Hepatolenticular degeneration is kind of an autosomal recessive genetic disease with diverse, complex and non-specific clinical manifestations, high misdiagnosis rate, rapid disease progression, poor drug treatment effect, and high mortality. It is one of the rare several genetic metabolic diseases in clinic that could be cured by liver transplantation method. Liver transplantation provides healthy P-type ATP enzyme through the donor liver, which can correct its genetic defects, improve copper metabolism disorders, relieve clinical symptoms, improve the quality of life, and improve the survival rate of patients. Liver transplantation is playing an increasingly important role as an important means to treat hepatolenticular degeneration. With the rapid development of partial living donor liver transplantation, auxiliary liver transplantation, domino-assisted liver transplantation and cross-assisted domino liver transplantation, a new way has been provided for patients with hepatolenticular degeneration, alleviating the problem of donor liver shortage and shortening the waiting time of recipients, which has certain clinical value and development prospects. In this paper, a review of the research progress in the treatment of hepatolenticular degeneration with liver transplantation was made with reference to the relevant literature at home and abroad.

Objective To assess effect of ultrasound-guided erector spinae plane block(ESPB)combined with patient controlled intravenous analgesia(PCIA)on postoperative analgesia after cesarean section.Methods A total of 120 full-term singleton pregnant women who underwent cesarean section under spinal anesthesia at Jiaxing Maternal and Child Health Care Hospital from May 2022 to August 2023 were selected.Participants were randomly divided into three groups using a random number table:Group E(ESPB combined with PCIA),group T[transversus abdominis plane(TAP)block combined with PCIA],and control group(PCIA alone),with 40 women in each group.Visual analogue scale(VAS)and Bruggrmann comfort scale(BCS)scores during rest and coughing were recorded at 4h,8h,12h,24h,and 48h postoperatively.Number of effective PCIA presses,total sufentanil dosage,proportion of rescue analgesia and maternal satisfaction were also documented within 48h.Additionally,adverse reactions and neonatal outcomes were observed within the same 48h period.Results Postoperatively,VAS scores for rest and coughing in group E at 8h,12h,24h were significantly lower than those in group T,those in two groups were lower than those in control group(P＜0.05).Postoperatively at 8h,12h,and 24h,BCS scores in group E were significantly higher than those in group T,with both higher than those in control group(P＜0.05).Within 48h after surgery,the number of effective PCIA presses,proportion of rescue analgesia and total sufentanil dosage in group E were lower than those in group T,both lower than those in control(P＜0.05).Moreover,maternal satisfaction score in group E was significantly higher than that in group T,that in two groups was significantly higher than that in control group(P＜0.05).Within 48h after surgery,there were no significant differences in adverse reactions or neonatal outcomes among the three groups(P＞0.05).Conclusion Ultrasound-guided ESPB combined with PCIA outperforms TAP block combined with PCIA,with reducing analgesic dose and enhancing maternal satisfaction and comfort.

Objective:To investigate the clinical characteristics of psoriasis and status quo of medical care for patients in county areas of China.Methods:This study was a cross-sectional investigation. Based on the “Qianxian Wuyin” Project (a national project for upgrating ability for psoriasis care at county level), an online questionnaire survey was conducted in the dermatology departments of 459 county hospitals in 404 pilot administrative counties across China from February to June 2023. The questionnaire included demographic information of patients (gender, ethnicity, age, place of residence, education, marital status), and clinical characteristics of psoriasis (disease course, type, comorbidities, body surface area (BSA) and previous treatment. The Dermatology Life Quality Index (DLQI) and Psoriasis Area and Severity Index (PASI) were applied for assessing the quality of life and disease severity, and completed by patients or guardian and doctors, respectively.Results:A total of 16 935 patients completed the questionnaire. The age of patients was 1-102(44.17±11.58)years, and 71.0% (12 036/16 935) were 30-59 years old. The ratio of male to female was 2.21∶1; 24.3%(4 117/16 935) of patients had high school education; there were 9 940 patients(58.7%) with previous or current smoking and/or alcohol use; 42.8%(7 218/16 855) of patients had a disease course of 1-5 years. There were 15 630 patients(92.3%) with DLQI≥10, 8 346 patients(49.7%) with PASI≥10, 15 017 patients(89.2%) with BSA≥10%. The plaque type was the most common disease type ( n=14 965, 88.7%), and spotting type ranked the second ( n=1 141, 6.8%). The most common initial site was the trunk ( n=12 309, 72.9%). Among the comorbidities, hypertension was the most common one ( n=1 681, 10.0%). There were 7 650 reports of treatment response to conventional topical drug therapy and 3 112 reports of treatment response to systemic drug therapy, with 6 269 (81.9%) and 2 493 (80.1%) reporting poor or no response, respectively. Conclusions:The survey shows that in the county areas of China, the majority of psoriasis patients are severe patients with short course of disease, plaque type is the most common type, and hypertension is the most common comorbidity; and the conventional treatment is less effective for most patients.

Objective To summarize clinical experience of transabdominal pericardial anastomosis of suprahepatic vena cava of the donor and right atrium of the recipient in liver transplantation for Budd-Chiari syndrome (BCS) complicated with liver cancer. Methods Clinical data of a BCS patient complicated with liver cancer undergoing transabdominal pericardial anastomosis of suprahepatic vena cava and right atrium in liver transplantation were retrospectively analyzed. Results The hepatic vein and suprahepatic vena cava were partially occluded in the patient. Liver transplantation was completed by transabdominal pericardial anastomosis of suprahepatic vena cava and right atrium with beating-heart. In addition, due to pathological changes of the recipient's hepatic artery, splenic artery of the recipient was cut off, distal ligation was performed, and the proximal end was reversed and anastomosed with the common hepatic artery of the donor liver, and the reconstruction of hepatic artery was completed. The surgery was successfully performed. At approximately postoperative 1 week, the function of the liver allograft was gradually restored to normal, and no major complications occurred. The patient was discharged at postoperative 25 d. No signs of BCS recurrence was reported after 8-month follow-up. Conclusions It is safe and feasible to treat BCS by liver transplantation with transabdominal pericardial anastomosis of suprahepatic vena cava and right atrium. BCS patients complicated with liver cancer obtain favorable prognosis.

A 24-year-old male patient was admitted to the Organ Transplant Department of the 900th Hospital of the Joint Logistic Support Force on March 13, 2000, due to repeated abdominal distension accompanied by edema of both lower limbs for more than 7 months and aggravated for 1 month. Clinical diagnosis: hepatolenticular degeneration, metabolic encephalopathy, decompensated stage of cirrhosis. Orthotopic liver transplantation was performed under general anesthesia on March 24, 2000. The postoperative recovery is smooth, and the patient has survived for more than 23 years, with normal life and work.

Objective:To investigate the effect of different spray-coagulation time of argon plasma coagulation (APC) injury on the Glisson system primary branche(G1) in the hepatic portal of pigs.Methods:Fifty clean healthy domestic pigs (27 females and 23 males, aged 7 to 14 months) were selected, with the body weighted (100.0±9.5) kg. They were randomly divided into five groups (A, B, C, D, and E), with 10 pigs in each group. G1 models were made and sprayed by APC for 1, 2, 3, 4, and 5 seconds. The damage, maximum damage area, maximum damage depth, and damage of the three branches of the Glisson system (the first branches of the portal vein, intrahepatic bile duct, and hepatic artery) were compared among the groups. The pigs were divided into two groups based on whether the three branches were damaged or not: the three-branch damage group ( n=23) and the control group ( n=27). The maximum damage area and maximum damage depth were compared between the two groups. Results:After the APC spraying, circular or elliptical damage appeared on the surface of the G1, with changes such as yellow-brown color, brown color, charred appearance, and defects. Under the microscope, G1 capsule was found to be deficient, the fibrous tissue beneath the capsule was ruptured, and the structures of small blood vessels and small bile ducts were incomplete. " Burn marks" and damage to the three branches of the Glisson system in G1 were also observed, and the damage was more severe at the center of the spray-coagulation. As the spray-coagulation time increased, the maximum damage area of the G1 model also increased, and the two were positively correlated ( r=0.90, P<0.001). The maximum damage depth was also positively correlated with spray-coagulation time ( r=0.97, P<0.001). The numbers of pigs with damage to the three branches of the Glisson system in Groups A-E were 0, 2, 5, 6, and 10, respectively, and the number of pigs with damage increased with the spray-coagulation time. In the three-branch damage group, the spray-coagulation time, maximum damage area, and maximum damage depth were all higher than those in the control group (without three-branch damage), and the differences were statistically significant (all P<0.05). Conclusion:The degree of damage to G1 caused by APC is positively correlated with the spray-coagulation time, and damage to the three branches of the Glisson system in G1 is related to the maximum damage area, maximum damage depth, and APC spray-coagulation time.

Objective To evaluate the effect of different techniques of hepatic artery reconstruction on postoperative hepatic artery complications and clinical prognosis in liver transplantation. Methods Clinical data of 140 liver transplant recipients were retrospectively analyzed. All recipients were divided into the conventional hepatic artery reconstruction group (n=123) and special hepatic artery reconstruction group (n=17) according to hepatic artery reconstruction methods. Intraoperative and postoperative clinical indexes, the incidence of postoperative hepatic artery complications and survival rate were compared between two groups. Results The alanine aminotransferase (ALT) and aspartate aminotransferase (AST) levels at postoperative 1 d, total bilirubin (TB) at postoperative 7 d and prothrombin time international normalized ratio (PT-INR) at postoperative 30 d in special hepatic artery reconstruction group were higher than those in conventional hepatic artery reconstruction group, and the differences were statistically significant (all P < 0.05). There were no significant differences in the operation time, anhepatic phase, intraoperative blood loss, intraoperative transfusion volume of red blood cells, cold or warm ischemia time, the length of intensive care unit (ICU) stay, the length of hospital stay and postoperative blood flow of liver allograft between two groups (all P > 0.05). In the conventional hepatic artery reconstruction group, 5 recipients developed hepatic artery complications, whereas no hepatic artery complications occurred in the special hepatic artery reconstruction group, with no significant difference between two groups (P > 0.05). In the special hepatic artery reconstruction group, the 1-, 3- and 5-year cumulative survival rates were equally 82.4%, compared with 85.0%, 78.9% and 75.6% in the conventional hepatic artery reconstruction group, respectively. There was no significant difference between two groups (all P > 0.05). Conclusions When hepatic artery variations and (or) lesions are detected in donors and recipients, use of special hepatic artery reconstruction may effectively restore the hepatic arterial blood flow of liver allograft after liver transplantation, and will not affect the incidence of hepatic artery complications and survival rate of the recipients following liver transplantation.

Objective:To evaluate the effects of pegylated interferon (Peg-IFN) alfa-2b combined with nucleotide analogues (NAs) on the recurrence of hepatitis B-related liver cancer after resection, and to explore the changes of HBsAg and HBV DNA in patients with chronic hepatitis B liver cancer during postoperative treatment.Methods:The prospective study was conducted. Clinical data of 43 patients with hepatitis B-related liver cancer who underwent radical resection treated in 900th Hospital of People′s Liberation Army were prospectively analyzed from January 2020 to December 2021. Among 43 patients, there were 39 males and 4 females, the age was 30-76 years. According to different treatment methods they were divided into two groups, the patients treated by Peg-IFN alfa-2b combined with NAs were devided into the IFN group( n=10), and those treated by NAs alone into the NAs group( n=33). Two-pair semi-quantitative were collected every 3 months after operation. The recurrence-free survival rate, recurrence time after 2 years in the two groups, the clearance rate and the negative rate of HBsAg and HBV DNA in the two groups. Peg-IFN alfa-2b was evaluated in improving the prognosis of hepatitis B-related liver cancer. The measurement data of normal distribution were expressed by mean±standard deviation ( ± s), and t-test was used for comparison between the two groups. Chi-square test was used for comparison between the two groups of count data. Repeated analysis of measurement variance was used for analysis HBsAg and HBV DNA changes of the interferon group overall survival time and recurrence-free surrival time of patients was estimated using Kaplan-Meier method and the difference between groups was assessed using Log-rank test. Results:HBsAg and HBV DNA: The HBsAg clearance rate at 24 weeks and that at 48 weeks in the IFN group were 24.6% and 59.0% respectively. The HBsAg negative rate at 48 weeks was 16.7%. The HBV DNA clearance rate at 24 weeks and that at 48 weeks were 33.9% and 53.8% respectively. The HBV DNA negative rate was 0 at 48 weeks. The levels of HBsAg and HBV DNA in the IFN group decreased gradually with time. There were statistically differences between the levels of HBsAg and HBV DNA at 0 weeks, 24 weeks and 48 weeks( P<0.05). The 2-year overall survival rates of IFN group and NAs group were 100% and 90.9% respectively. The 2-year recurrence-free survival rates were 90.0% and 63.6% respectively. There were no significant statistical differences in the overall survival rate and recurrence-free survival rate between the groups ( P>0.05). The postoperative recurrence time of the IFN group and the NAs group were (15.00±7.07) months and (5.78±3.39) months respectively. The difference between the two groups was statistically significant ( t=3.160, P<0.01). Conclusion:Long-term antiviral therapy of Peg-IFN alfa-2b combined with NAs can prolong the recurrence time of liver cancer, reduce the levels of HBsAg and HBV DNA in serum, and potentially improve the survival rate of the patients compared with therapy of NAs alone.

OBJECTIVE: To evaluate the performance of recombinase-aided amplification (RAA) assay in detection of Clonorchis sinensis metacercariae in freshwater fish samples, so as to provide insights into standardization and field application of this assay. METHODS: Wild freshwater fish samples were collected in the rivers of administrative villages where C. sinensis-infected residents lived in Jiangyan District, Xinghua County and Taixing County of Taizhou City, Jiangsu Province from June to September 2022. Genomic DNA was extracted from six freshwater fish specimens (5 g each) containing 0, 1, 2, 4, 8 and 16 C. sinensis metacercariae for fluorescent RAA assay, and the diagnostic sensitivity was evaluated. Fluorescent RAA assay was performed with genomic DNA from C. sinensis, Metorchis orientalis, Haplorchis pumilio and Centrocestus formosanus metacercariae as templates to evaluate its cross-reactions. In addition, the detection of fluorescent RAA assay and direct compression method for C. sinensis metacercariae was compared in field-collected freshwater fish samples. RESULTS: Positive amplification was found in fresh-water fish specimens containing different numbers of C. sinensis metacercariae, and fluorescent RAA assay was effective to detect one C. sinensis metacercaria in 5 g freshwater fish specimens within 20 min. Fluorescent RAA assay tested negative for DNA from M. orientalis, H. pumilio and C. formosanus metacercariae. Fluorescent RAA assay and direct compression method showed 5.36% (93/1 735) and 2.88% (50/1 735) detection rates for C. sinensis metacercariae in 1 735 field-collected freshwater fish samples, with a statistically significant difference seen (χ² = 478.150, P < 0.001). There was a significant difference in the detection of C. sinensis metacercariae in different species of freshwater fish by both the direct compression method (χ² = 11.20, P < 0.05) and fluorescent RAA assay (χ² = 20.26, P < 0.001), and the detection of C. sinensis metacercariae was higher in Pseudorasbora parva than in other fish species by both the direct compression method and fluorescent RAA assay (both P values < 0.05). CONCLUSIONS Fluorescent RAA assay has a high sensitivity for detection of C. sinensis metacercariae in freshwater fish samples, and has no cross-reactions with M. orientalis, H. pumilio or C. formosanus metacercariae. Fluorescent RAA assay shows a higher accuracy for detection of C. sinensis infections in field-collected freshwater fish than the direct compression method.
Animals ; Clonorchis sinensis/genetics* ; Metacercariae/genetics* ; Recombinases ; Fresh Water ; Fishes ; DNA ; Fish Diseases/diagnosis*

Objective:To explore the expression features of cytochrome C oxidase subunit Ⅰ (MT-CO1), BCL2 interacting protein 3 (BNIP3) and interleukin (IL)-1β in the liver of MRL/lpr lupus mice.Methods:The mRNA and protein levels of MT-CO1, BNIP3, IL-1β, p16 and p21 in lupus mice and control mice were detected by polymerase chain reaction (PCR) and Western blot, the IL-1β expression site were detected by hematoxylin and eosin (HE) staining and immunohistochemical method, and themalondialdehyde (MDA) was detected by colorimetry. Hepatocytes and macrophages were stimulated with lipopolysaccharide (LPS), while hepatocytes were also cultured with supernatants obtained after macrophages stimulated with LPS, and the mRNA and protein levels of MT-CO1, BNIP3 and LC3B, as well as p16 and p21 expression, were determined by qPCR and Western blot. The expression of mitochondrial reactive oxygen species (mtROS) was detected by immunofluorescence. One way Analysis of Variance (ANOVA) was used to compare the mean of each group, and LSD method was used to compare the means of multiple samples, and Tamhane's T2 method was used to compare the means of multiple samples when the variance was uniform. Results:The results of PCR showed that the mRNA levels of MT-CO1 and BNIP3 in the liver tissue of the lupus group (0.14±0.04; 0.16±0.05) were significantly lower than those of the control group (0.11±0.04; 0.16±0.06), and the differences were statistically significant ( t=7.16, P<0.001; t=4.54, P<0.001). The expression levels of IL-1β, p16 and p21 in the lupus group (2.06±0.69; 0.37±0.14; 0.16±0.06) were significantly higher than those of the control group (0.23±0.06; 0.25±0.08; 0.11±0.04) ( t=9.58, P<0.001; t=24.35, P<0.001; t=22.36, P<0.001). The results of Western blot were consistent with those of PCR. HE staining showed lymphocyte infiltration in the liver tissue of lupus mice, and immunohistochemistry showed IL-1β in the liver tissue of lupus mice. The positive cells were mainly concentrated in the sinusoids, and the expression of hepatic parenchymal cells was not rearkable. The content of MDA in liver tissue of the lupus group (0.19±0.10) was higher than that of the control group (0.17±0.09), and the difference was statistically significant ( t=4.33, P=0.005). LPS directly stimulated AML12 hepatocytes (0.069±0.028; 0.17±0.07). The PCR results showed that compared with the control group (0.176±0.072; 0.08±0.03), the expression of MT-CO1, and BNIP3 were not significantly different ( t=1.01, P=0.337; t=0.88, P=0.399). The expression of IL-1β was significantly higher when incubated with the supernatants of LPS stimulated macrophages (0.28±0.09) compared than that of the control group (0.15±0.05) ( t=28.26, P<0.001). The results of PCR showed that the mRNA levels of MT-CO1 and BNIP3 in the LPS stimulated group (0.046±0.026; 0.17±0.05) were significantly lower than those in the control group (0.143±0.083; 0.18±0.06), and the differences were statistically significant ( t=7.52, P<0.001; t=4.24, P<0.001), The expression of p16 and p21 in LPS stimulated group (0.29±0.09; 0.27±0.09) were significantly higher than those in the control group (0.18±0.06; 0.22±0.07) ( t=13.54, P<0.001; t=8.69, P<0.001). The results of Western blot were consistent with those of PCR. Immunofluorescence showed that the fluorescence intensity of mtROS in LPS stimulated group (0.25±0.10) was higher than that in the control group (0.08±0.03), and the difference was statistically significant ( t= 4.86, P<0.001). Conclusion:Immune-mediated inflammation in the liver tissue of lupus mice can stimulate liver parenchymal cells to cause intracellular mitochondrial dysfunction. However, the mechanism of liver organ damage in lupus mice is not limited to the immune-mediated inflammation of immune active cells, but also include parenchymal cell mitochondrial dysfunction.