Objective To analysis the imaging findings of tetralogy of Fallot and related pathological basis. Methods The clinical,pathologic and right ventriculography findings of 124 tetralogy of Fallot which were confirmed by surgery were retrospectively aralyzed. Results 124 tetralogy of Fallot were separated into type Ⅰ 62, Ⅱ 31, Ⅲ 6, Ⅳ 25 by Gray's classification,and slight,midrange,severe stenosis were 71 (0.51～0.6) ,33(0.41～0.5) ,20(<0.4) by Partiole's classcification. As to ventricular septal defect(VSD) ,infra-cristal ventricular septal defect were found in 116/124 in which crista supravcntricalaris appeared as girdle-shaped filling defect in lateral position, suprac-ristal ventricular septal defect were found in 8/124 in which crista supraventricularis appeared as round filling defect in orthophoric position. Conclusions Right ventriculography can precisely display stenosis of pulmary and ventricular septal defect(VSD). It has great valne in diagnosis and surgical therapy of tetralogy of Fallot.

Objective:To evaluate the effect of resveratrol combined with γ-ray irradiation on the biological behavior of cervical cancer cells, and to explore its possible mechanism.Methods:The proliferation of cell populations after different concentrations of resveratrol solution±γ-ray irradiation was detected by CCK-8 assay. Scratch test and Transwell chamber test were used to detect cell migration and invasion. Flow cytometry and Annexin V-FITC/PI double staining were employed to assess cell apoptosis. Western blot was performed to measure the expression levels of PI3K, Akt, p-Akt, mTOR and p-mTOR proteins.Results:Compared with the normal control (NC) group, the resveratrol group±γ-ray irradiation could inhibit the proliferation, migration, and invasion and promote cell apoptosis of human cervical cancer Hela cells, and the combined effect was more obvious. Compared with the NC group, resveratrol and γ-ray irradiation could significantly down-regulate the expression levels of Bcl-2, PI3K, p-Akt and p-mTOR proteins, up-regulate the expression level of Bax protein, but did not significantly alter the expression levels of Akt and mTOR proteins in human cervic1 255al cancer Hela cells.Conclusions:Resveratrol combined with γ-ray irradiation can dramatically inhibit the proliferation, migration, invasion, and promote the apoptosis of cervical cancer Hela cells. The mechanism may be related to the inhibition of the PI3K/Akt/mTOR signaling pathway and down-regulating the expression levels of downstream related proteins.

Objective:To analyze the characteristics of lung function in patients with spinal muscular atrophy (SMA) to provide evidence for multidisciplinary management of SMA.Methods:A total of 30 patients with SMA treated in the SMA multidisciplinary clinic of the Children′s Hospital, Zhejiang University School of Medicine from July 2019 to March 2021 were enrolled, including 1 child with type I, 18 patients with type Ⅱ and 11 children with type Ⅲ.There were 17 males and 13 females; the age ranged from 4 years to 21 years and 10 months old.A retrospective study was conducted to analyze the clinical features, spinal imaging findings and lung functions of patients with different clinical types of SMA and explore the factors influencing the lung functions of patients with SMA.Pulmonary function was measured by forced expiratory flow-volume curve.Forced vital capacity (FVC), forced expiratory volume in one second (FEV 1), FEV 1/FVC and peak expiratory flow (PEF) were measured.The results were expressed as the percentage of the measured value to predicted value.The Cobb angle was measured to evaluate scoliosis. Pearson correlation analysis and multiple linear regression analysis were used to evaluate the relationship between lung function and age and Cobb angle in patients with type Ⅱ SMA. Pearson correlation analysis and univariate linear regression analysis were used to evaluate the relationship between Cobb angle and age in patients with type Ⅱ SMA. Results:Pulmonary function in 1 type I patient showed decreased FVC and FEV 1; Among 18 patients with type Ⅱ, 14 cases had abnormal lung function (77.8%): FVC decreased in 12 patients (66.7%), FEV 1 decreased in 10 patients (55.6%), PEF decreased in 12 patients (66.7%). Among 11 patients with type Ⅲ, one had decreased FVC (9.1%). FVC, FEV 1 and PEF of patients with type Ⅱ were significantly lower than those of patients with type Ⅲ [(62.4±31.8)% vs.(90.8±11.0)%, (66.3±33.3)% vs.(97.8±9.9)%, (65.3±30.1)% vs.(98.6±21.1)%, all P<0.01]. Pearson correlation analysis showed that FVC of patients with type Ⅱ SMA was correlated with age and Cobb angle ( r=-0.864, -0.865, all P<0.001), FEV 1 was correlated with age and Cobb angle ( r=-0.878, -0.863, all P<0.001), PEF was correlated with age and Cobb angle ( r=-0.831, -0.783, all P<0.001), and Cobb angle was related to age ( r=0.922, P<0.001). Multiple linear regression analysis indicated that FVC of patients with type Ⅱ SMA was linearly correlated with Cobb angle ( R2=0.748, P<0.001), FEV 1 was linearly correlated with age ( R2=0.770, P<0.001), PEF was linearly related to age ( R2=0.690, P<0.001). Univariate linear regression analysis revealed that Cobb angle of patients with type Ⅱ SMA was linearly related to age ( R2=0.851, P<0.001). Conclusions:FVC, FEV 1 and PEF may decrease in patients with SMA.The degree of lung function damage is different in different types of SMA patients.With the increase of age, Cobb angle increases and FVC, FEV 1 and PEF decrease in patients with type Ⅱ SMA.Understanding the factors influencing the pulmonary function damage in patients with SMA is conductive to carrying out individual multidisciplinary management.

Objective:To explore the clinical efficacy of disease-modifying drug nusinersen on children with spinal muscular atrophy.Methods:The baseline and longitudinal clinical data of 15 children who were treated with nusinersen in the Children′s Hospital, Zhejiang University School of Medicine from October 2019 to October 2021 were retrospectively collected. The general data (gender, age, genotype, and clinical classification, etc.), motor function, nutritional status, scoliosis and respiratory function were analyzed. Wilcoxon rank-sum test was used for comparing multi-system conditions before and after treatment.Results:The age of 15 cases (7 males, 8 females) was 6.8 (2.8, 8.3) years, with 2 cases of type 1, 6 cases of type 2, and 7 cases of type 3 respectively, and the course of disease was 55.0 (21.0, 69.0) months. After 9.0 (9.0, 24.0) months of treatment, the motor function scale evaluations of the Hammersmith neurological examination section 2 (13.0 (7.0, 23.0) vs. 18.0 (10.0, 25.0) scores, Z=-2.67, P=0.018) of 15 children, the Hammersmith functional motor scale expanded (38.0 (18.5, 45.5) vs. 42.0 (23.0, 51.0) scores, Z=-2.38, P=0.018), and the revised upper limb module (27.0 (19.5, 32.0) vs. 33.0 (22.5, 35.5) scores, Z=-2.52, P=0.012) of children with type 2 and 3 had significantly improved. Thirteen patients achieved clinically significant motor function improvement, and 2 of them had kept stable scale scores. Subjective reports also indicated that the muscle strength and daily exercise ability of these children improved after treatment, and no serious adverse reactions were reported. Supplemented by the multi-disciplinary team management, the levels of some indicators such as Cobbs angle of scoliosis and forced vital capacity all had significantly improved (all P<0.05). Conclusions:Nusinersen can improve the motor function of patients with 5q spinal muscular atrophy, which is also proved safe to be used in children. The drug treatment supplemented by the multi-disciplinary team management is helpful to improve the multi-system function of the children with spinal muscular atrophy.

OBJECTIVE：To study the co rrelation of the chro maticity value of Schizonepeta tenuifolia charcoal of different processing time（0-40 min，similarly herein after）with multiple indicators ，and to reveal the quality change law of S. tenuifolia charcoal during processing and confirm the terminal time. METHODS ：The contents of ethanol-soluble extracts from S. tenuifolia charcoal decoction pieces of different processing time were determined. UPLC fingerprint of S. tenuifolia decoction pieces and S. tenuifolia charcoal decoction pieces of different processing time were established ，and the similarity evaluation was also conducted. The chromatographic peaks were confirmed by comparison with substance control. The same UPLC conditions were used to determine the contents of index components （hesperidin，rosmarinic acid ，menthone）in S. tenuifolia charcoal decoction pieces of different processing time. The colorimetric method was used to measure the chromaticity value of S. tenuifolia charcoal decoction pieces of different processing time. Meanwhile ，sample of processing 0 min was used as a control to calculate the total color value （E）and the total color difference value （ΔE）. Pearson correlation analysis ，cluster analysis and orthogonal partial least squares discriminant analysis （OPLS-DA）were performed on the ethanol-soluble extracts ，index component contents ，chromatographic peak area and chromaticity value. The terminal time of processing S. tenuifolia charcoal was conf irmed，and validation test was also conducted. RESULTS ：With the extension of processing time ， the content of ethanol-soluble extract in S. tenuifolia charcoal qq.com decoction pieces gradually decreased. A total of 17 chromato- graphic peaks were identified in 12 batches of S. tenuifolia decoction piece ，and its si milarity with the control fingerprint was greater than 0.9. 21 chromatographic peaks were identified in S. tenuifolia charcoal decoction pieces of different processing time，and its similarity with the chromatogram of sample of processing 0 min decreased with the processing time ，and the similarity after 18 min was lower than 0.9. The chromatographic peak 9 was hesperidin ，peak 10 was rosmarinic acid and peak 17 was menthone. The determination of content and chromaticity value showed that with the extension of processing time ，the contents of hesperidin ，rosmarinic acid and menthone decreased gradually ；the color L，b and E values of S. tenuifolia charcoal decoction piece powder decreased gradually ，and the a and ΔE values increased gradually. Pearson correlation analysis showed that the contents of ethanol-soluble extract ，hesperidin，rosmarinic acid and menthone ，the peak areas of 15 chromatographic peaks （peak 2，7-15，17-21）were significantly positively correlated with E value（P＜0.01）；the peak areas of 5 chromatographic peaks （peak 1，3-6）were significantly negatively correlated with E value（P＜0.01），but peak area of peak 16 was not related to E value（P＞ 0.05）. Results of cluster analysis showed that S. tenuifolia charcoal decoction pieces of different processing time were divided into 2 categories；the first category was processed for 0-16 min，and the second category was processed for 18-40 min. The results of OPLS-DA showed that the VIP values of peak 6 area（2.800 75），L value（2.327 54），peak 3 area（1.793 39），b value（1.735 78） and peak 5 area（1.244 04）were greater than 1. The final processing time of S. tenuifolia charcoal was 18 min. The results of validation experiment showed that the L，a and b values of S. tenuifolia charcoal decoction piece were 20.22-22.00，4.44-7.67， 9.78-13.00，and ΔE were 13.50-14.12，respectively. CONCLUSIONS ：The chromaticity value of S. tenuifolia charcoal decoction pieces of different processing time is closely related to the contents of ethanol-soluble extract ，hesperidin，rosmarinic acid ， menthone and the area of 20 chromatographic peaks. It is suggested that the terminal time of processing S. tenuifolia is 18 min.