Construction of performance management system for pharmacy department of tertiary public hospitals under new situation / 中国药房

OBJECTIVE To provide reference for improving the work efficiency of staff and promoting the discipline construction of pharmacy department. METHODS By analyzing the current situation of performance management in the pharmacy department of our hospital， the key successful factors were sorted out， strategic decoding was carried out and key performance indicators were extracted. The quarterly and annual performance appraisal forms were formulated for the departments of pharmacy warehouse， outpatient pharmacy， ward pharmacy， clinical pharmacy department， prescription examination center， laboratory and other departments； the performance management information platform was built. The work efficiency and output of each department were compared half a year before and after the implementation of the performance management plan. RESULTS After the implementation of the program， the average queuing time for drug collection in the outpatient department was shortened from 5 minutes to 3 minutes， the average number of dispensing infusion bags per hour in the pharmacy intravenous admixture services increased from 50 bags to 60 bags， and antibacterial use density of the hospital decreased from 42.7 DDD（defined daily doses） to 40.2 DDD. The number of academic papers published had increased from 8 to 10， and the satisfaction of clinical departments with ward pharmacies increased from 85% to 95%. CONCLUSIONS The performance management system has been successfully established in pharmacy department of our hospital， which can improve the enthusiasm of pharmacists， reflect the value of pharmaceutical care， and promote the discipline construction of pharmacy.

Effect of Qianyang Yuyin Granules on Mesenchymal Transdifferentiation of Renal Tubular Epithelial Cells Induced by TGF-β1 / 中国实验方剂学杂志

ObjectiveBy observing the effect of Qianyang Yuyin granules on the phenotype of renal tubule epithelial cells， the intervention of Qianyang Yuyin granule on renal interstitial fibrosis was investigated. MethodThe renal tubular epithelial cells （HK-2） were treated with different concentrations of transforming growth factor （TGF）-β1 （5， 10， 15， 20， 25 μg·L-1） for 24 hours， and cell morphology and growth state were observed with an inverted phase contrast microscope. The 20 μg·L-1 was selected as the most appropriate concentration of TGF-β1 according to Western blot results for subsequent experiments. HK-2 cells were divided into six groups： blank group， TGF-β1 group （concentration of 20 μg·L-1）， low， medium， and high dose Qianyang Yuyin granule groups （concentration of 0.5， 1， 2 g·L-1）， and valsartan group （1 × 10-5 mol·L-1）. The cell activity was measured by cell proliferation and cell counting kit-8 （CCK-8）. The cell migration ability was detected by scratch test. The Transwell method was used to detect the invasiveness of cells. Western blot was used to detect levels of fibronectin （FN）， E-cadherin， α-smooth muscle activator （α-SMA）， Vimentin， collagen type Ⅰ（Col Ⅰ）， collagen type Ⅳ（Col Ⅳ）， and other related proteins. ResultTGF-β1 stimulating epithelial-mesenchymal transition （EMT） in renal tubular epithelial cells was time- and concentration-dependent. Compared with the blank group， higher concentration in the TGF-β1 group indicates longer intervention time and more obvious long spindle change of cells， and the migration and invasion ability of the cells was significantly enhanced. The protein expression level of FN， α-SMA， Vimentin， Col Ⅰ， and Col Ⅳ increased significantly （P<0.05， P<0.01）， while the expression level of E-cadherin protein decreased （P<0.05）. Compared with the TGF-β1 group， Qianyang Yuyin granule groups could maintain normal cell morphology， and the migration and invasion ability of the cells was inhibited. The protein expression level of FN， α-SMA， Vimentin， Col Ⅰ， and Col Ⅳ decreased （P<0.05， P<0.01）， and the expression of E-cadherin protein was significantly restored （P<0.05）. ConclusionQianyang Yuyin granule can reverse TGF-β1-induced interstitial transformation of renal tubular epithelial cells by reducing the phenotypic expression of mesenchymal cells and increasing the phenotypic expression of epithelial cells.

Kinematics and Plantar Pressure Analysis of Varus Femur and Varus Tibia / 医用生物力学

Objective To compare the differences in kinematic parameters and plantar pressures for two types of knee varus with tibial and femoral origins in gait analysis, so as to provide biomechanical theoretical basis for different types of genu varus. Methods Twenty-six patients with unilateral knee osteoarthritis (KOA) varus genu were enrolled, with 13 from femoral and 13 from tibial sources. Using Noraxon MyoMotion three-dimensional (3D) motion capture system and Footscan plantar pressure test system, the gait of the subjects during natural walking was measured, the temporal and spatial parameters of the gait, the kinematics parameters of lower limb joints and plantar pressures were collected, to make comparative analysis between the two groups. Results The range of knee flexion and extension of tibial varus, the peak of hip abduction, the range of motion (ROM) of hip adduction and abduction and the peak of ankle pronation were larger than those of femoral lateral genu varus. The peak of knee flexion and hip adduction was smaller than that of femoral lateral genu varus. Compared with femoral varus, subjects with tibial varus had increased stress time and peak pressure on the plantar of the 4th and 5th metatarsals (P<0.05). In the 3rd metatarsal region, the impulse of healthy femoral limb was greater than that of healthy limb with tibial deformity. While in the medial calcaneal region, the impulse of healthy femoral limb was smaller (P<0.05). Conclusions There are some differences in kinematic parameters and plantar pressures between two different types of unilateral genu varus patients. The results of this study are helpful to understand the abnormal gait caused by genu varus, and provide reliable reference for postoperative rehabilitation and limb exercise for different types of genu varus.

Research progress in comorbid bipolar disorder and obsessive-compulsive disorder / 中国基层医药

Bipolar disorder and obsessive-compulsive disorder comorbidity is becoming more and more common and has aroused clinical physicians' increasing interest. This review summarizes the epidemiology, clinical characteristics, neurobiochemistry, and treatment of comorbid bipolar disorder and obsessive-compulsive disorder and discusses the neurobiochemical mechanism. Findings from this review will provide evidence for identifying the clinical symptoms of bipolar disorder and obsessive-compulsive disorder comorbidity and for treatment of the comorbidity.

Introduction and evaluation of multidisciplinary collaborative blood glucose management mode of the whole hospital with the participation of clinical pharmacists / 中国药房

OBJECTIVE To intr oduce multidisciplinary collaborative blood glucose management mode （hereinafter referred as to blood glucose management mode of the whole hospital ）of the whole hospital with the participation of clinical pharmacists ，and to evaluation it effects on the blood glucose management of perioperative diabetes patients. METHODS The process of blood glucose management mode of the whole hospital in Drum Tower Hospital Affiliated to Medical School of Nanjing University and the work content of clinical pharmacists were introduced. Three hundred patients with type 2 diabetes mellitus who underwent elective surgery were included and randomly divided into general consultation group （control group ）and hospital-wide glucose management group （intervention group ）. The effect of glucose management and indicators such as preoperative waiting time and total length of stay were compared between 2 groups. RESULTS In the blood glucose management team of the whole hospital ，the physicians，clinical pharmacists and nurses of blood glucose management in endocrinology department were the core members ，and the management process was divided into initial management ，daily management ，discharge management and follow-up. As the team secretary ，the clinical pharmacists were mainly responsible for daily summarizing and managing the blood glucose level and special conditions of patients ，regularly evaluating the management effect ，carrying out pharmaceutical ward rounds ，medical order review， pharmaceutical care ， timely assessing the blood glucose of patients and guiding the rational use of drugs. Compared with before intervention ， after 3 days ofintervention，the levels of fasting plasma glucose （FPG）and postprandial blood glucose （PBG） were decreased significantly in 2 groups（P＜0.05）；intervention group was significantly lower than control group （P＜0.05）. Compared with control group ， the fluctuation of blood glucose in intervention group was significantly reduced （P＜0.05），standard deviation of blood glucose had reached the targeted control level ， and the postprandial glucose excursion and the largest amplitude of glycemic excursion were close to the targeted control level ；the rate of bl ood glucose reaching the standard before operation and at discharge were significantly increased （P＜0.05）；the preoperative waiting time and hospitalization days were significantly shortened （P＜0.05）. CONCLUSIONS The multidisciplinary collaborative blood glucose management mode of the whole hospital with the participation of clinical pharmacists can control the blood glucose level of diabetic patients in the perioperative period more stably and effectively ，and has practical significance for the disease treatment and prognosis of patients.

Current status and development trend of coagulation factor Ⅷ activity / 中华检验医学杂志

The detection of coagulation factor Ⅷ activity plays an important role in the diagnosis, typing, efficacy monitoring and detection of inhibitor titer in hemophilia A, acquired hemophilia A and von Willebrand disease. However, due to the diversity of detecting systems, the difference of reagent composition, the existence of interfering substances and other influence factors, the detection of coagulation factor Ⅷ activity in the laboratories in China still needs to be improved.

HbA 1C variability increases the risk of decline in glomerular filtration rate in elderly patients with type 2 diabetes / 中华内分泌代谢杂志

Objective:To evaluate the association of HbA 1C level and variability with annual decline in glomerular filtration rate in elderly patients with type 2 diabetes. Methods:A total of 527 elderly type 2 diabetic patients with baseline estimated glomerular filtration rate(eGFR)≥60 mL·min -1·(1.73 m 2) -1 at the diabetes center of a tertiary hospital in Jiangsu province were included and followed up. The mean value and the variability of HbA 1C, including standard deviation(HbA 1C-SD), variation coefficient(HbA 1C-CV), and adjusted standard deviation(Adj-HbA 1C-SD) were calculated. According to the annual decreased rate of eGFR, the patients were divided into △eGFR>5% group and △eGFR≤5% group. Cox proportional risk regression model was used to evaluate the relationship between HbA 1C variability and the risk of decreased glomerular filtration rate. Results:With a mean follow-up time of 19 months, there were 176 patients whose △eGFR>5%. Compared with △eGFR≤5% group, the HbA 1C-mean and HbA 1C variability were significantly higher in △eGFR>5% group( P<0.05). Cox regression analysis showed that HbA 1C-mean, HbA 1C-SD, HbA 1C-CV, and Adj-HbA 1C-SD were significantly correlated with decreased glomerular filtration rate. After adjustment for age, gender, HbA 1C-mean, and other factors, only Adj-HbA 1C-SD was correlated with renal insufficiency [ HR=3.32(1.68-6.57)]. Conclusions:HbA 1C variability is independently associated with annual decline in glomerular filtration rate in elderly patients with type 2 diabetes. The Adj-HbA 1C-SD is the most sensitive indicator in predicting decreased glomerular filtration rate.

Executive function correlates with community integration after a traumatic brain injury / 中华物理医学与康复杂志

Objective:To explore the relationship between executive functioning and community integration after a traumatic brain injury and to identify the main factors influencing community integration.Methods:A cross-sectional study of 30 traumatic brain injury survivors was conducted recording their gender, age, years of education, days in coma, living status and mobility. Their executive functioning was assessed using the Disorders of Executive Function Questionnaire (DEX) and the Wisconsin Card Sorting Test (WCST). Their community integration was evaluated using the Community Integration Questionnaire (CIQ). The independent correlations of demographic characteristics, life status, mobility and executive functioning with CIQ score were quantified.Results:The average total CIQ score was negatively correlated with the average WCST-RP, DEX and WCST-RPE scores, but it was positively correlated with mobility. It was also significantly correlated with life status. DEX scores and WCST-RP scores were significant independent predictors of community integration. The average CIQ family integration score was negatively correlated with days spent in a coma and significantly correlated with living status. The average CIQ social integration score was positively correlated with mobility (and negatively correlated with DEX, WCST-RP and WCST-RPE score. WCST-RP score and mobility were significant independent predictors of CIQ social integration scores. The average CIQ productive activity scores correlated negatively with the DEX, WCST-RP and WCST-RPE scores, and with the DEX and WCST-RP executive function scores. They were significant independent predictors of CIQ productive activity scores.Conclusions:Executive functioning can predict community integration, especially its social integration dimension.

Value of autism screening checklists in the early identification of autism spectrum disorder / 中国当代儿科杂志

OBJECTIVE@#To evaluate the value of autism screening checklists in the early identification of autism spectrum disorder (ASD).@*METHODS@#A total of 2 571 children who attended the Children's Hospital of Chongqing Medical University and completed autism screening and diagnostic test were enrolled as subjects, among whom 2 074 were diagnosed with ASD, 261 were diagnosed with global developmental delay (GDD), 206 were diagnosed with developmental language disorder (DLD), and 30 had normal development. The sensitivity, specificity, and optimal threshold value of the Modified Checklist for Autism in Toddlers (M-CHAT) and the Autism Behavior Checklist (ABC) for the early identification of ASD were evaluated by the receiver operating characteristic (ROC) curve.@*RESULTS@#The M-CHAT had a high sensitivity of 88.3% but a low specificity of 36.0% for the identification of ASD. Its sensitivity decreased with age, and was maintained above 80% for children aged 16 to < 48 months. The ABC had a high specificity of 87.3% but a low sensitivity of 27.2%, with an optimal cut-off value of 47.5 based on the ROC curve analysis. The multivariate linear regression model based on a combination of the M-CHAT and ABC for screening of ASD showed a specificity of 85.8% and a sensitivity of 56.6%.@*CONCLUSIONS@#The M-CHAT has a high sensitivity and a low specificity in the identification of ASD, with a better effect in children aged 16 to < 48 months. The ABC has a high specificity and a low sensitivity. The multiple linear regression model method based on the combined M-CHAT and ABC to screen ASD appears to be effective.

Effect of Fangji Huangqitang on DBA/1 Mice Collagen Induced Arthritis and Synovial Angiogenesis / 中国实验方剂学杂志

Objective:To observe the effect of Fangji Huangqitang （FJHQT） on collagen induced arthritis （CIA） and synovial angiogenesis in DBA/1 mice. Method:DBA/1 mice were randomly divided into normal group， CIA group and FJHQT group. DBA/1 mice in CIA group and FJHQT group were immunized with bovine type Ⅱ collagen and complete Freund's adjuvant on the first day， and DBA/1 mice were immunized with bovine type Ⅱ collagen and incomplete Freund's adjuvant on the 21^st day to establish CIA model. On the day of the second immunization， the drug was given by gavage once a day for 28 days. On the 22^nd day， the arthritis score and other symptoms of CIA mice were observed. On the 49^th day， Hematoxylin eosin （HE） staining was carried out to observe the angiogenesis in the synovium of CIA mice， the expression of vascular endothelial cell marker platelet endothelial cell adhesion molecule-1 （CD31） and vascular endothelial growth factor （VEGF） in the synovium of CIA mice were detected. Immunofluorescence double staining was used to detect the mature and immature vessels in the synovium of CIA mice. And the microvascular growth of the rat thoracic aortic ring was induced by VEGF （20 μg·L^-1）. The effects of FJHQT （0.25， 0.5， 1 g·L^-1） at different concentrations were observed under microscope. Result:Compared with the normal group， the inflammation， joints， red and swelling of the inflammatory joints of the CIA group were significantly increased （<italic>P</italic><0.01）. The scores of clinical arthritis， the incidence rate， synovial inflammation and angiogenesis were significantly increased （<italic>P</italic><0.01）. The density of blood vessels， the positive expression of CD31 and VEGF， the number of immature vessels in synovial membrane were significantly increased （<italic>P</italic><0.01）. And compared with the CIA group， the inflammation， joint swelling， and malformation of the FJHQT group were significantly improved， the clinical arthritis score， incidence rate， synovial inflammation and angiogenesis were significantly reduced （<italic>P</italic><0.01）. The vascular density， the positive expression of CD31 and VEGF， and the number of immature blood vessels in synovial membrane were significantly increased （<italic>P</italic><0.01）. Compared with blank group， VEGF could significantly induce the growth of microvasculature in rat thoracic aortic ring （<italic>P</italic><0.01）. Compared with VEGF group， FJHQT（0.25， 0.5， 1 g·L^-1） could significantly inhibit the formation of microvasculature in rat thoracic aortic ring （<italic>P</italic><0.01）. Conclusion:FJHQT can effectively alleviate the clinical symptoms and condition of CIA mice， reduce the clinical arthritis score and incidence rate，and inhibit the synovial angiogenesis of CIA mice joints and VEGF induced microvascular formation in rat thoracic aortic rings.

Altered white matter microarchitecture in Parkinson's disease: a voxel-based meta-analysis of diffusion tensor imaging studies / 医学前沿

This study aimed to define the most consistent white matter microarchitecture pattern in Parkinson's disease (PD) reflected by fractional anisotropy (FA), addressing clinical profiles and methodology-related heterogeneity. Web-based publication databases were searched to conduct a meta-analysis of whole-brain diffusion tensor imaging studies comparing PD with healthy controls (HC) using the anisotropic effect size-signed differential mapping. A total of 808 patients with PD and 760 HC coming from 27 databases were finally included. Subgroup analyses were conducted considering heterogeneity with respect to medication status, disease stage, analysis methods, and the number of diffusion directions in acquisition. Compared with HC, patients with PD had decreased FA in the left middle cerebellar peduncle, corpus callosum (CC), left inferior fronto-occipital fasciculus, and right inferior longitudinal fasciculus. Most of the main results remained unchanged in subgroup meta-analyses of medicated patients, early stage patients, voxel-based analysis, and acquisition with 30 diffusion directions. The subgroup meta-analysis of medication-free patients showed FA decrease in the right olfactory cortex. The cerebellum and CC, associated with typical motor impairment, showed the most consistent FA decreases in PD. Medication status, analysis approaches, and the number of diffusion directions have an important impact on the findings, needing careful evaluation in future meta-analyses.

Advances in platelet transcriptomics / 中国输血杂志

As special non-nuclear blood cells, platelets play an important role in organism. Over the years, the mechanism of platelets, as well as the production, maturation, metabolism, apoptosis in the body, and the separation and preservation of platelets during in vitro blood transfusion have been continuously studied. There are many kinds of RNAs in platelets. Therefore, platelet RNAs have become an important target for the study of platelet genetic material. With the development of transcriptome, the study of platelet transcriptome has been further studied. This review presents the research methods of transcriptome and the cutting-edge technology in recent years, and discusses the research of platelet transcriptome.

Novel coronavirus disease with conjunctivitis and conjunctivitis as first symptom: Two cases report / 中华实验眼科杂志

As the frontline health care workers at the center of the novel coronavirus disease (COVID-19) outbreak, we have encountered many asymptomatic COVID-19 patients or patients with mild symptoms since December 2019.A number of COVID-19 cases with conjunctivitis or conjunctivitis as the first symptom have been observed in our clinical work.This paper reports the diagnosis and treatment of one COVID-19 patient with conjunctivitis as the first symptom and one COVID-19 patient with conjunctivitis.In case one conjunctivitis occurred at the third day after patient came in close contact with determined COVID-19 patient and visited an eye doctor, and the symptom of conjunctivitis following the topical administration of anti-viral eyedrops for 1 week, followed by COVID-19.Her 2019-nCoV RNA detection of nasopharynx swab was positive but that of conjunctival sac swab was a negative result.Case two had a positive epidemiological history and simultaneous onset of COVID-19 and conjunctivitis.She presented positive results of 2019-nCoV RNA detection in both nasopharynx and conjunctival sac swabs, and other lab results supported the diagnosis of COVID-19 but she had a normal CT findings of the chest.The ocular symptoms were disappeared after topical administration of anti-viral eyedrops for 1 week.

Clinical analysis of 93 cases of adult patients with acute lymphoblastic leukemia / 国际肿瘤学杂志

Objective:To analyze the survival and prognostic factors of adult acute lymphoblastic leukemia (ALL) with different consolidation regimens after complete remission by induction therapy.Methods:A total of 93 adult patients with ALL were enrolled from January 2012 to June 2019 in Peking University Shenzhen Hospital. All the patients achieved complete remission induced by VDLCP regimen, and were divided into the standard group, intensive group and transplantation group according to the consolidation treatment. Thirty-four patients in the standard group received an ALL-like chemotherapy regimen based on VDLCP or Hyper-CVAD consolidation for 4-6 courses. Twenty-nine patients in the intensive group received BFM90/95 consolidation treatment for 2 years. Thirty patients in the transplantation group received allogeneic hematopoietic stem cell transplantation (allo-HSCT) after 2-3 courses of consolidation with the original induction regimen. The median follow-up was 18 (3-96) months, and the main follow-up indicators were overall survival (OS) and disease free survival (DFS). Prognostic factors of adult ALL patients and treatment-related deaths in each group were analyzed.Results:The 3-year OS rates of the standard group, intensive group and transplantation group were 54.0% (95% CI: 35.3%-72.6%), 71.8% (95% CI: 41.0%-94.5%), 62.3% (95% CI: 43.6%-80.9%), with a statistically significant difference ( χ2=6.110, P=0.047). The 3-year DFS rates of the three groups were 31.4% (95% CI: 12.9%-49.8%), 72.1% (95% CI: 52.3%-91.9%), 65.7% (95% CI: 45.3%-86.1%), with a statistically significant difference ( χ2=13.831, P=0.001). There were no significant differences in OS and DFS between the intensive group and the transplantation group ( χ2=0.709, P=0.400; χ2=0.046, P=0.830). OS and DFS of the intensive group were better than those of the standard group ( χ2=5.346, P=0.021; χ2=10.326, P=0.010). Multivariate analysis suggested that bone marrow minimal residual disease (MRD) negative on day 14-21 of chemotherapy was an independent prognostic factor affecting adult ALL ( HR=0.114, 95% CI: 0.015-0.841, P=0.033). The 3-year OS rates of Ph + ALL patients who received and did not receive allo-HSCT were 53.5% (95% CI: 23.1%-83.8%), 52.4% (95% CI: 23.8%-81.0%), the 3-year DFS rates were 77.1% (95% CI: 54.2%-100.0%), 35.0% (95% CI: 4.8%-65.2%), and there were no significant differences between the two groups ( χ2=3.600, P=0.223; χ2=3.824, P=0.050). The treatment-related mortalities of the non-transplantation group (standard group + intensive group) and the transplantation group were 3.2% (2/63) and 20.0% (6/30), and the treatment-related mortality of the non-transplantation group was significantly lower than that of the transplantation group ( χ2=7.318, P=0.007). Conclusion:Adult ALL has a poor prognosis. The 3-year OS rate and 3-year DFS rate of BFM intensive consolidation therapy are better than those of standard consolidation therapy, achieving a similar effect to allo-HSCT, but treatment-related mortality does not increase significantly. Patients with bone marrow MRD negative on the day 14-21 of chemotherapy have the better OS and DFS.

Novel coronavirus disease with conjunctivitis and conjunctivitis as first symptom: Two cases report / 中华实验眼科杂志

As the frontline health care workers at the center of the novel coronavirus disease (COVIN-19) outbreak, we have found many asymptomatic COVIN-19 patients or patients with mild symptoms since December 2019. A number of COVIN-19 cases with conjunctivitis or conjunctivitis as the first symptom have been observed in our clinical work. This paper reports the diagnosis and treatment of one COVIN-19 patient with conjunctivitis as the first symptom and one COVIN-19 patient with conjunctivitis. Case one occurred conjunctivitis at the third day after closely contacted with determined COVID-19 patient and visited to eye doctor, and the symptom of conjunctivitis following the topical administration of anti-virual eyedrops for 1 week, followed by COVID-19. Her 2019-nCoV RNA detection of nasopharynx swab was positive but that of conjunctival sac swab was a negative result. Case two had a positive epidemiological history and simultaneous onset of COVID-19 and conjunctivitis.She presented positive results of 2019-nCoV RNA detection in both nasopharynx and conjunctival sac swabs, and other lab results supported the diagnosis of COVID-19 but she had a normal CT findings of the chest. The ocular symptoms were disappeared after topical administration of anti-virual eyedrops for 1 week.

Analysis of the Relationship between Cytogenetic Changes and Course Evolution of Patients with CML during TKI Treatment / 中国实验血液学杂志

OBJECTIVE@#To analyze the relationship between cytogenetic changes and the progression in the patients with chronic myeloid leukemia (CML) during the treatment with tyrosine kinase inhibitor (TKI).@*METHODS@#The chromosome G banding of 150 patients with CML treated in our hospital, was carried out to analyze the karyotype by the 24 h short-term culture or direct method of bone marrow cells, and the point mutation of the ABL kinase area was detected, the relationship between cytogenetic changes and the evolution of the disease course was analyzed.@*RESULTS@#The indirect fluorescence in situ hybridization showed that the BCR-ABL fusion gene of 150 patients was positive, out of which 142 cases showed positive Philadelphia (Ph) (94.67%), 8 cases with Ph negative (5.33%). Among 142 cases with Ph positive on the first diagnosis, and 14 cases (9.86%) with additional chromosome abnormality (9.86%), 4 cases (2.82%) with mutation translocation with 124 cases (87.32%), standard translocation t (9; 22) (q34; Q11) were found. Out of the 14 patients with additional chromosomal abnormalities, 8 cases with "main pathmay" abnormalities, 2 case with -Y abnormalities, and 4 cases with "secondary pathway" abnormalities were observed. During TKI treatment, additional chromosomal abnormalities were found in 46 patients with standard translocation and abnormal number of chromosomes, and the incidence of disease progression and point mutation were higher (P＜0.05). Compared with patients with the standard translocation, the disease-free survival rate of the patients diagnosed as CML at 1st visit and with additional chromosome abnormality was significantly decreased (P＜0.05), but the overall survival rate showed no significantly different (P＞0.05). Compared with patients without additional cvtogenetic aberrations, the disease free and overall survival rate of the patients with additional cytogenetic aberrations during the TKI treatment of CML in chronic phase were significantly decreased (P＜0.05).@*CONCLUSION@#Some CML patients may have additional chromosomal abnormalities during the onset and development of the disease, and these patients are at higher risk of disease progression.

Analysis of a pedigree affected with congenital dysfibrinogenemia due to heterozygous Gln195Arg mutation of fibrinogen γ chain gene / 中华检验医学杂志

Objective:To analyze the phenotype and genotype of a Chinese pedigree with congenital dysfibrinogenemia and investigate the molecular mechanism of the disease.Methods:Pedigree analysis. Peripheral blood samples were collected from 7 members of the pedigree and routine coagulation tests were conducted. The activity of fibrinogen was measured using Clauss method, and fibrinogen antigen was measured by immunoturbidimetry. All the exons and exon-intron boundaries of FGA, FGB and FGG genes were amplified using PCR, which was followed by direct sequencing. Electrophoretic and immunological analysis of fibrinogen, fibrinogen clottability measurement, fibrin polymerization measurement and scanning electron microscopy were used to investigate the pathogenesis of this disease. Results:The proband showed normal activated partial thromboplastin time (APTT) , prolonged prothrombin time(PT), thrombin time (TT),and reptilase time (RT).The antigen level of fibrinogen in the proband (1.6 g/L) decreased slightly, while the activity level of fibrinogen (0.7 g/L) decreased significantly. His father and grandmother showed normal APTT and PT, prolonged TT and RT. The antigen levels of fibrinogen in both of them were normal (2.0 g/L and 2.2 g/L, respectively), while the activity levels of fibrinogen were low (1.0 g/L and 1.1 g/L, respectively). The results of other members from the pedigree were all within the normal range. Genetic analysis revealed a heterozygous A>G mutation at nucleotide 4774 in exon 6 of FGG gene in the proband, which was predicated to be a novel Gln195Arg mutation. The mutation was also found in his father and grandmother.Western blot results showed that no abnormal bands of plasma fibrinogen were found in the proband, his father and grandmother. The fibrinogen clottability in the proband was 49.3%, while that in the heathy control was 98.9%. Both thrombin-induced fibrin polymerization and reptilase-induced fibrin polymerization were significantly impaired in the proband, compared to that in the heathy control. Scanning electron microscopy revealed that compared with the heathy control, the average fiber diameters of the fibrin clot in the proband increased significantly ( P<0.001), while the density of fibers decreased and the arrangement of fibers was sparse. Conclusions:The heterozygous Arg19Gly mutation, which probably damages functions of fibrinogen, should be responsible for the congenital dysfibrinogenemia in this pedigree. This mutation has not been reported.

Clinical application evaluation of chromogenic substrate assay in detecting coagulation factor Ⅷ activity / 中华检验医学杂志

Objective:To establish clinical detection of coagulation factor Ⅷ activity by chromogenic substrate assay and evaluate its clinical application.Methods:A total of 40 hemophilia Apatients, 20 acquired hemophilia A patients, 26 patients with positive lupus anticoagulant and 60 apparently healthy people were enrolled from January 2018 to May 2019 in Ruijin Hospital of Medical College, Shanghai Jiaotong University. According to Clinical and Laboratory Standards Institute(CLSI), the accuracy, within-run and between-run imprecision, lower detection limit, linear range, carryover rate, reference range, and reportable range of chromogenic substrate assay for detecting coagulation factor Ⅷ activity was evaluated, and compared with coagulation assay. The clinical application was evaluated by detecting F Ⅷ activity in acquired hemophilia A patients and patients with lupus anticoagulant by chromogenic substrate assay.Results:The results of two constant quality control products were within range provided by the manufacturer(the bias was 3.93%-6.79%). The within-run imprecision was 1.86%-2.06%(≤5%). The between-run imprecision was 4.83%-6.90%(≤15%). The chromogenic substrate assay had a good performance in sensitivity(CV=11.23%<20%). The recommended reference range was appropriate for our laboratory. The maximum dilution was 1∶16. The linear range was 5.00%-193.50% (a=1.0243, R 2=1.000). The clinical reportable range was 0.50%-387.00%. The method had a low carryover rate (0.04%). The chromogenic substrate assay had good consistency with coagulation assay in detecting coagulation factor Ⅷactivity (R 2=0.961) as well as the titer of FⅧ inhibitor(R 2=0.973).The difference of FⅧ activity in patients with lupus anticoagulant between these two assays was statistically significant(t=9.232,P<0.05). Conclusion:The chromogenic substrate assay has a good performance in clinical detection of coagulation factor Ⅷactivity with wider clinical application and less interference.

The diagnostic value of flow cytometry in patients with lymphoma associated hemophagocytic syndrome / 中华内科杂志

Objective:To evaluate the reliability of flow cytometry (FCM) for diagnosing lymphoma associated hemophagocytic syndrome (LAHS).Method:The clinical data in 57 patients with hemophagocytic lymphohistiocytosis (HLH)were retrospective analyzed at Peking University Shenzhen Hospital from July 2010 to July 2019. All patients were performed bone marrow FCM and bone marrow pathological examination before final diagnoses were made. The golden diagnosis criterion was based on clinical, biochemical and histopathological evidence, which was regarded as the standard to evaluate the sensitivity and specificity of FCM analysis in diagnosing LAHS.Results:Among 57 cases, 36 cases were eventually diagnosed with LAHS, including 15 B-cell lymphoma(14 diffuse large B-cell lymphoma, 1 B-cell lymphoma with reactive T-cell hyperplasia), 13 aggressive NK/T cell lymphoma/leukemia, 2 cases of gamma-delta T-cell lymphoma, 4 angioimmunoblastic T-cell lymphoma, 1 enteropathy-associated peripheral T-cell lymphoma and 1 anaplastic T-cell lymphoma. Lymphoma cells in bone marrow were detected in all patients by FCM except one ENTCL patient. The sensitivity and the specificity of FCM in LASH compared to bone marrow biopsy were 97.2%( P=0.014)and 90.5%( P=0.488) respectively. In the other 21 non-LAHS patients, T cell receptor Vβ (TCRVβ) rearrangement was detected in 2 patients with Epstein-Barr virus (EBV) associated primary HLH. Conclusions:FCM effectively detects lymphoma cells in bone marrow of lymphoma patients with LHL, suggesting that FCM could be an important indicator for the diagnosis of LAHS. FCM also has the advantage in differentiating LAHS from other HLH.

Effects of PRECEDE-PROCEED Model on Behavior of Patients after Total Hip Arthroplasty / 中国康复理论与实践

Objective:To explore the effect of PRECEDE-PROCEED model on health behavior and quality of life of patients after total hip arthroplasty. Methods:From February to December, 2018, 103 patients who underwent primary total hip arthroplasty were divided into control group (n = 51) and intervention group (n = 52). The control group received routine perioperative guidance, while the intervention group received PRECEDE-PROCEED model in addition, in accordance to the nine links of the model, multi-dimensional intervention measures should be formulated with tendency factors, contributing factors and strengthening factors as the core. They were followed up for six months after discharge, and were assessed with Health Promoting Life style Profile II (HPLP II) and the MOS Item Short From Health Survey (SF-36) one, three and six months after discharge, respectively. Results:There was no significant difference in the scores of HPLP II and SF-36 between two groups at discharge (P > 0.05). The scores of HPLP II and SF-36 were higher than in the intervention group than in the control group one, three and six months after discharge (t > 2.307, P < 0.05). Conclusion:PRECEDE- PROCEED model could change the undesirable life behavior after operation, and improve the health behavior and quality of life of patients with total hip arthroplasty.