Shinyoung Jang, Su Jin Kim, Myeongseob Lee, Hae In Lee, Ahreum Kwon, Junghwan Suh, Kyungchul Song, Hyun Wook Chae, Ho-Seong Kim
{"title":"Comparison of the effect of gonadotropin-releasing hormone agonist dosage in girls with central precocious puberty.","authors":"Shinyoung Jang, Su Jin Kim, Myeongseob Lee, Hae In Lee, Ahreum Kwon, Junghwan Suh, Kyungchul Song, Hyun Wook Chae, Ho-Seong Kim","doi":"10.6065/apem.2244210.105","DOIUrl":"10.6065/apem.2244210.105","url":null,"abstract":"<p><strong>Purpose: </strong>There are no definite guidelines on the optimal dosage of gonadotropin-releasing hormone (GnRH) agonist for treatment of central precocious puberty (CPP). We compared growth outcomes of GnRH agonist at different dosages in girls with idiopathic CPP to assess the optimal dosage.</p><p><strong>Methods: </strong>This retrospective study included 86 girls with idiopathic CPP who had been treated with GnRH agonist for at least one year and had attained their final adult height. Leuprolide was given as fixed dosage (3.75 mg every 4 weeks in body weight >20 kg, n=72) or weight-based dosage (60-85 μg/kg every 4 weeks, n=14). We compared suppression of advanced puberty and treatment response between the 2 groups.</p><p><strong>Results: </strong>Peak estradiol and luteinizing hormone and bone age (BA)/chronological age after injection of GnRH agonist were effectively suppressed in both groups. In both groups, the height standard deviation score (SDS) for BA increased after treatment. Final adult height (FAH) (fixed dosage group,160.8±4.1 cm and weight-based dosage group, 161.2±4.4 cm) was significantly higher than the initial predicted adult height (PAH) (155.5±3.3 and 156.1±3.6 cm, respectively) (both P<0.001) and similar to midparental height (159.8±3.3 and 160.6±3.7 cm, respectively). There were no differences in gain in height SDS for BA and gain in height (FAH-PAH at the start) between the 2 groups.</p><p><strong>Conclusion: </strong>There were no differences in treatment outcome between fixed dosage (3.75 mg/4 wk) and weight-based dosage (60-85 μg/kg/4wk) of GnRH agonist. Therefore, a fixed dosage of GnRH agonist can be used more conveniently for CPP treatment without growth oversuppression.</p>","PeriodicalId":44915,"journal":{"name":"Annals of Pediatric Endocrinology & Metabolism","volume":" ","pages":"283-288"},"PeriodicalIF":2.8,"publicationDate":"2023-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10765025/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9238295","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Kyujung Park, Kyung In Lim, Young Bae Sohn, Hae Sang Lee, Jin Soon Hwang
{"title":"Using low-dose octreotide with diazoxide-resistant congenital hyperinsulinism resulting from compound heterozygous mutations in the ABCC8 gene.","authors":"Kyujung Park, Kyung In Lim, Young Bae Sohn, Hae Sang Lee, Jin Soon Hwang","doi":"10.6065/apem.2244068.034","DOIUrl":"10.6065/apem.2244068.034","url":null,"abstract":"","PeriodicalId":44915,"journal":{"name":"Annals of Pediatric Endocrinology & Metabolism","volume":" ","pages":"S23-S24"},"PeriodicalIF":2.8,"publicationDate":"2023-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10783923/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40578825","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Commentary on \"Effects of once-weekly dulaglutide on juvenile type 2 diabetes mellitus and obesity in Korea: a pilot study\".","authors":"Han Hyuk Lim","doi":"10.6065/apem.2322098edi010","DOIUrl":"10.6065/apem.2322098edi010","url":null,"abstract":"","PeriodicalId":44915,"journal":{"name":"Annals of Pediatric Endocrinology & Metabolism","volume":"28 4","pages":"235-236"},"PeriodicalIF":2.8,"publicationDate":"2023-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10765026/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139088908","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"A novel compound variant in GNRHR causing congenital idiopathic hypogonadotropic hypogonadism in a young male Korean patient.","authors":"Gimin Lee, Mi Seon Lee, Rosie Lee, Jung Eun Moon","doi":"10.6065/apem.2244070.035","DOIUrl":"10.6065/apem.2244070.035","url":null,"abstract":"","PeriodicalId":44915,"journal":{"name":"Annals of Pediatric Endocrinology & Metabolism","volume":" ","pages":"S20-S22"},"PeriodicalIF":2.8,"publicationDate":"2023-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10783930/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"40490284","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ha Young Jo, Hyun Ji Jang, Young Mi Kim, Soo-Han Choi, Kyung Hee Park, Hye Won Yoo, Su Jeong Park, Yoon Hee Jo, Min Jung Kwak
{"title":"Turner syndrome due to Xp22.33 deletion combined with 7p22.3 duplication.","authors":"Ha Young Jo, Hyun Ji Jang, Young Mi Kim, Soo-Han Choi, Kyung Hee Park, Hye Won Yoo, Su Jeong Park, Yoon Hee Jo, Min Jung Kwak","doi":"10.6065/apem.2244122.061","DOIUrl":"10.6065/apem.2244122.061","url":null,"abstract":"","PeriodicalId":44915,"journal":{"name":"Annals of Pediatric Endocrinology & Metabolism","volume":" ","pages":"S14-S16"},"PeriodicalIF":2.8,"publicationDate":"2023-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10783925/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9195127","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Na Won Lee, Ji Eun Jeong, Young Hwan Kim, Chang Seok Ki, Jin Kyung Kim
{"title":"A case of type A insulin resistance syndrome in a 14-year-old adolescent girl without common clinical features.","authors":"Na Won Lee, Ji Eun Jeong, Young Hwan Kim, Chang Seok Ki, Jin Kyung Kim","doi":"10.6065/apem.2244106.053","DOIUrl":"10.6065/apem.2244106.053","url":null,"abstract":"","PeriodicalId":44915,"journal":{"name":"Annals of Pediatric Endocrinology & Metabolism","volume":" ","pages":"S17-S19"},"PeriodicalIF":2.8,"publicationDate":"2023-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10783927/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9195131","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Commentary on \"Glycemic control and complications of type 2 diabetes mellitus in children and adolescents during the COVID-19 outbreak\".","authors":"Chong Kun Cheon","doi":"10.6065/apem.2322107edi009","DOIUrl":"10.6065/apem.2322107edi009","url":null,"abstract":"","PeriodicalId":44915,"journal":{"name":"Annals of Pediatric Endocrinology & Metabolism","volume":"28 4","pages":"233-234"},"PeriodicalIF":2.8,"publicationDate":"2023-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10765030/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139088909","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Kyeong Eun Oh, Yu Jin Kim, Ye Rim Oh, Eungu Kang, Hyo-Kyoung Nam, Young-Jun Rhie, Kee-Hyoung Lee
{"title":"Glycemic control and complications of type 2 diabetes mellitus in children and adolescents during the COVID-19 outbreak.","authors":"Kyeong Eun Oh, Yu Jin Kim, Ye Rim Oh, Eungu Kang, Hyo-Kyoung Nam, Young-Jun Rhie, Kee-Hyoung Lee","doi":"10.6065/apem.2244214.107","DOIUrl":"10.6065/apem.2244214.107","url":null,"abstract":"<p><strong>Purpose: </strong>This study aimed to investigate the impact of coronavirus disease 2019 (COVID-19) on type 2 diabetes mellitus (T2DM) in children and adolescents.</p><p><strong>Methods: </strong>Children and adolescents diagnosed with T2DM who visited the Korea University Hospital in 2019 and 2020 were retrospectively analyzed, including changes in body mass index (BMI)-standard deviation score (SDS), glycated hemoglobin (HbA1c), diabetes complications, and diabetes management from 2019 to 2020.</p><p><strong>Results: </strong>Patient mean age and disease duration were 15.48±2.15 and 2.56±1.51 years, respectively. Obese patients accounted for 70.6% of the study population. From 2019 to 2020, mean BMI-SDS (2.21±1.25 vs. 2.35±1.43, P=0.044), HbA1c level (6.5%±2.72% vs. 7.3%±3.70%, P<0.001), blood pressure (BP), total cholesterol, and non-high-density lipoprotein cholesterol level in all patients increased significantly. Obesity was an independent predictor of increased HbA1c (95% confidence interval, 1.071-50.384; P=0.042). HbA1c levels did not increase significantly in nonobese patients, whereas HbA1c (6.45%±2.30% vs. 7.20%±3.05%, P<0.001), BMI-SDS (2.88±0.75 vs. 3.08±0.98, P=0.045), diastolic BP (P=0.037), and total cholesterol values (P=0.019) increased in obese patients in 2020 compared to 2019.</p><p><strong>Conclusion: </strong>During the COVID-19 outbreak, glycemic control and diabetic complications worsened in children and adolescents with T2DM, particularly in obese patients. Close monitoring for glycemic control and diabetic complications is necessary in children and adolescents with T2DM, especially those with obesity.</p>","PeriodicalId":44915,"journal":{"name":"Annals of Pediatric Endocrinology & Metabolism","volume":"28 4","pages":"275-282"},"PeriodicalIF":2.8,"publicationDate":"2023-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10765022/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143774569","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Da Hye Lee, Sung-Chan Kang, Seung-Sik Hwang, Yun Jeong Lee, Hwa Young Kim, Seong Yong Lee, Choong Ho Shin, Jaehyun Kim
{"title":"Establishing reference values for percentage of appendicular skeletal muscle mass and their association with metabolic syndrome in Korean adolescents.","authors":"Da Hye Lee, Sung-Chan Kang, Seung-Sik Hwang, Yun Jeong Lee, Hwa Young Kim, Seong Yong Lee, Choong Ho Shin, Jaehyun Kim","doi":"10.6065/apem.2244268.134","DOIUrl":"10.6065/apem.2244268.134","url":null,"abstract":"<p><strong>Purpose: </strong>The association between appendicular skeletal muscle mass (ASM) and cardiometabolic risk has been emphasized. We estimated reference values of the percentage of ASM (PASM) and investigated their association with metabolic syndrome (MS) in Korean adolescents.</p><p><strong>Methods: </strong>Data from the Korea National Health and Nutrition Examination Survey performed between 2009 and 2011 were used. Tables and graphs of reference PASM were generated using 1,522 subjects, 807 of whom were boys aged 10 to 18. The relationship between PASM and each component of MS in adolescents was further analyzed in 1,174 subjects, 613 of whom were boys. Moreover, the pediatric simple MS score (PsiMS), the homeostasis model assessment of insulin resistance (HOMA-IR), and the triglyceride-glucose (TyG) index were analyzed. Multivariate linear and logistic regressions adjusting for age, sex, household income, and daily energy intake were performed.</p><p><strong>Results: </strong>In boys, PASM increased with age; the trend was different in girls, in whom PASM declined with age. PsiMS, HOMA-IR, and TyG index showed inverse associations with PASM (PsiMS, β=-0.105, P<0.001; HOMA-IR, β=-0.104, P<0.001; and TyG index, β=-0.013, P<0.001). PASM z-score was negatively associated with obesity (adjusted odds ratio [aOR], 0.22; 95% CI, 0.17-0.30), abdominal obesity (aOR, 0.27; 95% CI, 0.20-0.36), hypertension (aOR, 0.65; 95% CI, 0.52-0.80), and elevated triglycerides (aOR, 0.67; 95% CI, 0.56-0.79).</p><p><strong>Conclusion: </strong>The probability of acquiring MS and insulin resistance decreased as PASM values increased. The reference range may offer clinicians information to aid in the effective management of patients. We urge clinicians to monitor body composition using standard reference databases.</p>","PeriodicalId":44915,"journal":{"name":"Annals of Pediatric Endocrinology & Metabolism","volume":" ","pages":"237-244"},"PeriodicalIF":2.8,"publicationDate":"2023-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10765024/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9405266","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"A Korean male with Kleefstra syndrome presented with micropenis.","authors":"Rosie Lee, Mi-Seon Lee, Jung Eun Moon","doi":"10.6065/apem.2244174.087","DOIUrl":"10.6065/apem.2244174.087","url":null,"abstract":"<p><p>Kleefstra syndrome is caused by chromosome 9q34.3 deletion or heterozygous mutations in the euchromatin histone methyl transferase 1 (EHMT1) gene. It can be accompanied by intellectual disability, distinctive facial features, microcephaly, psychiatric disorders, hypotonia in childhood, hearing loss, heart defects, renal defects, epilepsy, speech anomalies, and obesity. Furthermore, genital anomalies are present in 30%-40% of male patients with Kleefstra syndrome, but their mechanisms have not been elucidated. Herein, we report a patient with Kleefstra syndrome presenting with micropenis. The patient was transferred to Kyungpook National University Children's Hospital for management of imperforate anus on the day of birth. Physical examination revealed micropenis with stretched penile length of 0.9 cm and facial dysmorphisms, including hypertelorism and anteverted nares. Chromosomal microarray revealed 424-kb heterozygous deletion at chromosome 9q34.3 (arr[hg19] 9q34.3 (140,234,315-140,659,055)x1). Among the involved main OMIM genes, phenotypically relevant genes were EHMT1 and NSMF. Endocrinological investigation showed low basal gonadotropin and testosterone levels. Anterior pituitary hormones and steroid hormone levels were in the normal range. Testicular function was normal based on human chorionic gonadotropin stimulation test. The patient experienced improvement in penile length growth with intramuscular testosterone enanthate injection initiated at 4 months of age. The purpose of this study is to describe the etiology, endocrine laboratory tests, and treatment of micropenis in Kleefstra syndrome.</p>","PeriodicalId":44915,"journal":{"name":"Annals of Pediatric Endocrinology & Metabolism","volume":"28 4","pages":"308-311"},"PeriodicalIF":2.8,"publicationDate":"2023-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10765021/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139088905","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}