hrp0084p2-544 | Puberty | ESPE2015

Increasing BMI is Associated with Lower Luteinising Hormone Levels in Girls with Central Precocious Puberty at the Early Pubertal Stage

Lee Hae Sang , Kim Eun Young , Hwang Jin Soon

Background: Girls with precocious puberty have high LH levels and bone age was advanced over chronological age by 1 year. Obese children start puberty at an earlier age than non obese children. The mechanisms that cause obese children to grow faster starting from early childhood are not well defined.Objective and hypotheses: We analysed the effects of obesity on luteinising hormone (LH) secretion by using gonadotropin-releasing hormone (GnRH) tests in gi...

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...

hrp0097p1-316 | Growth and Syndromes | ESPE2023

The effects of androgen treatment on growth in patients with 5-α-Reductase type 2 deficiency

Kwon Ahreum , Kim Sujin , Seo Junghwan , in Lee Hae

Introduction: 5-α-Reductase type 2 deficiency (5αRD2) is 46,XY disorder of sex development that requires androgen treatment for normal male external genitalia development. Despite concerns regarding precocious puberty and stunted growth associated with androgen treatment, limited research has been conducted on its effects on individuals with 5αRD2. The present study aims to evaluate the impact of androgen treatment on bone age (BA) and height...

hrp0095p1-32 | Bone, Growth Plate and Mineral Metabolism | ESPE2022

Six-year clinical outcome of enzyme replacement therapy for patients with perinatal lethal and infantile hypophosphatasia in Korea

Yoon Cho Sung , Kim Min-Sun , Noh Eu-Seon , young Bae Ga , won Park Sung , Jin Dong-Kyu , Kim Insung

Hypophosphatasia (HPP) is a genetic skeletal disease caused by loss-of-function mutations of ALPL encoding tissue-nonspecific alkaline phosphatase (TNSALP). The clinical presentation of HPP varies greatly, ranging from stillbirth without bone mineralization to findings in later life, such as delayed walking, short stature, skeletal deformities, bone pain, and pathologic fractures. The diagnosis is based on clinical examination, radiographic findings, biochemical parameters of ...

hrp0092p2-158 | GH and IGFs | ESPE2019

Clinical and Genetic Characteristics of Eleven Korean Patients with Hypochondroplasia and Outcomes of Growth Hormone Therapy

Kim Min-Sun , Im Minji , Park Hyojung , Park Mi Jung , Kim Shin Hye , Cho Sung Yoon , Jin Dong-Kyu

Hypochondroplasia (HCH) is an autosomal dominant inherited skeletal dysplasia with abnormal growth pattern and inadequate pubertal growth spurt. Achondroplasia and HCH have many similar phenotype, however, HCH show the mildest phenotype among FGFR3-associated skeletal dysplasia, and the radiologic findings are usually so subtle. We investigated to evaluate clue for the hypochondroplasia, and clinical and genetic characteristics of eleven Korean patients with HCH. We a...

hrp0092p2-209 | Multisystem Endocrine Disorders | ESPE2019

Endocrine Complications of Patients with Hepatic Type of Glycogen Storage Disease

Lee Yena , Kim Yoo-Mi , Oh Arum , Kim Gu-Hwan , Hee Lee Beom , Choi Jin-Ho , Yoo Han-Wook

Background: Glycogen storage disease (GSD) is an inherited metabolic defect of metabolic defect of glycogenolysis and gluconeogenesis. Patients with GSDs are associated with endocrine abnormalities such as short stature, delayed puberty, fasting hypoglycemia, and dyslipidemia. In addition, patients with GSD 1b are also at risk of autoimmune hypothyroidism. Therefore, this study was performed to investigate endocrine complications in patients with GSD.<p cl...

hrp0092p2-218 | Pituitary, Neuroendocrinology and Puberty | ESPE2019

The Difference of Body Mass Index (BMI) Score Before and After Gonadotropin-Releasing Hormone Agonist (GnRHa) Treatment in Central Precocious Puberty Girls

Choi Yujung , Lee Seonhwa , Kim Seulki , Ahn Moonbae , Kim Shinhee , Cho Wonkyoung , Cho Kyoungsoon , Jung Minho , Suh Byungkyu

Purpose: We investigated the difference of body mass index (BMI) score before and after gonadotropin-releasing hormone agonist (GnRHa) treatment in central precocious puberty girls (CPP).Methods: Medical records of 188 girls with CPP treated with GnRHa were reviewed. All patients completed the therapy. The patients were categorized into two groups according to initial BMI; normal weight group (BMI < 85 percentile) an...

hrp0089p3-p248 | Growth &amp; Syndromes P3 | ESPE2018

Prediction of Response to Growth Hormone Treatment in Korean Girls with Turner Syndrome

Jung Mo Kyung , Kim Se Young , Lee Ji-Eun , Kim Hae Soon , Yu Jeesuk , Yoo Eun-Gyong

Purpose: Growth hormone (GH) treatment has become common practice in Turner syndrome (TS) to improve final adult height. However, there are only a few studies on the analysis of good responders to GH treatment in TS. The aim of this study is to predict the responsiveness to growth hormone therapy in Turner syndrome.Methods: Among 197 TS patients registered in LG Growth study, 92 patients were excluded because of systemic illness or hypothyroidism. The cl...

hrp0089p2-p366 | Sex Differentiation, Gonads and Gynaecology or Sex Endocrinology P2 | ESPE2018

Genetic Etiologies and Gender Outcomes of Patients with Disorders of Sex Development Presenting with Asymmetric Gonads

Oh Arum , Kim Yoon-Myung , Hun Seo Go , Kim Gu-Hwan , Choi Jin-Ho , Yoo Han-Wook

Purpose: Patients with mixed gonadal dysgenesis (MGD) and ovotesticular disorders of sex development (DSD) usually present with asymmetric gonads. Differential diagnosis of these conditions is based on karyotype and pathological findings of gonads. However, it is difficult to determine sex of rearing and to predict long-term outcomes. This study investigated the clinical features, karyotype, sex of rearing, and pubertal outcomes of patients with MGD and ovotesticular DSD.<...

hrp0089p2-p392 | Thyroid P2 | ESPE2018

Clinical Characteristics and Predictive Factors for the Detection of Thyroid Cancer in Children with Thyroid Nodules

Suh Junghwan , Choi Han Saem , Kwon Ah Reum , Chae Hyun Wook , Kim Duk Hee , Kim Ho-Seong

Thyroid nodules in children are less common than adults. However, pediatric thyroid nodules have higher rate of malignancy compared with adults, and also have increased risk of metastasis and recurrence. In this study, we analyzed clinical features, laboratory findings, and thyroid ultrasound (US) of children with thyroid nodules to determine predictive factors of thyroid cancer. Total 268 patients under 18 years of age with thyroid nodule whom visited Severance Children’...