Rapid and living guidelines are those developed in response to public health emergencies in a short period of time using a scientific and standardized approach. Subsequently， they provide timely and credible recommendations for decision makers through regular and frequent updates of clinical evidence and recommendations. In this paper， we introduced the definition of rapid and living guideline as well as analyzed the basic characteristics of eight rapid and living guidelines in the field of traditional Chinese medicine （TCM） published till 2023 June， summarizing three core methodological issues in relation to how to rapidly develop guidelines， how to formulate recommendations when there is lack of evidence， and how to ensure the timeliness of guidelines. Based on the analysis of current rapid and living guidelines， it is implicated that there is necessity to carry out rapid and living guideline in the field of TCM， and the methodology of rapid integration of multivariate evidence in the field of TCM needs to be further explored； furthermore， it is necessary to further explore the obstacles of implementation of guidelines and promote timely updating， all of which provide certain theoretical references for relevant guideline developers and researchers.

In developing rapid and living guidelines of traditional Chinese medicine （TCM） in response to public health emergencies， it is important that evidence continue to be reviewed， and clinical questions and recommendations updated if necessary， due to the rapid changes in disease progression and the continuous generation of relevant research evidence. This paper proposed that the updating scope in dynamic mode should first be identified； then evidence monitoring should be carried out in four aspects， including clinical research， related guidelines or laws and regulations， disease progression， as well as clinical use of recommendations and clinical needs； finally， based on the results of the evidence monitoring， different options should be made， including revising the clinical questions， updating the evidence and recommendations， and withdrawing the guideline.

The Ehlers-Danlos syndrome(EDS)is a rare inherent connective tissue disorder.The prev-alence of EDS in the population is estimated at one out of ten thousand to one out of a hundred thousand.The vascular EDS(vEDS)are rare among the subtypes but are the worst in prognosis.The article reports a case of vEDS admitted to the hospital.The patient was a young man complaining of a sudden onset of aphasia in right hemiparalysis and severe left abdominal pain for unknown reasons.The diagnosis was made after the genetic testing.The patient suffered from vEDS.Then,the multi-disciplinary team(MDT)made a treatment plan tailored to this young patient.The complexity in classification and delusive presentations of the EDS make the correct diagnosis very challenging.This article hopes to report this case and to share the experiences to the bet-ter understanding of this disease.

Objective To observe the value of ultrasound-guided intra-articular injection of hypertonic glucose(HG)for treating knee osteoarthritis in plateau area.Methods Ninety-one patients with knee osteoarthritis in plateau area(124 affected knee joints)were enrolled.The knee joints were divided into 20％HG group(n=67),25％HG group(n=42)or sodium hyaluronate group(n=15)based on the medication.Clinical and ultrasound scores were compared before and after treatment,and the efficacy of injection of HG was evaluated.Results At the 12th and 48th weeks after treatment,visual analog scale(VAS)scores decreased in all 3 groups(all P＜0.05),and Lysholm scores of 20％HG group and 25％HG group increased compared to those before treatment(all P＜0.05).The difference of Lysholm score before and in the 12th week after treatment,and of VAS score before and in the 48th week after treatment of 25％HG group were higher than those of 20％HG group(both P＜0.05).The joint exudation score of 20％HG group decreased in the 48th week after treatment compared to that before treatment(P＜0.05),and the synovial blood flow score decreased in the 12th and 48th weeks after treatment compared to those before treatment(both P＜0.05).The joint exudation score of 25％HG group decreased in the 48th week after treatment compared to that before treatment(P＜0.05),and the synovial hyperplasia and synovial blood flow score decreased in the 12th and 48th weeks after treatment compared to before treatment(all P＜0.05).The joint exudation score of sodium hyaluronate group decreased in the 48th week after treatment compared to that before treatment(P＜0.05),and the synovial hyperplasia score decreased in the 12th and 48th weeks after treatment compared to that before treatment(both P＜0.05).Conclusion Ultrasound-guided intra-articular injection of HG,esp.25％HG,had certain value for treating knee osteoarthritis in plateau area.

Objective:To explore the influencing factors of left ventricular thrombus (LVT) in patients with non-ischemic heart failure (NIHF) and to construct a nomogram prediction model for NIHF patients with LVT.Methods:This study was a case-control study. A total of 2 592 patients with NIHF hospitalized in Beijing Anzhen Hospital affiliated to Capital Medical University from January 2018 to July 2022 were selected. Fifty-one patients with LVT identified by echocardiography and cardiac magnetic resonance were classified into LVT group. One hundred and sixty patients were selected as the non-LVT group using a 1∶3 propensity score matching based on age and gender. Multivariate logistic regression analysis was used to explore the influencing factors of LVT in patients with NIHF. A nomogram prediction model was constructed, and the area under (AUC) the receiver operating characteristic (ROC) curve was calculated to evaluate the predictive effect of the model.Results:A total of 211 patients were enrolled, with a median age of 40 years old and 160 males (76%). Compared with non-LVT group, LVT group had lower systolic blood pressure ((112±20) mmHg vs. (120±19) mmHg; 1 mmHg=0.133 kPa), lower left ventricular ejection fraction (LVEF; (27±12)% vs. (39±14)% ), lower proportion of patients with history of hypertension (28% (14/51) vs. 44% (70/160)) and atrial fibrillation (8% (4/51)vs.39% (62/160)), higher proportion of patients with New York Heart Association functional class Ⅲ to Ⅳ (class Ⅲ: 59% (30/51) vs. 41% (66/160); class Ⅳ: 28% (14/51) vs. 19% (31/160)), and larger left ventricular end-systolic diameter (LVESD; (56±14) mm vs. (50±15) mm). The levels of hemoglobin ((152±23) g/L vs. (142±30) g/L), D-dimer (508 (300, 1 105) μg/L vs. 158 (68, 379) μg/L), and N-terminal pro-brain natriuretic peptide (3 429 (2 462, 4 734) ng/L vs. 1 288 (422, 2 544) ng/L) were higher in LVT group than in non-LVT group ( P all<0.05). LVT group had a higher proportion of patients using beta-blockers (92% (47/51) vs. 78% (124/160)), angiotensin-converting enzyme inhibitors or angiotensin receptor blockers or angiotensin receptor neprilysin inhibitors (88% (45/51) vs. 72% (115/160)), and anticoagulant drugs (98% (50/51) vs. 32% (51/160)) than non-LVT group (all P <0.05). Multivariate logistic regression showed that reduced LVEF ( OR=1.08, 95% CI 1.02-1.15, P=0.008), decreased LVESD ( OR=1.07, 95% CI 1.01-1.12, P=0.013), and increased D-dimer levels ( OR=5.40, 95% CI 1.98-14.74, P=0.001) were independent influencing factors for LVT in patients with NIHF. The ROC curve showed that the AUC of the nomogram for predicting LVT in patients with NIHF was 0.793 (95% CI 0.710-0.876, P<0.001). Conclusion:Reduced LVEF, decreased LVESD, and elevated D-dimer are associated with LVT in NIHF patients. The predictive model developed based on the above indicators has certain value in predicting LVT in NIHF patients.

Objective:To explore the efficacy and safety of ibrutinib for the treatment of newly treated and relapsed refractory (R/R) lymphoplasmacytic lymphoma (LPL) /Waldenstr?m macroglobulinemia (WM) .Methods:Retrospectively collected clinical data of 98 cases of newly treated and R/R LPL/WM patients who received ibrutinib treatment at the Hematology & Blood Diseases Hospital of the Chinese Academy of Medical Sciences from March 2016 to June 2023, and analyzed their efficacy and safety.Results:A total of 98 LPL/WM patients were included, which consisted of 45 newly treated patients and 53 R/R patients. Of these, 74 were males (75.5%) and the cohort had a median age of 64 (42-87) years. Eighty-eight patients were eligible for efficacy evaluation with a median treatment time of 20.8 (2.1-55.0) months, a major remission rate (MRR) of 78.4%, and an overall response rate (ORR) of 85.2%. The MRR and ORR of the newly treated patients were 78.4% and 86.5%, respectively, whereas the MRR and ORR of the R/R patients were 78.4% and 84.3%, respectively. There were no statistically significant differences in MRR and ORR between the initial treatment and R/R patients (all P values >0.05) . The median follow-up period was 29.1 (2.9-50.3) months and the median overall survival time for newly treated and R/R patients was not reached. The median progression-free survival time was 23.5 (95% CI 10.5-36.5) months and 45.0 (95% CI 34.0-56.0) months, respectively, with no statistically significant differences (all P values >0.05) . There were 25 deceased patients and no deaths were related to ibrutinib treatment. The main adverse reactions of ibrutinib were thrombocytopenia (5.1%) , pneumonia (8.1%) , and hyperuricemia (21.4%) . The incidence of atrial fibrillation was 2.0%. Conclusion:Ibrutinib exhibits good efficacy and safety for newly treated and R/R LPL/WM patients.

Humans ; Waldenstrom Macroglobulinemia/genetics* ; East Asian People ; Chromosome Aberrations ; Lymphoma

OBJECTIVE To establi sh the fingerprint of Cnidium monnieri and a method for the content determination of 4 kinds of coumarins. METHODS Ultra-high performance liquid chromatography （UPLC） method was adopted to establish the fingerprints of 21 batches of C. monnieri ； their similarities were evaluated with Similarity Evaluation System of TCM Chromatographic Fingerprint （2012 edition）；common peaks were identification by comparison with reference substance. Using 10 common peak areas as variables ，cluster analysis was performed for 21 batches of C. monnieri by the method of between groups. The relative correction factors of xanthotoxin ，bergapten and imperatorin were calculated by the same UPLC method with osthole as the internal reference. The contents of them were calculated by quantitative analysis of multi-components by single marker （QAMS），and compared with the results of external standard method. RESULTS Totally 10 common peaks were identified in the fingerprints of 21 batches of C. monnieri ；the similarities ranged from 0.997 to 1.000. Peak 4 was identified as xanthotoxin ，peak 8 as bergapten ，peak 9 as imperatorin and peak 10 as osthole. A total of 21 batches of samples were divided into 3 categories，of which S 7 was clustered into one category ，S14 was clustered into one category ，and the other 19 batches were clustered into one category. The relative deviations of the contents of xanthotoxin ，bergapten and imperatorin determined by QAMS and external standard method were in the range of 0.88％ -1.07％ ，2.22％ -2.29％ ，0.67％ -2.93％ ，respectively. CONCLUSIONS UPLC fingerprint of C. monnieri is successfully established ，and QAMS method for content determination of 4 coumarins is also established.

Objective:To evaluate the role of miR-20a-5p in M1 microglia aggravating oxygen-glucose deprivation and restoration (OGD/R)-induced injury to neurons and the relationship with mitofusin2 (MFN2).Methods:The well-growing BV2 microglia (M0 type) were polarized into M1 phenotype by lipopolysaccharide (100 ng/ml) and IFN-γ (20 ng/ml) and identified by quantitative real-time polymerase chain reaction and immunofluorescence.The well-growing N2a cells were divided into 6 groups ( n=6 each) by the random number table method: control group (group C), OGD/R group, M0 microglia co-culture group (group M0), M1 microglia co-culture group (group M1), miR-20a-5p inhibitor transfection group (group I) and negative control group (group NC). The cells were routinely cultured in group C, and the cells were subjected to OGD for 3 h followed by restoration of oxygen-glucose supply to develop the model of OGD/R injury in group OGD/R.The cells were subjected to OGD for 3 h and were co-cultured with M0 microglia for 24 h during restoration of oxygen-glucose supply in group M0.The cells were subjected to OGD for 3 h and were co-cultured with M1 microglia for 24 h during restoration of oxygen-glucose supply in group M1.In group I and group NC, cells were transfected with miR-20a-5p inhibitor and negative control miRNA into M1 microglia, respectively, and N2a cells were subjected to OGD for 3 h and co-cultured with M1 microglia for 24 h during restoration of oxygen-glucose supply.The cell viability was determined by cell counting kit-8 assay, amount of lactate dehydrogenase (LDH) released was determined, the expression of miR-20a-5p and MFN2 mRNA was detected by quantitative real-time polymerase chain reaction, and MFN2 expression was detected by Western blot. Results:Compared with group C, the cell viability was significantly decreased, the amount of LDH released was increased, and the expression of MFN2 protein and mRNA was down-regulated in the other five groups, miR-20a-5p expression was significantly up-regulated in OGD/R, M0 and M1 groups, and miR-20a-5p expression was significantly down-regulated in group I ( P<0.05). There were no significant differences in the cell viability, amount of LDH released, and expression of miR-20a-5p, MFN2 protein and mRNA between group OGD/R and group M0 ( P>0.05). Compared with group OGD/R and group M0, the cell viability was significantly decreased, the amount of LDH released was increased, and the expression of MFN2 protein and mRNA was down-regulated, and miR-20a-5p expression was up-regulated in group M1 ( P<0.05). Compared with group M1, the cell viability was significantly increased, the amount of LDH released was decreased, the expression of MFN2 protein and mRNA was up-regulated, and miR-20a-5p expression was down-regulated in group I ( P<0.05). Conclusions:The mechanism by which M1 microglia aggravates OGD/R-induced damage to N2a cells may be related to the up-regulation of miR-20a-5p expression in M1 microglia and the inhibition of MFN2 expression in N2a cells.

Objective To evaluate the clinical efficacy of endoscopic retrograde cholangiopancreatography (ERCP)-based comprehensive minimally invasive treatment for biliary anastomotic stenosis (BAS) after liver transplantation. Methods Clinical data of 60 BAS recipients after liver transplantation were retrospectively analyzed, 54 male and 6 female, aged (48±10) years. ERCP was initially carried out. If it succeeded, plastic or metallic stents were placed into the biliary tract. If it failed, percutaneous transhepatic cholangial drainage (PTCD) or single-operator cholangioscopy (SpyGlass) was adopted to pass through the stenosis. If all these procedures failed, magnetic anastomosis or other special methods were delivered. The incidence and treatment of BAS after liver transplantation were summarized. The efficacy, stent removal and recurrence were observed. Results The median time of incidence of BAS after liver transplantation was 8 (4, 13) months. Within postoperative 1 year, 1-2 years and over 2 years, 39, 16 and 5 recipients were diagnosed with BAS, respectively. All 60 BAS recipients after liver transplantation were successfully treated, including 56 cases initially receiving ERCP, and 41 completing BAS treatment, with a success rate of 73%. The failure of guide wire was the main cause of ERCP failure. The success rates of PTCD, SpyGlass and magnetic anastomosis were 5/9, 5/7 and 7/8, respectively. Two recipients were successfully treated by percutaneous choledochoscope-assisted blunt guide wire technique and stent placement in the biliary and duodenal fistula. After 3 (3, 4) cycles of ERCP and 13 (8, 18) months of stent indwelling, 38 recipients reached the stent removal criteria, including 25 plastic stents and 13 metallic stents. The indwelling time of plastic stents was longer than that of metallic stents (P < 0.05). Six cases suffered from stenosis recurrence at 12 (8, 33) months after stent removal, and the recurrence rate was 16%. Six patients were treated with ERCP, and 5 of them did not recur after the stents were successfully removed. Multivariate analysis showed that delayed diagnosis of stenosis and frequent ERCP before stent removal were the independent risk factors for BAS recurrence (both P < 0.05). Conclusions ERCP-based comprehensive minimally invasive treatment may improve the success rate of BAS treatment after liver transplantation and yield satisfactory long-term efficacy. Delayed diagnosis of BAS and high frequent ERCP required for stent removal are the independent risk factors for BAS recurrence.