• Title/Summary/Keyword: Bile duct disease

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Oral Vancomycin Therapy in a Child with Primary Sclerosing Cholangitis and Severe Ulcerative Colitis

  • Buness, Cynthia;Lindor, Keith D.;Miloh, Tamir
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.19 no.3
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    • pp.210-213
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    • 2016
  • Primary sclerosing cholangitis (PSC), a rare progressive liver disease characterized by cholestasis and bile duct fibrosis, has no accepted, effective therapy known to delay or arrest its progression. We report a 15 year old female patient diagnosed with PSC and moderate chronic active ulcerative colitis (UC) who achieved normalization of her liver enzymes and bile ducts, and resolution of her UC symptoms with colonic mucosal healing, after treatment with a single drug therapy of the antibiotic oral vancomycin. We postulate that the oral vancomycin may be acting both as an antibiotic by altering the intestinal microbiome and as an immunomodulator. Oral vancomycin may be a promising treatment for PSC that needs to be further studied in randomized trials.

Treatment Outcome of Postoperative Radiotherapy in Extrahepatic Bile Duct Cancer (간외담관암의 수술 후 방사선치료 성적)

  • Choi, Young-Min;Cho, Heung-Lae
    • Radiation Oncology Journal
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    • v.21 no.1
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    • pp.27-34
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    • 2003
  • Purpose : To evaluate the efficacy of radiotherapy, and the factors affecting survival in patients of extrahepatic bile duct cancer, by analyzing the results of postoperative radiotherapy Materials and Methods : Between October 1991 and July 2001, 21 patients with extrahepatic bile duct cancer, who received radiotherapy after a radical resection, were retrospectively reviewed. The patients' ages ranged from 39 to 75 years, with a median of 61 years, and a male to female ratio of 16 to 5. The numbers of patients with proximal and distal bile duct cancer were 14 and 7, respectively. From the postoperative pathological examination, 19 of the patients were found to have microscopic residues, and 7 to be lymph node positive. Patients with AJCC stages I, II and III were 10, 10 and 1, respectively. The total radiation dose administered was 4,500$\~$6,300 cGy, with a median dose of 5,040 cGy. The follow up period was 20$\~$81 months, with a median of 57.5 months. Results : The overall and disease free survival rates at 3 and 5 years were 41.0 and 29.3$\%$, and 41.6 and 29.7$\%$, respectively. The influences of age, sex, tumor location, differentiation, microscopic residue, neural invasion, 7 and N stage, the stage itself, the dose of radiation and chemotherapy, on survival were evaluated. The T stage and the stage itself were found to be significant from a univariate analysis (p<0.05), but the degree of significance was limited by the small number of patients. A recurrence occurred in 12 patients (57.1$\%$), 5 in locoregional sites, 4 in distant sites and 3 in a combination of locoregional and distant sites, and the sites of distant metastasis were the liver, 6, and the bone, 1 Grade 2 or 3 acute leucopenias occurred in 2 patients and grade 2 chronic peptic ulcers occurred in 4, who were all recovered by conservative management. Conclusion : Postoperative radiotherapy is feasigbls in extrah데atic bile duct cancer, with tolerable toxicity, but prospective studies, with a large patient enrollment, are needed for the evaluation of the effects of postoperative radiotherapy and the related prognostic factors.

A CASE OF TYPE II7 MIRIZZI SYNDROME (Type II Mirizzi 증후군 1례)

  • Kim, Hong-Jin;Lee, Joo-Hyeong;Shin, Myeong-Jun;Kwun, Koing-Bo;Chang, Jae-Chun;Chung, Moon-Kwan
    • Journal of Yeungnam Medical Science
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    • v.7 no.2
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    • pp.197-202
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    • 1990
  • Mechanical obstruction of the common hepatic duct includes the following causes ; choledocholithiasis, sclerosis, cholangitis, pancreatic carcinoma, cholangiocarcinoma, postoperative stricture, primary hepatic duct carcinoma, enlarged cystic duct lymph nodes, and metastatic nodal involvement of the porta hepatis. Partial mechanical obstruction of the common hepatic duct caused by impaction of stones and inflammation surrounding the vicinity of the neck of the gallbladder had been reported on the "syndrome del conducto hepatico" in 1948 by Mirizzi. Nowadays, this disease was named by Mirizzi syndrome. Mirizzi syndrome is a rare entity of common hepatic duct obstruction that results from an inflammatory response secondary to a gallstone impacted in the cystic duct or neck of the gallbladder. It results from an almost parallel course and low insertion of the cystic duct into the common hepatic duct. In a varient of Mirizzi's syndrome, the cause of the common hepatic duct obstruction was a primary cystic duct carcinoma rather than gallstone disease. A 71-year-old man was admitted with a four-day history of right upper quadrant abdominal pain. Past medical history was unremarkable. On physical examination, the patient had a temperature of $38^{\circ}C$, icteric sclera and right upper quadrant tenderness. Pertinent laboratory findings included WBC 18,000/$cm^2$;albumin 2.6g/dl(normal 3.9-5.1) ; SGOT 183u/L(normal 0-50) ; SGPT167u/L(normal 0-65) ; bilirubin, 8.2mg/dl(normal 0-1) with the direct bilirubin, 4.4mg/dl(normal 0-0.4). Ultrasonography revealed a dilated extrahepatic biliary tree. ERCP showed that the superior margin was angular and more consistent with a calculus causing partial CHD obstruction(Mirizzi syndrome). At surgery a diseased gallbladder containing calculi was found. In addition, there was two calculi partially eroding through the proximal portion of the cystic duct and compressing the common hepatic duct. A cholecystectomy and excision of common bile duct was performed, with Roux-en-Y hepaticojejunostomy. The postoperative course was uneventful.

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Primary Sclerosing Cholangitis -One Case Report- (원발성 경화성 담관염 1예 보고)

  • Park, T.J.;Min, H.S.;Suh, B.Y.;Kwun, K.B.;Lee, Tae-Suk
    • Journal of Yeungnam Medical Science
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    • v.3 no.1
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    • pp.351-355
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    • 1986
  • Sclerosing Cholangitis is an uncommon disease which involves either all or part of the extrahepatic biliary duct system and, occassionally, affects the intrahepatic biliary radicles. The disease has also been called "obliterative cholangitis" and "stenosing cholangitis", in reference to a progressive thickening of the bile duct walls encroaching upon the lumen. Several authors have proposed that the temr "primary sclerosing cholangitis" be reserved for cases in which there arew no associated diseases, and that all other cases be classified as "secondary sclerosing cholangitis". Many rigid criterias have been established for the diagnosis of primary sclerosing cholangitis. Recently the authors experienced one case of primary sclerosing cholangitis which was coincided with rigid criterias and was confirmed by operation with histologic examination. We present our case and review the literatures.

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Catechol-O-Methyltransferase Activity in Cholestatic Rat's Liver Induced by Bile Duct Ligation

  • Mun, Kyo-Cheol
    • BMB Reports
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    • v.29 no.2
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    • pp.142-145
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    • 1996
  • To investigate the cause of increased plasma catecholamine levels in liver disease, catechol-O-methyltransferase (COMT), which provides a major route of catabolism for circulating catecholamines, was studied under the cholestasis induced by mechanical biliary obstruction in rats. Monoamine oxidase (MAO) activity and the $K_m$ and $V_{max}$ values for both enzymes were also measured. Cytosolic, microsomal, and mitochondrial COMT activities in the cholestatic liver were significantly decreased throughout the experiment. Microsomal, and mitochondrial MAO activity in the cholestatic liver were also significantly decreased. Vmax values of COMT and MAO were lower. Serum COMT and MAO activities were detected after CBD ligation. These results indicate that plasma catecholamine levels are increased in liver disease due to decreased hepatic degradation of catecholamines by decreased activities of COMT and MAO. The decreased activity of these enzymes is caused by decreased biosynthesis and by flowage into the blood from the damaged hepatocyte.

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A Study on the Hepatoprotective effect of PS-1 from Artemisia iwayomogi (한인전 추출물의 간장보호 작용에 관한 연구)

  • 이순복;조태순;윤기욱;이종찬;이선미;심성보
    • Biomolecules & Therapeutics
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    • v.6 no.2
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    • pp.119-129
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    • 1998
  • The dose-response effect of polysaccharide extracts(PS-1) from Artemisia iwayomogi was inves-tigated under various hepatic disease models. Silymarin, DDB and UDCA were used as reference compounds. We found that the maximal effective dose of PS-1 was 100 mg/kg b.wt. in $CCl_4$-induced hepatotoxicity, D-galactosamine-induced hepatitis, in ANIT-induced cholestasis and 300 mg/kg b.wt. in $CCl_4$-induced chronic liver disease, 30 mg/tg b.wt. in chronic bile duct ligation and chronic ethanol fatty liver. These findings suggest that PS-1 fraction protects the hepatocyte against various hepatic injuries, and this fracton might be of therapeutic value.

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Experience of Biliary Atresia-Long-term Survival (담도 폐색증 환자의 수술 치험 22례 와 장기 생존율)

  • Choi, Kyung-Hyun;Yoo, Jung-Jae;Shin, Yeon-Myung;Hur, Bang;Park, Jae-Sun
    • Advances in pediatric surgery
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    • v.13 no.2
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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.

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The Antifibrotic Effects of D-penicillamine in Liver Fibrosis Animal (간섬유화 동물에서 D-페니실라민의 항섬유화 효과 검색)

  • Kim, Gi-Yeong;Yun, Gi-Jung;Mun, Hyeong-Bae
    • YAKHAK HOEJI
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    • v.40 no.5
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    • pp.550-557
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    • 1996
  • One of therapeutics in liver disease (morbus wilson) is D-penicillamin (D-pen: D-3-mercapto-valin). Especially the cross-linking of collagen molecules could be inhibited by D-pe n in extracellular space. In this study we investigated the antifibrotic effects of D-pen in rats that were induced the liver fibrosis by bile duct ligation and scission (BDL/S). Rats were treated for 4 weeks with D-pen after BDL/S operation or sham operation. The balance between fibrogenesis-marker (PNIIIP) and the fibrolysis-maker (PNIVP) were observed in sera by RIA (radioimmunoassay), and the parameter of collagen deposition in liver tissue (hydroxyproline: HYP) was measured by colorimetry. The weight of liver in BDL/S operated group was increased significantly in compared with sham operation group (15.2g${\pm}$1.1, vs 11.9g${\pm}$3.9: p<0.005, p<0.05). The rats group treated by D-pen showed the lower level of PNIIIP (6.7ng/ml${\pm}$1.5, vs 9.5ng/ml${\pm}$2.8) and the higher value of PIVCP (14.0ng/ml${\pm}$1.9, vs 7.9ng/ml${\pm}$1.5) in sera that compared to untreated rats. The content of HYP was decreased by 141% in BDL/S with D-pen treated group than that of it in BDL/S group. No correlation was revealed between collagen parameters in sera and HYP in liver tissue of BDL/S operated and D-pen treated rats. The group treated with D-pen showed the lower value of clinical biochemistry parameters (GOT: glutamate oxalacetate transaminase, Total-Bilirubin) in compared with only BDL/S operated rats, but the value of GPT (glutamate pyruvate transaminase) and Alkaline phosphatase in two BDL/S groups was nearly same. In the histological finding, we observed mild bile duct proliferation, weak inflammation and fibrosis in BDL/S with D-pen treated group, but BDL/S operated group showed the formation of septum (island of hepatocytes), massive bile duct proliferation. This result represents that the BDL/S operation induces liver fibrosis (cirrhosis) in 4 weeks, and D-pen inhibits the synthesis of collagen weakly and stimulates the degradation of collagen in the extracellular space. We conclude that the monitoring of PNIIIP, PIVCP in sera is useful parameter for screening of antifibrotic effect, and D-pen delay the liver fibrosis.

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Implantation of Fetal Hepatocytes on Biodegradable Polymer Scaffolds (생분해성 고분자 담체를 이용한 태아 간세포의 이식)

  • 곽소정;최동호;백승삼;김상수;최차용;김병수
    • KSBB Journal
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    • v.19 no.3
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    • pp.210-214
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    • 2004
  • Whole liver transplantation, the currently available treatment of end-stage liver disease, has limitations including serious donor shortage, fatal surgical complications, risk of allograft rejection, and the requirement of life-long immunosuppression. In this study, we investigated the possibility of reconstructing liver tissues in vivo by implanting fetal hepatocytes on polymer scaffolds as a potential method to replace the current treatments. Fetal hepatocytes were freshly isolated from mice and seeded onto porous mesh scaffolds fabricated from polyglycolic acid, a biodegradable synthetic polymer. The seeded scaffolds were implanted into peritoneal cavity of athymic mice for one week. As a control, fetal hepatocytes were implanted without scaffold. One week after transplantation, liver-like tissues formed. Histological and immunohistochemical analyses indicated that the hepatocyles and liver tissue structures (bile ducts) were present in the newly formed tissues. In the control group, no transplanted hepatocytes were observed. Theses preliminary results suggest that liver tissues may be regeneration by transplanting fetal hepatocytes on polymer scaffolds.

Pancreatic Diseases: Genetics and Modeling Using Human Pluripotent Stem Cells

  • Yuri Lee;Kihyun Lee
    • International Journal of Stem Cells
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    • v.17 no.3
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    • pp.253-269
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    • 2024
  • Pancreas serves endocrine and exocrine functions in the body; thus, their pathology can cause a broad range of irreparable consequences. Endocrine functions include the production of hormones such as insulin and glucagon, while exocrine functions involve the secretion of digestive enzymes. Disruption of these functions can lead to conditions like diabetes mellitus and exocrine pancreatic insufficiency. Also, the symptoms and causality of pancreatic cancer very greatly depends on their origin: pancreatic ductal adenocarcinoma is one of the most fatal cancer; however, most of tumor derived from endocrine part of pancreas are benign. Pancreatitis, an inflammation of the pancreatic tissues, is caused by excessive alcohol consumption, the bile duct obstruction by gallstones, and the premature activation of digestive enzymes in the pancreas. Hereditary pancreatic diseases, such as maturity-onset diabetes of the young and hereditary pancreatitis, can be a candidate for disease modeling using human pluripotent stem cells (hPSCs), due to their strong genetic influence. hPSC-derived pancreatic differentiation has been established for cell replacement therapy for diabetic patients and is robustly used for disease modeling. The disease modeling platform that allows interactions between immune cells and pancreatic cells is necessary to perform in-depth investigation of disease pathogenesis.