Growth Hormone Therapy in Children with Prader-Willi Syndrome

Im, Minji Association for Research of MPS and Rare Diseases 2021 Journal of mucopolysaccharidosis and rare disease Vol.5 No.1

Prader-Willi syndrome is a complicated genetic disorder caused by a mutation on chromosome 15q11-13. The disease results in morbid obesity due to hyperphagia, growth disturbance, multiple endocrine problems from hypopituitarism, developmental delay, and cognitive or behavioral problems. Recombinant human growth hormone has been used to improve body composition and muscle mass, which plays a main role in treating patients with Prader-Willi syndrome. We describe previous studies showing the efficacy and safety of growth hormone treatment in children with Prader-Willi syndrome and provide treatment guidelines. Growth hormone therapy could be beneficial for children with Prader-Willi syndrome and improve their quality of life.

Growth hormone treatment for children with mucopolysaccharidosis I or II

Minji Im,Chiwoo Kim,Juyoung Sung,Insung Kim,Ji-Hoon Hwang,Min-Sun Kim,Sung Yoon Cho 대한의학유전학회 2023 대한의학유전학회지 Vol.20 No.2

Purpose: Despite enzyme replacement therapy (ERT) and/or allogeneic hematopoietic stem cell transplantation, individuals with mucopolysaccharidosis (MPS) I or II often experience significant growth deficiencies. This study aimed to assess the safety and efficacy of recombinant human growth hormone (hGH) treatment in children diagnosed with MPS I or II. Materials and Methods: A total of nine pediatric patients—four with MPS I and five with MPS II—underwent treatment with ERT and hGH at Samsung Medical Center. Results: The mean hGH dose administered was 0.26±0.03 mg/kg/week. In the MPS I group, three patients showed an increase in height Z-score from -4.09±0.83 to -3.68±0.43 after 1 year of hGH treatment, and to -3.10±0.72 by the end of the hGH regimen. In the MPS II group, while the height Z-score of four patients decreased according to standard growth charts, it improved from 1.61±1.79 to 2.71±1.68 based on the disease-specific growth chart through hGH treatment. Two patients discontinued hGH treatment due to lack of efficacy after 22 and 6 months each of treatment, respectively. No new-onset neurological symptoms or necessity for prosthetic or orthopedic surgery were reported during hGH treatment. Conclusion: This study provides insights into the impact of hGH on MPS patients, demonstrating its potential to reverse growth deceleration in some cases. Further research is needed to explore the long-term effects of hGH on changes in body composition, muscle strength, and bone health in this population.

Wilson disease diagnosed incidentally by targeted gene panel sequencing in a Korean boy with severe obesity

Im Minji,Song Ari,Kim Jiyeon,Kim Min-Sun,Lee Sae-Mi,Kim Mi Jin,Cho Sung Yoon,Jin Dong-Kyu 대한소아내분비학회 2022 Annals of Pediatirc Endocrinology & Metabolism Vol.27 No.3

Wilson disease (WD) is a relatively common genetic hepatic disease in children and is characterized by excessive copper accumulation, predominantly in the liver and brain. It is an autosomal recessive disease caused by an ATP7B mutation that causes brain degeneration and is potentially fatal if diagnosed late or untreated. In the early phase of WD, its initial presentation may include mild hepatic involvement. WD may be overlooked as a cause of liver disease due to severe obesity but should not be excluded from differential diagnosis. We report a case of WD with severe obesity and fatty liver diagnosed in the early phase by targeted gene panel sequencing and review the endocrine problems associated with WD. Early suspicion of WD is important for good prognosis.

Growth Hormone Therapy in PWS with Mobile Application-Living with PWS

Im, Minji Association for Research of MPS and Rare Diseases 2018 Journal of mucopolysaccharidosis and rare disease Vol.4 No.2

Prader-Willi syndrome (PWS) is a multisystemic complex disorder characterized by hyperphagia and impaired satiety which lead to severe and early obesity. In infancy, hypotonia and poor suck are main problems, and a child goes through Failure-to-thrive. During childhood, clinical manifestations change to food seeking as well as excessive weight gain, short stature, developmental delay, cognitive disability and behavioral problems. Also, growth hormone insufficiency is frequent. Most patients receive the recombinant growth hormone (rGH) therapy that provides improvement in growth, body composition, and physical attributes. The clinical care guideline for rGH therapy in PWS had been noticed in 2013. The rGH therapy helps in body fat, lean body mass, height SDS and head circumference. Also, the rGH therapy helps motor function, psychomotor development and cognition and behavioral issues.In Samsung medical center, there are clinical care guidelines for rGH therapy in PWS and an useful application for the patients. 'Living with PWS', the name of an moblie application for PWS patients, was introduced in the lecture. The application revised to version 2. It was made more convenient to users than in version 1. It helps caregivers to schedule the rGH therapy and to monitor height and weight.

Identification the DNA Polymorphism of Volvariella volvacea Genetic Resources by using Various RAPD Primers

Minji Oh,Youn-Lee Oh,Ji-Hoon Im,Kab-Yeul Jang,Won-Sik Kong 한국버섯학회 2017 버섯 Vol.21 No.2

Volvariella volvacea is mainly cultivated in subtropics area like South-East Asia. Because it is cultivated in rice straws, it is called a straw mushroom. That mushroom grows well in high temperature about 30~38°C and high humidity. Straw mushroom is a homothallic mushroom, so it is difficult to identify whether the offspring is different from the parents. This study was carried out to investigate RAPD primers that can be used for identification the DNA polymorphism of Volvariella volvacea genetic resources. 9 strains were collected from various countries like China, Vietnam etc. When ITS regions of their DNA were analyzed, they proved to be Volvariella volvacea. A cultivation test was conducted to measure the morphological characteristics of them 2 strains, KMCC04380 and KMCC04382 were selected for breeding resources because the mycelium of them grew well on medium and fruiting bodies were formed quickly. The Universal PCR Fingerprinting kits(UPF primers) were used to confirm the genetic polymorphisms of the 2 strains. As a result of confirming the DNA bands, 2 of 12 primers could be used to genetically distinguish 2 strains. About 50 spores were isolated from their fruiting bodies respectively and they also will be confirmed DNA polymorphisms by using UPF primers.

느타리 재배에 적용 가능한 최적 액체종균 선발 및 생산성 시험

Minji Oh,Ji-Hoon Im,Youn-Lee Oh,Jegadeesh Raman,Kab-Yeul Jang 한국버섯학회 2020 버섯 Vol.24 No.1

국내 버섯 생산량은 2018년 기준 약 16만 톤으로, 국내 버섯 주요 품목은 느타리, 큰느타리, 팽이, 양송이, 표고이며 이 중 큰느타리와 팽이는 대부분 액체종균을 사용하여 종균 생산부터 버섯 수확까지 자동화된 생산시스템으로 대량생산되고 있다. 생산량의 약 24%를 차지하는 느타리는 액체종균을 사용한 재배가 이전부터 몇 차례 시도되어 왔으나, 균덩이 현상 등 다양한 원인으로 아직까지 실용화되지 못하고 있다. 향후 수출이나 유통 등에 적합하며 완성형 배지에도 적용 가능한 종균을 개발하기 위해서 느타리 재배에 적용 가능한 최적 액체종균 배지를 선발하고 생산성을 시험하였다. 기존 팽이, 큰느타리에 사용하는 대두박배지 이외에 BSMY, YM, ME, PMP, MCM, PDB, 대두박 변형 액체배지를 제조하여 느타리 ‘솔타리’, ‘흑타리’ 품종의 원균을 접종하여 정치배양과 진탕배양으로 균사를 7일 간 배양하였다. 그 결과, 모든 배지에서 정치배양보다 진탕배양에서 균사 생장이 우수하였고, 배양된 균체량과 집락형성단위(CFU)는 BSMY, YM, MCM 배지에서 우수하였다. 선발된 액체종균을 활용하여 병재배를 통한 생산성을 시험하였고, 균사배양은 YM 배지의 종균 접종 시 우수하였고, MCM 배지의 종균을 접종 시 생육일수가 6일로 단축되었고, 수확량 또한 기존 대두박배지 액체종균보다 약 4% 증수되었다. 향후 병재배뿐만 아니라 봉지재배, 균상재배에서도 생산성을 검증하여 액체종균이 느타리 재배에서도 보급 및 활용될 수 있기를 기대해 본다.

의료데이터에서의 클래스 불균형 문제 해소를 위한 성향점수매칭의 활용

노민지(Minji Roh),주기훈(Gihun Joo),임현승(Hyeonseung Im),장재원(Jaewon Jang) Korean Institute of Information Scientists and Eng 2023 한국정보과학회 학술발표논문집 Vol.2023 No.12

대학생 학업소진 관련 국내 연구에 대한 통합적 문헌 고찰

박민지(Park, Minji),김수임(Kim, Soo-Im) 학습자중심교과교육학회 2021 학습자중심교과교육연구 Vol.21 No.22

목적 본 연구의 목적은 통합적 문헌고찰 방법을 통해 대학생의 학업소진과 관련된 변인들을 통합적으로 분석함으로써 변인들을 분류하고 변인 간 관계의 구조를 파악하는 데 있다. 방법 이를 위해 1차로 ‘대학생’, ‘소진’을 검색어로 118편의 논문을 검색하고 논문의 제목과 초록을 각각 검토하여 대학생 학업소진과 관련 없는 연구나 중복 논문을 삭제한 후 최종 17편의 분석 대상 논문을 선정하였다. 분석항목으로 연구대상 및 연구목적, 학업소진 측정도구, 학업소진 관련 변인 및 주요 연구결과 등을 확인하였다. 결과 대학생들의 학년별 분포는 개별 연구로는 차이가 있으나 전체적으로는 고른 편이었으며, 측정도구는 MBI-SS가 가장 많이 사용되었다. 학업소진을 매개변인이나 독립변인으로 설정한 연구 각 1편, 3편 외에 대부분의 연구는 학업소진을 종속변인으로 하여 학업소진에 미치는 관련 변인의 영향을 확인하고자 하였다. 연구자들의 합의과정을 통해 관련 변인들을 개인특성 차원, 진로⋅학습 차원, 관계 차원으로 분류하고 이들 관계의 구조를 도출하였다. 결론 학업소진 측정도구인 MBI-SS를 국내 대학생 대상으로 타당화하는 것이 필요하며, 대학생의 학업소진을 이해하기 위해서는 학업, 개인특성, 진로, 관계 등 관련 변인의 구조를 종합적으로 고려하는 것이 필요하다. Objectives The purpose of this study is to classify the variables and to understand the structure of the relationship between the variables by analyzing the variables related to academic burnout of college students through an integrated literature review method. Methods For this purpose, 118 papers were searched for as keywords ‘college student’ and ‘burnout’. After reviewing the title and abstract of the thesis and deleting any research not related to academic burnout or duplicate thesis, a total of 17 target articles were selected and analyzed As analysis items, research subjects, research objectives, academic burnout measurement tools, academic burnout-related variables, major research results, and factors affecting academic burnout were identified. Results Although the distribution of college students by grade was differed, they were generally similar, and MBI-SS was the most used measurement tool. In addition to one and three studies in which academic burnout was set as a mediator or independent variable, most of the studies used academic burnout as a dependent variable to determine the effect of related variables on academic burnout. Through the consensus process of the researchers, the related variables were classified into the individual characteristics dimension, the career/learning dimension, and the relationship dimension, and the structure of these relationships was derived. Conclusions In measuring academic burnout, it is necessary to validate MBI-SS for domestic college students. In order to understand the academic burnout of college students, it is necessary to comprehensively consider not only academic levels but also personal characteristics, careers, and relationships.

Breeding on new white and gold superior strains of Flammulina velutipes

Ji-Hoon Im,Youn-Lee Oh,Minji Oh,Kab-Yeul Jang,Won-sik Kong 한국버섯학회 2017 버섯 Vol.21 No.2

Flammulina velutipes belonging to white rot fungi is one of the commercially important edible mushrooms and is produced in large quantities due to the introduction of a automated and mechanized cultivation system in Korea. Despite the chief item of export among edible mushrooms, Flammulina velutipes has the lowest distribution rate of domestic cultivar, estimated that about 20 percent. As the result that most white cultivars of Flammulina velutipes produced and exported in Korea were introduced from Japan, farmers pay a large amount of royalties. Therefore, we try to develop a new pure domestic cultivars as a substitute for Japanese cultivars. To breed both white and gold superior strains, we selected the crossing mother groups including 10 white strains of ASI 4198 etc. and 7 brown strains of ASI 4049 etc. and mated each of the 17 strains by mon-mon hybridization. 19 white and 14 brown strains were chosen through two selection experiment over 2014 2016. In the third selection experiment this year, we finally selected one white(Fv 16 c 37) and the other gold(Fv 15 a 31) strain. Two selected strains were cultivated in the same environmental conditions. Spawn running period on the sawdust substrate required 30days at 20°C. The cultivation period and optimum temperature were 12±1 days at 14°C for primordia formation, 5 days at 4°C for inhibition phase, and 14±1 days at 7°C for fruiting body development. The length of pilei and stipes in two selected strains and Megumi as a control Japanese cultivar harvested in optimal stage was as follows: 10.5±0.81mm and 139.7±4.23mm in Fv 16 c 37, 10.8±0.43mm and 128..2±7.31mm in Fv 15 a 31, and 10.9±0.41mm and 141.8±4.64mm in Megumi respectively. The Yield of Fv 16 c 37, Fv 15 a 31 and Megumi was 271.2±11.84g, 237.7±9.05g and 270.7±16.87g per 1100ml in bottle cultivation.

PⅢ-5 Morphological characteristics of a new Flammulina veluptipes variety ‘Baeke’

Ji-Hoon Im,Youn-Lee Oh,Minji Oh,Seul-ki Lee,Kab-Yeul Jang,Won-sik Kong 한국버섯학회 2018 버섯 Vol.22 No.1