• Journal of Chest Surgery
• /
• 제23권5호
• /
• pp.844-853
• /
• 1990

We have performed 27 cases of orthotopic homologous cardiac transplantation using Korean mongrel dogs and one case of sham operation for the evaluation of harmful effect of cardiopulmonary bypass itself on the dog from April, 1989 to June, 1990. Our previous reports have already demonstrated basal hemodynamic and hematologic data on the canine homologous heart transplantation and the fundamental principles of transplantation of the heart. The mean body weight of recipients was 13.2$\pm$1.2kg with a rage of 11 ~ 15kg, and the hemodynamic and hematologic pictures were almost same as the result of previous reports from our hospital, except marked decrease in postoperative platelet count[from 3.18 $\pm$0.80x106/mm3 to 1.41$\pm$0 37x 106/mm3]. Mean survival time was 24.82$\pm$49.40 hours with the longest survival of 264 hours. Donor cardiectomy included coronary vasodilatation with diltiazem, potassium arrest, and the rapid cooling of the heart suspending in the specially designed ice-bath. Median sternotomy provided excellent exposure of the surgical field. 6 \ulcorner0 prolene suture was used for the anastomosis of both atrial cuffs and the great arteries, and we found the fact that stenosis, bleeding, thrombus formation around the anastomotic site could be decreased with the use of everted horizontal mattress suture techniques. Immunosuppression was done with a combination of lower dose Cyclosporin-A, Azathioprine, methyl-prednisolone, but our cases still showed too short survival to worry about graft rejection. Still poor was our quality control of experimental animal, we had much difficulties in postmortem evaluation of the dogs. Low cardiac output due to biventricular failure, intractable supraventricular or ventricular tachyarrhythmia, postoperative massive bleeding, sepsis were most frequent findings that could be thought as a cause of death. A few cases showed subendocardial patch hemorrhage in both ventricular cavity or atrial septum at autopsy, suggesting acute subendocardial infarction. Although our team overcome most of the technical problems of orthotopic heart transplantation, we should pile up further knowledges about donor heart preservation, quality control of animal, infection, rejection, the effect of the cardiopulmonary bypass to improve the results.

• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• 제23권1호
• /
• pp.63-71
• /
• 2020

Purpose: Autoimmune hepatitis (AIH) is a chronic disease that may lead to cirrhosis. The immunopathogenesis of AIH is not fully understood and it mainly involves T-cell mediated mechanism. Macrophage migration inhibitory factor (MIF) is a pro-inflammatory cytokine that promotes T cell response and its polymorphism may serve as a severity marker of AIH. No previous study has considered investigating MIF polymorphism in children with AIH. Methods: Forty-two children with definite diagnosis of AIH were enrolled along with 100 age and sex matched controls. All participants were tested for polymorphism at -173GC (rs755622) of MIF gene. All patients received the standard protocol of steroid plus azathioprine to achieve remission. Liver biopsy was performed at time of diagnosis for all patients and only 18 of them underwent a second biopsy after treatment. Results: No statistically significant differences in the frequency of the genotypes GG and GC or in allele distribution were found in both patient and control groups (p=0.590, 0.640 respectively). Initial alanine aminotransferase (ALT) levels at the time of presentation was significantly higher in the GC group than GG group (p=0.020). GC genotype significantly correlated with disease relapse (r=0.41, p=0.007). Regression of necroinflammation and the fibrosis score in the second liver biopsy was statistically significant in the GG group (p<0.0001, p=0.010 respectively). Conclusion: MIF -173GC polymorphism is associated with clinically significant markers of pediatric AIH, including increased initial serum ALT levels, may help predict necroinflammatory/fibrosis regression effectively, following immunosuppressive treatment.

• 한국임상약학회지
• /
• 제16권1호
• /
• pp.14-22
• /
• 2006

Purpose: To determine the short (1 year of transplant) and long-term (1-5 years of transplantation) risk factors affecting the graft and patient survival in kidney transplantation recipients. Methods: Records of 149 patients who received kidney transplantation in 1996 from Asan Medical Center were followed for 5 years retrospectively. Results: All patients initiated triple immunosuppressive therapy with cyclosporine, prednisone and azathioprine. One, two, three, four, five year patient and graft survival rates were 98.7%, 98.0%, 98.0%, 97.3%, 97.3%, and 96.6%, 95.2%, 94.6%, 92.5%, 91.8%, respectively. There were 30 cases of acute rejection (AR) and 6 cases of chronic rejection (CR) within $2.1{\pm}3.2$ months and $42.1{\pm}13.2$ months of transplantation, respectively. The risk factors for AR were donor's age older than 30 years (p=0.02) and cardiovascular disease (p=0.05). The risk factors for CR were AR (p=0.0169) and episode of complications (p=0.0330). Increasing period of dialysis (p=0.0473), episodes of AR (p<0.0001) and complication (p=0.0317) were significant factors for graft loss. Seven grafts were lost from noncompliance during 1-5 year period. The most com- mon cause of the graft loss for both periods was the graft rejection. The graft survival rate was significantly lower in patients with than without rejection episodes (77.4% vs. 90.0%, p=0.002). Conclusions: Survival rate of the graft with rejection was significantly lower. The risk factors affecting AR were donor's age older than 30years and CVD. AR and episode of complications within 1year were the risk factors for CR and graft loss.

• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• 제17권3호
• /
• pp.147-154
• /
• 2014

Purpose: Gender differences in paediatric patients with inflammatory bowel disease (IBD) are frequently reported as a secondary outcome and the results are divergent. To assess gender differences by analysing data collected within the Swiss IBD cohort study database since 2008, related to children with IBD, using the Montreal classification for a systematic approach. Methods: Data on gender, age, anthropometrics, disease location at diagnosis, disease behaviour, and therapy of 196 patients, 105 with Crohn's disease (CD) and 91 with ulcerative or indeterminate colitis (UC/IC) were retrieved and analysed. Results: The crude gender ratio (male : female) of patients with CD diagnosed at <10 years of age was 2.57, the adjusted ratio was 2.42, and in patients with UC/IC it was 0.68 and 0.64 respectively. The non-adjusted gender ratio of patients diagnosed at ${\geq}10$ years was 1.58 for CD and 0.88 for UC/IC. Boys with UC/IC diagnosed <10 years of age had a longer diagnostic delay, and in girls diagnosed with UC/IC >10 years a more important use of azathioprine was observed. No other gender difference was found after analysis of age, disease location and behaviour at diagnosis, duration of disease, familial occurrence of IBD, prevalence of extra-intestinal manifestations, complications, and requirement for surgery. Conclusion: CD in children <10 years affects predominantly boys with a sex ratio of 2.57; the impact of sex-hormones on the development of CD in pre-pubertal male patients should be investigated.

• Tuberculosis and Respiratory Diseases
• /
• 제61권1호
• /
• pp.65-69
• /
• 2006

저자들은 43세 남자환자에서 반복적인 구강궤양을 가지고 있으며 안증상으로 진단한 베체트병 환자에서 폐동맥 색전증, 폐동맥류, 상대정맥 협착등 다양한 폐혈관 침범과 함께 폐아스페르길루스종을 동반한 다발성 공동 형성을 경험하였기에 문헌고찰과 함께 보고하는 바이다.

• Journal of Chest Surgery
• /
• 제2권2호
• /
• pp.155-166
• /
• 1969

Heterotopic abdominal homograft of canine heart was carried out in 20 pairs of dogs. Of these 12 cases were subjected as a control and 8 were subjected to immunosuppressive group. The dosage of immunosuppressive agent was 5mg/kg/day of Imuran [Azathioprine] for 3 days preoperatively, 10mg/kg on operative day and 5mg/kg/day postoperatively. For reducing the metabolic demand, the donor heart was preserved in 4degree heparinized saline solution for approximately I4 minutes. In the most of the cases, transplantation was performed with the technique of end-to-side aorto-aortic anastomosis and end-to-side pulmonary artery-inferior vena cava anastomosis at the infrarenal portion. Five out of 20 grafted dogs were survived more than one day. The longest survived 18 days in the control group and survive more than 60 days in the treated group. The survival cases were 3 out of 8[37. 5%] in the group of dogs treated with lmuran and 2`out of 12 [16.6%] in the group of non-treated. A prominent gross findings of the grafted heart was a minimal to moderate degree of dilatation of the heart with or without thrombosis in the cardiac chambers and/or anastomotic site. The case number 10, 15, and 19 showed moderate hypertrophy in grossly. The microscopic findings were as follows; 1. There were early hypersensitive histologic reactions such as interstitial edema, cellular infiltrations and early degenerative changes in the myocardium in the cases of 3 hour survival. 2. In the cases of more than 6 hours survival, organizing thrombosis of myocardial vessels, vasculitis,myocardial necrosis and lymphocyte, plasma cell, round cell infiltrations were noted. In the cases of more than 12 hours survival, the degree of these histologic changes especially in the non-treated group were more intensified than in the treated. 3. In the cases which survived more than one day, so called homograft specific histologic changes were milder in the immunosuppressive group compared with the control. 4. All the host hearts showed no evidence of pathologic findings histologically. Among the homologous canine cardiac transplantation tissue reaction, was milder and suvival time longer in the group treated with immunosuppressive drug.

• Journal of Chest Surgery
• /
• 제34권12호
• /
• pp.956-959
• /
• 2001

이식 후 발생하는 임파구 증식성 질환은 장기 이식의 예가 늘어나면서 증가하는 추세이며 폐 ,심장 이식과 같이 면역 억제 요법의 강도가 심할수록 그 발생률이 높아진다. 특히 임파구 증식성 질환 중 악성 임파종은 치료가 어렵고 예후가 좋지 않아 신속한 진단 및 치료가 필요하다. 본원에서는 1999년 11월 말기 폐기종으로 호흡 곤란이 심해져 가는 37세 A형 여자 환자에 0형 남자 환자의 좌측 폐를 이식하였다. 폐 이식 수술 후 면역 억제를 위해 3제 치료(싸이클로스포린, 아자씨오프런, 프레드니졸론)를 시행하였고, 환자는 이식 후 특별한 문제없이 외래 추적 관찰 중 정기적으로 시행한 가슴 컴퓨터 단층 촬영 상 이식 폐에 비정상적 종괴가 보여 2001년 2월 gun biopsy 시행 후 악성 임파종으로 진단을 받았으며 면역 억제제 감량 후 항암 화학요법 2회 시행하고 경과 관찰 중 갑자기 발생한 이식 폐부종으로 사망하였다.

• Tuberculosis and Respiratory Diseases
• /
• 제82권4호
• /
• pp.277-284
• /
• 2019

Idiopathic nonspecific interstitial pneumonia (NSIP) is one of the varieties of idiopathic interstitial pneumonias. Diagnosis of idiopathic NSIP can be done via multidisciplinary approach in which the clinical, radiologic, and pathologic findings were discussed together and exclude other causes. Clinical manifestations include subacute or chronic dyspnea and cough that last an average of 6 months, most of which occur in non-smoking, middle-aged women. The common findings in thoracic high-resolution computed tomography in NSIP are bilateral reticular opacities, traction bronchiectasis, reduced volume of the lobes, and ground-glass opacity in the lower lungs. These lesions can involve diffuse bilateral lungs or subpleural area. Unlike usual interstitial pneumonia, honeycombing is sparse or absent. Pathology shows diffuse interstitial inflammation and fibrosis which are temporally homogeneous, namely NSIP pattern. Idiopathic NSIP is usually treated with steroid only or combination with immunosuppressive agents such as azathioprine, cyclophosphamide, cyclosporine, and mycophenolate mofetil. Prognosis of idiopathic NSIP is better than idiopathic pulmonary fibrosis. Many studies have reported a 5-year survival rate of more than 70%.

• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• 제7권2호
• /
• pp.186-196
• /
• 2004

목적: 자가면역성 간염은 원인 불명의 진행성 염증성 간질환으로 혈중 자가항체의 출현과 고감마글로불린혈증, 그리고 조직학적으로 문맥주위 염증을 특징으로 한다. 소아에서는 산발적인 증례 보고가 있었으나 국내에서는 아직까지 체계적인 연구가 없었다. 이에 저자들은 소아에서 자가면역성 간염의 임상적, 조직학적 소견과 치료 경과 및 예후에 대해 알아보고자 본 연구를 시행하였다. 방법: 1990년부터 2004년까지 서울대학교 어린이병원에서 자가면역성 간염으로 진단되었던 14명의 환자들의 임상적 특징, 혈액 검사 소견 및 간 조직 검사 소견을 분석하였다. 또한 이들 환자들에게 시행된 치료 약제 및 치료에의 반응, 그리고 재발 여부 등 치료 경과를 조사하였다. 결과: 대상 환자 14명의 진단시 연령은 평균 $9{\pm}3$세였다. 여자가 11명으로 전체의 78%를 차지하였고, 6명에서 급성 발병을 보였다. 임상 증상으로는 황달이 11명에서 호소하여 가장 흔하였으며, 이 외에도 피로감, 식욕부진, 진한색 소변, 상복부 불쾌감, 잦은 코피, 오심, 소양감 및 설사가 동반되었다. 신체 검진 소견에서는 간비종대가 흔하게 나타났다. 6명의 환자에서 다른 자가면역성 질환이 동반되었고 1명에서는 일등친 중 그레이브스병의 병력이 있었다. 혈중 자가항체 검사 결과 13명에서 항핵 항체가 양성으로 나왔으며 항핵 항체가 음성으로 나왔던 1명은 항평활근 항체가 양성으로 나와 대상 환자 모두가 제1형 자가면역성 간염에 해당되었다. 7명에서는 항핵 항체와 항평활근 항체가 모두 양성으로 나왔다. 혈청 IgG는 평균 $2971{\pm}2147mg/dL$였고, AST는 평균 $469{\pm}402\;U/L$, 총 빌리루빈은 평균 $4.9{\pm}5.5mg/dL$였다. 간 조직 검사 소견으로는 문맥 주위의 염증(10명), 간세포의 로제트 형성(3명), 소엽의 염증(6명)이 흔하게 나타났고 2명에서는 진단 당시 이미 조직학적으로 간경변이 동반되었다. 대상 환자 14명 중 6명은 UDCA만 사용하며 관찰 중 간수치가 호전되었고 나머지 8명에게는 스테로이드 치료가 시행되었다. 이중 7명이 초기 스테로이드 치료시 관해를 보였으나 스테로이드를 감량하거나 중지시 재발하였다. 스테로이드 재치료가 시행되었고 3명은 스테로이드 감량 중 관해 유지 중이고 2명은 azathioprine으로 관해 유지 중이다. 결론: 자가 면역성 간염은 원인 불명의 소아 만성 간 질환의 원인 중 하나이며, 적절한 치료로 예후가 개선될 수 있는 간질환이다. 그러므로 특히 여아에서 고감마글로블린혈증을 보이거나 다른 자가면역성 질환이 동반될 경우 자가면역성 간염을 의심하여 자가항체 선별 검사를 시행하고 적절한 진단 및 치료를 시행하는 것이 중요하리라고 생각된다.

• Childhood Kidney Diseases
• /
• 제3권1호
• /
• pp.80-87
• /
• 1999

목 적 : 루프스 신염은 전신성 홍반성 낭창(systemiclupuserythematosus,SLE)의 가장 심각한 합병증 가운데 하나이지만, 소아 연령에서 루프스 신염에 대한 국내 연구는 미약한 실정이다. 본 연구는 루프스 신염 환자를 대상으로 빈도, 임상상, 치료반응, 예후 등에 대한 기초 조사를 실시하여 향후 전국 규모의 다기관 연구를 위한 기반 자료로 사용하기 위함이다. 방 법 : 1986년부터 1997년 11년 동안 서울대학교 어린이병원 소아과에 입원한 SLE환아 53례 중 신생검상 루프스 신염이 확인된 46례를 대상으로 후향적 의무기록 고찰을 시행하였다. 결 과 : 1) 전체 남녀비는 1:2.5, 루프스 신염의 진단 당시 정중연령은 12.8세(2세-15세 8개월)이었다. 2) ARA 분류 기준 중 형광항핵항체($85.7\%$), 항ds-DNA항체($78.0\%$), 나비모양 홍반($60.8\%$) 등이 가장 빈번히 관찰되었으며, 기타 검사 소견중에서는 C3의 감소($88.9\%$)가 흔하였다. 3) 혈뇨($87.0\%$)는 가장 흔한 신장 증상이었고, 신생검상 41례에서 WHO class IV 병변이 확인되었다. 4) Prednisolone, azathioprine, 경구 또는 정맥 cyclophosphamide 등을 단독 또는 병합치료 결과 대부분 환자에서 질환 활성도가 정상 혹은 중증도 이하로 조절되었으며, 치료 종류에 따른 반응도의 차이는 관찰할 수 없었다. 5) 감염은 치료 경과중 가장 흔한 합병증이었으며, 특히 Varicella-Zoster virus와 candida 감염증이 흔하였다. 결 론 : SLE 환아중 루프스 신염의 빈도는 $87.0\%$이었고, 그중 $89.1\%$가 장기적 예후가 불량한 WHO class IV 로 확인되었다. 따라서 신염 초기의 적극적인 면역억제제 사용이 장기적 예후 향상에 도움을 주리라 사료되며, 이에 대한 장기간의 전향적-비교연구를 위하여 다기관 공동연구가 절실히 필요하다.