hrp0092p3-279 | Late Breaking Abstracts | ESPE2019

Relation between Levels of Atymullerian Hormone and Inhibin B and Spontaneous Puberty in Patients with Turner Syndrome – Preliminary Results

Ruszała Anna , Wójcik Małgorzata , Starzyk Jerzy B.

Introduction: Primary hypogonadism is stated as one of major features in patients with Turner Syndrome (TS), however it is not a constant finding. Variable markers of ovaries failure in TS patients are still missing. The present study aimed to evaluate the usefulness of atymullerian hormone and inhibin B assessment in predicting spontaneous puberty in patients with TS.Methods: The study included 35 TS patients. Gonadal a...

hrp0092p3-317 | Late Breaking Abstracts | ESPE2019

Body Mass Index and Incident Type 1 Diabetes in Children from Lesser Poland Over an 11 Year Observation Period

Wasyl-Nawrot Barbara , Wójcik Małgorzata , Nazim Joanna , Skupień Jan , Starzyk Jerzy

Background: One of the speculated causes of the decreasing age of onset of type 1 diabetes is the increase in body weight in children. This so-called accelerator hypothesis is, however, controversial. The aim of the study was to test whether younger age of type 1 diabetes onset is associated with higher BMI-SDS at the time of diagnosis.Methods: Retrospective data analysis from medical records of all patients und...

hrp0089p2-p034 | Adrenals and HPA Axis P2 | ESPE2018

Etiology of Primary Adrenal Insufficiency in Children: a 29-year Single Center Experience

Ma Huamei , Zhang Jun , Guo Song , Li Yanhong , Chen Hongshan , Chen Qiu , Du Minlian , Li Shaofu

Objective: To investigate the etiology and clinical features of Chinese children with PAI.Method: 427 children (age 0–18 years) with PAI followed at our institution between September 1989 and March 2016 were studied.Results: 1. 228 males and 199 female (1.14:1) were included. Median age at diagnosis was 1.66 (10th–90th, 0.06~8.73 yrs.2. An identified diagnosis(clinical or genetic) was obtained in 93...

hrp0089p1-p053 | Diabetes & Insulin P1 | ESPE2018

Neonatal Diabetes as a First Symptom of IPEX Syndrom

Brandt Agnieszka , Okońska Maja , Hennig Matylda , Mysliwiec Małgorzata , Młynarski Wojciech

Introduction: Immunodysregulation polyendocrinopathy enteropathy x-linked syndrome (IPEX) is characterized by systemic autoimmunity, typically beginning in the first year of life. Most commonly triad of symptoms of diarrhea, dermatitis and endocrinopathy is present.Case report: Presentatlion of male patient, born with body weight 3840 grams and 10 points in Apgar scale. In 13th day of life vomitting and tachypnoe were noted and in laboratory tests hyperg...

hrp0089p2-p136 | Fat, Metabolism and Obesity P2 | ESPE2018

Dyslipidemia and Its Related Factors in Chinese Children and Adolescents with Turner Syndrome

Huang Siqi , Chen Hongshan , Du Minlian , Li Yanhong , Ma Huamei , Chen Qiuli , Zhang Jun , Guo Song

Turner syndrome is associated with insulin resistance, increased incidence of type II diabetes, and hypertension, all of which are cardiovascular risk factors. The purpose of this study was to evaluate the lipid profile of Chinese girls with untreated Turner syndrome, (aged 2 to 15 years; 50.9% 45,XO) and age-matched, normal girls. A total of 108 girls with Turner syndrome and 99 normal girls had lipid profile measurements, including cholesterol, triglycerides,high-density lip...

hrp0089p3-p145 | Fat, Metabolism and Obesity P3 | ESPE2018

Thyroid Function, Lipid Profile and Carbohydrate Metabolism Parameters in Patients with Alstrom Syndrome

Okońska Maja , Brandt Agnieszka , Myśliwiec Małgorzata

Introduction: Alstrom syndrome is an autosomal recessive genetic disorder with mutation in the ALMS 2p12-13 gene and its characteristic features are: pigmented retinopathy, deafness, growth deficiency, obesity, metabolic syndrome, diabetes, thyroid dysfunction, nephropathy and cardiomyopathy.Aim of the study: Evaluation of anthropometric parameters, thyroid function, carbohydrate metabolism and lipid profile in five patients with diagnosed Alstrom syndro...

hrp0089p3-p183 | Fetal, Neonatal Endocrinology and Metabolism P3 | ESPE2018

Diagnosis and Treatment of Persistent Hyperkalemia in Newborn Twins – Rare Case Report of Gordon Syndrome

Zhang Jun , Ma Huamei , Li Yanhong , Guo Song , Du Minlian , Yu Muxue , Li Xiaoyu

Objectives: To summarize the diagnosis and treatment course of hyperkalemia in twins, review the diagnosis approach of hyperkalemia in neonate and guide clinical practice.Methods: The clinical manifestation, laboratory examination, the course of diagnosis and treatment of the two cases were summarized.Results: A pair of twin girls, 38 days after birth, came to our department because of ‘Hyperkalemia more than one month’. ...

hrp0089p2-p317 | Pituitary, Neuroendocrinology and Puberty P2 | ESPE2018

The Effect of Letrozole on the Reproductive Function and Linear Growth in the Early and Mid Puberty Boys

Ma Huamei , Lin Juan , Zhang Jun , Li Yanhong , Chen Qiuli , Du Minlian

Objectives: To investigate the effect of Letrozole on the reproductive function and linear growth in adolescent boys.Methods: 43 early and middle pubertal boy with seriously damaged predicted adult height(PAH), treated with letrozole 1.5mg/m2/d Po(≯2.5mg/d) with a duration of 3-18 months were enrolled as Short-, medium- and long- treatment group with letrozole of 3-6, 6-12, 12-18 months, respectively. 48 healthy pubertal boys were enrolled as control...

hrp0089p3-p286 | Pituitary, Neuroendocrinology and Puberty P3 | ESPE2018

Post-Traumatic Hypopituitarism Caused by Pituitary Stalk Transection

Ruszała Anna , Wojcik Małgorzata , Krystynowicz Andrzej , Wyrobek Łukasz , Starzyk Jerzy B

Common cause of hypopituitarism is congenital, genetically determined abnormality called pituitary stalk interruption syndrome (PSIS). It is characterised by hypoplastic anterior pituitary gland with thin or absent infundibulum and ectopic posterior pituitary location in MRI examination. Post-traumatic hypopituitarism may mimic this image. We present 7 years and 10 months old girl who was admitted to Pediatrics Institute due to hypoglycemia during an acute gastroenteritis. Hyp...

hrp0089p3-p321 | Sex Differentiation, Gonads and Gynaecology or Sex Endocrinology P3 | ESPE2018

Gonadal Tumor Incidence in Patients with Disorders of Sex Development Containing Y Chromosome or Y-derived Sequences – Experience from One Clinical Center

Gawlik Aneta , Antosz Aleksandra , Drosdzol-Cop Agnieszka , Kudela Grzegorz , Koszutski Tomasz , Małecka-Tendera Ewa

Background: Risk of developing germ cell tumors (GCTs) in disorders of sex development (DSD) patients with karyotypes contain Y-chromosome or it’s material (Y) increase with age. The appropriate timing for prophylactic gonadectomy in these patients is still controversial.Aim: To analyze the gonadal tumor incidence and histological assessment of gonads in DSD (Y) patients who were treated in a single institution between 1997 and 03/2018.<p class=...