多囊肾术前不切原肾同种异体尸肾移植11例报告

目的探讨多囊肾尿毒症患者肾移植术前小切多囊肾对肾移植的影响。方法对11例移植术前不切多囊肾尿毒症患者，在成功进行肾脏移植后进行经验总结。追踪术后移棺肾肾功能恢复及术后3年人／移植肾存活率和术后1年原肾体积及血尿变化情况。结果11例移植术前小切多囊肾的尿毒症患者，术后移植肾肾功能均能顺利恢复，占100％。人／移植肾3年存活率100％，移植后原肾体积逐步缩小，12个月内明显缩小20％-45％，血尿逐渐消失。2例术后因原多囊肾严重感染而手术切除（18％）。结论多囊肾尿毒症患者肾移植术前不切原病变肾也能收到满意的移植效果。     

多囊肾患者肾移植术后的临床效果

目的:探讨多囊肾患者肾移植的特点、并发症及其对移植效果的影响。方法:回顾性分析了42例多囊肾患者和80例非多囊肾患者肾移植的临床资料。对两组患者的术后并发症以及1年和5年的人、肾存活率进行比较。同时对多囊肾组术前切除原肾和不切除原肾的患者进行比较。结果:两组患者在术后移植肾功能延迟恢复,急性排斥反应,心脑血管并发症以及肺部感染的发生率上均无显著性差异。多囊肾组患者术后的泌尿系感染的发生率高于对照组(P<0.05)。多囊肾组和对照组患者,1年和5年人存活率分别为95.24%与97.50%和83.81%与88.92%;1年和5年肾存活率分别为90.48%与94.97%和69.55%与66.54%。多囊肾组术前切除原肾和不切除原肾的两组患者间,上述并发症以及人、肾存活率差异均无统计学意义。结论:多囊肾患者接受肾移植是可行的,术后的人肾存活率与对照组比较差异无统计学意义,不切除原病变肾脏能收到满意的移植效果。多囊肾患者肾移植术后易发生泌尿系感染,应积极采取有效的防治措施。     

多囊肾尿毒症患者肾移植时同期切除多囊肾的临床分析

目的 探讨多囊肾尿毒症患者在接受肾移植时是否同期切除多囊肾以及切肾对肾移植手术、术后并发症及患者预后的影响.方法 对63例接受肾移植治疗的多囊肾患者的临床资料进行回顾性分析.63例中,合并多囊肝者43例,胰腺囊肿者2例.对多囊肾体积较大影响手术操作、术前曾有血尿或泌尿系感染的31例患者,在肾移植的同时切除患者的多囊肾(切肾组),另32例保留多囊肾,仅行肾移植(保留组).术后采用环孢素A(或他克莫司)、霉酚酸酯和泼尼松预防排斥反应,观察比较两组患者的一般情况、移植肾功能恢复延迟(DGF)发生率、急性排斥反应发生率、手术并发症发生率、术后感染情况、患者和移植肾存活率等指标.结果 切肾组的手术耗时为(300±31)min,肾周引流管持续时间为(4.6±1.4)d,明显长于保留组(P＜0.01,P＜0.01),红细胞输注量为(4.31±1.05)U,明显多于保留组(P＜0.01).切肾组手术并发症发生率为29.0%(9/31),明显高于保留组的6.2%(2/32),差异有统计学意义(P＜0.05).保留组泌尿系感染发生率为31.2%(10/32),而切肾组只有6.5%(2/31),二者间比较,差异有统计学意义(P＜0.05),保留组因术后多囊肾感染而须再次手术切除多囊肾者占12.5%(4/32).切肾组和保留组术前各有24例血压偏高,切肾组术后8例(33.3%)血压恢复正常,而保留组只有2例(8.3%)血压恢复正常,两组间的差异有统计学意义(P＜0.05).两组在DGF发生率和急性排斥反应发生率、人/肾1年和5年存活率等方面的差异均无统计学意义.结论 只要操作细致,多囊肾患者接受肾移植时同期切除多囊肾是安全的,但切肾与否与人/肾存活率无关.     

肾移植治疗常染色体显性遗传性多囊肾病患者的临床效果分析（附43例报告）

目的探讨肾移植治疗常染色体显性遗传性多囊肾病（多囊肾）患者的疗效。方法多囊肾患者43例（多囊肾组）,在不切除原双侧肾脏的前提下,进行肾移植,以同期50例原发病为非多囊肾的肾移植患者作为对照组,进行随访研究。比较两组的术后1、3、5年人、肾存活率及排斥反应发生情况,通过肾脏B超检查多囊肾组患者术前与术后移植肾的体积变化,记录多囊肾组的并发症发生情况。结果多囊肾组肾移植术后1、3、5年人存活率分别为95.3%、90.6%、90.6%,术后1、3、5年肾存活率分别为95.3%、88.3%、83.7%。对照组相应为96.0%、92.0%、90.0%,94.0%、92.0%、88.0%,两组比较差异无统计学意义（P〉0.05）。两组的急性排斥反应发生率比较差异亦无统计学意义（P〉0.05）。多囊肾组术后3～6个月原肾明显缩小,1年后体积基本稳定,跟踪观察1～15年肾脏体积变化不明显。移植后血尿逐渐减轻,7～10d后消失。12例在移植后5～10周反复出现肉眼血尿,均经抗感染治疗后消失。多囊肾患者移植后仍需要应用药物控制血压。多囊肾组尿路感染发生率高达40%。32例多囊肾合并多囊肝,术后发生肝功能损害7例。结论多囊肾患者采用不切除原肾的肾移植效果满意,移植后应严密观察患者移植物肾功能、血尿和感染情况,及时对症处理。     

多囊肾与肾移植相关关系的研究

目的 研究多囊肾尿毒症患者肾移植术前是否需要切除多囊肾。方法 对30例多囊肾尿毒症患者肾移植后进行随访，比较生存率及生活质量。结果 切除多囊肾后肾移植的3、5年生存率分别为100％、70％，肾移植后患者可恢复正常工作。未切除多囊肾的肾移植3、5年生存率为70％、50％，肾移植后生活质量没有提高。结论 多囊肾尿毒症患者，肾移植前应常规切除多囊肾，以提高移植后3、5年生存率和生活质量。     

多囊肾与肾移植

多囊肾是泌尿外科常见病 ,由于肾囊肿呈进行性增大 ,对肾实质形成压迫 ,使功能性肾实质日益减少 ,最终导致终末期肾功能衰竭。我院自 1986年以来共作肾移植 82 0余例 ,其中因多囊肾尿毒症行肾移植者 30例 ,占 3 7%。报道如下。临床资料　本组 30例 ,年龄 5 0～6 0岁。女 10例 ,男 2 0例。血液透析时间 6个月～ 3年。病人均有高血压及腰部酸痛 ,两侧多囊肾肿大 ,3例在移植前作多囊肾去顶减压 ,有 2例脑中风偏瘫基本恢复 ,有 2例伴发肾输尿管结石已手术取石 ,有 8例病人并发血尿及尿路感染。 30例中合并多囊肝 2 1例 ,合并肝、胰腺囊肿 1例。3…     

肾移植术前尿毒症患者合并症的手术治疗体会

目的 总结肾移植术前尿毒症合并症的手术治疗体会。以提高肾移植预后，方法 回顾性分析１９７８年至今尿毒症合并症３９例的手术指征。围手术期治疗以及肾移植预后情况，其中成人性多囊肾９例、药物不可控制性高血压２１例、脾功能亢进４例、胃溃疡５例。结果 ９例多囊肾切除术后１～６个月行肾移植术。随访肾功能正常；２１例高血压患者术后血压不同程度下降，术后６～１２个月行同种肾移植术，肾功能稳定；４例脾脾切除患者肾移     

同种异体原位肾移植初探

同种异体原位肾移植２例，手术经过和术后恢复顺利，至今已随访二年余，移植肾功能正常。因多囊肾，顽固性肾性高血压，慢性肾盂肾炎等需切除自体肾脏时使用摘除受者肾脏的同一切口植入供肾，简化了手术过程。对于第二，三次肾移植，因膀胱病而需行尿流改道者，以及髂血管粥样硬化者，原位肾移植是一种可供选择的方法。     

表皮生长因子与肾功能关系的研究

本文就表皮生长因子及其受体在肾脏肿瘤诊断、治疗及预后判断和急性肾衰治疗、肾脏移植的缺血再灌注损伤、肾移植的急慢性排斥、颅脑手术后的肾缺血、先天性肾积水、多囊肾、ESWL、肾小球肾炎、糖尿病肾病、肾发育不良症进行了综述.     

表皮生长因子与肾功能关系的研究

本文就表皮生长因子及其受体在肾脏肿瘤诊断、治疗及预后判断和急性。肾衰治疗、肾脏移植的缺血再灌注损伤、肾移植的急慢性排斥、颅脑手术后的肾缺血、先天性肾积水、多囊肾、 ESWL、肾小球肾炎、糖尿病肾病、肾发育不良症进行了综述。     

Urate homeostasis in polycystic kidney disease: comparison with chronic glomerulonephritic kidney

Autosomal dominant polycystic kidney disease (ADPKD) might affect urate homeostasis and clearance. Renal tubular urate transport was studied by means of probenecid (PB) and pyrazinamide (PZA) tests in individuals with ADPKD and normal renal function as well as various degrees of renal failure (49 patients). Comparisons were made between polycystic and chronic glomerulonephritic kidney (CGNK), as well as with controls (men with normal renal function). Patients with ADPKD and normal renal function showed plasma urate levels within normal range and normal renal urate handling. In contrast higher plasma urate levels comparing to controls were found in patients with CGNK and normal renal function. During the evolution of renal failure ADPKD patients showed lower urate plasma levels and higher renal clearance as well as, fractional urate excretion, comparing to CGNK patients with the same degree of renal failure. In conclusion patients with ADPKD and normal renal function have normal urate handling and plasma urate levels within normal range. With increasing severity of disease and during evolution of renal failure CGNK patients showed higher urate plasma levels and lower clearances comparing to ADPKD patients. When renal disease becomes more advanced there was no difference in renal urate handling between ADPKD and CGNK patients.     

Expression of secreted frizzled related protein 4 and effect of induced-apoptosis in autosomal dominant polycystic kidney disease

Objective To evaluate the expression of secreted frizzled related protein 4 (sFRP4) in autosomal dominant polycystic kidney disease(ADPKD) and the effect of sFRP4 induced apoptosis in ADPKD. Methods (1)Serological method: serum samples of 12 healthy people and 20 ADPKD patients were collected and the levels of sFRP4 in serum were detected by ELSIA assay. (2)Tissue experiments: normal renal tissue was collected from radical nephrectomy for renal carcinoma; polycystic renal tissues were taken from ADPKD patients. The expression of sFRP4 in renal tissues was observed by immunohistochemistry; Real-time PCR was used to explore the mRNA level of sFRP4 and caspase-3; TUNEL method was applied to observe the apoptosis cells existing in ADPKD. (3)studies in vitro: HEK-293T cells were transfected with PcDNA6 and Flag. sFRP4.PcDNA6 respectively, after which Western blotting was performed to detect the expression of caspase-3 protein and flow cytometry was performed to estimate cell apoptosis rate. Results (1)ELISA results showed serum concentrations of sFRP4 in ADPKD were markedly higher than normal control (P＜0.05). (2)Compared with normal renal tissues, the sFRP4 expression was dramatically increased in ADPKD and mainly distributed in the cytoplasm of renal tubular epithelial cells. (3)Real-time PCR showed the expression of sFRP4 and Caspase-3 mRNA in ADPKD were up-regulated comparing with those in normal control (P＜0.05). (4)TUNEL assays revealed that elevated apoptosis appeared in tubular epithelial cells of ADPKD. (5)The level of caspase-3 protein and apoptosis rate were significantly increased after over-expressed sFRP4 in HEK-293T cells (all P＜0.05). Conclusions The expression of sFRP4 is strikingly up-regulated in ADPKD. In addition, abnormal apoptosis of tubular epithelial cells exists in ADPKD and over-expressed sFRP4 can induce apoptosis of HEK-293T cells. This phenomenon may be attributed to the elevated sFRP4.     

Clinical aspects of renal transplantation in polycystic kidney disease

BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) as a systemic disorder represents a special subgroup among patients with end-stage renal disease (ESRD). The different organ manifestations are potential risk factors for cardiovascular events or infections in the course after renal transplantation. Therefore, a long-term evaluation of ADPKD patients and of a control group was done. PATIENTS AND METHODS: 80 ADPKD patients were compared with 88 non-diabetic patients in a retrospective follow-up after renal transplantation. Patient and graft survival (1, 5 and 10 years after transplantation) as well as complications such as infections and cardiovascular events were evaluated. RESULTS: A comparable overall transplant (1 year, 5 years, 10 years: 83%, 73%, 67% ADPKD vs. 84%, 70%, 51% controls) and patient survival rate (1 year, 5 years, 10 years: 96%, 84%, 73% ADPKD vs. 91%, 79%, 58% controls) was found in both groups. Infectious complications with the exception of urinary tract infections (UTIs: ADPKD 42.5% vs. 26%) were diagnosed in similar frequency in the graft recipients. ADPKD patients were significantly more affected by UTIs than their control group (p < 0.05) and tended to suffer more often from lethal infections (ADPKD 7 vs. controls 3), but without statistical significance. Cardiovascular events were not observed to be significantly different between both groups (ADPKD 3 vs. controls 4). An obvious difference was found in patient (p < 0.01) and transplant survival rates (p < 0.05) of male and female ADPKD patients. The female group showed a significantly better outcome. CONCLUSIONS: The overall patient and graft survival rates did not differ between the ADPKD and control groups. The better outcome of female ADPKD graft recipients compared to the male group may be related to a gender-dependent disease severity, possibly due to hormonal effects. As UTIs and lethal septicemia were the leading complications in ADPKD patients, a careful monitoring for infections is important in the post-transplant follow-up.     

Autosomal Dominant Polycystic Kidney Disease Transplant Recipients After Kidney Transplantation: A Single-center Experience

Kidney transplantation is indicated for end-stage renal disease. Autosomal dominant polycystic kidney disease (ADPKD) causes structural degeneration of the kidney and eventually becomes end-stage renal disease. ADPKD patients usually have several renal and nonrenal complications. We analyzed our kidney transplantation activities between 1991 and 2010 regarding ADPKD. We followed up with patients to December 31, 2016. Data were collected as patient and graft survival rates, the prevalence of polycystic manifestation of the gastrointestinal tract and other organs, and the attendance of urinary tract infection. Among the 734 kidney transplantations, 10.9% (n = 80) had an ADPKD. Four patients (5%) had diverticulum perforation. The prevalence of post-transplantation urinary tract infection was higher in ADPKD patients (55.9%) compared to non-ADPKD patients (44.1%). The 1-, 3-, and 5-year overall survival rates in ADPKD recipients versus non-ADPKD patients are 77.5%, 70.0%, and 67.5% versus 86.4%, 83.0%, and 80.1%, respectively. Patients with ADPKD were transplanted at an elder age compared to others (median: 47.5 years vs. 39.9 years). Female patients had longer graft survival times than males. ADPKD implies multiple cystic degeneration of the kidneys; however, it can cause structural degeneration in other organs. It is typical for ADPKD patients to have an acute abdominal-like syndrome. Immunosuppressive drugs can hide the clinical picture, which makes early diagnosis difficult.     

Long-term outcome of renal transplantation in autosomal dominant polycystic kidney disease

This study was performed to determine the long-term outcome of renal transplantation in 54 patients with end-stage renal failure secondary to autosomal dominant polycystic kidney disease (ADPKD) and in 107 patients with renal diseases other than ADPKD or diabetes mellitus matched by gender, age, year of transplantation, and source of the allograft. The overall patient survival and patient survival with a functioning first renal allograft were similar in both groups. Infection and cardiovascular accidents were the leading causes of early and late death in both groups. No cause of death was greatly overrepresented in the ADPKD group. Serious complications from extrarenal manifestations of ADPKD following renal transplantation included a ruptured intracranial aneurysm in one patient, a dissection of the ascending thoracic aorta in one patient, and infected hepatic cysts in two patients. Neoplasia (other than skin or cervical) occurred in four ADPKD patients and in one control patient and included one lymphoma in each group. Two ADPKD and one control patient had monoclonal gammopathies of undetermined significance. No complications related to the retention of native kidneys were detected in 12 ADPKD patients with a mean follow-up of 3 years. Cysts were observed in the renal allografts of some patients in both groups at autopsy and in a prospective computed tomography (CT) study of the allograft. However, we failed to detect a significant difference in the occurrence and number of the cysts between ADPKD and control patients.     

CF gene and cystic fibrosis transmembrane conductance regulator expression in autosomal dominant polycystic kidney disease

Disease-modifying genes might participate in the significant intrafamilial variability of the renal phenotype in autosomal dominant polycystic kidney disease (ADPKD). Cystic fibrosis (CF) transmembrane conductance regulator (CFTR) is a chloride channel that promotes intracystic fluid secretion, and thus cyst progression, in ADPKD. The hypothesis that mutations of the CF gene, which encodes CFTR, might be associated with a milder renal phenotype in ADPKD was tested. A series of 117 unrelated ADPKD probands and 136 unaffected control subjects were screened for the 12 most common mutations and the frequency of the alleles of the intron 8 polymorphic TN: locus of CF. The prevalence of CF mutations was not significantly different in the ADPKD (1.7%, n = 2) and control (3.7%, n = 5) groups. The CF mutation was DeltaF508 in all cases, except for one control subject (1717-1G A). The frequencies of the 5T, 7T, and 9T intron 8 alleles were also similar in the ADPKD and control groups. Two additional patients with ADPKD and the DeltaF508 mutation were detected in the families of the two probands with CF mutations. Kidney volumes and renal function levels were similar for these four patients with ADPKD and DeltaF508 CFTR (heterozygous for three and homozygous for one) and for control patients with ADPKD collected in the University of Colorado Health Sciences Center database. The absence of a renal protective effect of the homozygous DeltaF508 mutation might be related to the lack of a renal phenotype in CF and the variable, tissue-specific expression of DeltaF508 CFTR. Immunohistochemical analysis of a kidney from the patient with ADPKD who was homozygous for the DeltaF508 mutation substantiated that hypothesis, because CFTR expression was detected in 75% of cysts (compared with <50% in control ADPKD kidneys) and at least partly in the apical membrane area of cyst-lining cells. These data do not exclude a potential protective role of some CFTR mutations in ADPKD but suggest that it might be related to the nature of the mutation and renal expression of the mutated CFTR.     

Renal transplantation in patients with autosomal dominant polycystic kidney disease: pre-transplantation evaluation and follow-up

Autosomal dominant polycystic kidney disease (ADPKD) which accounts for 15% of all renal transplantations emerges as the third cause of kidney transplantation in France. In addition to routine evaluation before transplantation, the ADPKD patient requires special assessment of three aspects: should potential kidney complications (recurrent upper tract infection or haemorrhage) or kidney size assessed by computed tomography require nephrectomy prior to transplantation? Is it advisable to detect intracranial aneurysm (ICA) in patients with a relative having experienced ruptured ICA? When transplantation from a living relative is considered, the existence of ADPKD in the donor should be formally ruled out by imaging or genetic studies. The risk of recurrence of ADPKD post-transplantation does not exist. Nevertheless other complications may occur. Thus, an increased incidence of colonic perforation has been reported. In addition, as compared to non-ADPKD patients, an increased risk for both skin cancer and new-onset post-transplant diabetes mellitus has been reported recently after kidney transplantation. Finally, because these patients suffer from an inherited syndrome, physicians should carefully consider the personal and familial history before and after transplantation in order to respond to fatalism in some cases, or to attenuate excessive enthusiasm in the others. Altogether, it apears that a specific approach is needed for ADPKD patients when considering renal transplantation.     

Evaluation of colonic diverticular disease in autosomal dominant polycystic kidney disease without end-stage renal disease.

A previous study had shown an increased prevalence (83%) of diverticula among patients with autosomal dominant polycystic kidney disease (ADPKD) with end-stage renal disease (ESRD) compared with other ESRD patients without ADPKD (32%). Others have also suggested an increased risk for diverticular complications in renal transplant recipients with ADPKD. To determine whether there was an increased occurrence of diverticula among non-ESRD patients with ADPKD, we studied 55 patients with ADPKD who were not receiving renal replacement therapy compared with 12 unaffected family members (non-ADPKD) and 59 random patients who had undergone barium enemas (control [C]). No study patient had a history of diverticular disease. All patients underwent a double-contrast barium enema after administration of glucagon. The occurrence, number, location, and size of diverticula were noted. There was no significant difference among the three groups in regard to sex (men: ADPKD, 42% versus non-ADPKD, 42% versus C, 37%) or age (ADPKD, 49.3 +/- 0.7 versus non-ADPKD, 51.2 +/- 2.1 versus C, 49 +/- 1 years). There was no significant difference in the percentage of patients with diverticula (ADPKD, 47% versus non-ADPKD, 58% versus C, 59%), the percentage with only right-colon diverticula (ADPKD, 5% versus non-ADPKD, 17% versus C, 5%), the mean number of diverticula in patients with diverticulosis (ADPKD, 13.8 versus non-ADPKD, 7.9 versus C, 9.9 diverticula), or the size of the largest diverticula (ADPKD, 9.5 versus non-ADPKD, 10.4 versus C, 10.5 mm). There was no significant difference in these variables between the patients with ADPKD with a creatinine clearance greater than 70 mL/min/1.73 m(2) (n = 25) or less than 70 mL/min/1.73 m(2). This study does not show the greater prevalence of diverticular disease in non-ESRD patients with ADPKD compared with the general population. Thus, patients with ADPKD need not be considered at greater risk for diverticular disease than the general population.     

Favorable graft survival in renal transplant recipients with polycystic kidney disease

Graft survival in the autosomal dominant polycystic kidney disease (ADPKD) transplant population at our center was compared to other end stage renal disease (ESRD) transplant recipients (excluding diabetics). There were 1512 adult cadaveric renal transplants carried out at our center between 1989 and 2002. After exclusions, 1372 renal grafts were included in the study. Using Kaplan-Meier methods, patient and graft survival were determined and compared between the two groups. Mean age at transplant was significantly older for the ADPKD group of patients. The age adjusted graft survival at 5 years was 79% for ADPKD patients compared to 68% in the controls. Patient survival for ADPKD patients improved from 89% at 5 years to 95% when age adjusted. Using the Cox proportional hazards models to compare ADPKD with other causes of ESRD (including recipient age and other variables) in a multifactorial model, ADPKD was significant at the 5% level (p=0.036). This study demonstrates a graft and patient survival advantage in ADPKD patients when age-matched compared to other ESRD patients.     

Cystic fibrosis and the phenotypic expression of autosomal dominant polycystic kidney disease

Recent experiments in cultured cyst epithelial cells from kidneys of patients with autosomal dominant polycystic kidney disease (ADPKD) have shown that the cystic fibrosis (CF) transmembrane conductance regulator (CFTR) is present in the apical surface of these cells and mediates chloride (Cl-) and fluid secretion in vitro. To determine whether the presence of CF with the expression of mutated CFTR proteins modifies cyst formation in ADPKD, we studied a large family with both inherited diseases. ADPKD in this family is linked to PKD1. The family is composed of 26 members; 11 members with ADPKD, 4 members with CF, and 2 members with both diseases. Renal volumes measured by computerized tomography (CT), calculated creatinine clearances, and other clinical parameters in the family members with ADPKD and CF were compared with those in the family members with ADPKD alone, as well as to a large population of patients with ADPKD. The patients with CF and ADPKD, but not the CF heterozygote carriers with ADPKD, had less severe polycystic kidney and liver disease, as indicated by normal renal function; smaller renal volume, even when corrected for height and body surface area; and the absence of hypertension and liver cysts. These observations suggest that the coexistence of CF may reduce the severity of ADPKD.