hrp0097p1-421 | Bone, Growth Plate and Mineral Metabolism | ESPE2023

Bisphosphonate, sirolimus, atenolol treatment in a 4-year old child diagnosed with Gorham-Stout disease

Jin Park Su , Yeun Sim Soo , Suh Byung-Kyu , Bae Ahn Moon

Gorham-Stout disease(GSD), also known as vanishing bone disease is an extremely rare skeletal disorder characterized by idiopathic intraosseous proliferation of lymphatic vascular structures resulting in progressive resorption of bone. Herein, we report a case of a 4-year-old girl with GSD treated with the combination treatment with bisphosphonate, sirolimus, and atenolol. A 4-year-old girl presented with prolonged back pain for 2 weeks. The thoracolumbar spine radiography rev...

hrp0097p1-32 | Diabetes and Insulin | ESPE2023

Continuous Glucose Monitoring: A possible aid to detect hypoglycemia event during insulin challenge tests

Yeun Sim Soo , Jin Park Su , Bae Ahn Moon , Suh Byung-Kyu

Combined pituitary function test is a dynamic function test used to evaluate the anterior pituitary gland in patients suspected with hypopituitarism. The test comprises insulin challenge test where intravenous insulin injection in order to induce symptomatic hypoglycemia (serum blood glucose <40mg/dL). Insufficient increase in growth hormone and cortisol after the stimulation confirms the diagnosis of growth hormone deficiency and/or adrenal function insufficiency. However,...

hrp0097p1-463 | Fat, Metabolism and Obesity | ESPE2023

Association between lean mass and metabolic syndrome risk in Korean children and adolescents

Suk Shim Young , Sang Lee Hae , Rim Jeong Hwal , Sub Lim Jung , Soon Hwang Jin

Introduction: Skeletal muscle plays a crucial role in glucose disposal, and studies have shown a positive relationship between muscle mass and insulin sensitivity. However, an elevated lean mass has also been associated with metabolically adverse outcomes. This study aimed to evaluate the association between the risk of metabolic syndrome (MetS) and lean mass using dual-energy X-ray absorptiometry (DXA) in a nationally representative sample from the Korea Nati...

hrp0097p1-498 | GH and IGFs | ESPE2023

The relationship between body mass index and therapeutic effect in children with idiopathic growth hormone deficiency

Sang Lee Hae , Suk Shim Young , Soon Hwang Jin , Sub Lim Jung

Purpose: The aim of this study is to investigate the influence of body mass index (BMI) on therapeutic effect in prepubertal children with idiopathic growth hormone deficiency (IGHD).Methods: We conducted a retrospective study by chart review in a single center. A total of 138 patients (male n=90, female n=48) with idiopathic growth hormone deficiency who were was treated growth hormone for at least 2 y...

hrp0097p1-353 | Pituitary, Neuroendocrinology and Puberty | ESPE2023

Clinical features of hyperprolactinemia in children and adolescents

Sang Lee Hae , Suk Shim Young , Sub Lim Jung , Soon Hwang Jin

Background/Purpose: Hyperprolactinemia is a rare endocrine disorder in childhood and there are limited etiological, clinical, and demographic data. The purpose of this study was to evaluate the clinical features and course of hyperprolactinemia in childhood and adolescents and to help diagnose and plan the management.Methods: In this single-center retrospective study included 21 patients with hyperprolactinemia from Ajou...

hrp0095p1-463 | Fat, Metabolism and Obesity | ESPE2022

Clinical characteristics of sitosterolemic children with xanthomas as the first manifestation

Zhang Jun , Chen Qiu-li , Guo Song , Li Yan-hong , Li Chuan , Zheng Ru-jiang , Ma Hua-mei

Background: Sitosterolemia (STSL) is an extremely rare genetic disease. Xanthomas as the first symptom is frequently misinterpreted as familial hypercholesterolemia (FH) in children. Inappropriate treatment may deteriorate the condition.Objectives: The goal of this study was to summarize the clinical characteristics of children with STSL who had xanthomas as their first symptom and to provide clues for early clinical dia...

hrp0095p1-123 | Growth and Syndromes | ESPE2022

Single center analysis of the characteristics of 24-hour ambulatory blood pressure and related factors in patients with Turner syndrome

Zheng Rujiang , Chen Hongshan , Huang Huiling , Ma Huamei , Li Yanhong , Chen Qiuli , Zhang Jun , Guo Song , Wang Bing , Du Minlian

Background and Objectives: In the patients with Turner syndrome (TS), the risk of hypertension is higher in childhood and adulthood. The aim of the study was to retrospectively analyze 24-hour ambulatory blood pressure monitoring (ABPM) in children and adolescents with TS and its related factors.Materials and Methods: A retrospective study was conducted involving TS patients admitted to our pediatric endocrine specialist...

hrp0095p2-233 | Pituitary, Neuroendocrinology and Puberty | ESPE2022

Central precocious puberty in a girl with vasculitis-related moyamoya syndrome

Xie Dandan , Chen Qiuli , Ma Huamei , Jiang Bo , Li Yanhong , Zhang Jun , Guo Song , Zheng Rujiang , Chen Zhixin

Object: To raise the awareness of clinical manifestations about vasculitis-related moyamoya syndrome by summarizing the clinical characteristics of a girl with central precocious puberty, vasculitis-related moyamoya syndrome and reviewing the literature. Method: We carried on the review analysis to the patient with central precocious puberty, vasculitis-related moyamoya syndrome and reviewed the literature. Result: The bilateral breast development was the prim...

hrp0092p3-241 | Sex Differentiation, Gonads and Gynaecology or Sex Endocrinology | ESPE2019

46XY, DSD with Hemolytic Uremic Syndrome as the Primary Manifestation——Denys–Drash Syndrome Caused by WT1 Gene Mutation

zhang jun , guo song , chen qiuli , ma huamei , li yanhong , chen hongshan , du minlian , cheng cheng , ye minyi

Objective: To summarize the diagnosis and treatment of a rare 46XY DSD cause: Denys-Drash syndrome.Methods: To summarize the clinical manifestations, laboratory tests, diagnosis and treatment of a rare 46XY DSD cause presenting with hemolytic uremic syndrome: Denys-Drash syndrome (WT1 mutation).Results: Female, 2 years and 4 months, were admitted to the hospital at 2018-11-7 becaus...

hrp0086p1-p459 | Fat Metabolism and Obesity P1 | ESPE2016

Erythropoietin Activates Classical Brown Adipose Tissue Through the Erythropoietin Receptor/STAT3 Pathway, Improving Obesity and Glucose Homeostasis in High Fat Diet-induced Obese Mice

Kodo Kazuki , Nakajima Hisakazu , Sugimoto Satoru , Itoh Ikuyo , Syota Fukuhara , Shigehara Keiichi , Nishikawa Taichiro , Mori Jun , Kosaka Kitaro , Hosoi Hajime

Background, aims and objectives: We hypothesized that classical brown adipose tissue (cBAT) could play a crucial role in the anti-obesity effects of erythropoietin (EPO). Our study highlights the mechanism in which EPO treatments could upregulate energy expenditure and improve glucose homeostasis through cBAT in obese mice fed with a high-fat diet (HFD).Method: C57BL/6J mice had been fed with HFD since the age of 4 weeks (HFD mice). We administered recom...