Primary biliary cholangitis (PBC) is a chronic progressive cholestatic liver disease of unknown etiology characterized by highly specific anti-mitochondrial antibody in serum and immune-mediated non-pyogenic destructive infection in the small intrahepatic bile ducts,which can lead to portal inflammation and fibrosis and finally progress to liver cirrhosis and liver failure.At present,ursodeoxycholic acid (UDCA) is the only drug approved for the treatment of PBC with a recommended dose of 13-15 mg · kg-1 · d-1.There are significant improvements in the survival rate of patients achieving biochemical response after UDCA treatment.However,about 40％ of PBC patients do not respond to UDCA,and such patients have a risk of disease progression and are in urgent need of other drugs.With reference to recent clinical studies and guidelines,this article summarizes the basic concepts and latest advances in pharmacotherapy for PBC,as well as the perspectives of new drugs in clinical trials,in order to bring new hopes to PBC patients with poor response to UDCA.

Objective To explore the correlation of single nucleotide polymorphism ( SNP ) in Nrf2 gene with acute mountain sickness ( AMS) among Han populations in China .Methods As a nested case-control study , 603 Chinese Han young men who had been quickly exposed to 3700 m were adopted and divided into case group and control group ( 369 vs 234,respectively) by Lake Louise Self-assessment Scoring System(LLSS).The Sequenom Mass Array system was used to detect the SNPs of rs10497511 and rs2364722 in Nrf2 gene.Results Alleles of rs10497511 and rs2364722 were respec-tively detected in both case and control groups , which were T-C and A-G.Statistically significant difference was not found in allele frequencies ( P>0.05 ) .Further analysis showed that there was still no significant difference between the codomi -nant, dominant and recessive genotype models (P>0.05).Conclusion rs10497511 and rs2364722 of Nrf2 gene may not be related to susceptibility to AMS in Chinese Han populations .

The clinical features and genetic variants of the patient with sitosterolemia who was referred to Shanghai Children′s Medical Center, Shanghai Jiaotong University School of Medicine from June 2019 to January 2020 were retrospectively analyzed.The patient was treated with Ezetimibe tablets combined with diet control, and the follow-up was performed regularly.Besides, a relevant literature review was conducted.A 7-year and 5-month-old boy was referred to the hospital for " repeated thrombocytopenia for 7 months" with normal serum cholesterol.The whole exome sequencing showed that compound heterozygous mutations (p.Arg446*, p.Gln251*) in ABCG5 gene were inherited from their parents respectively.Hence, he was diagnosed with sitosterolemia.After 29 days of treatment with Ezetimibe tablets combined with diet control, the patient′s platelets returned to normal values without obvious adverse reactions related to drugs.Children with sitosterolemia may present with rare thrombocytopenia, and the therapeutic effects of Ezetimibe tablets combined with diet control are favorable.

Background and objectivePostoperative radiotherapy (PORT) for thymic tumor is still controversial. The object of the study is to evaluate the role of PORT for stage I/II/III thymic tumor.MethodsThe database of Chinese Al-liance of Research for Thymomas (ChART) was retrieved for patients with stage I/II/III thymic tumor who underwent surgi-cal therapy without neoajuvant therapy between 1994 and 2012. Univariate and multivariate survival analyses were performed. Cox proportional hazard model was used to determine the hazard ratio for death.Results 1,546 stage I/II/III patients were identiifed from ChART database. Among these patients, 649 (41.98%) underwent PORT. PORT was associated with gender, histologic type (World Health Organization, WHO), surgical extent, complete resection, Masaoka stage and adjuvant che-motherapy. The 5-yr and 10-yr overall survival (OS) rates and disease-free survival (DFS) rate for patients underwent surgery followed by PORT were 90% and 80%, 81% and 63%, comparing with 96% and 95%, 92% and 90% for patients underwent surgery alone (P=0.001,P<0.001) respectively. In univariate analysis, age, histologic type (WHO), Masaoka stage, complete-ness of resection, and PORT were associated with OS. Multivariable analysis showed that histologic type (WHO)(P=0.001), Masaoka stage (P=0.029) and completeness of resection (P=0.003) were independently prognostic factors of OS. In univari-ate analysis, gender, myasthenia gravis, histologic type (WHO), Masaoka stage, surgical approach, PORT and completeness of resection were associated with DFS. Multivariable analysis showed that histologic type (WHO) (P<0.001), Masaoka stage (P=0.005) and completeness of resection (P=0.006) were independently prognostic factors of DFS. Subgroup analysis showed that patients with incomplete resection underwent PORT achieved the better OS and DFS (P=0.010, 0.017, respectively). However, patients with complete resection underwent PORT had the worse OS and DFS (P<0.001,P<0.001, respectively). ConclusionThe current retrospective study indicated that PORT atfer incomplete resection could improve OS and DFS for patients with stage I/II/III thymic tumor. But for those atfer complete resection, PORT may not help improve prognosis on the whole.

Objective:To evaluate the effect of continuous pump ropivacaine in rectus sheath in the postoperative analgesia after abdominoplasty.Methods:Patients with abdominoplasty who were admitted to the Plastic Surgery Hospital of the Chinese Academy of Medical Sciences from August 2020 to December 2021 were retrospectively analyzed. They were divided into 2 groups: the control group received patient-controlled intravenous analgesia (PCIA) after the operation, sufentanil 100 μg + normal saline diluted to 100 ml, the rate was 2 ml/h, lasted for 48 h, locking time 15 min, Bolus: 0.5 ml; the study group underwent postoperative rectus abdominis intrathecal intubation and continuously infused 0.262 5% ropivacaine saline mixture 100 ml, the rate was 2 ml/h, lasted for 48 h, the locking time is 15 min, Bolus: 0.5 ml. The method of catheter placement in the study group: a catheter was placed unilaterally in the anterior sheath of the rectus abdominis under direct vision, and an incision was made 2 cm below the umbilical plane. The other end is pierced from the umbilicus and connected to the analgesic pump. The visual analogue scale (VAS) for pain and the number of analgesic pump boluses at 24 h and 48 h after operation, the occurrence of postoperative adverse drug reactions and the patient satisfaction at 1 month after operation were collected. The normally distributed measurement data were expressed as Mean±SD, and the comparison between the two groups was analyzed by t-test; the count data was expressed by the frequency (percentage), and the Fisher’s test was used for analysis. Results:A total of 56 patients were included, including 9 males and 47 females, ranging in age from 27 to 53 years old, with ASA graded from 1 to 2, body mass index (BMI) ranged from 26 to 33 kg/m 2. Their rectus abdominis separation ranged from 3 to 7 cm. Compared with the control group, the pain VAS score of the study group at 48 hours after the operation was lower (3.9±1.5 vs. 5.2±1.4, P<0.05), and the number of analgesic boluses was reduced (2.9±0.3 vs. 3.9±0.4, P<0.05), the incidence of adverse reactions such as nausea, vomiting decreased( P<0.05). The satisfaction rate 1 month after operation in the study group was significantly higher than that in the control group [100.0% (30/30) vs. 42.3% (11/26), P<0.05]. Conclusions:Continuous infusion of ropivacaine through anterior rectus intrathecal cannula can effectively relieve abdominal pain after total abdominoplasty, reduce adverse reactions caused by postoperative analgesics, improve patient comfort during hospitalization, and improve overall surgical satisfaction.

Objective:To evaluate the effect of continuous pump ropivacaine in rectus sheath in the postoperative analgesia after abdominoplasty.Methods:Patients with abdominoplasty who were admitted to the Plastic Surgery Hospital of the Chinese Academy of Medical Sciences from August 2020 to December 2021 were retrospectively analyzed. They were divided into 2 groups: the control group received patient-controlled intravenous analgesia (PCIA) after the operation, sufentanil 100 μg + normal saline diluted to 100 ml, the rate was 2 ml/h, lasted for 48 h, locking time 15 min, Bolus: 0.5 ml; the study group underwent postoperative rectus abdominis intrathecal intubation and continuously infused 0.262 5% ropivacaine saline mixture 100 ml, the rate was 2 ml/h, lasted for 48 h, the locking time is 15 min, Bolus: 0.5 ml. The method of catheter placement in the study group: a catheter was placed unilaterally in the anterior sheath of the rectus abdominis under direct vision, and an incision was made 2 cm below the umbilical plane. The other end is pierced from the umbilicus and connected to the analgesic pump. The visual analogue scale (VAS) for pain and the number of analgesic pump boluses at 24 h and 48 h after operation, the occurrence of postoperative adverse drug reactions and the patient satisfaction at 1 month after operation were collected. The normally distributed measurement data were expressed as Mean±SD, and the comparison between the two groups was analyzed by t-test; the count data was expressed by the frequency (percentage), and the Fisher’s test was used for analysis. Results:A total of 56 patients were included, including 9 males and 47 females, ranging in age from 27 to 53 years old, with ASA graded from 1 to 2, body mass index (BMI) ranged from 26 to 33 kg/m 2. Their rectus abdominis separation ranged from 3 to 7 cm. Compared with the control group, the pain VAS score of the study group at 48 hours after the operation was lower (3.9±1.5 vs. 5.2±1.4, P<0.05), and the number of analgesic boluses was reduced (2.9±0.3 vs. 3.9±0.4, P<0.05), the incidence of adverse reactions such as nausea, vomiting decreased( P<0.05). The satisfaction rate 1 month after operation in the study group was significantly higher than that in the control group [100.0% (30/30) vs. 42.3% (11/26), P<0.05]. Conclusions:Continuous infusion of ropivacaine through anterior rectus intrathecal cannula can effectively relieve abdominal pain after total abdominoplasty, reduce adverse reactions caused by postoperative analgesics, improve patient comfort during hospitalization, and improve overall surgical satisfaction.