肾脏轻/重链沉积病诊治进展

轻/重链沉积病是临床少见病,肾脏是其最常见受累脏器,临床常表现为大量蛋白尿和肾功能不全。血/尿游离轻链检测具有重要诊断价值。肾脏病理是诊断肾脏轻/重链沉积病的重要手段,且需要光镜、电镜及免疫病理共同诊断,缺一不可。结节样肾小球硬化是光镜下典型的肾脏病理改变。自体干细胞移植被认为有利于改善肾脏受累患者的预后。本文对该病的发病机制、诊治进展及预后进行了综述。     

吕仁和教授对“肾络瘕瘕说”的认识及分期辨治隐匿性肾小球肾炎

隐匿性肾小球肾炎是临床上常见的肾小球疾病,大多数患者仅表现为无症状性血尿、无症状性蛋白尿或两者并见。对隐匿性肾小球肾炎的预后主要取决于病理形态学的诊断,然而仍有5％～15％的患者在。肾穿刺病理检查后因肾组织基本正常而难下定论。因该病表现隐匿,故常于体检时才被发现;而且常因无临床表现或临床表现轻,因而患者未予重视,常常失去早期诊治的机会,因此早期诊断及治疗对于隐匿性肾小球肾炎患者有着重要意义。     

148例肾小球轻微病变患者的临床和电镜病理探讨

目的    对肾小球轻微病变（glomerular minor lesion,GML）进行临床及电镜病理的分类总结,进一步明确该病理表现的性质及意义,探讨其与临床表现的相关性。 方法    纳入北京协和医院2003年1月7日至2008年12月2日经光镜及免疫荧光病理诊断为GML、同时电镜病理资料完整的患者148例,回顾性分析其临床及病理资料。 结果    孤立性血尿是患者最常见的临床表现,其次为血尿合并少量蛋白尿及单纯蛋白尿,部分患者可并存高血压、特殊自身免疫表现、肝炎标志物阳性等。经电镜检查明确,GML可最终呈现各种不同的病理表现：薄基底膜肾病（66.2%）、系膜增生性肾小球肾炎（20.3%）、Alport综合征（2.7%）、膜性肾病（3.4%）以及正常组织（4.7%）等。结合临床与病理,表现为孤立性血尿的GML患者,经电镜病理检查诊断为薄基底膜肾病的可能性最大（76.9%）。部分患者存在蛋白尿、高血压等提示不良预后的情况。 结论    GML只是一种对肾脏病理表现的粗泛描述,对应的电镜病理具有较大异质性,临床意义各异,需积极完善电镜病理,结合临床特点,明确最终诊断,指导治疗。
        

透射电镜检查在冷球蛋白血症肾损害诊断中的作用

目的 探讨透射电镜检查在冷球蛋白血症肾损害诊断中的作用。方法 我院近6年来接受的肾活检病例中,透射电镜观察发现肾组织内有类似冷球蛋白沉积形成的特殊有形结构的病例共16例,对其临床、病理特征及超微结构改变进行分析。结果 4例血冷球蛋白检测阳性,诊断为冷球蛋白血症肾损害;其余12例未进行血冷球蛋白的检测,诊断为可疑的冷球蛋白血症肾损害。临床上表现为蛋白尿及镜下血尿,部分病例有肾病综合征、高血压及轻至中度肾功能不全。病理改变以膜增生性肾小球肾炎为主要病理类型,伴有毛细血管内细胞增生及单核细胞浸润,可见肾小球内皮细胞下及毛细血管腔内的嗜复红蛋白沉积形成白金耳或微血栓样结构。透射电镜检查可见肾小球内皮细胞下及毛细血管腔内的沉积物形成特殊的有形结构,表现为微管状、纤维样、晶格样或颗粒样等结构。大部分病例是通过电镜检查首先观察到肾组织内的特殊结构后才提示冷球蛋白血症肾损害的存在。结论 透射电镜检查发现肾组织内特殊结构的沉积物是诊断冷球蛋白血症肾损害的重要依据。     

糖尿病肾病合并肾小球微小病变1例

目的探讨糖尿病合并肾小球微小病变的病理诊断和治疗方案。方法回顾性分析2018年5月30日在中国人民解放军北部战区空军医院收治的1例糖尿病合并肾小球微小病变患者的临床资料。结果该患者肾脏病理可见结节性糖尿病肾小球硬化等改变, 同时电镜下可见足突弥漫融合, 起病急, 颜面部及周身浮肿明显, 联合激素及免疫抑制剂治疗后, 尿量明显增多, 预后良好。结论糖尿病肾病合并肾小球微小病变诊治需结合病理诊断、临床表现及其对激素的治疗效果综合评估, 临床医师应予高度重视。     

触须样免疫性肾小球病

触须样免疫性肾小球病（immunotactoid glomerulopathy，ITG）是指电镜下肾小球系膜区和／或基底膜内存在直径30～51nm、排列规则的类似淀粉纤维丝样物质或呈中空微管样结构的纤维样物质，但对淀粉样蛋白质特殊染色阴性，一般不伴有系统性疾病的。肾小球疾病。该病于1980年由Schwatz等首次报道，他在电镜下观察到肾小球内较粗的平行排列管样物质，类似昆虫触须，因而命名为触须样免疫性肾小球病。据不完全统计，迄今为止国内外报道已超过20例。     

膜性肾病并发纤维样肾小球病一例

纤维样肾小球病(fibrillary glomerulonephritis,FGN)是少见的肾小球病,电镜显示肾小球内存在杂乱排列的、直径约20 nm的纤维丝样结构为其特点.现报告1例纤维样肾小球病并发膜性肾病.     

电子致密物沉积病的临床及病理研究

目的：探讨电子致密物沉积病（DDD）的临床及病理特点。方法：通过5例DDD的临床及病理资料并结合文献复习,对其临床表现和组织形态学的多样性以及与治疗和预后的关系进行综合分析。结果：DDD占经肾活检证实的原发性肾小球疾病的0．2％,占膜增殖性肾炎的2％,5例中的3例临床表现为肾病综合征,组织学呈膜增生性肾炎（MPGN）,并伴有血C3降低,2例表现为慢性肾炎综合征,组织学1例呈MPGN伴有血C3降低;另1例呈系膜增生性病变,血C3正常,4例免疫荧光表现为以C3为主沿毛细血管壁呈颗粒状沉积,并有系膜区闭块状沉积,电镜下均可见电子致密物在肾小球毛细血管基底膜呈弥漫,均匀沉积,其中4例包曼囊及肾小管基底膜亦可见弥漫或节段电子致密物沉积,2例呈肾病综合征表现者对糖皮质激素治疗不敏感,结论：电子致密物沉积病是超微结构的诊断,其临床表现呈多样性,大量蛋白尿,严重高血压和较重的病理改变预示其预后不佳。     

肾小球纤维样物质沉积病（附六例病例报告）

肾小球纤维样物质沉积病（附六例病例报告）徐志雄章友康王素霞邹万忠王海燕电镜下肾小球内有纤维样物质沉积的疾病统称肾小球纤维样物质沉积病（ｆｉｂｒｉｌａｒｙｄｅｐｏｓｉｔｉｏｎｇｌｏｍｅｒｕｌｏｐａｔｈｉｅｓ，ＦＤＧＰ），主要包括肾淀粉样变、纤维样肾病（...     

原发性支气管肺淀粉样变性10例临床分析

目的探讨原发性支气管肺淀粉样变性临床表现、诊断、治疗及预后。方法回顾性分析10例原发性支气管肺淀粉样变性患者的临床特点。结果原发性支气管肺淀粉样变性患者临床表现为咳嗽、咳痰、咯血、气促,可经纤维支气管镜、肺穿刺或胸腔镜手术活检病理检查确诊,刚果红染色阳性为典型病理特征。结论本病临床少见,病理检查为确诊金标准。目前无疗效确切的治疗药物,气管受累及弥漫性病变者预后差。     

Vasopressor hormone response following mesenteric traction during major abdominal surgery

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_1α (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.     

A Teaspoon Pump for Pumping Blood with High Hydraulic Efficiency and Low Hemolysis Potential

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.     

Microspheres Based Detoxification System: A New Method in Convective Blood Purification

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.     

Tissue perfusion in relation to cardiac output during continuous positive-pressure ventilation and administration of propranolol or verapamil

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.     

Volatile anaesthetics reduce adhesion of blood platelets under low-flow conditions in the coronary system of isolated guinea pig hearts

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.     

Bariatric Surgery in Some "Central and East-European (former Eastern bloc)"Countries - Current Status and Prediction for the Next Millennium

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.     

Is the recovery profile of mivacurium independent of the rate of decay of its plasma concentration in patients with normal plasma cholinesterase activity?

Background: The duration of action of muscle relaxants is poorly correlated to the rate of decay of their plasma concentration. The plasma concentration of mivacurium may rapidly decrease below its active concentration because of the extensive hydrolysis of mivacurium. By inflating a tourniquet on one upper limb for 3 min after the administration of atracurium, mivacurium or vecuronium, we studied the influence of the initial decline of their plasma concentration on their effect. Methods: In 50 patients anaesthetised with thiopental, isoflurane and fentanyl, the effect of bolus doses of 0.15 or 0.25 mg . kg^?1 mivacurium (MIV 15, MIV 25), 0.3 or 0.5 mg . kg^?1 atracurium (ATR 30, ATR 50) and 0.06 or 0.1 mg . kg^?1 vecuronium (VEC 06, VEC 10) were measured on both arms (evoked response of the adductor pollicis to train-of-four stimulation every 12 s), a tourniquet being applied on one arm just before and during 3 min after the muscle relaxant bolus. Results: Tourniquet inflation of 3 min almost abolished the neuromuscular effect of mivacurium. In the vecuronium groups and in the ATR 50 group, tourniquet inflation did not modify the maximum degree of depression of the twitch response. Also, the duration of action of vecuronium was unaffected by the tourniquet. In the ATR 30 group, times to return of the twitch response to 25% (duration 25%) and 75% (duration 75%) of control response were significantly shorter in the cuffed arm, 23 min vs 27 min, and 41 min vs 45 min, respectively. In the ATR 50 group, only duration 25% was significantly shorter in the cuffed arm (41 min vs 45 min). Conclusion: The results suggest that the rate of decline of the plasma concentration of mivacurium is so rapid, that a very low and almost clinically ineffective concentration is present as soon as 3 min after its administration. The results also indicate that the recovery from a mivacurium-induced neuromuscular blockade is not influenced by the rate of decay of its plasma concentration in patients with genotypically normal plasma cholinesterase.     

Membranes in Artificial Organs

Abstract: Membrane processes play a pivotal and enabling role in modern replacement therapy for acute and chronic organ failure and in the management of immunologic diseases. In fact, virtually all contemporary extracorporeal blood purification methods employ membrane devices, and the next generation of artificial organs and tissue engineering therapies are almost certain to be similarly grounded in membrane technology. In this short essay, we comment on the similarities and differences among synthetic membranes and their natural counterparts and also provide a critical overview of the demographics and technology of hemodialysis, hemofiltration, apheresis, oxygenation, and emerging membrane technologies and applications.     

Influence of dopexamine hydrochloride on haemodynamics and regulators of circulation in patients undergoing major abdominal surgery

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.     

Synergistic interaction between the effects of propofol and midazolam with fentanyl on phrenic nerve activity in rabbits

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.