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排序方式: 共有1854条查询结果,搜索用时 24 毫秒
91.
Yu Kobayashi Jun Tohyama Yukitoshi Takahashi Tomohide Goto Kazuhiro Haginoya Takeshi Inoue Masaya Kubota Hiroshi Fujita Ryoko Honda Masahiro Ito Kanako Kishimoto Kazuyuki Nakamura Yasunari Sakai Jun-ichi Takanashi Manabu Tanaka Koichi Tanda Koji Tominaga Seiichiro Yoshioka Naomichi Matsumoto 《Brain & development》2021,43(4):505-514
ObjectivePatients with pathogenic cyclin-dependent kinase-like-5 gene (CDKL5) variants are designated CDKL5 deficiency disorder (CDD). This study aimed to delineate the clinical characteristics of Japanese patients with CDD and elucidate possible appropriate treatments.MethodsWe recruited patients with pathogenic or likely pathogenic CDKL5 variants from a cohort of approximately 1,100 Japanese patients with developmental and epileptic encephalopathies, who underwent genetic analysis. We retrospectively reviewed clinical, electroencephalogram, neuroimaging, and genetic information.ResultsWe identified 29 patients (21 females, eight males). All patients showed severe developmental delay, especially in males. Involuntary movements were observed in 15 patients. No antiepileptic drugs (AEDs) achieved seizure freedom by monotherapy. AEDs achieving ≥ 50% reduction in seizure frequency were sodium valproate in two patients, vigabatrin in one, and lamotrigine in one. Seizure aggravation was observed during the use of lamotrigine, potassium bromide, and levetiracetam. Adrenocorticotrophic hormone (ACTH) was the most effective treatment. The ketogenic diet (KD), corpus callosotomy and vagus nerve stimulation did not improve seizure frequency in most patients, but KD was remarkably effective in one. The degree of brain atrophy on magnetic resonance imaging (MRI) reflected disease severity. Compared with females, males had lower levels of attained motor development and more severe cerebral atrophy on MRI.ConclusionOur patients showed more severe global developmental delay than those in previous studies and had intractable epilepsy, likely because previous studies had lower numbers of males. Further studies are needed to investigate appropriate therapy for CDD, such as AED polytherapy or combination treatment involving ACTH, KD, and AEDs. 相似文献
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PurposeThe interface phenomena between anodized-hydrothermally treated commercially pure titanium (SA-treated c.p.Ti) endosseous implants and biological tissue are important in osseous wound healing. Therefore purpose of this study was to characterize the morphological, structural and chemical features of SA-treated c.p.Ti surfaces after immersion in Hanks’ balanced salt solution (HBSS).MethodsAnodic oxide (AO) c.p.Ti and SA-treated c.p.Ti disks were immersed for 14 and 28 days in HBSS (37 °C, pH 7.4). These samples were analyzed by X-ray photoelectron spectroscopy, X-ray diffraction, scanning electron microscopy, electron probe microanalyzer, and ion chromatography.ResultsX-ray photoelectron spectroscopy and X-ray diffraction patterns were similar in each sample before and after HBSS immersion. Scanning electron microscopy performed after 28 days immersion in HBSS revealed deposits completely covering SA-treated c.p.Ti. However, deposits were not observed on AO c.p.Ti after 14 or 28 days immersion. Electron probe microanalyzer results revealed an even distribution of calcium and phosphorus on SA-treated c.p.Ti after 28 days immersion, however there was no change in AO c.p.Ti disks during all immersion periods. Ion chromatography showed increased levels of calcium and phosphorus on SA-treated c.p.Ti disks compared to AO c.p.Ti disks, after 28 days immersion.ConclusionsThe results indicate that the deposits formed on SA-treated c.p.Ti surfaces contained calcium phosphate. These deposits may be formed as a consequence of the topography and physicochemical properties of SA-treated c.p.Ti surfaces and may play a key role in osteoconduction during osseous wound healing. 相似文献
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Kiyohide Fujimoto Yuzo Ichimori Hisako Yamaguchi Kunihiko Arai Takashi Futami Seiichiro Ozono Yoshihiko Hirao Tadao Kakizoe Masaaki Terada Eigoro Okajima 《Cancer science》1995,86(2):182-186
The present investigation was conducted to determine serum levels of basic fibroblast growth factor (FGF) by enzyme immunoassay in patients with various urogenital tumors. Renal cell carcinoma had a higher tendency (28 of 52, 53.8%) toward increased serum levels of basic FGF than any of the other urogenital tumors, and increased serum basic FGF was detected more frequently in patients with advanced renal cell carcinoma. Analysis of histological pattern indicated that renal cell carcinoma with a solid or tubular component is more likely to produce basic FGF. However, no significant difference was seen between the percentage of clear cell type tumor patients with increased serum basic FGF (50.0%) and the percentage of granular cell type tumor patients with increased serum basic FGF (66.7%). Five of 8 patients with renal cell carcinoma who underwent selective renal venous sampling before nephrectomy showed increased serum basic FGF in the renal vein from the affected kidney. After resection of the affected kidney to remove the cancer, serum basic FGF disappeared within 2 weeks. However, residual huge tumor or postoperative disease prolonged the increased levels of basic FGF in 2 patients, indicating that basic FGF is produced from and secreted by tumor tissue itself. These findings suggest that serum basic FGF can be useful in the diagnosis, and in evaluating the prognosis, of patients with renal cell carcinoma. 相似文献
97.
The exposure of blood to hemodialysis membranes results in numerous phenomena and/or complications in hemodialyzed patients, which have an influence on the quality of life (QOL) of those patients. A vitamin E-modified regenerated cellulose membrane (E-membrane) was developed to act as a scavenger for reactive oxygen species causing complications in hemodialysis patients. Neopterin (NEOP) is a metabolite derived from guanosine triphosphate with the production and release of NEOP being induced in monocytes and macrophages by cytokines such as interferon-gamma (IFN-gamma). Serum neopterin levels are shown to be a reactive marker of bioincompatibility of dialysis membranes in hemodialysis patients. The following report evaluates the usefulness of serum NEOP as a marker for the biocompatibility of the E-membrane hemodialyzer in a clinical study. In the clinical study, where extracorporeal ultrafiltration strategies with E-membranes were employed, the serum levels of NEOP were lower than those in patients using cellulose triacetate membranes (C-membranes). In the long-term evaluation of the biocompatibility of E- and C-membranes, the increase of serum neopterin levels in the C-membrane was higher than those in the E-membrane. In conclusion, the evaluation of serum neopterin levels during hemodialysis shows that the E-membrane has a good biocompatibility in hemodialyzed patients. 相似文献
98.
Combined cuff and suture technique for orthotopic whole intestinal transplantation in rats 总被引:4,自引:0,他引:4
Nakao A Tahara K Inoue S Mizuta K Takeichi T Uchida H Tanaka N Kobayashi E 《Microsurgery》2002,22(3):85-90
For the purpose of immunological study on small intestinal transplantation (SIT), rat SIT models using direct suture technique widely have been used, which requires at least several months of training for microsurgery. Alternatively, a simple cuff technique for SIT has been mainly used by us, which reduces warm-ischemic time and the training period, but the entire intestinal grafts usually obtain a limited blood supply. This report describes a modification of a combined cuff and suture technique for rat SIT to aid beginning microsurgical transplantation researchers. The advantages are 1) use of only arterial suturing, making it easier for beginners, with the cuff technique applied to the more difficult vein anastomosis; 2) achievement of better arterial inflow and graft survival than when the arterial cuff technique is used; and 3) doing only partial clamping of the aorta, which improves animal survival and success of the procedure. A very high successful rate in orthotopic whole SIT was achieved even by beginners. 相似文献
99.
Acute renal failure in neuroleptic malignant syndrome 总被引:2,自引:0,他引:2
We report a patient with neuroleptic malignant syndrome (NMS) who developed acute renal failure and was successfully treated by hemodialysis. A 60-year-old man with a 26-year history of schizophrenia had been treated with thioridazine and sulpiride. He was admitted to our hospital for muscular rigidity and oliguria. After the diagnosis of NMS with acute renal failure was established, thioridazine and sulpiride were discontinued and hemodialysis was instituted. Renal function gradually improved and hemodialysis was discontinued after 17th treatment. We also reviewed 57 cases of NMS with acute renal failure reported in the literature. To our knowledge, 26 years is the longest latency between initiation of neuroleptic drug treatment and onset of NMS. Our review of reported NMS cases with acute renal failure identified those risks for poor prognosis as high level of BUN, age and female gender. 相似文献
100.
Long-term follow-up of visual field progression after trabeculectomy in progressive normal-tension glaucoma 总被引:3,自引:0,他引:3
OBJECTIVE: To evaluate the long-term effects of trabeculectomy on the progression of visual field damage in patients with progressive normal-tension glaucoma (NTG). DESIGN: Retrospective, noncomparative, interventional case series. PARTICIPANTS: Twenty-three patients with NTG who had significant progression of visual field damage preoperatively and underwent trabeculectomy using antimetabolites. METHODS: Visual field testing using the Humphrey Visual Field Analyzer was periodically performed before and for at least 5 years after surgery (mean, 6 years). The time course of the mean deviation (MD) and mean of total deviations (TD(mean)) in four separate subfields, superior and inferior cecocentral and superior and inferior arcuate fields, were analyzed using a linear mixed effects model. MAIN OUTCOME MEASURES: Intraocular pressure (IOP), preoperative and postoperative regression coefficients of the time course of MD or TD(mean) in the four subfields, corresponding to the rate of progression of visual field damage. RESULTS: IOP significantly decreased from 16.2 +/- 1.8 mmHg preoperatively to approximately 11 mmHg during the postoperative follow-up period (P < 0.001). The preoperative regression coefficient of MD change was significantly negative (-1.05 [95% confidence interval, -1.28 to -0.82] dB/year, P < 0.001). After surgery, it increased significantly to -0.44 (95% confidence interval, -0.64 to -0.24) dB/year (P < 0.001), but the postoperative value was still significantly negative (P < 0.001). In the superior cecocentral, superior and inferior arcuate fields, preoperative regression coefficients of TD(mean) change were significantly negative (P < 0.001) and significantly increased after surgery (P < 0.01). In the inferior cecocentral field, the preoperative regression coefficient was not significantly different from 0 (P = 0.72) and did not change significantly after surgery (P = 0.15). CONCLUSIONS: Trabeculectomy was statistically associated with slowing further progression of visual field damage in patients with progressive NTG. The progression, however, did not completely stop over the 6-year postoperative follow-up period. 相似文献