hrp0092p2-184 | Growth and Syndromes (to include Turner Syndrome) | ESPE2019

Effect of Gonadotropin-Releasing Hormone Agonists on Auxological Outcomes of Korean Boys with Central Precocious Puberty and Early Puberty

Lee Hae Sang , Sohn Young Bae , Kum Chang Dae , Hwang Jin Soon , Lim Jung Sub

Objective: To determine the effect of gonadotropin-releasing hormone agonist (GnRHa) treatment on auxological outcome of Korean boys with central precocious puberty (CPP) and early puberty (EP).Methods: This study included 11 boys with CPP and 8 boys with EP who were treated with GnRHa for at least 2 years at the Pediatric Endocrine Unit of Ajou University Hospital from March 2003 to December 2015. All nineteen boys atta...

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...

hrp0086p1-p741 | Pituitary and Neuroendocrinology P1 | ESPE2016

Serum Anti-Mullerian Hormone Levels in Precocious Puberty Girls According to the Timing of GnRH Agonist Treatment

Nam Hyo-Kyoung , Kim Hye Ryun , Rhie Young Jun , Lee Kee-Hyoung

Background: Few studies have investigated the long-term effects of gonadotropin releasing hormone (GnRH) agonist treatment on reproductive function.Objective and hypotheses: We assessed ovarian function by analyzing serum anti-mullerian hormone (AMH) levels in central precocious puberty (CPP) girls according to GnRH agonist-treatment timing.Method: Our study included 505 CPP girls subdivided into five groups based on the timing of ...

hrp0086p2-p838 | Syndromes: Mechanisms and Management P2 | ESPE2016

A Novel Fibrillin-1 Gene Mutation Leading to Marfan Syndrome in Korean Girl

Nam Hyo-Kyoung , Nam Myung-Hyun , Rhie Young-Jun , Lee Kee-Hyoung

Background: Marfan syndrome is one of the most common over-growth conditions and the cardinal features occur in ocular, skeletal and cardiovascular systems. Clinical variation is common and signs are age-dependent.Case: A 9-year-old girl was referred to our pediatric endocrinology clinic for tall stature. Physical examination revealed a lens dislocation with strabismus, high palate, positive wrist and thumb signs, joint hypermobility, and pes plenus. Dil...

hrp0082p1-d2-212 | Reproduction (1) | ESPE2014

A Novel MKRN3 Mutation Discovered in a Korean Girl with Central Precocious Puberty

Sang Lee Hae , Sub Lim Jung , Soon Hwang Jin , Young Kim Eun

Context: It has recently been shown that mutations of MKRN3, the gene encoding makorin RING-finger protein 3, lead to central precocious puberty (CPP). The aim of this study was to investigate mutations of the MKRN3 gene in Korean girls with CPP.Methods: Two hundred and sixty Korean girls with idiopathic CPP were included in this study. Auxological and endocrine parameters were measured. The entire MKRN3 gene was directly seque...

hrp0082p2-d2-577 | Sex Development (1) | ESPE2014

A Novel Cyp19a1 Gene Mutation Identified in Three Turkish Families

Akcurin Sema , Durmaz Erdem , Kim Woo-Young , Turkkahraman Doga , Shin Joe-Gook , Lee Su-Jun

The CYP19A1 gene product cP450aromatase enzyme is responsible for estrogen synthesis and androgen/estrogen equilibrium in many tissues; placenta and gonads are being the leading tissues. cP450aromatase deficiency has important effects on clinical phenotype due to excessive amount of androgen accumulation and insufficient estrogen synthesis in the pre- and postnatal periods. We identified a new point mutation in the CYP19A1 gene causing aromatase deficiency in three Turkish fam...

hrp0082p3-d3-789 | Fat Metabolism & Obesity (2) | ESPE2014

Bone Age Advancement in Prepubertal Children with Overweight and Obesity

Lee Hae Sang , Lim Jung Sub , Hwang Jin Soon , Kim Eun Young

Objective: Obesity is associated with bone age (BA) advancement of unclear etiology. In animal study, insulin may directly modulate skeletal growth. Our objective was to investigate the association with BA maturation and insulin levels in children with overweight and obesity.Methods: In this cross-sectional study of 42 prepubertal children, anthropometric data and hormonal values during oral glucose tolerance test were measured. Subjects were divided int...

hrp0082p3-d1-881 | Perinatal and Neonatal Endocrinology | ESPE2014

A Case of Hyperinsulinism/Hyperammonemia Syndrome

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

Background: Hyperinsulinism/hyperammonemia (HI/HA) syndrome is a form of congenital hyperinsulinism (CHI) caused by a mutation in the GLUD1 gene. It is characterized by hyperinsulinemic hypoglycemia accompanying hyperammonemia.Objective and hypotheses: We report HI/HA syndrome with a 4-month-old male who hypoglycemic seizure.Method: A 4-month-old male infant presented with seizure caused by fasting-induced hypoglycemia. At the time...

hrp0082p3-d3-947 | Puberty and Neuroendocrinology (1) | ESPE2014

The Etiology of Central Precocious Puberty and Effect of GnRH Agonist for 2 years in Korean Boys

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

Background: GnRH agonist (GnRHa) are able to modify natural course of Central Precocious Puberty (CPP) in girls.Objective and hypotheses: We evaluated the etiology and the effect of GnRHa in Korean CPP boys.Method: Total 29 boys diagnosed for CPP from 2007 to 2012 were included in Ajou University Medical Center. Sellar MRI was performed in 26 of 29 patients (89.7%). CPP was diagnosed on the basis of i) onset evidence of testicular ...