Sequential Treatment of Biliary Atresia With Kasai Hepatoportoenterostomy and Liver Transplantation: Benefits, Risks, and Outcome in 393 Children

Introduction: Surgical treatment of biliary atresia (BA) is still based on sequential strategy with Kasai hepatoportoenterostomy (KP) followed by liver transplantation (LT), in case of complicated secondary biliary cirrhosis. Concerns have been expressed regarding the risks of LT related to previous KP, suggesting primary LT as an exclusive treatment of BA.Methods: Single-center retrospective analysis including 393 pediatric patients who underwent LT for BA from 1993 to 2018, categorized into two groups: with (KP) or without (NoKP) previous KP. Pre-LT clinical condition was estimated considering age at LT, time on waiting list, pediatric end-stage liver disease score (PELD), and presence of portal vein hypoplasia. Post-LT outcome was evaluated considering patient and graft survival rates, and need for early reoperation due to abdominal or graft-related complications (<45 days after LT).Results: Two-hundred ninety-six patients (75.3%) were categorized in the KP group, and 97 (24.7%) in the NoKP group. Median age at LT was 1.14 years in the KP group and 0.85 years in the NoKP group (p < 0.0001). PELD score was significantly less severe in KP patients (p < 0.05). One-year patient survival rates were 96.9 and 96.8% in the KP and NoKP groups, respectively (p = 0.43), and the corresponding graft survival was 92.5 and 94.8% (p = 0.97). The need for early reoperation was more frequent in the KP group (29.8%) vs. NoKP group (12.4%, p = 0.01). The rate of bowel perforation was non-significantly higher in the KP group (8.1%) vs. NoKP group (3.1%, p = 0.11).Conclusions: The sequential strategy including KP and LT allowed performing LT in patients with significant older age and better clinical conditions, when compared to those transplanted without previous KP. Patient and graft survivals were not impacted by previous KP. Although previous KP was associated with an increased rate of post-LT surgical complications, bowel perforation and bleeding did not occur significantly more frequently. Such results support the current strategy based on sequential treatment.

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Timing, Management, and Outcomes of Liver Transplantation in Primary Sclerosing Cholangitis

Seminars in Liver Disease ◽

10.1055/s-0037-1608655 ◽

2017 ◽

Vol 37 (04) ◽

pp. 305-313 ◽

Cited By ~ 12

Author(s):

Cynthia Levy ◽

Eric Martin

Keyword(s):

Liver Transplantation ◽

Liver Disease ◽

Primary Sclerosing Cholangitis ◽

Graft Survival ◽

Sclerosing Cholangitis ◽

Cholestatic Liver Disease ◽

Biliary Cirrhosis ◽

Immune Mediated ◽

End Stage ◽

Patient And Graft Survival

AbstractPrimary sclerosing cholangitis (PSC) is a chronic, immune-mediated cholestatic liver disease that often progresses to secondary biliary cirrhosis and end-stage liver disease. Short of liver transplantation (LT), there is no effective treatment for PSC. PSC accounts for approximately 5% of total adult LTs in the US and is currently the fifth most common indication for LT. Patient and graft survival for PSC is among the highest for all indications for LT. The main factors that impact outcomes after LT for PSC include biliary strictures, rejection, and recurrence of PSC. Recurrent PSC (rPSC) develops in 20% of LT recipients within 5 years of LT and is associated with negative patient and graft survival. LT is a viable option for recipients who develop rPSC and progress to graft failure.

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Liver Transplantation: Past Accomplishments and Future Challenges

Canadian Journal of Gastroenterology ◽

10.1155/1999/518751 ◽

1999 ◽

Vol 13 (3) ◽

pp. 257-263 ◽

Cited By ~ 4

Author(s):

William J Wall

Keyword(s):

Liver Transplantation ◽

Survival Rates ◽

Alcoholic Cirrhosis ◽

Liver Transplants ◽

Recidivism Rates ◽

Societal Challenges ◽

Therapeutic Value ◽

Future Challenges ◽

Will Force ◽

End Stage

Liver transplantation has evolved from a rare and risky operation of questionable therapeutic value to the preferred treatment for an extensive list of end-stage liver diseases. Superior immunosuppression (cyclosporine), and improvements in surgery and anesthesia brought liver grafting to its current level of success. Nearly 60,000 liver transplants have been performed, and survival rates are very good; however liver grafting faces serious immediate and long term challenges, mainly due to the widening gap between donor supply and recipient demand. Increasing numbers of sick candidates, recurrent disease (especially hepatitis C) and recidivism rates after transplantation for alcoholic cirrhosis will force increasingly difficult decisions on candidate selection and priority listing of potential recipients. Although xenotransplantation may be the ultimate solution, it has its own specific set of biological and societal challenges - the full extent of which should be revealed in the next several years.

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Adoption of MELD score increases the number of liver transplant

ABCD Arquivos Brasileiros de Cirurgia Digestiva (São Paulo) ◽

10.1590/s0102-67202014000300010 ◽

2014 ◽

Vol 27 (3) ◽

pp. 201-203 ◽

Cited By ~ 6

Author(s):

Lucas Souto NACIF ◽

Wellington ANDRAUS ◽

Rodrigo Bronze MARTINO ◽

Vinicius Rocha SANTOS ◽

Rafael Soares PINHEIRO ◽

...

Keyword(s):

Liver Transplantation ◽

Survival Rates ◽

Sao Paulo ◽

Meld Score ◽

The State ◽

São Paulo ◽

Liver Transplants ◽

Mean Values ◽

Before And After ◽

End Stage

BACKGROUND: Liver transplantation is performed at large transplant centers worldwide as a therapeutic intervention for patients with end-stage liver diseases. AIM: To analyze the outcomes and incidence of liver transplantation performed at the University of São Paulo and to compare those with the State of São Paulo before and after adoption of the Model for End-Stage Liver Disease (MELD) score. METHOD: Evaluation of the number of liver transplantations before and after adoption of the MELD score. Mean values and standard deviations were used to analyze normally distributed variables. The incidence results were compared with those of the State of São Paulo. RESULTS: There was a high prevalence of male patients, with a predominance of middle-aged. The main indication for liver transplantation was hepatitis C cirrhosis. The mean and median survival rates and overall survival over ten and five years were similar between the groups (p>0.05). The MELD score increased over the course of the study period for patients who underwent liver transplantation (p>0.05). There were an increased number of liver transplants after adoption of the MELD score at this institution and in the State of São Paulo (p<0.001). CONCLUSION: The adoption of the MELD score led to increase the number of liver transplants performed in São Paulo.

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Liver Grafts with Major Extended Donor Criteria May Expand the Organ Pool for Patients with Hepatocellular Carcinoma

Journal of Clinical Medicine ◽

10.3390/jcm8101692 ◽

2019 ◽

Vol 8 (10) ◽

pp. 1692 ◽

Cited By ~ 1

Author(s):

Vladimir Lozanovski ◽

Larissa Kerr ◽

Elias Khajeh ◽

Omid Ghamarnejad ◽

Jan Pfeiffenberger ◽

...

Keyword(s):

Hepatocellular Carcinoma ◽

Liver Transplantation ◽

Graft Survival ◽

Patient Survival ◽

Laboratory Model ◽

Transplant Patients ◽

One Year ◽

End Stage ◽

Donor Criteria ◽

Extended Donor Criteria

The major extended donor criteria (maEDC; steatosis >40%, age >65 years, and cold ischemia time >14 h) influence graft and patient outcomes after liver transplantation. Despite organ shortages, maEDC organs are often considered unsuitable for transplantation. We investigated the outcomes of maEDC organ liver transplantation in patients with hepatocellular carcinoma (HCC). Two hundred and sixty-four HCC liver transplant patients were eligible for analysis. Risk factor analysis was performed for early allograft dysfunction; primary nonfunction; 30-day and 90-day graft failure; and 30-day, 90-day, and 1-year patient mortality. One-year graft survival was higher in recipients of no-maEDC grafts. One-year patient survival did not differ between the recipients of no-maEDC and maEDC organs. The univariate and multivariate analyses revealed no association between maEDC grafts and one-year patient mortality. Graft survival differed between the recipients of no-maEDC and maEDC organs after correcting for a laboratory model of end-stage liver disease (labMELD) score with a cut-off value of 20, but patient survival did not. Patient survival did not differ between recipients who did and did not meet the Milan criteria and who received grafts with and without maEDC. Instead of being discarded, maEDC grafts may expand the organ pool for patients with HCC without impairing patient survival or recurrence-free survival.

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Alagille Syndrome Candidates for Liver Transplantation: Differentiation from End-Stage Biliary Atresia Using Preoperative CT

PLoS ONE ◽

10.1371/journal.pone.0149681 ◽

2016 ◽

Vol 11 (2) ◽

pp. e0149681 ◽

Cited By ~ 2

Author(s):

Sook Min Hwang ◽

Tae Yeon Jeon ◽

So-Young Yoo ◽

Ji Hye Kim ◽

Ben Kang ◽

...

Keyword(s):

Liver Transplantation ◽

Biliary Atresia ◽

Alagille Syndrome ◽

Preoperative Ct ◽

End Stage

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Case report: progressive familial intrahepatic cholestasis type 3 with compound heterozygous ABCB4 variants diagnosed 15 years after liver transplantation

BMC Medical Genetics ◽

10.1186/s12881-020-01173-0 ◽

2020 ◽

Vol 21 (1) ◽

Author(s):

Mariam Goubran ◽

Ayodeji Aderibigbe ◽

Emmanuel Jacquemin ◽

Catherine Guettier ◽

Safwat Girgis ◽

...

Keyword(s):

Liver Transplantation ◽

Liver Disease ◽

Intrahepatic Cholestasis ◽

Autoimmune Response ◽

Compound Heterozygous ◽

Biliary Cirrhosis ◽

Progressive Familial Intrahepatic Cholestasis ◽

End Stage Liver Disease ◽

End Stage ◽

Type 3

Abstract Background Progressive familial intrahepatic cholestasis (PFIC) type 3 is an autosomal recessive disorder arising from mutations in the ATP-binding cassette subfamily B member 4 (ABCB4) gene. This gene encodes multidrug resistance protein-3 (MDR3) that acts as a hepatocanalicular floppase that transports phosphatidylcholine from the inner to the outer canalicular membrane. In the absence of phosphatidylcholine, the detergent activity of bile salts is amplified and this leads to cholangiopathy, bile duct loss and biliary cirrhosis. Patients usually present in infancy or childhood and often progress to end-stage liver disease before adulthood. Case presentation We report a 32-year-old female who required cadaveric liver transplantation at the age of 17 for cryptogenic cirrhosis. When the patient developed chronic ductopenia in the allograft 15 years later, we hypothesized that the patient’s original disease was due to a deficiency of a biliary transport protein and the ductopenia could be explained by an autoimmune response to neoantigen that was not previously encountered by the immune system. We therefore performed genetic analyses and immunohistochemistry of the native liver, which led to a diagnosis of PFIC3. However, there was no evidence of humoral immune response to the MDR3 and therefore, we assumed that the ductopenia observed in the allograft was likely due to chronic rejection rather than autoimmune disease in the allograft. Conclusions Teenage patients referred for liver transplantation with cryptogenic liver disease should undergo work up for PFIC3. An accurate diagnosis of PFIC 3 is key for optimal management, therapeutic intervention, and avoidance of complications before the onset of end-stage liver disease.

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Prognosis of liver transplantation: Does postoperative ileus matter?

BMC Gastroenterology ◽

10.1186/s12876-021-02026-7 ◽

2021 ◽

Vol 21 (1) ◽

Author(s):

Ruiping Bai ◽

Rui An ◽

Kunyu Han ◽

Mengwen Xue ◽

Simei Zhang ◽

...

Keyword(s):

Liver Transplantation ◽

Survival Data ◽

Postoperative Ileus ◽

Total Bilirubin ◽

Survival Rates ◽

Major Complication ◽

Meld Score ◽

Gastrointestinal Dysfunction ◽

End Stage ◽

Relative Factors

Abstract Background Nowadays, liver transplantation has become a main therapy for end-stage liver disease. However, studies show that there are high mortality and severe complications after liver transplantation. Although gastrointestinal dysfunction is a common and major complication after liver transplantation, there was rarely relative research. This study aims to elucidate the factors about ileus after liver transplantation and patients’ survival. Methods We collected and analyzed the data (n = 318, 2016–2019) from the First Affiliated Hospital of Xi’an Jiaotong University. After excluding cases, a total of 293 patients were included for this study. The subjects were divided into a non-ileus group and an ileus group. We reviewed 38 variables (including preoperative, operative and postoperative relative factors). Additionally, other complications after liver transplantation and survival data were compared between two groups. Results Of the 293 patients, 23.2% (n = 68) experienced postoperative ileus. Ileus patients were not different with non-ileus patients in preoperative, operative and postoperative factors. HBV-positive patients with ileus had a lower MELD score (P = 0.025), and lower postoperative total bilirubin was correlated with ileus (P = 0.049). Besides, Child–Pugh score of HCC patients with ileus was low (P = 0.029). The complications after liver transplantation were not different between two groups. Compared with the patients without ileus, the patients with ileus had a higher mortality rate. Conclusion According to our research, ileus-patients had a lower 1-year survival rates. The preoperative MELD score and postoperative total bilirubin of HBV-positive patients with ileus were lower, and Child–Pugh score of HCC patients with ileus was also lower.

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Post Liver Transplantation Survival and Related Prognostic Factors among Adult Recipients in Tehran Liver Transplant Center; 2002–2019

Archives of Iranian Medicine ◽

10.34172/aim.2020.22 ◽

2020 ◽

Vol 23 (5) ◽

pp. 326-334

Author(s):

Elham Madreseh ◽

Mahmood Mahmoudi ◽

Mohssen Nassiri-Toosi ◽

Taban Baghfalaki ◽

Hojjat Zeraati

Keyword(s):

Liver Transplantation ◽

Prognostic Factors ◽

Cox Model ◽

Survival Rates ◽

Underlying Disease ◽

Historical Cohort ◽

Transplant Center ◽

Increasing Demand ◽

End Stage

Background: Liver transplantation is a standard treatment for patients with end-stage liver disease (ESLD). However, with increasing demand for this treatment and limited resources, it is available only to patients who are more likely to survive. The primary aim was to determine prognostic factors for survival. Methods: We collected data from 597 adult patients with ESLD, who received a single organ and initial orthotopic liver transplantation (OLT) in our center between 20 March 2008 and 20 March 2018. In this historical cohort study, univariate and multiple Cox model were used to determine prognostic factors of survival after transplantation. Results: After a median follow-up of 825 (0–3889) days, 111 (19%) patients died. Survival rates were 88%, 85%, 82% and 79% at 90 days, 1 year, 3 years, and 5 years, respectively. Older patients (HR = 1.27; 95% CI: 1.01–1.59), presence of pre-OLT ascites (HR = 2.03; 95% CI: 1.16–3.57), pre-OLT hospitalization (HR = 1.88; 95% CI:1.02–3.46), longer operative time (HR = 1.006; 95% CI: 1.004–1.008), post-OLT dialysis (HR = 3.51; 95% CI: 2.07–5.94), cancer (HR = 2.69; 95% CI: 1.23–5.89) and AID (HR = 2.04; 95% CI: 1.17–3.56) as underlying disease versus hepatitis, and higher pre-OLT creatinine (HR = 1.67; 95% CI: 1.10–2.52) were associated with decreased survival. Conclusion: In this center, not only are survival outcomes excellent, but also younger patients, cases with better pre-operative health conditions, and those without complications after OLT have superior survival.

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Detrimental graft survival of size-mismatched graft for high model for end-stage liver disease recipients in liver transplantation

Journal of Hepato-Biliary-Pancreatic Sciences ◽

10.1002/jhbp.355 ◽

2016 ◽

Vol 23 (7) ◽

pp. 406-413 ◽

Cited By ~ 2

Author(s):

Kyota Fukazawa ◽

Seigo Nishida ◽

Ernesto A. Pretto ◽

Youri Vater ◽

Jorge D. Reyes

Keyword(s):

Liver Transplantation ◽

Liver Disease ◽

Graft Survival ◽

End Stage Liver Disease ◽

End Stage

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Biliary Atresia: 50 Years after the First Kasai

ISRN Surgery ◽

10.5402/2012/132089 ◽

2012 ◽

Vol 2012 ◽

pp. 1-15 ◽

Cited By ~ 44

Author(s):

Barbara E. Wildhaber

Keyword(s):

Liver Transplantation ◽

Biliary Atresia ◽

Clinical Sign ◽

Great Degree ◽

Biliary Tree ◽

Unknown Etiology ◽

Biliary Cirrhosis ◽

Systematic Screening ◽

Life Threatening ◽

Pediatric Indication

Biliary atresia is a rare neonatal disease of unknown etiology, where obstruction of the biliary tree causes severe cholestasis, leading to biliary cirrhosis and death in the first years of life, if the condition is left untreated. Biliary atresia is the most frequent surgical cause of cholestatic jaundice in neonates and should be evoked whenever this clinical sign is associated with pale stools and hepatomegaly. The treatment of biliary atresia is surgical and currently recommended as a sequence of, eventually, two interventions. During the first months of life a hepatoportoenterostomy (a “Kasai,” modifications of which are discussed in this paper) should be performed, in order to restore the biliary flow to the intestine and lessen further damage to the liver. If this fails and/or the disease progresses towards biliary cirrhosis and life-threatening complications, then liver transplantation is indicated, for which biliary atresia represents the most frequent pediatric indication. Of importance, the earlier the Kasai is performed, the later a liver transplantation is usually needed. This warrants a great degree of awareness of biliary atresia, and the implementation of systematic screening for this life-threatening pathology.

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