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.

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。

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.

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.

OBJECTIVE: To evaluate the efficacy of standardized medication for patients with chronic rhinosinusitis. METHOD: According to the diagnosis and treatment guidelines on chronic rhinosinusitis formulated in 2008, by means of prospective study, we studied 54 patients suffering from chronic rhinosinusitis treated with standardized medication including, a combination of local intranasaI corticosteroids, macrolides, mucus discharging agent and nasal irrigation treatment and followed up for 3 months. Visual analogue scale (VAS), sino nasal outcome test-20 Chinese version scales (SNOT-20 CV), Lund-Mackay CT and Lund-Kennedy endoscopy methods were employed to conduct the subjective and objective assessment and comprehensively evaluate the clinical efficacy before and after treatment. RESULT: (1) After three months of standardized medication, the patients' total scores of VAS, SNOT-20 CV, CT and endoscopy were improved significantly compared with those before-treatment (P < 0.01 for all these scoring systems). (2) There was statistically significant difference between the clinical efficacies of chronic rhinosinusitis patients with and without nasal polyps groups (P < 0.01). After 3 months of standardized medication, the effective rates of the CRSwNP group evaluated by subjective assessment and CT evaluation were 66.7% and 94.4% respectively, while those of the CRSsNP groups were 91.7% and 97.2% respectively. (3) Betwecn CRSwNP and CRSsNP groups, there was no significant difference in the improvement rate or inefficiency rate in subjective assessment except for the cure rate, while there were significant differences in both cure rate and improvement rate in CT evaluation. (4) The CRS patients' self-testing-based questionnaires results showed positive correlation with objective assessments. CONCLUSION The standardized medication with combination of intranasal local glucocorticoid, macrolides (14-membered ring), the mucus discharging agent and nasal irrigation on CRS was effective.
Administration, Intranasal ; Adult ; Aged ; Chronic Disease ; Female ; Glucocorticoids ; administration & dosage ; therapeutic use ; Humans ; Macrolides ; administration & dosage ; therapeutic use ; Male ; Middle Aged ; Prospective Studies ; Quality of Life ; Rhinitis ; drug therapy ; Sinusitis ; drug therapy ; Treatment Outcome ; Young Adult

Objective To observe the feasibility of posterior internal fixation with pedicle screw rod system for upper cervical vertebra injury. Methods 16 patients with upper cervical vertebra injury accepted posterior pedicle screw system internal fixation were reviewed. Results Venous plexus behind C2 damaged in operation in a case, who needed a microscope for hemostasis. No complication, such as neurological symptoms worse, cerebrospinal fluid leakage, hematoma and infection of incision happened post operation. The neurological symptoms improved 81.8% in all the 7 cases who complained before operation. No complication was found in the follow-up 3 to 18 months after discharge. Their activities of upper cervical was basically unaffected. Conclusion Posterior internal fixation with pedicle screw rod system can provide stable support for patients with upper cervical injury.

Objective To study the effect of Coxsackie virus B3 (CVB3) infection on islet cells in vitro, and to explore the mechanism of islet cells caused by CVB3. Methods Bone marrow mesenchymal stem cells( BMSCs) were separated from the bone marrow and cultured. Then they were induced to differentiate into islet-like cells using nicotinamide and mercaptoethanol. Differentiated cells were detected by morphology , special staining and RT-PCR. Observe CVB3 infection on islet cells under inverse microscope and detect the specific gene fragment by RT-PCR. Results BMSCs showed half suspended shape and gathered to form a cluster after induction. Cells became red brown by dithizone specific staining. RT-PCR also proved the existence of mRNA expressing insulin. Infected islet cells appeared typical pathological changes like shrinks, refraction decreases. RT-PCR detected the desired specific gene fragment of 299 bp in infected islet cells. Conclusion CVB3 can directly injury islet cells, and damage the function of islet cells of secreting insulin.