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.     

Kidney Transplantation During Autoimmune Diseases

Herein, we report the results of kidney transplantation in 9 of 376 patients who underwent kidney transplantation at our center between 1986 and 2007 because of chronic renal failure associated with autoimmune disease. Four of the 9 patients had systemic lupus erythematosus, 3 had Wegener granulomatosis, and 2 had Goodpasture syndrome. Six patients received organs from living donors, and 3 received cadaver organs. Infections were frequent and included cytomegalovirus and urinary tract infection in most cases. There was no difference in occurrence of metabolic and cardiovascular complications in our study patients compared with other transplant recipients. Incidence of allograft loss (n = 1) was similar to that in our entire transplantation population, with an overall rate of 2.9%. We conclude that kidney transplantation is a reasonable therapeutic option in patients with autoimmune disease with end-stage renal disease because of good graft and patient survival compared with kidney recipients without autoimmune diseases.     

Native Nephrectomy in Patients With Autosomal Dominant Polycystic Kidney Disease Evaluated for Kidney Transplantation

Native nephrectomy (NN) in patients with autosomal dominant polycystic kidney disease (ADPKD) is indicated in cases of recurrent urinary tract infections and hematuria, neoplastic degeneration, and encumbrance. Timing, indication, and surgical approach of NN depends on the symptoms or policy of the center. The aim of our study is to evaluate our experience.In our retrospective study, we included 130 patients with a diagnosis of ADPKD from 530 patients evaluated for renal transplantation from 2011 to 2017. We analyzed the etiologic indication, the timing, and the complications of NN.In our cohort, 53 patients underwent open NN, 85% pre-kidney transplantation (KT), 13% post-KT, and only 1 case simultaneous with KT. In the pre-KT group, indications included: major indication was encumbrance in the. In the post-KT group, the major indication was infection followed by encumbrance, which developed after KT. Complications were: 3 cases of bleeding (1 required relaparotomy, 2 evolved into hematoma and radiological derange); 1 iatrogenic iliac artery injury, which was contextually repaired, and 5 cases of incisional hernia. At 35 ± 7.2 months follow-up, patients’ survival was 96%; 1 patient died at the induction of anesthesia and 1 patient from sepsis after double NN and removal of nonfunctional transplanted kidney.NN is not without complications and should be performed when clearly indicated. In our experience, we preferred to perform NN before KT.     

成人型多囊肾与肾移植(附8例报告)

本文报告我院对8例成人型多囊肾施行同种异体肾移植的经验和体会。结果表明一年人/肾存活率为75％。讨论了①是否应该对这些病人施行肾移植?②术前肾切除对肾移植效果的影响;③多囊肾行肾移植后感染等并发症问题。     

Autosomal-Dominant Polycystic Kidney Disease and Kidney Transplantation: Experience of a Single Center

Background

Autosomal dominant polycystic kidney disease (ADPKD) is a hereditary disease that frequently leads to end-stage renal disease and is a common indication for kidney transplantation. We sought to evaluate the demographic characteristics, graft and patient survival, and some posttransplantation complications among ADPKD recipients.

Methods

This retrospective study included 445 renal transplant recipients, among whom 48 had ADPKD. We excluded patients with pretransplantation diabetes mellitus. We evaluated patient and graft survivals as well as posttransplantation complications.

Results

There was no difference between the 2 groups with respect to demographic or transplant characteristics, except for older age among the ADPKD group (51.2 ± 8.6 years vs 44 ± 13.1 years; P < .001). We also observed no significant difference with regard to immediate graft function, immunological graft, or patient survival. Although not significant, there was a lower incidence of proteinuria and a greater number of acute rejections among ADPKD patients. As for posttransplantation complications, there was no difference regarding the prevalence of hypertension, but there was more erythrocytosis among the ADPKD group. The incidence of posttransplantation diabetes mellitus was significantly greater in ADPKD patients (33.3% vs 17.1%; P = .009), and remained significant after adjusting for confounding variables by multivariate analysis with an adjusted odds ratio of 2.3 (95% confidence interval, 1.008-5.136; P = .048).

Conclusion

Our results suggested that ADPKD patients display a greater incidence of diabetes mellitus posttransplantation; ADPKD emerged as an independent predictor for this complication.     

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.     

Management of end-stage autosomal dominant polycystic kidney disease with hemodialysis and transplantation

This is an analysis of the outcome of 35 patients with end-stage autosomal dominant polycystic kidney disease (ADPKD) at Toronto Western Hospital (TWH) during a 10-year period. The primary treatment in each case was hemodialysis. In the 15 patients managed exclusively with hemodialysis the one- and five-year actuarial survival was 93% and 77% respectively. Twenty patients ultimately received a total of 26 cadaveric renal allografts. Graft survival at one year was 76%. One- and five-year patient survival was 92% and 73% respectively. Beyond 5 years a trend towards increased survival in the transplant group was seen, compared with the exclusively hemodialyzed group. Bilateral nephrectomy prior to transplantation was associated with high morbidity and mortality, and did not change either graft or patient survival. In view of the similar survival and because it is accepted that transplantation offers the highest quality of life amongst the modalities of treatment for end-stage renal failure, transplantation should be considered the treatment of choice for end-stage ADPKD. There is no justification for routine bilateral nephrectomy before renal transplantation.     

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。     

Deceased donor kidney transplantation in autosomal dominant polycystic kidney disease: a single-center experience

Renal transplantation (RTx) has become the treatment of choice for end-stage renal disease (ESRD) in autosomal dominant polycystic kidney disease (ADPKD), the most common genetic kidney disease. Because of the inheritable nature of this disease, live related donors might be avoided due to the fear of future appearance of polycystic disease. This retrospective singlecenter study was undertaken to evaluate patient/graft survival function vis-a-vis serum creatinine (SCr), rejection episodes and mortality in ADPKD vs matched control patients. Between 2000 and 2009, 18 (7.4%) deceased donor renal transplant (DDRTx) were performed for ESRD due to ADPKD. Diagnosis of ADPKD was established by family history and ultrasound. An individualized approach was applied for the need of pre-transplant nephrectomy. All recipients received rabbit-anti-thymocyte globulin induction and maintenance triple immunosuppression. Delayed graft function was observed in 33% patients, and 16% had biopsy-proven acute rejection. Over mean follow-up of 4.67 ± 2.2 years, patient and graft survival rates were 72.22% and 83.33%, with mean SCr (mg/dL) of 1.44 ± 0.54, 1.78 ± 0.42 and 2.2 ± 0.6 at 1, 5 and 10 years. Overall, 44.4% (n-8) underwent pre-transplant nephrectomy. Infection and cardio/cerebrovascular events were the main causes of death. Patient, graft survival and acute rejection were similar between ADPKD and control group. DDRTx in ADPKD has acceptable patient and graft survival. Because of the inheritable nature of the disease, and unavailability of genetic linkage analysis as a routine, DDRTx is a viable option to avoid using unrelated donors.     

Experience With Autosomal Dominant Polycystic Kidney Disease in Patients Before and After Renal Transplantation: A 7-Year Observation

Objective

Autosomal dominant polycystic kidney disease (ADPKD) is characterized by the presence of multiple cysts in both kidneys. Symptoms of the disease may arise either from the presence of cysts or from increasing loss of kidney function. First symptoms usually appear in the third decade of life: lumbar pain, urinary tract infections, arterial hypertension, or renal colic due to cyst rupture or coexistent nephrolithiasis. An early diagnosis, male gender, large kidneys by sonography, arterial hypertension, hematuria, and urinary tract infections are predictive factors of a faster progression of the disease. Our aim was to establish the indications for nephrectomy among symptomatic ADPKD patients before kidney transplantation and to assess the risks of posttransplantation complications among ADPKD patients without nephrectomy.

Patients and Methods

The observed group consisted of 183 patients with ADPKD among whom 50 (27.3%) underwent kidney transplantation during a 7-year observation period (2000-2007). Among those subjects were 3 groups: (I) nephrectomy preceding transplantation; (II) nephrectomy during kidney transplantation; and (III) without nephrectomy.

Results

Among group I before transplantation we observed: arterial hemorrhage, wound infections, and splenectomy 4 weeks after ADPKD nephrectomy; afterward we observed: urinary tract infections and contralateral cyst infection. Among group II we only observed 1 case of wound infection. Among group III we observed: ascending urinary tract infections, cyst infections, and cyst hemorrhage. Cyst hemorrhage and cyst infections led mainly to ADPKD kidney nephrectomy. During the observation time, 80.95% of grafts were functioning.

Conclusions

Unilateral nephrectomy is a well-founded preliminary surgical treatment before kidney transplantation. Bilateral nephrectomy before or during transplantation eliminates ADPKD complications and does not significantly increase general complications. The greatest numbers of complications and of graft losses were observed among the group without pretransplantation nephrectomy.     

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。     

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.     

Combined liver-kidney transplantation in polycystic disease: case reports

Polycystic disease causes a progressive decrease in renal function and liver degeneration. The progression of the disease evolves separately between organs and transplantation options vary: simultaneous or sequential liver-kidney transplantation or single-organ transplantation. From September 2006 to June 2007 3 combined liver kidney transplantations (CLKT) were performed for polycystic disease with end-stage renal disease: 2 with polycystic liver disease, and 1 with hepatic failure due to congenital hepatic fibrosis. The widest dimensions of the polycystic liver of 50 and 60 cm diameter were due to extensive cystic degeneration. We performed 1 simultaneous CLKT and 2 sequential transplantations: 1 liver after kidney, and 1 kidney after liver. At present all patients are alive with 100% graft function. Median creatinine level at discharge was 0.9 mg/dL (ranges, +/-0.2). Good liver graft function was reported in all 3 cases. Transplant benefit in polycystic liver-kidney disease has been already demonstrated; conservative surgical options may result in a high incidence of complications in highly involved polycystic livers. Delaying transplantation results in a more difficult surgical technique, a higher rate of postoperative complications, and a disturbance of optimal graft retrieval because of the worse preoperative condition of the patients.     

Polyomavirus BK replication in adult polycystic kidney disease post-renal transplant patients and possible role of cellular permissivity

Cell division and differentiation but not proliferation seem to be necessary for BK virus (BKV) replication and reactivation of persistent infection. Only terminal differentiating cells are permissive to BKV replication. Renal tubular epithelial cells in human adult polycystic kidney disease (ADPKD) are characterized by increased proliferative activity leading to cyst growth with less cellular differentiation. The aim of this study was to evaluate the possibility of different BKV replication patterns in patients with polycystic kidney disease versus non-ADPKD patients. From May 2006 to April 2008, we performed renal transplantations in 47. Eleven patients were affected by ADPKD (Pc group) and 36 patients, non ADPKD (n-Pc group). BKV replication was evaluated by quantitative real-time polymerase chain reactions (PCR) on plasma and urine samples at 12 hours (T0/early) as well as 3 (T3) and 6 (T6) months after transplantation. BKV viremia occurred in 9%, 12.5%, and 20% among the Pc group versus 33.3%, 42.4%, and 50% among the n-Pc group at 12 hours as well as 3 and 6 months posttransplantation, respectively. A higher discordance (BKV-PCR blood −/urine +) was observed in plasma and urine BKV replication among Pc versus n-Pc groups. We hypothesized that the lower number of patients with active BKV plasma replication in the Pc group may be related to a lower cellular permissivity of the renal tubular epithelial cells in ADPKD.     

Kidney transplantation in patients with a urinary diversion

Summary Patients with congenital abnormalities of the lower urinary tract may develop end-stage renal failure, necessitating kidney transplantation. If their own bladder is unsuitable for implantation of the graft ureter, or if reconstructive surgery of the lower tract is not possible, the ureter can be implanted in an ileostomy or colostomy. The results of a follow-up study of 22 kidney transplants in 19 patients with a urinary diversion are reported. The immediate post-operative surgical complications were few, including one superficial wound abscess, one lymphocele, and one hematoma. One patient had a perforation of the ileal loop after the excision of an infected kidney. The late surgical complications involved excessive length of the ileal loop in two patients, nephrolithiasis in two, and ureteroileal anastomotic strictures in three. One patient died of liver failure 2 years posttransplantation with a well-functioning graft. Today, 16 of the remaining 18 patients have a well-functioning graft, one has impaired kidney function, and one is on dialysis. The 1- and 2-year graft survival rates are 90.6% and 74.7%, respectively, and the mean follow-up period is 5.5 years. Our results show that kidney transplantation in patients with a urinary diversion can be carried out with an acceptable complication rate and a very good patient and graft survival rate.     

Transplantation of a cadaveric polycystic kidney in a patient with autosomal dominant polycystic kidney disease: long-term outcome

INTRODUCTION: Kidneys from donors affected by autosomal dominant polycystic kidney disease (ADPKD) were considered unusable for transplantation. To the best of our knowledge, seven cases worldwide have now been described in the English literature since 1967 suggesting such donor organs may be acceptable under certain conditions. Most of these reports have only short-term follow-up. METHODS: We provide a review of these patients and share our experience with an ADPKD patient who had a cadaveric ADPKD transplant and has been closely followed for 10 years. RESULTS: During the 10-year period, the patient had three transplant biopsies without complication. This creatinine is currently 1.2 mg/dL. Serial computed tomography imaging indicated that the cystic disease slowly progressed during this time period. He eventually developed intractable pain in his native left kidney and underwent a laparoscopic nephrectomy. CONCLUSIONS: Normal functioning cadaveric kidneys that show early signs of polycystic kidney disease should be considered acceptable for renal donation. These organs provide the recipient a safe, reasonable period of graft survival and have not been shown to cause adverse effects.     

Autosomal dominant polycystic kidney disease in a kidney transplant population

AIM: To define specific manifestations of autosomal dominant polycystic kidney disease in kidney transplant patients. METHODS: Of 874 consecutive first renal transplant patients 1985-1993, 114 (13%) had autosomal dominant polycystic kidney disease (ADPKD). Mean age was 53 +/- 8 years, 62% were men, and 83% received cadaveric kidneys. Control patients were matched for sex, age and donor type. Median follow-up time was 63 months. One patient was lost to follow-up. Medical records before and after transplantation were reviewed. RESULTS: Survival of patients and grafts was similar in ADPKD patients and controls. Twenty- five ADPKD patients died, four of causes not seen in the controls; two aortic aneurysms, one urothelial cancer, one colon perforation. Four more ADPKD patients but no control had diverticulitis (P = 0.03), two with perforation. Cardiovascular morbidity was not increased. Eight patients had subarachnoidal haemorrhage before transplantation and two during follow-up. Nineteen patients had undergone nephrectomy before transplantation, 11 because of voluminous kidneys, five for infection, pain or bleeding, two for suspected malignancy, one for hypertension. After transplantation, seven patients underwent nephrectomy, only one related to kidney size. During the first year, need of phlebotomy occurred in 14% of patients versus 4% of controls, P = 0.02. Urinary tract infection rates were not increased. No morbidity was related to liver cysts. CONCLUSION: The specific features of kidney transplantation to patients with ADPKD were few: enlarged kidneys, relevant only before transplantation, erythrocytosis, and as rare but serious events, diverticulitis with perforation.      

Outcomes of renal transplantation in patients with autosomal dominant polycystic kidney disease: a nationwide longitudinal study

Renal transplantation in patients with autosomal dominant polycystic kidney disease (ADPKD) is a medical and surgical challenge. Detailed longitudinal epidemiological studies on large populations are lacking and it is mandatory to care better for these patients. The success of such a project requires the development of a validated epidemiological database. Herein, we present the results of the largest longitudinal study to date on renal transplant in patients with ADPKD. The 15‐year outcomes following renal transplantation of 534 ADPKD patients were compared with 4779 non‐ADPKD patients. This comprehensive, longitudinal, multicenter French study was performed using the validated database, DIVAT (Données Informatisées et VAlidées en Transplantaion). We demonstrate that renal transplantation in ADPKD is associated with better graft survival, more thromboembolic complications, more metabolic complications, and increased incidence of hypertension, whereas the prevalence of infections is not increased. This study provides important new insights that could lead to a better care for renal transplant patients with ADPKD.     

Renal transplantation in polycystic kidney disease.

Thirty-one renal transplantations were performed in 25 patients with end-stage polycystic disease of the kidneys. Of the 14 recipients transplanted with both polycystic kidneys in situ and followed for at least 6 months, 10 had a previous history of urinary tract infection. Four of these 14 recipients had relapsing urinary tract infection after transplantation, the other 10 have been at risk on immunosuppressive therapy for a total of 152 months and have had no trouble that could be attributed to the presence of the polycystic kidneys. Despite the potentiality of polycystic kidneys to be a source of post-transplant infection when left in situ, a good outcome of renal transplatation could be achieved without preparative bilateral nephrectomy. The one-year patient and graft survivals are comparable to those obtained in our total transplantation series and renal transplantation is considered to be an acceptable therapy in polycystic disease.     

Emergent early markers of renal progression in autosomal-dominant polycystic kidney disease patients: implications for prevention and treatment

Autosomal-dominant polycystic kidney disease (ADPKD) is the most common single cause of end-stage renal disease after diabetes, hypertension and glomerulonephritis. The clinical course of ADPKD is highly variable. Even with optimal care and therapy monitoring, currently the progression of ADPKD is slowed but not stopped. Newer treatments will no doubt become available in the future, but their side effect profiles will always need to be considered. Therefore, markers to distinguish ADPKD patients with a poor versus a good prognosis will be helpful. Several risk factors influencing kidney disease progression in ADPKD have been identified in the current era. The present review will discuss the spectrum of early markers of ADPKD renal disease progression. Specifically, the volume of total kidney, hypertension, glomerular hyperfiltration, renal blood flow, microalbuminuria, uric acid, and urinary molecular markers will be discussed. On this background, implications for the prevention and treatment of kidney disease progression in ADPKD are also discussed.