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Objective:To analyze the clinical features and ultrasonographic features of Williams syndrome(WS) in children, and to explore the diagnostic value of ultrasonography in this disease.Methods:A retrospective analysis was performed on 19 children with abnormal echocardiography at Hunan Children′s Hospital from January 2016 to December 2020, and the abnormal echocardiography was evaluated by WS phenotypic scoring method of American Academy of Pediatrics.Cardiological CT and genetic tests were performed, and the clinical data and ultrasound image characteristics were analyzed.Results:The WS score of 19 cases was more than six, which was highly suspicious clinically.In 19 cases, supra-aortic stenosis and pulmonary stenosis were the most common findings on echocardiography, accounting for 63.2%(12/19), among which three cases were complicated with aortic arch constriction and one case with mitral lobe deformity.There were 15.8%(3/19)children with supra-aortic stenosis associated with pulmonary artery velocity increase, and 10.5%(2/19)children with left and right pulmonary artery branch stenosis associated with aortic inner diameter narrowing.Other cardiac malformations were found in 42.1%(8/19) of the children.Chromosome 7 microdeletions accounted for 68.4%(13/19) in genetic examination, and no abnormalities were found in three cases(15.8%, 3/19). The genetic results of three cases were still under follow-up.Conclusion:Echocardiography can accurately and sensitively detect cardiac lesions in children with WS.Combined with WS score of American Academy of Pediatrics, the diagnosis rate of WS can be improved.
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Objective:To explore the feasibility of artificial intelligence ultrasound to diagnose of biliary atresia (BA) based on deep learning.Methods:A total of 531 gallbladder ultrasound images in 177 cases of BA patients (BA group) and 585 gallbladder ultrasound images in 195 cases of Non-BA patients (Non-BA group) were collected in Hunan Children′s Hospital from September 2018 to October 2020. For the BA and Non-BA groups, all images were divided into training set and test set according to the ratio of 2∶1. The Mask R-CNN model was trained by training samples, and then the model was tested, according to patient and image as a unit respectively, to evaluate the gallbladder organ detection rate and the diagnostic accuracy of BA. In addition, the images of the test set were randomly numbered.Four sonographers were invited to interpret the images to calculate the diagnostic accuracy individually. Last, the diagnostic accuracy of the Mask R-CNN model was compared with that of sonographers.Results:In terms of the automatic detection of gallbladder organs, the detection rate in both BA and Non-BA group reached 100%, but there were 17 false alarms in 372 test images, with a false alarm rate of 4.57%. In terms of the diagnostic rate of gallbladders, when taking patient as a unit, the total diagnostic accuracy of the model in the test set was 95.97%, which was higher than that of the sonographers in other hospitals and the sonographer with intermediate professional title in our hospital, and the difference was statistically significant ( P<0.005). It was slightly higher than that of sonographer with senior professional title in our hospital (91.94%), but the difference was not statistically significant ( P=0.183). When taking picture as a unit, the total diagnostic accuracy of the model was 97.04%, which was higher than that of the sonographers in other hospitals and the sonographer with intermediate professional title in our hospital, and the difference was statistically significant ( P<0.001). It was slightly higher than that of sonographer with senior professional title in our hospital (94.09%), but the difference was not statistically significant ( P=0.05). Conclusions:The artificial intelligence technology based on Mask R-CNN can automatically and accurately detect gallbladder organs and diagnose BA, which is worthy of further study.
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Objective@#To explore the application value of pulmonary ultrasound for the treatment of severe acute respiratory distress syndrome in children with extracorporeal membrane oxygenation(ECMO).@*Methods@#Seven children with severe acute respiratory distress syndrome (ARDS) who were treated with ECMO in the PICU ward of Hunan Children Hospital from August 2018 to March 2019 underwent pulmonary ultrasound before treated with ECMO, after each bronchoscopy and lavage, before ECMO withdrawal, and within 24 hours after ECMO withdrawal. Performance of pulmonary ultrasound and clinical data were retrospectively reviewed.@*Results@#Seven patients treated with ECMO for 11.5(3-27)days, and 1 patient underwent ECMO transport. Six (86.7%) patients were successfully weaned from ECMO, and 1 (14.2%) patient failed to exit ECMO successfully. Four(57.1%) patients were discharged from hospital, and 3(42.8%) patients eventually died. In various diagnostic signs of pulmonary ultrasound were detected in 7 patients, including pulmonary edema (7 cases), lung consolidation (5 cases), followed by pleural effusion (4 cases), atelectasis (3 cases) and pneumothorax (3 cases). Pulmonary ultrasound showed that the lungs were re-expanded and suggested that the child could attempt to evacuate the ECMO.@*Conclusions@#Pulmonary ultrasound can comprehensively evaluate the various lung lesions in children with ECMO treatment, and can dynamically monitor the process of lung recruitment in real time, providing a reference for guiding the evacuation of ECMO.
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The lung ultrasound develops rapidly in recent years. We investigated the ultrasonic diag-nostic value of neonatal infectious pneumonia on the basis of our experience in diagnosing adults′ and chil-dren′s infectious pneumonia. The result confirms the reliability and effectiveness of lung ultrasound in diagno-sing neonatal infectious pneumonia. We believe that the diagnostic performance of lung ultrasound will be im-proved a lot with the popularization of lung ultrasound, in combination with the supervision of patients′con-ditions as well as routine clinical diagnosis and treatment procedures.
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Objective To explore the application value of pulmonary ultrasound for the treatment of severe acute respiratory distress syndrome in children with extracorporeal membrane oxygenation( ECM O ) . Methods Seven children with severe acute respiratory distress syndrome ( ARDS ) w ho were treated with ECM O in the PICU ward of Hunan Children Hospital from August 2018 to M arch 2019 underwent pulmonary ultrasound before treated with ECM O , after each bronchoscopy and lavage , before ECM O withdrawal ,and within 24 hours after ECM O withdrawal . Performance of pulmonary ultrasound and clinical data were retrospectively reviewed . Results Seven patients treated with ECM O for 11 .5( 3－27) days ,and 1 patient underwent ECM O transport . Six ( 86 .7% ) patients were successfully weaned from ECM O ,and 1 ( 14 .2% ) patient failed to exit ECM O successfully . Four( 57 .1% ) patients were discharged from hospital , and 3( 42 .8% ) patients eventually died . In various diagnostic signs of pulmonary ultrasound were detected in 7 patients ,including pulmonary edema ( 7 cases ) ,lung consolidation ( 5 cases ) ,followed by pleural effusion ( 4 cases) ,atelectasis ( 3 cases) and pneumothorax ( 3 cases ) . Pulmonary ultrasound showed that the lungs were re‐expanded and suggested that the child could attempt to evacuate the ECM O . Conclusions Pulmonary ultrasound can comprehensively evaluate the various lung lesions in children with ECM O treatment ,and can dynamically monitor the process of lung recruitment in real time ,providing a reference for guiding the evacuation of ECM O .
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Gene therapy is a rapidly developing field. The most widely used technique for foreign gene transfer is lentiviral-mediated gene therapy. Lentiviral vector has been developed from the first generation to the third generation in terms of safety. The preparation of lentiviruses with high titer remains difficult. In this study, a Fibra-Cel sheet carrier was used as an HEK293T cell carrier matrix, and several sterile cell culture spinners were combined and cultured on a roller bottle machine to scale up the adherent cells. The virus titer was maximized by screening the factors to optimize the lentivirus titer in the third-generation lentivirus packaging process one by one. Fibra-Cel sheet vector was successfully used as the matrix of HEK293T cell adhesion to culture adherent cells at large scale. The optimal conditions for large-scale preparation of the third-generation lentivirus by bottle roller were screened and three batches of lentiviruses were produced on pilot scale. The production time of lentivirus was shortened from 120 hours to 54 hours from plasmid transfection to virus collection; in terms of cost, a rolling bottle machine was used instead of a bioreactor, leading to lower cost and no need for repeated sterilization during the whole process. The safe, effective and low-cost operation of successful production will provide a technical base for the large-scale preparation of lentivirus and thus lay a firm foundation for its clinical application.
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Humans , Genetic Vectors , HEK293 Cells , Lentivirus , Transduction, Genetic , TransfectionABSTRACT
Objective To explore the clinical value of two-dimensional speckle tracking imaging (2DE-STI) by measuring global longitudinal strain (GLS) in normal children.Methods Totally 176 normal children underwent cardiac ultrasound examination,the left ventricular GLS were measured,and the difference of GLS between gender was analyzed.And the changes of GLS with body surface area (BSA) and age were discussed.Results GLS had no significant difference between male and female ([-24.90 ± 2.06]％ vs [-24.93 ± 2.01]％,t =0.83,P =0.934).GLS had significant differences among different BSA groups (F=3.84,P =0.003),and the differences of GLS in BSA＞ 1.0 group and the other five groups were statistically significant (all P＜0.05).GLS had statistically significant differences among different age groups (F=4.81,P=0.001),and the differences of GLS in ＞9 years old group and the other four groups were statistically significant (all P＜0.05).Conclusion Left ventricular GLS presents certain regularity with children's growth.
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Patent ductus venous is a kind of the congenital portosystemic shunts (CPSS). In recent years, the related case report increased gradually, but the domestic research is rare. In this article, we review the current diagnosis and treatment of neonatal patent ductus venous based on literature available.