Oxalate Nephropathy After Kidney Transplantation: Risk Factors and Outcomes of Two Phenotypes.

Describing risk factors and outcomes in kidney transplant recipients with oxalate nephropathy (ON) may help elucidate the pathogenesis and guide treatment strategies. We used a large single-center database to identify patients with ON and categorized them into delayed graft function with ON (DGF-ON) and late ON. Incidence density sampling was used to select controls. A total of 37 ON cases were diagnosed between 1/2011 and 1/2021. DGF-ON (n = 13) was diagnosed in 1.05% of the DGF population. Pancreatic atrophy on imaging (36.4% vs. 2.9%, p = 0.002) and gastric bypass history (7.7% vs. 0%; p = 0.06) were more common in DGF-ON than with controls with DGF requiring biopsy but without evidence of ON. DGF-ON was not associated with worse graft survival (p = 0.98) or death-censored graft survival (p = 0.48). Late ON (n = 24) was diagnosed after a mean of 78.2 months. Late ON patients were older (mean age 55.1 vs. 48.4 years; p = 0.02), more likely to be women (61.7% vs. 37.5%; p = 0.03), have gastric bypass history (8.3% vs. 0.8%; p = 0.02) and pancreatic atrophy on imaging (38.9% vs. 13.3%; p = 0.02). Late ON was associated with an increased risk of graft failure (HR 2.0; p = 0.07) and death-censored graft loss (HR 2.5; p = 0.10). We describe two phenotypes of ON after kidney transplantation: DGF-ON and late ON. Our study is the first to our knowledge to evaluate DGF-ON with DGF controls without ON. Although limited by small sample size, DGF-ON was not associated with adverse outcomes when compared with controls. Late ON predicted worse allograft outcomes.

Intravenous Human Immunoglobulin in the Treatment of BK Virus Nephropathy in Kidney Transplant: A Case Report.

The BK virus infection is common in the immunocompetent population and is asymptomatic in the majority of cases. However, in renal transplant patients, reactivation and replication can occur, leading to the development of BK virus-associated nephropathy (BKVN), which is associated with renal injury and graft loss. The objective of this case report was to demonstrate a case of BKVN that showed a good response to the use of human immunoglobulin. A 37-year-old man who underwent a second transplant received rabbit-derived antithymocyte human immunoglobulin at a dose of 6 mg/kg intravenously as induction immunosuppressive therapy, and maintenance therapy with tacrolimus, prednisone, and mycophenolate sodium (MFS). At 3 months post-transplant, he presented sustained BK virus viremia (70,000-100,000 copies/mL), leading to a reduction in the dose of MFS and tacrolimus. A biopsy diagnosed BKVN class 2/B2, and viremia increased to over 1 million copies/mL at 22 months, prompting the discontinuation of tacrolimus without response. Intravenous human immunoglobulin (IVIG) was administered at 2 g/kg at 22 months and again at 33 months, with viremia peaking at 2 million copies 3 months later. However, it steadily declined to 5500 copies/mL at 52 months post-transplant. Currently, the only proven therapy for BKVN is the reduction of immunosuppression. However, in patients who do not respond, IVIG is considered as an option, with good results demonstrated in case reports, as shown here. Nevertheless, the data are based on case reports or case series, and the development of controlled clinical trials is necessary for confirmation of the efficacy.

Association of BKV viremia and nephropathy with adverse alloimmune outcomes in kidney transplant recipients.

BACKGROUND: Immunosuppression reduction for BK polyoma virus (BKV) must be balanced against risk of adverse alloimmune outcomes. We sought to characterize risk of alloimmune events after BKV within context of HLA-DR/DQ molecular mismatch (mMM) risk score. METHODS: This single-center study evaluated 460 kidney transplant patients on tacrolimus-mycophenolate-prednisone from 2010-2021. BKV status was classified at 6-months post-transplant as "BKV" or "no BKV" in landmark analysis. Primary outcome was T-cell mediated rejection (TCMR). Secondary outcomes included all-cause graft failure (ACGF), death-censored graft failure (DCGF), de novo donor specific antibody (dnDSA), and antibody-mediated rejection (ABMR). Predictors of outcomes were assessed in Cox proportional hazards models including BKV status and alloimmune risk defined by recipient age and molecular mismatch (RAMM) groups. RESULTS: At 6-months post-transplant, 72 patients had BKV and 388 had no BKV. TCMR occurred in 86 recipients, including 27.8% with BKV and 17% with no BKV (p = .05). TCMR risk was increased in recipients with BKV (HR 1.90, (95% CI 1.14, 3.17); p = .01) and high vs. low-risk RAMM group risk (HR 2.26 (95% CI 1.02, 4.98); p = .02) in multivariable analyses; but not HLA serological MM in sensitivity analysis. Recipients with BKV experienced increased dnDSA in univariable analysis, and there was no association with ABMR, DCGF, or ACGF. CONCLUSIONS: Recipients with BKV had increased risk of TCMR independent of induction immunosuppression and conventional alloimmune risk measures. Recipients with high-risk RAMM experienced increased TCMR risk. Future studies on optimizing immunosuppression for BKV should explore nuanced risk stratification and may consider novel measures of alloimmune risk.

Impact of kidney disease on perioperative outcomes of endoscopic BPH surgery: a propensity score matched analysis from the NSQIP database.

INTRODUCTION: To assess the impact of kidney function in patients with BPH undergoing surgery prior to Transurethral resection of prostate (TURP), Laser enucleation of the prostate (LEP), and Laser Vaporization of the prostate (LVP) on operative and post-operative outcomes using the ACS-NSQIP database. METHODS: The ACS-NSQIP database was reviewed for patients that underwent TURP, LEP and LVP for treatment of patients with BPH between the years of 2008 and 2021. Demographics, comorbidities, bleeding disorders, operative time, and surgical procedure performed were collected for comparison between Kidney function groups: G1, normal/high function; G2-G3, mild/moderate kidney disease; and G4-G5, severe kidney disease. The 30-day peri-operative complications were measured and a multivariate logistic regression analysis was performed while adjusting for all confounding variables. Propensity score matching was performed between the G1 and G4-G5 cohorts. RESULTS: A total of 83,020 patients were included. On multivariable regression, in the G2-G3 cohort, patients were at significantly increased risk for renal complications with OR = 2.43[1.56-3.79]. After propensity score matching, the G4-G5 cohort showed increased odds of pneumonia OR = 4.02[1.343-12.056], renal complications with OR = 7.62[2.283-25.411], cardiac complications OR = 4.53[1.531-13.411], and sepsis/septic shock OR = 1.76[1.091-2.834]. They also had a higher need for blood transfusion OR = 3.58[2.242-5.714], and prolonged hospital stay with OR = 1.49[1.296-1.723]. CONCLUSION: Pre-operative kidney disease may pose an increased risk of complications for patients undergoing endoscopic BPH surgery. The literature lacks information on the effect of pre-operative kidney disease on endoscopic BPH surgeries. Further studies are required to compare post-operative outcomes of LEP and LVP as compared to TURP across kidney function status.

Management of Nontraumatic Spontaneous Renal Hemorrhage (Wünderlich Syndrome) through Robotic-Assisted Laparoscopic Nephrectomy: A Case Series.

BACKGROUND Wünderlich syndrome (WS) is a rare diagnosis of nontraumatic spontaneous renal hemorrhage into the subcapsular, perirenal, or pararenal spaces. Prompt and effective intervention is necessary for an accurate pathological diagnosis and preservation of life. In the current literature, open surgery is the primary option when conservative treatment fails, but there can be serious trauma and corresponding consequences. Herein, we present 3 cases of Wünderlich syndrome managed by robot-assisted laparoscopic nephrectomy via a retroperitoneal approach. CASE REPORT Patient 1 was a 44-year-old woman with right flank pain for 6 h. Patient 2 was a 53-year-old woman with a history of diabetes who had pain in her right flank pain and nausea for 1 day. Patient 3 was a 45-year-old man with left flank pain for 1 day. All cases of WS were confirmed by CT. All 3 patients were treated with retroperitoneal robot-assisted nephrectomy after conservative treatment failed. Pathological examination confirmed that patient 1 had angiomyolipoma, and patients 2 and 3 had renal clear cell carcinoma. At the 9-month follow-up, renal function was good and no evidence of recurrence or metastasis has been detected. CONCLUSIONS These cases have highlighted the importance of the clinical history and imaging findings in the diagnosis of Wünderlich syndrome, and show that rapid management can be achieved using robot-assisted laparoscopic nephrectomy. However, it is crucial to have a skilled surgical team and adequate preoperative preparation.

Recurrent disease after pediatric renal transplantation.

BACKGROUND: Recurrent disease after kidney transplant remains an important cause of allograft failure, accounting for 7-8% of graft loss and ranking as the fifth most common cause of allograft loss in the pediatric population. Although the pathophysiology of many recurrent diseases is incompletely understood, recent advances in basic science and therapeutics are improving outcomes and changing the course of several of these conditions. METHODS: Review of the literature. RESULTS: We discuss the diagnosis and management of recurrent disease. CONCLUSION: We highlight new insights into the pathophysiology and treatment of post-transplant primary hyperoxaluria, focal segmental glomerulosclerosis, immune complex glomerulonephritis, C3 glomerulopathy, lupus nephritis, atypical hemolytic uremic syndrome, and IgA nephropathy.

Concurrent JCPyV-DNAemia Is Correlated With Poor Graft Outcome in Kidney Transplant Recipients With Polyomavirus-associated Nephropathy.

BACKGROUND: Co-infection of JC polyomavirus (JCPyV) and BK polyomavirus (BKPyV) is uncommon in kidney transplant recipients, and the prognosis is unclear. This study aimed to investigate the effect of concurrent JCPyV-DNAemia on graft outcomes in BKPyV-infected kidney transplant recipients with polyomavirus-associated nephropathy (PyVAN). METHODS: A total of 140 kidney transplant recipients with BKPyV replication and PyVAN, 122 without concurrent JCPyV-DNAemia and 18 with JCPyV-DNAemia were included in the analysis. Least absolute shrinkage and selection operator regression analysis and multivariate Cox regression analysis were used to identify prognostic factors for graft survival. A nomogram for predicting graft survival was created and evaluated. RESULTS: The median tubulitis score in the JCPyV-DNAemia-positive group was higher than in JCPyV-DNAemia-negative group ( P  = 0.048). At last follow-up, the graft loss rate in the JCPyV-DNAemia-positive group was higher than in the JCPyV-DNAemia-negative group (50% versus 25.4%; P  = 0.031). Kaplan-Meier analysis showed that the graft survival rate in the JCPyV-DNAemia-positive group was lower than in the JCPyV-DNAemia-negative group ( P  = 0.003). Least absolute shrinkage and selection operator regression and multivariate Cox regression analysis demonstrated that concurrent JCPyV-DNAemia was an independent risk factor for graft survival (hazard ratio = 4.808; 95% confidence interval: 2.096-11.03; P  < 0.001). The nomogram displayed favorable discrimination (C-index = 0.839), concordance, and clinical applicability in predicting graft survival. CONCLUSIONS: Concurrent JCPyV-DNAemia is associated with a worse graft outcome in BKPyV-infected kidney transplant recipients with PyVAN.

Penile Agenesis Associated with Anorectal Malformation and Bilateral Renal Agenesis: A Case Report and a Review of the Literature.

ABSTRACT: Penile agenesis is complete absence of the penis in an otherwise normal phenotypic and genotypic male at birth that results from failure of development of the genital tubercle. It is an extremely rare anomaly that may be associated with anomalies in other organ systems, the extent and severity of which may affect the prognosis. The management is challenging and may have far reaching implications for the individual and family. While gender reassignment with bilateral orchidectomy and feminising genitoplasty has been carried out for most patients, significant psychosexual and social issues related to the male identity may occur due to foetal or postnatal sex steroid imprinting. We report a neonate with penile agenesis with bilateral renal agenesis and anorectal malformation.

Predictive Value of the Modified Mehran Score for Contrast-Induced Nephropathy After Transcatheter Aortic Valve Implantation.

Considering the increasing use of the transcatheter aortic valve implantation (TAVI) procedure, the relationship of contrast-induced nephropathy (CIN) with post-TAVI mortality has become important. The Mehran score was developed to detect the risk of CIN development after cardiac intervention. We aimed to compare the role of the modified Mehran score, which can be calculated pre-procedure, in predicting CIN development and compare it with the original Mehran score. We retrospectively collected data from TAVI procedures at our institution between December 2016 and June 2021; of 171 patients, 44 (25.7%) had CIN. We found no association between contrast media volume and CIN (387 ± 120 vs 418 ± 139 mL, P = .303). High and very high modified Mehran score and preoperative C-reactive protein (CRP) level were independent risk factors for CIN development after TAVI procedure. The area under curve (AUC) was .686 with 95% CI: .591-.780 and P < .001, and also, with a cut-off point of >7.5 points, there was 79.5% sensitivity and 63.0% specificity; otherwise, with a cut-off point of >9.5 points, there was 54.5% sensitivity and 71.7% specificity, for the modified Mehran score. The modified Mehran score comes into prominence compared with the original Mehran score since it can be calculated pre-procedure.

Native nephrectomy in advanced pediatric kidney disease: indications, timing, and surgical approaches.

In pediatric kidney failure, native kidneys may pose a risk to successful transplant outcomes. The indications and timing of native nephrectomy represent a controversial management decision. A lack of high-quality, outcomes-based data has prevented development of evidence-based guidelines for intervention. In this article, we review the published literature on medical indications for native nephrectomy and current knowledge gaps. In addition, we provide a surgical perspective regarding timing and approach.

Treatment of BK Polyomavirus-Associated Nephropathy in Paediatric Kidney Transplant Recipients: Leflunomide Versus Cidofovir.

OBJECTIVES: BK polyomavirus-associated nephropathy is a clinicopathological entity that negatively affects graft function in kidney transplant recipients. We compared the efficacy of leflunomide and cidofovir to treat BK polyomavirus-associated nephropathy in pediatric kidney transplant recipients. MATERIALS AND METHODS: Medical records of pediatric recipients with BK viremia for the period 2004 through 2019 were reviewed retrospectively, and patients diagnosed with BK polyomavirusassociated nephro-pathy were included in the study. A serum BK virus level above 104 copies/mL was accepted as BK viremia. We defined BK polyomavirusassociated nephropathy as detection of BK virus SV40 antigen on immunochemistry staining of renal graft tissue accompanied by signs of tubulointerstitial nephritis or elevated serum creatinine in addition to BK viremia. RESULTS: Of 304 kidney transplant recipients, 53 had persistent BK viremia; 36 of these patients (61.1% male) were included in the study with the diagnosis of BK polyomavirus-associated nephropathy. Twelve patients (33.3%) received cidofovir, and 14 (38.8%) received leflunomide. Results were similar between the cidofovir and leflunomide groups for serum creatinine level at last follow-up (0.91 ± 0.29 vs 0.94 ± 0.37 mg/dL, respectively; P = .843) and graft failure rate (8.3% vs 14.2%, respectively; P = .632). Graft failure was observed in 8.3% of patients with BK polyomavirus-associated nephropathy. CONCLUSIONS: Leflunomide and cidofovir showed similar efficacy for treatment of BK polyomavirus-associated nephropathy.

Pre-operative factors associated with the development of distal ureteral stump syndrome after upper pole heminephrectomy.

INTRODUCTION: For children with duplex systems and severe hydroureteronephrosis of the upper pole, heminephrectomy is one of many suitable treatments, particularly if there is no associated lower pole reflux. Distal ureteral stump syndrome (DSS) is a very difficult complication and manifests as stump empyema, urinary tract infection and/or vulvar discharge and can occur months to years later in 10-20 percent of patients. Secondary distal ureterectomy is an extremely difficult surgery due to inflammation and adhesions. To avoid DSS, distal ureterectomy at the time of heminephrectomy can be performed concurrently but carries a risk of lower pole ureter devascularization and injury. Current literature on DSS has shown associations with subtotal ureterectomy or long ureteral stumps. We hypothesized that there may be preoperative variables prior to heminephrectomy that are associated with the development of DSS. OBJECTIVE: Identify pre-operative risk factors for the development of DSS in pediatric patients who underwent upper pole heminephrectomy for duplex kidneys. STUDY METHODS: Retrospective analysis of pediatric patients who underwent upper pole heminephrectomy at single, academic institution from 1999 to 2021. Pre-operative patient age, gender, history, imaging, and lab results were extracted from patient charts to assess for factors that may predict the development of DSS. Patient groups with and without DSS were compared using Fischer's Exact Test. RESULTS: Five (14%) of 36 patients developed DSS and required secondary distal ureterectomy at a median time of 22 months (IQR 6-27) after heminephrectomy. The presence of ureteral debris (80% of DSS) on preoperative ultrasound (p < 0.001), reflux into the upper pole (p = 0.005), and mucus discharge (100% of DSS) (p < 0.001) prior to surgery were found to be significantly associated with those who developed DSS, compared to those who did not. These three pre-operative factors had high specificity (97-100%) and negative predictive value (94-97%). DISCUSSION: Substantial experience has shown that less than 20% of patients benefit from distal ureterectomy during upper heminephrectomy. Whether using an open or laparoscopic approach, selection of at-risk patients should lower operative time and avoid injury and devascularization of the lower pole ureter for most patients. CONCLUSION: The presence or absence of ureteral debris, mucus discharge and/or upper pole reflux prior to heminephrectomy may be useful guides in selecting which patients would benefit from concurrent distal ureterectomy and conversely which patients may safely avoid the additional dissection.

Multidisciplinary and multidimensional approaches to transplantation in children with rare genetic kidney diseases.

In this review, we describe the multidisciplinary, multidimensional care required to optimize outcomes for pediatric transplant recipients with rare genetic kidney diseases. Transplant success, recipient survival, and improvement in quality of life depend on collaboration between patients, families, and a team of specialists with medical, as well as nonmedical expertise. A multidisciplinary transplant team composed of experts from medicine, surgery, nursing, nutrition, social services, transplant coordination, psychology, and pharmacology, is now standard in most transplant centers and is critical to the success of a transplant. In addition to these professionals, other specialists, such as cardiologists, urologists, geneticists, metabolic disease specialists, occupational therapists, case management, child life, chaplain, and palliative care services, have a crucial role to play in the preparation, surgery, and follow-up care, especially when a pediatric patient has a rare genetic disorder leading to renal involvement, and the need for transplantation. In order to describe this multidisciplinary care, we divide the genetic renal diseases into five subgroups-metabolic and tubular disorders, glomerular diseases, congenital anomalies of the kidney and urinary tract, ciliopathies including cystic diseases, and miscellaneous renal conditions; and describe for each, the need for care beyond that provided by the standard transplant team members.

Minimally invasive approach to Herlyn-Werner-Wunderlich syndrome with giant endometriotic cyst: a case report.

Herlyn-Werner-Wunderlich syndrome is a rare congenital anomaly of the Mullerian and Wolffian ductal system, manifesting as a triad of dipelphys uterus, obstructed hemivagina, and ipsilateral renal agenesis. Patients usually remain asymptomatic till menarche and experience progressive dysmenorrhea, suprapubic lump, and/or features of infection (pyometra, pelvic collection, etc.) afterward. We hereby present a case of a young lady with Herlyn-Werner-Wunderlich syndrome with a large endometriotic cyst, likely arising from the right hemiuterus. She presented with dysmenorrhea and progressive abdominal distention for seven years. She was treated by laparoscopic ovarian cyst excision and right hemihysterectomy that relieved her symptoms.

Endometriosis in an ectopic kidney: a rare case report and literature review.

BACKGROUND: Endometriosis mainly occurs in female pelvic organs. Endometriosis in the kidney is extremely rare. CASE PRESENTATION: We herein describe a case of a 19-year-old girl with occasional mild abdominal pain associated with an ectopic left kidney. SPECT-CT showed no abnormal radioactive distribution in the left pelvis, suggesting loss of function of the ectopic kidney. Laparoscopic left ectopic kidney resection was subsequently performed. Histopathology revealed endometriosis of the ectopic left kidney. CONCLUSIONS: In female patients with clinical manifestations of abdominal pain and gross hematuria, the possibility of renal endometriosis should be considered.

BK DNAemia and native kidney polyomavirus nephropathy following lung transplantation.

BK virus DNAemia (BKPyV) and nephropathy are common after kidney transplant; however, there are limited data on BK infections in nonrenal solid organ transplant recipients. We examined the frequency, clinical and pathologic features, and kidney and lung outcomes of BKPyV and BK virus native kidney nephropathy (BKVN) in lung transplant recipients at our center. Among 878 recipients transplanted from 2003 to 2019, 56 (6%) developed BKPyV at a median of 30.1 months after transplant (range, 0.6-213) and 11 (1.3%) developed BKVN at a median of 46 months after transplant (range, 9-213). The incidence of end-stage kidney disease was significantly higher in patients with peak viral load ≥10 000 copies/mL (39% vs 8%, P < .001). All cases of BKVN were in patients with peak viral load of ≥10 000 copies/mL, and 55% of these patients developed end-stage kidney disease. Despite the reduction of immunosuppression to treat BKVN, only 1 patient developed acute rejection, and lung function was stable >1 year. BKPyV and nephropathy are more common after lung transplantation than previously reported. Routine screening for BKPyV should be considered in all lung transplant recipients.

Evaluation of non-hematologic toxicity in patients with cervical cancer after Type 3 radical hysterectomy followed by concurrent chemoradiotherapy: a retrospective study.

OBJECTIVE: To investigate the safety of concurrent chemoradiotherapy after Type 3 radical hysterectomy, focusing on non-hematologic toxicity. METHODS: Between January 2010 and December 2017, 236 patients diagnosed with cervical cancer Stages IB1-II (FIGO2008) and who had undergone Type 3 radical hysterectomy at the Jikei Medical University School-related four hospitals were included. Of these 236 patients, 134 had undergone adjuvant concurrent chemoradiotherapy after Type 3 radical hysterectomy (radical hysterectomy + concurrent chemoradiotherapy group), and 102 received no adjuvant therapy after Type 3 radical hysterectomy (radical hysterectomy group). The frequency of non-hematologic toxicities, especially lymphedema, pelvic infection, renal dysfunction, ileus and diarrhea, was investigated in the radical hysterectomy + concurrent chemoradiotherapy and radical hysterectomy groups using univariate and multivariate analyses. In these analyses, age, extent of lymph node dissection and preoperative clinical stage were included as risk factors for five complications. The risk factors for grade ≤ 2 adverse events were statistically evaluated. RESULTS: The frequency of lower extremity lymphedema (22 vs. 10%), renal dysfunction (13 vs. 3%), and diarrhea (13 vs. 0%) was significantly higher in the radical hysterectomy + CRRT group than that in the radical hysterectomy group. Logistic regression analysis revealed that adjuvant concurrent chemoradiotherapy significantly affected the occurrence of grade ≥ 2 lymphedema (P < 0.01) and renal dysfunction (P < 0.01). CONCLUSIONS: Concurrent chemoradiotherapy after Type 3 radical hysterectomy is associated with a higher incidence of renal dysfunction, lower extremity lymphedema and diarrhea. A more appropriate adjuvant therapy needs to be established.