• Journal of Chest Surgery
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• 제55권4호
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• pp.313-318
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• 2022

Fewer patients undergo pediatric lung transplantation (PLT) than adult lung transplantation. Size mismatch is the key factor that limits the availability of potential donors. Every candidate for PLT is in a different scenario in terms of age, height and weight, size of structures, indications for PLT, the concomitant presence of a cardiac anomaly, and other individual-specific factors; thus, a thorough understanding of pediatric patients' medical problems is essential. Living-donor lobar lung transplantation (LDLLT) has only been performed once in Korea to date. However, since each step in the LDLLT is a well-established procedure, including intrapericardial lobectomy, lung procurement, and lobar lung transplantation, qualified surgeons and lung transplantation teams are competent to perform LDLLT in clinically necessary situations.

• Clinical and Experimental Pediatrics
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• 제64권2호
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• pp.49-59
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• 2021

Since the initial International Society of Heart Lung Transplantation registry was published in 1982, the number of pediatric heart transplantations has increased markedly, reaching a steady state of 500-550 transplantation annually and occupying up to 10% of total heart transplantations. Heart transplantation is considered an established therapeutic option for patients with end-stage heart disease. The long-term outcomes of pediatric heart transplantations were comparable to those of adults. Issues affecting long-term outcomes include acute cellular rejection, antibody-mediated rejection, cardiac allograft vasculopathy, infection, prolonged renal dysfunction, and malignancies such as posttransplant lymphoproliferative disorder. This article focuses on medical issues before pediatric heart transplantation, according to the Korean Network of Organ Sharing registry and as well as major problems such as graft rejection and cardiac allograft vasculopathy. To reduce graft failure rate and improve long-term outcomes, meticulous monitoring for rejection and medication compliance are also important, especially in adolescents.

• Clinical and Experimental Pediatrics
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• 제58권12호
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• pp.459-465
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• 2015

Postinfectious bronchiolitis obliterans (PIBO) is an irreversible obstructive lung disease characterized by subepithelial inflammation and fibrotic narrowing of the bronchioles after lower respiratory tract infection during childhood, especially early childhood. Although diagnosis of PIBO should be confirmed by histopathology, it is generally based on history and clinical findings. Irreversible airway obstruction is demonstrated by decreased forced expiratory volume in 1 second with an absent bronchodilator response, and by mosaic perfusion, air trapping, and/or bronchiectasis on computed tomography images. However, lung function tests using spirometry are not feasible in young children, and most cases of PIBO develop during early childhood. Further studies focused on obtaining serial measurements of lung function in infants and toddlers with a risk of bronchiolitis obliterans (BO) after lower respiratory tract infection are therefore needed. Although an optimal treatment for PIBO has not been established, corticosteroids have been used to target the inflammatory component. Other treatment modalities for BO after lung transplantation or hematopoietic stem cell transplantation have been studied in clinical trials, and the results can be extrapolated for the treatment of PIBO. Lung transplantation remains the final option for children with PIBO who have progressed to end-stage lung disease.

• Pediatric Infection and Vaccine
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• 제29권2호
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• pp.105-109
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• 2022

다제내성 반코마이신 저항성 장내구균(multidrug-resistant vancomycin-resistant enterococci, VRE)에 의한 침습감염의 치료는 특히 기저질환을 가진 소아 환자들에게 어려운 점이 있다. 소아환자들에게서 VRE 감염을 치료하기 위한 새로운 항생제에 대한 연구가 충분히 이루어지지 않았다. 본 증례는 심폐이식을 받은 생후 6개월 된 영아에서 linezolid 중단 이후 재발된 VRE 균혈증에 대해 daptomycin과 tigecycline 조합으로 성공적으로 치료하여 이를 보고하는 바이다.

• Journal of Chest Surgery
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• 제50권5호
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• pp.317-325
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• 2017

Background: Extracorporeal membrane oxygenation (ECMO) has become an important treatment modality in pediatric patients with cardiopulmonary failure, but few studies have been conducted in Korea. Methods: We conducted a retrospective review of pediatric patients younger than 18 years who were placed on ECMO between January 2004 and December 2014 at Samsung Medical Center. Results: We identified 116 children on ECMO support. The overall rate of successful weaning was 51.7%, and the survival to discharge rate was 37.1%. There were 39, 61, and 16 patients on ECMO for respiratory, cardiac, and extracorporeal cardiopulmonary resuscitation, respectively. The weaning rate in each group was 48.7%, 55.7%, and 43.8%, respectively. The survival rate was 43.6%, 36.1%, and 25.0%, respectively. Sixteen patients on ECMO had functional single ventricle physiology; in this group, the weaning rate was 43.8% and the survival rate was 31.3%. Ten patients were on ECMO as a bridge to transplantation (8 for heart and 2 for lung). In patients with heart transplantation, the rate of survival to transplantation was 50.0%, and the overall rate of survival to discharge was 37.5%. Conclusion: An increasing trend in pediatric ECMO utilization was observed. The outcomes were favorable considering the early experiences that were included in this study and the limited supply of specialized equipment for pediatric patients.

• Childhood Kidney Diseases
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• 제24권1호
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• pp.47-52
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• 2020

Pneumocystis pneumonia (PCP) is a rare disease in healthy people but a potentially fatal opportunistic infection by Pneumocystis jirovecii in immunocompromised patients with organ transplantation. We present three cases of PCP after kidney transplantation in pediatric patients. First case was a 4-year-old boy diagnosed with Denys-Drash syndrome and received living-donor kidney transplantation from his mother at age of 1. Second case was a 19-year-old male, with polycystic kidney disease, who received kidney transplantation from his mother at the age of 18. Third case was a 19-year-old female with chronic kidney disease of unknown etiology, who received kidney transplantation from her father at age of 15. These three patients who were on immunosuppressive therapy and completed of routine PCP prophylaxis for 6 months had presented with cough and dyspnea more than 1 year after transplantation. Chest x-ray all showed diffuse haziness of both lung fields, and bronchoalveolar lavage from bronchoscopy revealed Pneumocystisjirovecii infection. All patients showed clinical resolution with intravenous trimethoprim-sulfamethoxazole (TMP-SMX) therapy for at least 3 weeks and had continued secondary prophylaxis for another 6-12 months. This report suggests that clinicians should have suspicion for the possibilities of opportunistic infection such as PCP after kidney transplantation in children.

• Journal of Chest Surgery
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• 제42권5호
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• pp.630-634
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• 2009

International Society for Heart and Lung Transplantation (ISHLT)는 2007년 보고에서 선천성 심질환, 1세 미만의 나이, 이식 당시 체외 막형 산소화 보조가 심장 이식 후 사망의 유의한 위험 요소임을 보고하였으며, 몸무게 차이가 클 경우에도 사망의 위험도를 높인다고 하였다. 이 증례의 환아는 심한 좌심실 유출로 협착, 승모판 폐쇄 부전이 있어 Ross수술과 승모판 성형술을 시행하였으나 술 후 심한 좌심실 기능부전으로 체외 막형 산소화 보조를 적용하였다. 체외 막형 산소화 보조 30일이 경과한 생후 3개월 경 공여자-수용자간 몸무게 비가 4.42에 이르는 매우 큰 심장을 성공적으로 이식하였기에 이식의 기술적인 주요 사항을 문헌 고찰과 함께 보고하는 바이다.

• Journal of Chest Surgery
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• 제27권12호
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• pp.977-983
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• 1994

From April 1989 to December 1993, total 39 patients who were unable to be weaned off CPB or expected fatal immediate postoperiatively, were treated with ventricular assist device [VAD] or extracorporeal membrane oxygenator[ECMO] at the Royal Children`s Hospital, Melbourne. Ages ranged from 3 day to 19.4 year old and body weights from 2.0Kg to 70Kg. Twenty-seven[69.2%] of 39 patients were weaned to be decannulated successfully and sixteen[41.0%] survived to hospital discharge and late survival rate was twelve[30.8%] of 39 patients. The total follow-up period was 4 to 56 months [32.92$\pm$20.77months] and most of the late survivals showed good myocardial recovery state. From the viewpoint of the assist modality, 29 patients were treated with VAD and among them, 23 were weaned from assist successfully, but among the 8 ECMO patients, only 3 could be weaned, and both modalities were performed to the 2 patients with one weaned. The total duration of assist was from 8 to 428 hours and there was a significant difference between hospital discharged group and hospital death group, which were 83.13$\pm$31.29 hours vs 147.52$\pm$112.03 hours[P=0.032]. Conclusively, at the critical postcardiotomy situation of the paediatrtic patients including various congenital complex disease and procedures, we can choose this VAD or ECMO treatment strategy as the reasonable life saving way except transplantation.

• Clinical and Experimental Pediatrics
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• 제46권1호
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• pp.42-50
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• 2003

목 적 : 소아의 급성 호흡 곤란 증후군은 직접적 혹은 간접적 폐 손상 후 급성적으로 폐포-모세혈관의 손상이 심하게 진행하는 질환군으로 이에 대한 임상 및 연구 자료들이 아직은 부족한 실정이다. 연자들은 단일 기관에서 경험한 소아의 급성 호흡 곤란 증후군에 대한 발생 양상, 임상 경과, 예후 및 사망 원인들을 분석하여 향후 이 질환에 대한 진단과 치료에 도움이 되고자 하였다. 방 법 : 1992년 1월부터 2001년 12월까지 10년간 신촌세브란스병원 소아과에 내원하여 급성 호흡 곤란 증후군으로 진단되었거나 의심되었던 환아 45명 중 1994년 American-European Consensus Conference에서 제안한 진단 기준에 부합하는 33례를 대상으로 임상 양상과 치료에 대한 후향적 자료 분석을 하였다. 결 과 : 대상 환아 33명의 연령별 발병 빈도는 1세 미만에서 가장 많았으며(7례, 21.2%) 4세 미만의 환아의 수는 20례로 전체의 60.6%를 차지하였다. 반면 연령별 사망률은 1세 미만에서 57.1%로 가장 낮아 전체 사망률 78.8%(26례)에 비해 상대적으로 치료성적이 좋았으나 통계적 의미는 없었다. 남아가 20명이고 여아가 13명으로 남녀비는 약 6 : 4였고 각각의 사망률의 차이는 없었다. 계절별 발병 빈도는 봄철(3-5월)에 12례(36.4%)로 비교적 높았으며 특히 5월에 7례로(21.2%) 가장 높았다. 유발요인으로는 폐렴, 패혈증, 흡인성 사고 등의 순이었으며, 골수 이식 수혜자 1례를 포함하여 면역 결핍 상태의 환아들이 전체의 18.2%(6례)를 차지하였는데 이들 모두가 사망하였다. 내원 당시 흉부 방사선 검사에서 grade 4 이상의 소견을 보인 환아들 중 88.9%(8/9)가 사망하였으나 흉부 방사선 소견에 따른 사망률의 통계학적 차이는 없었다. 진단 시 호흡 부전만 보인 군의 사망률 33.3%(1/3)에 비해 타 장기의 기능 부전이 동반된 군의 사망률이 83.3%(25/30)로 증가되었지만 두 군 사이의 사망률의 통계학적 차이는 보이지 않았다. 생존군과 사망군의 비교에서는 진단후 7일째 헤마토크리트, 동맥혈 산소 분압, 동맥혈 이산화탄소 분압, $PaO_2/FiO_2$, 그리고 호기말 양압 등에서 유의한 차이를 나타냈다. 결 론 : 급성 호흡 곤란 증후군의 사망률에는 호흡 부전이 중요한 영향을 미치며 생존을 위한 보다 적극적인 치료가 연구되고 도입되어야 할 것으로 사료된다.