• Journal of Chest Surgery
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• 제26권3호
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• pp.224-227
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• 1993

We experienced one case of orthotopic cardiac transplantation in a patient with end stage dilated cardiomyopathy. This 50 year-old female recipient was suffered from NYHA functional class IV cardiac failure and dependent upon intravenous inotropic support for 2 months [recipient category 1]. Her preoperative condition was grave with left ventricular ejection fraction of 20% and estimated systolic pulmonary arterial pressure [from Doppler study] was 50mmHg. The brain-dead donor was 31 year-old male with head trauma. The body sizes [weight, height] of the donor/recipient were 70 Kg, 165 cm / 43 Kg, 160 cm and appropriately overmatched. Preoperatively, identical ABO/Rh blood group [A+] and nonreacting HLA crossmatching were confirmed. On November 11 1992 cardiac transplantation was performed without complication. Multiple organ procurement team and heart transplantation team were organized the operation schedule appropriately to minimize the ischemic time. The pump time was 126 minutes and aortic crossclamping time of recipient heart was 73 minutes and, as a result, total ischemic time of the transplanted heart was 75 minutes. Postoperatively, the vital signs were stable with minimal inotropic support. The immunosuppressive therapy was commenced from preoperatively and cyclosporine, azathioprine, and corticosteroid were used as a combination therapy as scheduled and monitored with blood drug concentration, WBC count, renal function and most importantly regular endomyocardial biopsy.Now, 5 months after transplantation, the patient is in NYHA functional class II with minimal cardiac drug support.

• Childhood Kidney Diseases
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• 제1권2호
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• pp.183-188
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• 1997

Familial juvenile hyperuricemic nephropathy is an autosomal dominant disease characterized by progressive renal disease and hyperuricemia or gout, affecting young people of either sex equally. There are two biochemical markers of this disorder. The first is hyperuricemia disproportionate to the degree of renal dysfunction; the second is a grossly reduced clearance of uric acid relative to creatinine, dispropotionate to age, sex and degree of renal failure. We experienced 2 family members with hyperuricemia. One family member, a 13-year-old girl who had suffered from tophaceous gout and chronic renal failure. Her younger brother also had hyperuricemia and moderately reduced renal function. Their urinary excretion fractions of uric acid($FE_{uric\;acid}$) were reduced and renal biopsy specimens showed interstitial fibrosis with tubular atrophy and interstitial urate crystal deposition. We have treated these two patients with allopurinol but we have done renal transplantation because she progressed to end stage renal disease at 16 year old age.

• 한국임상약학회지
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• 제31권1호
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• pp.44-52
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• 2021

Background: Post-transplant immunosuppression with calcineurin inhibitors (CNIs) is associated with kidney function impairment while mammalian target of rapamycin (mTOR) inhibitors, such as everolimus, can be used for its renal-sparing effects. In this study, we compared the efficacy and safety of everolimus with low dose tacrolimus (EVR+Low TAC) and conventional dose tacrolimus (TAC) in liver transplantation recipients. Methods: Medical records of recipients who received liver transplantation at Seoul National University Bundang Hospital from January 1st 2009 to December 31st 2018 were retrospectively reviewed. Cohort entry date was defined as the day everolimus was initiated and tacrolimus dosage was reduced. All patients were followed up for 1 year. Indicator of efficacy was the incidence of rejection and safety was evaluated by incidence of drug adverse events including renal function. Results: Among 118 patients, there were 40 patients (33.9%) in EVR+Low TAC group. Incidence of rejection, including both biopsy proven acute rejection and clinical rejection, was similar in two groups [7.5% (n=3) vs. 6.4% (n=5), p=1.000]. Renal dysfunction was less frequent in EVR+Low TAC [17.5% (n=7) vs. 35.9% (n=28), p=0.038]. However, incidence rates of dyslipidemia, oral ulcer were more frequent in EVR+Low TAC [45.0% (n=18) vs. 21.8% (n=17), p=0.009; 15.0% (n=6) vs. 1.3% (n=1), p=0.006]. Conclusions: In terms of prevention of rejection, EVR+Low TAC was as effective as TAC and had renal-sparing effect but was associated with increased risk of dyslipidemia and oral ulcer. This study demonstrates that EVR+Low TAC could be an alternative to liver transplant recipients with nephrotoxicity after administration of conventional dose tacrolimus.

• Clinical and Experimental Pediatrics
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• 제52권10호
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• pp.1069-1074
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• 2009

Peritoneal dialysis is a preferred modality of replacement therapy in children and adolescents with end-stage renal disease waiting for kidney transplantation. Recent development of pediatric swan-neck catheters with cuffs, novel dialysis solutions, and cyclers for automated peritoneal dialysis enabled more flexible prescriptions of dialysis with less complication, and improved patients' activities as well as the dialysis adequacy. Principles and practical issues of chronic peritoneal dialysis in children and adolescents are reviewed and utility of a web-based Korean Pediatric CRF Registry is explained.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제24권3호
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• pp.288-294
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• 2021

Purpose: Despite aggressive medical and nutritional management, patients with methylmalonic acidemia (MMA) often suffer from multi-organ damage. Early deceased donor liver transplantation (DDLT) has emerged as an intervention to prevent disease progression. We investigated the efficacy of living donor LT (LDLT) with a potential carrier of MMA and a small volume of graft in patients with MMA as an alternative to DDLT. Methods: Of five patients (three male, two female; median age 5.7 years; range, 1.3-13.7 years), four underwent carrier LDLT, while one underwent non-carrier auxiliary LDLT. All patients received pre- and post-LT continuous renal replacement therapy and were provided with minimal restriction diet according to serum MMA level after LT. MMA levels in the serum and urine, the incidence of metabolic crisis, and clinical findings before and after LT were compared. Results: The survival rate was 100% during 2.2 years of follow up period after LT. In all five cases, MMA titer in the serum after transplantation decreased with less restrictive diet. Metabolic crisis was not observed during the follow-up period. In addition, no patient showed progression of severe renal impairment requiring hemodialysis. Progression of delayed cognitive development was not observed. Social functioning with improved neuropsychiatric development was observed. Conclusion: This study showed that LDLT achieved improved quality of life with less restrictive diet, therefore it could be a feasible alternative option to DDLT for the treatment of patients with MMA, even with an auxiliary LT.

• Journal of Chest Surgery
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• 제33권4호
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• pp.338-341
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• 2000

Renal transplant recipients who received immunosuporessive agent are in high risk of development Kaposi's sarcoma. In Korea a few report of Kaposi's sarcoma has been pubilshed but any report of intrathoracic Kaposi's sarcoma provedn by open lung biopsy has not been pulbilshed until now. We report a case of intrathoracic Kaposi's sarcoma developed in a 25 year old Korean man, who had been operated renal transplantation due to end stage renal disease and received cyclosporine and prednisolone as immunosuppessive agent, without any other organ involvment and was proven by open lung biopsy. Although discontinuation of immunosuppressive agent, temporary symptomatic and radilolgic improvement were observed, he died 11 days later after open lung biopsy because of intractable resiratory failure.

• Childhood Kidney Diseases
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• 제7권2호
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• pp.245-252
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• 2003

Posttransplant lymphoproliferative disease(PTLD)는 이식 후 발생하는 림프증식성 질환으로 이식장기의 거부반응을 억제하기 위한 면역억제제의 사용 및 이에 따른 EBV 감염과 연관이 있다고 알려져 있다. 소아 PTLD의 경우 성인에 비해 EBV의 초감염 또는 재활성이 더 많은 것으로 보고되고 있으며 최근 더 강력한 면역억제제들의 개발 및 사용에 따라 발생이 증가하고 있다. 본 증례는 14세 여아로 신이식 44개월 후에 EBV 감염의 증거 없이 지발성 PTLD가 발생하였으며 골수 검사상 B-세포 급성 림프구성 백혈병으로 진단되어 항암화학요법 치료를 시작하였고, 치료 후 완전 관해는 이루어졌으나 심한 중성구 감소증에 따른 패혈성 쇼크로 입원 77일만에 사망하였다.

• 대한핵의학회지
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• 제35권4호
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• pp.251-257
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• 2001

목적: 신 이식 이틀째 촬영한 Tc-99m DTPA 스캔 소견에서 나타난 이식신의 기능 저하가 이식신의 장기 생존에 영향을 미치는 지 알아보았다. 대상 및 방법: 생체 신 이식 총 64예에서 신장 스캔 소견에 따른 이식신 누적 생존율의 차이를 Kaplan-Meier법으로 분석하고, Cox proportional hazard model을 이용하여 이식신 장기 생존율 예측능을 알아보았다. Chi-square test로 두 군 간에 급성 거부반응의 빈도 차이를 보았다. 결과: 신장 스캔 비정상군의 이식신 장기 생존율이 정상군에 비하여 낮았으나 그 차이가 통계적으로 유의하지 않았다. 이식 직후 신장 스캔 소견 상 나타나는 이식신 기능저하도 이식신 장기 생존의 예후 인자가 아니었다. 단, 신장 스캔 비정상군에서 급성 거부 반응 빈도가 정상군에 비하여 유의하게 높았다. 결론: 생체 신이식 예에서 이식 직후 신장 스캔에서 이식신의 기능 저하 소견은 이식신의 장기 생존에 직접적 영향은 없으나 급성 거부 반응을 증가시켜 간접적 영향이 있을 것으로 판단된다.

• Childhood Kidney Diseases
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• 제9권2호
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• pp.275-281
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• 2005

저자들은 생체신장이식을 받은 후 발열과 빈혈을 주소로 내원한 2명의 환아에서 혈청 PCR을 통해 PV B19 감염을 진단한 후 면역글로불린 정맥투여로 싱공적으로 치료하고 재발 없이 관찰중인 2례를 경험하였기에 보고하는 바이다.

• 대한핵의학회지
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• 제29권4호
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• pp.504-510
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• 1995

목적 : 신장 이식후 발생한 무혈관성 골괴사의 빈도를 알아보고, 골스캔과 SPECT, MRI의 역할을 비교해 보고자 하였다. 대상 및 방법 : 1989년 6월부터 1995년 9원까지 본원에서 신장 이식을 받은 301명중 임상적으로 무혈관성 골괴사를 진단받은 환자의 발생빈도 및 침범 부위를 알아보고, 무혈성 골괴사 환자에서 시행한 골스캔과 SPECT, MRI 소견을 비교하여 보았다. 결과 : 무혈관성 골괴사의 발생빈도는 3.3%(10/301)였고, 침범 부위는 대퇴골두 16병변과 종골 융기 2병변이었다. 또한 전신 골스캔상 천골의 불충분 골절을 보인 경우가 1례 있었다. 골스캔 소견은 열소에 둘러싸인 냉소 13병변, 열소 3병변, 냉소 1병변, 정상 1병변의 소견을 보였고 열소만을 보인 경우는 SPECT에서는 작은 냉소를 둘러싸는 열소로, 냉소나 정상으로 보인 경우는 SPECT상 반응성 열소 없는 냉소로 나타났다. SPECT와 MRI를 같이 시행한 6례중 모두양성인 경우가 5례였고, 증상이 있었던 1례에서 SPECT상 냉소로 보였으나 MRI에서는 정상소견을 보였다. 결론 : 신장 이식후 발생한 무혈관성 골괴사의 발생빈도는 3.3%였고, 전신골스캔으로 골괴사외의 전신골 병변을 관찰할 수 있었으며 증상은 있으나 골스캔상에서 병변이 뚜렷하지 않거나 MRI에서 음성인 경우 등에 SPECT가 진단에 도움이 되었다. 그러므로 증상은 있으나 MRI에서 음성인 경우 SPECT를 시행하는 것이 신장 이식후의 환자에서 발생하는 무혈관성 골괴사의 진단에 유용하리라고 생각된다.