首页 | 本学科首页   官方微博 | 高级检索  
相似文献
 共查询到20条相似文献,搜索用时 62 毫秒
1.
Ⅰ型神经纤维瘤病软骨细胞生物学特性的研究   总被引:1,自引:0,他引:1  
目的检测神经纤维瘤蛋白在Ⅰ型神经纤维瘤病(type 1 neurofibromatosis,NF1)脊柱侧凸患者软骨细胞中的表达,观察NF1脊柱侧凸患者软骨细胞的生物学特性。方法先天性脊柱侧凸患者7例,NF1脊柱侧凸患者6例,两组年龄和Cobb角接近。脊柱后路手术中取髂骨生长板行软骨细胞分离和培养。取第2代软骨细胞分别检测其增殖活性和Ⅱ型胶原、可聚蛋白多糖等软骨细胞特异性分化指标,并用免疫沉淀和Western blot检测神经纤维瘤蛋白在两组软骨细胞中的表达。结果NF1脊柱侧凸患者软骨细胞中神经纤维瘤蛋白表达水平明显低于先天性脊柱侧凸组(灰度积分比值分别为1.31±0.53和2.17±1.02,P=0.03),其Ⅱ型胶原水平明显低于先天性脊柱侧凸患者组(26.6±11.3ng/mgpro和31.7±16.2ng/mgpro,P=0.03),而可聚蛋白多糖水平两组无明显差异(63.0±129.6ng/mgpro和76.7±519.4ng/mgpro,P=0.21)。NF1脊柱侧凸患者软骨细胞显示相对活跃的增殖活性(增殖倍数分别为2.9±0.04和2.49±0.11,P=0.02)。结论NF1脊柱侧凸患者软骨细胞神经纤维瘤蛋白表达降低的同时,其细胞增值相对活跃,细胞分化功能也存在缺陷。  相似文献   

2.
目的:检测神经纤维瘤蛋白在先天性脊柱侧凸患者成骨细胞和软骨细胞中的表达。方法:6例先天性脊柱侧凸患者,在后路手术时取髂骨及髂骨生长板,分离、培养成骨细胞和软骨细胞,分别行碱性磷酸酶染色和甲苯胺蓝染色。逆转录-多聚酶链反应(RT—PCR)检测神经纤维瘤蛋白mRNA.间接免疫荧光和Westemblot检测神经纤维瘤蛋白在成骨细胞和软骨细胞中的表达。结果:先天性脊柱侧凸患者成骨细胞和软骨细胞中存在Ⅱ型神经纤维瘤蛋白表达,该蛋白主要分布在细胞浆,所表达蛋白为三磷酸鸟苷酶活化蛋白(GAP)活性较弱的Ⅱ型异构体。结论:先天性脊柱侧凸患者成骨细胞和软骨细胞中存在神经纤维瘤蛋白表达,但该蛋白是否通过对成骨细胞和软骨细胞的影响导致骨骼系统异常还有待于进一步研究。  相似文献   

3.
本文探讨Ⅰ型神经纤维瘤病患者骨量降低的发病机制.结果 表明,Ⅰ型神经纤维瘤病常伴有骨骼病变,包括胫骨假关节形成、蝶骨翼发育不良和脊柱侧凸.最近研究表明Ⅰ型神经纤维瘤病患者常表现出不同程度的骨量减少或骨质疏松.对于骨量降低或骨质疏松的研究又集中于患者神经纤维瘤蛋白表达异常或功能缺陷,成骨细胞、破骨细胞、成纤维细胞和肥大细胞功能障碍,血清25-(OH)VD降低,生物力学因素和患者体力活动下降,以及骨骼周围血管功能异常等.结论 为Ⅰ型神经纤维瘤病患者骨量降低和骨质疏松发病机制是多种因素作用的结果.  相似文献   

4.
目的:观察Ⅰ型神经纤维瘤病(NF1)营养不良性脊柱侧凸患者椎体生长板软骨细胞的功能变化,探讨其在脊柱营养不良性改变中的作用。方法:共有8例NF1营养不良性脊柱侧凸患者纳入本研究,男5例,女3例,年龄9~15岁,平均11.8±2.0岁。在矫形术中取营养不良区(顶椎区,A组)、非营养不良区(端椎区,B组)椎体生长板软骨及髂软骨(C组)。采用两步酶消化结合植块法进行软骨细胞培养。应用Real-time PCR法检测软骨细胞中可聚蛋白多糖、Ⅱ型胶原及神经纤维瘤蛋白的mRNA表达。结果:A组软骨细胞中可聚蛋白多糖mRNA的表达量为0.04±0.02,B组为0.09±0.04,C组为0.13±0.07,A组显著低于B组和C组(P<0.05)。A组Ⅱ型胶原的mRNA表达量为0.91±0.04,B组为0.96±0.07,C组为1.03±0.10,A组显著低于C组(P<0.05),A组与B组差别无统计学意义(P>0.05)。A组神经纤维瘤蛋白的mRNA表达量为0.39±0.30,B组为1.34±0.63,C组为1.00±0.51,A组较B、C组均明显降低(P<0.05)。各指标在B组与C组间均无统计学差异(P>0.05)。结论:NF1营养不良性脊柱侧凸患者营养不良区椎体生长板软骨细胞分化功能存在明显缺陷,其可能是脊柱营养不良性改变的基础。  相似文献   

5.
【摘要】 目的:探讨Ⅰ型神经纤维瘤病(neurofibromatosis type 1,NF1) 伴脊柱侧凸患者骨密度(BMD)与血清骨钙素(BGP)及骨代谢生化指标的相关性。方法:选取2012年2月~2013年3月我院脊柱外科收治的17例NF1伴脊柱侧凸(NF1-S)患者为观察对象(病例组),年龄8~18岁,Cobb角40°~143°;17例正常儿童及青少年作为对照组,年龄7~19岁。两组均采用双能X线骨密度吸收仪测量非优势侧的股骨近端和腰椎(L2~L4)的BMD,同时采用免疫分析仪检测BGP水平,血生化分析仪检测碱性磷酸酶(ALP)、血钙(血Ca)、血磷(血P)、尿钙/肌酐比值(尿Ca/Cr)和尿磷/肌酐比值(尿P/Cr)。应用SPSS 16.0软件进行统计分析,采用独立样本t检验分析两组BMD及各项骨代谢生化指标之间的差异;Pearson相关分析病例组患者腰椎BMD与对应各项骨代谢生化指标的相关关系。结果:两组受试者年龄、性别构成比无统计学差异(P>0.05)。病例组所测各部位BMD均明显低于正常对照组,差异有显著性(P<0.05);BGP(135.6ng/ml)、血Ca(2.59mmol/L)、血P(1.75mmol/L)、尿Ca/Cr(0.10mg/mg)均高于正常对照组(79ng/ml、2.45mmol/l、1.47mmol/l、0.06mg/mg),差异均具有显著性(P<0.05),而ALP(141.6u/L vs 141.8u/l,t=3.611,P=0.991)和尿P/Cr(0.47mg/mg vs 0.46mg/mg,t=0.054,P=0.957)则无显著性差异(P>0.05)。NF1-S患者腰椎BMD与BGP(r=-0.916,P=0.000)、尿Ca/Cr(r=-0.968,P=0.000)存在显著性负相关。结论:NF1-S患者BMD明显低于正常人;BGP、血Ca、血P和尿Ca/Cr均高于正常人,测定BGP、尿Ca/Cr水平是监测NF1-S患者BMD变化较为敏感的方法之一。  相似文献   

6.
Ⅰ型神经纤维瘤病(neurofibromatosis1,NF-1)是人类最常见的单基因疾病之一,它以神经嵴细胞的异常增生为特征,儿童及成人均可发病,发病率约为1/4 000~1/3 000[1],本病可以涉及人体皮肤、神经及骨骼等多个系统,脊柱侧凸是NF-1最常见的骨骼表现之一。NF-1合并脊柱侧凸由Gould在1918年首先报道[2],其后发现NF-1性脊柱侧凸在脊柱外科领域较为常见。作为一种特殊类型的脊柱畸形,NF-1性脊柱侧凸不论是病因,还是临床表现、治疗策略等都与其他类型的脊柱侧凸显著不同,有着其自身的特点,现综述如下。1 NF-1性脊柱侧凸的病因导致脊柱侧凸的原因…  相似文献   

7.
<正>I型神经纤维瘤病(neurofibromatosis type 1,NF1)是一种累及人体多个系统的常染色体显性遗传性疾病,发病率为1/3 0001/3 500[1]。NF1病人发生骨骼畸形的概率为10%1/3 500[1]。NF1病人发生骨骼畸形的概率为10%25%[2],其中最常见的表现是脊柱侧凸,大约为10%25%[2],其中最常见的表现是脊柱侧凸,大约为10%30%[3]。临床上按有无脊柱结构性改变可分为两种类型:即非萎缩性脊柱侧凸和萎  相似文献   

8.
<正>1型神经纤维瘤病(neurofibromatosis type 1,NF1)是最常见的常染色体显性遗传病之一,发病率约为1/3000~1/4000,NF1基因突变导致神经纤维瘤蛋白失活是NF1发病的遗传基础~([1])。骨骼营养不良占所有NF1患者的50%~([2]),其中脊柱侧凸是最常见的骨损害,发病率为10%~30%~([3])。脊柱侧凸又分为营养不良型和非营养不良型,营养不良型  相似文献   

9.
Ⅰ型神经纤维瘤病是一种常见的常染色体显性遗传病,主要由NF1基因突变导致。临床表现包括皮肤色斑和良性神经纤维瘤,病变可累及神经、骨骼、血管等多种组织结构。血管病变是NF1的常见并发症,患病率和死亡率较高。本文针对NF1中血管性病变的研究进展进行综述。  相似文献   

10.
目的:探讨骨密度与青少年Ⅰ型神经纤维瘤病(neurofibromatosis1,NF-1)性脊柱侧凸患者Cobb角的关系。方法:NF-1伴脊柱侧凸组共有患者18例,年龄10~18岁,平均14.9岁,侧凸Cobb角40°~132°(平均81.4°);同年龄非脊柱畸形患者(对照组)25例,年龄11~19岁,平均15.6岁,所有研究对象均行双能X线吸收仪测量非优势侧的股骨近端和腰椎(L2~L4)的骨密度(bone mineral density,BMD),将两组受试者的测量结果进行t检验比较,并对NF-1伴脊柱侧凸组患者的BMD检测结果与侧凸Cobb角进行相关性分析。结果:两组受试者在年龄、性别比例无明显差异(P0.05),青少年NF-1伴脊柱侧凸患者所测各部位的骨密度均明显低于对照组(P0.01),腰椎(L2~L4)平均骨密度(0.61g/cm2)明显低于对照组(1.03g/cm2),腰椎骨密度的降低比股骨明显,NF-1伴脊柱侧凸组患者腰椎及非优势侧股骨近端BMD与侧凸Cobb角无显著性相关(P0.05)。结论:青少年NF-1伴脊柱侧凸患者存在全身性的骨量减低,其与侧凸的严重程度无关,可能与NF-1伴脊柱侧凸的发病机理有关。  相似文献   

11.
Background : We investigated the vasopressor hormone response following mesenteric traction (MT) with hypotension due to prostacyclin (PGI2) release in patients undergoing abdominal surgery with a combined general and epidural anesthesia. Methods : In a prospective, randomized, placebo-controlled study we administered 400 mg ibuprofen (i.v.) in 42 patients scheduled for abdominal surgery. General anesthesia was combined with epidural anesthesia (T4-L1). Before as well as 5, 15, 30, 45, and 90 min after MT we recorded plasma osmolality, hemodynamics and measured 6-keto-PGFlα (stabile metabolite of PGI2), TXB2 (stabile metabolite of thromboxane A2) active renin, and arginine vasopressin (AVP) plasma concentrations by radioimmunoassay. Catecholamine levels were assessed by high-pressure liquid chromatography (HPLC) with electrochemical detection. Results : Following MT, arterial hypotension occurred along with a substantial PGI2 release. This was completely abolished by ibuprofen administration. Although plasma levels of 6-keto-PGF (1133 (708) vs. 60 (3) ng/L, median (median absolute deviation), P=0.0001, placebo vs. ibuprofen) remained significantly elevated, blood pressure was restored within 30 min after MT in the placebo group. At the same point in time plasma concentrations of TXB2 (164 (87) vs. 58 (1) ng/L, P=0.0001), epinephrine (46 (33) vs. 14 (6) ng/L, P=0.001), AVP (41 ± (18) vs. 12 (7) ng/L, P=0.0004), and active renin (27 (12) vs. 12 (4) ng/L, P = 0.001) were significantly higher in placebo-treated patients. Conclusion : Under combined general and epidural anesthesia arterial hypotension following MT due to endogenous PGI2 release is associated with enhanced release of AVP, active renin, epinephrine and thromboxane A2, presumably contributing to hemodynamic stability within 30 min after MT.  相似文献   

12.
Don Dame 《Artificial organs》1996,20(5):613-617
Abstract: Virtually all blood pumps contain some kind of rubbing, sliding, closely moving machinery surfaces that are exposed to the blood being pumped. These valves, internal bearings, magnetic bearing position sensors, and shaft seals cause most of the problems with blood pumps. The original teaspoon pump design prevented the rubbing, sliding machinery surfaces from contacting the blood. However, the hydraulic efficiency was low because the blood was able to "slip around" the rotating impeller so that the blood itself never rotated fast enough to develop adequate pressure. An improved teaspoon blood pump has been designed and tested and has shown acceptable hydraulic performance and low hemolysis potential. The new pump uses a nonrotating "swinging" hose as the pump impeller. The fluid enters the pump through the center of the swinging hose; therefore, there can be no fluid slip between the revolving blood and the revolving impeller. The new pump uses an impeller that is comparable to a flexible garden hose. If the free end of the hose were swung around in a circle like half of a jump rope, the fluid inside the hose would rotate and develop pressure even though the hose impeller itself did not "rotate"; therefore, no rotating shaft seal or internal bearings are required.  相似文献   

13.
Background: Halothane inhibits in vitro and in vivo activity of cytochrome P-450 (CYP) 2E1. There are several fluorinated volatile anaesthetics besides halothane, and most of them are defluorinated by CYP2E1. It is unclear whether other fluorinated anaesthetics inhibit the in vivo activity of CYP2E1.
Methods: We compared the inhibitory effects of therapeutic concentrations of four inhalational anaesthetics, halothane, enflurane, isoflurane, and sevoflurane, on chlorzoxazone metabolism in rabbits receiving artificial ventilation.
Results: All four inhalational anaesthetics decreased arterial blood pressure and increased plasma chlorzoxazone concentration. However, no significant differences in the plasma chlorzoxazone concentration were found between the four anaesthetics. The estimated chlorzoxazone clearance increased after beginning inhalation with all four agents, but no significant difference in clearance was noted between agents.
Conclusions: At therapeutic concentrations, the in vivo inhibitory effect on chlorzoxazone metabolism was similar for all four inhalational anaesthetics examined, even though their chemical characteristics and extent of hepatic metabolism differ considerably.  相似文献   

14.
Abstract: A variety of protein-bound or hydrophobic substances, accumulating as a result of pathologic conditions such as exogenous or endogenous intoxications, are removed poorly by conventional detoxification methods because of low accessibility (hemodialysis), insufficient adsorption capabilities (hemosorption), low efficiency (peritoneal dialysis), or economic limitations (high-volume plasmapheresis). Combining advantages of existing methods with microspheric technology, a module-based system was designed. Major operating parameters of the latter can be modified to allow for adjustment to individual clinical situations. An extracorporeal blood circuit including a plasmafilter is combined with a secondary high-velocity plasma circuit driven by a centrifugal pump. Different microspheric adsorbers can be combined in one circuit or applied in sequence. Thus, a prolonged treatment can be tailored using specially designed selective adsorber materials. Comparing this system with existing methods (high-flux hemodialysis, molecular adsorbent recycling system), results from our in vitro studies and animal experiments demonstrate the superior efficiency of substance removal.  相似文献   

15.
Background : Our objective was to determine whether administration of propranolol or verapamil modifies the hemodynamic adaptation to continuous positive-pressure ventilation (CPPV), in particular the regional distribution of cardiac output (CO).
Methods : General hemodynamics and regional blood flows assessed by microsphere technique (15 (μm) were recorded in 16 anesthetized pigs during spontaneous breathing (SB) and CPPV with 8 cm H2O end-expiratory pressure (CPPV8) before and after intravenous administration of propranolol (0.3 mg · kg−1 followed by 0.15 mg · kg−1 · h−1, n=8) or verapamil (0.1 mg · kg−1 followed by 0.3 mg · kg−1 · h−1, n=8).
Results : CPPV8 depressed CO by 25% without shifts in its relative distribution with the exception of a noteworthy increase in adrenal perfusion. Propranolol increased arterial blood pressure, and due to a fall in heart rate, CO dropped by 25%. The kidneys and, to a lesser extent, the splanchic region and central nervous system received increased fractions of the remaining CO at the expense of skeletal muscle flow. Similar patterns were seen during SB and CPPV8 such that the combination of propranolol and CPPV8 depressed CO by 50%. The circulatory effects of verapamil were less evident but myocardial perfusion tended to increase.
Conclusions : The combination of propranolol or verapamil with CPPV does not result in any specific hemodynamic interaction in anesthetized pigs, except that the combined effect of propranolol and CPPV may severely reduce CO.  相似文献   

16.
Background: Obesity is increasing globallly, including in the formerly "Eastern Bloc" countries. Methods: A survey was made of obesity and bariatric surgery. Results: In the 8 East and Central European countries studied, with total population 300 million, roughly 43% of the population was overweight (BMI 25-30), 23% obese (BMI > 30), with about 15 million people morbidly obese (BMI > 40). From 0-10 morbidly obese individuals/100,000/year undergo bariatric surgery. Conclusion: Most countries were found to provide inadequate treatment for obesity.The majority of the morbidly obese are not treated effectively. However, health-care awareness of obesity and bariatric surgeons are slowly increasing.  相似文献   

17.
Background : Inhibitory effects of volatile anaesthetics on platelet aggregation have been demonstrated in several studies. However, the influence of volatile anaesthetics on intracoronary platelet adhesion has not been elucidated so far.
Methods : Isolated hearts of guinea pigs were perfused with buffer in the absence or presence of volatile anaesthetics (0.5 and 1 MAC) at constant coronary flow rates of 5 ml/min for 25 min, then 1 ml/min for 30 min and again 5 ml/min for 10 min. Before, during and after low-flow perfusion, a bolus of human platelets was applied into the coronary system. To simulate thrombogenic conditions, 0.3 U/ml human thrombin was infused during low-flow perfusion and reperfusion. The number of platelets sequestered to the endothelium was calculated from the difference between coronary in- and output of platelets. The myocardial production of lactate and consumption of pyruvate and coronary perfusion pressure were also determined.
Results : At a flow rate of 5 ml/min only about 3% of the applied platelets did not emerge from the coronary system, in any group. In contrast, 13.1±1.2% (mean±SEM) of infused platelets became adherent in low-flow perfusion in the control group without anaesthetic. The adherence was reduced with each 1 MAC isoflurane (to 6.2±1.2%), sevoflurane (to 4.4±0.9%) or halothane (to 3.2±1.5%) (each P <0.05 vs. control). Volatile anaesthetic, 0.5 MAC, did not inhibit platelet adhesion to a statistically significant extent in any case. Perfusion pressure and metabolic parameters were not statistically different between the control and the hearts exposed to anaesthetics.
Conclusion : Volatile anaesthetics in a concentration of 1 MAC can reduce the adhesion of platelets in the coronary system under reduced flow conditions. This action does not arise from vasodilation or inhibition of ischaemic stress.  相似文献   

18.
Background: It has been shown that the depressive effects of both propofol and midazolam on consciousness are synergistic with opioids, but the nature of their interactions on other physiological systems, e. g. respiration, has not been fully investigated. The present study examined the effect of propofol and midazolam alone and in combination with fentanyl on phrenic nerve activity (PNA) and whether such interactions are additive or synergistic. Methods: PNA was recorded in 27 anaesthetised and artificially ventilated rabbits. In three groups, propofol, fentanyl and midazolam were administered intravenously in incremental doses to construct dose-response curves for the depressant effects of each one on PNA. In another two groups, the effect of pretreatment with either fentanyl 1 μg · kg?1 i. v. or midazolam 0.05 mg · kg?1 i. v. on the effects of propofol and fentanyl respectively on PNA were studied. Results: Propofol and fentanyl caused a dose-dependent depression of PNA with complete abolition at the highest total doses of 16 mg · kg?1 i. v. and 32 μg · kg?1 i. v., respectively. In contrast, midazolam in incremental doses to a total of 0.8 mg · kg?1 reduced mean PNA by 63%, but approximately 12% of PNA remained at a total dose as high as 6.4 mg · kg?1. The mean ED50s, calculated from dose-response curves, were 5.4 mg · kg?1, 3.9 μg · kg?1 and 0.4 mg · kg?1 for propofol, fentanyl and midazolam, respectively. Initial doses of either fentanyl 1 μg · kg?1 i. v. or midazolam 0.05 mg · kg?1 i. v. acted synergistically with subsequent doses of either propofol or fentanyl to abolish PNA at total doses of 8 mg · kg?1 and 8 μg · kg?1, respectively. Conclusion: Fentanyl has a synergistic interaction with both propofol and midazolam on PNA and hence potentially on respiration.  相似文献   

19.
Background: Catecholaminergic support is often used to improve haemodynamics in patients undergoing major abdominal surgery. Dopexamine is a synthetic vasoactive catecholamine with beneficial microcirculatory properties. Methods: The influence of perioperative administration of dopexamine on cardiorespiratory data and important regulators of macro- and microcirculation were studied in 30 patients undergoing Whipple pancreaticduodenectomy. The patients received randomized and blinded either 2 μg · kg?1 · min?1 of dopexamine (n=15) or placebo (n=15, control group). The infusion was started after induction of anaesthesia and continued until the morning of the first postoperative day. Endothelin-1 (ET-1), vasopressin, atrial natriuretic peptide (ANP), and catecholamine plasma levels were measured from arterial blood samples. Measurements were carried out after induction of anaesthesia, 2 h after onset of surgery, at the end of surgery, 2 h after surgery, and on the morning of the first postoperative day. Results: Cardiac index (CI) increased significantly in the dopexamine group (from 2.61±0.41 to 4.57±0.78 1 · min?1 · m?2) and remained elevated until the morning of the first postoperative day. Oxygen delivery index (DO2I) and oxygen consumption index (VO2I) were also significantly increased in the dopexamine group (DO2I: from 416±91 to 717±110 ml/m2 · m2; VO2I: from 98±25 to 157±22 ml/m2 · m2), being significantly higher than in the control group. pHi remained stable only in the dopexamine patients, indicating adequate splanchnic perfusion. Vasopressive regulators of circulation increased significantly only in the untreated control patients (vasopressin: from 4.37±1.1 to 35.9±12.1 pg/ml; ET-1: from 2.88±0.91 to 6.91±1.20 pg/ml). Conclusion: Patients undergoing major abdominal surgery may profit from prophylactic perioperative administration of dopexamine hydrochloride in the form of improved haemodynamics and oxygenation as well as beneficial influence on important regulators of organ blood flow.  相似文献   

20.
A concept of balanced analgesia using nonsteroidal anti-inflammatory drugs (NSAIDs), paracetamol (acetaminophen), opioids, and corticosteroids can also be used in patients with pre-existing illnesses. NSAIDs are the most effective treatment for acute pain of moderate intensity in children; however, these drugs should be avoided in patients at increased risk for serious side effects, e.g. patients with renal impairment, bleeding tendency, or extreme prematurity. NSAIDs can be given with minimal risks to the younger child with mild to moderate asthma, and, in these patients, the use of steroids can be encouraged; in addition to their antiemetic and analgesic action, a beneficial effect on asthma symptoms can be expected. In the non-intubated child with cerebral trauma, exaggerated sedation caused by opioids and increased bleeding tendency caused by NSAIDs must be avoided. In neonates and small infants, the oral administration of sucrose or glucose is helpful to minimize pain reaction during short uncomfortable interventions.  相似文献   

设为首页 | 免责声明 | 关于勤云 | 加入收藏

Copyright©北京勤云科技发展有限公司  京ICP备09084417号