Objective: To investigate the application of antigen avoidance pattern for compatible platelets matching. Methods: Samples from two patients with immune-mediated platelet transfusion refractoriness were screened for platelet antibodies using solid-phase red blood cell adhesion assay (SPRCA). The genotypes of HLA-A, -B loci were determined via ploymerase chain reaction sequence. The specificity of HLA class I antibodies was detected using Luminex technology. Results: Platelet antibody screening via SPRCA yielded positive results in both samples. Antibody specificity testing showed the presence of antibodies against HLA-B65, A80, B13, as well as antibodies against HLA-A11, B52, A24 respectively, with both patients exhibiting 80 kinds of positive antibodies. The antibody avoidance pattern successfully selected compatible platelets for transfusion. The bleeding symptoms of two patients were improved after compatible platelets transfusion. Conclusion: For blood stations with limited platelet gene bank resources, antibody avoidance pattern for compatible platelets matching represents an effective strategy for immune-mediated platelet transfusion refractoriness.

Hepatitis B vaccination is the most economical and effective way to prevent HBV infection.The advances in molecular biology and genetic engineering have continuously improved the manufacturing process of vaccines,and hepatitis B vaccine has gradually developed from the initial plasma-derived vaccine to the currently used recombinant vaccine.Preventive hepatitis B vaccine has been clinically tested in patients with HBsAg seroclearance to increase the level of anti-HBs,with certain safety and efficacy.As one of the multiple targets for new drugs in the treatment of chronic hepatitis B,a therapeutic hepatitis B vaccine based on HBsAg is already in the stages of research and development and clinical trial.

Objective To investigate the status of occult hepatitis B virus infection(OBI)among blood donors in Quzhou,Zhejiang,and to analyze the mutation of S region in blood donors with anti-HBc+alone and non anti-HBc+alone.Methods The OBI samples were screened by ELISA and NAT;the HBV DNA was amplified and sequenced;20 anti-HBc+alone and 25 not anti-HBc+alone samples were obtained.Results The detection rate of OBI in Quzhou was 0.10%(155/161 045),and the positive rate of anti-HBc was 74.19%(115/155).The detection rate of OBI increased with the age of blood donors(P＜0.05),but was not related to gender.The positive rate of anti-HBc+alone was 22.58%(35/155),and that of not anti-HBc alone was 51.61%(80/155).Among the 45 OBI sequencing samples,the proportion of B and C geno-type was73.33%(33/45)and 20.00%(9/45),respectively.The mutation sites of blood donors in the anti-HBc+alone group were more than those in the not anti-HBc+alone group,and the mutation rates of S114T and V168A on MHR were significantly different(P＜0.05).Conclusion The genotype of OBI infection in Quzhou is mainly type B.The mutation sites of blood donors with anti-HBc+alone are higher than those with not anti-HBc+alone,which may be more suitable as one of the OBI screening indicators.

Objective To investigate the imaging manifestations and pathological basis of hepatic angiosarcoma(HAS).Methods The CT and MRI findings of 12 patients with HAS confirmed by pathology were analyzed retrospectively and compared with pathological findings.Results Based on morphological classification,the 12 cases of HAS were categorized into three types:massive patterns(n=3),mixed patterns of mass with nodules(n=2),and diffuse infiltration patterns(n=7).Hemorrhage was observed in 9 cases,and necrosis was present in all 12 cases.The massive patterns exhibited peripheral or nodular,patchy,annular,and cord-like enhancement patterns during the arterial phase,with increasing enhancement during the portal and delayed phases.The mixed patterns of mass with nodules demonstrated mild enhancement around the margin or in patchy and spotty structures during the arterial phase,progressing to expansive enhancement during the portal and delayed phases.Four of the seven diffuse infiltration patterns presented with mesh enhancement during the arterial phase,which expanded and became diffuse during the portal phase,accompanied by progressively enlarged enhancing nodules.In the delayed phase,the lesions were fused.The other three cases showed diffuse nodular enhancement during the arterial phase followed by increased enhancement during both portal phase and delayed phase.Regardless of subtype,focal fusion occurred during the delayed phase when multiple intrahepatic lesions were present,and the hemorrhagic and necrotic parts did not enhance.Conclusion The imaging characteristics of HAS include heterogeneous and progressive enhancement,often accompanied by hemorrhage,cystic change,and necrosis.

Hepatitis B virus (HBV) DNA integration occurs during the reverse transcription process of HBV replication, which develops in the early stages of HBV infection and accompanies the entire disease course. The integration of HBV DNA is detrimental to the attainment of clinical cure goals and also raises the risk of developing liver cancer. Theoretically, nucleos(t)ide analogs can reduce the synthesis of new double-stranded linear DNA, but there is no clearance function for hepatocytes that have already integrated HBV. Therefore, patients with serum HBV DNA-negative conversions still have the risk of developing liver cancer. As an immunomodulatory drug, interferon can not only inhibit viral replication but also inhibit or even eliminate existing clonally amplified hepatocytes carrying integrated HBV DNA fragments. However, there are currently few studies on the effects of nucleos(t)ide analogues and interferon therapy on HBV DNA integration. Thus, large-scale clinical studies are urgently needed for further clarification.

Wilson's disease (WD) is a kind of inherited metabolic liver disease in which most patients need lifelong medication to maintain copper homeostasis in the body. Zinc is one of the most commonly used drugs for WD treatment. However, there are currently few high-quality, large-sample, and prospective clinical trials on zinc agent-treated WD. The selection and application of zinc agents are mainly based on patients' clinical phenotype, tolerance to zinc agents, and physicians' experience in treating WD. This article summarizes the application of zinc agents in WD.

Background/Aims: To investigate the autoantibody against fumarate hydratase (FH), which is a specific liver failure-associated antigen (LFAA) and determine whether it can be used as a biomarker to evaluate the prognosis of acute-on-chronic liver failure (ACLF). Methods: An immunoproteomic approach was applied to screen specific LFAAs related to differential prognosis of ACLF (n=60). Enzyme-linked immunosorbent assay (ELISA) technology was employed for the validation of the frequency and titer of autoantibodies against FH in ACLF patients with different prognoses (n=82). Moreover, we clarified the expression of autoantibodies against FH in patients with chronic hepatitis B (n=60) and hepatitis B virus-related liver cirrhosis (n=60). The dynamic changes in the titers of autoantibodies against FH were analyzed by sample collection at multiple time points during the clinical course of eight ACLF patients with different prognoses. Results: Ultimately, 15 LFAAs were screened and identified by the immunoproteomic approach.Based on ELISA-based verification, anti-FH/Fumarate hydratase protein autoantibody was chosen to verify its expression in ACLF patients. ACLF patients had a much higher anti-FH autoantibody frequency (76.8%) than patients with liver cirrhosis (10%, p=0.000), patients with chronic hepatitis B (6.7%, p=0.022), and normal humans (0%, p=0.000). More importantly, the frequency and titer of anti-FH protein autoantibodies in the serum of ACLF patients with a good prognosis were much higher than that of patients with a poor prognosis (83.9% vs 61.5%, p=0.019; 1.41±0.85 vs 0.94±0.56, p=0.017, respectively). The titer of anti-FH autoantibodies showed dynamic changes in the clinical course of ACLF. Conclusions The anti-FH autoantibody in serum may be a potential biomarker for predicting the prognosis of ACLF.

Objective To investigate the ability of combined baseline serum markers, i.e., HBV DNA, HBV RNA, HBsAg, and HBcrAg, to predict HBeAg seroconversion in patients with HBeAg-positive chronic hepatitis B (CHB) treated by nucleos(t)ide analogues. Methods A retrospective analysis was performed for 83 HBeAg-positive patients selected as subjects from the prospective CHB follow-up cohort established by Difficult & Complicated Liver Diseases and Artificial Liver Center, Beijing YouAn Hospital, Capital Medical University, from June 2007 to July 2008, and the baseline serum levels of HBV DNA, HBV RNA, HBsAg, and HBcrAg were analyzed. The t -test or the Mann-Whitney U test was used for comparison of continuous data between two groups, and the chi-square test was used for comparison of categorical data between two groups. The Spearman method was used for correlation analysis. A Cox regression model was established to calculate HBeAg seroconversion prediction score, and the time-dependent receiver operating characteristic curve was used to evaluate the ability of combined markers in predicting HBeAg seroconversion. The Kaplan-Meier method was used to calculate cumulative seroconversion rate in each group, and the Log-rank test was used for comparison between groups. Results For the 83 HBeAg-positive patients, the median follow-up time was 108 months, and 44.58%(37/83) of these patients achieved HBeAg seroconversion. Compared with the non-seroconversion group, the HBeAg seroconversion group had significantly lower baseline serum levels of HBV DNA [6.23(1.99-9.28) log 10 IU/mL vs 7.69(2.05-8.96) log 10 IU/mL, Z =-2.345, P =0.019] and HBV RNA [4.81(1.40-7.53) log 10 copies/mL vs 6.22(2.00-8.49) log 10 copies/mL, Z =-1.702, P =0.010], and there were no significant differences in the levels of HBsAg and HBcrAg between the two groups ( P > 0.05). The Cox regression equation constructed based on the above serum markers showed a median score of 0.95(range 0.37-3.45) for predicting HBeAg seroconversion. In the total population, the combined score was negatively correlated with HBsAg, HBV DNA, HBV RNA, and HBcrAg ( r =-0.697, -0.787, -0.990, and -0.819, all P < 0.001). Based on the median prediction score, the patients were divided into high HBeAg seroconversion group and low HBeAg seroconversion group; as for the prediction of HBeAg seroconversion rate at 36, 60, and 84 months, the high HBeAg seroconversion group had a seroconversion rate of 43.90%, 51.20%, and 63.10%, respectively, while the low HBeAg seroconversion group had a seroconversion rate of 9.60%, 17.00%, and 19.8%, respectively, and there was a significant difference between the two groups ( χ 2 =11.6, P < 0.001). Conclusion The combined prediction score based on baseline serum HBV markers can predict HBeAg seroconversion in CHB patients treated by nucleos(t)ide analogues.

Objective:To investigate the family gene features in Crigler-Najjar syndrome (CNS) type II.Methods:The UGT1A1 gene and related bilirubin metabolism genes were comprehensively analysed in a CNS-II family (3 CNS-II, 1 Gilbert syndrome, and 8 normal subjects). The genetics basis of CNS-II were investigated from the perspective of family analysis. Results:In three cases, compound heterozygous mutations at three sites of the UGT1A1 gene (c.-3279T > G, c.211G > A and c.1456T > G) caused CNS-II. Gilbert syndrome and CNS-II were not significantly associated with distribution or diversity loci. Conclusion:The compound heterozygous pathogenic mutations (c.-3279T > G, c.211G > A, and c.1456T > G) at three loci of the UGT1A1 gene may be the feature of the newly discovered CNS-II family genes based on the CNS-II family study.

Inherited metabolic liver diseases can occur in multi-age groups such as children, adolescents, adults, and others. With the improvement of diagnosis and treatment levels, more and more patients with childhood-onset diseases are surviving into adulthood. Some diseases originally faced by pediatric hepatologists also appear in adult hepatology clinics. This raises new challenges for adult hepatologists, requiring them to master more professional knowledge. However, specific data on the incidence rate of most inherited metabolic liver diseases is still lacking in our country. This article reviews the research progress of hereditary metabolic liver diseases and summarizes the epidemiological characteristics of familiar hereditary metabolic liver diseases in China.