Seema Alam1, Bikrant Bihari Lal2, Vikrant Sood2, Rajeev Khanna2, Guresh Kumar3. 1. Department of Pediatric Hepatology, Institute of Liver and Biliary Sciences, New Delhi, 110070, India. seema_alam@hotmail.com. 2. Department of Pediatric Hepatology, Institute of Liver and Biliary Sciences, New Delhi, 110070, India. 3. Institute of Liver and Biliary Sciences, New Delhi, India.
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.
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.