hrp0084p2-524 | Puberty | ESPE2015

Pulsatile GnRH is Superior to hCG in Therapeutic Efficacy in Adolescent Boys with Hypogonadotropic Hypogonadodism

Gong Chunxiu , Liu Ying , Qin Miao , Wu Di , Wang Xiaoling

Background: We investigate the efficacy and safety of two different treatments which have not been evaluated in peri-puberty boys with HH.Objective and hypotheses: To assess the effectiveness and safety of pulsatile GnRH or hCG treatment in adolescent boys with hypogonadotropic hypogonadodism.Method: 12 patients received 8–10 μg of GnRH, subcutaneously injected every 90 min using a pump. Another 22 patients received hCG, ...

hrp0095p1-558 | Pituitary, Neuroendocrinology and Puberty | ESPE2022

Simultaneous quantification of 51 steroids in pediatric plasma and urine samples by LC–MS/MS

Wu Xiaoyu , Chen Yao , Li Xiaoqing , Xue Peng , Tang Jingyi , Yu Xiaodan , Zheng Liang , Wang Xiumin , Liu Shijian

Quantitative analysis of endogenous and exogenous steroid hormones is an important step for our understanding of the causes of endocrine disorders in pediatric. In this study, we developed a method to simultaneously quantify endogenous and exogenous steroids in pediatric urine and plasma. Ultra-performance liquid chromatography-tandem mass spectrometry (LC-MS/MS) was used to simultaneously quantify steroid hormones in children plasma and urine. Under the optimum conditions, th...

hrp0092p3-12 | Adrenals and HPA Axis | ESPE2019

Clinical Follow-Up of a Novel NR0B1 Mutation in a Case of Adrenal Hypoplasia Congenital

yang haihua , wei haiyan , Shen Linghua , Wang Huizhen , Chen Qiong , Chen Yongxing , Liu Xiaojing

We have examined a male child patient aged about 15 years old to confirm the diagnosis of adrenal hypoplasia congenital (AHC) at the Genetics in-patient department, Children's Hospital Affiliated to Zhengzhou University.When the child was 5 years and 9 months,he was diagnosed as abmormal phenotypic characteristics who had skin hyperpigmentation, penis enlargement, rapid growth over the past 2 years, along with fatigue, drowsiness, and pubic hair appearing for 2 months.Base...

hrp0089p1-p101 | Fat, Metabolism and Obesity P1 | ESPE2018

Circulating Mots-C Levels are Decreased in Obese Male Children and Adolescents and Associated with Insulin Resistance

Du Caiqi , Zhang Cai , Wu Wei , Liang Yan , Hou Ling , Wang Anru , Ning Qin , Luo Xiaoping

Background and aims: A novel bioactive peptide, mitochondrial-derived peptide (MOTS-c), has recently attracted attention as a potential prevention or therapeutic option for obesity and type 2 diabetes mellitus (T2DM). MOTS-c profiles have not yet been reported in human obesity and T2DM. We aimed to determine circulating MOTS-c levels in obesity and explore the association between MOTS-c levels and various metabolic parameters. Methods: In this case-control study, 40 obese chil...

hrp0097p1-553 | Pituitary, Neuroendocrinology and Puberty | ESPE2023

A 12-month, open-label, single-arm, phase 3 trial of the efficacy and safety of triptorelin 3-month formulation in Chinese children with central precocious puberty (CPP)

Luo Xiaoping , Zhang Cai , Yang Yu , Xu Xu , Cheng Xinran , Wei Haiyan , Wang Lanying , Huang Frank , Cabri Patrick

Background: CPP is commonly treated with triptorelin, a gonadotropin-releasing hormone (GnRH) analogue. It is available as 1-month and 3-month prolonged-release (PR) formulations, but only the former is approved for CPP in China. Overseas studies have proved the efficacy and safety of triptorelin 3-month PR formulation; this study evaluated efficacy and safety in Chinese children with CPP.Methods: In this 12-month, open-...

hrp0094p2-379 | Pituitary, neuroendocrinology and puberty | ESPE2021

An Observational, Retrospective Study to Evaluate Long Term Safety and Effectiveness of Leuprorelin in the Treatment of Central Precocious Puberty

Fu Junfen , Dong Guanping , Li Pin , Gong Yan , Yang Yu , Yang Li , Gu Wei , Yuan Xuewen , Luo Xiaoping , Hou Ling , Zhong Yan , You Cheng , Wang Winston , Guo Ziheng ,

Objectives: To describe safety and effectiveness of high (≥90-180 µg/kg) and low (<90-30 µg/kg) dose leuprorelin in treating central precocious puberty (CPP).Methods: In this observational, retrospective study, effectiveness was evaluated based on regression or no progression of Tanner staging as the primary outcomes. LH, FSH, estradiol or testosterone suppression, and decrease in bone age to chronolo...

hrp0094p2-324 | Growth and syndromes (to include Turner syndrome) | ESPE2021

A Patient with Turner Syndrome (45X/46XX) and Congenital Adrenal Hyperplasia: A Case Report and Literature Review

Wang Yirou ,

Background: The combination of Turner syndrome (TS) and congenital adrenal hyperplasia (CAH) caused by 21-hydroxylase deficiency is rare. These two diseases affect children’s growth and development through different endocrine hormone metabolic dysfunction.Case presentation: A patient suffering from ambiguous genitalia was referred to our hospital when she was 2 months old. Her length was 58 cm (+0.9 standard deviation, SD) and weigh...

hrp0097lb8 | Late Breaking | ESPE2023

Mutant MCM8 induces apoptosis and S Phase Arrest in Premature Ovarian Insufficiency

Fei Wang

Keywords: Primary ovarian insufficiency; Microsomal maintenance complex 8; Cell apoptosis; Cell cycle; PI3K/AKTBackground: Primary ovarian insufficiency (POI) with a chromosome karyotype of 46,XX in children, compared to adults, is difficult to diagnose and often seek medical attention due to delayed puberty or short stature. We have reported the two novel pathogenic mutations p.C242R and p.S445*of MCM8 gene in a pedigre...

hrp0082p2-d2-603 | Thyroid (1) | ESPE2014

Euthyroid Sick Syndrome in Children with Diabetic Ketoacidosis

Hu Yanyan , Li Guimei , Wang Qian , Wang Zengmin , Wang Fengxue

Background: The correlation between free thyroid hormones and poor diabetic control in children with diabetic ketoacidosis (DKA) and the effects of thyroid hormone therapy on euthyroid sick syndrome (ESS) remain unclear.Objective and hypotheses: To investigate characteristics of ESS in children with DKA and the effects of thyroid hormone therapy on ESS. In children with DKA, free thyroid hormones may be associated with the severity of DKA and thyroid hor...

hrp0095rfc4.4 | Fat, Metabolism and Obesity | ESPE2022

New insights of monogenic hypercholesterolemia in pediatric patients: basing on a Chinese cohort

Zhang Qianwen , Wang Xiumin

Background and objectives: Dyslipidemia especially hypercholesterolemia attracts much more attention recently. Given different management and treatment of multiple categories, precise diagnosis becomes increasingly important. However, studies in pediatric patients lacks around the world, especially in China. Consequently, this study was designed to confirm the molecular defect of persistent hypercholesterolemia with next-generation sequencing basing on a singl...