• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• 제2권2호
• /
• pp.262-266
• /
• 1999

Obstruction of the extrahepatic bile ducts is the most common cause of conjugated hyperbilirubinemia in early infancy. More than 90% of such obstructive lesions are accounted for by extrahepatic biliary atresia. A rare lesion is obstruction of the common duct by impacted, thickened secretions and bile. Bile plug syndrome is defined as extrahepatic obstruction of the bile ducts by bile sludge in term infants without anatomic abnormalities, congenital chemical defects of bile, or hepatocellular lesions. Obstruction of extrahepatic ducts by plugs of biliary material apperas to be due to the inspissation and precipitation of bile and mucus within the lumen of the ducts. Cholestasis and precipitation of bile develop in association with abnormal composition of bile in cystic fibrosis, hepatocellular damage, prolonged erythroblastic jaundice, altered biliary dynamics with total parenteral nutrition, gut dysfunction, diuretic therapy, exchange transfusions and perinatal hemolysis. In those cases, the term inspissated bile syndrome is used. The clinical and laboratory findings in bile plug syndrome are identical to those observed in biliary atresia and choledochal cyst. The diagnosis can be suspected based on the findings of clinical and laboratory examinations together with hepatobiliary imaging, ultrasonography, radionuclide scan and liver biopsy. We experienced a case of spontaneous resolution of bile plug syndrome in a 4-year-old girl. We report this case with brief review related literatures.

• Applied Microscopy
• /
• 제39권2호
• /
• pp.117-124
• /
• 2009

간흡충(Clonorchis sinensis)은 우리나라에서 높은 감염률(2.9%)을 나타내는 기생충으로(KAHP, 2004), 간흡충에 감염된 담관은 간흡충의 흡반에 의한 물리적 자극과 대사산물 및 분비물 등의 화학적 자극에 의해 담관염이 일어나고, 간흡충이 성장할 때 충체 주위의 담관 상피세포의 증식, 탈락, 담관 주위의 염증 및 섬유화가 일어난다. 담관 점막에 분포하는 섬유모세포는 결합조직을 구성하는 세포의 한 종류로서 세포질돌기들이 잘 발달된 형태적 특징이 있으며, 세포질 내에 세포의 형태 유지, 신호전달, 인접세포와의 연접 등에 관여한다. 또한 조직을 발달시키고, 조직이 손상된 부위에서 콜라겐 층을 형성하여 손상된 조직이 복구되도록 하기도 한다. 상처의 반흔(scar) 형성과 지방축적, 염증(inflammation) 발생 과정에서 섬유모세포의 작용이 제대로 조절되지 못하면 섬유증(fibrosis)이 발달하게 된다는 연구 보고도 있다. 본 연구에서는 정상 흰쥐에서 분리된 담관 섬유모세포와 간흡충 감염 흰쥐에서 분리된 담관 섬유모세포를 배양하고, 각각의 실험군에 간흡충 분비배설 물질(Cs excretory-secretory product, ESP)을 첨가하여 배양하였다. 배양된 섬유모세포의 미세구조 변화와 세포 표면에 존재하는 sialic acid 및 actin의 분포를 전자현미경으로 관찰하여, 간흡충 감염에 따른 섬유모세포의 변화 및 간흡충 분비배설 물질의 자극에 따른 섬유모세포의 변화를 관찰하여 흰쥐의 담관 섬유모세포와 간흡충 감염과의 연관성을 알아보고자 하였다. 정상 흰쥐 담관에서 분리한 섬유모세포(G1)에 비하여 간흡충에 감염된 흰쥐 담관에서 분리한 섬유모세포(G2)와 간흡충 분비배설 물질을 첨가하여 배양한 섬유모세포(G1-1, G2-1)의 증식속도가 느린 것이 확인되었다. 세포질돌기의 수는 간흡충에 감염된 흰쥐의 담관으로부터 분리된 섬유모세포(G2)에서 가장 많은 수가 관찰되었고, 배양배지에 분비배설 물질을 첨가하면 정상 담관의 섬유모세포에서도 세포질돌기가 증가하였다. 따라서 간흡충 대사물질은 섬유모세포의 세포질돌기형성을 촉진시키는 것으로 생각된다. 간흡충에 감염된 흰쥐 담관에서 분리한 섬유모세포(G2)의 소포체는 정상 담관에서 분리된 섬유모세포(G1)의 것에 비하여 감소하는 양상을 나타내었고, 여기에 분비배설 물질을 첨가하면 섬유모세포의 소포체가 증가하는 것이 관찰되었다. 그리고 세포표면에 분비되는 sialic acid는 주로 세포질의 소포낭 주변에서 관찰되었으며, 정상 섬유모세포(G1, G1-1)보다 감염된 섬유모세포(G2, G2-1)에서 증가하였다. Actin은 세포표면과 세포질돌기에서 주로 관찰되었으며, 정상 섬유모세포(G1, G1-1)보다 감염된 섬유모세포(G2, G2-1)에서 반응이 증가하였고, 간흡충 분비배설 물질을 첨가하면 G1-1은 반응이 증가하고, G2-1은 반응이 감소하는 것이 관찰되었다. 이상의 결과로 간흡충에 감염된 흰쥐 담관의 섬유모세포는 세포질돌기들이 매우 발달하며, actin단백과 sialic acid가 증가하여 세포변형을 초래하게 된다. 또한 간흡충 감염으로 손상된 담관의 섬유모세포로 구성된 결합조직은 정상으로 회복되지 않으며, 세포질 부피 및 세포질돌기의 증가는 이루어지지만 간흡충 대사물질의 영향으로 섬유모세포의 분열 및 성장 속도가 억제되는 것으로 확인되었다. 이 결과 간흡충 감염으로 손상된 숙주의 담관 결합조직과 섬유모세포들은 간흡충 대사물질에 의하여 변형을 일으키고, 세포 활성 및 증식이 저하되므로 팽대된 담관은 간흡충이 사멸된다 하더라도 원상회복이 불가능할 것으로 생각된다.

• Advances in pediatric surgery
• /
• 제14권2호
• /
• pp.178-182
• /
• 2008

Forme fruste choledochal cyst (FFCC) is one of the diverse types of choledochal cyst with little or no dilatation of the extrahepatic bile duct (EHBD). It is considered that FFCC has to do with the pancreatobiliary malunion (PBM). In children, 3 to 6 millimeters of EHBD is assumed to be normal. Even though there is no clear-cut definition, FFCC is likely to be associated with bile duct dilatation less than 10 millimeters. Almost all cases have PBMs and symptoms of the pancreatitis or cholangitis. We experienced a case of FFCC in a 4-year-old boy. His EHBD measures 10 mm diameter. He had symptoms of pancreatitis and elevated hepatic transaminases. The pancreatobiliary common channel was 28 millimeters. He underwent EHBD resection and Roux-en-Y hepaticojejunostomy and was discharged with no specific complications.

• Advances in pediatric surgery
• /
• 제3권2호
• /
• pp.98-107
• /
• 1997

To determine whether bile juice exclusion can prevent the mucosal damage, and Insulin-like growth factor-I can promote mucosal regeneration in ischemia-reperfusion injury of the bowel, 39 weanling rats with 10 cm of Thiry-Vella loop were studied. Animal groups were; Control, BL(common bile duct ligation), IGF{insulin-like growth factor-I(IGF-I) infusion} and IGF-BL(combined treatment). IGF-I(1.5 mg/kg/day) was continuously delivered through a subcutaneously implanted miniosmotic pump. After 15 minutes of superior mesenteric artery clamping, a tissue specimen(P) was taken after 30 minutes of reperfusion. Intestinal continuity was restored to allow oral feeding. A specimen of main tract(M) and another of the Thiry-Vella loop(T) were collected for histomorphometry after 48 hours of reperfusion and free feeding. Villus size ratio(VSR), crypt depth(CD), crypt-depth/villus-height ratio(CVR) and injury score(IS) were measured in 15 consecutive villi. The postoperative mortalities of bile duct ligation groups(BL and IGF-BL) were higher than those of other groups. In control group, VSR of M was lower(P<0.05) than P or T, but not in the other groups. VSR of M in control was lower than those in other groups. CD of T in control, IGF and IGF-BL group were higher than those of M. CD of M and T showed gradual increments from control, IGF and IGF-BL group, respectively. CVR of M and T in IGF group were higher than those in control. CVR in IGF-BL group, T was higher than M, and M was higher than P. About IS, M of BL($20.1{\pm}2.5$) and IGF-BL($20.9{\pm}3.3$) groups were significantly lower than that of control($32.4{\pm}2.5$). These results suggest that the exclusion of bile juice reduces the severity of the reperfusion injury of the mucosa, by inability to activate pancreatic enzymes and IGF-I stimulates mucosal regeneration in injured bowel, and the effect is potentiated by bile juice exclusion.

• Journal of Yeungnam Medical Science
• /
• 제7권2호
• /
• pp.197-202
• /
• 1990

저자들은 최근 4일간의 우측 상복부 동통 및 경미한 황달을 주소로 내원한 71세 남자 환자에서 임상적으로 경피적 간담관조영술, 복부 초음파검사, 간기능 검사, 복강 동맥 조영술을 시행하여 Type II Mirizzi 증후군으로 진단된 1예를 치험하였기에 문헌고찰과 함께 보고하는 바이다.

• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• 제19권4호
• /
• pp.286-290
• /
• 2016

Inspissated bile syndrome (IBS) is a rare condition in which thick intraluminal bile, including bile plugs, sludge, or stones, blocks the extrahepatic bile ducts in an infant. A 5-week-old female infant was admitted for evaluation of jaundice and acholic stool. Diagnostic tests, including ultrasound sonography, magnetic resonance cholangiopancreatography, and a hepatobiliary scan, were not conclusive. Although the diagnosis was unclear, the clinical and laboratory findings improved gradually on administration of urodeoxycholic acid and lipid emulsion containing omega-3 polyunsaturated fatty acids (PUFAs) for 3 weeks. However, a liver biopsy was suggestive of biliary atresia. This finding forced us to perform intraoperative cholangiography, which revealed a patent common bile duct with impacted thick bile. We performed normal saline irrigation and the symptom was improved, the final diagnosis was IBS. Thus, we herein report that IBS can be treated with omega-3 PUFAs as an alternative to surgical intervention.

• Radiation Oncology Journal
• /
• 제9권2호
• /
• pp.253-263
• /
• 1991

1985년부터 1990년까지 연세대학교, 연세암센터 치료방사선과에 내원하였던 27명의 간외담도종양환자를 대상으로 외부방사선조사 및 관내 근접조사(intraluminal brachytherapy)를 병용하였을 경우 적절한 국소관해율을 얻을 수 있는지에 대한 후향적 분석을 하였고 이러한 치료방법에 대한 결과를 불완전한 절제술후 방사선치료를 시행한 군의 치료성적과 비교분석하였다. 16명은 남성이었고 11명은 여성이었으며, 평균나이는 58세($34\~70$세)였다. 외부방사선치료는 10 MV Linear Accelerator, 4 문조사방법을 이용하였고 총 조사량은 $45\~55$ Gy이었다. 관내 근접조사는 외부방사선조사후 시행하였고 Ir-192를 사용한 고선량치료기기인 Gamma-Med 12i를 이용하여 총 15Gy를 조사하였다. 최소 추적조사기간은 12개월이었다. 치료방법에 따라 3개의 군으로 대별해 보았는데, 1군(6명)은 불완전 절제술후 미시적 잔류암존재로 방사선치료를 시행했던 경우이고 2군(13명)은 수술불가능하여 방사선치료 단독으로 치료하였던 경우이며, 3군(8명)은 외부방사선조사와 관내근접조사를 병용하였던 경우로 분류하였다. 치료실패요인은 대부분 국소실패였고 원격전이는 관찰되지 않았다. 중앙생존기간은 10개월이었고 2년 생존율은 $30\%$이었다. 각 치료군에 따른 치료실패율은 1군 $67\%(4/6),\;2군\;79\%(10/13),\;3군\;38\%(3/8)$이었으며 중앙생존기간은 각각 10개월, 6개월, 10개월이었다. 또한 2년 생존율은 각각 $56\%,\;18\%,\;50\%$로서 종합적으로 1군과 3군간의 생존율과 중앙생존기간은 의미있는 차이는 없었다. 따라서 본 저자들은 간외담도종양 환자의 경우 외부 방사선치료만으로는 만족할 만한 결과를 얻을 수 없었으며 외부방사선 치료와 관내근접조사를 병용하였을 경우 수술적 절제술이 시행되었던 경우와 비슷한 치료성적을 얻었기에 이를 보고하는 바이며 향후 이러한 결과를 바탕으로 생존율을 향상시키기 위해 적극적인 방사선치료 방법이 모색되어야 하리라 생각하는 바이다.

• Nuclear Medicine and Molecular Imaging
• /
• 제42권3호
• /
• pp.253-255
• /
• 2008

A 61-year-old man had a F-18 FDG PET/CT scan for evaluation of a common bile duct cancer identified on CT. The PET/CT image showed a hypermetabolic mass in the common bile duct, and a focal area of increased F-18 FDG uptake in segment IV of the liver, which corresponded to a hypoattenuated lesion on non-enhanced CT, and was consistent with hepatic metastasis. The patient underwent choledochojejunostomy with hepatic resection, and pathologic findings were compatible with an eosinophilic abscess in the liver. This case demonstrates that F-18 FDG uptake by an eosinophilic abscess can mimic hepatic metastasis in a patient with a malignancy.

• 대한의생명과학회지
• /
• 제10권2호
• /
• pp.93-98
• /
• 2004

The effects of intravenously administered of high concentration of taurocholic acid (TCA) on $\alpha$-D- and $\beta$-D-mannosidase activities in rat liver lysosomes were studied. These liver lysosomal enzymes, and serum lysosomal $\alpha$-D- and $\beta$-D-mannosidase isozymes activities were determined in experimental rats with common bile duct ligation (CBDL). The liver lysosomal $\beta$-D-mannosidase activity as well as the serum lysosomal $\alpha$-D- and $\beta$-D-mannosidase isozymes activities were found to be significantly increased in the CBDL plus TCA injected group than in the control group such as CBDL alone group. However, the liver lysosomal $\alpha$-D-mannosidase activity was found to be significantly decreased in the CBDL plus TCA injected group. The above results suggest that TCA repress the biosynthesis of the lysosomal $\alpha$-D-mannosidase and induce the biosynthesis of the lysosomal $\beta$-D-mannosidase in the liver. And that the elevated serum lysosomal $\alpha$-D- and $\beta$-D-mannosidase isozymes activities are most likely due to increased hepatocyte membrane permeability caused by TCA mediated liver cell necrosis.

• 대한의생명과학회지
• /
• 제10권2호
• /
• pp.99-105
• /
• 2004

Liver and serum rhodanese activities were determined in acute ethanol intoxicated rats with extrahepatic cholestasis induced by common bile duct ligation (CBD) to manifest the biochemical background of alcohol drinking hazard under the hepatobiliary disease. Liver cytosolic and microsomal rhodanese activities and these Vmax values in CBD ligated rats with acute ethanol intoxication were found to be decreased much more than that in CBD ligation alone. However, the difference of Km value on above hepatic enzyme was not found between the experimental groups. On the other hand, serum rhodanese activity in CBD ligated rats with acute ethanol intoxication was greater increased more than that in CBD ligation alone. These results indicate that the biosynthesis of the hepatic rhodanese decreases and the serum rhodanese activity increases in cholestasis combined with acute ethanol intoxication, reflecting damage of aggravated hapatocytic membrane. Accordingly, the resulting data supported the fact that alcoholic drinks were enzymologically harmful to the hepatobiliary disease.