UNASSIGNED: We analyzed the admission characteristics of 68 patients with WD presenting with features of ACLF among a cohort of WD patients from 1997 to 2017. WD was diagnosed as per European association for the study of the liver (EASL)/Leipzig criteria and ACLF by the Asia-Pacific Association of Study of Liver and World Gastroenterology Organization consensus criteria. Factors associated with mortality were analyzed by univariate followed by multivariate analysis and receiver operating characteristic curve.
UNASSIGNED: Of the 272 patients with WD, 68 fulfilled criteria for ACLF. The mean age was 14.4 years (Range 5-42 years). Males constituted 38/68 (56%). Acute viral or drug induced hepatitis as precipitating factors was seen in 11.7%. Forty-nine patients (49/67; 73%) died including 30/32 (93.8%) with encephalopathy and 45/62 (72.6%) with ascites. Prognostic factors on univariate analysis significant for mortality included encephalopathy, international normalized ratio, white blood cell count and model for end-stage liver disease (MELD) score. On multivariate analysis, only encephalopathy was significant with 82% accuracy in differentiating survivors versus non-survivors. Post mortem liver biopsy in 21 patients and explant biopsy in 2 patients showed features of cirrhosis in all.
UNASSIGNED: WD with ACLF is associated with a high mortality Precipitating factors such as viral and drug-induced hepatitis was seen in 11.7% patients. Liver histology in patients subjected to biopsy showed cirrhosis in all. Only encephalopathy is a prognostic marker of non-survival with an accuracy of 82%.