Objective To observe the changes of Akt1,Akt2,Akt3 mRNA expression level in rat pulmonary arterial smooth muscle cells(PASMC) induced by hypoxia and to investigate the value of Akt signaling pathway in vascular reconstruction during phyoxia pulmonary hypertension(HPH).Methods The pure PASMCs were established by tissue-sticking methods.Semi-quantitative reverse transcription polymerase chain reaction(RT-PCR) were used to examine the Akt1,Akt2 and Akt3 mRNA expression after PASMCs were exposed to normoxia(21%O_2) or hypoxia(3%O_2) for 2,8,12 and 24 h respectively.Results The pure PASMCs were established.The mRNA of Akt1,Akt2,Akt3 could be detected in all groups.Exposed to hypoxia,the mRNA expression levels gradually increased,reached the peak at 8 h,then decreased,and at 24 h down to the level under normoxia.As compared with that exposed to normoxia,the mRNA expression levels of Akt1 at 2,8,12,24 h,Akt2 at 8 h,Akt3 at 8 h in PASMCs exposed to hypoxia showed significant difference(P

Objective To study the drifting law of floats and potential risks of Oncomelania hupensis diffusion in the water diversion rivers of the east route of South?to?North Water Diversion Project. Methods The O. hupensis snails in the river chan?nels were monitored by the salvage method and snail luring method with rice straw curtains,and the diffusion possibility of snails along with water was assessed through the drift test of floats with GPS. Results In the flood seasons from 2006 to 2013, totally 8 338.0 kg of floats were salvaged,and 2 100 rice straw curtains were put into water in the Li Canal and Jinbao shipping channel,but no Oncomelania snails were found. The drift test of floats with GPS before water diversion showed that the flow ve?locity on water surface(northbound)was 0.45 m/s,the average drift velocity of the floats was 0.56-0.60 m/s,and the average drift distances each time were 999.70- 1 995.50 m in the Gaoshui River section,while there were no obvious drift in Jinbao shipping channel section. During the water diversion period,the flow velocity on water surface(northbound)was 0.45 m/s,the average drift velocity of the floats was 0.35-0.41m/s,and the average drift distances each time were 1 248.06-1 289.44 m in the Gaoshui River,while in Jinbao shipping channel section,the flow velocity on water surface was 0.28 m/s,the average drift velocity of the floats was 0.25- 0.27 m/s,and the average drift distances each time were 477.76- 496.38 m. The drift test showed that the floats gradually closed to the river bank as affected by water flow,wind direction and ship waves,when blocked by the reeds,water plants or other obstacles,and they would stopped and could not continue to drift without outside help. Con?clusions There are no Oncomelania snails found in the river channels of the east route of South?to?North Water Diversion Proj?ect. The drifting distance of the floating debris along with the water is restricted by the flow rate and shore environment.

Objective:To investigate the short-term and medium-term changes of the left ventricular ejection fraction (LVEF) and the predictive value of relevant electrocardiogram (ECG) indexes in children with dilated cardiomyopathy (DCM) complicated with complete left bundle branch block (CLBBB).Methods:Children clinically diagnosed with DCM in the Department of Heart Center, Women and Children′s Hospital, Qingdao University and Beijing Anzhen Hospital, Capital Medical University between November 2011 and August 2020 were retrospectively recruited.According to the combination of CLBBB, they were divided into CLBBB group and non-CLBBB group.Echocardiogram and ECG were regularly performed.Short-term and medium-term changes of LVEF based on the 1-5-year follow-up data were compared between groups.COX proportional hazards model and Kaplan-Meier multiplicative limit method were used to analyze the predictive value of ECG indexes of LVEF changes in children with DCM combined with CLBBB.Results:Ninety-four children with DCM were enrolled, including 35 cases in CLBBB group and 59 cases in non-CLBBB group.There was no difference in baseline LVEF between groups.However, significant differences were found in QRS duration, corre-cted QT interval(QTc), R peak time in lead V 5 (T V5R) and QRS notching or slurring between groups ( P<0.05). LVEF of all children showed an upward trend within one year after onset, while the Z value of eft ventricular end diastolic diameter(LVEDd) showed a downward trend, and the two indexes tended to be stable within 1 - 5 years.The Z value of LVEDd in CLBBB group was significantly higher than that of non-CLBBB group, while LVEF was significantly lower (all P<0.05). The mean LVEF of CLBBB group slightly fluctuated around 50%, that of LVEF in non-CLBBB group was 60%.The multivariate COX regression analysis showed that QRS duration ( HR=0.979; 95% CI: 0.960-0.999, P<0.05) and QTc ( HR=0.988; 95% CI: 0.979-0.998, P<0.05) were independent predictors of LVEF recovery in children with DCM.Kaplan-Meier method showed a significant difference of LVEF normalization between DCM children with different QRS durations ( P<0.05), which was also detected in those with QTc interval ( P<0.05). Conclusions:LVEF of children with DCM combined with CLBBB increases in the short term after standard treatment, and then being stable.CLBBB can affect the recovery of left ventricular systolic function in children with DCM.Moreover, QRS duration and QTc interval are independent predictors of LVEF recovery in DCM children.

Objective:To summarize the clinical features, gene mutations and experience of standardized enzyme replacement therapy (ERT) of Pompe disease (PD) in children.Methods:A retrospective analysis was performed on the clinical data of 13 children with PD, who were hospitalized in Qingdao Women and Children′s Hospital from December 2016 to August 2021.According to the age at onset, the children were divided into the infantile-onset Pompe disease (IOPD) group and late-onset Pompe disease (LOPD) group.At the same time, they were divided into the ERT group and non-ERT group according to whether recombinant human acid alpha-glucosidase (rhGAA) was infused.Furthermore, the ERT group was divided into the standard ERT group and non-standard ERT group.The standard ERT group received a dose of 20 mg/kg every 2 weeks for 52 weeks.The survival rate was compared between groups by using the Kaplan-Meier method.Results:Among the 13 children with PD, there were 7 males and 6 females.Ten cases belonged to the IOPD group and 3 cases belonged to the LOPD group.The most common cause of initial consultation in the IOPD group was cardiac involvement, which accounted for 60.0% (6/10 cases), while the LOPD group mainly presented with myasthenia, cardiac involvement and respiratory tract infection at the first diagnosis.The serum level of creatine kinase (CK) in all cases increased to varying degrees.Acid alpha-glucosidase (GAA) was completely deficient in 1 case and decreased in 12 cases.All the children in the IOPD group showed myocardial hypertrophy, electrocardiograph (ECG) suggested a short PR interval, increased QRS voltage and extensive T-wave inversion.Three new mutations were found by GAA gene analysis, and they were c. 1861T>G (p.Trp621Gly), c.2278A>T (p.K760X), and c. 949G>A (p.A317T). Five cases in the IOPD group were given ERT.Two of them were given standard ERT for 52 weeks, and the other 3 cases were treated with non-standard ERT.At the end of follow-up, 2 cases treated with standardized ERT survived and the remaining 8 cases died of heart failure or respiratory failure.In the LOPD group, only 1 case was given ERT one time.Finally, 2 cases survived and one died of respiratory failure.The total fatality rate was 69.2%(9/13 cases). The survival rate of the ERT group (50.0%) and standard ERT group (100.0%) was significantly higher than that of the non-ERT group (14.3%) ( Log Rank P=0.037, 0.044). Conclusions:The clinical manifestations of PD are diverse.GAA activity examination and GAA gene analysis are important for clinical diagnosis of PD.Standardized ERT can significantly delay the progression of PD and even reverse myocardial hypertrophy in children with IOPD.

OBJECTIVE：To investigate inhibitory effects of Qing fei baoyu an capsule on airway inflammation in rats with chronic obstructive pulmonary disease （COPD），and its effects on NLRP 3 signaling pathway. METHODS ：Totally 60 SD male rats were randomly divided into blank control group ，model group ，dexamethasone group （positive control ，0.2 mg/kg），Qingfei baoyuan capsule high-dose ，medium-dose and low-dose groups （1 232.0，616.0，308.0 mg/kg），with 10 rats in each group. Except for blank control group ，other groups were fumigated for 28 days and given intratracheal dripping of lipopolysaccharide twice to induce COPD model. Since the 29th day after modeling ，blank control group and model group were given constant volume of normal saline intragastrically ，and administration groups were given related medicine intragastrically. The administration volume was 10 mL/kg，once a day ，for consecutive 28 days. After last administration ，the lung function was detected. The pathological changes of lung tissue were observed by HE staining. The content of interleukin- 1β（IL-1β）in bronchoalveolar lavage fluid （BALF）were detected by ELISA ，and the number of leukocytes was counted ；the expression of NLRP 3 and Cleaved caspase- 1 in lung tissue of rats were detected by Western blotting assay. RESULTS：Three，two，one，one and two rats died in model group， dexamethasone group ， Qingfei baoyuan capsule high-dose，medium-dose and low-dose groups ，respectively. Compared with blank control group ，FEV0.3/FVC of rats in # model group was significantly decreased （P＜0.01）. A large number of inflammatory cells infiltration we re found in the lung tissue ，and lung tissue lesion was obvious. The content of IL- 1β and white blood cell count in BALF，relative expression of NLRP3 and Cleaved caspase- 1 protein in lung tissue were increased significantly （P＜0.05 or P＜0.01）. Compared with model group，FEV0.3/FVC of administration groups were increased significantly （P＜0.05 or P＜0.01）；lung tissue lesion of them were improved to different extents. The content of IL- 1β and white cell count in BALF，relative expression of NLRP 3 protein（except for Qingfei baoyuan capsule low-dose group ）and Cleaved caspase- 1 protein（except for Qingfei baoyuan capsule medium-dose and low-dose groups ）in lung tissue were decreased significantly （P＜0.05 or P＜0.01）. CONCLUSIONS ：Qingfei baoyuan capsule can relieve lung tissue lesion and improve lung function in COPD model rats ，the effects of which may be associated with inhibiting inflammation reaction by inhibiting NLRP 3 signaling pathway.