• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제18권3호
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• pp.175-179
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• 2015

Purpose: Infants with Alagille syndrome (AGS) are occasionally misdiagnosed as biliary atresia and subsequently undergo Kasai operation. The purpose of this study was to investigate the outcome of patients with AGS who had previously received Kasai operation during infancy. Methods: This retrospective study was conducted at the Department of Pediatrics, Samsung Medical Center. We compared the prognosis and mortality between those who had undergone Kasai operation during infancy (Kasai group) and those who had not (non-Kasai group). Results: Among the 15 children with AGS, five had received Kasai operation, while 10 had not. All subjects in the Kasai group revealed neonatal cholestasis, while 70% of the non-Kasai group showed neonatal cholestasis. Liver transplantation was performed in 100% (5/5) among the Kasai group, and 20.0% (2/10) among the non-Kasai group (p=0.007). Mortality was observed in 60.0% (3/5) among the Kasai group, and 10.0% (1/10) among the non-Kasai group (p=0.077). Conclusion: Although overall mortality rate did not significantly differ between the two groups, the proportion of patients receiving liver transplantation was significantly higher in the non-Kasai group. The relatively worse outcome in AGS patients who had received Kasai operation may be due to the unfavorable influences of Kasai operation on the clinical course of AGS, or maybe due to neonatal cholestasis, irrespective of the Kasai operation.

• Advances in pediatric surgery
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• 제17권1호
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• pp.1-14
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• 2011

Biliary atresia (BA) is an infantile cholestatic disease of progressive obliterative cholangiopathy with varying degrees of damage to both extra and intrahepatic bile ducts due to unknown causes. The diagnostic studies should be done to diagnose or exclude BA without unnecessary delay. Kasai portoenterostomy is the first choice of treatment for bile drainage from microscopic bile ductules present in the portal fibrous mass. The medical management after Kasai portoenterostomy should be done carefully to maintain bile excretion and prevent and treat complications Including cholangitis, hepatic fibrosis, portal hypertension and nutritional problem. The reported five years-survival rates after Kasai portoenterostomy range from 30 to 60 %. About 20 % of all patients undergoing Kasai portoenterostomy during infancy survive into adulthood with their native liver. Even if Kasai portoenterostomy remains as the first line of treatment In BA, liver transplantation serves as a good salvage treatment when portoenterostomy fails or liver function gradually deteriorates after initially successful establishment of bile flow, Overall 5-year survival rate in BA is about 90 % in recent series.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• 제14권1호
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• pp.74-80
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• 2011

목 적: 선천성 담도폐쇄증의 일차적 치료로 알려진 Kasai 수술에서의 가장 중요한 예후 인자로 수술 시기가 중요하다는 것은 기존의 연구 결과들을 통해 잘 알려져 있다. 본 연구에서는 선천성 담도 폐쇄증 환아들을 수술 시기별로 분류하고 간섬유화 스캔을 수술 전후에 시행하여 얻어진 간탄성률값을 이용하여 수술 전후의 간 섬유화 변화를 비교함으로서 본 연구에서 도출된 최적의 수술시기가 기존의 연구와 일치하는지 재확인 하고자 한다. 방 법: 2007년 10월부터 2010년 7월까지 단일 기관에서 Kasai 수술을 시행 받은 환자 중, 수술 전후로 간섬유화 스캔을 시행한 환자 34명을 대상으로 수술 시기별 간탄성률 결과값을 비교하였다. 결 과: 환자군의 수술 시기가 빠를수록 수술 전과 후 모두에서 간탄성률 결과값은 좋았고, 특히 8주 이전에 수술을 시행 받은 경우 그 이상에서 수술을 받은 환자군보다 수술 후의 간섬유화가 적게 진행되었고 일부에서는 회복되었다. 또한 8주 수술 전후의 각 탄성률 간의 차이값 또한 통계적으로 유의하였다. 결 론: Kasai 수술의 시기가 이를수록 수술 전 간 섬유화가 적고, 적어도 8주 이전에서 Kasai 수술을 시행하였을 때 수술 이후의 간섬유화가 양호하였으며 수술전후의 간섬유화 진행 정도가 크지 않았다. 따라서 본 연구에서는 간섬유화스캔을 바탕으로 적어도 8주 미만의 연령이 Kasai 수술을 시행 받기에 최적의 시기임을 재확인하였다.

• Advances in pediatric surgery
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• 제14권2호
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• pp.125-133
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• 2008

To evaluate the long-term prognosis of biliary atresia after Kasai operation, a total of 14 patients (of the 41 patients operated upon from 1982 to 1997), who had been followed up for more than 10 years, were included in this retrospective study. Eleven out of 14 patients survived with their native livers, and their data analyzed for age at operation, clearing time of jaundice, histological outcome, postoperative complications, effectiveness after the application of an intussusception anti-reflex valve, and quality of life. Average age at surgery was 62.8 days. Serum bilirubin was normalized within three months in all patients. Six among the eleven long-term survivors had ascending cholangitis as one of the postoperative complications. The application of an intussusception anti-reflux valve did not show any statistical significance in long-term survival. Most of long-term survivors appeared to enjoy good quality of life. Kasai operation might not be the definitive treatment for biliary atresia; however, Kasai operation made it possible to achieve long-term survival for patients with biliary atresia when the patients were detected and treated as early as possible.

• 한국산학기술학회논문지
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• 제12권5호
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• pp.2261-2266
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• 2011

초음파 컬러 유동 사상에서 가장 많이 사용되는 Kasai 알고리듬은 래그-1 자기상관 방법으로서 적은 계산량으로 비교적 우수하게 도플러 평균주파수를 추정한다. 하지만 특히 깊은 곳을 영상화하는 경우 낮은 신호 대 잡음비로 인해 추정성능이 저하된다. 본 논문에서는 낮은 신호 대 잡음비에서도 Kasai 알고리듬보다 우수한 디에일리어스된 래그-2 자기상관방법을 제안하고 시뮬레이션을 통해 성능을 검증하였다. 제안한 방법은 잡음이 존재하는 경우 도플러 평균주파수 추정 성능을 평균 자승 오차 측면에서 전반적으로 약 2~3dB 정도 개선시켜줌을 확인하였다.

• Advances in pediatric surgery
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• 제12권2호
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• pp.202-212
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• 2006

The prognostic factors for extrahepatic biliary atresia (EHBA) after Kasai portoenterostomy include the patient's age at portoenterostomy (age), size of bile duct in theporta hepatis (size), clearance of jaundice after operation (clearance) and the surgeon's experience. The aim of this study is to examine the most significant prognostic factor of EHBA after Kasai portoenterostomy. This retrospective study was done in 51 cases of EHBA that received Kasai portoenterostomy by one pediatric surgeon. For the statistical analysis, Kaplan-Meier method, Logrank test and Cox regression test were used. A p value of less than 0.05 was considered to be significant. Fifteen patients were regarded as dead in this study, including nine cases of liver transplantation. There was no significant difference of survival to age. The age is also not a significant risk factor for survival in this study (Cox Regression test; p = 0.63). There was no significant difference in survival in relation to the size of bile duct. However, bile duct size was a significant risk factor for survival (Cox Regression test; p = 0.002). There was a significant difference in relation to survival and clearance (Kaplan-Meier method; p = 0.02). The clearing was also a significant risk factor for survival (Cox Regression test; p = 0.001). The clearance of jaundice is the most significant prognostic factor of EHBA after Kasai portoenterostomy.

• Advances in pediatric surgery
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• 제13권2호
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• pp.135-143
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• 2007

Biliary atresia (BA) is an uncommon neonatal surgical disease that has a fatal outcome if not properly treated. The survival rates of the patients with native liver after Kasai's operation in countries outside Japan are not so good. We reviewed the results of 22 cases of biliary atresia treated in Kosin University Hospital between October 1987 and March 2001. There were 13 males and 9 females aged from 21 to 106 days (mean 52 days). There were 3 cases of Type I (13.6%), and 3 of Type II (13.6%), and 16 Type III (72.7%). The operative methods were resection of the common bile duct remnant and cyst followed by Roux-en-Y hepaticojejunostomy in 3 cases for Type I BA; Kasai I in 15 cases, Kasai II in 1 case, and Ueda's operation in 3 cases for Types II and III BA. There was no death within the first 30 days after operation. We were able to follow 21 of the 22 patients (95.4%) for more than 5 years. The actual 5 year survival rate (YSR) was 40.9%. One Type I case received a living-related liver transplantation at 6 years of age because of the multiple intrahepatic stones and liver cirrhosis. Five YSR after biliostomy group (Kasai II and Ueda op.) was 75 % (3/4) while that of Kasai I was 20% (3/15). One case had no bile duct in the resected fibrotic plaque on microscopic review and died 8 months after Kasai I operation, would have been a strong candidate for early liver transplantation. From the above result, our conclusions are as follows; (1) early liver transplantation should be considered for cases of no bile duct after pathologic examination of the resected specimen, (2) measures to prevent postoperative cholangitis and prevention of postoperative liver cirrhosis are needed, (3) liver transplantation program should be available for failed cases.

• 대한영상의학회지
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• 제83권5호
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• pp.1014-1031
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• 2022

신생아 황달의 주요 원인인 담도폐쇄증은 Kasai portoenterostomy가 기본적인 치료 술식이다. 수술 이후 다양한 합병증이 발생할 수 있는데 portoenterostomy를 통해 발생하는 상행성 담도염이 가장 흔하다. 성공적인 Kasai portoenterostomy 후에도 간 섬유화가 진행할 수 있어 이로 인한 간경화, 문맥 고혈압으로 인한 합병증이 많은 경우에서 발생하게 된다. 합병증이 조절되지 않거나 진행성 고빌리루빈혈증이 있는 경우 간이식을 시행하게 된다. Kasai portoenterostomy 이후 합병증 확인 및 장기 생존 환자의 추적 검사로 다양한 영상 진단이 주요 역할을 하고 있다. 또한 간이식이 필요한 경우 이식 전후로 공여자 및 수혜자 모두에서 수술 금기 확인 및 합병증 조기 발견을 위해 영상 검사들이 중요한 역할을 한다. 초음파 검사가 추적 관찰에 가장 먼저 사용되는 유용한 검사이며 추가적으로 CT 및 MRI를 통해 진단에 도움을 받을 수 있다.

• Advances in pediatric surgery
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• 제13권1호
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• pp.1-12
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• 2007

Biliary atresia (BA) is the result of fibrosing destructive inflammatory process affecting intrahepatic and extrahepatic bile ducts, which lead to cirrhosis and portal hypertension. Kasai portoenterostomy has been the standard operative procedure in biliary atresia. Recently, there has been remarkable increase in the survival rate in cases of BA. However, long-term survivors are not clearly evaluated in Korea. To define long-term prognosis factors of patients who underwent surgery for BA, a retrospective study was undertaken of 10 (37 %) patients surviving more than 10 years among 27 patients who underwent one of Kasai procedures between 1981 and 1995. Hepatomegaly was present in 4 and splenomegaly in 7 patients. Serum bilirubin was normalized at 1 year after operation. Aspartate aminotransferase (AST, GOT), Alanine aminotransferase(ALT, GPT) were normalized at 12 years and alkaline phosphatase(ALP) was normalized at 13.5 years. Cholangitis developed mainly within 5 years after operation so close follow up is needed. Life long follow-up is needed because of progressive deterioration of liver function even after 10 years.

• 대한영상의학회지
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• 제83권5호
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• pp.979-990
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• 2022

담도폐쇄증은 간외 담도의 진행성, 특발성, 섬유 폐쇄성 염증질환으로 신생아기에 담도 폐쇄가 나타나고 소아 간이식의 가장 흔한 적응증이다. 조기에 치료하지 않으면 간경화가 진행되어 2세 이전에 사망하게 된다. 오늘날 Kasai portoenterostomy와 간이식 수술기법의 발달로 담도폐쇄증 환자의 90% 이상이 성인기까지 생존한다. 따라서 Kasai portoenterostomy의 성공 가능성은 시간이 지날수록 줄어들기 때문에 조기 진단이 매우 중요하다. 이 고찰은 담도폐쇄증의 원인, 분류, 유병률, 임상양상, 치료와 예후를 최신 지견을 중심으로 종합적으로 검토하고자 한다.