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Article in Chinese | WPRIM | ID: wpr-817912


OBJECTIVE: To summarize the clinical features of spinal muscular atrophy complicated with pulmonary infection in children,and to improve clinicians' understanding of the disease and improve the prognosis. METHODS: The clinical data of 36 children with SMA complicated with pulmonary infection,who were admitted to Yuying Children's Hospital of the Second Affiliated Hospital of Wenzhou Medical University from January 1,2008 to December 31,2017,were retrospectively analyzed. RESULTS: Among of the 36 patients,19 were typeⅠ,9 were typeⅡ,and 8 were typeⅢ. The common clinical manifestations and signs were fever,cough,shortness of breath,laborious breathing,three-concave signs,and crackles in the lungs. Respiratory failure occurred in 11 children,including 7 children(63.6%)with typeⅠSMA,2 children(18.2%)with typeⅡ SMA and 2 children(18.2%)with typeⅢ SMA. Imaging findings showed 5 cases of scoliosis,3 cases being typeⅡ SMA,and 2 being typeⅢ SMA. Pathogenic tests were positive in 18 children:10 cases(55.5%)of type Ⅰ SMA,4 cases(22.2%)of typeⅡ SMA,4 cases(22.2%)of typeⅢ SMA;nosocomial mixed infection with conditioned pathogens was common,among which Burkholderia cepacia was the most common. Three patients died in the hospital,22 patients improved and discharged,and the remaining 11 patients gave up treatment. The number of hospitalizations,the incidence of severe pneumonia and respiratory failure was significantly different between the first 5 years(2008-2012)and the last 5 years(2013-2018)(P<0.05). CONCLUSION: SMA is verysusceptible to pulmonary infection. We should be alert to opportunistic pathogenic bacteria infection and use mechanical ventilation in time for respiratory failure patients. Active and effective respiratory care can reduce the incidence of pulmonary infection and improve the prognosis of SMA children.

Article in Chinese | WPRIM | ID: wpr-776519


OBJECTIVE@#To investigate the effects of optical genetic techniques on new neurons through the Wnt/β-Catenin pathway.@*METHODS@#Neural stem cells (ESCs)were extracted from the cerebral cortex of fetal rat and transfected by lentivirus carrying DCX-ChR2-EGFP gene and the expression of DCX of newborn neurons differentiated from neural stem cells were observed. All cells were divided into 3 groups(n=9): control group, NSCs+EGFP and NSCs+ChR2 groups. The control group was normal cultured NSCs (NSCs group); the neural stem cells in NSCs+EGFP group were transfected with lentivirus carrying EGFP gene. The neural stem cells in NSCs+ChR2 group were infected with lentivirus carrying DCX-ChR2-EGFP gene. After 48 hours of lentivirus infection, 470 nm blue laser irradiation was performed for 3 consecutive days. NeuN positive cell density(the maturation of neural stem cells)and the ratio of NeuN/Hoechst in each group were observed. Western blot was used to detect the expression levels of MAP2, NeuN, Neurog2, NeuroD1 and GluR2. Western blot was used to detect the expressions of β-catenin and TCF4 associated with Wnt/β-catenin signaling channel. Verapamil (100 μmol/L, L-type calcium channel blockers) and Dkk1 (50 μg/ml, β-catenin inhibitor) were used to treat stem cells of the NSCs+ChR2 group and then the expressions of MAP2, NeuN, Neurog2, NeuroD1 and GluR were detected by Western blot.@*RESULTS@#After 3 days of 470 nm blue laser irradiation, NeuN positive cell density(the maturation of neural stem cells)and the ratio of NeuN/Hoechst, the expression levels of the protein MAP2, NeuN, Neurog2, NeuroD1, GluR and the protein β-catenin and TCF4 associated with Wnt/β-catenin signaling channel detected by Western blot were significantly increased in the group of NSCs+ChR2, compared with NSCs and NSCs+EGFP groups. The expressions of MAP2, NeuN, Neurog2, NeuroD1 and GluR were remarkably decreased after treated by verapamil and Dkk1 in the group of NSCs+ChR2. It was proved that the opening of ChR2 channel producing cationic influx promoted the maturation of neural stem cells and induced by the Wnt/β-catenin signaling pathway.@*CONCLUSION@#Optical genetic promoted the maturation of newborn neurons through the Wnt/β-catenin signaling pathway.

Animals , Cells, Cultured , Neural Stem Cells , Cell Biology , Neurons , Cell Biology , Optogenetics , Rats , Transfection , Wnt Signaling Pathway