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.

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

외과전문의 자격 요건

남궁정만 대한외과학회 2016 대한외과학회 학술대회 초록집 Vol.2016 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.

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.

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.

대학안전문화 확산을 위한 사회적응 상담 및 심리지원 프로그램 : 전문대학생의 정규 수업 후 교내활동을 중심으로

남궁정(Nam Kung Jeong) 한국방과후학교학회 2017 방과후학교연구 Vol.4 No.1

이 글은 대학 안전문화 확산을 위한 사회적응 상담 및 심리지원 프로그램 운영에 관한 내용 및 결과에 대해 전문대학생의 정규 수업 후 교내활동의 한 모델을 제시하여 대학내에서의 교육의 지적 서비스에 대한 보완과 대학교육의 다양성과 질적 향상에 대한 연구이다. 안전사각지대에 놓여있는 대학 내 학생들에게 안전에 대한 중요성이 더욱 더 인지되고 있는 상황에서 학생들에게 안전 문화를 확산하고 이에 대한 심층적인 안전 의식을 고취하기 위해서 정규 수업 후 주로 교내에서 활동으로 먼저, 자살예방 프로그램을 진행하여 생명존중과 안전의식을 높이고 강화 하고자 하였다. 이 프로그램 수료 및 이수과정을 마친 학생들에게는 교내활동의 만족도 향상과 향후 학교안전 지도사 양성으로 발전 할 수 있는 가능성을 논의하였다. The following research article is based on a model that has resulted from social adjustment, counseling service and psychological support for the improvement of safety culture for university students’ after having a regular class schedule. This model was developed as a means to create a safe environment for them within a school where it is in a need of safety awareness and it is specifically designed for the school activities apart from regular classes. The main content of the program deals with raising awareness for suicide prevention, respect of life and safety consciousness. After that, the program suggests an improvement of safety culture within school while providing a relevant theory and practice for the program. The main reason for the program is aimed for a recent announcement for safety policy within education sector while considering timeliness and the appropriate action to be dealt with any other negative consequences arising from rapidly changing education environment.The program is not included in the regular school curriculum, but it was implemented to develop student’s personality, characteristics and to be connected with school-related activities to foster their academic success.In addition to that, it has been designed to enhance the qualitative service for post-secondary education and school activities of university students while it could have a potential to foresee a viability for students to be successful.

저마찰 인발유 및 인발공구 표면처리적용에 관한 연구

남궁정(J. Namkung),강희수(H. S. Kang),임광혁(K. H. Yim),박재현(J. H. Park),김정훈(J. H. Kim) 한국소성가공학회 2022 한국소성가공학회 학술대회 논문집 Vol.2022 No.12

생식내분비학 : 유전체 복제수 변이마커를 이용한 자궁내막증의 유전적소인연구 (초)

남궁정,김장흡,조현희,천근영,오은경,김진홍,신승훈,정연준,김미란 대한산부인과학회 2010 대한산부인과학회 학술대회 Vol.96 No.-

문학공간 답사를 통한 한국어 학습자의 문화교육 방안 연구

남궁정,김경훤 한국문화융합학회 2019 문화와 융합 Vol.41 No.2

The purpose of this study is to propose plans to promote cultural education for Korean learners through explorations of literary spaces. If learners connect literary spaces with the content of literary works, they will be able to experience Korean culture and the sentiments of the Korean people as well as understand the spaces and texts together. In other words, explorations of literary spaces will open the door to an advanced level of education in a way that surpasses simple experience- based field trips. Such explorations can also propose specific methods of practice to make literature a part of one’s life and self in that they substantialize fictional literary experiences through realistic spaces. The present study thus proposes a teaching and learning model for explorations of literary spaces. Stage 1, “understanding of literary works,” introduces the goals of the unit and consists of activities to encourage appreciation and understanding of literary works. Stage 2, “identification of literary spaces and surveying & planning,” involves identifying and surveying spaces in literary works and planning exploration schedules and activities for these literary spaces. Stage 3, “exploration of literary spaces,” offers opportunities to actually explore literary spaces, compare them with the spaces in literary works, and discover the culture of the target society in the literary spaces. Stage 4, “arrangement through criticism and discussion,” provides the following options for activities: to discuss the culture of the target society discovered in the literary spaces, criticize literary works freely based on what one felt or discovered in the literary spaces, write a travel essay about spaces based on literary works, and write a critical essay about literary works, thinking of their spaces. Cultural education for Korean learners through explorations of literary spaces holds significance in a range of areas in addition to Korean education. It can create opportunities to globally promote Korean authors, literary works, and literary. In addition, it can lead to the development of tourism content connected with literary works for Korean learners, and thus promote the vitalization of concerned areas.