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AARC-ACLF score: best predictor of outcome in children and adolescents with decompensated Wilson disease

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Abstract

Background and aims

Doubts have been raised about efficacy of New Wilson’s index (NWI) in predicting Liver Transplant (LT) or mortality in decompensated Wilson Disease (WD) patients. APASL ACLF Research Consortium (AARC) has introduced a new score (AARC-ACLF) which has not been studied in children.

Methods

Data of all pediatric WD cases were prospectively collected and analyzed. Cox regression and Area Under Receiver Operative Curve (AUROC) analyses were used to identify best predictive score for mortality at 90 days.

Results

Sixty-six confirmed cases of decompensated WD, 39 (59%) improved on medical management and 27 (41%) either died (20) or were transplanted (7). Among those with NWI ≥ 11 (42/66 cases) 19 survived versus those with NWI < 11 (24/66), 4 died. NWI (HR 1.23, 95% CI 1.07–1.42, p = 0.005), AARC-ACLF (HR 1.66, 95% CI 1.34–2.05, p = 0.000) and Chronic Liver Failure-Sequential Organ Failure Assessment score also known as CLIF–SOFA (HR 1.31, 95% CI 1.13–1.50, p = 0.000) were all significantly associated with death on univariate Cox regression analysis. On comparative evaluation of the predictive scores in the present cohort, the highest positive (6.02) and lowest negative (0.09) likelihood ratios as well as highest accuracy (87.88%) revealed AARC-ACLF as the best predictor of mortality. AARC-ACLF had the best predictability with AUROC of 0.939 and the minimum standard error of 0.027. For every unit increase in AARC-ACLF score, there is likelihood of 66% increase in 90 day mortality. The optimal cutoff for the AARC-ACLF score to predict mortality was 11 or more.

Conclusion

AARC-ACLF is the best score for the prediction of mortality at 90 days in decompensated WD cases.

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Authors and Affiliations

  1. Department of Pediatric Hepatology, Institute of Liver and Biliary Sciences, New Delhi, 110070, India

    Seema Alam, Bikrant Bihari Lal, Vikrant Sood & Rajeev Khanna

  2. Institute of Liver and Biliary Sciences, New Delhi, India

    Guresh Kumar

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  1. Seema Alam
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  2. Bikrant Bihari Lal
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  3. Vikrant Sood
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  4. Rajeev Khanna
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Correspondence to Seema Alam.

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Conflict of interest

Seema Alam, Bikrant Bihari Lal, Vikrant Sood, Rajeev Khanna, and Guresh Kumar declare that they have no conflict of interest.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. This was a retrospective study. The study was approved by the institutional ethical review board.

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Alam, S., Lal, B.B., Sood, V. et al. AARC-ACLF score: best predictor of outcome in children and adolescents with decompensated Wilson disease. Hepatol Int 13, 330–338 (2019). https://doi.org/10.1007/s12072-019-09938-3

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  • DOI: https://doi.org/10.1007/s12072-019-09938-3

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