The standardization of medical management is critical for facing the challenge of public health emergencies in primary medical and health institutions. During the COVID-19 epidemic a quality standard system for pharmacy management was developed in Shanghai Gubei Community Health Service Center, which included 4 standardized modules: medication management module, pharmacy administration modules, pharmacy service module and specialized disease management module. In this article the author discusses the construction of pharmacy quality management standards, its application and roles in COVID-19 epidemics, to provide reference for quality pharmacy management during public health emergencies in grass-roots medical and health institutions.

Objective:The phenotype and genotype of a pedigree with Glanzmann thrombasthenia caused by compound heterozygous mutation in the ITGA2B gene and its molecular pathogenesis were explored.Methods:The platelet aggregation rate of the proband and his family was detected by using a platelet aggregation test with adenosine diphosphate, collagen, epinephrine, arachidonic acid, and ristocetin. The expression levels of CD41 (αⅡb), CD61 (β3), and CD42b (GPⅠb) on the platelet surface was detected by flow cytometry. Gene sequencing technology was used for the genetic identification of the family. RT-PCR was used in the detection of mRNA splicing, and qRT-PCR was used in detecting the relative mRNA level of the ITGA2B gene. Bioinformatics analysis was used to evaluate the pathogenicity of mutation sites and their effects on protein structure and function. The expressions of total αⅡb and β3 in platelets were analyzed by Western blot.Results:Except ristocetin, the other four inducers could not induce platelet aggregation in the proband. Flow cytometry showed that the expression levels of αⅡb and β3 were only 0.25% and 9.76%, respectively, on the platelet surface of the proband, whereas GPⅠb expression was relatively normal. The expression levels of glycoproteins in the other family members were almost normal. c.480C>G and c.2929C>T mutations were detected in the proband through gene sequencing. The c.480C>G mutation was inherited from his mother, and the c.2929C>T mutation was inherited from his father. The RT-PCR and sequencing results showed that the c.480C>G mutation caused mRNA splicing in the proband and his mother, resulting in the deletion of 99 bases in c.476G-574A (p.S160-S192). qRT-PCR showed that the c.2929C>T variant reduced the mRNA level of the ITGA2B gene in the proband and his father. Bioinformatics analysis suggested that the c.480C>G mutation might form a binding sequence with hnRNP A1 protein and generate the 5′SS splice site. The three-dimensional structural model of the αⅡb subunit showed that the β-propeller domain of the p.S160-S192 deletion lost two β-strands and one α-helix in blade 2. The c.2929C>T nonsense mutation caused premature translation termination and produced a truncated protein with the deletion of p.R977-E1039, including the cytoplasmic domain, transmembrane domain, and a β chain of the extracellular Calf-2 domain. The total αⅡb expression of the proband was absent, and the relative expression of β3 was 11.36% of the normal level.Conclusion:The compound heterozygous mutation c.480C>G in exon 4 and c.2929C>T in exon 28 of the ITGA2B gene probably underlies Glanzmann thrombasthenia in this pedigree.

Objective To examine the influencing factors related to clinical efficacy and outcomes of adult primary immune thrombocytopenia (ITP).Methods The clinical data of 161 cases of ITP admitted in the Second Hospital of Shanxi Medical University from June 2013 to March 2017 were collected.The influencing factors related to clinical efficacy and prognosis of adult ITP patients were analyzed.Results There were 60 males and 101 females with a M/F ratio of 0.59∶1 and a median age of 45 years (18-84 years).There were 109 newly diagnosed ITP cases,14 persistent ITP cases and 38 chronic ITP cases in this series.Seventy nine patients received intravenous immunoglobulin g (IVIg) treatment and 82 patients received high dose-dexamethasone treatment.There were no significant differences in clinical efficacy [91.13％(72/79) vs.87.80％(72/82),x2=0.181,P=0.914] and relapse rate [36.11％(26/72) vs.30.55％(22/72),x2=0.189,P=0.910] between IVIg and high dose-dexamethosone groups.Multivariate regression analysis showed that bleeding score ≥2 was the independent risk factor for the lower clinical efficacy (RR=1.415,95％CI:1.008-1.986,P＜0.05).Patients were followed up for a median of 9.0 months (0.5-55.0 months),48 patients relapsed with a relapse rate of 33.33％ and a median relapse time of 1.8 months (0.5-24.0 months).Conclusions IVIg and high dose-dexamethasone have the similar clinical efficacy and relapse rate for treatment of adult ITP.The patients with the bleeding score ≥2 are more likely to get lower remission rate.

Objective To analyze the clinical features and risk factors for an emerging infection during the first induction chemotherapy in elderly patients with acute leukemia.Methods A retrospective analysis of clinical data of 79 elderly patients with newly diagnosed acute leukemia was performed in Second Hospital of Shanxi Medical University from January 2014 to May 2016.Results The 70 cases among 79 elderly patients with acute leukemia were suffered from infection with infection incidence rate of 88.6％ (70/79)during first induction chemotherapy.The infection-related fatality rate was 8.6 ％ (6/70).Being clear about sites of infection accounted for 90.0 ％ (63/70),and the top three infection sites were the lungs,gastrointestinal tract and the bloodstream.113 pathogenic strains were detected,including gram-negative bacilli accounting for 42.5 ％ (48/113),Gram-positive cocci for 30.1％ (34/113),fungi for 24.8％ (28/113),the virus for 2.7％ (3/113).Based on clinically and confirmatively diagnosis,the invasive fungal diseases mostly as Candida accounted for 30.4 ％ (24/79),mixed infections accounted for 34.3％ (24/70).Univariate analysis showed agranulocytosis and AML were risk factors for infection.Logistic multivariate regression analysis showed that agranulocytosis was a risk factor for infection (OR=12.010,95％CI:2.346-107.973,P=0.000).The infection does not affect a complete remission rate of acute leukemia (x2 =0.001,P=0.983).Conclusions For newly diagnosed elderly acute leukemia patients,an emerging infection during the first induction chemotherapy is characterized by a high incidence,high fungal infection rate,most common site in lung,Gram-negative bacteria as most common pathogen,and an increased infection rate by agranulocytosis.The infection does not affect the remission rate of acute leukemia.

Objective:To develop the intelligent management system for diabetes medication knowledge base with the help of community hospital information platform.Methods:Based on the China Type 2 Diabetes Prevention Guide (2013 edition),National Essential Drugs (2012 edition) and the relevant package inserts,the knowledge base of diabetes medication was established.Results:The information platform could make interception beforehand,warning in the process of medication and provide attentions for physicians after treatment to determine rational medication.Conclusion:The intelligent management system for diabetes medication knowledge base can promote physicians to firmly grasp the principles of drug treatment,simplify treatment regimen,select effective drugs with reasonable prices and provide individualized treatment.

Objective To construct pIRES2-ZsGreen1/FⅨ expression vector,using the pcDNA/ FⅨ plasmid containing FⅨ cDNA as template,and express in HEK-293 cells.Methods The total ORF of F Ⅸ gene was amplified from pcDNA/F Ⅸ plasmid,then the amplified fragment was clonded into the pIRES2-ZsGreen1 vector using the Infusion enzyme.The positive clones of eukaryotic expression vector of pIRES2-ZsGreen1/F Ⅸ were screened and expanded after transfection,then were constructed and confirmed by PCR and sequencing.Transient expression experiments were performed using HEK-293 cells transfected with the expression vectors and observed the expression of ZsGreenl protein by confocal laser microscope.The relative expression levels of F Ⅸ mRNA,protein and F Ⅸ activity (F Ⅸ ∶ C) were detected by real time PCR (RT-PCR),immunofluorescence microscopy,One-Stage method,respectively.Results The expression vector,pIRES2-ZsGreen1/F Ⅸ,was successfully constructed and expressed in HEK-293 cells.RT-PCR detected the expression of F Ⅸ mRNA in HEK-293 cells and the immunofluorescence microscopy showed F Ⅸ protein distributed in the surrounding of nucleus.F Ⅸ ∶ C of cell lysates and cell culture fluid transfected with the expression vectors were (92.03 ± 0.29)％ and (86.89 ± 8.78)％,respectively;while both F Ⅸ ∶ C of cell lysates and cell culture fluid transfected with or without the expression vectors were 0.Conclusion The experimental results showed the expression vector,pIRES2-ZsGreen1/F Ⅸ,was successfully constructed,which provided experiment basement for the follow study on the location,function and molecular pathology of hemophilia B.

Computational Biology ; Factor VII ; Factor VII Deficiency ; Genetic Testing ; Humans ; Pedigree

Objective To explore the relationship between T-cell acute lymphoblastic leukemia and the Notch signaling pathway.Methods Human T-cell acute lymphoblastic leukemia SupT1 cells were infected with the lentiviral vector made up specific Notch1-shRNA gene and nonspecific Notch1-shRNA gene.The inhibitive rate of SupT1 cells was detected by CCK-8.The rates of early apoptotic cells (Annexin V+/ 7-AAD-) and late apoptotic cells (Annexin V+/7-AAD+) were analyzed by flow cytometry and the expression levels of Notch1 receptor gene and downstream target genes were assessed by quantitative reverse transcription and polymerase chain reaction (QT-PCR).Results The cell inhibition rates of Notch1 interference group,control group and empty vector group at 96 h were 0.902±0.013,0,and 0.486±0.084,respectively,and it was increased obviously in Notch1 interference group (both P ＜ 0.05).The cell early apoptosis rates of the three groups were (15.27±0.31) ％,(5.57±0.25) ％,(5.80±0.20) ％,respectively.The cell early apoptosis rate of Notch1 interference group was increased obviously compared with the control group and empty vector group (both P ＜ 0.05).While the cell late apoptosis rates had no significant difference among the three groups (P ＞ 0.05).The mRNA expression levels of Notch1 receptor gene and its target genes (Hesl,c-myc,NF-κB) at 48 h,72 h and 96 h were higher than those in the control group and empty vector group (all P ＜ 0.05).Conclusions The specificity of Notch1-shRNA can effectively decrease the Notch1 mRNA expression,and reduce the expression level of downstream target genes.Notch1 cut can inhibit the proliferation of SupT1 cells,and promote the early apoptosis.

Adult ; Aged ; Autoimmune Diseases ; genetics ; Case-Control Studies ; Female ; Genetic Predisposition to Disease ; Genotype ; Humans ; Male ; Middle Aged ; Polymorphism, Single Nucleotide ; Receptors, IgG ; genetics ; Thrombocytopenia ; genetics ; Young Adult