serum IGF-1 >30ng/ml and a peak ...

hrp0098p2-260 | Sex Differentiation, Gonads and Gynaecology, and Sex Endocrinology | ESPE2024

Care of transgender children and adolescents in our region: nine years of experience of a multidisciplinary working group

Bertholt Zuber Laura , Portal Buenaga Marina , Alonso Rubio Pablo , Villar Bonet Aurelia , Pazos Toral Fernando , Canceller Meseguer Telva , Zorrilla Arce Ana

Background: In recent years, the number of consultations of transgender children and adolescents has gradually increased. In our region, this group receives care from a specialized multidisciplinary team, formed in 2015. It is basically composed of a psychosexologist, two adult endocrinologist, a pediatric endocrinologist, among other specialists.Objective: To describe the characteristics of the children and adolescents ...

hrp0092p3-196 | Pituitary, Neuroendocrinology and Puberty | ESPE2019

Precocious Pseudo-Puberty Presenting with Bilateral Ovarian Involvement and Progressing to Juvenile Granulosa Cell Tumor in a 2-Year-Old Girl

Barakizou Hager , Donaldson Malcolm , Huma Zilla , Amary Fernanda , Gannouni Souha

Background: Feminising preocious pseudo-puberty in McCune Albright syndrome (MAS) and juvenile granulosa cell tumour (JGCT) arises from bilateral and unilateral estradiol hypersecretion respectively. GNAS mutations have been described in some cases of JGCT as well causing MAS.Aim: To describe a case in which MAS was evoked initially, but then progressed to JGCTCase: A girl aged 2 y...

hrp0094p2-81 | Bone, growth plate and mineral metabolism | ESPE2021

Early-onset osteoporosis due to LRP5

Rios Beatriz , Acuna Pilar , Ochoa Fernanda , Cristian Seiltgens ,

Introduction: Primary osteoporosis are frequently linked to syndromic conditions such as osteogenesis imperfecta which also involves extra esqueletic tissues. With the advancement of medicine, this group has been reduced, due the ability to specifying the causal pathogenic variants. Among these, the LRP5 (Low-density lipoprotein receptor-related protein 5) gene mutations are the most frequent cause. LPR5 is a fundamental coreceptor in the wnt/betacatenin signa...

hrp0095rfc10.4 | GH and IGFs | ESPE2022

Recombinant PAPP-A2 induces sex-specific increases in body weight and length and changes in bone microstructure in adolescent mice with longitudinal growth reduction induced by Pappa2 deletion

del Mar Fernández-Arjona María , Rubio Leticia , López-Gambero Antonio , Rivera Patricia , Vargas Antonio , Rodríguez de Fonseca Fernando , A. Chowen Julie , Argente Jesús , Suárez Juan

Deficiency of pregnancy-associated plasma protein-A2 (PAPP-A2), a protease that regulates IGF-1 availability, causes postnatal growth failure and changes in bone size and density in humans and mice. The present study aimed to determine the effects of daily administration (from PND5 to PND35) of recombinant murine (rm) PAPP-A2, in comparison to rmGH and rmIGF1, on mouse auxology and bone microarchitecture in homozygous Pappa2 knock-out (ko/ko) mice of both sexes. Hormone treatm...

hrp0095p1-66 | Fat, Metabolism and Obesity | ESPE2022

Effect of gender and age on the mental well-being and self-esteem of adolescents living with obesity: subanalysis from the ACTION Teens global study

Pedro López Siguero Juan , A. Baur Louise , Bereket Abdullah , Bin-Abbas Bassam , Chen Walter , Fernández-Aranda Fernando , Garibay Nieto Nayely , C.G. Halford Jason , Maffeis Claudio , Mooney Vicki , Karenina Osorto Cynthia , Reynoso Ricardo , Rhie Young-Jun , Toro-Ramos Martín

Background: The ACTION Teens study aimed to identify attitudes, behaviours and perceptions among adolescents living with obesity (ALwO), caregivers of ALwO (CGs) and healthcare professionals (HCPs) who treat ALwO. This subanalysis assessed the mental well-being and self-esteem of ALwO.Methods: ACTION Teens was a global, cross-sectional study (NCT05013359) that surveyed 5275 ALwO (aged 12–<18 years), 5389 CGs and...

hrp0095p1-67 | Fat, Metabolism and Obesity | ESPE2022

Effect of unawareness of obesity on perceptions of obesity and food/diet among adolescents living with obesity and their caregivers: subanalysis from the ACTION Teens global study

Bereket Abdullah , A. Baur Louise , Bin-Abbas Bassam , Chen Walter , Fernández-Aranda Fernando , Garibay Nieto Nayely , C.G. Halford Jason , Pedro López Siguero Juan , Maffeis Claudio , Mooney Vicki , Karenina Osorto Cynthia , Reynoso Ricardo , Rhie Young-Jun , Toro-Ramos Martín

Background: This subanalysis of the ACTION Teens study aimed to explore perceptions of obesity and food/diet among adolescents living with obesity (ALwO) and caregivers of ALwO (CGs) who were unaware of their/their child’s obesity.Methods: The global, cross-sectional ACTION Teens survey study (NCT05013359) assessed perceptions, attitudes and behaviours among 5275 ALwO (aged 12–<18 years), 5389 CGs and 2323...

hrp0095p1-470 | Fat, Metabolism and Obesity | ESPE2022

High carbohydrate diet results in sex-specific differences in energy homeostasis in mice with PAPP-A2 deficiency

J. López Gambero Antonio , del Mar Fernández-Arjona María , De Ceglia Marialuisa , Rubio Leticia , Rivera Patricia , Vargas Antonio , Vera-Fernández Carlos , Rodríguez de Fonseca Fernando , A. Chowen Julie , Argente Jesús , Suárez Juan

The growth hormone (GH)-insulin-like growth factor (IGF-1) system is essential for optimal human growth and energy homeostasis. Deficiency of pregnancy-associated plasma protein-A2 (PAPP-A2), a protease involved in the liberation of free IGF-1, leads to problems in growth and bone density in humans and mice. Patients with PAPP-A2 deficiency also present lower body mass and mild glucose intolerance. The present study aimed to determine the influence of 1 month of high carbohydr...

hrp0089p3-p318 | Pituitary, Neuroendocrinology and Puberty P3 | ESPE2018

SIG (Special Interest Group)-ENDOPED/RUTE (Brazil): Seven Years Integrating Pediatric Endocrinology Centers throughout the Country

Arrais Ricardo Fernando , Alves Cresio Aragao Dantas , Junior Gil Guerra , Castro Luiz Claudio Goncalves , Filho Geraldo Miranda Graca , Kopacek Cristiane , Damiani Durval , Junior Raphael Del Roio Liberatore , Punales Marcia Khaled

Introduction: Telemedicine, or the use of CIT (Communication and Information Technology) to deliver and/or share medical remote assistance and knowledge, is of paramount importance, mainly in large countries, with social and economic disparities, as Brazil, by means of teleconferences, webconferences, webcasts and use of a wide range of interactive technologies, helping activities of assistance and professional health education. One of the activities provided by our RUTE (Univ...

hrp0084p3-818 | Endocrine Oncology | ESPE2015

Endocrine Evaluation in Children and Adolescents Submitted to Allogeneic Bone Marrow Tranplantation

Kuperman Hilton , Manna Thais Della , Dichtchkenian Vae , Filho Hamilton Cabral de Menezes , Steinmetz Leandra , Cominato Louise , Fernandes Juliana Folloni , Mandelli Angela , Mantovani Luiz Fernando , Zanichelli Maria Aparecida , Colassanti Maria Dulce , Cristofani Lilian Maria , Filho Vicente Odone , Damiani Durval

Background: Paediatric bone marrow transplantation (BMT) can lead to endocrine dysfunctions due to common pre-procedure protocols involving chemo and radiotherapy.Objective and hypotheses: To evaluate the prevalence and time-of-onset of endocrine dysfunctions after BMT in children and adolescents.Method: A retrospective cohort-study design was performed. The inclusion criteria were: age range less than 18 years old at the time of t...