hrp0092p1-101 | Pituitary, Neuroendocrinology and Puberty | ESPE2019

Identification of Novel Mutations in FGFR1 and Functional Characteristics in Patients with Isolated Gonadotropin-Releasing Hormone Deficiency

Lee Yena , Huh Juyoung , Oh Arum , Kim Gu-Hwan , Yoo Han-Wook , Choi Jin-Ho

Background: Isolated gonadotropin-releasing hormone (GnRH) deficiency (IGD) is caused by a deficiency in GnRH production, secretion or action and a highly heterogeneous disorder with wide phenotypic spectrum including Kallmann syndrome (KS) with anosmia and normosmic idiopathic hypogonadotropic hypogonadism (nIHH). More than 30 different causative genes have been identified in several studies. FGFR1 mutations have been identified in about 3–10&#3...

hrp0086p2-p309 | Diabetes P2 | ESPE2016

Clinical Characteristics of Latent Autoimmune Diabetes in Youth (Type 1.5 DM)

Lee Seung Ho , Huh Seung , Yu Jeesuk

Background: Diabetes mellitus (DM) in childhood was mostly type 1 DM (T1DM), but sometimes it is not easy to classify, especially in the case having both type 2 clinical phenotype and autoantibody positivity. It is named as latent autoimmune diabetes in youth or type 1.5 DM (T1.5DM).Objective and hypotheses: This study was designed to evaluate the clinical characteristics of T1.5DM who had autoantibody positivity with clinical phenotype of T2DM.<p cl...

hrp0097p2-222 | Growth and Syndromes | ESPE2023

Height control using estradiol valerate considering chronological and bone age in patient with Marfan Syndrome

Kim Insung , Hwang JiHoon , Sung Juyoung , Eun Kim Sung , Lee Dong-Yun , Kim Min-Sun , Yoon Cho Sung , Jin Dong-Kyu , Seok Choi Doo

Introduction: Estrogen treatment can be used for pediatric patients with Marfan syndrome who wish to control the rate of excessive height growth. However, the appropriate timing of treatment initiation is controversial and studies were limited. In this study, the authors aimed to find out when the initiation of estradiol therapy is most appropriate for controlling height growth rate in patients with Marfan syndrome.Methods:</stro...

hrp0092p1-268 | Sex Differentiation, Gonads and Gynaecology or Sex Endocrinology (1) | ESPE2019

Idiopathic Scoliosis in Girls with Central Precocious Puberty: Incidence and Effect of Gonadotropin-Releasing Hormone Agonists

Chung Lindsey Yoojin , Nam Hyo-Kyoung , Rhie Young-Jun , Huh Rimm , Lee Kee-Hyoung

Adolescent idiopathic scoliosis (AIS) is the most common form of scoliosis, affecting approximately 2 to 4 percent of adolescents. AIS by definition occurs in children between 10 to 18 years old, in periods of growth spurts and puberty changes. In patients with central precocious puberty (CPP), generally, growth spurts start earlier than their peers. Therefore, AIS in patients with CPP is expected to develop earlier in juvenile age. Especially in girls, both AIS and CPP are mo...

hrp0092lb-15 | Late Breaking Posters | ESPE2019

Efficacy of 3-Monthly Compare to Monthly Depot GnRH Agonist (Triptorelin Pamoate) in the Treatment of Girls with Central Precocious Puberty in Korea

Yoojin Chung Lindsey , Huh Rimm , Nam Hyo-Kyoung , Rhie Young-Jun , Lee Kee-Hyoung

Background: Triptorelin depot is largely used to treat central precocious puberty (CPP) in children and currently 3 monthly depot was introduced. No Korean data are available on 3-monthly GnRH agonist treatment in central precocious puberty.Aim: To compare the efficacy of Triptorelin 11.25mg 3-months depot with 3.75mg monthly depot in suppressing pubertal development from the beginning to 6 month and 1 year after the tre...

hrp0092p2-283 | Thyroid | ESPE2019

Changes of Thyroid Function in Girls with Central Precocious Puberty After 6-Month GnRH Agonist Treatment

Huh Rimm , Yoojin Chung Lindsey , Nam Hyo-Kyoung , Rhie Young-Jun , Lee Kee-Hyoung

Objectives: In our previous study, serum thyroid stimulating hormone (TSH) level in the central precocious puberty (CPP) group was higher than that of the non-CPP group. Serum free thyroxine (fT4) level in the CPP group was notably lower than that of the non-CPP group. And it was also showed that age and peak luteinizing hormone (LH) were independent predictors of serum TSH concentration. Elevated TSH in girls with CPP is supposed to be associated with puberta...

hrp0092p3-123 | Fat, Metabolism and Obesity | ESPE2019

Validity of Non-High-Density Lipoprotein Cholesterol for Detecting Dyslipidemia Among Korean Adolescents

Nam Hyo-Kyoung , Yoojin Lindsey , Huh Rimm , Rhie Young-Jun , Lee Kee-Hyoung

Objectives: Non-high-density lipoprotein (HDL) cholesterol is an alternative method to assess dyslipidemia and is not required fasting. We aimed to assess the validity of non-HDL cholesterol for detecting dyslipidemia among Korean adolescents.Subjects and Methods: Data for 6,989 adolescents aged 10 to 19 years (3,684 boys and 3,305 girls), were obtained from the Korean National Health and Nutrition Examination Surveys du...

hrp0084p1-154 | Miscelleaneous | ESPE2015

Novel Compound Heterozygous BMP1 Variants Associated with Osteogenesis Imperfecta

Yang Aram , Huh Rimm , Kim Jinsup , Cho Sung Yoon , Jin Dong-Kyu

Background: Osteogenesis imperfecta (OI) includes a group of disorders with a susceptibility to bone fractures, the presentation ranging from slightly increased fracture frequency to death in the perinatal period.Objective and hypotheses: Autosomal-dominant inheritance with type I collagen biosynthesis defects is the most common, but many autosomal-recessive genes have been previously reported.Method: Whole-exome sequencing was per...

hrp0084p2-450 | Growth | ESPE2015

Pharmacokinetics and Efficacy of a Long-Acting Human GH with Fc Fusion Protein

Kim Sujin , Jin Dong-Kyu , Cho Sungyoon , Huh Rimm , Kim Jinsup , Yang Aram , Kwak Hyunhee

Background: Recombinant human GH (rhGH) therapy requires daily s.c. injections, this inconvenient treatment regimen results to poor compliance of the patient. Thus, to improve patient compliance, long-acting rhGH products including various protein fusion techniques have been in development during past 15 years.Objective and hypotheses: In this study, we describe the pharmacokinetics and efficacy of a novel long-acting GH using Fc fusion protein (rhGH-Fc)...

hrp0084p3-1132 | Puberty | ESPE2015

Effects of GnRH Agonists and Antagonists on Danazol-Induced Precocious Puberty Rat

Yang Aram , Huh Rimm , Kim Jinsup , Cho Sung Yoon , Jin Dong-Kyu

Background: GnRH agonists are a common treatment modality for patients with central precocious puberty.Objective and hypotheses: Danazol-induced precocious puberty rats were used as an animal model to compare the effects of GnRH analogues and to assess combinations of treatment with agonistic and antagonistic GnRH analogues.Method: A 5-day-old female Sprague–Dawley rats were subcutaneously injected with a single dose of 300 &#...