hrp0089p3-p406 | Growth & Syndromes P3 | ESPE2018

Two Siblings with Prader-Willi Syndrome Caused by Microdeletion Derived from the Paternal Grandmother

Dong Guanping , Dai Yangli , Huang Ke , Zou Chaochun , Chen Xuefeng

Prader-Willi syndrome (PWS) is a complex neurobehavioral disorder characterized by infantile significant hypotonia and feeding difficulties, followed by morbid obesity secondary to hyperphagia, short stature, functionally deficient gonads, intellectual disabilities and behavioral problems. It is caused by lack of expression of imprinting genes on the paternally inherited chromosome 15q11.2-q13 region. The genetic mechanism responsible for Prader-Willi syndrome can rarely be in...

hrp0086p2-p784 | Pituitary and Neuroendocrinology P2 | ESPE2016

Body Mass Index and Body Fat Composition are Both Related to Central Precocious Puberty in Chinese Girls

Huang Ke , Fu Jun-fen , Dong Guan-ping

Background: Obesity in children and adolescents has become an increasing clinical and public health concern. It was reported that obesity is positively associated with sexual maturation in both boys and girls in China. However, the effect of obesity on pubertal development is incompletely elucidated.Objective and hypotheses: To evaluate the fat mass and body composition by dual-energy x-ray absorptiometry (DEXA) in Chinese precocious puberty girls, and t...

hrp0082p1-d2-117 | Fat Metabolism & Obesity (1) | ESPE2014

Ratio of Triglycerides to High-Density Lipoprotein Cholesterol Compared with Homeostasis Model Assessment Insulin Resistance Indexes in Screening the Metabolic Syndrome in Chinese Obese Children

Liang Jianfeng , Fu Junfen , Wang Jinling , Jiang Youjun , Dong Guanping

Background: IDF-2007 MS definition (MS-IDF2007) for children and adolescents has been challenged frequently in clinical practice and should be improved further. Therefore, it becomes imminent to explore an accessible and effective tool to screen obese children with the metabolic syndrome components.Objective and hypotheses: To investigate the optimal cut-off values and compare the accuracy for the triglycerides and HDL cholesterol ratio and homeostasis m...

hrp0082p2-d1-258 | Adrenals & HP Axis | ESPE2014

ACTH and Cortisol Levels are Associated with Cardiovascular Risk in Pediatric Obesity: a Cross-Sectional Study in China

Liang Jianfeng , Fu Junfen , Wang Jinling , Jiang Youjun , Dong Guanping

Background: The hypothalamic–pituitary–adrenal (HPA) axis, and cortisol in particular, has been reported to be involved in obesity-associated metabolic disturbances in adults and in specific populations of adolescents.Objective and hypotheses: To investigate the associations between serum ACTH and cortisol levels and cardiovascular risk factors in obese children and adolescents.Method: Of 1119 obese children and adolescen...

hrp0084p3-1076 | Hypo | ESPE2015

Hyperinsulinism Secondary to Congenital Portosystemic Shunt in a Neonate

Hong Yong Hee , Lee Dong Hoon , Kim Sung Shin

Background: Hyperinsulinism is a common cause of persistent hypoglycaemia in infant. Insulin secretion from pancreatic β-cells is unregulated and inappropriate for the level of blood glucose, causing glucose into the insulin-sensitive tissues, such as the muscle, liver and adipose tissue, leading to hyperinsulinaemic hypoglycaemia. But congenital portosystemic shunt, a rare vascular malformation, can cause hyperinsulinaemic hypoglycaemia rarely because glucose from portal...

hrp0084p3-1241 | Turner | ESPE2015

Renal Problems in Early Adult Patients with Turner Syndrome

Chung Woo Yeong , Oh Seung Hwan , Yu Dong Uk

Introduction: The prevalence of renal anomalies in Turner syndrome (TS) has been reported to vary from 30 to 70%. However, the influence of renal anomalies on renal function and morbidity have been less well investigated. We evaluate the status of renal function and the presence of urinary abnormalities in early adult TS patients.Patients and method: Sixty-three girls with TS, who are attending Pediatric Endocrine Clinics in Busan Paik Hospital, were stu...

hrp0097p1-354 | Pituitary, Neuroendocrinology and Puberty | ESPE2023

The effect of leuprolide acetate 11.25mg 3-month formulation in children with central precocious puberty: A systematic review and meta-analysis

Wu Wei , Huang Ke , Dong Guanping , Fu Junfen

Background: Central precocious puberty (CPP) results from the premature pubertal activation of the hypothalamic-pituitary-gonadal axis before eight years for girls or nine years for boys. A systematic review and meta-analysis was conducted to evaluate the long-term efficacy and safety of leuprolide acetate (LA) 11.25mg 3-month formulation in children with CPP.Methods: A systematic search in PubMed and Embase was conducte...

hrp0092p3-24 | Adrenals and HPA Axis | ESPE2019

Clinical Characteristics and Genetic Analysis in one Patient with Congenital Lipoid Adrenal Hyperplasia

Xi Li , Chang Zhuo , Pei Zhou , Li Xiaojing , Luo Feihong

Objective: To explore the clinical and molecular genetic characteristics of congenital lipoid adrenal hyperplasia (CLAH), and to sequence the acute regulatory protein (steroid acute regulatory, StAR) gene of the infant patient and her pedigree.Methods: Physical examination, laboratory tests, and imaging examination of the 1-month- old patient with CLAH were collected. DNA was extracted from blood samples of the patient a...

hrp0086fc9.4 | Pathophysiology of Disorders of Insulin Secretion | ESPE2016

The Protective Effects of Adenovirus-mediated IL-10 Gene and Anti-CD20 Monoclonal Antibody on the Pancreatic β Cells of NOD Mice in the Early Stage of Natural T1D Onset

Li Tang , Li Cheng , Fan Lei , Tian Fei , Tang Aiping

Background: Type 1 diabetes (T1D) is an autoimmune disease (AID) whose primary features include progressive pancreatic β cell damage and absolute insufficient endogenous insulin secretion. Recent studies have shown that a Th1/Th2 cell subset unbalance and excessively activated B lymphocytes are important pathogenic mechanisms.Objective and hypotheses: To investigate the protective effects of Adenovirus-mediated IL-10 gene and anti-CD20 monoclonal an...

hrp0082p1-d1-67 | Diabetes | ESPE2014

Protective Effects of Combined Intervention with Adenovirus Vector Mediated il10 and igf1 Genes on Endogenous Islet β Cells in Nonobese Diabetes Mice with Onset of Type 1 Diabetes Mellitus

Li Tang , Zhang Lijuan , Chen Yanyan , Li Cheng , Lin Xiaojie

Introduction: To investigate the protective effects of combined intervention with adenovirus vector mediated interleukin 10 (IL10) and IGF1 genes on islet β cells in nonobese diabetes (NOD) mice with type 1 diabetes mellitus (T1D) at early stage.Methods: Twenty-four female NOD mice at onset of diabetes and aged 17–20 weeks old were randomly divided into four groups. Mouse 1, 2, and 3 groups were i.p. injected 0.1 ml of Ad-mIGF1, Ad-mIL10, and c...