The Influence of Cholangitis on the Prognosis of Extrahepatic Biliary Atresia

Lünzmann, K.; Schweizer, Paul

doi:10.1055/s-2008-1072206

Cited by 47 publications

(31 citation statements)

References 6 publications

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“…However, the antimicrobial property for Escherichia coli in hepcidin is meaningful, as E. coli is the most common bacterium involved in postoperative cholangitis in BA (25,26). Postoperative cholangitis is the most common complication following KP for BA, which exacerbates preexisting liver fibrosis (27), leading to end-stage liver cirrhosis necessitating liver transplant in the late stage of BA. Patients who receive liver transplantation for 665 salvage experience more episodes of infection in the hepatobiliary system than those who survive with their native liver (28).…”

Section: Discussionmentioning

confidence: 99%

Liver Hepcidin and Stainable Iron Expression in Biliary Atresia

et al. 2006

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Hepcidin is a proposed mammalian host defense peptide that was identified on the basis of its antimicrobial activity, but it was later shown to be a crucial regulator of iron homeostasis and a mediator of the anemia of chronic inflammation. Hepcidin and stainable iron expression in biliary atresia (BA) were investigated in this study. Fresh liver tissues were obtained from 10 patients in the early stage of BA when they underwent Kasai's procedure, 9 in the late stage of BA when they received liver transplantation and 5 controls receiving liver resection for benign lesions other than cholestasis or fibrosis. Real-time quantitative reverse-transcription PCR (QRT-PCR), immunohistochemical staining and ELISA were performed to gauge hepcidin mRNA and protein expression in liver and plasma. Archival liver specimens from patients in the early and late stages of BA were treated with Perls' acid ferrocyanide technique for hepatic stainable iron. The results demonstrated that liver hepcidin mRNA expression was 100-fold lower in late-stage BA than in the early stage by QRT-PCR. Significantly weaker liver hepcidin immunostaining and lower plasma hepcidin levels were found in late-stage BA than in the early stage. There was also significantly lower stainable iron in the liver of late-stage BA. The major site of stainable iron was in Kupffer cells. The results support a role for hepcidin as a key regulator of mammalian iron metabolism and chronic inflammation, whose expression correlates with the degree of stainable iron in BA. (Pediatr Res 59: 662-666, 2006)

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Section: Discussionmentioning

confidence: 99%

Liver Hepcidin and Stainable Iron Expression in Biliary Atresia

et al. 2006

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“…da CPRE e da colangiorressonância no diagnóstico da atresia continuam sendo debatidos, e a laparoscopia combinada com colangiografia intra-operatória ainda é preconizada para os lactentes com suspeita de atresia Após a portoenterostomia, a complicação precoce mais freqüente é a colangite bacteriana ascendente, e seu tratamento é fundamental para a manutenção do fluxo biliar e do prognóstico do paciente, pois quanto maior o número de episódios de colangite, maior a chance de ocorrerem esclerose e perda dos ductos biliares intra-hepáticos remanescentes, com conseqüente progressão para cirrose hepática 77 . A colangite ascendente ocorre em 40 a 60% das crianças operadas, sendo mais comumente observada nos pacientes que .…”

Section: Figura 2 -Interações Dos Prováveis Mecanismos Patogênicos Daunclassified

Extrahepatic biliary atresia: current concepts and future directions

Carvalho¹,

Ivantes²,

Bezerra³

2007

J Pediatr (Rio J)

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ResumoObjetivos: Apresentar uma revisão atualizada sobre atresia das vias biliares extra-hepáticas, com ênfase em etiopatogenia, abordagens diagnósticas e terapêuticas e prognóstico. Conclusão:A atresia das vias biliares extra-hepáticas continua sendo a principal indicação de transplante hepático em crianças, e a mudança deste panorama depende de um melhor entendimento da etiopatogenia da obstrução biliar nos diferentes fenótipos da doença.Investigações futuras a respeito do papel do interferon gama e de outras citocinas são necessárias para avaliar se esses aspectos seriam potenciais alvos de intervenção terapêutica.J Pediatr (Rio J). 2007;83(2):105-120: Atresia das vias biliares extra-hepáticas, etiopatogenia, diagnóstico, tratamento e prognóstico. AbstractObjective: To provide an updated review on extrahepatic biliary atresia, focusing mainly on its etiopathogenesis, diagnosis, treatment and prognosis.Sources: MEDLINE and PubMed databases were searched using the following keywords: biliary atresia, etiopathogenesis, diagnosis, treatment, prognosis, children. Summary of the findings:Extrahepatic biliary atresia is the main indication for liver transplantation among pediatric patients. As to its

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“…Biliary atresia is generally considered an acquired condition with potential immunologic, inflammatory, infectious and abnormal developmental pathway contributing to the progressive destruction of the extra- and intrahepatic biliary tree leading to hepatic fibrosis. (3–9)…”

Section: Introductionmentioning

confidence: 99%

Dexamethasone Alters the Hepatic Inflammatory Cellular Profile Without Changes in Matrix Degradation During Liver Repair Following Biliary Decompression

Muratore

Harty

Papa

et al. 2009

Journal of Surgical Research

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Background Biliary atresia is characterized by extrahepatic bile duct obliteration along with persistent intrahepatic portal inflammation. Steroids are standard in the treatment of cholangitis following the Kasai portoenterostomy and were liberally adopted advocated for continued suppression of the ongoing immunological attack against intrahepatic ducts. Recent reports however have failed to demonstrate an improved patient outcome or difference in the need for liver transplant in postoperative patients treated with a variety of steroid regimes compared to historical controls. In the wake of progressive liver disease despite biliary decompression steroids are hypothesized to suppress inflammation and promote bile flow without any supporting data regarding their effect on the emerging cellular and molecular mechanisms of liver repair. We have previously shown in a reversible model of cholestatic injury that repair is mediated by macrophages, neutrophils and specific matrix metalloproteinase activity (MMP8); we questioned whether steroids would alter these intrinsic mechanisms. Methods Rats underwent biliary ductal suspension for 7 days followed by decompression. Rats were treated with IV dexamethasone or saline at the time of decompression. Liver tissue obtained at the time of decompression or after 2 days (D2) of repair was processed for morphometric analysis, immunohistochemistry, and quantitative RT-PCR. Results There was a dramatic effect of dexamethasone on the inflammatory component with the initiation of repair. Immunohistochemistry revealed a reduction of both ED1+ hepatic macrophages and ED2+ Kupffer cells in repair when compared to saline controls. Dexamethasone treatment also reduced infiltrating neutrophils by D2. TNF-α expression, increased during injury in both saline and dexamethasone groups, was markedly reduced by dexamethasone during repair (D2) whereas IL-6, IL-10 and CINC-1 remained unchanged when compared to saline controls. Dexamethasone reduced both MMP8 and TIMP1 expression by D2, whereas MMP9, 13, and 14 were unchanged when compared to sham controls. Despite substantial cellular and molecular changes during repair, collagen resorption was the same in both groups Conclusion Dexamethasone has clear effects on both the hepatic macrophage populations and infiltrating neutrophils following biliary decompression. Altered MMP and TIMP gene expression might suggest that steroids have the potential to modify matrix metabolism during repair. Nevertheless, successful resorption of collagen fibrosis proceeded presumably through other MMP activating mechanisms. We conclude that steroids do not impede the rapid intrinsic repair mechanisms of matrix degradation required for successful repair.

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The Influence of Cholangitis on the Prognosis of Extrahepatic Biliary Atresia

Cited by 47 publications

References 6 publications

Liver Hepcidin and Stainable Iron Expression in Biliary Atresia

Liver Hepcidin and Stainable Iron Expression in Biliary Atresia

Extrahepatic biliary atresia: current concepts and future directions

Dexamethasone Alters the Hepatic Inflammatory Cellular Profile Without Changes in Matrix Degradation During Liver Repair Following Biliary Decompression

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