hrp0094p2-346 | Pituitary, neuroendocrinology and puberty | ESPE2021

The clinical and biochemical spectrum of pituitary lesions in children compared to adults: experience from a single tertiary center

Galazzi Elena , Carrara Silvia , Soranna Davide , Zambon Antonella , Maria Fatti Letizia , Moro Mirella , Persani Luca ,

We present a monocentric cross-sectional study of 55 children referred between 2000 and 2020 for pituitary lesions, who underwent a complete pituitary function assessment. These data were compared to results obtained in a group of 295 adults carrying pituitary lesions undergoing a complete pituitary screening. Follow-up data was available for 50 children and 248 adults. The mean age at diagnosis in children was 12.2±3.7 years (range 2.1-17.9), while in adults was 48.7&#17...

hrp0092p1-120 | Pituitary, Neuroendocrinology and Puberty | ESPE2019

Role of Priming in Peri-Pubertal Growth Delays: Preliminary Results of A Large Multicenter Study

Galazzi Elena , Improda Nicola , Cerbone Manuela , Soranna Davide , Moro Mirella , Fatti Letizia Maria , Zambon Antonella , Salerno Mariacarolina , Dattani Mehul , Persani Luca

Introduction: Peripubertal children with delayed puberty frequently display a poor growth rate prompting endocrine work-up. Whether priming with sex steroids should routinely be performed in these patients to improve specificity of growth hormone stimulation tests (GHST) is unclear. Treatment with sex steroids in constitutional delay of growth and puberty (CDGP) is also debated.Patients and Methods: This multicenter retr...

hrp0089p2-p284 | Multisystem Endocrine Disorders P2 | ESPE2018

Endocrine and Metabolic Complications in Children and Adolescents with Sickle Cell Disease: An Italian Cohort Study

Bigi Elena , Bruzzi Patrizia , Palazzi Giovanni , Predieri Barbara , Lucaccioni Laura , Pancaldi Alessia , Lodi Mariachiara , Cellini Monica , Iughetti Lorenzo

Background: Children with Sickle Cell Disease (SCD) show endocrine complications and metabolic alterations. The physiopathology of these conditions is not completely understood: iron overload due to chronic transfusions, ischemic damage, and inflammatory state related to vaso-occlusive crises may be involved. Aims of this study were to evaluate the growth pattern and the endocrine and metabolic alterations in a cohort of children with SCD and to detect the relationship between...

hrp0084p3-982 | GH & IGF | ESPE2015

Experience of the Use of Genetically Engineered GH ‘Rastan’ by Children in Clinical Practice

Kulagina Elena , Savelyeva Elena , Chistousova Galina

Objective: To evaluate the efficacy of the drug ‘Rastan’, (manufacturer JSC ‘Pharmstandard-Ufa Vita’), in children with GH deficiency at the beginning of treatment at different ages.Materials and methods: We examined two groups of patients with GH deficiency who receive the drug for 3 years. In group 1, nine boys and two girls with age at debut of therapy 5.88±1.35 (from 3.3 to 7.1 years); in the 2nd group of ten boys and two gir...

hrp0092p3-166 | Growth and Syndromes (to include Turner Syndrome) | ESPE2019

Growth Hormone Treatment and Puberty in Patient with Pallister-Hall Syndrome

Pisareva Elena , Vitebskaya Alisa

Background: Pallister-Hall syndrome (PHS) is a rare autosomal dominant disorder characterized by a complex of different abnormalities (polydactyly, bifid epiglottis, hypothalamic hamartoma, imperforate anus). Syndrome is cuased by mutations in the GLI3 gene. PHS is associated with hypopituitarism, early or precocious puberty.Objective: to demonstrate a patient with PHS treated with growth hormone (GH) and gonadotropin re...

hrp0086p1-p561 | Perinatal Endocrinology P1 | ESPE2016

A Case of Hyperinsulinemic Hypoglycemia, Associated with Insulin Autoimmune Syndrome (IAS) in 3.5 Year Old Girl

Kuznetsova Elena , Melikyan Mariya

Background: Insulin Autoimmune Syndrome (IAS) is a rare cause of hyperinsulinemic hypoglycaemia with only few descriptions in children in the literature. Drugs containing the sulfhydryl group, such as methimazol, are known to be a causative factor of this syndrome. Diazoxide and octreotide are usually ineffective in such patients.Objective: We aim to describe a rare case of IAS in a child, with a good response to a short course of glucocorticoid therapy....

hrp0092p1-121 | Sex Differentiation, Gonads and Gynaecology or Sex Endocrinology | ESPE2019

Lower Urinary Tract Dysfunction and Infection in Girls with Disorders of Sex Development and Urogenital Sinus

Anikiev Alexander , Brovin Dmitriy , Volodko Elena , Okulov Alexey , Andreeva Elena

Introduction: Two-stage surgical feminization is a part of the multidisciplinary rehabilitation of girls with external genital virilization. The first stage involves clitoroplasty and labioplasty with preservation of urogenital sinus (US) in girls with third degree of virilization according to Prader classification and higher. US eliminating is performed by second stage in the puberty. Persistence US may cause dysfunction of urodynamics and infections of the l...

hrp0092p3-150 | GH and IGFs | ESPE2019

Features of Somatropin Replacement Therapy in a Patient with Floating Harbor Syndrome

Berseneva Olga , Bashnina Elena , Turkunova Mariia , Serebryakova Elena

Background: Growth hormone (GH) deficiency in children, confirmed by stimulation diagnostic tests, in some cases is accompanied by low effectiveness of somatropin replacement therapy, which may be associated with rare genetic syndromes.Aim: To study the growth effects of GH therapy in treating a patient with Floating-Harbor SyndromeMethods: A GH deficiency was diagnosed in a patien...

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...

hrp0089p3-p133 | Fat, Metabolism and Obesity P3 | ESPE2018

Nonclassical Manifestation of PWS

Bogova Elena , Volevodz Natalya , Peterkova Valentina

Background: Prader-Willi syndrome (PWS) is a complex, multisystem disorder and is the most frequent cause of syndromic obesity that arises from lack of expression of paternally inherited imprinted genes on chromosome 15q11-q13. Its major clinical features include neonatal hypotonia, short stature, developmental delay, behavioral abnormalities, hyperphagia, childhood onset obesity, hypothalamic hypogonadism, and characteristic appearance.Case history: We ...