Comparison between Retroperitoneal Laparoscopic Nephrectomy and Traditional Open Nephrectomy to Treat Polycystic Kidney Disease before Kidney Transplantation.

PURPOSE: To compare the efficiency and safety between retroperitoneal laparoscopic nephrectomy and traditional open nephrectomy to treat autosomal-dominant polycystic kidney disease before kidney transplantation. MATERIALS AND METHODS: A total of 57 patients diagnosed with huge autosomal-dominant polycystic kidney disease between 2000 and 2020 at our center were included in this study. Patients were divided into a retroperitoneal laparoscopic (RL; n=23) group and traditional open (TO; n = 34) group. We retrospectively analyzed and compared preoperative and perioperative variables between the two groups. RESULTS: Patients in the RL group showed a longer operation time (201.09±83.76min) compared to patients in the TO group (113.38 ± 51.84min, p < 0.001). The RL group also showed significantly less intraoperative blood loss (p = 0.025) and less intraoperative blood transfusion volume (p = 0.016) compared to the TO group. Meanwhile, time of gastrointestinal function recovery, bed leave, catheter indwelling and postoperative hospitalization in the RL group were 2.13 ± 0.63, 1.30 ± 1.0, 5.22 ± 2.09, 7.35±2.48 days, respectively, which were significantly shorter than the TO group (p < 0.05). Pain degree of patients during the first 48 hours after operation was similar between the RL and TO groups, but the opioid use percentage in the RL group was 8.70% (2/23) and was lower than the 26.47% (9/34) in the TO group (p = 0.022). Meanwhile, 5 and 23 patients exhibited postoperative complications in the RL and TO groups, respectively (p < 0.001). CONCLUSION: Both retroperitoneal laparoscopic nephrectomy and traditional open surgery are feasible to treat huge polycystic nephrectomy. However, patients who undergo retroperitoneal laparoscopic nephrectomy experience higher levels of safety and recover more rapidly.

[Stanford Type A Acute Aortic Dissection Associated with Polycystic Kidney Disease].

Whereas cerebral aneurysm is a well-known consequence of autosomal dominant polycystic kidney disease (ADPKD), acute aortic dissection has been rarely reported. A patient was a 44-year-old male with a diagnosis of ADPKD, who had previously undergone transcatheter arterial embolization for a renal cyst hemorrhage. He presented with sudden onset of back pain, which got worse at emergency service. Contrast-enhanced computed tomography (CT) revealed Stanford type A acute aortic dissection. The patient subsequently underwent partial aortic arch replacement with a vascular graft under circulatory arrest. His postoperative course was complicated by pneumonia and required ventilation support for a week. Peak creatinine level was 3.28 mg/dl, but hemodialysis was not required. Patients with ADPKD should be considered a high-risk cohort of aortic dissection.

Recurrent urinary tract infections in kidney transplant patients with polycystic kidneys.

INTRODUCTION: Kidney transplantation is now a routine method used to treat end-stage renal disease. About 10 % of kidney transplant patients are patients with autosomal dominant polycystic kidney disease (ADPKD). After successful kidney transplantation, recurrent urinary tract infections also occur in initially asymptomatic patients. MATERIAL AND METHODS: The group included 320 patients after kidney transplantation. We compared patients with ADPKD versus patients without ADPKD in terms of the presence of recurrent urinary tract infections. THE RESULTS: The incidence of recurrent urinary tract infections (rIMCs) was 18% in patients without ADPKD and 48% in patients without ADPKD. Nephrectomy after kidney transplantation due to recurrent urinary tract infections eliminated this infectious complication (in 86% of patients). CONCLUSION: Kidney transplant patients with ADPKD have a significantly higher incidence of recurrent urinary tract infections. Removal of polycystic kidneys is a suitable solution if the infection persists.

[Bilateral laparoscopic nephrectomy for polycystic kidney disease].

A clinical case of surgical treatment of a patient with autosomal dominant type of polycystic kidney disease, stage 5 of chronic kidney disease and secondary arterial hypertension is presented in the article. The technique of single-stage bilateral laparoscopic nephrectomy, patented by the authors, is described. The practicability and safety of a simultaneous bilateral procedures was demonstrated, as well as the advantage of laparoscopic access for this type of surgical interventions. The successful and prompt procedure allowed the patient to undergone to allotransplantation of a cadaveric kidney as soon as possible.

Simultaneous Robotic-Assisted Bilateral Native Nephrectomy and Kidney Transplantation for Autosomal Dominant Polycystic Kidney Disease in Recipients With High Body Mass Index: Report of 2 Cases.

Robotic kidney transplantation is a safe, reproducible, and less morbid technique in high body mass index and end-stage renal disease. Polycystic kidney disease is a relative contraindication to robotic-assisted kidney transplantation because of the mass effect of the native kidneys on the patient's pelvis that prevents ideal exposure. We report the first 2 cases of robotic-assisted simultaneous bilateral nephrectomy and kidney transplantation for patients with obesity and adult polycystic kidney disease. The recipients were 2 males, 50 and 53 years old, with a body mass index of 35.1 41.6 kg/m2 and 41.6 kg/m2, respectively. Both recipients had suitable living donors. The average operating time was 395 minutes and the estimated blood loss was on average 250 mL. The postoperative course was uneventful and the patients were discharged home on days 4 and 5. Performing robotic nephrectomies simultaneously with kidney transplantation can be done safely, allowing patients with obesity and polycystic kidney disease needing bilateral nephrectomy, to take full advantage of minimally invasive kidney transplantation.

Effect of ultra-mini percutaneous nephrolithotomy and ShuoTong ureteroscopy on the stress response, inflammatory indices, and urokinase level in patients with polycystic kidney disease complicated with renal calculus.

This study aimed to explore the effects of ultra-mini percutaneous nephrolithotomy (UMP) combined with ShuoTong ureteroscopy on the stress response, inflammation indicators and urokinase levels in patients with polycystic kidney disease and kidney stones. In this study, 42 patients with polycystic kidney disease and kidney stones diagnosed and treated in our hospital from February 2018 to February 2021 were selected as the research objects. They were randomly divided into the control group and the study group, with 21 cases in each group, and the control group adopted Ultra-mini percutaneous nephrolithotomy treatment; the study group was combined with ShuoTong ureteroscopy on the basis of the control group, and compared and analyzed the stress response, inflammation indexes, urokinase level, renal function, complications and stone clearance rate of the two groups of patients. After treatment, the average operation time, channel establishment time, hemoglobin decline, number of puncture channels, and hospital stay in the study group were significantly shorter than those in the control group (P<0.05); after treatment, the reduction in CRP and PCT in the study group was significantly higher than that in the control group The increase of Ang I and Ang II in the control group was significantly lower than that in the control group (P<0.05); the improvement of PGE2, NO, and LPO levels in the study group was significantly higher than that in the control group after treatment (P<0.05); the study group TH, Cys- The levels of C and ß2-MG were significantly lower than those of the control group; the UK levels of the study group were significantly lower than those of the control group (P<0.05); the complication rate of 14.29% in the study group after treatment was significantly lower than that of the control group 33.33%; the study group The stone clearance rate of 95.24% was significantly higher than that of the control group 80.95% (P<0.05). In general, UMP combined with ShuoTong ureteroscopy is used to treat polycystic kidney with kidney stones, accurately determine the tip position of the needle sheath, ensure that the needle sheath enters the collection system to adjust its position, reduce patient stress and inflammation, improve safety and feasibility, and is worthy of clinical practice Promote applications.

Neonatal multicystic dysplastic kidney with mass effect: A systematic review.

INTRODUCTION: Multicystic Dysplastic Kidney (MCDK) is a common cause of palpable abdominal mass in a neonate, yet reports are few of resultant life-threatening extrinsic compression. This systematic review is the first to review all known neonatal MCDK cases complicated by external compression due to mass effect. Our aim is to foster communication about these unusual cases to inform management of future comparable cases. METHODS: This systematic review followed Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Medical Subject Headings (MeSH) were used to search PubMed through June 8, 2021 as follows: ((((Polycystic Kidney Diseases/complications [MeSH Terms]) OR (Multicystic Dysplastic Kidney/complications [MeSH Terms])) AND (Humans [MeSH Terms]) AND (Infant, Newborn [MeSH Terms]) AND (Case Reports [Publication Type]) AND (("multicystic dysplastic kidney") OR ("multicystic kidney")))) OR ((unusual respiratory distress in newborn [Title]) AND (kidney)) OR (large MCDK). Resulting papers were screened and included if they reported neonatal MCDK complicated by external compression by mass effect. Excluded papers lacked an MCDK diagnosis, a neonatal patient, or clear indication of mass effect. Salient data was extracted from each case for comparison. RESULTS: Of 51 papers screened, seven met inclusion criteria, presenting eight neonatal MCDK patients exhibiting varied combinations of symptoms secondary to external compression, including respiratory distress, gastrointestinal obstruction, and contralateral ureteral obstruction. All eight cases, ultimately managed with neonatal nephrectomy, had positive outcomes. Unreported and undiagnosed cases are not included in this review, making it susceptible to publication bias. CONCLUSIONS: Large neonatal MCDK is typically managed conservatively, however it can be complicated by external compression due to mass effect. In this scenario, nephrectomy is a reasonable and definitive treatment. Less invasive management options may exist, e.g., aspiration with sclerotherapy. Communication about exceptional cases like these should be encouraged even when outcomes are poor.

Primary Renal Squamous Cell Carcinoma in Native Polycystic Kidney and Ureter 16 Years After Living Donor Kidney Transplant.

We describe a case of a 55-year-old woman with polycystic kidney disease who received a living donor kidney transplant 16 years earlier and was on immunosuppressive therapy with satisfactory renal function. The donor was her mother. The patient presented with flank pain on the right side and macrohematuria, and noncontrast computed tomography and magnetic resonance imaging led to the diagnosis of tumors in the remaining right native polycystic kidney and ureter, as well as secondary retroperitoneal dissemination. We performed right radical nephrectomy and ureterectomy with extirpation of 2 metastases; the left native kidney remained intact. Histology showed squamous metaplastic changes and invasive epithelial neoplasm in the lumen of the renal pelvis and ureter with extensive squamous differentiation positive for nuclear p63 as squamous cell immunohistochemical marker. After surgery, an immunosuppressive therapy with methylprednisolone was administered, without calcineurin inhibitors and mycophenolate mofetil. Twelve months later the patient was still alive, with a glomerular filtration rate of 29 mL/min. Needs remain for further treatment modalities in patients with primary squamous cell carcinoma in nonfunctioning kidneys and improvements in imaging technique accuracy.

Liver and kidney transplantation in polycystic liver and kidney disease. / Trasplante hepático y renal en la enfermedad poliquística hepatorrenal.

OBJECTIVE: To evaluate the results of isolated liver and combined liver and kidney transplantation in a retrospective series of 32 patients with hepatorenal liver and kidney disease. MATERIALS AND METHODS: A retrospective observational study that enrolled patients with polycystic liver disease (PLD) and polycystic liver and kidney disease (PLKD) who were evaluated for transplantation between January 1999 and December 2019 at Hospital Clínic de Barcelona [Clinical Hospital of Barcelona]. RESULTS: We included a total of 53 patients enrolled, 32 (60.3%) had indication for transplantation, of which 12 received a single liver transplant and 20 received a double liver and kidney transplant. The mean age was 52 years and 83.9% of the recipients were women. The main indication for liver transplantation was disabling symptomatic hepatomegaly (93.5%). Among the postoperative complications, in the combined liver and kidney transplant group, hepatic artery thrombosis in one case and renal artery thrombosis in other were detected. In both groups there was one case of inferior vena cava lesion. Three patients presented acute cellular rejection responding to corticosteroids and one presented humoral rejection which was treated with plasmapheresis. During the follow-up period of 80 (27-121) months, the liver transplant survival rate was 100% and the kidney transplant survival rate was 90%. Two patients in the combined liver and kidney transplant group died (one due to cardiovascular causes and the other due to intestinal adenocarcinoma). CONCLUSIONS: Isolated liver transplantation or combined liver and kidney transplantation in selected patients with polycystic disease yields excellent results, with few complications, very good transplant survival and excellent patient survival (93.8%).

Long-Term Outcomes and Prognostic Factors in Kidney Transplant Recipients with Polycystic Kidney Disease.

Background: Polycystic kidney disease (PKD) accounts for approximately 15% of kidney transplants, but long-term outcomes in patients with PKD who have received a kidney transplant are not well understood. Methods: In primary recipients of kidney transplants at our center (1994-2014), we compared outcomes of underlying PKD (N=619) with other native diseases (non-PKD, N=4312). Potential factors influencing outcomes in PKD were evaluated using Cox proportional-hazards regression and a rigorous multivariable model. Results: Patients with PKD were older and were less likely to be sensitized or to experience delayed graft function (DGF). Over a median follow-up of 5.6 years, 1256 of all recipients experienced death-censored graft failure (DCGF; 115 patients with PKD) and 1617 died (154 patients with PKD). After adjustment for demographic, dialysis, comorbid disease, surgical, and immunologic variables, patients with PKD had a lower risk of DCGF (adjusted hazard ratio [aHR], 0.73; 95% CI, 0.57 to 0.93; P=0.01) and death (aHR, 0.62; 95% CI, 0.51 to 0.75; P<0.001). In our multiadjusted model, calcineurin-inhibitor (CNI) use was associated with lower risk of DCGF (aHR, 0.45; 95% CI, 0.26 to 0.76; P=0.003), whereas HLA mismatch of five to six antigens (aHR, 2.1; 95% CI, 1.2 to 3.64; P=0.009) was associated with higher likelihood of DCGF. Notably, both pretransplant coronary artery disease (CAD) and higher BMI were associated with increased risk of death (CAD, aHR, 2.5; 95% CI, 1.69 to 3.71; P<0.001; per 1 kg/m2 higher BMI, aHR, 1.07; 95% CI, 1.04 to 1.11; P<0.001), DCGF, and acute rejection. Nephrectomy at time of transplant and polycystic liver disease were not associated with DCGF/death. Incidence of post-transplant diabetes mellitus was similar between PKD and non-PKD cohorts. Conclusions: Recipients with PKD have better long-term graft and patient survival than those with non-PKD. Standard practices of CNI use and promoting HLA match are beneficial in PKD and should continue to be promoted. Further prospective studies investigating the potential benefits of CNI use and medical/surgical interventions to address CAD and the immunologic challenges of obesity are needed. Podcast: This article contains a podcast at https://dts.podtrac.com/redirect.mp3/www.asn-online.org/media/podcast/K360/2021_02_25_KID0001182019.mp3.

African American polycystic kidney patients receive higher risk kidneys, but do not face increased risk for graft failure or post-transplant mortality.

African Americans (AA) are disproportionately affected by end-stage renal disease (ESRD) and have worse outcomes following renal transplantation. Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic condition leading to ESRD necessitating transplant. We explored this population with respect to race by conducting a retrospective analysis of the UNOS database between 2005 and 2019. Our study included 10,842 (AA n = 1661; non-AA n = 9181) transplant recipients whose primary diagnosis was ADPKD. We further stratified the AA ADPKD population with respect to blood groups (AA blood type B n = 295 vs AA non-B blood type n = 1366), and also compared this cohort to AAs with a diagnosis of DM (n = 16,706) to identify unique trends in the ADPKD population. We analyzed recipient and donor characteristics, generated survival curves, and conducted multivariate analyses. African American ADPKD patients waited longer for transplants (924 days vs 747 days P < .001), and were more likely to be on dialysis (76% vs 62%; p < .001). This same group was also more likely to have AA donors (21% vs 9%; p < .001) and marginally higher KDPI kidneys (0.48 vs 0.45; p < .001). AA race was a risk factor for delayed graft function (DGF), increasing the chance of DGF by 45% (OR 1.45 95% CI 1.26-1.67; p < .001). AA race was not associated with graft failure (HR 1.10 95% CI 0.95-1.28; p = .21) or patient mortality (HR 0.84 95% CI 0.69-1.03; p = .09). Racial disparities exist in the ADPKD population. They should be continually studied and addressed to improve transplant equity.

Technical Challenges and Innovations in Kidney Transplantation: Experience With Over 5000 Cases.

Kidney transplant has been the standard-of-care treatment for patients with end-stage renal disease for many years. To expand the acceptance and care of complicated situations in patients with end-stage renal disease, transplant teams should be ready to find innovative solutions to prevent and manage pretransplant, intraoperative, and posttransplant problems. In this report, we present our approach for the following scenarios: transplant in patients with urinary diversion and augmentation, polycystic disease in recipients, tumors in transplanted kidney and native kidneys, and the roles of laparoscopy and mini-laparoscopy in kidney transplant.

Investigation of the Causes and Risk Factors of Previous End-Stage Renal Disease in Kidney Transplant Recipients.

BACKGROUND: Chronic kidney disease (CKD) is a pathophysiological process with many etiologic causes, often leading to end-stage renal disease (ESRD). The distribution of the causes that lead to ESRD varies by country, race, and sex. Renal failure may be prevented by determining these differences and reducing the risk factors. OBJECTIVE: The purpose of the study was to determine the causes and risk factors of previous ESRD in kidney transplant (KT) recipients. METHODS: In this descriptive, cross-sectional study, 393 KT recipients fitting the study criteria gave written consent to participate. Data were collected in face-to-face interviews at the Transplant Center using survey forms prepared by the researchers. RESULTS: According to a multivariate logistic regression analysis of the dependent variable of ESRD diagnosis age of KT recipients, the factors affecting ESRD diagnosis age were found to be job (Odds ratio (OD) = 5.76; 95% CI [2.291-14.481]), diabetes mellitus (DM) (OD = 2.94; 95% CI [1.143-7.571]), polycystic kidney disease (PKD) (OD = 4.55; 95% CI [1.737-11.919]), hypertension (HT) (OD = 3.53; 95% CI [2.132-5.854]), family history of ESRD (OD = 0.57; 95% CI [0.341-0.963]), surgical procedure history (OD = 1.93; 95% CI [1.150-3.230]), and stress level (OD = 5.86, 95% CI [2.212-15.528]). CONCLUSION: It is important that we determine the changeable risk factors related to ESRD development in order to prepare strategies aimed at preventing ESRD, the frequency and prevalence of which is gradually increasing. Modifiable risk factors should be identified, particularly in KT recipients, to preserve the functions of the transplanted kidney.

Budd-Chiari Syndrome after Bilateral Nephrectomy for Polycystic Kidney Disease in a Kidney Transplant Recipient.

We report a rare case of Budd-Chiari syndrome (BCS) as a postoperative complication after bilateral nephrectomy in a kidney transplant recipient with polycystic liver and kidneys. Contrast-enhanced computed tomography of the abdomen showed a narrowed inferior vena cava, compressed by the polycystic liver that moved downwards after nephrectomy. A stenting angioplasty was performed, resulting in remarkable clinical improvement. This case highlights the need for careful evaluation of polycystic kidneys and their anatomic relationship with the liver before nephrectomy, as well as for considering BCS as a differential diagnosis in similar cases.

Use of pediatric donor en bloc kidneys along with bladder segment in pediatric liver-kidney and multivisceral-kidney transplantation.

The combination of pediatric multivisceral and kidney transplantation leads to additional recipient risks due to the number of anastomoses and to the small sizes of donor structures. The inclusion of donor kidneys, ureters, and a bladder patch en bloc with multivisceral organs decreases the number and complexity of anastomoses and has not yet been reported. Four patients were transplanted in this fashion; three underwent multivisceral-kidney and one underwent liver-kidney transplantation. The first patient was a 3-year-old male with polycystic kidney disease and congenital hepatic fibrosis. The second was a 7-year-old female with complications from necrotizing enterocolitis. The third was a 12-month-old male with megacystis microcolon intestinal hypoperistalsis syndrome and secondary hydronephrosis, and the fourth was a 3-year-old male with multiple intestinal resections secondary to incarcerated hernia. The third patient developed a right ureteral stenosis with an intact bladder patch. The fourth child expired from maintained abdominal sepsis. The first 3 patients maintained normal graft function. There were no cases of thrombosis, arterial stenosis, or urinary leakages. These reported cases demonstrate that small pediatric en bloc transplantation of the multivisceral organs and dual kidneys with a bladder patch anastomosis is a feasible and less complex alternative to the standard procedure.