hrp0097p1-445 | Fat, Metabolism and Obesity | ESPE2023

Impact of early-life overweight/obesity trajectory on insulin resistance at age 8: a prospective cohort study

Jin Lee Hye , Jeong Lee Yun , Lim Youn-Hee , Hong Yun-Chul , Ho Shin Choong , Ah Lee Young

Objective: To investigate whether the early-life overweight/obesity trajectory from ages 2, 4, to 8 affects insulin resistance (IR) in 8-year-old prepubertal children.Methods: From the Environment and Development of Children (EDC) cohort, 262 prepubertal children (147 boys and 115 girls) who visited Seoul National University Children’s Hospital for anthropometric measurements at ages 2, 4 and 8 were included. At ag...

hrp0097p1-338 | Multisystem Endocrine Disorders | ESPE2023

Long-term endocrine sequelae after hematopoietic stem cell transplantation in children and adolescents

Yoon Ji-Hee , Choi Yunha , Hwang Soojin , Lee Yena , Hye Kim Ja , Choi Jin-Ho , Yoo Han-Wook

Purpose: As the survival rate for pediatric cancers increases significantly with advances in treatment modalities, long-term endocrine complications have also risen. This study investigated the frequencies and risks of endocrine sequelae of childhood cancer survivors after hematopoietic stem cell transplantation (HSCT).Methods: This study included 200 pediatric patients who underwent HSCT. Clinical and endocrinological f...

hrp0097p2-96 | Sex Differentiation, Gonads and Gynaecology, and Sex Endocrinology | ESPE2023

Clinical outcomes and genotype-phenotype correlations in patients with complete and partial androgen insensitivity syndromes

Yoon Ji-Hee , Lee Nae-Yun , Hye Kim Ja , Hwang Soojin , Kim Gu-Hwan , Yoo Han-Wook , Choi Jin-Ho

Purpose: Androgen insensitivity syndrome (AIS) is a rare X-linked recessive disorder caused by unresponsiveness to androgen because of mutations in the AR gene. Here, we investigated the clinical outcomes and molecular spectrum of AR variants in patients with AIS attending a single academic center.Methods: This study included 19 patients with AIS who were confirmed by molecular analysis of AR. Clinical features and endoc...

hrp0092p3-145 | GH and IGFs | ESPE2019

Final Adult Height of Children with Idiopathic Short Stature: A Multicenter Study on GH Therapy Alone Started During Peripuberty

Wu Di , Chen Rui-min , Chen Shao-ke , Liu Ge-li , Chen Lin-qi , Yang Yu , Wang Xin-li , Peng Ya-guang , Gong Chun-xiu

Purpose: To evaluate the efficacy of GH in improving FAH in ISS children in a multicenter study.Methods: A real-world observation was carried out. Children with ISS in seven hospitals in China were enrolled. The height gain standard deviation score and the height gain over the target height were evaluated.Results: There were 344 ISS patients (217 boys and 127 girls). The baseline a...

hrp0089p2-p338 | Sex Differentiation, Gonads and Gynaecology or Sex Endocrinology P2 | ESPE2018

Physical Assessment in Chinese Children with 5a-reductase Type 2 Deficiency

Zhao Xiu , Song Yanning , Chen Shaoke , Wang Xiumin , Luo Feihong , Yang Yu , Chen Linqi , Chen Ruimin , Chen Hui , Su Zhe , Wu Di , Gong Chunxiu

Background: 5α - reductase type 2 deficiency (5αRD) is an autosomal recessive hereditary disease which has an incidence of 11.2%–15.5% among population with 46, XY DSD (disorders of sex development).Objective: To study the growth pattern in Chinese pediatric patients with 5αRD.Subjects: Data were from 187 patients with 5αRD (age from 0–16 years old,with homozygous or compound heterozygous mutations in ...

hrp0089lb-p14 | Late Breaking P1 | ESPE2018

Beta-cell Function in Chinese Youngsters with Type 1 Diabetes and Assessment of Surrogate Markers of Severe Insulin Deficiency

Yuan Jinna , Derraik Jose G B , Fu Junfen , Dong Guanping , Cutfield Wayne S , Wu Wei , Huang Ke , Jiang Youjun , Chen Xiaochun

Objective: We assessed whether beta-cell function progressively decreases over time with greater type 1 diabetes mellitus (T1DM) duration using a mixed-meal tolerance test (MMTT). We also assessed simpler and more practical surrogate parameters for clinical use.Methods: We studied 57 children and adolescents with T1DM in Hangzhou (China), mean age at diagnosis was 8.3 years (range 2.3 to 15.3 years), with an average diabetes duration of 2.5 years (range ...

hrp0095p1-257 | Diabetes and Insulin | ESPE2022

A novel mutation in INS gene in an infant with neonatal diabetes mellitus: A case report and functional study

Suh Junghwan , Jin Kim Su , In Lee Hae , Lee Myeongseob , Song Kyungchul , Saem Choi Han , Kwon Ahreum , Wook Chae Hyun , Kim Ho-Seong

Neonatal diabetes mellitus (NDM) is a hyperglycemic status usually diagnosed before first 6 months of life, which is caused by monogenic mutations. INS gene mutation is the second most common cause of permanent NDM, causing misfolding of proinsulin and accumulation in the endoplasmic reticulum, leading to apoptosis of the pancreatic beta cells. We report a case of NDM in a 2-month-old girl with a novel heterozygous mutation of the INS gene, and functional studies to confirm th...

hrp0095p1-498 | GH and IGFs | ESPE2022

A Long-Acting Growth Hormone Module For Paediatric Growth Hormone Deficiency In The Global Registry For Novel Therapies In Rare Bone & Endocrine Conditions – The GloBE-Reg LAGH Study

Miller Bradley , Savendahl Lars , Hickman Krystina , Smythe Christopher , Chen Ching , Choi Jin-Ho , Dou Xinyu , Gong Chunxiu , Hamza Rasha , Horikawa Reiko , Jorge Alexander , Faisal Ahmed S.

Introduction: Post-marketing surveillance registries provided extensive information about the safety and efficacy of daily growth hormone (GH) therapy during treatment. With the availability of novel long-acting GH (LAGH) therapies, it is important to determine whether the novel molecules or the different pattern of GH exposure lead to changes in the efficacy and safety profile. Therefore, new surveillance registries of LAGH are warranted.<p class="abstext...

hrp0095p1-499 | GH and IGFs | ESPE2022

Long-term safety and effectiveness of recombinant human growth Hormone inKorean pediatric patients with growth disorders: 9-year interim analysis fromLG Growth Study

Kim Yoo-Mi , Chung Sochung , Rhie Young-Jun , Hyun Kim Jae , Chae Hyun-Wook , Ho Choi Jin , Ah Lee Young , Sang Lee Hae , Tae Hwang Il

This study aimed to evaluate the long-term safety and effectiveness of recombinant human growth hormone (rhGH; Eutropin®, Eutropin®Pen, Eutropin®AQ, and Eutropin®Plus, LG Chem, Ltd.) treatment in Korean pediatric patients. This observational study has been conducted since 2011, and the data were collected up to August 2021 for interim analysis. The incidence rates of all adverse events (AEs) were assessed for safety a...

hrp0092lb-23 | Late Breaking Posters | ESPE2019

Long-Term Safety and Effectiveness of Recombinant Human Growth Hormone in Korean Pediatric Patients with Growth Disorders: 7-year Interim Analysis from LG Growth Study

Ah Lee Young , Chung Sochung , Rhie Young-Jun , Hyun Kim Jae , Chae Hyun-Wook , Yoo Jae-ho , Ho Choi Jin , Tae Hwang Il

Objectives: This study aimed to evaluate the long-term safety and effectiveness of recombinant human growth hormone (rhGH) (Eutropin® Inj.., Eutropin®Pen Inj., Eutropin®AQ Inj., and Eutropin®Plus Inj., LG Chem, Ltd.) based on the interim analysis of a 7-year accumulated data of the LG Growth Study (LGS) in Korean pediatric patients with growth disorders including growth hormone deficiency (GHD), T...