仅累及肾脏的Fabry病一例

正患者女,44岁,因"眼睑浮肿5年,蛋白尿2年,加重半月"2016年8月26日入院。患者5年前无诱因出现眼睑浮肿,晨起时明显,无明显双下肢浮肿,无肉眼血尿、泡沫样尿,未重视。2年前体检时查尿常规蛋白(+),2016年7月外院诊断"慢性肾炎",予来氟米特、雷公藤多苷片、至灵胶囊口服(具体剂量不详)治疗1个月,患者自觉无效停     

利妥昔单抗治疗冷球蛋白血症肾损害1例并文献复习

正临床资料朱某某,男,76岁,因"尿泡沫增多1月"于2014年4月第一次入院,查尿常规:PRO(+)、BLD(-),24 h尿蛋白定量600 mg,诊断为"慢性肾小球肾炎",予氯沙坦钾片50 mg,qd治疗,病情平稳。2年后出现双下肢浮肿伴尿中泡沫增多,查尿常规:PRO(++)、BLD(-),24 h尿蛋白定量2 g,临床考虑"糖尿病肾病",予氯沙坦钾片50 mg,bid、降糖等对症治疗。多次查尿蛋白定量2 g左右,Alb 33 g/L左右,肾功能正常。2018年8月因低热、乏力、双下肢浮肿、双踝关节疼痛入院。     

狼疮肾炎合并足细胞内陷性肾小球病一例

病例资料 患者,女,46岁,因"浮肿半个月"于2020年4月6日入院.患者半月前无明显诱因出现浮肿,主要为双下肢凹性浮肿,病前无受凉感冒病史,活动后下肢浮肿加重,后逐渐出现眼睑浮肿,晨起时眼睑浮肿明显,活动后眼睑水肿消失,伴面部发僵感,伴尿量减少,伴脱发、全身对称性关节疼痛,日晒后面部皮疹明显.外院查尿分析:尿蛋白(+...     

以肾脏表现为首发症状的Fabry病女性患者一例报告并文献复习

正病例资料患者,女,28岁,因"反复双下肢浮肿半年,蛋白尿、血尿2 d"于2013年6月18日入院。患者半年前无明显原因出现双下肢浮肿,以踝关节以下为重,夜间浮肿消退。患者未重视,未行任何检查治疗。3个月前自觉双下肢浮肿明显加重,且浮肿不能自行消退,遂于2 d前去医院查尿常规示蛋白尿(++)。为求进一步治疗,来我院门诊检查尿常规示蛋白尿(++),血尿(+),尿白细胞(+),门诊以"慢性肾炎"收治入院。入院查体:体温36.8℃,脉搏93次/分,呼吸20     

急性肾炎合并格林-巴利综合征1例

病例 患者严某,男,47岁,2003年6月入院.入院时患者泡沫尿,双下肢浮肿,伴恶心、四肢麻木、乏力、行走困难,无明显呼吸困难.入院前1月有咽痛、咳嗽史,无发热.     

肾病综合征合并乳糜腹水一例

患者,男,18岁,农民,因双下肢紫癜伴腹胀、腹痛、肉眼血尿及双下肢浮肿半年入院。患者于半年前无明显诱因出现对称性双下肢远端略高出皮面皮疹,痒,4周后消失,诊断为过敏性紫癜。后出现肉眼血尿及双下肢浮肿,腹痛,腹胀,腹围明显增加,外院查尿RBC10～15/HP,24小时尿蛋白定量3.6g,血ALB15g/L,抽取腹水为乳糜样,约10000ml,腹水增长很快,为进一步诊治收入病房。入院查体:神清、消瘦,BP18.6/9.3kPa     

浮肿伴大量蛋白尿

病历摘要 患者：女，40岁。因“间断浮肿七年”人院。患者七年前劳累后出现双下肢浮肿，无肉眼血尿、尿频、尿急、夜尿增多等，尿量无明显改变。在当地医院查尿常规示尿蛋白（＋＋），予输液治疗（具体不详）后浮肿消退。后浮肿反复发作，多次复查尿常规仍提示尿蛋白（＋＋）～（＋＋＋）。病程中无皮疹、腹痛，无颜面部红斑、四肢关节痛等。为求进一步诊治来我院，门诊以“慢性肾小球肾炎”收治。既往史：无特殊。家族史：父亲死于癌症（具体不详），母亲死于“肺出血”。个人史：无特殊。     

1例以腹痛、腹泻为首发症状合并ANCA阳性的狼疮性肾炎病例报道

<正>病例韩某某，女，50岁，因“腹痛、腹泻反复1月余，双下肢浮肿2周余”,于2020年12月3日入院。该患者1月前出现腹泻、腹痛，于当地医院给以抗炎、止泻等治疗症状时轻时重，2周前腹痛、腹泻加重，出现双下肢浮肿，于当地医院查胃镜显示慢性萎缩性胃炎、肠镜无异常，尿蛋白(++++),血浆白蛋白27.2 g/L,口服止痛药效果不佳，由门诊以“肾病综合征、腹膜炎?”收入院。入院时患者双下肢浮肿，腹痛、腹泻，尿中泡沫多，乏力，周身酸痛。     

糖尿病全身浮肿蛋白尿——附原发性肾病综合征1例报告

病例 患者,女性,58岁.因患糖尿病8年,全身浮肿,蛋白尿2个月,于2004年8月10日就诊.于8年前因全身乏力、消瘦,确诊为2型糖尿病,给予二甲双胍、优降糖等口服治疗,空腹血糖(Bs)控制在7 mmol/L 左右.2月前患者出现眼睑、双下肢浮肿,渐波及全身,伴胸闷,尿量减少(600～800 ml/d),泡沫多,夜尿无增多,无发热、腰痛、血尿,无关节痛,无咳嗽及喘憋.曾给予利尿剂治疗,无效.查尿常规示尿蛋白( ):Bs 9 mmol/L,肌酐 145 μmol/L,尿素氮18.6 mmol/L,于2004年8月5日拟"糖尿病肾病"收住内分泌科,给予胰岛素控制血糖,利尿等治疗,无好转,遂于2005年8月10日转入我科.患者无肝炎、结核病史,无外伤、手术及药物过敏史,无输血史,家族史无特殊.     

他克莫司治疗局灶节段性肾小球硬化一例

患者：男性,23岁,因“反复浮肿10个月,复发1个月”于2009年4月1日入院。10个月前患者无诱因出现四肢水肿,活动后明显。尿量减少。外院查尿常规：尿蛋白（＋＋＋＋）,诊断为肾病综合征,予泼尼松60mg／d”,利尿等治疗,8周后病情无好转。     

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.     

A comparison of the inhibitory effects of four volatile anaesthetics on the metabolism of chlorzoxazone, a substrate for CYP2E1, in rabbits

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.     

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.     

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.     

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.