Requiring Kidney Transplantation Research Articles

#2935 Kidney transplantation outcomes following non-kidney solid organ transplantation

Abstract Background and Aims Approximately 15% of recipients of non-kidney solid organ transplantation (NKSOT) develop end-stage chronic kidney disease (CKD), requiring kidney transplantation (KT). There are controversy whether KT after NKSOT outcomes are poor or whether these patients should be prioritized. Method Retrospective analysis of KT after NKSOT in Andalusia between 1978-2023. We performed a case-control study, choosing 2 controls for each case (1 recipient of a second KT [reKT] and 1 recipient of a first KT without previous NKSOT), the closest ones with KT date and similar age and gender between donor and recipient. Patients with renopancreatic transplantation were excluded. We compared clinical characteristics and outcomes. Results During this period, we followed 12217 KT in Andalusia. Of these, 35 (0.003%) are KT after NKSOT: 26 (74.3%) liver transplant recipients, 7 (20%) heart transplant recipients and 2 (5.7%) lung transplant recipients. All of them are recipients of a first KT. When comparing KT after NKSOT vs. reKT and first KT recipients, demographic characteristics and donor type are similar in all 3 groups. The reKT recipients were on dialysis longer before receiving the second KT, with no difference between the other 2 groups. Graft survival of reKT patients was lower than the rest (vs. KT after NKSOT, p = 0.014; vs. first KT p < 0.001). Survival between KT after NKSOT and first KT was slightly worse in those who had received a previous NKSOT, but without significant differences. In multivariate analysis, adjusted for time on waiting list, reKT remained a risk factor at the limit of statistical significance (p = 0.072). There was no difference in patient survival. Conclusion KT after NKSOT has similar graft and patient survival outcomes to recipients of a first KT, and better than in recipients of a second KT.

Remission of nephrogenic diabetes insipidus (arginine vasopressin resistance): description of a rare clinical case

Nephrogenic diabetes insipidus is a heterogeneous disease in the etiopathogenesis of which are involved acquired and congenital factors. In this case, the kidneys do not respond to vasopressin and continue to produce large concentrated volumes of urine. Distinctive features in the pathology of central genesis are the fact of trauma, brain tumor with involvement of pituitary gland, response to intranasal vasopressin in the form of decreased diuresis.For diagnosis it is important to perform a series of differential diagnostic tests.In this article we present a unique clinical case of a patient with a long-term course of diabetes insipidus with the development a spectrum of comorbid conditions, metabolic disorders, required kidney transplantation.

Phenotypic anomalies in Kabuki syndrome and their implications – an analysis of two cases in the light of scientific literature

Kabuki syndrome (KS1: OMIM 147920, KS2: OMIM 300867) is a rare disorder characterised by dysmorphic face and limbs, microcephaly, short stature, and concomitant multiorgan congenital defects. The prevalence is estimated at 1:32,000–86,000 live births. Most patients are also diagnosed with mild to moderate intellectual disability. Molecular diagnosis includes an analysis for the two most common mutations in KMT2D (KS1), also known as MML2, and KDM6A (KS2) genes. Children diagnosed with Kabuki syndrome require multidisciplinary care. This paper presents detailed case reports of two children diagnosed with Kabuki syndrome, who presented with different manifestations of kidney disease. One of the patients required kidney transplantation, which determines the length and quality of life.

#3074 CHANGES IN THE CLINICAL CHARACTERISTICS AND MANAGEMENT OF PATIENTS WITH AHUS OVER 10 YEARS: TRENDS FROM THE GLOBAL AHUS REGISTRY

Abstract Background and Aims Atypical haemolytic uraemic syndrome (aHUS) is a rare disease predominantly caused by alternative complement pathway dysregulation. Prior to 2011, before the targeted complement inhibitor eculizumab became available, aHUS frequently led to end-stage kidney disease (ESKD) and early death. Treatment with eculizumab led to notable improvements in outcomes; whether patient characteristics and management has continued to change over time is unknown. Using data from the Global aHUS Registry, we assessed clinical characteristics and management of patients with aHUS over 10 years to identify any potential trends. Method All patients enrolled in the Global aHUS Registry from 2012–2022 were included. Patients were categorised according to age at aHUS onset (adult [≥18 years] vs paediatric [<18 years]); onset of aHUS was defined as the earliest of initial symptom presentation, aHUS diagnosis, or first recorded thrombotic microangiopathy (TMA). Patient characteristics were summarised using descriptive analysis. Results Of the 1994 patients enrolled in the registry between 2012 and 2022, 33 (1.7%) were missing data on age at aHUS onset. Of the remaining 1961 patients, 813 (41.5%) were paediatric and 1148 (58.5%) were adult. Changes in patient characteristics and management are presented in Fig. 1. Plasma exchange/plasma infusion (PE/PI) prior to and including the year of enrolment was less common in paediatric than adult patients (58.5% vs. 71.3%) between 2012–2013 and declined substantially in paediatric (19.6%) relative to adult (55.1%) patients by 2020–2022. Similar proportions of paediatric patients required dialysis at the time of enrolment between 2012–2013 (5.1%) and 2020–2022 (5.9%), while numerically lower proportions of adults required dialysis over time (2012–2013, 15.2%; 2020–2022, 6.5%). The rate of paediatric patients requiring a kidney transplant prior and up to enrolment dropped from 2012–2013 (28.4%) to 2020–2022 (5.9%); however, the rate among adult patients remained comparable (2012–2013, 29.2%; 2020–2022, 27.1%). A decrease in the proportion of patients with a reported identified pathogenic variant and/or anti-CFH antibodies was observed in paediatric and adult patients between 2012–2013 (paediatric, 59.1%; adult, 45.5%) and 2020–2022 (paediatric, 49.0%; adult, 30.8%). The proportion of patients with a reported triggering event increased from 2012–2013 (paediatric, 6.3%; adult, 12.4%) to 2020–2022 (paediatric, 9.8%; adult, 28.0%). In this cohort, a total of 1208 patients were treated with eculizumab/ravulizumab between 2012 and 2022 (paediatrics, 476; adults, 732). Between 2012–2013, the median time from aHUS onset to treatment initiation was 94.9 days for paediatric patients (blue) and 51.1 days for adult patients (orange); this fell to 13.1 days in paediatrics and 24.1 days in adults between 2020–2022 (Fig. 2). Conclusion Utilisation of PE/PI to treat aHUS has decreased more in paediatric than adult patients over time. A decreased requirement for transplant in paediatric patients was evident, suggesting more paediatrics are now being diagnosed and treated earlier, leading to better outcomes with fewer patients progressing to renal failure and requiring kidney transplant. The proportion of both paediatric and adult patients with reported pathogenic variants and/or anti-CFH antibodies decreased over time and may reflect the rate at which a clinical constellation consistent with aHUS is identified. Moreover, aHUS may be recognised more frequently in the presence of a triggering condition, or a lower barrier for clinicians to suspect aHUS in recent years. Patients were more rapidly treated with a complement inhibitor, likely due to better awareness in the clinical community around complement inhibitors and the importance of early treatment initiation. Although patients with aHUS are being treated more promptly in recent years than 10 years ago, more work is needed, especially in adults, to move towards optimal clinical practice.

A randomized controlled trial of preemptive rituximab to prevent recurrent focal segmental glomerulosclerosis post-kidney transplant (PRI-VENT FSGS): protocol and study design.

Focal segmental glomerulosclerosis (FSGS) is a common cause of end-stage kidney disease requiring kidney transplantation and can recur in the allograft in 30-80% of recipients resulting in reduced graft survival. Plasmapheresis has shown efficacy in treating some cases of recurrent FSGS but isolated plasmapheresis has not demonstrated efficacy in preventing recurrent FSGS. Rituximab has had anecdotal success in preventing recurrence in a single center study but has not been studied in combination with plasmapheresis for preventing FSGS recurrence. We are conducting a randomized, controlled, multicenter clinical trial of adult and pediatric kidney transplant recipients with primary FSGS to assess whether plasmapheresis in combination with rituximab prevents recurrent disease post-transplantation. Rituximab combined with plasmapheresis is a promising, novel therapy to prevent recurrent FSGS, a disease with limited therapeutic options and no consensus guidelines for prevention or treatment. https://clinicaltrials.gov/ct2/show/NCT03763643, identifier NCT03763643.

Cystinosis in a routine ophthalmologic evaluation

Introduction: Cystinosis is a rare autosomal recessive metabolic disease. It occurs due to a chromosomal mutation that allows the accumulation of this amino acid in lysosomes. Cystine can form crystals in various tissues such as the kidneys, pancreas, thyroid, testicles, bone marrow, brain, eye, and muscles. In the eyes, crystals can cause deposits on the cornea and conjunctiva. In the cornea, deposits occur in the periphery and anterior stroma. As the disease progresses, the crystals move centripetally and posteriorly. Oral and topical treatments with cysteamine have been described. The ophthalmologic solution of cysteamine 0.55% used continuously every 2 hours has shown good results. Clinical Case: Patient in a routine ophthalmologic exam, with findings of cystine crystals in the cornea and past kidney transplantation without diagnosis. Discussion: The patient’s past history is relevant and corroborates with the ophthalmological findings. The patient had severe kidney disease, requiring kidney transplantation, but the disease was not elucidated. Therefore, the ophthalmological examination was fundamental for the diagnosis of the case

A decrease in the incidence of Shiga toxin-related hemolytic uremic syndrome as a cause of kidney transplantation at an argentine referral center.

In Argentina, Hemolytic uremic syndrome caused by Shiga toxin-producing Escherichia coli (STEC HUS), is the main cause of acute kidney injury and the second cause of end-stage renal disease (ESRD) in children. In recent decades, strategies have been implemented to reduce progression to ESRD, but it is not known whether the cumulative incidence of HUS requiring kidney transplantation (KTx) has decreased. We aimed to determine whether the cumulative incidence of STEC HUS in children undergoing KTx decreased and compared outcomes of HUS-related KTx vs. those related to other etiologies. All patients who underwent KTx at our institution were evaluated. The cohort was divided into quintiles (Q), and we compared the cumulative incidence of HUS-related KTx vs KTx due to other etiologies. A total of 1000 consecutive KTx were included. The cumulative incidence of HUS-related KTx was 11%. HUS was the second cause of KTx in Q1: 17% (1988-1995); Q2: 13.5% (1996-2003); Q3: 11.5% (2004-2009) and third cause in Q4: 10% (2010-2015) and Q5: 3% (2016-2021). The cumulative incidence of HUS-related KTx decreased in Q4 and Q5 compared to Q1, Q2, and Q3 and the decline was even steeper when comparing Q4 to Q5 (p:0.019). There was no difference in graft survival in patients with HUS vs. congenital anomalies of kidney and urinary tract (CAKUT) but better than in those with focal segmental glomerulosclerosis (FSGS). In this cohort, the cumulative incidence of HUS-related KTx decreased, which may have been due to the implementation of nephroprotective strategies.

Serum amyloidosis: a cardiac amyloidosis.

A 75-year old male with a history of membranoproliferative glomerulonephritis requiring kidney transplant, carpal tunnel, and peripheral neuropathy presented with new heart failure. An echocardiogram revealed moderate, concentric left ventricular hypertrophy (LVH) despite normal electrocardiographic voltage. To evaluate for the aetiology of unexplained LVH, a cardiac magnetic resonance (CMR) was performed which showed increased wall thickness with markedly increased T1 relaxation time and extensive, near-transmural late gadolinium enhancement throughout the myocardium (Panels A–C, green arrows), findings highly concerning for cardiac amyloidosis (CA). Despite this, a 21.5 mCi Tc-99 m pyrophosphate (Tc-PyP) scan and comprehensive serum (Panels D and E) and urine light chain analysis showed no evidence of transthyretin or immunoglobulin light chain CA, respectively. Despite these negative findings, the patient was referred for endomyocardial biopsy due to high clinical concern for CA. His biopsy revealed diffuse myocardial amyloid deposition (Panels E and F) which was ultimately identified as serum amyloid A (AA) protein on mass spectrometry. AA-CA is a rare form of cardiac amyloidosis caused by deposition of SAA protein—an acute phase reactant—in the myocardium. This disorder is most commonly observed in patients with longstanding chronic inflammatory conditions and is frequently missed by conventional light chain and Tc-PyP analysis. Renal dysfunction is a key feature of AA-CA and is present in a most cases. This case highlights the importance of having a low threshold to pursue further testing including invasive tissue biopsy when typical CA CMR features are present even in the absence of confirmatory non-invasive testing.

Long-term follow-up of HCV-infected patients with end-stage chronic kidney disease after sustained virological response with direct-acting antiviral therapy

Background and aimPatients with chronic kidney disease (CKD) and hepatitis C infection can be safely and effectively treated with direct-acting antivirals (DAAs). However, there is scarce data on the long-term impact of hepatitis C cure on CKD. The aim of this study was to assess the long-term mortality, morbidity and hepatic/renal function outcomes in a cohort of HCV-infected individuals with CKD treated with DAAs. Methods135 HCV patients with CKD stage 3b-5 who received ombitasvir/paritaprevir/ritonavir±dasabuvir in a multicenter study were evaluated for long-term hepatic and renal outcomes and their associated mortality. Results125 patients achieved SVR and 66 were included. Prior to SVR, 53 were under renal replacement therapy (RRT) and 25 (37.8%) had liver cirrhosis. After a follow-up of 4.5 years, 25 (38%) required kidney transplantation but none combined liver–kidney. No changes in renal function were observed among the 51 patients who did not receive renal transplant although eGFR values improved in those with baseline CKD stage 3b-4. Three (5.6%) subjects were weaned from RRT. Eighteen (27.3%) patients died, mostly from cardiovascular events; 2 developed liver decompensation and 1 hepatocellular carcinoma. No HCV reinfection was observed. ConclusionsLong-term mortality remained high among end-stage CKD patients despite HCV cure. Overall, no improvement in renal function was observed and a high proportion of patients required kidney transplantation. However, in CKD stage 3b-4 HCV cure may play a positive role in renal function.

Percutaneous Tibial Nerve Stimulation Improves Lower Urinary Tract Dysfunction and Infection Rate in a Kidney Transplant Recipient With Augmentation Cystoplasty: A Case Report and Literature Review

Almost 20% of patients with congenital posterior urethral valves (PUV) reach end-stage kidney disease requiring kidney transplantation (KT). An augmentation cystoplasty (AC) is performed to treat significant lower urinary tract dysfunction (LUTD) which can persist afterwards due to residual valve bladder and lead to recurrent urinary tract infections (UTIs) and a worse graft survival. Percutaneous tibial nerve stimulation (PTNS) is a minimally invasive second-line neuromodulation therapy approved for the treatment of non-neurogenic LUTD performed in the outpatient setting. Herein, we reported the case of significant improvement of bladder voiding pattern and yearly UTI rates through PTNS in a 32-year-old male KT recipient with a history of congenital PUV and AC. The patient was referred to our institution at the beginning of 2019 for urgency, high post-void residual volume (PVR) and recurrent clinically significant UTIs (3 to 4/y). After assessment, the patient attended once-weekly 30-minute sessions of PTNS for 12 consecutive weeks. A significant improvement of urodynamic and infectious outcomes was observed through 3-day bladder diaries, questionnaires, and laboratory exams during the subsequent 3 years of regular follow-up. The present results might be significant for the prevention of antibiotic resistance and for better graft survival in KT recipients. We successfully treated a patient with AC, implying that the technique may be appliable also in this case. Neuromodulation in KT is still an unexplored field of research, we advocate for prospective assessment of the efficacy of PTNS in these patients, as reproducible results might lead to life-changing improvements.

Chronic Kidney Disease: Challenges in Translational Medicine.

Chronic kidney disease (CKD) has long been recognized as a state of progressive decline in renal function. Morbidity and mortality are well correlated to the stage of renal function decline. Approximately one million deaths are estimated to be related to CKD worldwide. They are mostly associated with cardiovascular disease as a result of concurrent hypertension, accelerated atherosclerosis, and volume overload. Even with the best current treatment, disease progression is the general rule with a small fraction who reach CKD stage 5 requiring kidney transplantation or dialysis. Transplant patients show substantial reductions in mortality and cardiovascular events, as well as improvements in quality of life. However, the capacity of health systems to deliver kidney transplantation varies worldwide with worse indicators in low-income countries. Consequently, exploring novel and better therapeutic options for CKD is mandatory. Cell-based therapy is a promising strategy for treating CKD in preclinical models, and several clinical trials involving kidney disease exhibit a favorable safety profile. This chapter aims to provide an overview of CKD and the recent results of clinical trials of cell therapy in kidney diseases.

The Conundrum of Patients With Compensated Cirrhosis Requiring Kidney Transplantation; Kidney Alone or Simultaneous Liver Kidney Transplantation.

Patients with compensated cirrhosis and chronic kidney disease are increasing along with demand for simultaneous liver kidney transplant (SLKT) and shortages of organs for transplantation. Although these well-compensated patients may not need a liver organ, the alternative of kidney transplant alone (KTA) poses the risk of liver decompensation. Therefore, we aim to characterize outcomes among patients with compensated cirrhosis and chronic kidney disease listed for SLKT or receiving KTA to inform clinical decisions. The 2-part retrospective study included a national cohort of patients listed for SLKT in United Network for Organ Sharing from January 2003 to June 2019 with Child A cirrhosis, with model for end-stage liver disease <25, and receiving dialysis; and a cohort of patients who underwent KTA from 2004 to 2019 with Child A cirrhosis identified through a 4-center chart review. Waitlist outcomes (SLKT, death, and clinical improvement) and post-KTA liver decompensation and survival were evaluated in the cohorts, respectively. In the national SLKT cohort (N = 705, median age 56 y, 68.8% male), 5-y cumulative incidence of SLKT was 43.1%, death 32.1%, and clinical improvement 9.1%. Among SLKT recipients, 36.3% remained Child A without ascites or encephalopathy at transplant. In the local KTA cohort (N = 34, median age 54 y, 79.4% male), none had ascites or hepatic encephalopathy before KTA, but 15 had clinical portal hypertension. Five-y post-KTA incidence of liver decompensation was 36.8%, and survival was 89.2%. SLKT may not be necessary for some patients with compensated cirrhosis needing kidney transplant. KTA is safe for selected patients with intact liver biochemical function, even with portal hypertension but without hepatic encephalopathy or ascites.

Robotic Kidney Transplantation Under Regional Hypothermia.

The technique of robotic kidney transplantation (RKT) under regional hypothermia is being described. It may be performed in all patients requiring kidney transplantation with some relative contraindications. Unlike open transplantation, RKT is a transperitoneal procedure. Patient lies supine with low lithotomy and 15° to 20° reverse Trendelenburg position. Port placement is like standard radical prostatectomy with a multiport device placed at the umbilicus. The graft after harvest needs special care on the bench to ligate all vascular communications and placed in a gauze jacket for easy handling. After transplant bed preparation, the graft is introduced in peritoneal cavity through the umbilical opening and placed over the bladder flap bed with ice slush for hypothermia. End-to-side vascular anastomoses of renal vein and artery are done with the graft lying in pelvic hollow and renal vessels facing laterally to external iliac vessels. After vascular anastomoses, the graft is rotated laterally to iliac fossa to be fixed with preformed peritoneal flap. A stented ureterovesicostomy to the recipient is done using extravesical technique. The external opening for introducing the graft and the robotic port openings is closed after leaving a drain. Postoperative (PO) care and PO immunosuppression are like the open counterpart, except the treatment of drain, which is removed on second PO day. Obese recipients and multiple graft vessels may be handled comfortably during RKT with described techniques. It is important to follow certain checkpoints to avoid bleeding. With graft and patient survival of 95.2% and 94.5%, respectively, RKT outcomes are noninferior to open technique in the published data. The RKT has less incidence of PO pain, wound infection, and symptomatic lymphoceles.

Risk factors for end stage renal disease in children with anorectal malformation and outcome comparison to children with isolated urological anomalies

End stage renal disease (ESRD) requiring kidney transplant (KT) remains an important cause of morbidity in anorectal malformations (ARM) patients. Current literature is scarce on defining the risk factors for ESRD and the outcomes of KT in ARM patients. This study aimed to identify predisposing factors to ESRD in ARM patients and verify if the long term outcome of KT in these patients differs from pure urological anomalies (UA). Databases of ARM and KT patients treated at our center between 2000 and 2016 were used for comparing characteristics of ARM cases which developed ESRD and those who did not, and the outcome features of MAR-KT vs UA-KT. Out of 117 ARM patients, 9 developed ESRD. All of them had a complex ARM. Association with UA were significantly higher in ARM-KT compared to other ARM patients (100% vs. 52%, p=0.001). The most common UA associated to KT in ARM patients was renal dysplasia. During the same period 23 patients underwent KT as a sequelae of pure urological anomalies (UA-KT group). The most represented UA were primary vesico-ureteral reflux (65.2%) and posterior urethral valves (14%) in UA-KT (table 2). ARM-KT patients required more often hemodialysis before KT (50% vs. 8.7%, p=0.05) and an aorto-caval anastomosis at the transplant (75% vs. 30%, p=0.04) compared to UA-KT. Moreover ARM-KT patients experienced more often graft failure and and the need for a second KT (50% vs. 8.6%, p=0.02). To our knowledge, this is the first study describing the differences in terms of risk factors and outcomes of KT in ARM patients. We observed a need for KT in 7% of ARM, which is at the higher end of the range reported in the literature. Bilateral dysplasia and cloaca malformation seem the leading cause to ESRD for ARM patients compared to vesico-ureteral reflux and posterior urethral valve in UA-KT. Our experience showed that KT has worst outcome in ARM vs pure UA patients. Patients with complex ARM are more frequently associated to renal dysplasia which lead to KT. Graft in ARM patients seems to be the more vulnerable and prone to failure.

Vignette-Based Reflections to Inform Genetic Testing Policies in Living Kidney Donors.

Family history of kidney disease increases risk of end-stage kidney disease (ESKD) in donors. Pre-donation genetic testing is recommended in evaluation guidelines and regulatory policy. Collaborating across several institutions, we describe cases to illustrate the utility as well as practical issues in incorporating genetic testing in transplant protocols. Case 1 is from 2009, before pervasive genetic testing. A healthy 27-year-old Caucasian male had an uneventful donor evaluation for his mother, who had early onset ESKD of unclear cause. He participated in paired-exchange kidney donation, but developed progressive kidney disease and gout over the next 10 years. A uromodulin gene mutation (NM_003361.3(UMOD):c.377 G>A p.C126Y) was detected and kidney biopsy showed tubulointerstitial kidney disease. The patient subsequently required kidney transplantation himself. Case 2 was a 36-year-old African American female who had an uneventful kidney donor evaluation. She underwent gene panel-based testing to rule out ApolipoproteinL1 risk variants, for which was negative. Incidentally, a sickle-cell trait (NM_000518.5(HBB):c.20A>T p.Glu7Val) was noted, and she was declined for kidney donation. This led to significant patient anguish. Case 3 was a 26-year-old Caucasian female who underwent panel-based testing because the potential recipient, her cousin, carried a variant of uncertain significance in the hepatocyte nuclear factor-1-β (HNF1B) gene. While the potential donor did not harbor this variant, she was found to have a likely pathogenic variant in complement factor I (NM_000204.4(CFI):c.1311dup:p.Asp438Argfs*8), precluding kidney donation. Our cases emphasize that while genetic testing can be invaluable in donor evaluation, transplant centers should utilize detailed informed consent, develop care pathways for secondary genetic findings, and share experience to develop best practices around genetic testing in donors.

Immunosuppressive regimens and outcomes of inflammatory bowel disease patients requiring kidney transplantation.

Patients with inflammatory bowel disease (IBD) can develop extra-renal complications and as a result, suffer from end stage renal failure requiring kidney transplantation (KT). A brief review of available literature revealed that IBD patients undergoing KT have shorter overall survival rates compared to their controls. Literature reporting steroid regimens and survival outcomes specific to IBD and post kidney transplant are scarce and these studies have small sample sizes thus making it difficult to draw accurate conclusions. Further research is required in the form of a randomized controlled study to clarify the effect and mechanism of steroid immunosuppression on the prognosis of renal transplant recipients and explore new treatment schemes.

Impact of Race-Adjusted Glomerular Filtration Rate Estimation on Eligibility for Simultaneous Liver-Kidney Transplantation.

Estimated glomerular filtration rate (eGFR) is adjusted for Black race in commonly used formulas. This has potential implications for access to simultaneous liver-kidney transplantation (SLKT) as qualifying criteria rely on eGFR. We performed a retrospective study of United Network for Organ Sharing national transplant registry data between February 28, 2002, and March 31, 2019, to evaluate the proportion of Black patients who would be reclassified as meeting SLKT criteria (as defined per current policies) if race adjustment were removed from 2 prominent eGFR equations (Modification of Diet in Renal Disease-4 [MDRD-4] and Chronic Kidney Disease Epidemiology Collaboration [CKD-EPI]). Of the 7937 Black patients listed for transplant during the study period, we found that 3.6% would have been reclassified as qualifying for chronic kidney disease (CKD)-related SLKT with removal of race adjustment for MDRD-4, and 3.0% would have been reclassified with CKD-EPI; this represented 23.7% and 18.7% increases in SLKT candidacy, respectively. Reclassification impacted women more than men (eg, 4.5% versus 3.0% by MDRD-4; P<0.05). In an exploratory analysis, patients meeting SLKT criteria by race-unadjusted eGFR equations were significantly more likely to receive liver transplantation alone (LTA) compared with SLKT. Approximately 2.0% of reclassified patients required kidney transplantation within 1 year of LTA versus 0.3% of nonreclassified patients. In conclusion, race adjustment in eGFR equations may impact SLKT candidacy for 3.0% to 4.0% of Black patients listed for LTA overall. Approximately 2.0% of patients reclassified as meeting SLKT criteria require short-term post-LTA kidney transplantation. These data argue for developing novel algorithms for glomerular filtration rate estimation free of race to promote equity.

Utilization of Broad-Panel Genetic Testing for Collagen Disorders of the Basement Membrane Disorders in Patients Requiring Kidney Transplantation

Utilization of Broad-Panel Genetic Testing for Collagen Disorders of the Basement Membrane Disorders in Patients Requiring Kidney Transplantation

MO107CLINICAL CHARACTERISTICS OF A PATIENT POPULATION WITH ATYPICAL HAEMOLYTIC URAEMIC SYNDROME AND MALIGNANT HYPERTENSION: THE GLOBAL AHUS REGISTRY ANALYSIS

Abstract Background and Aims Atypical haemolytic uraemic syndrome (aHUS) is a rare disease that manifests as complement-mediated thrombotic microangiopathy (TMA), which can lead to severe organ damage. Some patients with aHUS may present with malignant hypertension (MHT); both conditions can result in TMA. The objective of this analysis was to characterise patients with aHUS and MHT. Method In this analysis, patients from the Global aHUS Registry (NCT01522183) were included if they were diagnosed with MHT and were followed ≥90 days after initial aHUS symptom presentation or diagnosis date; patients were excluded if they withdrew from the registry or discontinued treatment with eculizumab due to a diagnosis other than aHUS. Demographics and clinical characteristics were evaluated. Results Seventy-one of 1903 registry patients were included in the analysis. Clinical characteristics are presented in the table. Seventeen patients (24%) had a paediatric (&amp;lt;18 years) onset of disease, and 54 (76%) were adults at aHUS diagnosis; female patients were slightly overrepresented (61%). Sixty-nine percent of patients were reported to have MHT at around the same time as aHUS diagnosis (+/-2 months), while 11% and 13% experienced MHT before and after aHUS diagnosis, respectively. aHUS triggering conditions were reported in 6/71 patients (8%) (Table). Cardiovascular (27%) and gastrointestinal (21%) symptoms were the most commonly reported extra-renal manifestations. Eight patients (11%) had a reported family history of aHUS and 40 patients (56%) had a complement pathogenic variant or an anti-CFH-antibody. Thirty-three patients (46%) had a kidney transplant; of these, 20 were prescribed eculizumab in the peri- or post-transplant period. Conclusion In this analysis of patients with aHUS and MHT, the observed high prevalence of pathogenic variants in complement genes or anti-CFH antibodies, alongside the high proportion of patients with extrarenal manifestations and/or requiring kidney transplant, indicate a high severity of presentation and poor prognosis of aHUS associated with MHT.

Innovations in Renal Replacement Technology and Their Impact on the Risk of Renal Cancer in Kidney Transplant Patients

Introduction: The current standard of care for patients with end-stage renal disease (ESRD) is a kidney transplant, and when a donor organ is not available, patients undergo long-term dialysis. There is a growing gap between patients who require a kidney transplant and the availability of donor organs. Dialysis is more widely available, but does not replace full kidney functionality, leading to worse outcomes. The past 20 years have seen an increase in alternative renal replacement technology in an effort to address the aforementioned issues with current therapies. The risk of kidney cancer in kidney transplant patients is increased when compared with the general population; the risk is hypothesized to be most closely tied to immunosuppression after the transplant surgery and length of time on dialysis. Given the increased risk of kidney cancer associated with kidney transplantation, it is imperative to assess the capabilities of these new devices not only for their functionality as alternatives to a donor organ, but also their potential to reduce the risk of renal cancer in this patient population. Purpose: The purpose of this review is to assess the technological potential of the most recent advancements in renal replacement technology in reducing risk of renal cancer in ESRD patients who require kidney transplantation. Methods: We performed a PubMED database search in July-August 2020 with the following search terms: [artificial kidney], [wearable artificial kidney], [bioartificial kidney], [implantable bioartificial kidney], [kidney regeneration]. These search terms were used to identify publications about the recent innovations in kidney replacement technology. Each technology was analyzed for its ability to replace total kidney functionality as well as its potential to reduce the risk of renal cancer. The potential to reduce risk of renal cancer was assessed by the device’s impact on immunosuppression and dialysis timeframes. Results: Four of the newest innovations in renal replacement technology were identified and analyzed: the wearable artificial kidney (WAK), the automated wearable artificial kidney (AWAK), the implantable bioartificial kidney (IAK), and kidney regeneration technology. Of these innovations, the IAK and kidney regeneration technology were found to have the most promise in reducing the risk of renal cancer in ESRD patients requiring transplantation. Conclusions: With the volume of new medical devices and technologies rising every year, it is imperative to analyze the impact they have on patient outcomes. A new technology should be assessed to determine if it improves on a limitation of prior solutions. In this study, we identified two devices with the potential to reduce the occurrence of a major risk associated with patients requiring kidney transplantation: renal cancer. Additional studies are required to analyze these devices once they move into the human clinical trial testing phase.