Objective:To further improve the understanding of paroxysmal nocturnal hemoglobinuria (PNH), we retrospectively analyzed and summarized the clinical characteristics, treatment status, and survival status of patients with PNH in Zhejiang Province.Methods:This study included 289 patients with PNH who visited 20 hospitals in Zhejiang Province. Their clinical characteristics, comorbidity, laboratory test results, and medications were analyzed and summarized.Results:Among the 289 patients with PNH, 148 males and 141 females, with a median onset age of 45 (16-87) years and a peak onset age of 20-49 years (57.8% ). The median lactic dehydrogenase (LDH) level was 1 142 (604-1 925) U/L. Classified by type, 70.9% (166/234) were classical, 24.4% (57/234) were PNH/bone marrow failure (BMF), and 4.7% (11/234) were subclinical. The main clinical manifestations included fatigue or weakness (80.8%, 235/289), dizziness (73.4%, 212/289), darkened urine color (66.2%, 179/272), and jaundice (46.2%, 126/270). Common comorbidities were hemoglobinuria (58.7% ), renal dysfunction (17.6% ), and thrombosis (15.0% ). Moreover, 82.3% of the patients received glucocorticoid therapy, 70.9% required blood transfusion, 30.7% used immunosuppressive agents, 13.8% received anticoagulant therapy, and 6.3% received allogeneic hematopoietic stem cell transplantation. The 10-year overall survival (OS) rate was 84.4% (95% CI 78.0% -91.3% ) . Conclusion:Patients with PNH are more common in young and middle-aged people, with a similar incidence rate between men and women. Common clinical manifestations include fatigue, hemoglobinuria, jaundice, renal dysfunction, and recurrent thrombosis. The 10-year OS of this group is similar to reports from other centers in China.

Herpes simplex virus (HSV), the main pathogen of human herpetic diseases, is an attractive viral vector due to a wide range of host cell types and a large capacity of foreign gene. The manipulation on herpes simplex virus genome, up to 152 kb, has been the key process and has undergone the improvement from homologous recombination to bacterial artificial chromosome (BAC) and CRISPR/Cas9. However, homologous recombination is imperfect because the efficiency of recombination is relatively low and the product is too complex to purify. Construction of recombinant HSV based on BAC is time-and labor-consuming. Currently, CRISPR/Cas9 has been applied to genetic engineering of various species by more and more researchers due to its convenience, good repeatability, low cost, and then the manipulation of viral genome is more accurate and efficient. Application of CRISPR/Cas9 in the research of HSV gene function, HSV infection therapy and viral vector were summarized in this review, in order to provide reference for corresponding research.

Objective: To identify the pathophysiological characteristics of cortical stroke survivors′ swallowing. Methods: Sixty cortical stroke survivors with dysphagia and cognitive impairment were enrolled into the observation group, while another 16 with dysphagia but without cognitive impairment formed the unimpaired control group and 16 healthy counterparts were selected for a normal control group. Each subject was recorded videofluoroscopically while swallowing 5ml of a liquid of medium consistency. The occurrence of refusing to eat, mouth opening difficulty, incomplete oral closure, residue in the oral cavity, residue in the pharyngeal cavity, leakage and aspiration were observed. Each subject′s swallowing time and kinematic parameters were analyzed from the fluoroscopic videos. Results: The incidence of refusing to eat (37.5%) and/or incomplete mouth closure (68.75%) were significantly higher in the observation group than in the other two groups. The incidence of difficulty in opening the mouth (37.5%), residue in the oral cavity (81.25%), residue in the pharyngeal cavity (56.25%), leakage (56.25%) and aspiration (50%) of the observation group were significantly higher in the observation group than among the normal controls, but were not significantly different from those incidences among the group without cognitive impairment. The average oral transit time and soft palate elevation time of the observation group were significantly longer than those of the other two groups. The observation group′s average hyoid movement time was significantly longer than that of the normal control group, but not significantly different from that of the group without cognitive impairment. There were no significant differences among the groups in average upper esophageal sphincter opening time, larynx closure time or the kinematic parameters. Conclusions Stroke survivors with dysphagia and cognitive impairment present dysphagia characteristic of oral phase swallowing difficulties.

Ohjective To explore the impact of constraint-induced aphasia therapy (CIAT) on language function and neural activity in patients with chronic Broca's aphasia.Methods Two chronic aphasics whose use of language was recovering after standard language therapy were selected to receive 1.5 h of CIAT twice daily for two weeks (30 hours in total).Before and after the CIAT they were tested using a block-designed picture-naming task,fMRIs were taken and their use of language was examined.Results The language function assessments showed relatively large improvements in the subjects' use of language after 4 weeks of conventional language training,but not much further change after 8 and 12 weeks.After the two weeks of CIAT,their language function improved further to a certain extent.fMRI showed increased activation in the left inferior frontal gyrus with or without enhanced activation in the left hemisphere,and reduced activation in the right inferior frontal gyrus.Conclusions Functional reorganization induced in the brain by CIAT was associated with up-regulation of the left inferior frontal gyrus or down-regulation of the right inferior frontal gyrus.Even the whole language network may have been modified.

Background: It remains controversial whether palliative primary tumor resection (PPTR) can provide survival benefits to the patients with metastatic colorectal cancer (mCRC) who have unresectable metastases. The aim of this study was to evaluate whether PPTR could improve the survival of patients with mCRC. Methods: We conducted a retrospective study on consecutive mCRC patients with unresectable metastases who were diagnosed at Sun Yat?sen University Cancer Center in Guangzhou, Guangdong, China, between January 2005 and December 2012. Overall survival (OS) and progression?free survival (PFS) after first?line chemotherapy failure were compared between the PPTR and non?PPTR patient groups. Results: A total of 387 patients were identified, including 254 who underwent PPTR and 133 who did not. The median OS of the PPTR and non?PPTR groups was 20.8 and 14.8 months (P < 0.001), respectively. The median PFS after first?line chemotherapy was 7.3 and 4.8 months (P < 0.001) in the PPTR and non?PPTR groups, respectively. A larger proportion of patients in the PPTR group (219 of 254, 86.2%) showed local progression compared with that of patients in the non?PPTR group (95 of 133, 71.4%; P < 0.001). Only patients with normal lactate dehydrogenase (LDH) levels and with carcinoembryonic antigen (CEA) levels <70 ng/mL benefited from PPTR (median OS, 22.2 months for the PPTR group and 16.2 months for the non?PPTR group; P < 0.001). Conclusions: For mCRC patients with unresectable metastases, PPTR can improve OS and PFS after first?line chemo?therapy and decrease the incidence of new organ involvement. However, PPTR should be recommended only for patients with normal LDH levels and with CEA levels <70 ng/mL.

binding,cellular organelle membrane,and cellular metabolic process of GO enrichment.For the KEGG pathways, the target genes mainly concentrated on the focal adhesion pathway.Conclusion There is a different expression of novel microRNA between SLE and NC groups.The target genes from differently-expressed novel microRNA may play an important role in the pathogenesis of SLE and clinical symptoms and may be the unique target for further research.

Objective To evaluate the activation patterns in the cortexes of expressive aphasics after stroke so as to explore the pathogenic mechanism of expressive aphasia.Methods Blood oxygen level dependent functional magnetic resonance imaging (BOLD-fMRI) was the method of choice.It was administered to 9 subjects with expressive aphasia at 1 to 3 months post-stroke onset and to 10 healthy controls.Active areas in the patients' brains were observed using a block-designed picture-naming task,and language function was tested with the China Rehabilitation Research Center's aphasia examination (CRRCAE).The control group received BOLD-fMRI only.SPM8 software was used to process the fMRI data.Results Differences were observed in the mapping of activated areas between the two groups,but many activated areas showed no difference.Significant differences in activation were observed in areas associated with vision,language and cognition,including the bilateral inferior frontal gyrus,the bilateral superior temporal gyrus,the bilateral insula,the bilateral basal ganglia,the left superior frontal gyrus,the left middle frontal gyrus,the left precentral gyrus,the left thalamus,and the left middle temporal gyrus.All the patients had activated cortex regions associated with visual processing in the left and/or right hemisphere,such as the middle frontal gyrus,the middle temporal gyrus,the lingual gyrus and the fusiform gyrus.The activation volumes in the left hemisphere were significantly smaller than those in normal adults.Regions related to language such as the left inferior frontal gyrus (Broca's area),the left middle frontal gyrus,and the right inferior frontal gyrus (the mirror region of Broca's area) were activated in some of them.While the activation frequency,activated volume and activation intensity generally were all less in the patients than in the controls,the activation intensity in the right superior temporal gyrus,the bilateral superior parietal lobule and the left inferior temporal gyrus were stronger.Conclusions Language production may be associated with multiple,interconnected regions.The right hemisphere participates in natural language processing.Aphasia damages both linguistic and cognitive areas,reducing activation in Broca's aphasia.Activation areas in the left hemisphere and the right inferior frontal gyrus decrease significantly,while some regions in the right hemisphere are relatively more activated.The right inferior frontal gyrus may play a different role in language recovery at different periods of aphasia after stroke.

Objective To screen the factors that affect severity of language disorder in subcortex aphasia, and analyze its characteristics and therapeutic effect. Methods 10 moderate degree and 12 severe degree subcortex aphasics were evaluated with Chinese Rehabilitation Research Center Aphasia Examination (CRRCAE) and the Boston Diagnostic Aphasia Examination (BDAE) before and 4 weeks after language training. The factors affecting severity of subcortex aphasia and characteristics were analyzed before training, and effect of language training was examined before and after training. Results Severity of subcortex aphasia was related to age and the complication of apraxia of speech. There was a relatively high level in listening comprehension, reading, repeating and reading aloud abilities. After training, all kinds of language abilities improved significantly in moderate degree subcortex aphasics. Whereas, comprehension and verbal communication capabilities increased obviously, but written communication abilities advanced rarely in severe degree subcortex aphasics. Conclusion Age and the complication of aphasia of speech may be important factors influencing severity of language disorder in subcortex aphasia. There are specific clinical features in subcortex aphasia. Almost all language abilities can be improved by language training, but therapeutic effect is differrent between moderate degree and severe degree subcortex aphasics.

ObjectiveTo study the effects and feasibility of Smart Equitest Balance Master training for severe balance disorder caused by cerebellar hemorrhage in children. MethodsA 10-year-old boy with severe ataxia caused by cerebellar hemorrhage were trained with Smart Equitest Balance Master. He was assessed with Smart Equitest Balance Master, Modified Barthel Index and Berg Balance Scale. ResultsAfter 22 weeks of balance training, he improved in equilibrium, gait, posture control, activity of daily living, dysarthria, and return to school to continue his study. ConclusionSmart Equitest Balance Master training can significantly improve balance function，motor function and activities of daily living after cerebellar hemorrhage.

Objective To investigate the efficacy of two different methods of catheter balloon dilatation therapy in treating cricopharyngeal achalasia and benign strictures,and to correlate balloon capacity with improve-ments in swallowing function. Methods Twenty-three cricopharyngeal achalasia patients and 7 with benign stric-tures of the cricopharyngeal muscles were diagnosed using videonuoroscopic swallowing study(VFSS).All cases re-ceived active dilatation or passive balloon dilatation therapy,combined with manipulation,indirect training,direct training and feeding instruction.Efficacy and the correlation were evaluated using VFSS and a swallowing function scale. Results Passive and active dilatation had the same efficacy.The correlation coefficient between balloon ca-pacity and swallowing function was 0.92 1.Re-evaluation using VFSS showed that opening of the cricopharyngeal muscles had improved significantly.Conclusions Balloon catheter dilation is an effective treatment for cricopha-ryngeal achalasia and benign strictures.The improvement in swallowing function is highly correlated with balloon ca-pacity in balloon dilatation therapy.Comprehensive treatment for dysphagia would provide better outcomes.