The knowledge and understanding of myeloproliferative neoplasms (MPN) over the last hundred years has been reviewed in this article,focusing on clinical practice.The identification of JAK2 V617F gene mutation leads Philadelphia chromosome-negative (Ph) MPN into a new era of molecular biology.These advances not only provide a reliable diagnostic tool and important evidence for diagnosis of MPN,also induce a lot of investigation and manufacture of targeting drugs to JAK2 mutation.However,JAK2 V617F mutation is not the gold standard for the diagnosis of MPN,as unique as bcr-abl in CML.Certain routine lab results and differentiation with some other diseases are still necessary.A JAK1/JAK2 inhibitor,ruxolitinib,has been approved for clinical use,but indication should be followed.Further follow-up is needed to assess the longterm outcomes with respect to efficacy and safety.It is not time to give up conventional medicine,such as hydroxyurea or aspirin.

Objective To identify the pathogenic microorganism by MALDI-TOF MS.Methods A total of 560 strains were resuscitated,which included 260 gram-positive bacteria strains,180 gram-negative bacteria strains,60 yeast-like-fungi strains and 60 enteropathogenic bacteria strains.Comparing MALDI-TOF MS with Vitek2 Compact,the discordant results were validated by 16S rDNA sequencing.Results Comparing MALDI-TOF MS with Vitek2 Compact,the coincidence rate was 94.6％ (246/260) for gram-positive bacteria,96.7％ (174/180) for gram-negative bacteria,95％ (57/60) for yeast-like-fungi,and 93.3％(56/60) for enteropathogenic bacteria Fifteen strains were validated by 16S rDNA gene sequencing.Comparing with sequencing,the coincidence rate of two methods was 66.7％ ( 10/15 ) for MALDI-TOF MS and 26.7％(4/11 ) for Vitek2 Compact,respectively.Conclusion MALDI-TOF MS shows rapid turnaround time and modest reagent costs,and it will be another effective tool for microorganism diagnosis.

PurposeTo explore the gemstone CT with low tube voltage and low concentration contrast medium in evaluating renal artery imaging of preoperative living renal transplantation donor.Materials and Methods Fifty cases of living kidney donor underwent spiral CT angiography of renal artery before operation. The patients were randomly divided into double-low group (25 patients) with 100 kV and Visipaque (270 mg/ml), and control group (25 patients) with 120 kV and Iopromide (370 mg/ml). Image reconstruction of 40% ASiR was used in both groups. The image quality score, CT values of renal arteries, contrast-to-noise ratio (CNR), noise, radiation dose and the amount of contrast agent of the two groups were recorded and compared.ResultsKappa coefifcient analysis showed that the consistency in evaluating image quality between two radiologists was excellent (Kappa=0.82). The CT value of the renal arteries and image quality did not show signiifcant difference between the two groups (t=1.05, 0.07 and 1.62,P>0.05). The CNR, noise and average radiation dose were statistical different between the two groups (t=2.92,-6.95 and-2.21,P<0.05). The contrast medium dosage of double-low group was decreased by 27% when compared with that of the control group.Conclusion Satisfied image quality of renal artery CTA can be obtained with low tube voltage (100 kV) and low concentration contrast medium (270 mg/ml) combined with iterative reconstruction algorithm. It can be used as a routine method in preoperative examination of living renal donors.

Objective To compare the clinical effect of the primary total hip replacement (THR)and the secondary THR after failed internal fixation in elderly patients with displaced femoral neck fracture,so as to investigate the optimal treatment for displaced femoral neck fractures in elderly patients.Methods From January 2006 to December 2012,22 patients treated with a secondary THR after failed internal fixation were chosen as the observation group and 30 patients treated with a primary THR were chosen as the control group,the operation time,blood loss,postoperative Harris scores for hip joint and health-related quality of life (~ index score) were observed and analyzed in two groups.Results Both of the groups were followed up successfully and each of the groups had 1 patient died.There were no joint infection,re-operations and any other complications in both groups.The operation time in observation group was longer than that in control group [(114.82 ±32.13) min vs.(90.63 ± 16.24) min],blood loss was more than that in control group [(551.73 ± 241.62) ml vs.(314.46:± 156.72) ml],there was significant difference (P ＜ 0.05).But the Harris score and KPS index score between two groups had no significant differences (P ＞ 0.05).Conclusion The primary THR in elderly patients with displaced femoral neck fracture has lower risk in operation tocompare with the secondary THR after failed internal fixation,but the two ways are similar in the hip joint function recovery and improve patients quality of life.

Objective To compare the energy expenditure of a hemicorporectomy amputee moving with different mobility devices. Methods A middle-aged male hemicorporectomy amputee was measured with cardiopulmonary exercise test when moving with 4 kinds of transport: socket, cart, wheelchair, and prosthesis. Results The patient moved the slowest with prosthesis, fastest with wheelchair. The heart rate was the highest with prosthesis and lowest with cart. The absolute oxygen consumption, the relative volume of oxygen consumption and metabolic (Mets) were the most when hand walking with socket, and lowest with cart. Conclusion Hand walking with socket costs the largest energy when walking, and the rest are prosthesis, wheelchair, and cart.

Objective To investigate the cognition about rehabilitation among parents having retarded children aged 0～6 in Beijing. Methods237 retarded children diagnosed in Disabilities Sampling among 0～6 Years Children in Beijing in 2004 were followed up 3 years later, and their parents were investigated with questionnaire about their rehabilitation knowledge. Results25.99% of these children had received rehabilitation services in the 3 years at least once, and was higher in city than in country. 38.39% and 16.52% of their parents knew their children need early rehabilitation and family training. ConclusionIt is necessary to communicate the knowledge of rehabilitation to the parents of retarded children.

Objective To investigate the effect of adenovirus-mediated IL-24 (Ad-IL-24)gene expression on the apoptosis in a human squamous cell carcinoma cell line COLO 16, and to explore the underlying molecular mechanism. Methods Cultured COLO 16 cells were divided into two groups to be transfected with an adenovirus vector carrying the IL-24 gene (Ad-IL-24 group)or green fluorescent protein (Ad-GFP group), while those receiving no treatment served as the control group. After culture for different durations, qPCR was performed to quantify IL-24 gene expression, methyl thiazolyl tetrazolium (MTT) assay to evaluate the proliferative activity of COLO 16 cells, flow cytometry to detect the apoptosis of COLO 16 cells, laser scanning confocal microscopy (LSCM) to observe the morphological changes of COLO 16 cells, Western blot to determine the levels of Bax and Bcl-2 proteins and to evaluate the activation of caspase-3, qPCR to determine the levels of Bax and Bcl-2 mRNAs, an immunofluorescence assay to observe the expression of Bax and Bcl-2 proteins. Statistical analysis was carried out by a two-sample t-test with the SPSS 19.0 software. Results MTT assay showed that the proliferation of COLO 16 cells in the Ad-IL-24 group was significantly inhibited as early as 4 days after the transfection; thereafter, the inhibitory effect increased in a time-dependent manner, and peaked on day 6(P<0.05). However, there was no significant difference in cellular proliferative activity between the Ad-GFP group and control group (P>0.05). Flow cytometry revealed that the apoptosis rate was significantly higher in the Ad-IL-24 group(13.10%± 0.92%)than in the control group(3.69%± 0.36%, P<0.05)and Ad-GFP group(3.39%± 1.06%, P<0.05), but no significant difference was observed between the control group and Ad-GFP group (P>0.05). LSCM demonstrated that the apoptosis of COLO 16 cells was accelerated in the Ad-IL-24 group. The immunofluorescence assay, Western blot and qPCR all showed that the mRNA and protein expressions of Bax were increased, but those of Bcl-2 were decreased in the Ad-IL-24 group compared with the Ad-GFP group and control group. Moreover, Western blot showed a protein band that could specifically bind to the anti-cleaved caspase-3 antibody in the Ad-IL-24 group, but not in the Ad-GFP group or control group. Conclusions Ad-IL-24 can induce apoptosis in human COLO 16 squamous cell carcinoma cells, probably by up-regulating Bax expression, down-regulating Bcl-2 expression, and activating caspase 3.

Objective To discuss the value of color Doppler echocardiography in diagnosis of scimitar syndrome .Methods The echocardiographic results of 6 patients with a diagnosis of scimitar syndrome were reviewed retrospectively .Their sonographic and hemodynamic characteristics were also analyzed connected with the reports in the literature .Results Three cases had dextrocardia and the others had mesocardia .All cases got right ventricular dimension enlargement .Total or partial of right pulmonary venous connection to the inferior vena cava were 3 cases respectively .All cases had right pulmonary artery hypoplasia .All of 6 cases echocardiographic results were in accordance with the findings by CT angiography and 4 cases were confirmed by operation .Conclusions The sonographic features of scimitar syndrome were obvious ,and echocardiography was contribute to early diagnosis of scimitar syndrome .

Objective:To compare the clinical effects of high volume zero-balanced ultraifltration (ZBUF) with different replacement lfuid in infant patients.
Methods: A total of 40 infant patients who received aortic coarctation with deep hypothermic circulatory arrest operation in our hospital from 2012-11 to 2014-02 were summarized. The patients were randomized into 2 groups, Group A, the patients had ZBUF with plasmalyte A, n=21 and Group B, the patients had ZBUF with modiifed replacement lfuid n=19. All patients received ZBUF (15-20) ml/kg during re-warming period until the temperature reached 34°C at rectum. Blood gas analysis were conducted at 4 time points as T1 (before CPB), T2 (open ascending aorta and 5 min after calcium perfusion), T3 (ifnishing ZBUF) and T4 (end of CPB). The intra-operative CPB time and the post-operative recovery with the complication were recorded in all patients.
Results: For blood gas analysis, the calcium concentration was higher in Group B than that in Group A at T3 time point, P<0.01 and the base excess was higher in Group A than that in Group B at T3 time point, P<0.05. Those indexes were similar at T1, T2 and T4, all P>0.05. The other indexes were similar between 2 groups, P>0.05 and the post-operative recovery was similar between 2 groups, P>0.05.
Conclusion: ZBUF with modiifed replacement lfuid could avoid decreased calcium concentration and acidosis, therefore, provide a relative stable homeostasis in infant patients.

Objective To investigate the drug resistance,source and molecular epidemiology of methicillin-resistant Staphyloccus aureus(MRSA)causing nosocomial infection. Methods Fifty-seven pathogenic MRSA strains were isolated from Beijing Tongren Hospital during 2007 and 2008.K-B method,MIC assay,multiple PCR,automatic repetitive element sequence-based PCR(REP-PCR)typing platform and DL MRSA Library were used to identify the resistant phenotypes,Panton-Valentine leukocidin gene (pvl)and REP-PCR types of the MRSA.Results All strains were classified as 6 antibiotic resistant phenotypes(a-f)based on the resistance to rifampin,clindamycin,levofloxacin and cotrimoxazole.The MRSAs with Staphylococcal cassette chromosome mec(SCCmec)Ⅲ and SCCmec Ⅱ accounted for 91.23% (52/57)and 5.26%(3/57)of all strains,respectively.Only one strain was pvl positive.All strains were typed as REP-A-F(6 types)and three single clones by automatic REP-PCR typing platform,in which REP-C was predominant(30/57,52.63%).Three out of 6 REP-D strains were from laryngology wards.The REP-C-SCCmec Ⅲ were genetically most close to the Brazilian clone-SCCmec Ⅲ in DL MRSA Library.Conclusion s REP-C-SCCmec Ⅲ-a type are the major epidemic hospital-associated MRSA and the REP-D-SCCmec Ⅲ-d is usually isolated from patients received laryngeal surgery. Automatic REP-PCR typingplatform combined with DL MRSA Library database is an effective approach to study the nosocomial infection.