Objective To analyze the clinical efficacy of left subclavian artery (LSA) revascularization combined with thoracic endovascular aortic repair (TEVAR) for Stanford type B aortic dissection with insufficient proximal landing zone. Methods A retrospective analysis was conducted on the clinical data of patients with Stanford type B aortic dissection and insufficient proximal landing zone who underwent TEVAR combined with LSA revascularization or TEVAR alone at the Central Hospital of Wuhan from 2017 to 2021. Patients were divided into a revascularization group and a simple stent group based on the surgical approach. Perioperative data of the two groups were compared. Results A total of 144 patients were included. In the simple stent group, there were 113 patients, including 85 males and 28 females, with a median age of 56.0 (48.0, 68.0) years. In the revascularization group, there were 31 patients, including 23 males and 8 females, with a median age of 54.0 (48.2, 59.7) years. There were statistical differences in operation time, hospital stay, preoperative lesion diameter, and preoperative and postoperative right vertebral artery diameter between the two groups (P<0.05). The simple stent group had 12 (10.6%) patients of complications, which was lower than the revascularization group (9 patients, 29.0%) postoperatively. At three months postoperatively, the most common complication in the simple stent group was endoleak (5 patients), while in the revascularization group it was hoarseness (2 patients). There was no death in the two groups within 1 year postoperatively. Conclusion Both different surgical approaches have good effects on the treatment of type B aortic dissection with insufficient proximal landing zone, but further validation is needed through multicenter, large-sample, and long-term follow-up studies.

Esophagogastric variceal bleeding is a common complication and the leading cause of death in advanced liver cirrhosis， and endoscopic variceal ligation （EVL） and endoscopic cyanoacrylate injection （ECI） are commonly used treatment strategies. Thrombocytopenia is one of the most common hematological complications in liver cirrhosis， and patients with severe thrombocytopenia have the potential risk of bleeding， which may affect treatment decision-making by clinicians and endoscopists. This article reviews the evolution of guidelines and clinical research advances regarding EVL/ECI in China and globally， in order to provide a basis for decision making among clinicians.

Objective:To carry out clinical and genetic analysis for a child featuring Brain-Lung-Thyroid syndrome (BLTS).Methods:A child who had presented at the Children′s Hospital Affiliated to Shandong University on May 27, 2022 was selected as the study subject. Clinical data was collected. Trio-whole exome sequencing (Trio-WES) was carried out for the child and his parents, and candidate variant was verified by Sanger sequencing and bioinformatic analysis. The child was given individualized treatment following the diagnosis.Results:The child, a two-year-and-seven-month-old boy, had presented with global developmental delay, ataxia and hypothyroidism. WES revealed that he has harbored a heterozygous c. 674C>T variant of the NKX2-1 gene, based on which he was diagnosed with BLTS. CT scan revealed interstitial and parenchymal inflammation in his lungs, which was reduced by budesonide aerosol inhalation. Conclusion:Discovery of the novel c. 674C>T variant has enriched the mutational spectrum of the NKX2-1 gene. Budesonide aerosol may be used to treat lung inflammation associated with BLTS.

OBJECTIVE Amyotrophic lateral sclerosis(ALS)is a fetal neurodegenerative disease characterized by the progressive loss of upper and lower motor neu-rons,leading to skeletal muscle atrophy,weakness,and paralysis.Oxidative stress plays a crucial role in ALS pathogenesis,including the familial forms of the disease arising from mutations in the gene coding for superox-ide dismutase(SOD1).Additionally,the abnormal accu-mulation of TAR DNA-binding protein of 43 ku(TDP-43)is a pathological feature present in almost all patients,even though the pathogenesis of ALS is unclear.Current-ly,there is no drug that can cure ALS/FTLD.Tetramethyl-pyrazine nitrone(TBN)is a derivative of tetramethylapyr-azine,derived from traditional Chinese medicine Ligusti-cum chuanxiong,which has been extensively proven to have therapeutic effects on various models of neurode-generative diseases.METHODS We investigated the therapeutic effect of TBN in the SOD1G93A and TDP-43M337V ALS mouse models.In the SOD1G93A trans-genic mouse model,TBN was administered to mice via intraperitoneal or intragastric injection after the onset of motor deficits.We injected the TDP-43M337V virus into the striatum of mice unilaterally and bilaterally,and then administered TBN 30 mg·kg-1 intragastrically to observe changes in behavior and survival rate of mice.RESULTS TBN slowed the progression of motor neuron disease,as evidenced by improved motor performance,reduced spi-nal motor neuron loss and associated glial response,and decreased skeletal muscle fiber denervation and fibrosis.TBN treatment activated mitochondrial antioxidant activity through the PGC-1α/Nrf2/HO-1 pathway and decreased the expression of human SOD1.In the mice with unilateral injection of TDP-43M337V into the striatum,TBN improved motor deficits and cognitive impairment in the early stages of disease progression.In mice with bilateral injection of TDP-43M337V into the striatum,TBN not only improved motor function but also prolonged survival.Moreover,we demonstrate that its therapeutic effect may be through activation of the Akt/mTOR/GSK-3β and AMPK/PGC-1α/Nrf2 signaling pathways.CONCLUSION TBN shows promise as an agent for the treatment of ALS/FTLD.TBN is currently undergoing clinical investigation for several indications,including a Phase Ⅱ trial for ALS.

Objective    To investigate the safety and feasibility of early mobilization in critically ill patients with femoral catheters, and to provide reference for guiding clinical rehabilitation training. Methods    The literature on the safety and feasibility of early mobilization in critically ill patients with femoral catheters included in PubMed, EMbase, OVID, Springer-link, Wiley Online Library, and Web of Science up to June 2021 was searched, and relevant data were extracted for analysis. Results    Seventy-two papers were initially screened, and 12 papers that met the criteria were finally included, covering 1 056 patients, and 489 patients had femoral catheters. Patients underwent 6 495 sessions of physical therapy, and a total of 62 patients had adverse events, including 14 (2.86%, 14/489) patients with catheter-related adverse events. Conclusion    Although early mobilization in critically ill patients with femoral catheters may lead to adverse catheter-related events, the incidence is low. Therefore, the associated risks and benefits should be weighed in clinical practice, and femoral catheter is not recommended as a contraindication for early mobilization in critically ill patients.

Objective Conduct routine microbiological monitoring of laboratory rats and mice from vendors to provide an important basis for the scientific management of laboratory animal facility and ensure the reliability of relevant experimental data obtained from laboratory animals.Methods Taking the Department of Laboratory Animal Science of Fudan University as an example, between April 2021 and April 2023, rats and mice purchased from 7 vendors were sampled for microbiological quality according to the principle of simple random sampling on the arrival days of animal delivery. Then, surveillance tests were conducted to examine the microbiological contaminations according to the national standards of SPF laboratory animals.Results The total qualified rate was 80.36%, with 52.63% in SD rat, 82.76% in inbred mice, 86.67% in outbred mice and 86.36% in immunodeficient mice in details. The most frequent bacteria isolated were Staphylococcus aureus, Pseudomonas aeruginosa, Klebsilla pneumoniae and Rodentibacter heylii, and their detection rates were 10.76%, 3.16%, 2.53% and 0.63%, respectively. Serological assays demonstrated the highest prevalence for virus was Sendai virus, and the detection rate was 2.53%. In addition to the pathogens those must be excluded from SPF rodents, Entamoeba muris and Enterobacter spp. were also detected in inbred mice, and Klebsiella oxytoca was detected in immunodeficient mice, with the detection rates of 1.15%, 2.30% and 4.55%, respectively.Conclusion There are certain incidences of pathogen infections in laboratory rats and mice from vendors, and an efficient microbiological monitoring of laboratory animals should be implemented in animal facilities, in order to eliminate pathogen infections in laboratory animals, which is required for improving the accuracy of research results and protecting the occupational health of laboratory animal practitioners as well.

Objective:To investigate the efficiency and safety of pneumatic retinopexy for rhegmatogenous retinal detachment (RRD).Methods:A prospective case series study. From January 2020 to December 2021, 27 eyes of 27 RRD patients in Joint Shantou International Eye Center were included. Among them, there were 10 males and 17 females; the mean age was 36.0±11.8 years old. There was recurrent retinal detachment in 4 eyes. Best corrected visual acuity (BCVA) and optical coherence tomography were performed. According to whether the retina was successfully reattached after a single intravitreal injection, the patients were divided into a single operation group and a second operation group. All eyes underwent pneumatic retinopexy, the patients received intravitreal injection of 0.3-0.5 ml 100% C 3F 8. On the next day, laser photocoagulation was performed around the retinal breaks. The mean follow-up time was 15.0±6.0 months. Outcome measures included single operation success rate, the BCVA at the last follow up and complications. The age, gender, extent of retinal detachment, number of retinal breaks, and preoperative and postoperative BCVA of patients in the single operation group and the second operation group were compared using Mann-Whiteney test or Fisher's exact test. Results:Twenty of the 27 eyes (74.1%, 20/27) had retinal reattachment after single operation, 7 of the 27 eyes (25.9%, 7/27) had retinal reattachment after the second operation. Among the 7 eyes, retinal detachment recurrence after operation, retinal detachment recurrence after tear opening, and retinal detachment recurrence with new tears occurred in 1, 4, and 2 eyes, respectively; retinas were all reattached after scleral buckling or vitrectomy. The BCVA was stable and improved in 14 and 13 eyes, respectively. Compared with the single operation group, the retinal detachment in the second operation group was wider and the BCVA before operation was worse, the differences were statistically significant ( Z=-2.842,-2.233; P<0.05). Transient intraocular pressure elevation occurred in 5 eyes; no complications such as infectious endophthalmitis, intraocular hemorrhage and lens damage occurred. Conclusion:Pneumatic retinopexy is safe and effective in the treatment of RRD.

Objective:To investigate the efficacy and safety of fecal microbiota transplantation (FMT) in the treatment of irritable bowel syndrome (IBS), and to explore the effects of FMT on the gut microbiota of IBS patients.Methods:From September 2016 to August 2017, at Guangzhou First People′s Hospital, 28 hospitalized IBS patients who underwent FMT treatment were enrolled. Before FMT, four and 12 weeks after FMT, all the IBS patients completed the irritable bowel syndrome quality of life scale (IBS-QOL), irritable bowel syndrome severity scoring system (IBS-SSS) and gastrointestinal symptom rating scale (GSRS). 16S rDNA sequencing was performed before FMT and four weeks after FMT. The effects of FMT on gut microbiota diversity and microbiota structure of IBS patients were analyzed respectively from the level of phylum, family and genus, and linear discriminant analysis effect size (LEfSe) was further used to screen the different bacteria. Paired t test and paired rank sum test were used for statistical analysis. Results:Twelve weeks after FMT, the scores of the six dimensions of IBS-QOL including dysthymia, behavioral disorder, auto imagery, health concerns, eating avoidance, and relationship expansion were all lower than those before FMT (43.750, 22.656 to 56.250 vs. 48.438, 32.031 to 60.938; 37.500, 18.750 to 56.250 vs. 46.429, 21.429 to 62.500; 31.250, 14.063 to 42.188 vs. 31.250, 18.750 to 50.000; 41.667, 27.083 to 56.250 vs. 50.000, 41.667 to 66.667; 54.167, 43.750 to 72.917 vs. 66.667, 58.333 to 83.333; 8.333, 0.000 to 33.333 vs. 16.667, 8.333 to 33.333, respectively), and the differences were statistically significant ( Z=-2.157, -3.429, -2.274, -3.197, -3.042 and -2.329, all P<0.05). Twelve weeks after FMT, the scores of the two dimensions of IBS-QOL including behavioral disorder and relationship expansion were both lower than those of four weeks after FMT (37.500, 18.750 to 56.250 vs. 39.286, 19.643 to 62.500 and 8.333, 0.000 to 33.333 vs. 16.670, 2.083 to 41.667, respectively), and the differences were statistically significant ( Z=-1.998 and -2.110, both P<0.05). Four and 12 weeks after FMT, the scores of IBS-SSS and GSRS were both lower than those before FMT ((190.32±106.51), (201.43±102.48) vs. (245.93±86.10) and 5.50, 4.00 to 9.00 and 5.50, 4.00 to 8.75 vs. 7.00, 6.00 to 9.75), and the differences were statistically significant ( t=4.402 and 3.848, Z=-3.081 and -3.609; all P<0.01). No serious adverse reactions occurred in the patients after FMT. At the phylum level, after FMT the abundance of Verrucomicrobia in the feces of IBS patients was richer than that before FMT (6.74% vs. 0.37%); at the family level, after FMT the abundance of Verrucomicrobiaceae in the feces of IBS patients was richer than that before FMT (6.74% vs. 0.37%); at the genus level, after FMT the abundance of Akkermansia was richer than that before FMT (6.74% vs. 0.37%); and the differences were statistically significant (all Z=-2.589, all P=0.010). The results of LEfSe method indicated that four weeks after FMT the abundance of Akkermansia in the gut microbiota of IBS patients was richer than that before FMT (6.74% vs. 0.37%), and the difference was statistically significant (linear discriminant analysis value=4.5, P=0.049). Conclusions:FMT is safe and effective in the treatment of IBS. The mechanism may be through upregulating the diversity of gut microbiota and changing the structure of gut microbiota of IBS patients.

Objective:To investigate the effects of Clostridium butyricum on colitis and intestinal microbiota in mice with or without antibiotic pretreatment. Methods:Thirty specific pathogen free BALB/c mice were randomly divided into the blank control group, dextran sulfate sodium (DSS) group, antibiotic + DSS group, Clostridium butyricum + DSS group and antibiotic+ Clostridium butyricum + DSS group, with 6 mice in each group. After the mice were pretreated with quadruple antibiotics (ampicillin 1 g/L, neomycin 1 g/L, metronidazole 1 g/L, and vancomycin 0.5 g/L) in normal drinking water for 30 d, the mice colitis model was induced with DSS. At the same time, the mice in Clostridium butyricum + DSS group and antibiotics+ Clostridium butyricum + DSS group were given 1×10 6colony-forming unit (CFU) Clostridium butyricum by gavage. The effect of Clostridium butyricum on mice with colitis was evaluated by disease activity index (DAI), colon length and histopathological score. The level of serum inflammatory factors was detected by enxyme linked immunosorbent assay, and the effect of Clostridium butyricum on gut microbita in mice was determined by fecal 16S rRNA sequencing. Results:The general condition of mice of the blank control group were good, and their DAI scores fluctuated around 0. Since the fourth day after DSS drinking water was given, the mice of the DSS group showed signs of colitis such as weight loss, unformed stools and bloody stools. On the fourth day after intervention, the DAI score of Clostridium butyricum + DSS group was lower than that of DSS group (0.000±0.000 vs. 0.444±0.111), and the difference was statistically significant ( t=4.000, P=0.016 1). On the tenth and twelfth day after the intervention, the DAI scores of antibiotic+ Clostridium butyricum + DSS group were both lower than those of antibiotic+ DSS group (0.000±0.000 vs. 1.111±0.222, 0.667±0.000 vs. 1.889±0.222), and the differences were statistically significant ( t=5.000 and 5.500, both P<0.05). The histopathological score of mice colon tissue of Clostridium butyricum + DSS group was lower than that of DSS group (2.50±1.73 vs. 5.50±1.00), and the histopathological score of mice colon tissue of antibiotic+ Clostridium butyricum+ DSS group was lower than that of antibiotic+ DSS group (1.25±0.96 vs. 5.00±0.82), and the differences were statistically significant ( t=3.000 and 5.960, both P<0.05). The serum level of interleukin (IL)-1β Clostridium butyricum+ DSS group was higher than that of blank control group ((4.464±0.075) ng/L vs. (3.907±0.080) ng/L), the serum levels of tumor necrosis factor-α, IL-6 and IL-1β of Clostridium butyricum+ DSS group and antibiotic+ Clostridium butyricum + DSS group were all lower than those of DSS group ((2.402±0.383) ng/L , (1.845±0.345) ng/L vs. (6.958±1.084) ng/L, (1.752±0.146) ng/L, (1.307±0.048) ng/L vs. (3.537±0.608) ng/L, (4.464±0.075) ng/L, (4.066±0.190) ng/L vs. (7.477±0.339) ng/L), and the differences were statistically significant ( t=5.005, 3.964, 4.495, 4.693, 6.294, 8.674 and 8.774 , all P<0.05). The results of 16S rRNA sequencing showed that there were a significantly large number of anti-inflammatory or short-chain fatty acid producing bacteria in the gut microbiota of mice intervened by Clostridium butyricum, among which the dominant bacteria genus in Clostridium butyricum + DSS group and antibiotic+ Colstridium butyicum+ DSS group were Mucispirillum (linear discriminant analysis (LDA)=3.667 log10, P=0.004) and Stenotrophomonas (LDA=2.778 log10, P=0.044). In the antibiotic+ Clostridium butyricum+ DSS group, the dominant bacteria genus were Peptococcus (LDA=2.685 log10, P=0.018), Butyricimonas (LDA=2.712 log10, P=0.011), Bilophila (LDA=3.204 log10, P=0.014), Intestinimonas (LDA=3.346 log10, P=0.010), Candidatus- Saccharimonas (LDA=3.363 log10, P=0.029), Desulfovibrio (LDA=3.402 log10, P=0.025), Oscillibacter (LDA=2.870 log10, P=0.019) and Akkermansia (LDA=4.031 log10, P=0.005). Conclusions:Clostridium butyricum can effectively improve colitis in mice and regulate the intestinal microbial structure of mice, whlie antibiotic pretreatment can strengthen its regulation of intestinal microbiota to and enhance the efficacy of Clostridium butyricum.

OBJECTIVE: To validate the diagnosis of an infant with elevated urine 3-methylglutaconic acid (3-MGA) through sequencing of the CLPB gene. METHODS: Genomic DNA of the infant was sequenced by next generation sequencing (NGS), and candidate pathogenic variants were verified by Sanger sequencing and bioinformatics analysis. RESULTS: NGS has revealed that the infant has carried a c.1085G>A (p.Arg362Gln) and a c.1700A>C (p.Tyr567Ser) of the CLPB gene, which were respectively inherited from her parents. Among these, c.1085G>A (p.Arg362Gln) is a novel variant which was unreported previously, and based on the ACMG guidelines, it was predicted to be a possible pathogenic variant. CONCLUSION Compound heterozygous variants c.1085G>A (p.Arg362Gln) and c.1700A>C (p.Tyr567Ser) of the CLPB gene probably underlay the disease in this infant. Genetic testing has confirmed the diagnosis.