hrp0098p3-210 | Pituitary, Neuroendocrinology and Puberty | ESPE2024

Hypergonadotropic hypogonadism in a girl after bone marrow transplantation from a male unrelated donor.

Shuperliska Elizabeta , Shukarova-Angelovska Elena , Krstevska-Kostantinova Marina , Jancevska Aleksandra , Ivanova Daniela

Background: Primary hypogonadism in females is characterized by inadequate ovarian function, resulting in insufficient or absent production of estrogens. This case report aims to present a rare case of primary amenorrhea in a female child where bone marrow transplantation (BMT) was performed due to the beta-thalassemia.Case presentation: A 14-year-old girl approached the endocrinology department for evaluation due to del...

hrp0097p1-520 | Growth and Syndromes | ESPE2023

Does cervical medullary decompression have an impact on growth in children with achondroplasia?

Fava Daniela , Tedesco Caterina , Angelelli Alessia , Napoli Flavia , Pepino Carlotta , Pepe Alessia , Teruzzi Daniela , Pisati Angelica , Fay Cortella Maria , Binelli Maria , Maghnie Mohamad , Di Iorgi Natascia , Elsa Maria Allegri Anna

Background: Foramen magnum stenosis (FMS) is a life-threatening complication in children with achondroplasia (ACH) which may require cervicomedullary decompression (CMD). There is no evidence if FMS and CMD affects growth in children with ACH.Aim: To evaluate the impact of FSM and CMD surgery on anthropometric measurements in children with ACH.Methods: Sixty-five patients with ACH ...

hrp0095fc9.6 | Pituitary, Neuroendocrinology and Puberty | ESPE2022

Cerebral infarction in childhood-onset craniopharyngioma patients: results of KRANIOPHARYNGEOM 2007

Boekhoff Svenja , Bison Brigitte , Genzel Daniela , Eveslage Maria , Friedrich Carsten , Flitsch Jörg , Müller Hermann L.

Background: Cerebral infarction (CI) is a known vascular complication following treatment of suprasellar tumors. The purpose of our study was to determine the incidence of cerebral infarction (CI) in a cohort of 244 German childhood-onset craniopharyngioma (CP) patients recruited between 2007 and 2019 with a high degree of completeness in the prospective, randomized trial KRANIOPHARYNGEOM 2007 (Clinical Trial No. NCT01272622). Up to now, risk factors for CI, i...

hrp0095p1-118 | Growth and Syndromes | ESPE2022

A background review of several generic patient-reported outcome and clinician-reported outcome measures: Selecting measures for further evaluation in a qualitative study in achondroplasia.

D. Mathias Susan , Hoover-Fong Julie , Savarirayan Ravi , Cho Terry , Lee Anne , H. Colwell Hilary , Rogoff Daniela , Muslimova Elena

Objectives: No validated achondroplasia (ACH)-specific patient reported outcome (PRO) or clinician-reported outcome (ClinRO) measures are available. A detailed review of generic PRO and ClinRO measures was performed to select relevant tools for further evaluation in a qualitative study.Methods: Published literature describing medical challenges and impacts among children with ACH was reviewed to identify important concep...

hrp0095p2-304 | Late Breaking | ESPE2022

Diabetic ketoacidosis associated with hypertriglyceridemia in the pediatric age group – A case report

Zorron Mariana , Duarte Gomes Machado Raquel , Albiero Camargo Daniela , Seben Julia , Fernanda Vanti Macedo Paulino Maria , Mendonça Erika , Fanger Vanessa

Introduction: Diabetic ketoacidosis (DKA) is a potentially fatal hyperglycemic emergency primarily associated with type 1 diabetes mellitus (T1D). The association between DKA and severe hypertriglyceridemia has already been previously discussed with a prevalence ranging between 7.1%-25%, with pancreatitis present in the majority of cases. Epidemiological data in the pediatric age are scarce and not well established. Its etiology is not yet fully known. The &qu...

hrp0092p1-323 | Diabetes and Insulin (2) | ESPE2019

Diabetes Type 2 in Non-Obese Neurologically Impaired Children and Adolescents: A New Emerging Entity?

Calcaterra Valeria , Regalbuto Corrado , Montalbano Chiara , Vinci Federica , De Silvestri Annalisa , Pelizzo Gloria , Cena Hellas , Larizza Daniela

Background: Insulin resistance (IR) plays a key role in the pathogenesis of type 2 diabetes (T2D). In neurologically impaired (NI) children unfavorable cardio-metabolic risk profile with high prevalence of IR has been reported. We evaluated the prevalence of T2D in NI children and adolescents, in order to define if a dedicated glucose monitoring may be recommended in these subjects.Patients and Methods: We retrospectivel...

hrp0092p1-378 | Growth and Syndromes (to include Turner Syndrome) (2) | ESPE2019

Vascular Anomalies and Aortic Dilatation in Turner Syndrome Study in a Large Cohort of Young-Adult Patients

Scarano Emanuela , Varini Susanna , Tamburrino Federica , Perri Annamaria , Costa Margherita , Prandstraller Daniela , Lovato Luigi , Balducci Anna , Gibertoni Dino , Mazzanti Laura

Background: Patients with Turner Syndrome (TS) are at high risk for congenital heart disease (CHD), aortic dilatation and dissection with increased mortality and morbidity. Thoracic gadolinium-enhanced MRI angiography allows clear imaging of all great vessels and revealed a spectrum of silent vascular anomalies (VA), both venous and arterial, undetected at Echocardiography.The aim of this study is to retrospectively evaluate the prevalen...

hrp0092p3-98 | Fat, Metabolism and Obesity | ESPE2019

High Allostatic Load in Children with Excess of Weight

Calcaterra Valeria , Montalbano Chiara , Vinci Federica , Regalbuto Corrado , Casari Giulia , De Silvestri Annalisa , Cena Hellas , Larizza Daniela

Objective: Allostatic load (AL) refers to the physiological response that occurs in chronic stress burden, Excessive weight gain is an important source of physiological stress promoting chronic low-inflammation state detrimental for health. We estimated AL score among a pediatric population, in order to define a correlation between cumulative biological dysregulation and excess weight.Methods: We enrolled 164 children an...

hrp0092p3-115 | Fat, Metabolism and Obesity | ESPE2019

Gender-Based Differences in the Clustering of Metabolic Syndrome Factors in Children and Adolescents

Calcaterra Valeria , Regalbuto Corrado , Vinci Federica , Montalbano Chiara , Dobbiani Giulia , De Silvestri Annalisa , Cena Hellas , Larizza Daniela

Background: we depicted gender-differences in metabolic syndrome (MS) clustering before and after puberty in pediatrics, to identify early childhood prevention intervention and treatments.Methods: We considered 1079 children and adolescents. According to body mass index (BMI) percentiles the subjects were classified as normal weight BMI<75th, overweight BMI 75-95th and with obesity BMI>95th....

hrp0089p1-p033 | Bone, Growth Plate &amp; Mineral Metabolism P1 | ESPE2018

Comparison of Manual and Automated Bone Age Assessment in 1285 Children and Adolescents Aged 5 to 16 Years

Maratova Klara , Zemkova Daniela , Lebl Jan , Soucek Ondrej , Pruhova Stepanka , Kolouskova Stanislava , Snajderova Marta , Krasnicanova Hana , Sumnik Zdenek

Background: Skeletal maturation is the most reliable indicator of biological age in children and adolescents. The evaluation of hand and wrist X-Ray according to Tanner-Whitehouse (TW3) or Greulich-Pyle (GP) are the most commonly used methods for biological age assessment. Automated bone age assessment has recently become increasingly popular, however a large independent study comparing automated and manual evaluation of bone age is still missing. The aim of this study was to ...