Gitelman syndrome (GS) is an autosomal recessive kidney disorder characterized by low blood levels of potassium and magnesium, decreased excretion of calcium in the urine, and elevated blood pH. The disorder is named for Gitelman, an American nephrologist. He first described it in 1966, after observing a pair of sisters with the disorder. The disorder is caused by inactivating mutations in the SLC12A3 gene, resulting in improper function of the thiazide-sensitive sodium-chloride co-transporter located in the distal convoluted tubule of the kidney. GS was formerly considered a subset of Bartter syndrome until the distinct genetic and molecular bases of these disorders were identified. GS is usually managed by a liberal salt intake together with oral magnesium and potassium supplements. This review aims to establish an initial framework to enable clinical auditing and thus improve quality control of care.
Objective To evaluate the effectiveness of tacrolimus and cyclosporine A on acute rejection, chronic rejection and survival rate of patient and graft after renal transplantation. Methods We searched MEDLINE (1989 to Nov.2004), EMBASE (1989 to Nov.2004), The Chinese Biomedical Database (CBM) (1998 to Nov.2004), Cochrane Central Register of Controlled Trials (The Cochrane Library Issue 4, 2004) and handsearched 8 Chinese journals. Trials comparing tacrolimus with cyclosporine A after renal transplantation were included. The quality of included studies such as randomization, blinding, allocation concealment was evaluated and meta-analysis was performed using RevMan 4.2.7 software. Results Eighteen studies involving 3 738 patients were included. Tacrolimus was more effective in decreasing the incidence of acute rejection and chronic rejection than that of cyclosporine A with RR 0.65, 95％CI 0.56 to 0.75 at the end of 6 months; with RR 0.70, 95％CI 0.54 to 0.92 at the end of 12 months for number of patients of acute rejection. The pooled RR was 0.65 (95％CI 0.47 to 0.89) for number of patients of chronic rejection. Tacrolimus could reduce the severity of acute rejection. The relative risks of pathologic grade BanffⅠand Banff (Ⅱ＋Ⅲ) were 1.64 (95％CI 1.08 to 2.49) and 0.75 (95％CI 0.63 to 0.89) respectively. But there was no significant difference on the survival rate of patient and graft within 5 years between the two groups. The relative risk of 6, 12, 24, 36 and 60 months were 1.01 (95％CI 0.99 to 1.02), 1.00 (95％CI 0.99 to 1.02), 1.01 (95％CI 0.97 to 1.05), 1.00 (95％CI 0.97 to 1.03) and 0.97 (95％CI 0.88 to 1.07) respectively for the survival rate of patient and 1.04 (95％CI 1.01 to 1.07), 1.03 (95％CI 1.00 to 1.06), 0.99 (95％CI 0.91 to 1.07), 1.04 (95％CI 0.99 to 1.09) and 1.04 (95％CI 0.90 to 1.21) respectively for the survival rate of grafts. Conclusions On acute rejection and chronic rejection, tacrolimus is more effective than cyclosporine A, but there is no difference in the graft or patient survival rate.
Objective By means of evidence-based clinical practice, to find more effective treatment for a hepatitis B related nephritis patient with renal failure. Methods The following databases as Up to Date (May 2011), The Cochrane Library (Issue 5, 2011), PubMed (1978 to 2011) and CNKI (1978 to 2011) were searched to identify systematic reviews and randomized controlled trials (RCTs) of treating hepatitis B related nephritis with glucocorticoid, immunosuppressor or antiviral therapies, and the quality of collected clinical evidence was evaluated by using GRADEpro software. Results The glucocorticoid or combined immunosuppressors was not recommended for existing adverse effects and not acting on the remission of hepatitis B related nephritis and reduction of proteinuria. However, the antiviral therapy used alone was recommended for acting on the remission of hepatitis B related nephritis and the reduction of proteinuria. In view of adverse effects and expensive price of interferon, the nucleoside analogue antiviral agent was suggested. Considering the renal toxicity of adefovir and tenofovir, and possible drug-resistance of lamivudine, the entecavir (0.5 mg qd) was finally selected with patient’s agreement, and the supporting therapies such as lowering blood pressure, and protecting the kidney and liver were adopted continually. After one month treatment, 24-hour urinary protein got reduced, serum albumin got increased, kidney function got stable, and hepatitis B virus DNA quantity got reduced. Conclusion For treating hepatitis B related nephritis with kidney failure, entacavir can reduce 24-hour urinary protein, raise serum albumin, stabilize kidney function and reduce hepatitis B virus DNA in a short term, but its long-term efficacy still requires further studies.
Objective To improve arterial anastomosis method for rat renal transplantation. Methods Renal transplantations were performed on 72 wistar rats. The donor superior mesenteric artery was end-to-end anastomosed to the recipient left renal artery by using of sleeve anastomosis technique. The external diameters of the vessels anastomosed were 0.60±0.05 mm (left renal artery) or 0.80±0.07 mm (superior mesenteric artery). The procedure consisted of a guidingsuture and two fixing sutures. The guiding suture was used to “telescope” therecipient left renal artery into the donor superior mesenteric artery about 2 millimetre. Two fixing sutures were applied 180°apart from each other and tied. Three sutures passed through all layers of the donor superior mesenteric artery andconstricted the vessel lumen, but only penetrated the adventitia of the recipient left renal artery. Results The time for arterial anastomoses was approximately 6 to 8 minutes. The renal grafts perfused very well after the recipient left renal artery clamp was removed. Complications included anastomotic hemorrhage(1 case) and thrombosis (1 case). Histologic examination of 34 grafts at different postoperative time ranging from 6 to 30 days revealed that renal artery was fully patent, with no evidence of ischemic injury. Conclusion The modified arterial sleeve anastomosis technique is simple and feasible regardless of experimentalcondition and can be easily performed.
Objective To investigate the immature myocardial protection effects with renal ischemic preconditioning. Methods 18 neonatal rabbits were randomly divided into three groups. Ischemic/reperfusion(I/R) group underwent 45 min ischemia followed with 45 min reperfusion after Langendorff model performed. Cardiac ischemic preconditioning(CIP) group underwent 45 min ischemia followed with 45 min reperfusion after 5 min ischemia and then 5 min reperfusion for two times. Renal ischemic preconditioning(RIP) group underwent 45 min ischemia followed with 45 min reperfusion after renal artery obstruction for 5 min and 5 min reperfusion for three times.The left ventricular function recovery,myocardial water content(MWC), lactate dehydrogenase (LDH) and creatine kinase(CK) leakage, malondialdehyde(MDA) content,adenosine triphosphate(ATP) content, superoxide dismutase(SOD) activity, myocardial cell Ca2+ ［Ca2+］c content,mitochondrial Ca 2+ content ［Ca2+］m,synthesizing ATP activity of mitochondria ［ATP］m and Ca2+ATPase activity of mitochondria ［Ca2+ATPase］m were tested. Results The recovery of postischemic heart function in RIP group and CIP group were higher than that I/R group(Plt;0.01). There were no significant difference of HR, AF in three groups （Pgt;0.05）. There were significant difference of CF，CO，LVSP and LVEDP in RIP group and CIP group than those I/R group（Plt;0.01）. There were significant difference of MWC, CK, LDH, ATP content, MDA, SOD activity, ［Ca2+］c content, ［Ca2+ATPase］m， ［Ca2+］m and ［ATP］m in RIP group than those I/R group（Plt;0.01). There were no significant difference between RIP group and CIP group upon every index (Pgt;0.05). Conclusion RIP has the same cardioprotection to immature myocardium as ischemic preconditioning.
ObjectiveTo analyze the clinical manifestations and pathological patterns of renal diseases requiring percutaneous renopuncture, evaluate the clinical significance of renal biopsy and the value of clinical pathway for renal biopsy. MethodsWe retrospectively summarized and analyzed the clinical and pathological data, and the clinical pathway implementation of 224 patients who underwent renal biopsy between October 2009 and September 2014. ResultsIn the 224 patients, there were 62 cases of IgA nephropathy (27.68%), 50 cases of minimal change nephropathy (22.32%), 28 cases of lupus nephritis (12.5%), 26 cases of membrane nephropathy (11.6%), 26 cases of mesangial proliferative glomerulonephritis (11.6%), 6 cases of purpura nephritis (2.68%), 4 cases of focal segmental glomerular sclerosis (1.79%), 4 cases of hepatitis B virus-associated membrane nephropathy (1.79%), 4 cases of nodular diabetic glomerulosclerosis (1.79%), 4 cases of acute tubulointerstitial nephropathy (1.79%), 2 cases of hypertensive renal damage (0.89%), 2 cases of membrano-proliferative glomerulonephritis (0.89%), 1 case of lipoprotein kidney disease (0.45%), and 1 case of fibrillary glomerulopathy (0.45%). A total of 220 specimens in the 224 cases were qualified, accounting for 98.21%. Diagnosis of 70 patients in the qualified 220 cases were re-corrected according to their renal pathology reports, accounting for 31.81%. In the 224 cases, there were 16 cases of gross hematuria (7.14%) and 24 of peri-renal hematoma (10.71%) after renal biopsy. Patients who met the requirement of clinical pathway were divided into clinical pathway group and control group randomly. Average hospitalization time of the clinical pathway group was (7.6±1.2) days, and the average cost was (5 860±237) yuan, both lower than the control group [(11.8±2.3) days, (7 658±360) yuan)]. The difference was statistically significant. ConclusionsIgA nephropathy is the most common pathological type of primary glomerular diseases, and minimal change nephropathy the second. Lupus nephritis, membranous nephropathy, mesangial proliferative glomerulonephritis are still the most common types of glomerular diseases. Lupus nephritis becomes the first secondary glomerular disease. Ultrasound guided percutaneous renal biopsy is safe and has high success rate and high clinical application value. The implementation of clinical pathway can shorten the average length of hospital stay and reduce the average hospital cost.
ObjectiveTo systematically review the differential diagnostic value of contrast-enhanced ultrasound (CEUS) and contrast-enhanced CT (CECT) for renal solid space-occupying lesions. MethodsDatabases including EMbase, PubMed, The Cochrane Library (Issue 11, 2014), CNKI, CBM, VIP and WanFang Data were searched for diagnostic tests about CEUS and CECT for renal solid space-occupying lesions from inception to September, 2014. Two reviewers independently screened literature, extracted data and assessed the methodological quality of included studies. Then meta-analysis was performed using Meta-Disc 1.4 software. ResultsA total of 13 studies involving 754 specimens were included. The results of meta-analysis showed that:the sensitivity, specificity, positive likelihood ratio, negative likelihood ratio, diagnostic odds ratio (combined effect and its 95%CI) in the CEUS group were 0.96 (95%CI 0.94 to 0.97), 0.77 (95%CI 0.70 to 0.83), 3.82 (95%CI 2.93 to 4.97), 0.06 (95%CI 0.04 to 0.10), 64.33 (95%CI 36.79 to 112.51), and in the CECT group were 0.84 (95%CI 0.81 to 0.87), 0.73 (95%CI 0.65 to 0.79), 2.81 (95%CI 2.22 to 3.56), 0.23 (95%CI 0.16 to 0.34), 13.85 (95%CI 6.79 to 28.26). There were significant differences between the CEUS group (0.960 8, 95%CI 0.927 3 to 0.994 3) and the CECT group (0.866 8, 95%CI 0.788 8 to 0.944 8) in the area under the summary receiver operating characteristic (SROC) curve (P<0.05). The similar results were observed in cases with small renal tumors≤4 cm (AUC:0.973 7 vs. 0.861 3, P<0.05). ConclusionCEUS has higher differential diagnostic value than CECT for renal solid space-occupying lesions.
ObjectiveTo investigate the sonographic characteristics and diagnostic value of color Doppler sonography for patients with renal schwannoma. MethodsTen patients with pathologically confirmed renal schwannoma dimensional ultrasonography treated between January 2008 and May 2014 were included in this study. We analyzed and summarized their color flow distribution characteristics. ResultsThe ultrasound of the 10 patients showed substantial hypoechoic mass with clear boundary and complete capsule; color Doppler flow imaging displayed mass within the probe and a small amount of dotted blood flow information or no obvious blood flow information. Four patients were suspected to have schwannoma before surgery by ultrasound diagnosis, while the remaining 6 cases only showed benign lesions without clear diagnosis. All the patients underwent surgery subsequently under laparoscope, and resection of the tumor was performed with the integrity of normal kidney tissue retained. Postoperative recovery was good, and follow-up did not detect any recurrence. ConclusionColor Doppler ultrasound has a certain specificity on the diagnosis of renal schwannoma. Combined with other examination methods, it can not only give the clinicians a good suggestion, but avoid laparotomy.
Objective?To evaluate the value of various CT features in differentiating renal angiomyolipoma (RAML) with minimal fat and renal cell carcinoma (RCC). Methods?The Cochrane Library, PubMed, MEDLINE (OVID), EMBase, and the Chinese Periodical Wed (CNKI, CBM, VIP) were searched. They were searched from Jan 2001 to Nov 2008. Trials screening, quality assessment, and data extraction was conducted according to the inclusion criteria recommended by the Cochrane Collaboration. The SROC curve and meta-analyses were performed by Meta-disc 1.4. Results?Seven trials, involving 482 patients and 513 tumors, were included. The studies were highly homogonous. It was considered that 8 features including single or multiple lesions, scanning density, calcification, angle with cortex, levering-cortex-up sign, lesions pro-trusion, homogeneous enhancement, and prolonged enhancement, played certain roles in differentiating RAML with minimal fat and RCC. Among these features, interface with the cortex was the most important, and the features of homogeneous enhancement and prolonged enhancement were the second most important. Conclusion?Besides measuring lipoid tissue in the tumor, there are another 8 features which are valuable to the differentiation of RAML with minimal fat and RCC.
Abstract: Objective To evaluate the protective effects of Ulinastatin on the peri-operative liver and renal function in patients undergoing cardiac surgery for tetralogy of Fallot (TO F). Methods Thirty-eight patients with TOF were divided into Ulinastatin group and control group according to admission sequence, 19 cases in each group.For Ulinastatin group, intravenous Ulinastatin was given with a dosage of 10 000U /kg at 1h before operation, 1h and 24 h after operation. For control group, no Ulinastatin was given. 10 ml fresh urine and 2 ml blood samples were collected before operation, and postoperative 1h, 10h, 24h, 48h and 72h, respect ively. The liver and renal functions were measured. Fluid intake, urine output, chest drainage, dosage of furosemide, durations of mechanical ventilation and intensive care unit ( ICU ) stay were recorded. Results Neither arrhythmia nor low cardiac output syndrome occurred for both groups. No peri-operative death. Compared with control group, dose of furosemide, period of mechanical ventilation were lower, while urine output was higher in Ulinastat in group; the aberrant climax value of urine pro tein and N-acetylglucosam inidase (NAG) were lower in Ulinastatin group (10h post-operat ively, urinem icroalbum in: 65. 2 ± 58. 3mg/L vs. 71. 8 ±58. 9mg/L ; urine transferrin: 5. 8 ± 3. 6mg/L vs. 7. 4 ± 5. 4mg/L ; urine immunoglobulin G: 26. 9±20. 3mg/L vs. 31. 3±23. 3mg/L ; 1h post-operat ively; urine NAG: 61. 4±81. 6U /L vs. 76.1±48. 5 U /L ; P lt; 0. 05) and maintained in shorter period (P lt; 0. 05) , it returned to baseline value at 48h and 72 h post-operatively. The value of alanine aminotransferase (ALT) significantly increased post-operatively at every time points in control group (P lt; 0. 01) , w hile no obvious change in Ulinastat in group (P gt; 0. 05). The increased value of aspartate aminotransferase (AST ) in Ulinastatin group was significantly lower than that in control group (10h post-operat ively: 144. 4±20. 8U /L vs. 202. 7±74. 1U /L ; P lt; 0. 01). The value of AST returned to baseline value at 48h and 72h post-operat ively. Conclusion U linastatin is an effect ive strategy for protecting peri-operat ive liver and renal function of the patients with tetralogy of Fallot and the clinical application of Ulinastatin is safe and effective.