Coexisting presentation of two rare genetic variants of autosomal dominant polycystic kidney disease and Alport syndrome.

Alport syndrome and autosomal dominant polycystic kidney disease are monogenic causes of chronic kidney disease and end-stage kidney failure. We present a case of a man in his 60s with progressive chronic kidney disease, bilateral sensorineural hearing loss and multiple renal cysts. Genetic analysis revealed a heterozygous variant in COL4A3 (linked to Alport syndrome) and in the GANAB gene (associated with a milder form of autosomal dominant polycystic kidney disease). Although each variant confers a mild risk of developing end-stage kidney disease, the patient presented a pronounced and accelerated progression of chronic kidney disease, which goes beyond what would be predicted by adding up their individual effects. This suggests a potential synergic effect of both variants, which warrants further investigation.

Is aortic knob width a novel predictor of mortality in hemodialysis patients?

OBJECTIVE: Identifying reliable predictors of mortality in end-stage renal disease patients is crucial for patient outcomes. Aortic knob width is a radiographic parameter used to assess cardiovascular diseases and atherosclerosis. This study investigated the association between aortic knob width and mortality in hemodialysis patients. PATIENTS AND METHODS: The study included data collected between 2007 and 2022 from 103 patients aged between 18 and 85 who had been undergoing hemodialysis treatment for at least one year. Patients were divided into two groups: survivors and deceased. The aortic knob width was measured using a posterior-anterior chest radiograph after midweek hemodialysis. The relationship between aortic knob width and mortality was investigated. RESULTS: Deceased patients had significantly larger aortic knob widths compared with survivors. The deceased group's hemodialysis (HD) duration was shorter, median age was older, Kt/V, hemoglobin, and albumin levels were lower, and the frequency of patients with hypertension, diabetes, and aortic wall calcification was higher. Aortic knob width greater than 37.98 mm was identified as a predictor of mortality in hemodialysis patients. Survival rates for aortic knob width <37.98 mm are 98.1% for 1 year and 64.9% for 15 years. For aortic knob width larger than 37.98 mm, survival rates are 88% for three years, 68% for five years, 45.2% for ten years, and 25% for fifteen years. The most important risk factors for increased aortic knob width were age, male sex, aortic calcification, and hypertension. CONCLUSIONS: Age, male gender, aortic calcification, and hypertension are the primary risk factors for increased aortic knob width in hemodialysis patients. Aortic knob width greater than 37.98 mm, which can be measured simply and rapidly using posterior-anterior chest radiography, may be a predictor of mortality. Graphical Abstract: https://www.europeanreview.org/wp/wp-content/uploads/Graphical-Abstract-10.jpg.

Low platelet count at diagnosis of anti-neutrophil cytoplasmic antibody-associated vasculitis is correlated with the severity of disease and renal prognosis.

Antineutrophil cytoplasmic antibody-associated vasculitis (AAV) is an autoimmune disease that involves inflammation of blood vessels. There is increasing evidence that platelets play a crucial role not only in hemostasis but also in inflammation and innate immunity. In this study, we explored the relationship between platelet count, clinical characteristics, and the prognosis of patients with AAV. We divided 187 patients into two groups based on their platelet count. Clinicopathological data and prognostic information were retrospectively gathered from medical records. Univariate and multivariate regression analyses were used to identify risk factors for prognosis, including end-stage renal disease (ESRD) and mortality. The cutoff point for platelet count was set at 264.5 × 109/L, as determined by the receiver operating characteristic (ROC) curve for predicting progression to ESRD in patients with AAV. We observed patients with low platelet count (platelets < 264.5 × 109/L) had lower leukocytes, hemoglobin, complement, acute reactants, and worse renal function (P for eGFR < 0.001). They were also more likely to progress to ESRD or death compared to the high platelet count group (platelets > 264.5 × 109/L) (P < 0.0001, P = 0.0338, respectively). Low platelet count was potentially an independent predictor of poor renal prognosis in the multivariate regression analysis [HR 1.670 (95% CI 1.019-2.515), P = 0.014]. Lower platelet count at diagnosis is associated with more severe clinical characteristics and impaired renal function. Therefore, platelet count may be an accessible prognostic indicator for renal outcomes in patients with AAV.

A new index for the outcome of focal segmental glomerulosclerosis.

Focal segmental glomerulosclerosis (FSGS) is a common pathological form of nephrotic syndrome. This study analyzed the value of pathological lesions and clinical prognosis of different segmental glomerulosclerosis ratios in FSGS. Two hundred and six FSGS patients were collected from Dec 2013 to Apr 2016. The patients were divided into two groups according to the proportion of glomerular segmental sclerosis: F1 (SSR ≤ 15%, n = 133) and F2 (SSR > 15%, n = 73). The clinical and pathological data were recorded and analyzed, and statistical differences were observed between the serum uric acid level and the percentage of chronic renal failure. The pathological results showed significant differences in interstitial fibrosis and tubular atrophy (IFTA), degree of mesangial hyperplasia, vascular lesions, synaptopodin intensity, and foot process effacement between the two groups. Multivariate logistic regression analysis showed significant differences in creatinine (OR: 1.008) and F2 group (OR: 1.19). In all patients, the prognoses of urine protein and serum creatinine levels were statistically different. Multivariate Cox regression analysis revealed that F2 (hazard ratio: 2.306, 95% CI 1.022-5.207) was associated with a risk of ESRD (end stage renal disease). The proportion of segmental glomerulosclerosis provides a guiding value in the pathological diagnosis and clinical prognosis of FSGS.

Effects of rare kidney diseases on kidney failure: a longitudinal analysis of the UK National Registry of Rare Kidney Diseases (RaDaR) cohort.

BACKGROUND: Individuals with rare kidney diseases account for 5-10% of people with chronic kidney disease, but constitute more than 25% of patients receiving kidney replacement therapy. The National Registry of Rare Kidney Diseases (RaDaR) gathers longitudinal data from patients with these conditions, which we used to study disease progression and outcomes of death and kidney failure. METHODS: People aged 0-96 years living with 28 types of rare kidney diseases were recruited from 108 UK renal care facilities. The primary outcomes were cumulative incidence of mortality and kidney failure in individuals with rare kidney diseases, which were calculated and compared with that of unselected patients with chronic kidney disease. Cumulative incidence and Kaplan-Meier survival estimates were calculated for the following outcomes: median age at kidney failure; median age at death; time from start of dialysis to death; and time from diagnosis to estimated glomerular filtration rate (eGFR) thresholds, allowing calculation of time from last eGFR of 75 mL/min per 1·73 m2 or more to first eGFR of less than 30 mL/min per 1·73 m2 (the therapeutic trial window). FINDINGS: Between Jan 18, 2010, and July 25, 2022, 27 285 participants were recruited to RaDaR. Median follow-up time from diagnosis was 9·6 years (IQR 5·9-16·7). RaDaR participants had significantly higher 5-year cumulative incidence of kidney failure than 2·81 million UK patients with all-cause chronic kidney disease (28% vs 1%; p<0·0001), but better survival rates (standardised mortality ratio 0·42 [95% CI 0·32-0·52]; p<0·0001). Median age at kidney failure, median age at death, time from start of dialysis to death, time from diagnosis to eGFR thresholds, and therapeutic trial window all varied substantially between rare diseases. INTERPRETATION: Patients with rare kidney diseases differ from the general population of individuals with chronic kidney disease: they have higher 5-year rates of kidney failure but higher survival than other patients with chronic kidney disease stages 3-5, and so are over-represented in the cohort of patients requiring kidney replacement therapy. Addressing unmet therapeutic need for patients with rare kidney diseases could have a large beneficial effect on long-term kidney replacement therapy demand. FUNDING: RaDaR is funded by the Medical Research Council, Kidney Research UK, Kidney Care UK, and the Polycystic Kidney Disease Charity.

Association between physical activity and quality of life in haemodialysed and peritoneal dialyzed patients in Hungary.

BACKGROUND: Patients with end-stage kidney disease (ESKD) receiving peritoneal dialysis (PD) or haemodyalisis (PD) appear to be less physically active than healthy persons, a situation that could lead to reductions in quality of life. The aim of the present study was to assess and compare physical activity and health-related quality of life in renal patients on HD and PD programs. METHODS: In May 2020, 130 patients (106 HD and 24 PD) were enrolled in a study of chronic dialysis programs. All participants received a questionnaire containing information on demographics, treatment, and co-morbidities. Physical activity was assessed using the International Physical Activity Questionnaire (IPAQ) short form, and quality of life was measured using the Kidney Disease Quality of Life-Short Form 12 (KDQOL-SF-12) questionnaire comprising mental (MCS) and physical components (PCS). Non-parametric statistical tests were executed with 0.05 as the level of significance. RESULTS: The physical activity of patients treated in both HD and PD programs could be considered as low, without a statistically significant difference between the two modalities. For the quality of life measures, we found a significant (p = .004) difference regarding Physical Component Summary (PCS) scores, with higher PCS scores in patients treated in the PD programme compared to HD. Furthermore, higher physical activity levels were associated with better quality of life parameters in both groups. CONCLUSION: This study confirms the importance of physical activity among dialysis patients with ESKD, suggesting that greater activity could be associated with a better quality of life.

The Effect of Aortic Angulation on Clinical Outcomes of Patients Undergoing Transcatheter Aortic Valve Replacement.

INTRODUCTION: The aim of this study was to assess the impact of aortic angulation (AA) on periprocedural and in-hospital complications as well as mortality of patients undergoing Evolut™ R valve implantation. METHODS: A retrospective study was conducted on 264 patients who underwent transfemoral-approach transcatheter aortic valve replacement with self-expandable valve at our hospital between August 2015 and August 2022. These patients underwent multislice computer tomography scans to evaluate AA. Transcatheter aortic valve replacement endpoints, device success, and clinical events were assessed according to the definitions provided by the Valve Academic Research Consortium-3. Cumulative events included paravalvular leak, permanent pacemaker implantation, new-onset stroke, and in-hospital mortality. Patients were divided into two groups, AA ≤ 48° and AA > 48°, based on the mean AA measurement (48.3±8.8) on multislice computer tomography. RESULTS: Multivariable logistic regression analysis was performed to identify predictors of cumulative events, utilizing variables with a P-value < 0.2 obtained from univariable logistic regression analysis, including AA, age, hypertension, chronic renal failure, and heart failure. AA (odds ratio [OR]: 1.73, 95% confidence interval [CI]: 0.89-3.38, P=0.104), age (OR: 1.04, 95% CI: 0.99-1.10, P=0.099), hypertension (OR: 1.66, 95% CI: 0.82-3.33, P=0.155), chronic renal failure (OR: 1.82, 95% CI: 0.92-3.61, P=0.084), and heart failure (OR: 0.57, 95% CI: 0.27-1.21, P=0.145) were not found to be significantly associated with cumulative events in the multivariable logistic regression analysis. CONCLUSION: This study demonstrated that increased AA does not have a significant impact on intraprocedural and periprocedural complications of patients with new generation self-expandable valves implanted.

Transplantation: platform to study recurrence of disease.

Beyond the direct benefit that a transplanted organ provides to an individual recipient, the study of the transplant process has the potential to create a better understanding of the pathogenesis, etiology, progression and possible therapy for recurrence of disease after transplantation while at the same time providing insight into the original disease. Specific examples of this include: 1) recurrence of focal segmental glomerulosclerosis (FSGS) after kidney transplantation, 2) recurrent autoimmunity after pancreas transplantation, and 3) recurrence of disease after orthotopic liver transplantation (OLT) for cirrhosis related to progressive steatosis secondary to jejuno-ileal bypass (JIB) surgery. Our team has been studying these phenomena and their immunologic underpinnings, and we suggest that expanding the concept to other pathologic processes and/or transplanted organs that harbor the risk for recurrent disease may provide novel insight into the pathogenesis of a host of other disease processes that lead to organ failure.

Prognostic value of transthoracic echocardiography score for the prognosis of continuous ambulatory peritoneal dialysis patients.

BACKGROUND: We devoted ourselves to proving that the initial transthoracic echocardiography score (TTES) had predictive significance for patients with continuous ambulatory peritoneal dialysis (CAPD). METHODS: In this retrospective analysis, 274 CAPD patients who had PD therapy were recruited sequentially. TTE exams were performed three months following the start of PD therapy. All patients were divided into two groups based on the strength of their TTES levels. TTES's predictive value for CAPD patients was then determined using LASSO regression and Cox regression. RESULTS: During a median of 52 months, 46 patients (16.8%) died from all causes, and 32 patients (11.7%) died from cardiovascular disease (CV). The TTES was computed as follows: 0.109 × aortic root diameter (ARD, mm) - 0.976 × LVEF (> 55%, yes or no) + 0.010 × left ventricular max index, (LVMI, g/m2) + 0.035 × E/e' ratio. The higher TTES value (≥ 3.7) had a higher risk of all-cause death (hazard ratio, HR, 3.70, 95% confidence index, 95%CI, 1.45-9.46, P = 0.006) as well as CV mortality (HR, 2.74, 95%CI 1.15-19.17, P = 0.042). Moreover, the TTES had an attractive predictive efficiency for all-cause mortality (AUC = 0.762, 95%CI 0.645-0.849) and CV mortality (AUC = 0.746, 95%CI 0.640-0.852). The introduced nomogram, which was based on TTES and clinical variables, exhibited a high predictive value for all-cause and CV mortality in CAPD patients. CONCLUSION: TTES is a pretty good predictor of clinical outcomes, and the introduced TTES-based nomogram yields an accurate prediction value for CAPD patients.

Population-wide long-term study of incidence, renal failure, and mortality rates for lupus nephritis.

OBJECTIVE: Given limited regional data, we investigate the state-wide epidemiology, renal and patient outcomes for lupus nephritis (LN) in Western Australia (WA). METHODS: Patients hospitalized with incident SLE (≥2 diagnostic codes in the state-wide WA Health Hospital Morbidity Data Collection) in the period 1985-2015 were included (n = 1480). LN was defined by the presence of glomerulonephritis and/or raised serum creatinine. Trends over three study decades for annual incidence rate (AIR)/100.000 population, mortality (MR), and end-stage renal disease (ESRD) rates/100 person years were analyzed by least square regression and compared with a matched control group (n = 12 840). RESULTS: Clinical evidence of LN developed in 366 SLE patients (25.9%) after a median disease duration of 10 months (IQR 0-101) with renal biopsy performed in 308 (84.2%). The AIR for LN (0.63/100.000) did not change significantly over time (R2 = .11, p = .85), while point prevalence reached 11.9/100.000 in 2015. ESRD developed in 14.1% (n = 54) of LN patients vs. 0.2% in non-LN SLE patients and 0.05% in controls (all p ≤ 0.01). ESRD rates increased over time in LN patients (0.4 to 0.7, R2 = .52, p = .26). The odds ratio for death was 8.81 (CI 3.78-22.9) for LN and 6.62 (CI 2.76-17.9) for non-LN SLE patients compared to controls and MR for LN patients increased over time (1.3 to 2.2, R2 = .84, p = .26). CONCLUSIONS: The incidence rate of LN in WA remained unchanged over 30 years. A lack of improvement in renal failure and mortality rates illustrates the pressing need for better long-term treatment options and/or strategies in LN.

External Validation of the International Prognosis Prediction Model of IgA Nephropathy.

BACKGROUND: The International IgA Nephropathy (IgAN) Network developed and validated two prognostic prediction models for IgAN, one incorporating a race parameter. These models could anticipate the risk of a 50% reduction in estimated glomerular filtration rate (eGFR) or progression to end-stage renal disease (ESRD) subsequent to an IgAN diagnosis via renal biopsy. This investigation aimed to validate the International IgA Nephropathy Prediction Tool (IIgANPT) within a contemporary Chinese cohort. METHODS: Within this study,185 patients diagnosed with IgAN via renal biopsy at the Center for Kidney Disease, Second Affiliated Hospital of Nanjing Medical University, between January 2012 and December 2021, were encompassed. Each patient's risk of progression was assessed utilizing the IIgANPT formula. The primary outcome, a 50% decline in eGFR or progression to ESRD, was examined. Two predictive models, one inclusive and the other exclusive of a race parameter, underwent evaluation via receiver-operating characteristic (ROC) curves, subgroup survival analyses, calibration plots, and decision curve analyses. RESULTS: The median follow-up duration within our cohort spanned 5.1 years, during which 18 patients encountered the primary outcome. The subgroup survival curves exhibited distinct separations, and the comparison of clinical and histological characteristics among the risk subgroups revealed significant differences. Both models demonstrated outstanding discrimination, evidenced by the areas under the ROC curve at five years: 0.882 and 0.878. Whether incorporating the race parameter or not, both prediction models exhibited acceptable calibration. Decision curve analysis affirmed the favorable clinical utility of both models. CONCLUSIONS: Both prognostic risk evaluation models for IgAN exhibited remarkable discrimination, sound calibration, and acceptable clinical utility.

Low rate of severe-end-stage kidney disease after SABR for localised primary kidney cancer.

BACKGROUND: Stereotactic ablative body radiotherapy (SABR) is an emerging treatment for patients with primary renal cell carcinoma (RCC). However, its impact on renal function is unclear. This study aimed to evaluate incidence and clinical factors predictive of severe to end-stage chronic kidney disease (CKD) after SABR for RCC. METHODS AND MATERIALS: This was a Single institutional retrospective analysis of patients with diagnosed primary RCC receiving SABR between 2012-2020. Adult patients with no metastatic disease, baseline estimated glomerular filtration rate (eGFR) of ≥ 30 ml/min/1.73 m2, and at least one post-SABR eGFR at six months or later were included in this analysis. Patients with upper tract urothelial carcinoma were excluded. Primary outcome was freedom from severe to end-stage CKD, determined using the Kaplan-Meier estimator. The impact of baseline CKD, age, hypertension, diabetes, tumor size and fractionation schedule were assessed by Cox proportional hazard models. RESULTS: Seventy-eight consecutive patients were included, with median age of 77.8 years (IQR 70-83), tumor size of 4.5 cm (IQR 3.9-5.8) and follow-up of 42.2 months (IQR 23-60). Baseline median eGFR was 58 mls/min; 55% (n = 43) of patients had baseline CKD stage 3 and the remainder stage 1-2. By last follow-up, 1/35 (2.8%) of baseline CKD 1-2, 7/27 (25.9%) CKD 3a and 11/16 (68.8%) CKD 3b had developed CKD stage 4-5. The estimated probability of freedom from CKD stage 4-5 at 1 and 5 years was 89.6% (CI 83.0-97.6) and 65% (CI 51.4-81.7) respectively. On univariable analysis, worse baseline CKD (p < 0.0001) and multi-fraction SABR (p = 0.005) were predictive for development of stage 4-5 CKD though only the former remained significant in multivariable model. CONCLUSION: In this elderly cohort with pre-existing renal dysfunction, SABR achieved satisfactory nephron sparing with acceptable rates of severe to end-stage CKD. It can be an attractive option in patients who are medically inoperable.

Second and Third Kidney Transplants.

Renal transplant is the best procedure for patients with end-stage renal disease. Although an ideal kidney transplant should survive for the lifetime of each recipient, there may be a need for a second, third, or even a fourth retransplant. The outcomes of these kidney allografts, surgical approaches, immunology issues, and drug therapies warrant greater focus. Pediatric kidney retransplant is even more important because these patients are more immunologically responsive to donor antigens and because they need longer allograft survival. Although kidney retransplant provides a survival advantage for patients who would otherwise remain on the wait list and/or hemodialysis, careful patient selection is crucial for second, third, and fourth renal transplants. Despite the shortage of donor organs, outcomes, manageable complications, and economic considerations support earlier kidney retransplants rather than delayed retransplants. Preoperative vascular imaging, appropriate induction therapy, regular monitoring of renal function, and regular surveillance for malignancy and infection are more important in the retransplanted kidneys than in cases of first kidney transplants. The lack of robust data on optimal clinical management of these retransplant recipients has contributed to substantial variations in clinical practice among different centers. In this review, we discuss medical and surgical approaches in the cases of second and third kidney transplants.

Sleep Disorders in Kidney Transplant Recipients.

OBJECTIVES: Kidney transplant is the primary treatment option for end-stage renal disease, owing to its favorable outcomes in terms of survival, healthcare expenses, and overall quality of life. However, sleep disturbances are common among patients with chronic kidney disease and may not always improve after kidney transplant. This review aims to summarize the available literature pertaining to sleep issues in kidney transplant recipients. MATERIALS AND METHODS: We conducted a comprehensive search by using PubMed and Ulakbim databases, without imposing any restrictions on publication dates. The primary objective of the search was to identify relevant studies involving the keywords "kidney transplantation," "sleep disorders," and "nursing care." RESULTS: Restful sleep is a key component in the recovery process after kidney transplant. Posttransplant physical conditions should be evaluated in terms of the side effects of surgery and drugs, as well as other factors that may have serious effects on the sleep cycle. The frequency of insufficient sleep before and after kidney transplant was shown to be 37.5% before transplant, 37.5% after 3 months, and 20.0% after 6 months. In addition, both kidney transplant recipients and hemodialysis patients had lower sleep quality than people with normal kidney function. CONCLUSIONS: Sleep disorders can have detrimental effects on kidney allograft function, emphasizing the crucial need for systematic screening and management to ensure the survival of both the graft and the recipient. In light of this, it is imperative for nurses to regularly assess the sleep health of transplant recipients and, when deemed necessary, employ specific nursing interventions to address sleep problems and enhance overall sleep quality during the provision of care.

Global epidemiology of hepatitis C virus in dialysis patients: A systematic review and meta-analysis.

Dialysis is a replacement therapy for patients with End-Stage Renal Disease (ESRD). Patients on dialysis are at high risk of acquiring hepatitis C virus (HCV), which has become a leading cause of morbidity and mortality in this population. There is a wide range of prevalence of HCV in dialysis populations around the world. It is still unknown how prevalent HCV infection is among worldwide dialysis patients (including those undergoing hemodialysis and peritoneal dialysis). A review was conducted to estimate the global epidemiology of hepatitis C in dialysis patients. We searched PubMed, Excerpta Medica Database (Embase), Global Index Medicus and Web of Science until October 2022. A manual search of references from relevant articles was also conducted. Heterogeneity was evaluated by the χ2 test on Cochrane's Q statistic, and the sources of heterogeneity were investigated using subgroup analysis. In order to assess publication bias, funnel plots and Egger tests were conducted, and pooled HCV prevalence estimates were generated using a DerSimonian and Laird meta-analysis model. The study is registered with PROSPERO under CRD42022237789. We included 634 papers involving 392160 participants. The overall HCV case fatality rate was 38.7% (95% CI = 28.9-49). The global prevalence of HCV infection in dialysis population group were 24.3% [95% CI = 22.6-25.9]. As indicated by UNSD region, country, dialysis type, and HCV diagnostic targeted; Eastern Europe had the highest prevalence of 48.6% [95% CI = 35.2-62], Indonesia had 63.6% [95% CI = 42.9-82], hemodialysis patients had 25.5% [95% CI = 23.8-27.3], and anti-HCV were detected in 24.5% [95% CI = 22.8-26.2]. Dialysis patients, particularly those on hemodialysis, have a high prevalence and case fatality rate of HCV infection. Hemodialysis units need to implement strict infection control measures.

High Ferritin Is Not Needed in Hemodialysis Patients: A Retrospective Study of Total Body Iron and Oral Iron Replacement Therapy.

In vivo iron levels can be adjusted through intestinal iron absorption to be maintained at a suitable level; however, optimal iron levels in hemodialysis (HD) patients are unclear. In this study, we investigated total body iron (TBI), calculated as the sum of red blood cell (RBC) iron and iron stores, during courses of low-dose oral iron replacement therapy, and evaluated in vivo iron sufficiency and its indicators in HD patients. We analyzed data on 105 courses of low-dose iron replacement therapy administered to 83 patients on maintenance HD over 7 months. We evaluated changes in TBI, RBC iron, and iron stores from the initiation of treatment to month 7 in two groups of patients, namely, iron-therapy responders and non-responders. TBI showed significant increases until month 4 and plateaued thereafter in iron-therapy responders, and tended to increase and then reached a similar plateau in non-responders (month 7: 1900 ± 447 vs. 1900 ± 408 mg). Steady-state TBI was strongly correlated with body surface area (y = 1628.6x - 791.91, R2 = 0.88, p < 0.001). We observed constant TBI during oral iron replacement therapy suggesting the activation of a "mucosal block". The results suggest that body surface area has utility for estimating the required TBI with regression equations.

[Primary hyperoxaluria type 1-a rare hereditary metabolic disorder as cause of livedo racemosa]. / Primäre Hyperoxalurie Typ 1 ­ eine seltene hereditäre Stoffwechselstörung als Ursache einer Livedo racemosa.

Livedo racemosa is characterized by a bizarrely configurated lightning figure-like appearance with striated to reticulated, livid erythematous macules and results from a reduced perfusion of the respective skin area, which can have different underlying pathophysiologies. A rare but relevant cause, especially in young patients with end-stage kidney failure, is primary hyperoxaluria type 1 (PH1), a hereditary metabolic disorder in which oxalate accumulates in the body.

Safety and utility of panniculectomy in renal transplant candidates and end stage renal disease patients.

BACKGROUND: As the obesity crisis in the United States continues, some renal transplantation centers have liberalized their BMI criteria necessary for transplant eligibility. More individuals with larger body-habitus related comorbidities with End-Stage Renal Disease (ESRD) now qualify for renal transplantation (RT). Surgical modalities from other fields also interact with this patient population. METHODS: In order to assess surgical outcomes of panniculectomy in the context of renal transplantation and ESRD, the authors performed a systematic review following the Preferred Reporting Items for Systematic Review and Meta-Analyses (PRISMA) 2020 guidelines. Due to a paucity of existing primary studies, we retrospectively collected data on patients with ESRD undergoing panniculectomy from the American College of Surgeons' National Surgical Quality Improvement Program (NSQIP) to evaluate outcomes of body contouring in this patient population. RESULTS: From the systematic review, a total of 783 ESRD patients underwent panniculectomy among the studies identified. Of these, 91 patients underwent panniculectomy simultaneously to RT while 692 had their pannus resected prior to kidney transplant. The most common complication was hematoma followed by wound dehiscence. From the NSQIP database, 24 868 patients met the inclusion criteria for analysis. In the setting of renal transplant status, patients with diabetes, hypertension requiring medication, and requiring dialysis were more likely to suffer postoperative complications (OR 1.31, 1.15, and 2.2, respectively). However, upon sub-analysis of specific types of complications, the only retained association was between diabetes and wound complication. CONCLUSION: Preliminary data show that panniculectomy in ESRD patients appears to be safe, though with a nominal increased risk for complications. Pannus resection does not appear to impact post-transplantation outcomes, including long-term allograft survival. Larger, higher powered, randomized studies are needed to confirm the safety, utility, and medical benefit of panniculectomy in the context of renal transplantation.

Risk factors for renal dysfunction after isolated intestinal transplantation.

INTRODUCTION: Kidney dysfunction is a known complication of intestinal transplantation; however, the rate of development and risk factors for chronic kidney disease (CKD) remain poorly defined. METHODS: This was a single-center retrospective review of isolated adult intestinal allograft recipients from 2011 to 2019. Patients who died or experienced graft loss within 1-year or had a prior transplant were excluded. Estimated glomerular filtration rate (eGFR) was calculated using the CKD-EPI equation at 0-, 6- and 12-months post-transplant, and multivariable linear regression was performed to identify variables associated with adjusted eGFR at 1-year. Independent variables included age, ethnicity, BMI, history of diabetes/hypertension, vasopressor use, TPN and stoma days, urinary or bloodstream infections, intravenous contrast exposure, rejection, concomitant immunosuppression, and time above the therapeutic range of tacrolimus. Variables with a p < .1 in univariate analysis were considered for multivariable modeling. RESULTS: Thirty-three patients were included with a mean age of 43.9 ± 13.0. A mean 42.3% decline in eGFR was observed at 1-year post-transplant, with 15.2% of patients developing new stage 4/5 CKD. Factors associated with a greater decline in adjusted eGFR in the univariate model included increasing age, decreased BMI, stoma days, and vasopressor use. In the adjusted multivariable model patient age (ß = -.77, p < .01) and stoma days (ß = -.06, p < .01) remained significant. Tacrolimus and sirolimus exposure were not associated with decline in eGFR at 1 year. CONCLUSIONS: Renal dysfunction is common following intestinal transplantation. The need for stoma creation should be carefully considered, and reversal should be performed when feasible for renal protection.

Effects of Extracorporeal Blood Flow Rates on Patient Tolerance for LIXELLE® Treatment during Outpatient Hemodialysis.

INTRODUCTION: Accumulation of ß2-microglobulin (B2M) in dialysis patients contributes to several comorbidities of end-stage kidney disease (ESKD). The LIXELLE® device adsorbs B2M from blood using sorbent bead technology. Studies in Japan showed that LIXELLE treatment during hemodialysis (HD) at blood flow rates up to 250 mL/min removes B2M above HD alone and is well tolerated. We investigated tolerance for LIXELLE treatment during HD at higher HD blood flow rates standard in the USA. METHODS: A prospective, open-label, non-randomized, single-arm, early-feasibility study (EFS) assessed tolerance and safety of LIXELLE treatment during HD at blood flow rates up to 450 mL/min. ESKD patients (40-75 years old) on thrice weekly outpatient HD were eligible. After a 1-week HD run-in, patients received LIXELLE plus HD at a blood flow rate of 250 mL/min (1 week), followed by LIXELLE plus HD at a blood flow rate up to 450 mL/min (1 week). These blood flow rates were tested with three LIXELLE column sizes in sequence (treatment = 6 weeks). B2M removal was assessed for each combination. RESULTS: Ten patients with a historic intradialytic hypotension (IDH) rate of 0.42 events/HD session/patient were enrolled. Nine patients completed all combinations without IDH events (treatment IDH rate: 0.56 events/HD session/patient). No treatment-emergent serious adverse events or significant changes in red blood cell, platelet, or complement indices except haptoglobin were reported. B2M reduction ratios and removal of select proteins (<40 kDa) increased with escalating column size and blood flow rate. CONCLUSION: LIXELLE plus HD across all column sizes was safe and well tolerated at blood flow rates up to 450 mL/min. Extent of B2M removal corresponded to column size-blood flow rate combinations. This EFS provides a risk profile to guide further studies of LIXELLE in ESKD patients at US-standard blood flow rates.