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        find Keyword "congenital" 113 results
        • Minimally invasive techniques for treatment of special cardiac malformations

          ObjectiveTo share the experience of treating special cardiac malformations by applying minimally invasive techniques.MethodsEight children with special cardiac malformations admitted to our hospital from July 2014 to September 2020 were recruited, including 3 males and 5 females, aged 0.8-1.2 (1.1±0.4) years, and weighted 7.8-11.5 (9.6±2.9) kg. There were 2 patients of huge muscular ventricular septal defect (VSD), 3 perimembranous cribriform VSD, 1 right coronary-right atrial fistula, 1 right coronary-right ventricular fistula, and 1 young, low-weight child with large aortopulmonary. All were treated with minimally invasive techniques using transesophageal echocardiography (TEE) as a guiding tool. All children received intraoperative TEE immediately to evaluate the curative effect of the surgery, and all went to outpatient clinic for reexamination of echocardiography, electrocardiogram and chest X-ray after discharge.ResultsEight children underwent minimally invasive surgery successfully without any incision infection, intracardiac infection, arrhythmia or pericardial effusion. None of the 8 children were lost to follow-up, and the results of all reexaminations were satisfactory.ConclusionThe application of minimally invasive techniques is a bold and innovative attempt for the treatment of a few special types of cardiac malformations. It has significant advantages in reducing trauma and medical costs in some suitable patients, and has certain clinical reference values.

          Release date:2022-12-28 06:02 Export PDF Favorites Scan
        • Application of amniotic membrane-living skin equivalent in repairing skin defect after removal of congenital giant nevus

          Objective To investigate the feasibility of human amniotic membrane-living skin equivalent (AM-LSE) in repairing the skin defect. Methods A 5-year-old boy with giant nevus at neck, shoulder, and back was admitted in July 2016. Normal skin tissue of the patient was harvested and keratinocytes and dermal fibroblasts were separated and expanded in vitro. Human AM was donated from a normal delivery and de-epithelialized for constructing an LSE as a matrix. Keratinocytes were seeded on the epithelial side of the AM which was previously seeded with fibroblasts on the stromal side and then the complex was lifted for air-liquid surface cultivation for 10 days and observed under naked eyes and sampled for histological study. The nevus was excised to deep fascia and the skin defect in size of 20 cm×15 cm was covered with artificial skin of collagen sponge for 2 weeks to enhance granulation tissue formation, and then the AM-LSE grafts of stamp size were grafted on. The dressing was changed until the wound healed. Results After 10 days of air-liquid surface cultivation, the AM-LSE developed a multilayered and differentiated epidermis with the fibroblasts-populated amnion as the dermal matrix. The LSE stamps survived and expanded to cover the whole wound. The grafted area showed normal skin color and soft contexture at 6 months after operation, and histological study showed well developed epidermis with compactly aligned basal cells, stratified and well differentiated squamous, granular layers and stratum corneum and well vascularized dermal compartment without inflammatory cells infiltration. Conclusion The cultivated AM-LSE with autologous cells can repair skin defect and survive for a long term without rejection.

          Release date:2017-12-11 12:15 Export PDF Favorites Scan
        • Transcatheter closure of congenital heart disease under the guidance of transthoracic echocardiography

          Objective To explore the feasibility of transcatheter closure of congenital heart disease (CHD) under the guidance of transthoracic echocardiography (TTE). Methods A total of 37 patients with CHD who received transcatheter closure under the guidance of transthoracic echocardiography from November 2013 through November 2015 in our hospital were recruited. There were 15 males and 22 females, aged 1 to 16 years. Among them 32 patients suffered atrial septal defect and 5 patients had patent ductus arteriosus. The transcatheter closure of CHD was performed under the guidance of TTE. The patients underwent echocardiography follow-up at one, three and six months after surgery. Results Closure devices were successfully implanted in 37 patients under TTE guidance. The procedure was simple and safe. During the follow-up, no severe complication such as valvular injury, pericardial effusion, residual shunt and peripheral vascular injury occurred. Conclusion Transcatheter closure of CHD under TTE guidance is a feasible method and worth further clinical application.

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        • Sex hormone levels have little impact on outcomes of cardiac surgery in fertile women with congenital heart disease: A retrospective cohort study

          Objective To examine the influence of hormonal fluctuations on the perioperative outcomes of patients undergoing congenital heart surgery. Methods We conducted a retrospective analysis of clinical data from fertile women diagnosed with congenital heart disease at the Guangdong Provincial People's Hospital, between January 1, 2015, and July 30, 2019. Initially, patients were categorized into groups based on serum progesterone levels: a low progesterone group (n=31) and a high progesterone group (n=153). Furthermore, based on serum estrogen levels, they were divided into a low estrogen group (n=10), a medium estrogen group (n=32), and a high estrogen group (n=118) for comparative analysis. A control group (n=24) consisted of patients who received progesterone injections before their menstrual period. Results We finally included 184 patients. The patients’ average age was 27.6±5.7 years, with 142 (77.17%) presenting with complex congenital heart conditions. There were statistically significant differences in total postoperative standard thoracic drainage volume and postoperative albumin level between the high and low progesterone groups (P<0.05), while other perioperative outcome indicators showed no statistical differences (P>0.05). Among the different serum estrogen level groups, there were statistically significant differences in postoperative blood urea nitrogen levels, total postoperative standard thoracic drainage volume, and hospital stay (P<0.05), while other perioperative outcome indicators showed no statistical differences (P>0.05). ConclusionConsidering the overall clinical significance, the physiological changes in sex hormone levels appear to have a negligible effect on the perioperative outcomes of fertile women with congenital heart disease.

          Release date:2024-05-28 03:37 Export PDF Favorites Scan
        • Analysis of dominant views in ultrasound examination of common congenital heart diseases in children

          Objective To explore the dominant views with positive results when performing echocardiography on common congenital heart diseases (CHD) in children using the "Seven-Step Screening Method for Pediatric Echocardiography". MethodsThe echocardiographic data of children with atrial septal defect, patent foramen ovale, ventricular septal defect, and patent ductus arteriosus were collected from September 2021 to February 2022. The disease type distribution, view distribution, and the dominant view distribution were analyzed. Dominant view refered to the view with a high ratio of positive result images per view to the total image in each disease. Results A total of 8 353 images of 1 633 children with common CHD were collected. There were 813 males and 820 females at age of 0-7 years. Including 3 613 images in 701 patients with atrial septal defect, 1 178 images in 206 patients with patent foramen ovale, 2 857 images in 576 patients with ventricular septal defect, and 705 images in 150 patients with patent ductus arteriosus. The dominant views of atrial septal defect were subxiphoid 2-chamber view (92.96%), subxiphoid 4-chamber view (85.61%), parasternal 4-chamber view (62.07%), and parasternal short-axis view (38.50%). The dominant views of patent foramen ovale were subxiphoid 2-chamber view (82.69%) and subxiphoid 4-chamber view (65.41%). The dominant views of ventricular septal defect were parasternal 5-chamber view (79.73%), parasternal short-axis view (79.41%), parasternal 4-chamber view (58.18%), and parasternal long-axis view (51.11%). The dominant view of patent ductus arteriosus were parasternal short-axis view (98.80%). Conclusions The analysis of the lesion key areas of common CHD showed that there were 4 dominant views for atrial septal defect and ventricular septal defect, 2 for patent foramen ovale, and only 1 for patent ductus arteriosus. Clarifying the dominant views of common CHD were conducive to rapid and accurate diagnosis of diseases, clinical, teaching and scientific research.

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        • Effect of 3D-printed heart model on congenital heart disease education: A systematic review and meta-analysis

          Objective To evaluate the effect of the 3D-printed heart model on congenital heart disease (CHD) education through systematic review and meta-analysis. Methods The literature about the application of the 3D-printed heart model in CHD education was systematically searched by computer from PubMed, Web of Science, and EMbase from inception to November 10, 2022. The two researchers independently screened the literature, extracted data and evaluated the quality of the literature. Cochrane literature evaluation standard was used to evaluate the quality of randomized controlled trials, and JBI evaluation scale was used for cross-sectional and cohort studies. ResultsAfter screening, 23 literatures were included, including 7 randomized controlled trials, 15 cross-sectional studies and 1 cohort study. Randomized controlled trials were all at low-risk, cross-sectional studies and and the cohort study had potential bias. There were 4 literatures comparing 3D printing heart model with 2D image teaching and the meta-analysis result showed that the effect of 3D printing heart model on theoretical achievement was more significant compared with 2D image teaching (SMD=0.31, 95%CI –0.28 to 0.91, P=0.05). Conclusion The application of the 3D-printed heart model in CHD education can be beneficial. But more randomized controlled trials are still needed to verify this result.

          Release date:2024-08-02 10:43 Export PDF Favorites Scan
        • Characteristics 0f fundus fluorescein angiography and optical coherence tomography in juvenile retinoschisis

          Objective To observe the characteristics of fundus fluorescein angiography(FFA)and optical coherence tomography(OCT)in juvenile retinoschisis. Methods The photochromes of the ocular fudus of 7 cases(14 eyes)who were diagnosed as juvenile retinoschisis were taken,among whom,5(10 eyes)were examined bv FFA,and 6(12 eyes)bv OCT. Results In 8 eyes with cystiform stellate maculopathy under ophtalmoscope,the result of FFA showed granular fluorescence in different density and shape without exact connection of the configuration between these granules and the cystlike maculopathy.In 2 eyes with pigment disorder in the macula under ophthalmoscope,blocky fluorescence was found in FFA.In 3 eyes with peripheral schisis,FFA discovered distorted and dilated retinal capillaries with different extent,and flecks of non-perfusion area.OCT images revealed thickening of the macular neuroepithelium with laminal separation,and cystic low-reflect areas in the inner layer. Conclusions In juvenile retinoschisis, pigment proliferation and degeneration in the macular area could be found.Granular fluorescence and cystic low—reflect areas could be seen in FFA and OCT,respectively. (Chin J Ocul Fundus Dis,2004,20:5-7)

          Release date:2016-09-02 05:58 Export PDF Favorites Scan
        • Inhaled nitric oxide improves postoperative hemodynamics in patients with cyanotic congenital heart disease combined with decreased pulmonary blood flow

          ObjectiveTo explore the hemodynamic effects of inhaled nitric oxide (iNO) on postoperative hemodynamic in patients with cyanotic congenital heart disease (CHD) combined with decreased pulmonary blood flow.MethodsFrom 2014 to 2018, there were 1 764 patients who received corrective repair of cyanotic CHD with decreased pulmonary blood flow in the Department of Pediatric Cardiac Surgery of Fuwai Hospital. We included 61 patients with the ratio of right ventricular systolic pressure to systolic blood pressure (SBP) ≥75% after weaning from cardiopulmonary bypass. There were 41 males and 20 females, with the age of 20.5 (9.0, 39.0) months and weight of 12.5±7.8 kg. The patients were divided into two groups: a conventional group (33 patients, conventional therapy only) and a combined therapy group (28 patients, iNO combined with conventional therapy). The hemodynamics during the first 24 hours after iNO therapy and the in-hospital outcomes of the two groups were investigated and compared.ResultsThere was no statistical difference between the two groups in demographic characteristics and surgical parameters (P>0.05). The hemodynamic effects of iNO within 24 hours included the decrease in the vasoactive inotropic score (VIS, 21.6±6.6 vs. 17.3±7.2, P=0.020) along with the increase in blood pressure (SBP: 73.7±9.7 mm Hg vs. 90.8±9.1 mm Hg, P<0.001) , the decrease in central venous pressure (10.0±3.1 mm Hg vs. 7.9±2.1 mm Hg, P=0.020), the decrease in lactate (2.2±1.7 mmol/L vs. 1.2±0.5 mmol/L, P<0.001) and increase in urine output [2.8±1.7 mL/(kg·h) vs. 4.9±2.2 mL/(kg·h), P<0.001]. The decrease of VIS at 24 h after the surgery in the conventional therapy group was not statistically significant (22.1±7.9 vs. 20.0±8.5, P=0.232). Besides, we discovered that the need for renal replacement therapy (RRT) was less in the combined therapy group than that in the conventional therapy group, especially in the moderate complicated surgery [risk adjustment in congenital heart surgery (RACHS-1) ≤3] subgroup (9.5% vs. 40.7%, P=0.016).ConclusionIn pediatric patients after corrective repair of cyanotic and pulmonary blood follow decreased CHD with increased pulmonary vascular resistance, iNO combined with conventional therapy can improve the hemodynamics effectively. Compared with the conventional therapy, the combined therapy with iNO can decrease the VIS and the need for RRT, which is beneficial to the postoperative recovery of patients.

          Release date:2021-12-27 11:31 Export PDF Favorites Scan
        • The relationship of retinal morphological features and visual acuity of X-linked juvenile retinoschisis

          ObjectiveTo observe the optical coherence tomography (OCT) features of X-linked juvenile retinoschisis (XLRS) and its relationship and visual acuity. Methods Twenty-three XLRS patients (38 eyes) were enrolled in this study.All patients underwent best corrected visual acuity (BCVA) and frequency-domain optical coherence tomography (SD-OCT) examination. The international standard visual acuity chart was used for BCVA test and the results were then converted into logarithm of the minimum angle of resolution (logMAR) visual acuity. The mean logMAR BCVA of all patients was 0.41±0.28. Topcon 3D-OCT 1000 and Topcon DRI-OCT were applied to detect the retinal layers of retinoschisis. Based on the OCT features, retinoschisis was classified into 3 types, including splitting between outer nuclear layer (ONL) and outer plexiform layer (OPL), inner nuclear layer morphology (INL) splitting, splitting between ganglion cell layer (GCL) and nerve fiber layer (NFL). Based on the OCT features of fovea, there were photoreceptor inner segment (IS)/outer segment (OS) presence group and IS/OS absence group. ResultsThere were 17 eyes (44.7%) with splitting between ONL/OPL, 38 eyes (100%) with INL splitting, 13 eyes (34.2%) with splitting between GCL/NFL. INL splitting can coexist with splitting in other retinal layers. There were 25 eyes (65.8%) with splitting in 2 or more retinal layers. The mean logMAR BCVA were 0.32±0.17 and 0.44±0.22 respectively in the IS/OS absence group and presence group, the difference was statistically significant (t=6.531, P=0.008).The mean fovea thickness (FT) were(517.10±96.92)and(523.08±72.84) μm respectively in the IS/OS absence group and presence group, the difference was not statistically significant (t=2.282, P=0.061). There was no correlation between logMAR BCVA and FT (r=0.200, P=0.929). ConclusionsIn patients with XLRS, the foveomacular schisis involved in multiple retinal layers and most frequently in the INL. The defect of IS/OS is the important reason of the low visual acuity.

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        • Clinical characteristics of familial exudative vitreoretinopathy associated rhegmatogenous retinal detachment

          ObjectiveTo investigate the clinical characteristics and surgical management of the familial exudative vitreoretinopathy (FEVR)-associated rhegmatogenous retinal detachment (RRD). MethodsRetrospective noncomparative case series. Thirty-three eye of 32 patients were diagnosed FEVR-associated RRD by Fluorescein fundus angiography. There were 26 males and 6 females. The male to female ratio is 4.3:1 with an average age of 19.35±8.83 years. The detection of best corrected visual acuity (BCVA), refraction status, fundus photograph and fluorescein fundus angiography(FFA)were underwent in all patients. FEVR was confirmedby FFA and positive family history. The BCVA, refraction status, morphology of retinal detachment, location, size and shape of retinal hole, presence and grade of proliferative vitreoretinopathy (PVR), and subretinal proliferation were recorded. ResultsAs for the refractive status, the scope of refraction was +2.0 D to-13.0 D andthe BCVA were range from light perception to 0.7. Atrophy holes which located at the temporal half were responsible for retinal detachment in all cases. Besides, horseshoe tears were noted in 6 eyes (18.18%), while macular tears were noted in 2 eyes of RRD (6.06%). The PVR greater than stage C2 was noticed in 10 eyes (30.30%), while subretinal proliferation was presented in 23 eyes (69.70%). ConclusionsMale predominance, juvenile onset and associated with moderate to high myopia are the main characteristics in FEVR-associated RRD. Atrophy holes at the temporal half and the subretinal proliferations were most commonly in FEVR-associated RRD. Detailed fuduns and FFA examination of the fellow eye should be undergone to avoid misdiagnosis.

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