Grade IV Fibrosis Interferes in Biliary Drainage After Kasai Procedure

Netto, Alcides Augusto Salzedas; Chinen, E.S.; Oliveira, de; Pasquetti, Alberto; Azevedo, Ramiro Anthero de; Patricio, F.F. da Silva; Cury, Edson Khodor; Gonzalez, A.M.; Vicentine, Fernando Pompeu Piza; Martins, José Luiz

doi:10.1016/j.transproceed.2014.05.045

Cited by 16 publications

(13 citation statements)

References 5 publications

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“…Previous studies have reported 42% at 2 years, 23%‐45% at 4‐5 years, 15%‐40% at 10 years, 29% at 15 years, and 13% at 20 years of survival with native liver . Neither advanced histologic fibrosis nor nodular appearance of the liver at the time of KPE reliably predict outcome after surgery . However, KPE in infants with cirrhosis and ascites may precipitate hepatic decompensation.…”

Section: Indications For Liver Transplantationmentioning

confidence: 94%

Biliary atresia: Indications and timing of liver transplantation and optimization of pretransplant care

et al. 2016

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Biliary Atresia is a progressive, fibro-obliterative disorder of the intra and extrahepatic bile ducts in infancy. The majority of affected children will eventually develop end-stage liver disease and require liver transplantation. Indications for liver transplant in biliary atresia include failed Kasai portoenterostomy, significant and recalcitrant malnutrition, recurrent cholangitis, and the progressive manifestations of portal hypertension. Extra-hepatic complications of this disease, such as hepatopulmonary syndrome and portopulmonary hypertension, are also indications for liver transplantation. Optimal pre-transplant management of these potentially life threatening complications and maximizing nutrition and growth require the expertise of a multi-disciplinary team with experience caring for biliary atresia. The timing of transplant for biliary atresia requires careful consideration of the potential risk of transplant versus the survival benefit at any given stage of disease. Children with biliary atresia often experience long wait times for transplant unless exception points are granted to reflect severity of disease. Family preparedness for this arduous process is therefore critical.

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Section: Indications For Liver Transplantationmentioning

confidence: 94%

Biliary atresia: Indications and timing of liver transplantation and optimization of pretransplant care

et al. 2016

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“…For patients with BA, the progression of hepatic fibrosis is aggressive and rapid [ 29 ]. Severe fibrosis at the time of KPE is a determinant of outcomes [ 3 4 ]. Thus, the correct detection of liver fibrosis before KPE with a non-invasive tool might help pediatric surgeons select appropriate therapy methods.…”

Section: Discussionmentioning

confidence: 99%

“…An accurate method to monitor the progression of liver fibrosis can lead to better prognosis and management of pediatric patients with chronic liver diseases [ 1 2 ]. For example, in patients with biliary atresia (BA), the stage of fibrosis at the time of Kasai hepatoportoenterostomy (KPE) can influence the outcomes [ 3 4 ]. Although liver biopsy is still considered the reference standard for diagnosing and staging liver fibrosis in children, its limitations [ 5 ] (e.g., invasiveness and interobserver and sampling variability) are well known.…”

Section: Introductionmentioning

confidence: 99%

Two-Dimensional Shear Wave Elastography Predicts Liver Fibrosis in Jaundiced Infants with Suspected Biliary Atresia: A Prospective Study

et al. 2021

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Objective This study aimed to evaluate the role of preoperative two-dimensional (2D) shear wave elastography (SWE) in assessing the stages of liver fibrosis in patients with suspected biliary atresia (BA) and compared its diagnostic performance with those of serum fibrosis biomarkers. Materials and Methods This study was approved by the ethical committee, and written informed parental consent was obtained. Two hundred and sixteen patients were prospectively enrolled between January 2012 and October 2018. The 2D SWE measurements of 69 patients have been previously reported. 2D SWE measurements, serum fibrosis biomarkers, including fibrotic markers and biochemical test results, and liver histology parameters were obtained. 2D SWE values, serum biomarkers including, aspartate aminotransferase to platelet ratio index (APRi), and other serum fibrotic markers were correlated with the stages of liver fibrosis by METAVIR. Receiver operating characteristic (ROC) curves and area under the ROC (AUROC) curve analyses were used. Results The correlation coefficient of 2D SWE value in correlation with the stages of liver fibrosis was 0.789 ( p < 0.001). The cut-off values of 2D SWE were calculated as 9.1 kPa for F1, 11.6 kPa for F2, 13.0 kPa for F3, and 15.7 kPa for F4. The AUROCs of 2D SWE in the determination of the stages of liver fibrosis ranged from 0.869 to 0.941. The sensitivity and negative predictive value of 2D SWE in the diagnosis of ≥ F3 was 93.4% and 96.0%, respectively. The diagnostic performance of 2D SWE was superior to that of APRi and other serum fibrotic markers in predicting severe fibrosis and cirrhosis (all p < 0.005) and other serum biomarkers. Multivariate analysis showed that the 2D SWE value was the only statistically significant parameter for predicting liver fibrosis. Conclusion 2D SWE is a more effective non-invasive tool for predicting the stage of liver fibrosis in patients with suspected BA, compared with serum fibrosis biomarkers.

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“…Although early surgical intervention with Kasai portoenterostomy may restore biliary drainage, infants often progress to cirrhosis and end-stage liver disease (Bessho and Bezerra, 2011). Consequently, BA is the most common cause of pediatric liver transplantation, accounting for 50% of all liver transplants in the pediatric population (Salzedas-Netto et al, 2014). …”

Section: Introductionmentioning

confidence: 99%

Rhesus rotavirus VP6 regulates ERK-dependent calcium influx in cholangiocytes

et al. 2016

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The Rhesus rotavirus (RRV) induced murine model of biliary atresia (BA) is a useful tool in studying the pathogenesis of this neonatal biliary obstructive disease. In this model, the mitogen associated protein kinase pathway is involved in RRV infection of biliary epithelial cells (cholangiocytes). We hypothesized that extracellular signal-related kinase (ERK) phosphorylation is integral to calcium influx, allowing for viral replication within the cholangiocyte. Utilizing ERK and calcium inhibitors in immortalized cholangiocytes and BALB/c pups, we determined that ERK inhibition resulted in reduced viral yield and subsequent decreased symptomatology in mice. In vitro, the RRV VP6 protein induced ERK phosphorylation, leading to cellular calcium influx. Pre-treatment with an ERK inhibitor or Verapamil resulted in lower viral yields. We conclude that the pathogenesis of RRV-induced murine BA is dependent on the VP6 protein causing ERK phosphorylation and triggering calcium influx allowing replication in cholangiocytes.

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Grade IV Fibrosis Interferes in Biliary Drainage After Kasai Procedure

Cited by 16 publications

References 5 publications

Biliary atresia: Indications and timing of liver transplantation and optimization of pretransplant care

Biliary atresia: Indications and timing of liver transplantation and optimization of pretransplant care

Two-Dimensional Shear Wave Elastography Predicts Liver Fibrosis in Jaundiced Infants with Suspected Biliary Atresia: A Prospective Study

Rhesus rotavirus VP6 regulates ERK-dependent calcium influx in cholangiocytes

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