Hereditary hemolytic anemia(HHA)is a hereditary disorder with main clinical presentation of hemolysis and hemolytic anemia.It's the most common disease of hereditary disorders in the world.According to the pathogenic mechanism,the disorders can be divided into three groups:abnormalities of the red cell membrane,red cell enzyme disorders and hemoglobinopathies.The most common HHA are hereditary spherocytosis,G6PD deficiency and the thalassemias.The clinical presentation of HHA may be heterogeneity.No anemia may occur in the early stage or mild HHA.In order to detect HHA early,we should pay more attention to the morphology of red cell in the peripheral blood film.Recommendation of screening tests with high sensitivity and specificity will be improving the diagnosis of HHA.For correct diagnosis,the medical centres should establish red cell membrane protein electropheresis,direct quantitative assay of red cell enzyme,gene diagnosis and prenatal diagnosis of hemoglobinopathy.

Objective To investigate the clinical efficacy of Chinese herbal medicine and liquid wax in treating oral apatinib-induced hand-foot syndrome. Method Thirty patients with gastric or gastroesophageal junction adenocarcinoma, who had developed hand-foot syndrome after oral administration of apatinib for four weeks, with grade 0 in 3 cases, grade Ⅰ in 16 cases, grade Ⅱ in 9 cases and grade Ⅲ in 2 cases, were enrolled and treated by external application of Chinese herbal medicine and liquid wax on both hands and feet for three weeks. Result The 30 patients with hand-foot syndrome had grade 0 in 14 cases, grade Ⅰ in 14 cases, grade Ⅱ in 2 cases and grade Ⅲ in 0 case after treatment with Chinese herbal medicine and liquid wax. There was a definite therapeutic effect and a statistically significant difference (P<0.05). Conclusion External application of Chinese herbal medicine and liquid wax on both hands and feet for hand-foot syndrome not only has a definite therapeutic effect but is also an ideal means of prevention and treatment because of its noninvasive procedure and the high compliance of the patients.

Objective:To explore the humanistic care model and its effects in emergency nursing.Methods:Through literature research,analysis process and interviews,we constructed the humanistic care model,formulated corresponding humanistic care nursing measures,and implemented them in clinical practice.The humanistic care satisfaction questionnaire was used to evaluate the effectiveness.Results:The satisfaction rate of humanistic care was improved,and the difference was statistically significant (P ＜ 0.001).Conclusion:The construction of humanistic care model can significantly improve the satisfaction of emergency patients,and the standardized process should be established in the implementation of humanistic care.

Objective To describe the status and analyze the influencing factors of nurses' compassion fatigue and compassion satisfaction in a certain tertiary hospital,which may provide evidences for the emotional management of nurses by nursing managers.Methods A cross-sectional survey was used in this study and 246 nurses were recruited totally.They investigated about compassion fatigue and compassion satisfaction by questionnaires.Results The average score of nurses' compassion fatigue was (30.76± 5.61)points.The average score of nurses' compassion satisfaction was (25.68±4.12)points.There was a significant difference for nurses' compassion fatigue in various wards,and ICU nurses had the top score.There were no significant differences for nurses' compassion satisfaction.Conclusions The nurses' compassion fatigue and compassion satisfaction in this hospital was relatively moderate.ICU nurses' compassion fatigue score was higher than other wards.Education background,professional title,relationship with colleagues,extra work,support from leaders and family members,wards,education background and relationship with colleagues were the influencing factors of nurses' compassion satisfaction score.

Objective To investigate the management of prevention and control of catheter-related bloodstream in-fection (CRBSI)in intensive care units (ICUs)of tertiary hospitals in a city,and explore stategies.Methods Data about CRBSI prevention and control as well as quality management were collected and surveyed by self-developed questionnaires.Results Of ICUs in 8 hospitals,the incidence of CRBSI was 4.81 ‰,case infection rate was 5.02‰.6 (75.00%)hospitals had standard operating instruction for the prevention and control of CRBSI, 2 (25.00%)hospitals had maintenance record on central venous catheter,and 2(25.00%)hospitals implemented maximum sterile barrier and catheterization combination strategy,none hospital adopted anti-infective dressing and chlorhexidine bathing.Conclusion There are many problems in the standard operating instruction and implementa-tion of measures for the prevention and control of CRBSI in ICUs of tertiary hospitals in this city,which needs to be improved.

Objective To determine risk factors of environment and lifestyles involved in acute hematology departments of 29 secondary-care and tertiary-care hospitals in Shanghai. There were 206 hospitalized patients with de novo AML during June 2003 to April 2007 meeting the diagnostic criteria for AML by the World Health Organization, excluding those with definite history of radiotherapy or chemotherapy, and 412 age- and gender-matched controls with non-hematological other diseases from the same hospitals. All the participants were interviewed face-to-face using a standard structured questionnaire,including personal medical history, family medical history, history of medications, traditional Chinese herb medicines, exposure to occupational hazards, lifestyle factors, etc. in the past five years prior to onset of the disease. Conditional univariate and multivariate logistic regression analyses were performed to identify risk factors for AML. Results Logistic regression analysis showed that taking traditional Chinese herb medicine might be a protective factor for AML, with an odds ratio (OR) of 0. 39 and its 95% confidence interval (CI) of 0. 16-0. 98. Potential risk factors for AML included history of diabetes and taking hypoglycemic agents ( OR: 1.39, 95% CI: 0.67 -2. 87), occupational exposure to metal ( OR: 1.73, 95% CI: 0. 77 -3. 91 ), glue, paint and other coating materials, etc. ( OR: 1.72, 95% CI: 0. 93 - 3.2), raising animals (OR: 1.33, 95% CI: 0. 79 -2. 24). No association of living within a distance of 100 meters from high voltage power transmission lines, home renovation and decoration in the past year, hair dyeing, smoking,alcohol drinking with AML in adults was found. Conclusions Factors mentioned above such as occupational exposure to metal, glue, paint and other coating materials potentially increase risk for AML in adults,however taking traditional Chinese herb medicine is possibly a protective factor for it.

Objective To survey the incidence of acquired adult aplastic anemia (AA) in Shanghai, China. Meanwhile, we compared it with the previous data from China in 1986 and other countries in order to explore the trends. Methods Newly diagnosed AA patients were registered in 6 districts (Jingan, Xuhui, Huangpu, Changning, Putuo, Yangpu ) in Shanghai from 2004 to 2006. Then we calculated the crude and age-adjusted incidence of AA according to the population data from Shanghai Statistic Yearbook. Results There were 38 adult patients with acquired AA. The average crude incidence of AA was 0. 33/100 000 from 2004 to 2006. The incidences per 100 000 persons per year were 0. 40, 0. 14and 0. 64 in 18-34, 35-59 and ≥ 60 years, respectively. The rate of severe AA was 0. 17/100 000.Conclusion The incidence of severe AA has no marked change, but the total rate is a little decreased compared with the data from China in 1986.

Objective To examine the pathological changes in the liver of an AIDS patient with complicated infection of Pneumocystis carinii(PC). \ Methods\ A liver biopsy was made. The tissue was stained with HE, PAS, Giemsa, GMS, and acid\|fast staining, and examined under light microscope and transmission electron microscope. \ Results\ Granulomas (acid\|fast negative) in the tissue and numerous pathogens (PAS positive) in hepatic sinusoids were detected. Giemsa and GMS staining and electron microscopy all confirmed that the pathogen was Pneumocystis carinii. \ Conclusion\ The pathological findings revealed a diffuse extrapulmonary infection of Pneumocystis carinii in the patient of AIDS.

Objective To investigate the effects of early continuous renal replacement thempy(CRRT)on acute lung injury and prognosis in severe acute pancreatitis (SAP)patients.Methods 46 SAP patients were divided into the two groups randomly:the control group and CRRT treatment group.The levels of IL-1β,IL-6,TNF-α,the APACHEⅡscore,oxygenation index,the incidence of acute lung injury(ALI)/acute respiratory distress syndrome (ARDS),ICU stay were compared between the two groups.Results The level of interleukin-6(IL-6)in the con-trol group was significantly higher than that in CRRT group in day 1(t=2.265,P<0.05);The levels of interleukin-1β(IL-1β),interleukin-6 (IL-6 ),tumor necrosis factor-α(TNF -α)in the control group were significantly higher than that in CRRT group in day 3(t=2.305,2.471,2.293,all P<0.05);the oxygenation index in the control group was significantly lower than that in CRRT group in day 3(t=2.386,P<0.05);the incidence of ALI/ARDS, the ICU stay days,the fatality rate in the control group were significantly higher than that in CRRT group(P<0.05);there was no significant differences between late group than that early group in gender,age,APACHE Ⅱ score (all P>0.05).Conclusion Early CRRT therapy can eliminate the IL-1β,IL-6 and TNF-αin SAP patients,which can improve the oxygenation index and reduce the incidences of ALI/ARDS,may provide more clinical benefits in the early phase of SAP.

Objective To evaluate whether the functional MRI could be used to reflect the change of angiogenesis after drug treat-ment,and obtain the related semi quantitative and quantitative parameters by dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI).Methods The subcutaneous transplantation colorectal cancer were constructed in 20 nude mice,which were treated with Xiaoaifeimi.The transplanted tumor microvascular density,VEGF and PCNA were monitored.The therapeutic effect and MRI monitoring results were evaluated by combining DCE-MRI with the pharmacokinetic model.The semi quantitative and quantitative parameters were obtained for evaluating the effection of medicine.Half of nude mice were sacrificed to obtain the immunohistochem-ical staining.Correlation between pathological findings and parameters were analyzed.Results Compared to the control group,hu-man colon HT-29 cell proliferation rate was significantly decreased (44.87%)(P <0.05),and the apoptosis rate of HT-29 cells was increased 2.45 times (P <0.05)after Uyghur medicine treatment.The correlation between pathological examination and DCE-MRI parameters showed that Ktrans value and Kep were positively correlated with VEGF,MVD and PCNA (P <0.05).Conclusion A good relationship is showed between immunohistochemistry and DCE-MRI quantitative parameters in Uygur drug treating human colon cancer mice.