Pediatric living donor liver transplantation for biliary embryonal rhabdomyosarcoma: a case report of a case showing disease-free survival over 2 years

남궁정만,황신,박길천,권현희,Suhyeon Ha,Oh Seak Hee,Kim Kyung Mo 대한이식학회 2022 Korean Journal of Transplantation Vol.36 No.2

Biliary rhabdomyosarcoma is a rare tumor, but it is still the most common tumor of the biliary tract in children. We report a case of a 6-year-old boy with biliary embryonal rhabdomyosarcoma and liver metastasis, which were treated with neoadjuvant and adjuvant chemotherapy combined with living donor liver transplantation (LDLT). Ini- tial imaging studies showed a low-attenuation intraductal mass from the left hepatic duct to the intrapancreatic common bile duct with diffuse upstream dilatation of the intrahepatic duct and liver metastasis. Endoscopic biopsy revealed embryonal rhab- domyosarcoma. After tumor size reduction through neoadjuvant chemotherapy, LDLT was planned to remove the tumor completely. A left lateral section graft weighing 330 g was harvested from his 38-year-old mother and the graft-to-recipient weight ratio was 1.94%. Routine pediatric LDLT operation was performed with deep excavation of intra- pancreatic distal bile duct. The explant liver showed minimal residual embryonal rhab- domyosarcoma with no lymph node metastasis. The patient recovered uneventfully from LDLT operation. Scheduled adjuvant chemotherapy was performed for 6 months. The patient is doing well without any evidence of tumor recurrence for 26 months after LDLT. In conclusion, liver transplantation could be an effective treatment for unresect- able biliary rhabdomyosarcoma in children according to the location of tumor.

외과전문의 자격 요건

남궁정만 대한외과학회 2016 대한외과학회 학술대회 초록집 Vol.2016 No.5

Pediatric split liver transplantation in a patient with biliary atresia polysplenia syndrome and agenesis of inferior vena cava

남궁정만,황신,김대연,하태용,Gi-Won Song,정동환,김경모,오석희 대한이식학회 2020 Korean Journal of Transplantation Vol.34 No.4

Biliary atresia (BA)-polysplenia syndrome (PS) is diagnosed in a small proportion of BA patients. We present a case of split liver transplantation (LT) successfully performed in a pediatric recipient with BA-PS. The recipient was 29-month-old boy who underwent Kasai procedure. The coexisting malformations included agenesis of the inferior vena cava with azygos vein continuation, polysplenia, intestinal malrotation, truncated pancreas, and preduodenal portal vein and annular pancreas. With pediatric end-stage liver disease score of 33, the patient was allocated for split LT. Under this condition, the left lateral section graft was equivalent to a graft-recipient weight ratio of 2.6%. The recipient surgery was performed according to the standard procedures of pediatric LT. The graft hepatic vein was directly anastomosed with the suprahepatic confluence of the recipient hepatic veins. An external iliac vein homograft was interposed for portal vein reconstruction. Portal collateral veins were embolized intraoperatively to secure portal vein inflow. No surgical complications were developed. Currently, the patient has been doing well for 4 years after transplantation. Our pediatric patient with BA-PS had various anatomical malformations. Thorough preoperative and intraoperative assessment of these anomalies, adoption of customized reconstruction techniques of LT, and careful posttransplant monitoring are necessary for successful LT.

체관 내 유두상 점액종양의 형태적 분류에 따른 외과적 치료의 고찰

남궁정만,문기명,김송철,안철수,김기훈,황신,박광민,이영주,한덕종,이승규 대한외과학회 2006 대한외과학회 학술대회 초록집 Vol.2006 No.5

Indications and outcomes of liver transplantation for post-Kasai biliary atresia in young adults

남궁정만,황신,안철수,문덕복,하태용,송기원,정동환,박길천,윤영인,김경모,이성규 대한이식학회 2021 Korean Journal of Transplantation Vol.35 No.3

Background: Some young adults have a long survival period with native liver after Kasai portoenterostomy (KPE) for biliary atresia (BA). However, a considerable proportion of these patients require liver transplantation (LT). This study aimed to analyze the indica- tions and outcomes of LT in young adults after a long survival period with native liver after KPE. Methods: We selected seven patients who were 18 years or older at the time of LT out of 116 BA patients who underwent primary LT from 2008 to 2019 at Asan Medical Center. Results: The mean ages at KPE and LT were 2.1±0.9 months and 22.0±5.1 years, respec- tively. Mean serum total bilirubin level and model for end-stage liver disease score at LT were 7.91±7.22 mg/dL and 15.3±6.0, respectively. The main reasons for LT were liver cirrhosis with portal hypertension-associated complications in five patients and intracta- ble cholangitis in two patients. There were five cases of living donor LT and two cases of deceased donor LT. All the seven patients are currently alive during the mean follow-up period of 74.7±40.9 months. One patient suffered from outflow graft vein obstruction re- quiring endovascular stenting. Another patient showed core antibody-positivity-induced de novo hepatitis B virus infection, which was well managed with antiviral therapy. Conclusions: Young adult patients with BA are a unique group of patients requiring spe- cialist care regarding transition from pediatric to adult services. The outcomes of LT in young adult BA patients were excellent. Therefore, LT should be considered in patients showing serious BA-associated complications.

Whole liver deceased donor liver transplantation for pediatric recipients: single-center experience for 20 years

남궁정만,황신,김대연,하태용,Gi-Won Song,정동환,Gil-Chun Park,김경모,오석희 대한이식학회 2020 Korean Journal of Transplantation Vol.34 No.4

Background: We investigated the incidence and outcomes of pediatric deceased donor liver transplantation (DDLT) using whole liver grafts in a high-volume liver transplantation (LT) center. Methods: The study was a retrospective single-center analysis of whole LT in pediatric recipients. The study period was set as 20 years between January 2000 and December 2019. We defined pediatric recipients and donors to be aged ≤18 years. Results: During the study period, there were 98 cases of pediatric DDLT, and 34 patients (34.7%) received whole liver grafts. The age range of the deceased donors was 3 months to 56 years and that of pediatric recipients was 7 months to 17 years. Common primary diseases for LT were biliary atresia in 13, acute liver failure in four, Wilson disease in four, congenital portal vein agenesis in three, and genetic metabolic diseases in three. Pediatric-to-pediatric and adult-to-pediatric whole LTs were 22 (64.7%) and 12 (35.3%), respectively. A good correlation was noted between the donor and the recipient’s body weight, and the recipient’s body weight and allograft’s weight. Graft and overall patient survival rates were 91.2% and 91.2% at 1 year, 88.0% and 88.0% at 3 years, and 88.0% and 88.0% at 5 years, respectively. Conclusions: The results of this study revealed that Korean Network for Organ Sharing (KONOS) regulations with donor-recipient body weight matching exhibited good performance. Considering the reciprocal trades of liver organs among pediatric and adult donors and recipients, it is necessary to establish a policy for pediatric donor liver grafts to pediatric recipients on a priority basis.

인터랙티브 스토리텔링을 활용한 서책형 현대시 교육의 디지털 교과서화

남궁정 국어교육학회 2011 국어교육연구 Vol.49 No.-

In today's knowledge and information society in which a tremendous amount of information is digitalized and provided, modern poetry education cannot be one exception. According to a recent announcement from the Ministry of Education, Science, and Technology, all the current paper textbooks will be replaced with digital ones in future. The advent of digital textbooks era is to meet the demands of the times and environment and involves a continuous evolution into new forms according to the development of science and technology. Getting prepared for those changes, researches on literary education should be able to offer future directions for digital textbooks with a focus on the learners' enjoyment of poetry and literary experiences. In that vein, this study set out to propose an idea of turning modern poetry education into a digital textbook by utilizing interactive storytelling, showing that digital textbooks could induce interest, commitment, and new effects with regard to modern poetry education and study. It then introduced successful cases of educational content that utilized interactive storytelling, pointed out problems with cyber home study, a digital teaching material, and examined the interactive elements required for turning modern poetry education into a digital textbook. In particular, the investigator drew a blueprint for modern poetry education applicable in a digital textbook by giving various examples that used a branch narrative structure. Digital textbooks can offer answers to how to teach poetry to learners in today's environment and accordingly should be developed in a way that helps to preserve the nature and heritage of poetry, allow the students to enjoy the truth and value of poetry, and achieve the educational objectives. It is beyond a doubt that digital textbooks will bring a positive turning point to modern poetry education.

생식내분비학 : 점막하 자궁근종 환자에서 자궁경하 근종절제술 시행 전 자궁동맥 색전술-multidisplinary approach

남궁정,곽세현,정윤지,유영옥,김장흡,천호종,김미란 대한산부인과학회 2012 대한산부인과학회 학술대회 Vol.98 No.-

크기가 큰 점막하 자궁근종 환자에서 자궁경하 근종절제술 시행 전에 자궁동맥 색전술을 시행하여 수술적 접근이 용이하였던 5명의 환자를 보고하고자 한다. 2009년 1월부터 2011년 6월까지 본원 산부인과에서 점막하자궁근종으로 진단받고 자궁동맥색전술 시행 후 자궁경하 근종제거술을 받은 5명의 환자를 대상으로 하였다. 대상 환자 5명 모두 월경과다 및 생리통을 호소하였으며 4명에서는 혈색소 8g/dl 미만의 빈혈을 보이고 있었다. 모든 환자에서 자궁 동맥색전술 시행 전 촬영한 MRI 및 골반초음파에서 점막하자궁근종이 관찰되었으며 근종의 부피는 평균 71.2cm3 이었으며 1명을 제외한 모든 환자에서 자궁동맥색전술 후 크기의 감소를 보였다. UAE 시술 후 자궁경하 근종제거술을 시행하였고 조직검사상 초자변성을 동반한 자궁근종으로 나왔다. 5명의 환자 모두 치료 후 월경과다의 증상이 호전되었으며 1년간의 추적관찰 중 재발의 소견은 없었다. 월경과다를 일으키는 크기가 큰 점막하자궁근종의 경우 자궁동맥색전술을 시행한 후 자궁경하 자궁근종 절제술 시행시 출혈 등의 합병증을 줄일 수 있으며 좀 더 안전하고 쉽게 접근할 수 있다.

인터랙티브 스토리텔링을 활용한 소설교육 e-Learning 콘텐츠 개발

남궁정 ( Nam Kung Jung ) 한국언어문화학회 2011 한국언어문화 Vol.0 No.44

Despite the latest changes to the digital media environment, there has been the absence of full-scale discussions about them in Korea`s novel education community. Upon the realization that the novel education community should take a priority in those discussions to respond to the needs of the times, this study set out to analyze the development of e-learning content for novel education and propose the applicability of novel education content to interactive storytelling. Interactive storytelling is a new type of narrative that combines the emotional and dramatic aspects of narrative with the interactivity of the computer. It allows the user to influence storytelling and thus alter the direction of the story`s development. There is a need to put interactive storytelling into active use in order to deliver information to the students effectively and induce active participation from them. Based on those discussions, Chapter 2 defined interactive storytelling and examined its educational significance. Chapter 3 examined the interactivity components of content offered in conjunction with the Korean subject, the utilization cases of interactivity in each e-learning content in the Korean subject to figure out the degree of the interactivity components being utilized, and the specific cases of e-learning content for novel education provided by Edunet to understand the degree of interactivity being utilized in the e-learning content currently available to the students. Chapter 4 pointed out the limitations and problems with the current e-learning content for novel education, proposed plans to improve them, and designed new e-learning content for novel education by employing the interactive storytelling structure proposed by Glassner. The application of interactive storytelling to novel education content enables the users to highlight the emotional and dramatic aspects of narrative, have diverse, colorful experiences and obtain knowledge according to their choices, and increase the level of immersion and fun they are having. That is why attention must be paid to interactive storytelling. If interactive storytelling is put to great use, it will allow for the development of more advanced e-learning systems and content than the ones currently provided by Edunet.

보문 : 월악산 소나무림의 낙엽생산과 낙엽을 통한 영양염류 이입량

남궁정 ( Jeong Namgung ),문형태 ( Hyeong Tae Mun ) 한국환경생물학회 2009 환경생물 : 환경생물학회지 Vol.27 No.3

월악산 국립공원 내의 소나무군락에서 2005년 5월부터 2008년 4월까지 3년 동안 월별 낙엽의 생산량과 낙엽을 통해 임상에 이입되는 영양염류의 양을 조사하였다. 2005, 2006, 2007년의 낙엽생산량은 각각 3.070, 3.066, 3.099 ton ha-1 yr-1이었으며, 3년 동안의 평균 낙엽생산량은 3.078±0.018 ton ha-1 yr-1이었다. 낙엽 구성원 중 침엽, 가지 및 수피, 생식기관 그리고 기타가 차지하는 비율은 각각 61.9, 10.4, 5.2, 22.5%으로 침엽이 차지하는 비율이 가장 컷다. 조사지소에서 3년 동안 낙엽을 통해 임상에 이입되는 질소, 인, 칼륨, 칼슘 그리고 마그네슘의 평균 양은 18.014, 0.878, 4.240, 7.349 그리고 2.172 kg ha-1 yr-1이었다. Litter production, nutrient concentration of each component of litterfall and amount of nutrients input to forest floor via litterfall were investigated for three years from May 2005 through April 2008 in Pinus densiflora forest at Mt. Worak National Park. Amount of litterfall in 2005, 2006, 2007 were 3.070, 3.066, 3.099 ton ha-1 yr-1, respectively. Average amount of litterfall for three years was 3.078±0.018 ton ha-1 yr-1. Average percentage of leaf litter, branch and bark, reproductive organ and the miscellaneous for three years were 61.9, 10.4, 5.2, 22.5%, respectively. Average amount of N, P, K, Ca and Mg returned to forest floor via litterfall for three years in this P. densiflora forest were 18.014, 0.878, 4.240, 7.349 and 2.172 kg ha-1 yr-1, respectively.