Abstract: Objective To review the shortterm outcome of modified Nikaidoh operation, aortic translocation and biventricular outflow tract reconstruction as an alternative surgical procedure for the treatment of transposition of the great arteries with ventricular septal defect and pulmonary stenosis (TGA/VSD/PS). Methods Between January 2004 and December 2005, 8 consecutive patients had undergone Nikaidoh procedure for the treatment of TGA/VSD/PS at Shanghai Children’s Medical Center. All patients had ventriculoarterial discordance and atrioventricular concordance. Associated lesions included a straddling atrioventricular valve in one patient and hypoplastic left pulmonary artery. The median age at operation was 11.4±7.6months (4 to 29months). Weight of body was 8.0±1.9kg (5.2 to 11.0kg). No patient had previous palliative procedure. The surgical technique used was a modification of the Nikaidoh procedure. Results The median total cardiopulmonary bypass time was 176±50 minutes (range,112 to 250 minutes), and the median aortic crossclamp time was 101±27 minutes (range, 73 to 139minutes). The median length of stay in the intensive care unit was 12±9 days, with a median hospital stay of 19±12 days. There was 1 hospital death as a esult of severe left ventricle failure. There was no residual left ventricular outflow tract obstruction (LVOTO) and right ventricular outflow tract obstruction (RVOTO), but 3 patients with mild to moderate pulmonary regurgitation and 4 patients with moderate. At a median follow-up of 8.8 months (range, 3 to 18months), all patients were alive. All have the normal ventricular function. There were ejection fraction (EF) 0.64±0.02 and fractional shortening (FS) 0.33±0.02. None of the patients developed aortic insufficiency and progressed LVOTO or RVOTO. Conclusions Nikaidoh procedure is a valuable surgical option for TGA/VSD/PS in infant, especially in the presence of “inadequate anatomy” for a Rastelli repair. Big evidence and longer follow-up are required to fully assess the potential longterm benefits of this procedure compared with the Rastelli repair.
ObjectiveTo study the expressions of Ras trapping to Golgi (RasTG) genes in pancreatic carcinoma tissues and to observe the growth, proliferation and the impact of tumors formation of human pancreatic cancer cells (PANC-1), and to explore its mechanism. MethodsMade PANC1 as a target to research, transfected RasTG genes into PANC-1, used RNAi technology and observed the growth, proliferation and the impact of tumors formation of the cells. Meantime, contrasted the RasTG expressions between pancreatic ductal cancer and adjacent tissue by tissue microarray technology. Results①The expression of RasTG gene in tissues was not very differential， which was higher in the brain, liver, and adrenal gland. ②The expression of RasTG protein in pancreatic ductal carcinoma was significantly higher than that in adjacent tissues （Plt;0.05）. ③After RasTG RNAi in PANC-1 cells, the ability of growth and proliferation were decreased. ④The ability of tumors formation in PANC-1 cells after RNAi was decreased, carcinoma’s volume of transfected group was significantly smaller than that in the nontransfected group (Plt;0.05). ConclusionsRasTG gene is widely distributed in animals. RasTG protein in pancreatic carcinoma tissues is higher than that in adjacent tissues. The ability of proliferation, transformation and tumors formation in PANC-1 cells after RNAi of RasTG gene are restrained, RasTG gene is a positive regulatory factor.
ObjectiveTo detect FoxM1 expression in thyroid papillary carcinoma TPC-1 cells,and explore influence of FoxM1 expression on important genes (RAS gene and CDK1 gene) of mitogen activated protein kinase (MAPK) signal pathway. MethodsThe hFoxM1-RNA interference was used to deal with the thyroid papillary carcinoma TPC-1 cells (experiment group),another untreated TPC-1 cell was as control group.Then the real-time quantitative PCR was used to detect the FoxM1,Ras,and CDK1 gene expressions in all the TPC-1 cells. ResultCompared with the control group,the FoxM1 gene expression was significantly decreased (0.452 9 versus 1.005 0,t=24.692 9,P<0.01),the Ras gene expression was significantly elevated (1.319 0 versus 1.001 2,t=14.218 5,P<0.01),and the CDK1 gene expression was significantly decreased (0.767 5 versus 1.008 1，t=10.763 4,P<0.01) in the experiment group. ConclusionFoxM1 gene expression level in thyroid papillary carcinoma TPC-1 cells could influence Ras and CDK1 expression,which suggests that its role in thyroid papillary carcinoma might be associated with MAPK signal pathway.
ObjectiveTo detect expressions of epidermal growth factor receptor (EGFR), BRAF, and K-Ras genes in colorectal carcinoma tissues and explore pathogenesis in colorectal carcinoma. MethodThe expressions of EGFR, BRAF, and K-Ras genes were detected in these 136 colorectal carcinoma tissues and their corresponding adjacent normal colorectal tissues by immunohistochemistry. ResultsThe expressions of EGFR and BRAF in the colorectal carcinoma tissues were significantly higher than those in their corresponding adjacent normal colorectal tissues (P<0.05), but the expression of K-Ras had no significant difference between these two tissues (P>0.05). The expression of EGFR gene was related to the TNM stage, lymphatic metastasis, or degree of differentiation. The expression of BRAF gene was related to the TNM stage. The expression of K-Ras gene wasn,t related to the TNM stage, lymphatic metastasis, or degree of differentiation. The correlation analysis results showed that there was no relation among the EGFR, K-Ras, or BRAF expression. ConclusionsUp-regulated of EGFR and BRAF gene expressions might be related to development of colorectal carcinoma, and role of K-Ras is unclear. Anti EGFR and BRAF target therapy might be benefited for patients with colorectal carcinoma.
ObjectiveTo summarize the clinical phenotype, electrophysiological characteristics, imaging characteristics, surgical treatment and prognosis of Rasmussen encephalitis (RE), so as to deepen the understanding of the disease. MethodsThe clinical data of patients with RE who underwent surgical therapy from October 2014 to October 2019 at Children's Epilepsy Center in Peking University First Hospital were retrospectively reviewed. Demographic characteristics, seizure forms, electroencephalogram (EEG), cranial nuclear magnetic resonance (MRI), operative methods as well as surgical outcomes evaluated by Engel classification during follow-up of the subjects were collected and analyzed. ResultsTotally 21 pediatric patients were enrolled, including 8 males and 13 females. The age at onset was (5.0±2.0) years old, the age at the time of surgery was (6.9±2.7) years old, and the disease duration at the time of surgery was (1.7±1.3) years. Twenty (20/21, 95.2%) patients had focal motor seizures, and 10 (10/21, 47.6%) patients had 2 or 3 forms of focal motor seizures. Fifteen patients (15/21, 71.4%) had epilepsia partialis continua (EPC), which occurred (0.7±0.6) years after the onset. All patients had hemiplegia, which appeared at (0.9±0.6) years after the onset. All patients showed a slow rhythm at their affected hemispheres during the EEG monitoring and 4 of them also showed slow rhythm at the contralateral hemispheres as the disease progressed. All patients had epileptiform discharges at the involved hemisphere, and 6 patients also had independent epileptiform discharges on the contralateral side. All 21 patients underwent hemispheric disconnection. The duration of follow-up was 2 to 7 years, and all patients achieved Engel class I after the surgery. The neurological dysfunction recovered to varying degrees during the postoperative period. ConclusionRE mostly occurs around the school age. Focal motor seizures are the main manifestations and the most common onset symptoms. With the progress of the disease, the condition of patients worsened gradually. The EEG of patients was mainly characterized by broad slow wave and spike wave in the affected hemisphere. Some patients can also have bilateral involvement, which was obviously asymmetrical. Through surgical treatment, the patients all achieved good results in terms of seizures and development.
Rasmussen’s encephalitis (RE) is a rare neurologic disorder, with an incidence of 0.18 per 100,000 population, primarily affecting children, with an average onset at 6-7 years of age. Clinical manifestations include focal refractory epilepsy, progressive neurological deterioration, and cognitive decline. In imaging, magnetic resonance imaging (MRI) typically shows an increase in volume and sequences with T2/fluid-attenuated inversion recovery (FLAIR) signal, followed by atrophy. Positron emission tomography (PET) demonstrates a decline in metabolism in one hemisphere. Pathologically, neuronal loss, perivascular lymphocytic cuffing, and small glial nodules are prominent, with 10% of cases exhibiting dual pathology, primarily cortical dysplasia. Functional hemispherectomy remains the only therapeutic option, albeit resulting in permanent motor and cognitive deficits. Immunomodulatory therapy provides only temporary relief. Currently, the etiology and pathogenesis of RE remain unclear, presenting three major challenges: early diagnosis before hemisphere atrophy and neurocognitive impairment, managing immune therapies targeting inflammation, and determining rehabilitation post-surgery to maximize neurological recovery. Emerging evidence suggests that alterations in the brain’s immune microenvironment play a pivotal role in disease progression. This article focuses on the immunopathological aspects of RE, elucidating the roles of T lymphocytes, small glial cells, and astrocytes in the development of RE.
ObjectiveTo observe the effects of RasGRP4 gene deletion on the structure and function of the retina in diabetic mice, and to explore the mechanism of RasGRP4 in diabetic retinopathy (DR) by transcriptome sequencing in conjunction with bioinformatics analysis. MethodsA total of 12 male C57BL/6J mice were divided into normal group, diabetic group (DM group), with 6 mice in each group. Six male RasGRP4 knockout mice were uesd as RasGRP4 knockout diabetic group (DM-KO group). Mice in the DM group and DM-KO group were fed with high-fat diet combined with intraperitoneal injection of streptozotocin to establish diabetic model and body weight and blood glucose were monitored regularly. Three months after modeling, optical coherence tomography (OCT) was used to detect the retinal thickness. Electroretinography (ERG) was used to detect the function of the retina in mice under dark-adapted conditions. Total RNA was extracted from the retinas of the DM group and DM-KO group for transcriptome sequencing and bioinformatics analysis. The relative expression of IL-8, TGF-β, IFN-γ, NLRP3, Caspase-1, and IL-1β mRNA in retina were detected by real-time quantitative polymerase chain reaction (qRT-PCR). One-way analysis of variance was used to compare groups. ResultsThere was no statistically significant difference in blood glucose and body weight between mice in the DM group and DM-KO group (t=0.12、2.02、0.22、0.10、0.59、0.41、1.35、0.31、1.12、1.58、1.47、1.20、1.24、0.39、0.66、0.14, P＞0.05). Compared with the normal group, the retinal thickness and ganglion cell layer thickness were significantly decreased in the DM group, while the retinal thickness and ganglion cell layer thickness were significantly increased in the DM-KO group compared with the DM group, with statistical significance (F=30.43、7.81, P＜0.0001、0.01). Compared with the normal group, the a-wave and b-wave amplitudes were significantly decreased in the DM group, while the a-wave and b-wave amplitudes were significantly increased in the DM-KO group compared with the DM group, with statistical significance (F=16.46、35.58, P＜0.001、0.0001). Compared with the DM group, 184 differential genes (DEG) were screened in the DM-KO group, among which 39 up-regulated and 145 down-regulated genes were detected, respectively. The results of the MCODE plug-in analysis showed that Col1a2, Fbln1, Fbn1, Col6a3, Fmod, Ogn, Tgfb, Mfap4, Vcan, Nid2, and Col18a1 were core genes in the DEG. Cytohubba plug-in analysis showed that Col1a2, Mrc1, Cd47, Fbn1, Cybb, Cd163, Fbln1, Fmod, Adgre1, and Col6a3 were the core genes in DEG. The results of the GO functional enrichment analysis showed that DEG was mainly involved in hemoglobin complexes, MHC class II protein complex, apical plasma membrane, inflammasome complex, immunological synapse, response to bacterium, inflammatory response, immune system processe, response to hypoxia, and cell adhesion were significantly enriched. qRT-PCR results showed that compared with the normal group, the relative expression levels of IL-8, TGF-β, IFN-γ, NLRP3, Caspase-1, and IL-1β mRNA in the retina of mice in DM group were significantly increased, while the relative expression levels of IL-8, TGF-β, IFN-γ, NLRP3, Caspase-1, and IL-1β mRNA in the retina of mice in DM-KO group were significantly decreased compared with the DM group, with statistical significance (F=12.43、15.41、70.09、29.04、11.79、41.28, P＜0.01). Conclusion RasGRP4 deficiency plays a therapeutic role in the development of DR through inhibition of inflammatory factor secretion and NLRP 3 inflammasome pathway activation.
目的：采用Rastelli术治疗合并室间隔缺损、肺动脉狭窄、完全性大动脉转位病人，探讨围手术期麻醉管理特点。方法：选用大剂量芬太尼静脉复合麻醉方案，在深低温、低流量体外循环下实施Rastelli手术并进行多指标监测和临床观察。结果：麻醉经过平稳，围手术期患者生命体征稳定。结论：选择恰当的麻醉方案，有效的心脏保护,保持酸碱平衡和水、电解质稳定是成功进行麻醉管理的必要条件。
The oncogene ras p21 expression and DNA content in 46 cases of colorectal tumor were analysed quantitatively with flow cytometry and cyto-immunofluorescence staining technique. The results showed that the positive rate of ras p21 expression was 65.7% and the rate of DNA aneuploid was 74.3% in colorectal carcinomas. Ras p21 expression was higher in colorectal adenocarcinomas than that of the adenomas and normal mucosa. DNA ploid and proliferative index had some association with ras p21 expresssion. Detection of ras p21 expression and DNA content in tumors may be helpful in predicting the outcome of colorectal cancer patients.
目的 观察下调Ras同源类似物E (RhoE)表达对人乳腺癌细胞231生物学行为的影响。 方法 蛋白质印迹技术检测小干扰RNA（siRNA）转染前后RhoE在乳腺癌细胞231中的表达；RhoE siRNA的细胞转染 用lipofectamine?2000脂质体法；Cell Counting Kit-8检测转染细胞及对照细胞的增殖变化；损伤刮擦试验和体外侵袭实验（Transwell小室）分别检测转染细胞及对照细胞的迁移与侵袭能力。 结果 RhoE在乳腺癌细胞231中的表达较高；成功转染RhoE siRNA的乳腺癌细胞，蛋白质印迹显示RhoE的表达被明显的抑制；RhoE的表达被抑制后对乳腺癌细胞的增殖、迁移和侵袭有着明显的促进作用。 结论 下调RhoE 表达能够明显促进乳腺癌细胞的增殖﹑迁移和侵袭，RhoE可能在乳腺癌的发生发展中起着重要作用。