Acute-on-chronic liver failure：recent update

Acute on chronic liver failure（ACLF） was first described in 1995 as a clinical syndrome distinct to classic acute decompensation.Characterized by complications of decompensation,ACLF occurs on a background of chronic liver dysfunction and is associated with high rates of organ failure and significant short-term mortality estimated between45%and 90%.Despite the clinical relevance of the condition,it still remains largely undefined with continued disagreement regarding its precise etiological factors,clinical course,prognostic criteria and management pathways.It is concerning that,despite our relative lack of understanding of the condition,the burden of ACLF among cirrhotic patients remains significant with an estimated prevalence of 30.9%.This paper highlights our current understanding of ACLF,including its etiology,diagnostic and prognostic criteria and pathophysiology.It is evident that further refinement of the ACLF classification system is required in order to detect high-risk patients and improve short-term mortality rates.The field of metabolomics certainly warrants investigation to enhance diagnostic and prognostic parameters,while the use of granulocyte-colony stimulating factor is a promising future therapeutic intervention for patients with ACLF.

Recurrent infection triggered encephalopathy syndrome in a pediatric patient with RANBP2 mutation and severe acute respiratory syndrome coronavirus 2 infection

Introduction:Acute necrotizing encephalopathy(ANE),a fatal subtype of infection-triggered encephalopathy syndrome(ITES),can be triggered by many systemic infections.RANBP2 gene mutations were associated with recurrent ANE.Case presentation:Here we report a 1-year-old girl with recurrent ITES and RANBP2 mutation.She was diagnosed with influenza-associated encephalopathy and made a full recovery on the first episode.After severe acute respiratory syndrome coronavirus 2 infection,the patient presented with seizures and deteriorating mental status.Brain magnetic resonance imaging revealed necrotic lesions in bilateral thalami and pons.Methylprednisolone,immunoglobulin,and interleukin 6 inhibitors were administered.Her consciousness level was improved at discharge.Nineteen cases of 2019 coronavirus disease-related ANE have been reported,of which 22.2%of patients died and 61.1%had neurologic disabilities.RANBP2 gene mutation was found in five patients,two of whom developed recurrent ITES.Conclusion:Patients with RANBP2 mutations are at risk for recurrent ITES,may develop ANE,and have a poor prognosis after relapse.

Plasma exchange therapy for acute necrotizing encephalopathy of childhood

Importance Acute necrotizing encephalopathy(ANE)is a rare disease with high mortality.Plasma exchange(PLEX)has recently been reported to treat ANE of childhood(ANEC),but its efficacy is uncertain.Objective This study aimed to investigate the effectiveness of PLEX on ANEC.Methods A retrospective study was conducted in four pediatric intensive care units from December 2014 to December 2020.All patients who were diagnosed with ANEC were included;however,these patients were excluded if their length of stay was less than 24 h.Participants were classified into PLEX and non-PLEX groups.Results Twenty-nine patients with ANEC were identified,10 in the PLEX group and 19 in the non-PLEX group.In the PLEX group,C-reactive protein,procalcitonin,alanine aminotransferase,and aspartate aminotransaminase levels were significantly lower after 3 days of treatment than before treatment(13.1 vs.8.0,P=0.043;9.8 vs.1.5,P=0.028;133.4 vs.31.9,P=0.028;282.4 vs.50.5,P=0.046,respectively).Nine patients(31.0%,9/29)died at discharge,and a significantly difference was found between the PLEX group and non-PLEX group[0 vs.47.4%(9/19),P=0.011].The median follow-up period was 27 months,and three patients were lost to follow-up.Thirteen patients(50.0%,13/26)died at the last follow-up,comprising three(33.3%,3/9)in the PLEX group and ten(58.8%,10/17)in the non-PLEX group,but there was no significant difference between the two groups(P=0.411).Three patients(10.3%,3/29)fully recovered.Interpretation PLEX may reduce serum C-reactive protein and procalcitonin levels and improve liver function in the short term.PLEX may improve the prognosis of ANEC,and further studies are needed.

Hippocampus hypoxia-inducible factor-1 alpha and heme oxygenase-1 expression in the delayed encephalopathy after acute carbon monoxide poisoning rat model

Objective To research the expression of hypoxia-inducible factor-1 alpha(HIF-1α)and heme oxygenase-1(HO-1)in hippocampus of rats with delayed encephalopathy after acute carbon monoxide poisoning(DEACMP)and its functions.Methods One hundred and fiftysix rats were selected and randomly divided into