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 explore the feasibility and clinical effects of spinous proscess forming in posterior in the treatment of lower lumbar diseases. [Methods]Seventy-seven cases suffered from lower lumbar diseases were treated using spinous proscess forming as interbody fusion material.The cases included 35 of lumbar disc herniation,21 of spondylolysis,11 of degenerative spondylolisthesis and 10 of spinal canal stenosis.The clinical data and imaging results were investigated.[Results]Seventy-two patiens were followed up for 1 to 2 years while 5 were lost.During following-up,no loosened internal fixation,broken nail or stick,infravertebra prolapsed,or detachment recurrence was found.Preoperative the height of intervertebral space was 8.5?1.9 mm,10.9?1.8 mm at 2 weeks postoperatively and was 10.7?1.7 mm at last follow-up.The height of intervertebral space were significantly increased postoperatively with statistically significant difference(P

Objective To observe the effects of baicalin on forms of hepatic tissue, liver apoptosis, mRNA expressions of iNOS, NF-κB and protein expression of Caspase-3 in rats with ischemia reperfusion injury; To discuss its mechanism of action.Methods The rat models of liver ischemia reperfusion were performed according to the Pringle's method. Rats were randomly divided into sham-operation group, model group and baicalin group. Sham-operation group and model group were given normal saline for gavage, while baicalin group was given baicalin for gavage. Morphological characteristic was observed by HE staining. Hepatocyte apoptosis was determined by TUNEL. The mRNA expressions of iNOS and NF-κB were determined by RT-PCR. The protein expression of Caspase-3 was determined by Western blot.Results Compared with the sham-operation group, mRNA expressions of iNOS and NF-κB and the protein expression of Caspase-3 in the model group increased, as well as liver apoptosis rate (P<0.05,P<0.01); compared with the model group, mRNA expressions of iNOS and NF-κB and the protein expression of Caspase-3 in the baicalin group decreased, as well as liver apoptosis rate (P<0.05), and the hepatic lesion significantly improved in the baicalin group.Conclusion Baicalin can restrain Caspase-3 induced apoptosis by reducing the expressions of iNOS and NF-κB, with a purpose to realize the hepatoprotective effect for liver of rats with ischemia reperfusion injury.

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 In order to measure the nursing unit workload scientifically,we used the Delphi method to investigate the influencing factors of nursing unit workload.Methods After consulting literature and discussing with experts,alternative indicators were designed,then two rounds of consultations were made with experts by using the Delphi method.Results The questionnaires revealed that the valid recovery rate,after two rounds of consultations,were 95％ and 100％ respectively,which reflected the positivity of the experts which participated in this study.The expert authority coefficient was 0.896,which indicated the high reliability of the consulting.The coordination indexes of expert opinions were 0.467 and 0.239 respectively,which showed the expert advices had good coordination and the results had high reliability.Conclusions The influencing factors of nursing unit workload determined by Delphi method have high reliability,which is significant for the scientific measuring of the nursing unit workload.

Objective Based on our previous established cohort of myelodysplastic syndrome (MDS), we investigated the potential effect of beta-tubulin (TUBB) gene in the transformation of MDS into acute leukemia Methods From our nested case-control study cohort of MDS patients, we chose 11 paired transformed and nontransformed MDS patients.TUBB gene expression was tested by quantitative real-time PCR.TUBB-siRNA transfection was used to down-regulate TUBB gene expression in SKM-1 cell line.The function of TUBB gene in SKM-1 cell line was evaluated by cell proliferation, soft agar clone formation and electron microscope.Results TUBB gene expression in MDS patients in transformed group were significantly higher than that in control group (2.91 ± 0.41 vs 0.90 ± 0.23, P ＜0.01).After TUBB-siRNA transfection, A450/630nm of SKM-1 cells at 24 h, 48 h and 72 h were 0.299 ± 0.045, 0.526 ± 0.034 and 0.652 ± 0.035, respectively, which were significantly decreased than those in negative-siRNA group (0.438 ±0.074, 0.858 ±0.064 and 0.974 ±0.044) (P ＜0.05).Soft agar clone formation in TUBB-siRNA group was (7.0 ±0.2)％, which was significantly reduced than that of negative-siRNA group (25.0 ± 0.2)％ (P ＜ 0.01).Electron microscope showed significant apoptotic signs in TUBB-siRNA group, including vacuoles in cytoplasm and karyorrhexis.Conclusion Our results indicate that TUBB gene may play a role in the transformation of MDS into acute leukemia by affecting the proliferation of malignant clones.

Objective To explore the effects of homocysteine (Hcy) on neural stem cell (NSC) proliferation and the mRNA expression level of Notch1 and Hes1 related Notch signaling pathway from neonatal rats in vitro. Methods NSCs from neonatal rats were cultured by serum-free culture method in vitro. Cells were divided into four groups: control group (Hcy-C), low dose Hcy (Hcy-L) group, middle dose Hcy (Hcy-M) group and high dose Hcy (Hcy-H) group. NSCs were iden- tified by immunofluorescent staining using the antibodies against Nestin, β-tubulin Ⅲ and GFAP. The proliferation ability of NSCs was detected by MTT. The mRNA expressions of Notch1 and Hes1 were detected by Real-time PCR. Results In the serum free suspension medium, neurospheres that consisted of a great number of nestin-positive cells were found. β-tu- bulin Ⅲ positive neurons and GFAP positive astrocytes were detected by immunofluorescence staining on the 6 th day of cell induction. MTT assay showed that the cell viability was significantly lower in three Hcy treatment groups than those of con- trol group (P ＜ 0.05). And the effect of concentration-dependent was observed. The results of RT-PCR showed that mRNA expression of Hes1 was significantly lower in three Hcy treatment groups than that in control group (P ＜ 0.05). The mRNA ex- pression of Notch1 was significantly lower in Hcy-H group than that of other three groups (P ＜ 0.05). The mRNA expression of Notch1 was significantly lower in Hcy-M group than that of Hcy-L group and control group (P ＜ 0.05). Conclusion Hcy could inhibit the proliferation of NSCs by down-regulating mRNA expression levels of Notch1 and Hes1 genes related to Notch signal pathway.

Objective To investigate the characteristics of dysplasia in myeledysplastic syndrome (MDS). Methods 716 samples of adult patients with abnormal blood routine and unelear cause were collected between July 04, 2003 and March 14, 2007. Based on the gold diagnostic standard of WHO MDS classification, all eases were detected on cytomorphology, cytochemical stain, bone marrow pathological assay,cytogenetics, flow eytometry et al. The cytological study of bone marrow on some abnormal hematopaietie cells has a diagnostic value to determine clonal or non-clonal diseases and assess sensitivity and specificity. Results in the complicated various dysplasia of hematopeiefic ceils, the following characteristics can be the main basis of cytomorphological diagnosis: one of granular Auer bodies, micronuclens (MN), or nuclear budding, erythroid nuclear budding, megakaryocytes presented in peripheral blood, myeloblast or prorubricyte exhibited in peripheral blood, ringed sideroblasts>1%. The subordinate basis of cytomorphological diagnosis development of nuclei, ring-shaped nuclei, and aggregation of nuclear chromafin, erythroid multi-nuclei, odd nucleus, mother-daughter nucleus, nuclear fragmentation, vacuole, anisoeytosis and mieromegakaryocytes.Conclusion Cytomorphologic assay is the base for the diagnosis of MDS, however, it presents certain limit,especially when eytomnrphoiogical change does not possess specificity for early MDS. Hereby, it requires to combine other deteetion methods.

PurposeTo explore the relationship between expression of apoptosis-modulating proteins and chemotherapeutic efficacy in acute leukemia. MethodsImmunocytochemical method was used to detect the expression of Bcl-2、Bcl-XL、Bax and Bak in 36 cases of acute leukemia including previously untreated/ drug-sensitive group and refractory/relapse group. ResultsThe average positive cell rates of Bcl-2 and Bcl-XL in refractory/relapse group were (41.68 ± 14.39) % and (35.96 ± 9.95 ) %, while the rates in previously untreated/drug-sensitive group were (15.64 ± 8.51 )% and (12.91 ± 8.63 )%. Statistical analysis showed the average positive cell rates of Bcl-2 and Bcl-XL in refractory/relapse group were higher than those in previously untreated/drug-sensitive group (P ＜ 0.01 ). There was no significant difference in average positive cell rates of Bax and Bak between refractory/relapse group (25.28 ± 15.49) %, (15.53 ± 10.64) % and previously untreated/drug-sensitive group (21.55 ± 12.58)%, (13.23 ± 8.36)%. The Logistic regression of expression of Bd-2 、Bcl-XL、Bax and Bak to complete remission rate (CR) of 36 cases of acute leukemia showed that Bcl-XL was the most risk factor in reducing the CR.ConclusionsBcl-2 and Bcl-XL might play important roles in multi-drug resistance of acute leukemia and Bcl-XL was more important than Bcl-2.