End-stage liver disease (ESLD) is a life-threatening clinical syndrome with significantly increased mortality when the patients are complicated with infections. For patients with ESLD, infections can induce or aggravate the liver decompensation. In turn, infections are among the most common complication with the disease progression. Experts from Society of Infectious Diseases, Chinese Medical Association firstly formulated "Expert Consensus on Diagnosis and Treatment of End-Stage Liver Disease Complicated with Infection" in July 2018, which is extensively revised based on clinical evidence of recent three years. This consensus summarizes the up-to-date knowledge and experiences across Chinese colleagues, intending to provide principles and wording procedures for clinicians to diagnose and treat ESLD patient complicated with infections.
Consensus ; Disease Progression ; End Stage Liver Disease/complications* ; Humans

End Stage Liver Disease ; complications ; Humans ; Malnutrition ; etiology ; therapy

End-stage liver disease (ESLD) is a life threaten clinical syndrome with significantly increasing mortality when patients complicated with infections. For patients with ESLD, infections can induce or aggravate the occurrence of liver decompensation. In turn, infections are among the most common complications under, disease progression. There is lacking of working procedures for early diagnosis and appropriate management for patients of ESLD complicated with infections, neither guidelines nor consensus at home and abroad. This consensus assembled up-to-date knowledge and experience across Chinese colleagues, providing principles as well as working procedures for clinicians to diagnose and treat an ESLD patient complicated with infections.
Bacterial Infections/complications* ; Coinfection ; Consensus ; Disease Progression ; End Stage Liver Disease/therapy* ; Humans ; Liver Transplantation

Objective: To investigate the ABC prognostic classification and the updated version of Model for End-stage Liver Disease (MELD) score 3.0 and Chinese Group on the Study of Severe Hepatitis B ACLF Ⅱ score (COSSH-ACLF Ⅱ score) to evaluate the prognostic value in acute-on-chronic liver failure (ACLF). Methods: ABC classification was performed on a 1 409 follow-up cohorts. The area under the receiver operating characteristic curve (AUROC) was used to analyze MELD, MELD 3.0, COSSH-Ⅱ and COSSH-Ⅱ score after 3 days of hospitalization (COSSH-Ⅱ-3d). The prognostic predictive ability of patients were evaluated for 360 days, and the prediction differences of different classifications and different etiologies on the prognosis of ACLF were compared. Results: The survival curve of 1 409 cases with ACLF showed that the difference between class A, B, and C was statistically significant, Log Rank (Mantel-Cox) χ²=80.133, P<0.01. Compared with class A and C, χ²=76.198, P<0.01, the difference between class B and C, was not statistically significant χ²=3.717, P>0.05. AUROC [95% confidence interval (CI)] analyzed MELD, MELD 3.0, COSSH-Ⅱ and COSSH-Ⅱ-3d were 0.644, 0.655, 0.817 and 0.839, respectively (P<0.01). COSSH-Ⅱ had better prognostic predictive ability with class A ACLF and HBV-related ACLF (HBV-ACLF) for 360-days, and AUROC (95% CI) were 0.877 and 0.881, respectively (P<0.01), while MELD 3.0 prognostic predictive value was not better than MELD. Conclusion: ACLF prognosis is closely related to ABC classification. COSSH-Ⅱ score has a high predictive value for the prognostic evaluation of class A ACLF and HBV-ACLF. COSSH-Ⅱ score has a better prognostic evaluation value after 3 days of hospitalization, suggesting that attention should be paid to the treatment of ACLF in the early stage of admission.
Humans ; Acute-On-Chronic Liver Failure ; Prognosis ; End Stage Liver Disease/complications* ; Retrospective Studies ; Severity of Illness Index

Acute Kidney Injury ; etiology ; End Stage Liver Disease ; complications ; Hepatorenal Syndrome ; etiology ; Humans

BACKGROUNDThere are increasing numbers of patients who survive more than one year after liver transplantation. Many studies have focused on the early mortality of these patients. However, the factors affecting long-term survival are not fully understood. This study aims to evaluate prognostic factors predicting long-term survival and to explore measures for improving the survival outcomes of patients who underwent liver transplantation for benign end-stage liver diseases.

METHODSThe causes of late death after liver transplantation and potential prognostic factors were retrospectively analyzed for 221 consecutive patients who underwent liver transplantation from October 2003 to June 2008. Twenty-seven variables were assessed using the Kaplan-Meier method, and those variables found to be univariately significant at P < 0.10 were entered into a backward step-down Cox proportional hazard regression analysis to identify the independent prognostic factors influencing the recipients' long-term survival.

RESULTSTwenty-eight recipients died one year after liver transplantation. The major causes of late mortality were infectious complications, biliary complications, and Hepatitis B virus recurrence/reinfection. After Cox analysis, the five remaining co-variables were: age, ABO blood group, cold ischemia time, post-infection region, and biliary complications.

CONCLUSIONSThe major causes of late mortality were infection, biliary complications and Hepatitis B virus recurrence/reinfection. Five variables (Age, ABO blood group, cold ischemia time, infection, and biliary complications) had significant impacts on patient survival.

End Stage Liver Disease ; mortality ; surgery ; Hepatitis B ; mortality ; Humans ; Liver Transplantation ; Postoperative Complications ; mortality ; Retrospective Studies

OBJECTIVETo identify the risk factors of hepatorenal syndrome in patients with hepatitis B virus (HBV)-related acute-on-chronic liver failure(ACLF).

METHODSA total of 726 hospitalized patients with HBV-ACLF were retrospectively analyzed. Data of demographic and clinical parameters (sex, age, family history, and presence of liver cirrhosis and diabetes), common complications (spontaneous bacterial peritonitis, pulmonary infection, hepatic encephalopathy, and upper gastrointestinal hemorrhage), and baseline biochemical parameters (albumin, globulin, total bilirubin, direct bilirubin, alanine aminotransferase, aspartate aminotransferase, gamma-glutamyl transferase, alkaline phosphatase, cholesterol, cholinesterase, K+, Na+, plasma thromboplastin antecedent, alpha-fetoprotein, HBV DNA, white blood cell count, hemoglobin, and platelet count) were collected from the medical records database. Univariate and multiple regression analyses were performed to determine the risk factors of hepatorenal syndrome.

RESULTSMultiple logistic regression analysis indicated that upper gastrointestinal hemorrhage [risk (R) = 1.313, relative hazard (RH) = 3.716, 95% confidence interval (CI): 2.156-6.404], hepatic encephalopathy (R = 1.120, RH = 3.065, 95% CI: 1.900-4.945), spontaneous bacterial peritonitis (R = 1.005, RH = 2.733, 95% CI: 1.379-5.417), pulmonary infection (R = 1.051, RH = 2.862, 95% CI: 1.783-4.592), and white blood cell count (R = 0.056, RH = 1.058, 95% CI: 1.010-1.107) were independent risk factors for hepatorenal syndrome development in patients with HBV-ACLF.

CONCLUSIONSeveral risk factors were significantly associated with the development of hepatorenal syndrome in HBV-ACLF, including upper gastrointestinal hemorrhage, hepatic encephalopathy, spontaneous bacterial peritonitis, pulmonary infection, and elevated white blood cell count.

Adult ; Causality ; End Stage Liver Disease ; complications ; Female ; Hepatitis B, Chronic ; complications ; Hepatorenal Syndrome ; etiology ; Humans ; Liver Failure ; complications ; etiology ; Male ; Middle Aged ; Retrospective Studies ; Risk Factors

Erythropoietic protoporphyria (EPP) is an inherited disorder of the heme metabolic pathway that is characterized by accumulation of protoporphyrin in the blood, erythrocytes, and tissues, and cutaneous manifestations of photosensitivity, all resulting from abnormalities in ferrochelatase (FECH) activity due to mutations in the FECH gene. Protoporphyrin is excreted by the liver, and excess protoporphyrin leads to cholelithiasis with obstructive episodes and chronic liver disease, finally progressing to liver cirrhosis. Patients with end-stage EPP-associated liver disease require liver transplantation. We describe here a 31-year-old male patient with EPP who experienced acute-on-chronic liver failure and underwent deceased-donor liver transplantation. Surgical and postoperative care included specific shielding from exposure to ultraviolet radiation to prevent photosensitivity-associated adverse effects. The patient recovered uneventfully and was doing well 24 months after transplantation. Future prevention and treatment of liver disease are discussed in detail.
Acute Disease ; Adult ; End Stage Liver Disease/etiology/pathology/*therapy ; Ferrochelatase/genetics/metabolism ; Humans ; Liver Cirrhosis/diagnosis ; *Liver Transplantation ; Male ; Mutation ; Protoporphyria, Erythropoietic/complications/*diagnosis/pathology

Objective: To identify the predisposing factors, clinical characteristics, and risk factors of disease progression to establish a novel predictive survival model and evaluate its application value for hepatitis B virus-related acute-on-chronic liver failure. Methods: 153 cases of HBV-ACLF were selected according to the guidelines for the diagnosis and treatment of liver failure (2018 edition) of the Chinese Medical Association Hepatology Branch. Predisposing factors, the basic liver disease stage, therapeutic drugs, clinical characteristics, and factors affecting survival status were analyzed. Cox proportional hazards regression analysis was used to screen prognostic factors and establish a novel predictive survival model. The receiver operating characteristic curve (ROC) was used to evaluate predictive value with the Model for End-Stage Liver Disease (MELD) and the Chronic Liver Failure Consortium Acute-on-Chronic Liver Failure score (CLIF-C ACLF). Results: 80.39% (123/153) based on hepatitis B cirrhosis had developed ACLF. HBV-ACLF's main inducing factors were the discontinuation of nucleos(t)ide analogues (NAs) and the application of hepatotoxic drugs, including Chinese patent medicine/Chinese herbal medicine, non-steroidal anti-inflammatory drugs, anti-tuberculosis drugs, central nervous system drugs, anti-tumor drugs, etc. 34.64% of cases had an unknown inducement. The most common clinical symptoms at onset were progressive jaundice, poor appetite, and fatigue. The short-term mortality rate was significantly higher in patients complicated with hepatic encephalopathy, upper gastrointestinal hemorrhage, hepatorenal syndrome, and infection (P < 0.05). Lactate dehydrogenase, albumin, the international normalized ratio, the neutrophil-to-lymphocyte ratio, hepatic encephalopathy, and upper gastrointestinal bleeding were the independent predictors for the survival status of patients. The LAINeu model was established. The area under the curve for evaluating the survival of HBV-ACLF was 0.886, which was significantly higher than the MELD and CLIF-C ACLF scores (P < 0.05), and the prognosis was worse when the LAINeu score ≥ -3.75. Conclusion: Discontinuation of NAs and the application of hepatotoxic drugs are common predisposing factors for HBV-ACLF. Hepatic decompensation-related complications and infection accelerate the disease's progression. The LAINeu model can predict patient survival conditions more accurately.
Humans ; Hepatitis B virus ; Hepatic Encephalopathy/complications* ; Acute-On-Chronic Liver Failure/diagnosis* ; End Stage Liver Disease/complications* ; Severity of Illness Index ; Risk Factors ; ROC Curve ; Prognosis ; Retrospective Studies

OBJECTIVETo study the clinical characteristics of hepatitis B virus-related acute-on-chronic liver failure patients with familial aggregation.

METHODS275 patients with hepatitis B virus--related acute-on-chronic liver failure were investigated. The patients were divided into familial aggregation and non-familial aggregation group basis on their epidemiological features. Clinical data and biochemical indicators between the two groups were analyzed statistically.

RESULTS93 of 275 patients (33.82%) case were family aggregation. There was no significant difference compared with chronic hepatitis B patients (38.3%). The mean age of the two groups was 45.98 and 43.61 years old, respectively (P > 0.05). The rates of liver cirrhosis in family aggregation group were significant higher than non-familial aggregation group (73.91% vs 58.24%, p < 0.05). Serum total (TBil) and prothrombin activities (PTA) were no significant difference between the two groups, but ALT level in familial aggregation group was much higher (407.80 U/L vs 256.45 U/L, P 0.05).

CONCLUSIONFamilial aggregation were not related to acute-on-chronic liver failure in chronic HBV hepatitis patients. But the rate of liver cirrhosis were higher in patients with familial aggregation.

Acute Disease ; Adolescent ; Adult ; Aged ; Alanine Transaminase ; blood ; End Stage Liver Disease ; etiology ; genetics ; Family ; Female ; Hepatitis B ; complications ; Humans ; Liver Cirrhosis ; epidemiology ; Male ; Middle Aged