Objective:To establish auto verification rules for the routine coagulation assays,and to provide reference for clinical laboratories to improve the quality and efficiency of results verification.Methods:A total of 24,510 specimens of sodium citrate anticoagulation routine coagulation test from the laboratory departments of eight hospitals including the First Medical Center,Chinese PLA General Hospital during January to March 2020 were collected and randomly divided into a rule establishment group and a rule verification group,with 6,670 specimens in the rule establishment group,including 2,056 Delta checks,and 17,840 specimens in the rule validation group,including 3,210 Delta checks.The activities of prothrombin time(PT),activated partial thromboplastin time(APTT),fibrinogen(Fib),thrombin time(TT),D-dimer(DD)and/or antithrombin(AT)were detected by Stago STA R Max automatic coagulation analyzer and supporting reagents.Taking the manual verification results as the standard,the auto verification and manual false negative rate(invalid verification),false positive rate(invalid interception),pass rate,positive coincidence rate,negative coincidence rate,verification consistency rate and specimen turnaround time(TAT)of the two groups were calculated.Results:The auto verification rules and the application process were preliminarily established,including internal quality control,alarm information,auto verification scope,critical value and deviation value inspection.In the rule establishment group,the single item pass rate was 82.6%-92.4%,and the overall pass rate was 73.8%.The consistency rate between auto verification and manual verification was 98.2%,and the positive coincidence rate and negative coincidence rate were 24.4%and 73.8%,respectively.In the rule verification group,the single item pass rate was 86.4%-91.5%,and the overall review pass rate was 71.5%.By simulating the application of auto verification rules,the average TAT of two hospitals among the eight hospitals was shortened by 1.5 hours and 2.1 hours,respectively.Conclusion:The application of auto verification rules can reduce workload of manual verification,and significantly shorten the TAT,and improve the report efficiency of the laboratory.

Objective To compare the approval status of orphan drugs in China and the United States(the U.S.)from 2018 to 2022,and to further understand the trends and reasons for the differences in the development of orphan drugs between the two countries.Methods Data on orphan drug approvals in China and the U.S.were retrieved from the National Medical Products Administration of China and the U.S.Food and Drug Administration(FDA),and the number,types,Marketing Authorisation Holder(MAH),target diseases,and new drugs marketed in China and the U.S.were classified and compared.Results From 2018 to 2022,426 approvals of orphan drugs in the U.S.and 63 approvals in China.Average annual growth rates of drug approval numbers in the U.S.and China were-4.81％and 8.38％,respectively.The most common types of orphan drugs approved in both countries were antineoplastic drugs and immunomodulators,of which 235(55.16％)were in the U.S.versus 15 types(23.81％)in China.Among the types of rare diseases targeted by these orphan drugs,the U.S.had the most rare tumor diseases(197 types,46.24％),while China had the most rare neurologrical and psychiatric diseases(22 types,34.92％).The U.S.marketed 36 first-in-class orphan drugs with breakthrough therapeutic value.And China marketed 40 innovative new drugs for the treatment of rare diseases.Conclusion The number of orphan drugs marketed in the U.S.has far surpassed that in China,but has shown a slight downward trend,while an increasing trend in China.The concentration of the domestic orphan drug industry in the U.S.is high,while China's orphan drugs still rely on imports.China has made substantial progress in making domestic innovative drugs for rare diseases,but there is a huge gap between China and the U.S.,especially in terms of advanced technology and innovative drugs.Therefore,intensify efforts should be made in the research and development(R & D)of new drugs for rare diseases in China,and actively participate in global synchronized research and development to achieve win-win cooperation.

Objective:To explore the treatment of thoracoabdominal combined injury in a child with heart rupture caused by sheep horns under limited medical conditions.Methods:To summarize the diagnosis and treatment of a child with thoracoabdominal combined injury and cardiac rupture who were treated with epidural anesthesia and having spontaneous breath, and even more there was no oxygen supply and perioperative monitoring during operation.Results:After the complete of thoracoabdominal exploration, the repair of heart, stomach and diaphragm, postoperative anti-infection, nutritional support and symptomatic treatment, the little patient recovered successfully.Conclusions:Thoracoabdominal injuries combined cardiac rupture requires rapid judgment of the patient’s condition, control of bleeding, discharge cardiac tamponade and cardiac repair as soon as possible. In order to save life, unconventional measures can be used when it is necessary.

Objective To study the medication law of Professor Qian Ying in the treatment of primary liver cancer based on data mining technology;To provide ideas for the clinical treatment of primary liver cancer.Methods Outpatient TCM prescriptions of Professor Qian Ying for the treatment of liver cancer from November 2008 to August 2020 were collected,and a data table was established after sorting.The drug frequency,property and taste and tropism were analyzed using Excel 2019.The medical case analysis module of the Great Physician Inheritance Platform was used to analyze the core drugs,the symbiosis analysis between drug pairs,the drug association analysis,and the drug clustering analysis of the screened TCM prescriptions.Results Totally 108 prescriptions were included,involving 188 kinds of Chinese materia medica,with a total frequency of 1 322 times.High-frequency drugs included Hedyotis Sinensis,Angelicae Sinensis Radix,Visci Herba,Curcumae Radix,Salviae Miltiorrhizae Radix et Rhizoma,etc.The medicinal properties were mainly cold,mild and warm,and the tastes were mainly bitter,sweet and pungent,and the main meridians were liver meridians,spleen meridians,kidney meridians and stomach meridians.There were 9 pairs of high frequency drug combinations in drug association,such as Curcumae Radix-Polygoni Orientalis Fructus,Visci Herba-Curcumae Rhizoma.In the correlation analysis of drug disease,the ones with higher correlations include"stomachache-Salviae Miltiorrhizae Radix et Rhizoma""abdominal mass-Paeoniae Radix Rubra and Citri Reticulatae Pericarpium""tinnitus-Adenophorae Radix,Lycii Fructus,Visci Herba""prolonged sublingual collaterals-Curcumae Rhizoma,Polygoni Orientalis Fructus,Salviae Miltiorrhizae Radix et Rhizoma"and so on.Drug clustering could be divided into three potential drug clusters.Conclusion Professor Qian Ying often uses heat-clearing drugs,tonifying drugs,and promoting qi and blood circulation drugs to treat liver cancer,with Huqi Powder as the main formula and modified according to the syndromes.Clearing heat and detoxifying,soothing liver and relieving depression,removing blood stasis and regulating collatrals are used to treat its symptoms,and tonifying qi and invigorating spleen,regulating liver and nourishing liver and kidney are used to treat its essence.

Objective:To explore the risk factors of bortezomib-related peripheral neuropathy (BIPN) and the clinical and electrophysiological characteristics of patients in treatment of multiple myeloma (MM).Methods:The clinical data of 71 newly diagnosed MM patients treated with BD (bortezomib + dexamethasone) regimen in Yancheng First People's Hospital from March 2016 to December 2019 were retrospectively analyzed. The bone marrow morphology, immunology, cytogenetics, molecular biology (MICM), routine electrophysiological examination before and after treatment were performed. All patients were divided into the peripheral neuropathy (PN) group and the non-PN group according to the presence or not of BIPN, and the clinicopathological differences of both groups were also compared; a binary logistic regression model was used to analyze the factors affecting the occurrence of PN. The electrophysiological characteristics were summarized and fluorescence in situ hybridization (FISH) was used to detect karyotype of BIPN patients.Results:Among 71 MM patients, there were 40 cases (56.3%) of PN and 31 cases (43.7%) of non-PN. The proportion of patients at international staging system (ISS) staging Ⅲ, and the levels of IgA, IgG, IgM, serum creatinine, β 2-microglobulin (β 2-MG) in the PN group were higher than those in the non-PN group, and hemoglobin (Hb) level in the PN group was lower than that in the non-PN group, and the differences were statistically significant (both P < 0.05). Binary logistic regression analysis showed that increased IgA ( OR = 1.151, 95% CI 1.012-1.309, P = 0.033), increased IgG ( OR = 1.055, 95% CI 1.000~1.112, P = 0.049), increased IgM ( OR = 1.010, 95% CI 1.001-1.018, P = 0.022), increased serum creatinine ( OR = 1.037, 95% CI 1.011~1.065, P = 0.005), increased β 2-MG ( OR = 1.564, 95% CI 1.039-2.354, P = 0.032) were risk factors for BIPN. Among 40 patients with BIPN, 33 cases (82.5%) of sensory nerve conduction velocity (SCV) were abnormal, 23 cases (57.5%) of motor nerve conduction velocity (MCV) were abnormal; 31 cases (77.5%) showed demyelination damage, 9 cases (22.5%) had axonal damage. Among 40 patients with BIPN, 24 cases underwent FISH detection, including 19 cases (79.2%) with chromosomal mutations, of which 12 cases (50.0%) were mixed subtype abnormal. Conclusions:MM patients with high levels of β 2-MG, IgA, IgG, IgM and serum creatinine are more prone to PN when treated with bortezomib. The electrophysiology of patients with BIPN is mainly characterized by demyelination of sensory nerves.

Sarcopenia is a serious health concern that must be payed attention by aged people.The occurrence of sarcopenia in obese older individuals may be intricately linked to lipotoxic substances such as free fatty acids(FFA),diacylglycerols(DAG),and ceramides,which are toxic to skeletal muscle cells.Lipotoxic substances in-duce inflammatory responses,oxidative stress,endoplasmic reticulum stress,and insulin resistance in skeletal mus-cle cells,disrupting protein metabolism and impairing regeneration and then lead to the onset of sarcopenia.Up to the present,the precise mechanisms by which lipotoxicity in skeletal muscle results in the development of sarcope-nia remain incompletely understood.Further research may highlight the orientation for the prevention and treatment of sarcopenia.

Objective:The investigation and research on the application status of Hepatic Venous Pressure Gradient (HVPG) is very important to understand the real situation and future development of this technology in China.Methods:This study comprehensively investigated the basic situation of HVPG technology in China, including hospital distribution, hospital level, annual number of cases, catheters used, average cost, indications and existing problems.Results:According to the survey, there were 70 hospitals in China carrying out HVPG technology in 2021, distributed in 28 provinces (autonomous regions and municipalities directly under the central Government). A total of 4 398 cases of HVPG were performed in all the surveyed hospitals in 2021, of which 2 291 cases (52.1%) were tested by HVPG alone. The average cost of HVPG detection was (5 617.2±2 079.4) yuan. 96.3% of the teams completed HVPG detection with balloon method, and most of the teams used thrombectomy balloon catheter (80.3%).Conclusion:Through this investigation, the status of domestic clinical application of HVPG has been clarified, and it has been confirmed that many domestic medical institutions have mastered this technology, but it still needs to continue to promote and popularize HVPG technology in the future.

Objective:To detect IgG and neutralizing antibodies response to SARS-CoV-2 vaccine by comparing enzyme-linked immunosorbent assay (ELISA), commercial magnetic particle chemiluminescence assay(CLIA) and neutralization test(NT).Methods:ELISA, CLIA and NT were used to detect 143 healthy people before and after 28 days immunization with 2 doses of SARS-CoV-2 vaccine, and calculate the positive conversion rate, quantitative results and analysis the consistency of the three methods.Results:The positive conversion rate of SARS-CoV-2 vaccine antibody detected by ELISA, CLIA and NT were respectively 97.9%, 98.6% and 85.3%. The geometric mean of the highest dilution of the serum quantitatively detected by ELISA was 586.6; The mean of CLIA S/CO value was 11.26; The geometric mean titer of the NT was 7.6. The correlation coefficient between ELISA, CLIA and NT were respectively 0.69( P<0.01) and 0.65( P<0.01), and the correlation coefficient between ELISA and CLIA was 0.79( P<0.01). Conclusions:The three methods all detected high levels of antibodies response to SARS-CoV-2 vaccine immunization. ELISA and CLIA are more consistent to detect IgG antibody, and have a good correlation with the quantitative detection results of the NT.

Objective To investigate the effect of occipitocervical fusion in patients with craniovertebral junction deformity combined with atlantoaxial instability. Methods The clinical data of 59 patients with craniovertebral junction deformity combined with atlantoaxial instability from March 2013 to June 2017 were retrospectively analyzed. The patients were treated with occipitocervical fusion. The atlantoaxial distance (ADI), distance between the odontoid and Chamberlain line and cervicomedullary angle (CMA) were used to evaluate atlantoaxial dislocation, vertical displacement and spinal cord compression, and the Japan Orthopedic Association (JOA) score was used to evaluate the effect before operation and 7 days after operation. Results The operation time was 109 to 214 (148.5 ± 20.1) min, the transoperative bleeding was 55 to 210 (122.0 ± 16.7) ml, and no injury of spinal cord nerve and vertebral artery occurred during operation. The CMA and JOA score 7 d after operation were significantly higher than that before operation: (153.8 ± 5.4)° vs. (131.2 ± 7.1) ° and (12.7 ± 1.8) scores vs. (10.6 ± 1.4) scores, and the ADI and distance between the odontoid and Chamberlain line 7 d after operation were significantly lower than that before operation: (2.9 ± 0.7) mm vs. (8.3 ± 2.7) mm and (3.2 ± 1.4) mm vs. (8.2 ± 2.3) mm, and there were statistical difference (P＜0.05). One case (1.7%, 1/59) died of massive brainstem infarction 1 d after operation; 2 patients (3.4%, 2/59) had undergone a revision operation due to fusion failure 3 months after operation; other 56 patients in reduction, internal fixation and bone graft fusion. The patients were followed up for 6 to 38 (15.9 ± 7.2) months, and the symptom relief was in 56 cases. The rate of symptom relief was 94.9% (56/59). The JOA score at last follow-up was significantly higher than that before operation: (13.3 ± 1.8) scores vs. (10.6 ± 1.4) scores, and there was statistical difference (P＜0.05). Conclusions The occipitocervical fusion can effectively relieve spinal cord compression and atlantoaxial instability in patients with craniovertebral junction deformity combined with atlantoaxial instability.

Objective To evaluate the effect of weight-bearing alignment training (WBAT) in correcting anterior pelvic tilt in children with cerebral palsy.Methods Twenty-seven children with cerebral palsy and anterior pelvic tilt were recruited and randonly assigned to a WBAT group,a strengthening group and a standing group,each of 9.In addition to routine medication and rehabilitation training,the 3 groups received WBAT,strengthening training or standing training respectively for 20 minutes a day,5 times a week for 4 weeks.Before and immediately after the treatment,the subjects' anterior superior iliac spine-posterior superior iliac spine angles (ASIS-PSIS angles) were assessed along with their anterior-posterior angles (A-P angles) and distances in a 1-minute walk test.Results The WBAT group showed significant improvement in their average ASIS-PSIS angle (to 18.61 ± 3.13°),A-P angle (to 23.31±3.81°) and the distance in the 1-minute walk test after the treatment.The standing group had significant progress in improving their A-P angles (to 24.48±4.33°),and the strengthening group had significant improvement in the distance walked in the 1-minute walk test.The average improvements in the ASIS-PSIS angle and walk distance in the WBAT group were significantly better than in the other 2 groups.Conclusion WBAT is superior to strengthening and standing training in improving the ASIS-PSIS angle,A-P angle and walking ability of children with cerebral palsy and anterior pelvic tilt.