Prognostic markers at adolescence in patients requiring liver transplantation for biliary atresia in adulthood

Jain, Vandana; Burford, Charlotte; Alexander, Emma; Sutton, Harry; Dhawan, Anil; Joshi, Deepak; Davenport, Mark; Heaton, Nigel; Hadžić, Nedim; Samyn, Marianne

doi:10.1016/j.jhep.2019.03.005

Cited by 37 publications

(43 citation statements)

References 36 publications

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“…Cholangitis is another potentially lethal complication after KPE and is postulated to be responsible for the worsening of cholestasis. In a study by Jain et al, a 4-fold increase in the risk of future LT is found in patients with late onset of cholangitis (18). Our data revealed that 27.5% of our patients developed recurrent cholangitis, a gure lower than a recent study by a Korean group reporting 76.2% of cholangitis would recur (21).…”

Section: Discussioncontrasting

confidence: 69%

“…This policy should continue when the patient is transited to adult care as OGV can continue to develop after adolescence. Even though PHT is considered as a predictor for future LT in a recent study by Jain et al (18), proper management of its complications could potentially keep them from LT. Recently, non-invasive measurement of liver brosis is available for clinical use and this should be incorporated in the follow up visit of BA patient as an indirect monitoring of PHT (19).…”

Section: Discussionmentioning

confidence: 99%

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Stretching the Limit of Native Liver Survival in Biliary Atresia After Kasai Portoenterostomy – a 37-year Territory-wide Study

Chung

Chan

Yeung

et al. 2021

Preprint

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ObjectiveWe present a 37 years’ experience in the management of biliary atresia (BA) and discuss long-term complications after Kasai portoenterostomy (KPE).MethodsA retrospective territory-wide study from 1980 to 2017 on 231 patients with open KPE from three tertiary paediatric surgical centres was performed. The jaundice clearance (JC) rate, native liver survival (NLS) rate and complications were analyzed.ResultsThe mean follow up period was 15.4 +/- 6.2 years. Over 60% of patients remained JC within 2 years after KPE. Seventy patients (30.3%) received liver transplant (LT) at a median age of 6.2 years (range: 0.5 to 25 years). The NLS rates at 10, 20 and 30 years were 70.7%, 61.5% and 53.0% respectively with no significant change over the study period. The median bilirubin level among all native liver survivors (n=153) was 24 µmol/L (2 to 162 µmol/L). Portal hypertension (PHT) and recurrent cholangitis were found in 51.6% and 27.5% of them respectively.ConclusionWith a vigilant follow up program, more than 60% of BA patients could remain stable with the disease and achieve long term survival without LT. Although portal hypertension and recurrent cholangitis are common, they do not need to be the indications for LT if managed properly.

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Section: Discussioncontrasting

confidence: 69%

Section: Discussionmentioning

confidence: 99%

Stretching the Limit of Native Liver Survival in Biliary Atresia After Kasai Portoenterostomy – a 37-year Territory-wide Study

Chung

Chan

Yeung

et al. 2021

Preprint

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show abstract

“…Cholangitis is another potentially lethal complication after KPE and is postulated to be responsible for the worsening of cholestasis. In a study by Jain et al, a fourfold increase in the risk of future LT is found in patients with late onset of cholangitis 22 . Our data revealed that 27.5% of our patients had developed recurrent cholangitis, a figure lower than a recent study by a Korean group that reported 76.2% 25 .…”

Section: Discussionmentioning

confidence: 93%

“…This policy should continue when the patient is transitioned to adult as OGV can continue to develop after adolescence. Even though PHT is considered as a predictor for future LT in a recent study by Jain et al 22 , proper management of its complications could potentially keep them from LT. Recently, the value of liver stiffness measurement to monitor PHT has been reported and if available, this should be incorporated in the follow up protocol of BA patients 23 .…”

Section: Discussionmentioning

confidence: 99%

Life long follow up and management strategies of patients living with native livers after Kasai portoenterostomy

Chung

Chan

Yeung

et al. 2021

Sci Rep

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We present a 37 years’ experience in the management of biliary atresia (BA) and discuss long-term complications after Kasai portoenterostomy (KPE). A retrospective territory-wide study from 1980 to 2017 on 231 patients with open KPE from three tertiary paediatric surgical centres was performed. Outcome parameters were clearance of jaundice (COJ), native liver survival (NLS) and long-term complications. Factors affecting the operative outcomes were analyzed. The median duration of follow up was 17.5 (IQR: 13.5–22) years. Over 66% of patients became jaundice-freed at 1 year after KPE. Seventy patients (30.3%) received liver transplant (LT) at a median age of 6.2 (IQR: 4.3–8.4) years. The NLS rates at 10 and 20 years were 70.7% and 61.5% respectively with no significant change over the study period. The median age at KPE was 59 (IQR: 49–67) days. KPE performed before 70 days was associated with higher odd ratios for successful drainage but the age of KPE did not have an impact on the long-term NLS. Among all native liver survivors (n = 153), the median bilirubin level was 24 (IQR: 16–36) µmol/L. Portal hypertension (PHT) and recurrent cholangitis were found in 51.6% and 27.5% of them respectively. With a vigilant follow up program, more than 60% of BA patients could remain stable with the disease and achieve long-term survival without LT. Although cholestasis, portal hypertension and recurrent cholangitis are common in long-term NLS, with a comprehensive follow management strategy, they do not always necessitate LT. Our study serves as an example for countries where deceased donor organs are scarce due to very low donation rate.

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“…(25) Similarly, a retrospective Although this may be helpful in guiding surveillance of liver disease progression during transition to adulthood, earlier intervention before adolescence and before liver damage occurs would be more beneficial. (26) The Netherlands registry reported factors associated with LT; however, analysis was limited in its retrospective nature, the small cohort, and use of only very early laboratory values. (27) The biochemical parameters chosen for this analysis are obtained routinely by clinicians.…”

Section: Discussionmentioning

confidence: 99%

Modeling Outcomes in Children With Biliary Atresia With Native Liver After 2 Years of Age

Venkat

Magee

et al. 2020

Hepatol. Commun.

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Approximately 50% of infants with biliary atresia (BA) undergoing Kasai portoenterostomy show survival with native liver (SNL) at age 2 years. Predictors of disease progression after age 2 years are unknown, despite estimates of 20%-30% undergoing liver transplant (LT) between age 2 and 18 years. We sought to address this knowledge gap by developing prognostic models in participants of the multicenter prospective National Institutes of Health-supported Childhood Liver Disease Research Network. We extracted 14 clinical and biochemical variables at age 2 years to develop two models for future outcomes: 1) LT or death (LTD) and 2) first sentinel event (SE), either new onset ascites, hepatopulmonary syndrome (HPS), or gastrointestinal (GI) bleed. A total of 240 participants, enrolled between 2004 and 2017, were followed until a median age of 5.1 years (range, 2.0-13.3 years). Of these participants, 38 underwent LT (n = 37) or death (n = 1); cumulative incidence, 23.7% (95% confidence interval [CI], 16.2%-32.0%). Twenty-seven experienced either new-onset ascites (n = 13), HPS (n = 1), or GI bleed (n = 14). One participant had ascites and GI bleed concurrently; cumulative incidence, 21.5% (95% CI, 14.2%-29.8%) by age 10 years. The Cox proportional hazard model predicted risk of LTD, using total bilirubin, albumin, platelet count, and history of either ascites or cholangitis (BA LTD model), with a C-index of 0.88 (range, 0.86-0.89). A cause-specific hazard competing risk model predicted SE using platelet count and gamma glutamyltransferase levels (BA SE model) with a C-index of 0.81 (range, 0.80-0.84). Internal model validity was assessed using Harrell's C-index with cross-validation. Conclusion: Stratification using these models identified risk of poor outcomes in patients with BA SNL after age 2 years. The models may identify those who would benefit from enhanced clinical surveillance and prioritization in clinical trials. (Hepatology Communications 2020;4:1824-1834). B iliary atresia (BA) is a congenital, idiopathic, obliterative, neonatal cholangiopathy that rapidly leads to end-stage liver disease if untreated. There is no medical treatment for BA. Even after surgical intervention with Kasai portoenterostomy (KPE), where the fibrotic biliary remnant is excised and drainage of the biliary tree attempted with Roux-en-Y, almost 50% of patients with BA will undergo liver transplant (LT) before 2 years of age. (1-3)

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Prognostic markers at adolescence in patients requiring liver transplantation for biliary atresia in adulthood

Cited by 37 publications

References 36 publications

Stretching the Limit of Native Liver Survival in Biliary Atresia After Kasai Portoenterostomy – a 37-year Territory-wide Study

Stretching the Limit of Native Liver Survival in Biliary Atresia After Kasai Portoenterostomy – a 37-year Territory-wide Study

Life long follow up and management strategies of patients living with native livers after Kasai portoenterostomy

Modeling Outcomes in Children With Biliary Atresia With Native Liver After 2 Years of Age

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