| OBJECTIVE: To analyze the chronic liver disease,acute precipitating events,clinical characteristics,laboratory results,treatment and prognosis,as well as to analyze the prognostic factors in children with Pediatric acuteon-chronic liver failure(PALF)and verify the prognostic value of the AARC score in children for clinical decision making.METHODS: The clinical data of 52 children with PALF was collected at Children’s Hospital of Chongqing Medical University from January2000 to December 2020 retrospectively.All children were divided into survival and death groups,and the data of the two groups were statistically analyzed.RESULTS:1.among 52 children,35 were male and 17 were female,median age was 9.4 months(1 month to 16 years),and median length of hospitalization was 10 days(1 day to 62 days).2.The cause of chronic liver disease was unknown(cryptogenic chronic liver disease)in 32 cases,genetic metabolic disease in 12 cases,viral hepatitis in 6 cases,autoimmune hepatitis in 1 case,and common bile duct cyst in 1 case.The causes of acute liver injury were drug-related in 11 cases,infection-related in 7 cases,and unknown in the remaining 34 cases.3.16 children did not develop hepatic encephalopathy(HE),22 cases were graded 1-2,14 cases were graded 3-4 in HE,48 cases(92.3%)showed jaundice,31 cases(59.6%)showed ascites,31 cases(59.6%)showed bleeding tendency,and 42 cases(80.8%)showed multiple organ failure.4.Total bilirubin at admission was 282.2±171.9 mg/dl,peak total bilirubin was 321.0±134.8 mg/dl,ALT 654.6±897.3 U/L,AST902.1±1576.8 U/L,peak ALT 655.0±875.4 U/L,AST 770.8±835.4 U /L,albumin 32.3(25.3,38.5)g/L,low value 29.2 ± 6.3 g/L.Blood ammonia74.1 ± 48.6 umol/L,peak 100.8 ± 53.0 umol/L,lactate 4.5 ± 3.0 mmol/L,peak 5.5 ± 3.5 mmol/L.INR was 3.7 ± 5.4,peak 4.4 ±5.9.ALT(p=0.012)and AST(p=0.033)were statistically significant in the genetic metabolic disease group compared with other groups,and peak total bilirubin(p=0.017)and peak lactate(p=0.017)in viral hepatitis compared with other groups.5.25(48.1%)cases had only plasma transfusion during hospitalization,17(32.7%)had plasma replacement,10(19.2%)had mechanical ventilation,12(23.1%)had hemodilution,19 cases died at the 90-day follow-up outcome,20 survived,13 were lost,and no child had liver transplantation.6.age(p=0.010),length of hospitalization(p=0.008),incidence of MODS(p=0.008),encephalopathy(p=0.004)and severe encephalopathy(p=0.003),indirect bilirubin(p=0.019),INR(p=0.030),prothrombin time(p=0.013),peak prothrombin time(p=0.008),peak blood ammonia(p=0.001),peak lactate(p=0.020),peak albumin(p=0.003),WBC(p=0.026),hemoglobin(p=0.010),creatinine(p=0.006),and AARC score(p=0.001)were associated with poor prognosis,and peak hemoglobin(p=0.022)and creatinine(p=0.029)were independent risk factors affecting the prognosis of slow plus acute liver failure.The area under the AARC score curve(AUROC)was 0.81(95% confidence interval 0.61-0.91).The optimal cutoff value was 10,with a sensitivity of 90.00% and a specificity of 57.89%.Conclusion: Children with PALF have high mortality.Cryptogenic chronic liver disease and metabolic liver disease are the commonest chronic liver disease.Unknown causes and drug-related events often induce PALF.Poor outcome was associated with peak blood ammonia and creatinine.AARC score is of moderate diagnostic value,and more prospective large sample studies are still necessary in the future. |
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