Cyclic neutropenia (CN) is a kind of hereditary disease with periodic onset of neutrophil reduction and recurrent infection,which can be sporadic or familial.CN was firstly reported in 1910,and it was not until 1999 that the cause of the CN was further identified by investigators.Because the incidence of CN is low,the disease is rarely reported in China.CN is caused by mutations in the gene for neutrophil elastase (ELA-2 or ELANE),and it is a rare,inherited autosomal dominant disorder.Diagnosis of CN is based on the medical history and blood cycle characteristics.With the continuous development of medical technology,gene testing has certain significance for the diagnosis of CN.For the treatment,the most important is infection prevention,symptomatic treatment,the use of granulocyte colony-stimulating factor,and so forth.Although CN is a hematological disease,most cases have good prognosis,and will not result in malignant hematological diseases such as leukemia.At present,the doctors' awareness of CN has been lacking,which leads to misdiagnosis.The symptom and sign are not specific at the onset of CN,thus there is often misdiagnosis.In order to deepen the understanding of this disease and improve the skills of diagnosis and treatment,this review summarizes the pathogenesis,clinical manifestation,clinical characteristics,diagnosis and treatment of CN.

In recent years,the studies have shown that the incidence of type 1 diabetes(T1D)is closely related to the infection of human enteroviruses,particularly Coxsackie B Viruses(CVB).On one hand.the researches show that vires infection can induce T1D,and it is associated with the viral amount.the viral strain,and the host microenvironment.On the other hand,the studies confirm that viral infection also can inhibit the incidence of T1D.In terms of the factors,which is related to the vires infection-inducing.T1D.and the possible mechanism of the virus infection-inhibiting-T1D,the article aims to explore the relationship between CVB infection and the incidence of T1D based on referring to foreign literatures in recent years.

Objective To investigate the behavior characteristics of children or juveniles with accident proneness and to explore the influencing factors of children and juveniles accident proneness in behavior.Methods The study was carried out bv case-control method,and 1 601 children and juveniles from a rural area of hunan province were investigated with homemade general injury questionnaire.Accident proneness children or juveniles screened out from cross-sectional studv were selected into research group,while uninjured children or juveniles whose gender,age and grade were the same with the research group were included into control group with 1:1 matched.Achenbach Child Behavior Checklist was used to evaluate and compare these two groups of children and juveniles' behavior.Results 147 accident proneness children and juveniles were screened out from the study and the accident proneness incidence of children and juveniles in rural areas was 9.42％.The total score and each factor score of behavior problem and behavioral problems detection rate iu research group were higher than that of the control group and there were statistically significant (P＜0.01).Multivariate analysis showed that attention problem (OR=1.110,95％ CI =1.041-1.185) and aggressive behavior (OR=1.123,95％ CI =1.068-1.181) were risk factors of accident proneness.Conclusion Accident proneness children and juveniles' behavior is different from the uninjured,which is a risk factors of accident proneness.

Libraries are faced with the crisis of being marginalized in the era of network.Medical academic library should thus adapt to the changing information behaviors of users in study , teaching and scientific research and pro-vide their service according to the user needs-orientedsubject librarian service model , mobile library service model, and sharing commons service model.The service provided in the top 18 medical academic libraries in China according to the 3 models was analyzed, which showed their low popularization rate, insufficient depth and repeti-tion.Measures that should be taken for the solution of such problems were put forward.

Objective To investigate the kidney function changes and the risk factors in elderly patients aged 80 years and over.Methods A total of 1054 hospitalized patients aged ≥80 years in our hospital were selected.Serum levels of creatinine,urea,uric acid and urinate were tested.Cockcroft-Gault equation was used to estimate glomerular filtrate rate (GFR).Results The renal function in 87.1 ％ of the patients was reduced.The percentage of patients with 30≤eGFR ＜60 ml · min-1 · 1.73 m-2 was 66.2％.The percentage of patients with 15≤eGFR ＜30 ml · min-1 · 1.73 · m-2had a gender difference (P＜0.05).Serum creatinine level was not an ideal indicator for early renal dysfunction in the elderly,especially in emaciated elderly people.Multivariate logistic regression analysis showed that gender,hypertension,coronary heart disease and hyperuricemia were independent risk factors for the decline of renal function (OR=1.937,1.602,1.842,7.020,respectively,all P＜0.05).Conclusions The rate of renal dysfunction is high in very old people.Many risk factors affect renal dysfunction.

Insulin resistance model of 3T3-L1 adipocytes were prepared with plamotic acid.Adipocytes with generated insulin resistance were cultured with different concentrations of globular domain of adiponectin(gAd:250,500,1 000 ng/ml).The cell culture medium glucose content was detected with the glucose oxidase method,the mRNA expressions of insulin receptor substrate-1 (IRS-1),phosphatidylinositol-3 kinase(PI3K),and protein kinase B(PKB) were detected with real-time quantitative PCR method.The phosphorylation of IRS-1 was detected by Western blot.Compared with the control group,the experimental group showed significantly increased glucose consumption (P ＜ 0.01),and with the increasing gAd concentration,glucose consumption was gradually increasing.IRS-1 phosphorylation was increased gradually with the increasing concentration of gAd.These results suggest that gAd can promote glucose uptake by 3T3-L1 adipocyte model with generated insulin resistance.This may be correlated with promoting insulin signal transduction and improving insulin resistance in adipocytes.

目的探讨后外侧小切口行全髋置换术(THA)后患者康复护理。方法后外侧入路小切口患者45例48髋,经精心护理后与常规THA患者在手术切口、围手术期出血量、术后疼痛、住院天数、早期活动方面比较。结果在相同条件下,小切口手术患者减少围手术期出血量、术后疼痛、住院天数及术后早期康复快,缩短住院时间。结论康复护理有助于THA患者的恢复。

Objective To evaluate the efifcacy and inlfuencing factors of 3%hypertonic saline (HS) inhalation in treatment of bronchiolitis. Methods Clinical data together with the detection of 16 types of respiratory tract virus from hospitalized pediatric patients with primary diagnosis of bronchiolitis from June 2009 to December 2012 were retrospectively analyzed. The endpoint indicators for evaluation on the efifcacy of nebulized 3%HS inhalation were the percent decrease of clinical severity (CS) score after 2 days' treatment and the hospitalization time. Factors affecting efifcacy were further explored. Results The CS score in nebulized 3%HS treated group decreased in average of 42.86%(11.11%-66.67%), signiifcantly higher than that (26.79%, 0.00%-50.00%) in the untreated group (P=0.006). No difference of between the two groups (P=0.26). Multiple linear regression analysis showed that nebulized 3%HS inhalation has better efifcacy on the patients older than 3 months having breastfeeding, respiratory synthetic syncytial virus (RSV) infection and extensive wheezing sound auscultation of the lungs. The multiple linear regression analysis model was statistically signiifcant (R2=0.58, P<0.001). Conclusions After 2 days' treatment with 3%hypertonic saline inhalation, the CS score of bronchiolitis patients was decreased. The treatment can be recommended in hospitalized patient older than 3 months with breastfeeding, RSV infection, and extensive wheezing sound auscultation of the lungs.

Objective To study the effectiveness of niacinamide in treating maintenance hemodialysis patients with hyperphosphatemia.Methods It was a prospective and randomized controlled trial.Patients with hyperphosphatemia (serum phosphate ＞ 1.45 mmol/L) were randomly assigned into two groups:control group (continue their original phosphate binder and rocaltrol treatment) and niacinamide therapy group (additionally received niacinamide,titrated from 600 mg/d to 1200 mg/d).The treatment lasted for 8 weeks.Serum phosphate and calcium were tested every 2 weeks and normalized protein catabolic rate and other relevant indexes were tested monthly.Results 100 patients were recruited and 93 of them completed the trial,including 44 from the therapy group and 49 from the control group.By the repeated measures analysis of variance,changes of serum phosphate in two groups displayed a statistical significant difference,but the levels of serum calcium in both remained steady.At the end of trial,compared to control group,therapy group appeared decreased serum phosphate levels [(1.59±0.36) mmol/L vs (1.94±0.25) mmol/L,P ＜ 0.001] and increased serum HDL levels [(1.32±0.54) mmol/L vs (1.09±0.41) mmol/L,P=0.02].Meanwhile,two groups showed no significant difference in intact parathyoid hormone and alkaline phosphatase.Adverse reactions including thrombocytopenia and gastrointestinal dysfunction were observed in niacinamide therapy group.Conclusions Niacinamide is effective on controlling hyperphosphatemia along with phosphate binder in maintenance hemodialysis patients.It also increases the serum HDL levels.Nonetheless,it is important to monitor the number of platelet.

Objective To investigate the therapeutic efficiency of intravenous implanted endothelial progeni-tor cells (EPCs) pretreated with stromal cell derived factor-1 (SDF-1) in rat model of acute myocardial infarction (AMI). Method The bone-marrow derived EPCs were cultured. The effects of SDF-1 on the migration and sur-rival of EPCs were evaluated in vitro. The rat models of AMI were produced and randomly divided into SDF-1 +EPCs group (n=12), EPCs group (n=12) and control group (n=12). EPCs pretreated with or without SDF-1 were infused via tail vein 24 hours after AMI modelled. The EBM-2 culture medium without cell was infuaed in control group. Vessel density, cardiac function and infarct area were measured on 14 days and 28 days after cell implantation. Results Fourteen days after cell implantation, the vessel density of SDF-1+EPCs group was higher than that of EPCs group and control group. Twenty-eight days after cell implantation, the cardiac function of SDF-1+EPCs group was better than that of EPCs group and control group. Conclusions EPCs pretreatment with SDF-1 can improve the survival and migratory capacity of EPCs, and increase the therapeutic efficiency for myocardial infarction.