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.

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.

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.

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。

Background Evaluation of intra-retinal layer thickness plays an important role in the diagnosis and monitor of various eye diseases,and spectral domain optical coherence tomography (OCT) is a frequently used tool.Software analysis method was used to measure the retinal thickness in previous study,but the study on the reliability of automatic layered software is lack.Objective This study was to evaluate the repeatability and reproducibility of thickness profile measurement of intra-retinal layers determined by an automated algorithm applied to OCT images from RTVue100 OCT instrument.Methods In this prospective cross-sectional study,retinal thickness images at 6 mm around fovea were obtained from 18 right eyes of 18 normal subjects with RTVue100 OCT instrument.The retinal images were segmented into retinal nerve fiber layer (RNFL),ganglion cell layer and inner plexiform layer(GCL+IPL),inner nuclear layer (INL),outer plexiform layer (OPL),outer nuclear layer (ONL),inner segment (IS),outer segment (OS) and retinal pigment epithelial (RPE) layer using automated algorithm method.Then Matlab software was used to analyze the measuring outcome.Interclass correlation coefficient (ICC) and coefficients of reproducibility (COR) were calculated from the results of two-time examination by the same examiner to evaluate the repeatability and from the results of two different examiners to assess the reproducibility.Written informed consent was obtained from each subject prior to any medical procedure.Results The entire retinal thickness measured by RTVue-OCT was (303.22± 14.10) μm in the horizontal meridian and (306.68 ± 13.32) μm in the vertical meridian,with the maximum values of retinal thickness in the GCL+ IPL and ONL.Whether in the horizontal meridian or in the vertical meridian,the ICC and COR were ＜0.60 in the OPL,IS and OS;while those in the RNFL,GCL+IPL,INL,ONL and RPE layer were ＞0.70.Conclusions RTVue OCT with automated algorithm is a useful and reliable approach to the measurement of intra-retinal layer thickness.Automated segmentation can offer accurate and repeatable thickness profile of OCT retinal image.This method may improve the diagnosis and monitoring of retinal diseases.

Background Ultra-long scan depth OCT can achieve imaging of full range of human ocular anterior segment.However,the measurement of the dimension of anterior segment from the OCT image with high speed and precision is a challenge at present.The software of automatic data processing is still lack in analyzing spectral domain OCT.Objective This study was to perform the automatic biometry and data processing of human ocular anterior segment OCT image by using self-developed automatic detection software and evaluate the accuracy and repeatability of this method.Methods Twenty eyes of 10 normal subjects were included in Eye Hospital of Wenzhou Medical University from June to July 2013.The OCT image of anterior eye segments were obtained with custom-made ultra-long scan depth OCT under the informed consent.Anautomatic software algorithm was developed for the biometric measurement on these OCT images,including boundary segmentation,image registration and optical correction of OCT images.The boundary segmentation algorithm utilized the axial gradient information of OCT images and the shortest path search principal based on the dynamic programming to optimize edge finding.Central corneal thickness (CCT),anterior chamber depth (ACD),pupil diameter (PD),lens thickness (LT),radius of lens anterior curvatures (LAC) and radius of lens of posterior curvatures (LPC) were automatically and manually measured,and the validity of automatic detection algorithm was assessed by calculating the intraclass correlation coefficient (ICC) between the automatic and manual measurements,and the repeatability was validated by calculating the coefficient of repeatability (COR) between repeated measurement.This study was approved by the Ethic Committee of Wenzhou Medical University and informed consent was obtained from all subjects.Results There were no significant differences in the results of CCT,ACD,PD,LT,LAC and LPC between the automatic and manual measurements (P =0.205,0.167,0.285,0.127,0.102,0.074).The results were consistent between automatic and manual measurements (all at ICC＞0.75).The repeated measurement values were consistent in CCT,ACD and LT in both automatic and manual modes (all at ICC＞0.75).The reproducibilities of automatic biometry in PD and LAC (ICC =0.793,0.872;COR =2.90,5.79) were better than those of manual mode (ICC =0.631,0.579;COR =5.62,10.46);while the reproducibility of automatic biometry in LPC (ICC =0.663;COR =6.17) was lower than that of manual mode (ICC =0.794,COR =4.79).Conclusions Self-developed automatic detection software appears to be accurate and repeatable in measuring dimension of spectral domain OCT images.This automatic software algorithm can be used for the biometry and monitor of human ocular anterior segment.

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.