Objective:To explore the influence factors of neurodevelopmental disorders in children with SCN8A-related early-onset epilepsy through analyzing their clinical characteristics and following up their neurodeve-lopmental status. Methods:A retrospective analysis was carried out on 21 children (13 males and 8 females, the age ranged from 4 months to 8 years, average 31.6 months)with SCN8A-related early-onset epilepsy treated in Guangzhou Women and Children′s Medical Center and Kunming Children′s Hospital between January 2017 and February 2021.All patients underwent whole-exome sequencing and Sanger sequencing.The pathogenicity was estimated according to the American College of Medical Genetics and Genomics guidelines.The clinical data of all patients were also collected, including the age of onset of the disease, forms of seizures, seizure frequency, neurological development at onset, electroencephalogram (EEG) and brain magnetic resonance imaging (MRI). Besides, the patients were followed up to acquire the effect of sodium channel blockers after the onset of seizures, the process or improvement of neurodeve-lopment, EEG evaluation and neurodevelopmental outcomes.Patients were grouped based on data analysis results.The Fisher′s exact test was conducted to measure the effect of various factors on the neurodevelopmental process and outcome, and corresponding coe-fficients were calculated. Results:The average onset age of 21 patients was 0-9 months.The follow-up duration was 4 months-8 years.Three cases died.Sixteen cases (76.2%) had early infantile epileptic encephalopathy (EIEE), 5 cases (23.8%) had epilepsy without encephalopathy, and 1 case had benign infantile epilepsy.Fourteen cases (66.7%) belonged to drug resistant epilepsy.Only one child showed normal neurodevelopment.Eleven children showed delayed neurodevelopment, but improvement was observed.Nine children were retrogressed and stagnated in terms of neurodevelopment.Small age at onset ( Fisher=9.517, P=0.020, r=0.571), high seizure frequency ( Fisher=10.512, P=0.003, r=0.572), EEG background ( Fisher=10.512, P=0.003, r=0.572), epileptic discharges ( Fisher=8.288, P=0.008, r=0.542), and EEG changes before and after treatment ( Fisher=10.437, P=0.009, r=0.586) were important factors affecting the neurodevelopmental process.Neurodevelopmental outcome was normal in only 1 case, 1 child belonged to mild mental retardation (MR), 7 children belonged to moderate MR, 3 children belonged to severe MR, and 9 children belonged to profound MR.Statistical analysis indicated that the clinical phenotype ( Fisher=10.059, P=0.004, r=0.739) and drug resistance ( Fisher=13.706, P=0.001, r=0.640) were significantly correlated with neurodevelopmental outcomes.However, the forms of seizures, EEG findings at onset and mutation sites were not related to neurodevelopmental disorders. Conclusions:Most children with SCN8A-related early-onset epilepsy are accompanied with neurodevelopmental retardation of varying degrees.Epileptic encephalopathy and poor response to drug treatment will lead to severe neurodevelopmental disorders.

Objective:To examine the survival and influential factors of an integrated approach of novel agents, autologous hematopoietic stem cell (auto-HSCT) , and maintenance therapy in patients with multiple myeloma (MM) patients from a single center over the past 15 years.Methods:In our center, 300 MM patients who received an integrated strategy of new agents, auto-HSCT, and maintenance therapy over 15 years were retrospectively and prospectively analyzed.Results:The complete remission rates (CR) and ≥very good partial remission rates (VGPR) following induction therapy, transplantation, and maintenance therapy were respectively 35.3% and 55.2% , 72.4% and 80.0% , 89.2% , and 93.4% . When compared to patients receiving double-drug induction, the ≥VGPR and ORR of patients receiving triple-drug induction were improved. No difference existed in CR, ≥VGPR, and ORR between the PAD (bortezomib + liposome doxorubicin+ dexamethasone) and RAD (lenalidomide + liposome doxorubicin + dexamethasone) regimens, but the benefits speed differed. The negative rate of flow minimal residual disease following induction, transplantation, and maintenance was 18.8% (54 cases) , 41.4% (109 cases) , and 58.7% (142 cases) , respectively. The median time to progress (TTP) was 78.7 months and the median overall survival (OS) was 109 months. The median TTP for RISS-Ⅰ-Ⅲ patients were 111.8 months, 77.4 months, and 30.6 months, and the median OS was 118.8 months, 91.4 months, and 48.5 months, respectively. At various points during treatment, the TTP and OS of patients obtaining CR and MRD negative were longer than those of patients who did not obtain CR and MRD negative. TTP was noticeably shorter in high-risk cytogenetic patients compared to standard-risk patients even when CR was acquired during induction. There was no difference in TTP between patients with high-risk cytogenetics and those with standard-risk cytogenetics if MRD negative was acquired during induction. According to a multivariate analysis, the R-ISS stage was a poor predictor of TTP and OS at various treatment intervals. Therapeutic effectiveness was a newly independent prognostic factor following treatment.Conclusion:A median survival of almost 10 years is possible for MM patients who receive an integrated strategy of induction regimens followed by auto-HSCT and maintenance therapy, which significantly improves prognosis. However, this approach did not significantly benefit high-risk cytogenetic MM patients.

Objective:To explore the construction and effect of biobank in biological research in nursing field.Methods:Through the establishment of a biological research plan for nursing in Affiliated Jinhua Hospital, Zhejiang University School of Medicine, the biobank in biological research for nursing was constructed from aspects of biological samples, instruments and equipment, operating software, biobanking team formation, personnel training and standardized process formulation.Results:In August 2020, the Nursing Department and Central Laboratory of Affiliated Jinhua Hospital, Zhejiang University School of Medicine established a biobank for biological research in the nursing field, formed a biobank team, conducted personnel training and formulated standardized procedures. From December 1, 2020 to June 30, 2021, biological samples were collected from 264 gastric cancer patients and 56 healthy controls, mainly including whole blood, serum and urine.Conclusions:The construction of biobank for biological research in nursing field can collect abundant biological samples, which lays a foundation for subsequent independent biological research in nursing field.

Objective: To study the efficacy, safety and long-term outcomes of integrated strategy of bortezomib-based induction regimens followed by autologous hematopoietic stem cell (ASCT) and maintenance therapy in Chinese multiple myeloma (MM) patients. Methods: 200 MM patients receiving integrated strategy of bortezomib--based induction regimens followed by ASCT and maintenance therapy were retrospectively and prospectively analyzed from December 1. 2006 to April 30. 2018. Results: The complete remission rates (CR) and better than very good partial remission rates (VGPR) after induction therapy, transplantation and maintenance therapy were respectively 31% and 75.5%, 51.8% and 87.7%,73.6% and 93.4%. There was no difference between 4 cycles and more than 5 cycles induction chemotherapy. The negative rate of MRD detection by flow cytometry was 17.6% and 38.2% respectively after induction and 3 months after transplantation. The negative rate of MRD gradually increased during the maintenance therapy. The success rate of high dose CTX combined with G-CSF mobilization was 95.5% and transplantation related mortality (TRM) was zero. The median time to progress (TTP) was 75.3 months and the median overall survival (OS) was 99.5 months. TTP of patients obtaining CR and negative MRD after induction were longer that those of no CR and positive MRD. TTP and OS of patients receiving triple-drug induction and ASCT in early stage were longer than those of double-drug induction and ASCT in late stage. LDH≥240 U/L, high risk cytogenetics, ISS II+III stage and HBsAg positive were prognostic factors at diagnosis. However, only MRD and high risk cytogenetics were independent prognostic factors after transplantation and maintenance therapy. The clinical characteristics of patients of TTP ≥6 years were listed below: light-chain type M protein, ISS I stage, normal level of hemoglobin and platelet, normal LDH, HBsAg negative, chromosome 17p-negative, good response and sustained good response. Conclusions Integrated strategy of bortezomib-based induction regimens followed by ASCT and maintenance therapy can significantly improve the short-term and long-term efficacy. The prognostic factors of TTP in different disease stages were different. Response to treatment, especially MRD, played a more important role in prognostic factors.

Objective? To explore the effect of cardiovascular family-care APP on self-management behavior of patients with coronary heart disease. Methods? The hospitalized patients with coronary heart disease who were admitted in the department of cardiovascular medicine of a Class Ⅲ Grade A hospital from May 2016 to May 2018 were recruited by non-probability sampling method. All the research subjects were randomly divided into intervention group and control group by random number table. The intervention group consisted of 143 cases and control group consisted of 147 cases. The intervention lasted 12 months. The intervention group received health management of cardiovascular family-care APP, while the control group received routine discharged-guidance and outpatient clinic follow-up. Self-management behavior and risk factors of coronary heart disease were evaluated at baseline and 6 months and 12 months after intervention. Results? At 6 months after intervention, the total score of self-management behavior, medical management and emotional management in the intervention group were significantly higher than those in the control group, with statistical significance (P＜0.05). At 12 months after intervention, the total score of self-management behavior and medical management in intervention group were significantly higher than those in control group, with statistical significance (P＜0.05). After 6 months of intervention, BMI, alcohol consumption, blood pressure control of the intervention group were better than those of the control group, with statistical significance (P＜0.05). After 12 months of intervention, the control of blood pressure in the intervention group was better than that in the control group (P＜ 0.05). Conclusions? Cardiovascular family-care APP can promote the establishment of good self-management behavior in patients with coronary heart disease. Self assessment on smoking, drinking, diet, exercise, stress, anxiety and depression can be increased in app,and relevant research can be carried out

Objective To analyze the epidemiological and clinical characteristics of 403 local cases with dengue fever , and to provide guidance for dengue fever's prevention , diagnosis and treatment . Methods The epidemical data ,clinical characteristics ,laboratory results ,therapy and outcomes of 403 cases with dengue fever treated in Mengchao Hepatobiliary Hospital of Fujian Medical University ,Fuzhou from July to November 2016 were collected and analyzed retrospectively .Results Among the 403 cases , 56 cases (13 .89％ ) were younger than 20 years old ,160 cases (39 .71％ ) were 21 to 50 years old ,and 187 cases (46 .40％ ) were over 50 years old .The peak incidence period of dengue fever was from August to October ,accounting for 94 .29％ of the cases .The clinical classification was dominated by typical dengue fever (98 .01％ ) .Most of the cases had sudden onset .The main clinical symptoms included fever in 391 cases (97 .02％ ) , body aches in 355 cases (88 .09％ ) , and rash in 156 cases (38 .71％ ) . All patients were tested for serology and etiology of dengue virus at the early stage of disease .Among them , the positive rate of NS1 antigen was 95 .04％ ;the positive rate of IgM antibody was 13 .90％ ,and the positive rate of IgG antibody was 8 .19％ . Thrombocytopenia was founded in 332 cases (82 .38％ ) .Neutrophil count decreased in 293 cases (72 .70％ ) , w hite blood cell count decreased in 274 cases (67 .99％ ) .Aspartate aminotransferase increased in 256 cases (63 .52％ ) and alanine aminotransferase increased in 146 cases (36 .23％ ) .Creatine kinase elevated in 130 cases (32 .26％ ) and prothrombin time was prolonged in 40 cases (9 .93％ ) .A total of 289 cases (82 .75％ ) had bilateral lesions in both lungs and 25 cases (6 .20％ ) had pleural effusions . Color doppler ultrasound found 46 cases (11 .41％ ) of hepatomegaly ,84 cases (20 .84％ ) of splenomegaly ,8 cases (1 .99％ ) of a small amount of ascites . Conclusions Most of the 403 cases of dengue fever show typical clinical manifestations .Dengue fever mainly affects the blood system ,liver ,heart ,lung ,kidney ,skeletal muscle system ,and immune system . Clinicians should carefully observe the condition ,master the warning signs of severe dengue fever ,and achieve early identification and timely treatment of severe cases ,thereby reducing the mortality rate .

Objective To explore the application of "disease-characteristics nursing quality improvement" program in the nursing practice of atrial fibrillation patients. Methods On basis of patient-centered and evidence-based nursing, the cardiovascular department of Peking University First Hospital made the "disease-characteristics nursing quality improvement" program. The differences of patients′ satisfaction, the index of work satisfaction of nurses, the index of disease management before (2014)and after(2015)the implementation of "disease-characteristics nursing quality improvement"program were observed and compared. Results Before and after the "disease-characteristics nursing quality improvement" program, the patients′ satisfaction were 90.0%(135/150) and 96.8%(149/154), the rate of well warfarin knowledge were 74.3%(78/105) and 90.2%(101/112), the compliance rate of international standardization ratio were 79.0%(83/105) and 91.1%(102/112), the rate of telephone follow-up as requested were 57.3%(86/150) and 85.7%(132/154), the rate of outpatient follow-up as requested were 72.0%(108/150) and 83.1%(128/154). The differences all above were statistically significant (χ2=5.408-30.171,all P<0.05).Before and after the"disease-characteristics nursing quality improvement"program, the scores of the index of work satisfaction were(135.19±19.17)and(178.37±18.46)points.The difference was statistically significant (t=-13.577, P<0.05). Conclusions The "disease-characteristics nursing quality improvement"program is an effective method to improve the nursing quality of patients with atrial fibrillation.

Objective To investigate the effects of Dihuangyinzi(DHYZ) on behaviors and RAGE/p38 pathway in APP/PS1 mice.MethodTwenty APP/PS1 dementia mice were randomly divided into model group(n=10) and Chinese medicine group(n=10).The blank group was C57 BL/6 J normal mouse(n=10).The mice in Chinese medicine group were intragastric administration with DHYZ (9.75 g·kg-1·d-1).The mice in model group and blank group were treated with distilled water.After 30 days,the abilities of learning and memory of mice were detected by Morris water maze.The expression of amyloid-beta1-42(Aβ1-42) in the hippocampus and cortex was detected by immunohistochemistry.Reactive oxygen species of brain tissue were detected by DCFH-DA Methods in the brain of APP/PS1 mice.Gene expression level of receptor for advanced glycation end products(RAGE) was measured by real-time polymerase chain reaction (RT-PCR) in the cortex and hippocampus of APP/PS1 mice.The expression of phospho-mitogen-activated protein kinases (p38) was analyzed with Western blot and immunofluorescence analysis in the cortex and hippocampus of APP/PS1 mice.Results Behavioral Results showed that DHYZ significantly increased the distance((23.088±7.083)cm) and residence time((1.961±1.230)s)of effective area in Morris water maze on the fifth day(P<0.05,P<0.01)and remarkably increased the number of effective area crossings((1.607±0.405) times) and plats((0.893±0.283) times) in Morris water maze on the fifth day(P<0.01,P<0.05).DHYZ also significantly reduced the intracelluar ROS level(122.611±7.630) in the brain(P<0.01),and DHYZ could depress the expression of RAGE(1.467±0.081,7.983±0.136) and phosphorylation of p38 (0.376±0.026,0.538±0.016)in the cortex and hippocampus of APP/PS1 mice(P<0.01,P<0.05).Conclusions The Results demonstrate that DHYZ can partly improve memory impairment of APP/PS1 mice by the inhibition of RAGE/p38 pathway.

Objective To investigate the significance of CD64 combined with C-reactive protein (CRP) and procalcitonin(PCT) in the diagnosis of neonatal sepsis.Methods A total of 70 neonates diag-nosed with neonatal sepsis(sepsis group),35 cases of non-infectious diseases(non-infected group),and 40 healthy newborns(healthy control group) were enrolled in the Department of Pediatrics,Fuzhou First Hospital Affiliated to Fujian Medical University from July 2015 to June 2016. Serum CD64 was detected by flow cytometry.Serum CRP and PCT were detected by automatic biochemical analyzer,and the results were com-pared and analyzed.Results The levels of serum CD64,CRP and PCT in sepsis group were significantly higher than those in non-infected group and healthy controls(P <0.05).The sensitivity and specificity of CD64 combined with CRP and PCT in the detection of neonatal sepsis were 97.14% and 96.00%,and the sensitivity and specificity in the combined detection were higher than those in three indicators alone.Conclusion CD64 combined with CRP and PCT in the detection of neonatal sepsis can improve the specificity,and provide the basis for early diagnosis.

Objective To investigate the value of neutrophil CD64 combined with monocyte chemotactic protein 1 (MCP-1),interleukin-8 (IL-8) and interferon-inducible protein-10 (IP-10) in the clinical diagnosis of neonatal sepsis.Method Cases of neonatal sepsis from March 2015 to June 2016 in the department were chosen as sepsis group.35 neonates with non-infection diseases were selected as noninfection group and 40 healthy newborn infants were assigned as control group.The level of CD64 in blood were detected by flow cytometry,while the level of MCP-1,IL-8 and IP-10 of the serum in the three groups were detected by automatic biochemical analyzer.The differences between groups were compared by single factor ANOVA.The ROC curves of sepsis diagnosed by whole blood CD64,serum MCP-1,IL-8 and IP-10 were drew.Result The level of CD64,IL-8 and IP-10 of the neonatal blood in the sepsis group were significantly higher than that in the non-infection group (P ＜ 0.05) and control group (P ＜ 0.05).There were no significant difference between the sepsis group and non-infection group in MCP-1 (P ＞ 0.05),but significantly higher than that in the control group (P ＜ 0.05).The levels of serum MCP-1 and IP-10 in the non-infection group were significantly higher than those in the control group (P ＜ 0.05),but there was no significant difference between the non-infection group and control group in CD64 and IL-8 (P ＞0.05).The optimal thresholds of blood CD64,MCP-1,IL-8 and IP-10 in the diagnosis of sepsis were 35.0 MFI,58.6 ng/L,60.3 ng/L,0.46 μg/L.The sensitivity and specificity of the diagnosis of sepsis were 92.8％ and 90.6％ in CD64,70.0％ and 42.6％ in MCP-1,78.5％ and 68.0％ in IL-8,72.8％ and 54.6％ in IP-10,97.1％ and 94.6％ when combined.Conclusion The combination test of CD64,MCP-1,IL-8 and IP-10 can improve the sensitivity and specificity of the diagnosis of sepsis.