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

• 약학회지
• /
• 제46권2호
• /
• pp.129-136
• /
• 2002

The pathogenesis of cholestatic liver injury as well as the modulation of hepatic fibrogenesis is causally associated with involvement of reactive oxygen species (ROS) and free radical reactions. In this study, we investigated whether dried extracts of oriental medicine (LH) have antioxidant and antifibrotic effect under the biliary liver fibrosis (cirrhosis) c ondition. The female Sprague-Dawley rats were divided in six groups (Normal, N-LH, op-2, op-4, opLH-2, opLH-4) and were observed in 2 weeks or 4 weeks. For this purpose the rats were operated by bile duct ligation/scission (BDL/S), which induced to liver fibrosis and cirrhosis. After surgery, the prepared LH was administered p.o. 2 mι/day/rat in 2 weeks or 4 weeks for opLH groups. During the observation period, jaundices appeared in eyes, ears and tail of all BDL/S operated rats. And at the time of sacrifice, cholestasis was observed in proximal bile duct, especially the color of bile juice and urine in opLH-4 group showed more clear than op-2, op-4 and opLH-2 group. The value of clinical parameters and product of lipid peroxidation (MDA) in sera and the hydroxyproline (hyp) content in liver tissue were significantly increased in all liver fibrosis (cirrhosis) developed rats (p＜0.001~0.05). Among the clinical parameters of sera, value of BUN, ALP in opLH-4 group showed significantly lower than in op-4 group (p＜0.05, p＜0.001). The content of hyp in opLH-2, opLH-4 group (478.0 $\pm$ 134.3 $\mu\textrm{g}$/g 897.5 $\pm$ 118.2 $\mu\textrm{g}$/g) showed lower than in op-2, op-4 group (528.9 $\pm$ 220.7 $\mu\textrm{g}$/g, 1023.8 $\pm$ 277.1 $\mu\textrm{g}$/g) and then the value of MDA in opLH-4 was also significantly reduced to 59.4% of that in op-4 group (p＜0.001). The histological change (bile duct proliferation, fibrosis, collagen bundle) was similarly observed in op-2 group and in opLH-2 group, but the weak fibrosis and bile duct proliferation were observed in opLH-4 group compared with in op-4 group. Our data indicate that the 4 weeks treatment with LH extract suppressed lipid peroxidation and inhibited fibrotic (cirrhosis) process, and experimental cholestatic liver disease is associated with increased lipid peroxidation in BDL/S operated rats. Hence we concluded that the measurement of MDA and hyp can be useful monitor for the screening of antioxidant and antifibrotic effect in experimental liver fibrosis (cirrhosis), and LH has been shown to have hepatoprotective effect, antifibrotic effect and antioxidant effect.

• 약학회지
• /
• 제41권2호
• /
• pp.220-224
• /
• 1997

This study was carried out to investigate the dose dependent antifibrotic effects of polysaccharide from mycelium of Ganoderma lucidum. The experimental hepatic cirrhosis was induced by bile duct ligation/scission (BDL/S) in rats. BDL/S rats in each group were dosed 0.5 mg, 2.0 mg, 5.0 mg or 10.O mg/rat/day orally for 4 weeks after the operation. Antifibrotic effects were evaluated by serum biochemical values, hydroxyproline contents, and light microscopical histology. The results obtained were as follows: 1) Hydroxyproline contents in liver of 5.0 and 10.0mg polysaccharide-treated BDL/S rats were significantly reduced 2) In serum test, ALT, AST, ALP values in polysaccharide from Ganoderma lucidum-treated group were lower than BDL/S control group 3) The hepatic damage such as hepatocellular necrosis, inflammation, bile duct proliferation and fibrosis was less severe in the livers of 2.0 mg and 5.0 mg polysaccharide-treated rats. These results suggest that polysaccharide from mycelium of Ganoderma lucidum to be a promising agent for the inhibition of hepatic cirrhosis.

• Clinical and Experimental Pediatrics
• /
• 제48권1호
• /
• pp.104-107
• /
• 2005

저자들은 20개월 유아에서 복통이나 담즙정체 등의 특이 증상이 동반되지 않으면서도 간, 비장종대를 동반하고 간 조직검사에서 간경화가 있었으나, 종괴제거 수술 후 간기능검사 뿐 아니라 간, 비장 종대가 급격하게 해결된 총수담관낭을 경험하였다. 총수담관낭을 조기에 발견하여 적절하게 제거하는 것이 간기능 검사의 호전 뿐 아니라 조직학적으로 입증된 간경화를 호전시키는 데에도 중요함을 시사한다고 하겠다.

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

• 약학회지
• /
• 제45권5호
• /
• pp.513-521
• /
• 2001

The chronic cholestasis induce to biliary liver fibrosis (cirrhosis) and the increased products of ROS(reactive oxygen species) cause to the liver damage. In this study ; the antioxidant and antifibrotic effect of dried extracts of oriental medicine (DW) was investigated under the liver fibrotic (cirrhotic) condition. The female Sprague-Dawley rats were divided in 5 groups (Normal, Op-2, Op-4, OpDW-2, OpDW-4). Except for normal group, the rats were induced to biliary liver fibrosis (cirrhosis) by the operation of bile duct ligation/scission (BDU/S) and were observed in 2 weeks or 4 weeks. And the prepared DW was treated p.o.2 ml/day/rats in 2 weeks or 4 weeks for OpDW groups. At the time of sacrifice, the liver, kidney, spleen were weighed and the ratio of organ weight/body weight was calculated. The MDA, the hyp and biochemical parameters (GOT GTP, ALP, t-bili) were measured in sera and liver tissue of rats. The biochemical change was observed on liver tissue. In the result, the hepatomegaly and spleenomegaly appeared in all BDL/S operated rats, and significantly lower liver weight was observed in OpDW-4 group compared with in Op-4 group (p<0.05). The level of clinical parameters in sera of all liver fibrosis (cirrhosis) developed rats was higher than in normal group. Especial1y, the value of GOT in OpDW-2 group and ALP in OpDW-4 group showed significantly lower than in Op-2 group and Op-4 group (p<0.01, p<0.005). The content of hyp in all operation groups was significantly higher than in normal group (p<0.05∼<0.005), and showed significantly lower value in the OpDW-4 group than in Op-4 group (p<0.05). The product of lipid peroxidationUDA) increased significantly under the fibrotic(cirrhotic) condition (p<0.05∼ <0.005), and the MDA value in OpDW-4 group decreased significantly in Op-4 group (p<0.005). The histological change (bile duct proliferation, fibrosis, collagen bundle) was similarly observed in Op-2 group and in OpDW-2 group, but the weak fibrosis and bile duct proliferation were observed in OpDW-4 group compared with in Op-4 group. In conclusion, lipid peroxidation and severe liver damage were activated by bile duct obstruction, and the measurement of MDA and hap can be useful monitor for the screening of antioxidant and antifibrotic effect in experimental liver fibrosis (cirrhosis). The 4 weeks treatment with DW extracts suppressed lipid peroxidation and inhibited fibrotic (cirrhotic) process in BDL/S operated rats.

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

• Tuberculosis and Respiratory Diseases
• /
• 제62권5호
• /
• pp.421-426
• /
• 2007

진행성 간질환의 합병증으로 발생하는 문맥폐고혈압은, 이차성 폐동맥고혈압 중에서 진행성 간질환에 의한 문맥고혈압과 연관된 폐동맥고혈압으로 분류되며, 독특한 임상적, 병태생리학적 특징을 보인다. 임상양상은 점진적인 운동성 호흡곤란, 흉통 등을 호소하지만, 증상 없이 우연히 발견되는 경우도 있다. 심초음파 검사를 통해 예비적으로, 우심장 도관삽입으로 직접 우심실 및 평균 폐동맥압을 측정하여 진단할 수 있다. 치료는 칼슘통로차단제, 프로스타노이드 등을 사용하지만, 근본적인 치료를 위해서는 간 및 폐이식을 고려해야 한다. 저자들은 53세 여자로 20년 전에 간경변을 진단받은 후 호흡곤란으로 내원한 환자에게서, 간경변의 원인으로 원발성 담관성 간경변을 진단하고, 호흡곤란의 원인으로 문맥폐고혈압을 진단하여 치료하였기에 문헌고찰과 함께 보고한다.

• 대한핵의학회지
• /
• 제12권2호
• /
• pp.17-22
• /
• 1978

Carcinoembryonic antigen was initially known as tumor specific antigen and had a potential diagnostic value in the detection of digestive tract malignancies. However, subsequent studies showed CEA and CEA-like antigen present in benign disease, particullary in liver. We had collected sera from 58 patients who had liver scan and later were diagnosed clinically and histologically as liver disease. We estimated CEA values and correlations were made with liver function tests in liver cirrhosis cases. The results: 1) The raised plasma carcinoembryonic antigen level were found in 13 (68.4%) of 19 patients in liver cirrhosis, 5(27.8%) of 18 patients in hepatoma, 5(71. %) of 7 patients in chronic active hepatitis, all 3 patients in liver abscesses, 2(66.7%) of 3 patients in liver ablscesses, 2(66.7%) of 3 patients in obstructive biliary disease and none in each one patient of traumatic liver hematoma, subphrenic abscess and clonorchiasis. 2) There is no linear correlation between carcinoembryodic antigen level and liver function tests including serum bilirubin, alkaline phosphatase, SGOT and prothrombin time in liver cirrhosis patients.

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