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21.
We report a hitherto unknown, lethal osteochondrodysplasia in two Japanese siblings born to consanguineous parents. The skeletal
abnormalities are characterised by mesomelic brachymelia with bowed forearms, a round pelvis with shortened greater sciatic
notches, an ossification defect of the pubic bones, and absence of ossification centers in the cervical vertebral bodies.
The associated visceral anomalies comprised periportal fibrosis and cystic dysplasia of the intrahepatic bile ducts, pancreatic
ductal ectasia, a simple renal cyst, microcephaly with multifocal laminar necrosis and ectopic gray matter, dysplastic tracheobronchial
cartilage, abnormal lobulation of the lung, diaphragmatic hernia, and stenotic pulmonary valve. Thrombocytopenia was present
but megakaryocytes were slightly increased in the bone marrow. The patients showed various dysmorphic features including aniridia,
a long palpebral fissure, prominent nasal bridge, beaked nose, flat philtrum, low-set fleshy ears, micrognathia with submucosal
cleft palate, and multiple joint contractures.
Received: 23 May 1997 Accepted: 27 June 1997 相似文献
22.
Caffeine induces apoptosis of human umbilical vein endothelial cells through the caspase-9 pathway. 总被引:2,自引:0,他引:2
Shozo Matsuoka Toshitake Moriyama Noriyuki Ohara Kenji Tanimura Takeshi Maruo 《Gynecological endocrinology》2006,22(1):48-53
Caffeine is known to modulate placental and fetal umbilical circulation. It is demonstrated that apoptosis of human umbilical vein endothelial cells (HUVECs) is associated with placental umbilical vascular diseases. The present study was conducted to investigate the effects of caffeine on apoptosis of HUVECs. Isolated HUVECs were cultured under serum-free conditions for 24 h, and then treated with graded concentrations of caffeine (30, 100 and 300 microM) for additional 24 h and 48 h. The number of viable HUVECs was determined by cell counting. Apoptotic HUVECs were assessed by Hoechst33342 dye staining. The expression of caspase-9, caspase-8, caspase-3 and poly(ADP-ribose) polymerase (PARP) was assessed by Western blot analysis. Caffeine induced a dose- and time-dependent decrease in the number of viable HUVECs. Caffeine at concentrations higher than 100 microM significantly increased the percentage of apoptotic HUVECs. Caffeine at concentrations higher than 100 microM significantly increased cleaved caspase-9, caspase-3 and PARP expression in HUVECs at 24-h treatment compared with untreated cultures, whereas 30 microM caffeine significantly increased only caspase-3 expression at 24 h. Caffeine did not affect cleaved caspase-8 expression at 48 h. These results suggest that high concentrations of caffeine inhibit cell growth of HUVECs and induce apoptosis through the caspase-9 pathway. 相似文献
23.
24.
Katsumi Hirose Takahiro Kato Takaomi Harada Tomoaki Motoyanagi Hiroki Tanaka Akihiko Takeuchi Ryohei Kato Shinya Komori Yuhei Yamazaki Kazuhiro Arai Noriyuki Kadoya Mariko Sato Yoshihiro Takai 《Journal of radiation research》2022,63(4):620
The irradiation field of boron neutron capture therapy (BNCT) consists of multiple dose components including thermal, epithermal and fast neutron, and gamma. The objective of this work was to establish a methodology of dosimetric quality assurance (QA), using the most standard and reliable measurement methods, and to determine tolerance level for each QA measurement for a commercially available accelerator-based BNCT system. In order to establish a system of dosimetric QA suitable for BNCT, the following steps were taken. First, standard measurement points based on tissue-administered doses in BNCT for brain tumors were defined, and clinical tolerances of dosimetric QA measurements were derived from the contribution to total tissue relative biological effectiveness factor-weighted dose for each dose component. Next, a QA program was proposed based on TG-142 and TG-198, and confirmed that it could be assessed whether constancy of each dose component was assured within the limits of tolerances or not by measurements of the proposed QA program. Finally, the validity of the BNCT QA program as an evaluation system was confirmed in a demonstration experiment for long-term measurement over 1 year. These results offer an easy, reliable QA method that is clinically applicable with dosimetric validity for the mixed irradiation field of accelerator-based BNCT. 相似文献
25.
Yasumitsu Ogra Yu-ki Tanaka Noriyuki Suzuki 《Journal of Clinical Biochemistry and Nutrition》2022,71(1):2
Copper (Cu) participates in the biological redox reaction in the body, and its deficiency is fatal to the body. At the same time, Cu is extremely toxic when it exists in excess. Thus, the body has to tightly and spatiotemporally regulate the concentration of Cu within a physiological range by several groups of Cu-regulating proteins. However, entire mechanisms underlying the maintenance of Cu homeostasis in body and cells have not fully understood. It is necessary to analyze Cu itself in a body and in a cell to reveal the Cu homeostasis. In this review, recent advances in the analytical techniques to understand the Cu metabolism such as speciation, imaging and single-cell analysis of Cu were highlighted. 相似文献
26.
27.
Maeda N Osuga K Higashihara H Tomoda K Mikami K Nakazawa T Nakamura H Tomiyama N 《Cardiovascular and interventional radiology》2012,35(1):82-89
Purpose
The purpose of this retrospective study was to investigate the efficacy of transarterial chemoembolization (TACE) using cisplatin as a second-line treatment for advanced hepatocellular carcinoma (HCC) unresponsive to TACE using epirubicin–Lipiodol emulsion at our institution. 相似文献28.
Computer-aided detection in computed tomography colonography: current status and problems with detection of early colorectal cancer 总被引:1,自引:0,他引:1
Morimoto T Iinuma G Shiraishi J Arai Y Moriyama N Beddoe G Nakijima Y 《Radiation Medicine》2008,26(5):261-269
PURPOSE: The aim of this study was to evaluate the usefulness of computer-aided detection (CAD) in diagnosing early colorectal cancer using computed tomography colonography (CTC). MATERIALS AND METHODS: A total of 30 CTC data sets for 30 early colorectal cancers in 30 patients were retrospectively reviewed by three radiologists. After primary evaluation, a second reading was performed using CAD findings. The readers evaluated each colorectal segment for the presence or absence of colorectal cancer using five confidence rating levels. To compare the assessment results, the sensitivity and specificity with and without CAD were calculated on the basis of the confidence rating, and differences in these variables were analyzed by receiver operating characteristic (ROC) analysis. RESULTS: The average sensitivities for the detection without and with CAD for the three readers were 81.6% and 75.6%, respectively. Among the three readers, only one reader improved sensitivity with CAD compared to that without. CAD decreased specificity in all three readers. CAD detected 100% of protruding lesions but only 69.2% of flat lesions. On ROC analysis, the diagnostic performance of all three readers was decreased by use of CAD. CONCLUSION: Currently available CAD with CTC does not improve diagnostic performance for detecting early colorectal cancer. An improved CAD algorithm is required for detecting flat lesions and reducing the false-positive rate. 相似文献
29.
Midterm results of stent-graft repair of acute and chronic aortic dissection with descending tear: the complication-specific approach 总被引:12,自引:0,他引:12
Kato N Shimono T Hirano T Suzuki T Ishida M Sakuma H Yada I Takeda K 《The Journal of thoracic and cardiovascular surgery》2002,124(2):306-312
BACKGROUND: Endovascular stent-graft placement for the treatment of patients with aortic dissection is emerging as an attractive alternative to conventional cardiac operations. However, there has been no report of longer-term follow-up. The purpose of this study is to describe our midterm results with endovascular stent-graft repair for the treatment of patients with aortic dissections. METHODS: Thirty-eight patients with aortic dissections with descending tears were treated with endovascular stent-grafting. Ten patients had acute type A, 14 patients had acute type B, and 14 patients had chronic type B dissection. Stent grafts fabricated from expanded polytetrafluoroethylene-covered Z stents were placed to close entry tears in all patients through the delivery systems introduced from the femoral or the iliac arteries. RESULTS: Two patients with complicated acute type B dissection, who would have required surgical intervention, died within 30 days of the procedure, although no other patients died within the same period. There were no late deaths during the mean follow-up period of 27 months. Early and late complication rates were 33% and 36%, respectively, in patients with acute dissection, whereas rates were 4% and 0% (P <.05 vs patients with acute dissection) in patients with chronic dissection. CONCLUSIONS: Entry closure with endovascular stent-graft placement may be a safe and effective method for the treatment of patients with aortic dissection. It could be an alternative to conventional surgical intervention in selected patients with chronic dissection. However, strict patient selection and close follow-up seem mandatory in patients with acute dissection receiving Z stent-based stent-grafts. Stent-graft repair should be delayed for acute type B dissection without complications. 相似文献
30.
Naoki Ishii Noriyuki Horiki Toshiyuki Itoh Masataka Maruyama Michitaka Matsuda Takeshi Setoyama Shoko Suzuki Shino Uchida Masayo Uemura Yusuke Iizuka Katsuyuki Fukuda Koyu Suzuki Yoshiyuki Fujita 《Surgical endoscopy》2010,24(6):1413-1419