• Advances in pediatric surgery
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• 제4권2호
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• pp.125-130
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• 1998

To assess the clinical and nutritional status of long-term survivors of biliary atresia, history taking, medical record review, physical examination (height, weight, MAC, TSF), blood tests (LFT, prothrombin time, platelet count, prealbumin, calcium) and liver needle biopsy were performed in 12 patients in whom Kasai procedure were performed more than 10 years ago at Department of Pediatric Surgery in Seoul National University Hospital. None were below the 5th percentile in height and weight. TSF was above the 75th percentile in all patients and showed good subcutaneous fat deposition. MAC was above the 5th percentile in all patients. Serum prealbumin level was abnormal in 2 patients with abnormal liver function and revealed visceral protein malnutrition. Serum calcium level was decreased below normal range in 4 patients with abnormal liver function. One patient had mild ascites. Five patients had abnormal liver function and 7 patients showed clinical manifestation of portal hypertension. Liver needle biopsy was performed in 5 patients and no cirrhotic change was observed. Although some patients who have survived for more than 10 years after Kasai procedure developed protein malnutrition and vitamin deficiencies, growth and development and nutritional status were generally satisfactory. Five patients(42%) showed normal liver function and no portal hypertension. In conclusion, Kasai procedure is satisfactory as a primary treatment in biliary atresia but significant portion of long-term survivors had abnormal liver function and portal hypertension. Continuous and careful follow-up is necessary to determine when liver transplantation may be indicated.

• Tuberculosis and Respiratory Diseases
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• 제66권1호
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• pp.47-51
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• 2009

간폐증후군은 간질환이 있는 환자에서 저산소증이 유발되는 상태로 폐내 혈관 확장으로 인해 폐내 동맥혈 산소공급에 결함이 발생하는 질환이다. 현재까지 간이식 외에 간폐증후군에 대한 다른 효과적인 치료법은 없는 것으로 알려져 있다. 국내에서는 간폐증후군의 증례 보고가 드물고, 특히 Child-Pugh 분류 A군에서의 발생 빈도는 낮은 것으로 보인다. 이에 저자들은 Child-Pugh 분류 A군인 대상성 간경화 환자에서 발생한 간폐증후군 1예를 경험하여 문헌 고찰과 함께 보고하는 바이다.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제6권1호
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• pp.32-38
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• 2003

목 적: HBcAb 양성인 공여자로부터 생체 부분 간이식을 시행할 때 HBsAg 음성인 수혜자가 B형 간염 바이러스에 감염될 가능성이 높다. 본 연구에서는 삼성서울병원의 소아 생체 부분 간이식에서 능동 면역을 주로 사용하였던 초창기와 Hepatitis Bimmunoglobulin (HBIg) 단독 요법을 사용하였던 후기를 비교 분석하여 간이식 후 de novo hepatitis B발생에 대한 HBIg 단독 요법의 예방 효과에 대해 조사해 보고자 하였다. 방 법: 1996년 5월부터 2002년 6월까지 시행한 생체 부분 간이식에서 공여자가 HBcAb 양성이었으며 수혜자가 HBsAg 음성인 소아는 15명이었다. 다른 이유로 사망한 2명을 제외한 13명 중 11명은 HBsAb 양성, 2명은 naive (HBsAb 음성, HBcAb 음성)였다. 모든 환자는 간이식 전 B형 간염 바이러스 예방 접종을 실시하였다. 초기 단계에는(1997년 1월~1997년 11월, 3명) 수혜자가 HBsAb 양성인 경우 간이식 후 B형 간염 추가접종을 실시하였다. 후기 단계에서는(1997년 12월 이후, 10명) 간이식 전후에 모두 B형 간염 예방 접종을 실시한 이후 항체 양성인 수혜자에게 HBsAb 항체가를 200 IU/L 이상 유지하기 위해 HBIg를 단독 유지요법으로 사용하였다. HBIg의 심한 부작용으로 인해 1명의 경우 Lamivudine을 사용하였다. De novo hepatitis B의 예방효과를 병력 고찰을 통하여 후향적으로 분석하였다. 결 과: 13명 중 3명(23.1%)에서 de novo hepatitis B가 발생하였다. 능동 면역만을 시행한 초기 단계에서 3명 중 3명 모두 7~19개월에 HBsAg 양성으로 혈청 변환을 하였다. 1명은 간이식 전 naive 혈청 소견이었고 2명은 HBsAb 양성인 상태였다. HBIg를 사용한 후기 단계에서는 10명 모두 관찰 기간 7~55개월 동안 HBsAg 음성으로 남아 있다. 결 론: HBIg 단독 요법은 HBcAb 양성인 공여자의 간을 이식받은 HBsAg 음성 수혜자에서 de novo hepatitis B를 예방하는데 효과적이다.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제4권1호
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• pp.63-70
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• 2001

목 적: 본 연구는 생체 부분 간이식이 환아의 성장에 미치는 영향에 대하여 분석하고자 하였다. 방 법: 1994년 10월부터 1999년 2월까지 서울중앙병원에서 시행된 생체 부분 간이식을 받은 환아 중 생존율이 1년 이상인 환아 36명을 대상으로 하였다. 이식 전후 외래에서 추적 관찰하며 계측한 신장 기록을 후향적으로 조사하여 이식 후 개월에 따른 평균 zH를 비교하였다. 또한 이식 연령이 6세 이하로 사춘기 급성장의 영향이 배제된 소아에서의 이식 후 성장 변화와 원인 질환, 이식 전 성장 지연, 간이식편의 기능, 면역 억제제의 종류에 따른 이식 후 성장 변화에 대해 분석하였다. 결 과: 전체 환아의 평균 zH는 이식 전 -1.58에서 이식 후 24개월째 0.33으로 현저하게 지속적인 성장이 이루어짐을 보였다(p<0.05). 사춘기 성장의 급증에 의한 신장을 배제하기 위해 이식 당시 나이가 6세 이하였던 환아들에서 비교한 평균 zH도 이식 후 신장의 성장을 보였다(p<0.05). 만성질환이 있는 경우 이식 전에 비해 이식 후 성장이 향상되었고 전격성 간염의 경우 이식 후에도 지속적인 성장이 유지되었다. 이식 전 성장 지연이 있었던 환아의 경우(평균 zH=-2.70) 이식 후에 따라잡기 성장이 이루어졌고 정상적인 성장을 보였던 경우 이식 후에도 성장이 지속적으로 유지되었다. 만성 이식 거부반응이나 PTLD 같은 이식편의 간기능 장애가 동반된 경우에는 이식 후 성장의 지연을 보였다. 면역 억제제의 종류에 따른 성장의 변화에는 통계학적으로 유의한 차이는 보이지 않았다. 결 론: 생체 부분 간이식은 성공적인 간이식을 통해 장기 생존률의 증가 뿐만 아니라 술 후 정상적인 환아의 성장을 기대해 볼 수 있다.

• 대한미세수술학회:학술대회논문집
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• 대한미세수술학회 1996년도 제16차 학술대회
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• pp.158-158
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• 1996

• 한국보건간호학회지
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• 제19권1호
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• pp.129-138
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• 2005

As the patients who need to undergo liver transplant operation continues to grow. the number of livers that are donated can not keep pace with the demand. With the development of surgery skills, the necessity for operations from living donors is increasing. Nevertheless, satisfactory research has been conducted on the factors which generally affect the living donors. In this article. therefore. researchers focused on the factors which generally affect the donating liver donor in order to design a plan for recommending liver donation from living donors. The subjects were 91 living liver donors in C university hospital from October 1. 2000 to December 31. 2003. The results on the uncertainty of living donor, by test sheet. were analyzed with SAS program. The final results were as follows: 1. The uncertainty of the living donors was 51.54 marks per full credit 100. 2. The factor with the greatest effect on donation was the possibility of survival of the donor, followed by the admission period. marriage status and age. In recommending the living donation, the rate of donor survival after the operation was 5.2 times higher than death, 5.2 times higher when the admission period was under 20 days. 5.0 times higher when married. and 27.3 times higher when the family-related donation was very active at the age of 20s than in the 50s. These results suggest that all medical staffs should care for living donors with more interest and activity to give them the least complaints in admission and the lowest possibilities for complication. To enhance the survival rate and improve the surgical success rate. on-going monitoring should include regular health-checks. and continual efforts and education should be made to care for the health condition of the living donors after donation.

• Advances in pediatric surgery
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• 제13권2호
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• pp.144-154
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• 2007

Liver tumors in children are rare, relatively complex, and encompass a broad spectrum of disease processes. This study reviews our experience of liver tumors during the last 10 years. Medical records of 36 cases of liver tumors in children, treated at Samsung Medical Centers, from October 1994 to December 2005, were reviewed in this study. We analyzed disease characters and survival rates as a whole and by specific disease. The median age was 3.6 years. Male and female ratio was 1:1. The most common symptom was the palpable mass in 15 cases. Others were abdominal distension in 9 cases, jaundice in 2, vomiting in 2, weight loss in 2, and pubic hair growth in 1. CT or US and liver biopsy were performed for diagnosis. There were 28 malignant tumors: malignant rhabdoid tumor (1 case), hepatocellular carcinoma (3 cases), hemangioendothelioma type II (3 cases), angiosarcoma (1 case), and hepatoblastoma (20 cases). Eight tumors were benign; hepatic adenoma (1 case), focal nodular hyperplasia (2 cases), hemangioendothelioma type I (2 cases), mesenchymal hamartoma (3 cases). In this study the clinical characteristics were not different from the other reports. Liver transplantation was performed in 3 cases-1 with hepatoblastoma and 2 with hepatocelleular carcinoma. Accurate and early diagnosis, and individualized multimodality therapeutic approaches might be important for better outcome.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제25권5호
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• pp.353-375
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• 2022

No systematic review to date has examined histopathological parameters in relation to native liver survival in children who undergo the Kasai operation for biliary atresia (BA). A systematic review and meta-analysis is presented, comparing the frequency of native liver survival in peri-operative severe vs. non-severe liver fibrosis cases, in addition to other reported histopathology parameters. Records were sourced from MEDLINE, Embase, and CENTRAL databases. Studies followed the PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines and compared native liver survival frequencies in pediatric patients with evidence of severe vs. non-severe liver fibrosis, bile duct proliferation, cholestasis, lobular inflammation, portal inflammation, and giant cell transformation on peri-operative biopsies. The primary outcome was the frequency of native liver survival. A random effects meta-analysis was used. Twenty-eight observational studies were included, 1,171 pediatric patients with BA of whom 631 survived with their native liver. Lower odds of native liver survival in the severe liver fibrosis vs. non-severe liver fibrosis groups were reported (odds ratio [OR], 0.16; 95% confidence interval [CI], 0.08-0.33; I²=46%). No difference in the odds of native liver survival in the severe bile duct destruction vs. non-severe bile duct destruction groups were reported (OR, 0.17; 95% CI, 0.00-63.63; I²=96%). Lower odds of native liver survival were documented in the severe cholestasis vs. non-severe cholestasis (OR, 0.10; 95% CI, 0.01-0.73; I²=80%) and severe lobular inflammation vs. non-severe lobular inflammation groups (OR, 0.02; 95% CI, 0.00-0.62; I²=69%). There was no difference in the odds of native liver survival in the severe portal inflammation vs. non-severe portal inflammation groups (OR, 0.03; 95% CI, 0.00-3.22; I²=86%) or between the severe giant cell transformation vs. non-severe giant cell transformation groups (OR, 0.15; 95% CI, 0.00-175.21; I²=94%). The meta-analysis loosely suggests that the presence of severe liver fibrosis, cholestasis, and lobular inflammation are associated with lower odds of native liver survival in pediatric patients after Kasai.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제11권sup2호
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• pp.35-43
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• 2008

Jaundice is common in breast-fed infants. Any infant noted to be jaundiced at 2 weeks of age need to be evaluated for cholestasis with measurement of total and direct serum bilirubin. The most common causes of cholestatic jaundice in infants are biliary atresia and neonatal hepatitis. Genetic causes of the neonatal hepatitis syndrome are increasingly recognized and idiopathic neonatal hepatitis is decreasing. Cholestasis should be investigated using a structured protocol. Early detection and timely, accurate diagnosis is important for successful treatment and a favorable prognosis. In particular, a Kasai portoenterostomy for biliary atresia has the best outcome if performed before the infant is 8 weeks of age. The management of cholestasis is mainly supportive, including nutritional support and alleviation of symptoms to improve the quality of life. Specific treatments are available for some causes of neonatal hepatitis syndrome and should be started as soon as possible. For decompensated liver disease, liver transplantation yields a better outcome.

• Journal of Chest Surgery
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• 제55권2호
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• pp.171-173
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• 2022

A 65-year-old man underwent right trisectionectomy of the liver and reconstruction of the chest wall and diaphragm with a 2-mm Gore-Tex membrane due to recurrent hepatocellular carcinoma. After 3 years, the Gore-Tex membrane in the diaphragm migrated to the abdominal cavity and perforated the colon. We report a rare complication of a Gore-Tex membrane after diaphragm repair.