1. Department of Hepatitis C and Drug Induced Liver Injury, Beijing YouAn Hospital, Capital Medical University, Beijing 100069, China 2. Beijing Artificial Liver Treatment & Training Center, Beijing YouAn Hospital, Capital Medical University, Beijing 100069, China 3. Collaborative Innovation Center of Infectious Diseases, Beijing 100069, China 4. Division of Gastroenterology and Hepatology, University of California, Irvine, Medical Center, Orange, CA 92868, USA
Liver failure (LF) is defined as severe dysfunction in hepatic synthesis, detoxification, and metabolism induced by various etiologies. Clinical presentation of LF typically includes severe jaundice, coagulation disorder, hepatic encephalopathy, and ascites. LF can be classified into acute LF, acute-on-chronic LF (ACLF), and chronic LF. ACLF has been demonstrated as a distinct syndrome with unique clinical presentation and outcomes. The severity, curability, and reversibility of ACLF have attracted considerable attention. Remarkable developments in ACLF-related conception, diagnostic criteria, pathogenesis, and therapy have been achieved. However, this disease, especially its diagnostic criteria, remains controversial. In this paper, we systemically reviewed the current understanding of ACLF from its definition, etiology, pathophysiology, pathology, and clinical presentation to management by thoroughly comparing important findings between east and west countries, as well as those from other regions. We also discussed the controversies, challenges, and needs for future studies to promote the standardization and optimization of the diagnosis and treatment for ACLF.
No specific criteria (China, the only ?factor is PTA<40%)
Pathology
Extensive necrosis
Liver fibrosis in various degrees, ?acute hepatocyte injury, and necrosis
Diffuse hepatic fibrosis and forma-?tion of pseudo-lobules accompa-?nied with distributed hepatocyte ?necrosis
Clinical presentation
HE, brain edema, coagulation defects
Coagulation defects, hemodynamic ?dysfunction, immune function failure, ?SIRS, and multiple organ failure
Multiple organ failure, and SIRS
Therapy
Liver transplantation
Spontaneous recovery, transplantation
Liver transplantation
Prognostic indices
King’s college score
Liver specific model (CTP and MELD), ?general model (SAPS II and APACHE), ?and organ failure model (OSF, SOFA, ?and CLIF-SOFA)
MELD
Fatality rate
High
Higher than cirrhosis with same MELD ?score
Tab.1
CMA*
APASL
EASL-AASLD
Definition
Acute (usually within four weeks) liver ?decompensation in patients with CLDs
Acute hepatic insult manifesting as ?jaundice and coagulopathy, complicated ?within four weeks by ascites and/or ?encephalopathy in a patient with pre-?viously diagnosed or undiagnosed CLD
Acute deterioration of pre-existing CLD, ?usually related to a precipitating event and ?associated with increased mortality at three ?months due to multi-system organ failure
Diagnostic criteria
1. Progressively deepening jaundice ?(TBil ?≥10?mg/dl or daily increase ??≥1?mg/dl)2. PTA≤40% or INR?≥1.53. with or without HE or other ?complications4. divided into 3 grades
1. Previously diagnosed or undiagnosed ?CLD2.TBil>?5?mg/dl and INR>1.5 or PTA??<?40%3.Ascites and/or encephalopathy in four ?weeks4. No grades
ACLF-1: renal failure or a non-renal organ ?failure associated with creatinine 1.5–2 ?mg/dl and/or grades I–II encephalopathy?ACLF-2: two organ failures?ACLF-3: three or more organ failures
Predisposition
Compensated liver disease
Compensated liver disease
Stable compensated or decompensated ?cirrhosis to date
Precipitating factors
Not mentioned
Infectious and noninfectious causes direct ?hepatic insult
Infectious and noninfectious causes direct ?hepatic insult or not (especially infection)
Tab.2
Fig.1
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