Association Between Low Anti-spike Antibody Levels After the Third Dose of SARS-CoV-2 Vaccination and Hospitalization due to Symptomatic Breakthrough Infection in Kidney Transplant Recipients

Background@#Whether anti-severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) antibody levels post-third coronavirus disease (COVID-19) vaccination correlate with worse outcomes due to breakthrough infection is unclear. We evaluated the association between anti-SARS-CoV-2 antibody levels and symptomatic breakthrough infection or hospitalization during the Omicron surge in kidney transplant recipients. @*Methods@#In total, 287 kidney transplant recipients expected to receive a third vaccination were enrolled between November 2021 and February 2022. The Abbott SARS-CoV-2 IgG II Quant test (Abbott, Chicago, IL, USA) was performed within three weeks before and four weeks after the third vaccination. The incidence of symptomatic breakthrough infection and hospitalization from two weeks to four months post-third vaccination was recorded. @*Results@#After the third vaccination, the seropositive rate and median antibody titer of the 287 patients increased from 57.1% to 82.2% and from 71.7 (interquartile range [IQR] 7.2– 402.8) to 1,612.1 (IQR 153.9–5,489.1) AU/mL, respectively. Sixty-four (22.3%) patients had symptomatic breakthrough infections, of whom 12 required hospitalization. Lower anti-receptor-binding domain (RBD) IgG levels ( < 400 AU/mL) post-third vaccination were a risk factor for symptomatic breakthrough infection (hazard ratio [HR] = 3.46, P < 0.001).Anti-RBD IgG levels < 200 AU/mL were a critical risk factor for hospitalization (HR = 36.4, P = 0.007). @*Conclusions@#Low anti-spike IgG levels after third vaccination in kidney transplant recipients were associated with symptomatic breakthrough infection and, particularly, with hospitalization during the Omicron surge. These data can be used to identify patients requiring additional protective measures, such as passive immunization using monoclonal antibodies.

The Clinical Course and Prognosis of Patients With Nontuberculous Mycobacterial Pulmonary Disease After Solid Organ Transplantation

Background@#Due to impaired cell-mediated immunity, solid organ transplantation (SOT) recipients are at increased risk of developing nontuberculous mycobacterial pulmonary disease (NTM-PD). However, the clinical course of NTM-PD in SOT patients and the impact of SOT on the prognosis of NTM-PD remain unclear. @*Methods@#We analyzed patients who developed NTM-PD after receiving SOT between January 2001 and December 2020, at a tertiary referral hospital in South Korea. Baseline characteristics, clinical course, and prognosis were evaluated. Propensity score-matched analysis was performed to assess the impact of SOT on long-term survival in patients with NTM-PD. @*Results@#Among 4,685 SOT recipients over 20 years, 12 patients (median age, 64 years;interquartile range [IQR], 59–67 years; men, 66.7%) developed NTM-PD. Seven (58.3%) and five (41.7%) patients underwent kidney and liver transplantation, respectively, before the diagnosis of NTM-PD. The incidence of NTM-PD was 35.6 cases per 100,000 person-years among kidney transplant recipients and 28.7 cases per 100,000 person-years among liver transplant recipients. The median time between transplantation and the diagnosis of NTMPD was 3.3 (IQR, 1.5–10.8) years. The most common mycobacterial species was Mycobacterium avium (50.0%). Antibiotic treatment was initiated in five (41.7%) patients, and two patients (40.0%) achieved microbiological cure. Two patients died during a median follow-up of 4.2 (IQR, 2.3–8.8) years and NTM-PD was assumed to be the cause of death in one patient. When matched to patients without a history of SOT, patients with a history of SOT did not show worse survival (P value for log-rank test = 0.62). @*Conclusion@#The clinical course of NTM-PD in SOT recipients was comparable to that of patients without SOT, and SOT did not increase the risk of all-cause mortality in patients with NTM-PD.

Flat Pattern Peaks of Tacrolimus Absorption and Associated Pharmacogenomic Variants in Kidney Transplantation Recipients

Background@#Tacrolimus is the most commonly used immunosuppressive drug in solid organ transplantation. After administering a conventional twice-daily dose of tacrolimus, peak levels were achieved within the first 1.5 to 2 hours. A group of patients showed different early absorption phase of tacrolimus after kidney transplantation. @*Methods@#Trough(C0 ) and 1.5-hour blood levels (C1.5 ) of tacrolimus were measured in 95 kidney transplantation recipients. Patients with a C1.5 /C0 1.5 were defined as those having flat pattern peaks and as controls, respectively. Transplantation outcomes were compared between the groups. Whole exome sequencing was performed to investigate the genetic susceptibility to flat pattern peaks. @*Results@#Twenty-eight patients showed flat pattern peaks. The mean C1.5 /C0 values were 1.13 ± 0.22 and 3.78 ± 1.25 in the flat pattern peak and control groups, respectively. In multivariate analysis, flat pattern peak was an independent risk factor for biopsy-proven acute rejection (BPAR) and/or borderline change (P = 0.014). Patients having flat pattern peaks showed significantly lower post-transplant 36-month estimated glomerular filtration rate (P = 0.001). Two single nucleotide variants in ABCB1 genes, rs1922242 and rs2235035, were associated with flat pattern peaks (P = 0.019 and P = 0.027, respectively). @*Conclusion@#Both of C1.5 and C0should be measured to distinguish the patients showing unique initial absorption. A C1.5 /C0 ratio lower than 1.5 was associated with an increased risk of BPAR and/or borderline change. Single nucleotide variants s in ABCB1 gene might influence the flat pattern peaks of tacrolimus absorption.

Kidney Transplantation From Deceased Donors With Bloodstream Infection: A Multicenter Retrospective Study

Background@#The use of organs from donors with infection is limited because of the possibility of transmission. We aimed to investigate the transmission after deceased donor transplantation with bloodstream infection (BSI). @*Methods@#A retrospective study of patients undergoing kidney or pancreas transplantation at five tertiary centers in Korea from January 2009 and November 2019 was performed. We analyzed the outcomes after transplantation from deceased donors with BSI. @*Results@#Eighty-six recipients received transplantation from 69 donors with BSI. The most common isolated pathogens from donors were Gram-positive bacteria (72.0%), followed by Gram-negative bacteria (22.7%), and fungi (5.3%). Appropriate antimicrobial agents were used in 47.8% of donors before transplantation. Transmission occurred only in 1 of 83 recipients (1.2%) from bacteremic donors and 1 of 6 recipients (16.7%) from fungemic donors. One-year patient and graft survival was 97.5%and 96.3%, respectively. There was no significant difference in graft and patient survival between patients who received organs from infected donors and noninfected donors. @*Conclusion@#Using organs from donors with bacteremia seems to be a safe option with low transmission risk. The overall prognosis of using organs from donors with BSI is favorable.

Better Efficacy of Balloon Assisted Maturation in Radial-Cephalic Arteriovenous Fistula for Hemodialysis

Purpose@#Native arteriovenous fistula (AVF) is the first choice for hemodialysis access; however, the maturation failure rate remains high. Hence, balloon-assisted maturation (BAM) is increasingly being used to overcome maturation failure. This study evaluated the outcomes of BAM and compared the differences between radial-cephalic (RC) and brachial-cephalic (BC) AVF. @*Materials and Methods@#Between January 2013 and December 2017, 1,622 new AVFs were created. BAM was considered if the AVF did not satisfy the criteria for hemodynamic maturation (6-mm diameter and 500-mL/min flow rate within 8 weeks after the operation). @*Results@#Of the 1,622 AVFs, BAM was performed in 142 patients (8.75%). There were 92 RC and 50 BC AVFs. Multivariate analyses revealed that ipsilateral central vein catheter history was the sole risk factor for maturation failure after BAM. Oneyear functional primary patency (FPP) and functional secondary patency (FSP) in RC AVFs were higher than those in BC AVFs without statistical significance (FPP, RC vs. BC: 70.9% vs. 50.9%, P=0.099; FSP, 95.5% vs. 81.1%, P=0.146). Further, based on the multivariate analysis, the independent risk factors for FPP in the RC and BC AVFs were the number of BAMs (odds ratio [OR], 3.05; 95% confidence interval [CI], 1.11-8.37; P=0.03) and age (OR, 1.04; 95% CI, 1.00-1.07; P=0.04), respectively. @*Conclusion@#BAM is a relatively good salvage method with tolerable patency. However, the risk factors for patency and the outcomes of BAM differ between RC and BC AVFs.

Intravascular Fasciitis in the Femoral Vein with Hypermetabolic Signals Mimicking a Sarcoma:The Role of Preoperative Imaging Studies with Review of Literature

Intravascular fasciitis (IVF) is a very rare disease that is difficult to diagnose preoperatively. Frequently, it can be misdiagnosed as a malignancy or deep vein thrombosis. A 26-year-old man presented with a 6-month history of intermittent cramping pain in the right calf. Duplex ultrasonography, computed tomography, magnetic resonance imaging, and positron emission tomography were performed in various hospitals. The work-up revealed a hypermetabolic mass in the femoral vein, suggestive of a malignancy, such as leiomyosarcoma. The tumor was located inside the femoral vein with no invasion, and the mass was resected en bloc with the vein wall. Intraoperative frozen section biopsy revealed no malignancy, and the final pathological diagnosis was IVF. Herein, we report a case of IVF and discuss the role of imaging studies in its preoperative diagnosis, with an extensive literature review.

Better Efficacy of Balloon Assisted Maturation in Radial-Cephalic Arteriovenous Fistula for Hemodialysis

Purpose@#Native arteriovenous fistula (AVF) is the first choice for hemodialysis access; however, the maturation failure rate remains high. Hence, balloon-assisted maturation (BAM) is increasingly being used to overcome maturation failure. This study evaluated the outcomes of BAM and compared the differences between radial-cephalic (RC) and brachial-cephalic (BC) AVF. @*Materials and Methods@#Between January 2013 and December 2017, 1,622 new AVFs were created. BAM was considered if the AVF did not satisfy the criteria for hemodynamic maturation (6-mm diameter and 500-mL/min flow rate within 8 weeks after the operation). @*Results@#Of the 1,622 AVFs, BAM was performed in 142 patients (8.75%). There were 92 RC and 50 BC AVFs. Multivariate analyses revealed that ipsilateral central vein catheter history was the sole risk factor for maturation failure after BAM. Oneyear functional primary patency (FPP) and functional secondary patency (FSP) in RC AVFs were higher than those in BC AVFs without statistical significance (FPP, RC vs. BC: 70.9% vs. 50.9%, P=0.099; FSP, 95.5% vs. 81.1%, P=0.146). Further, based on the multivariate analysis, the independent risk factors for FPP in the RC and BC AVFs were the number of BAMs (odds ratio [OR], 3.05; 95% confidence interval [CI], 1.11-8.37; P=0.03) and age (OR, 1.04; 95% CI, 1.00-1.07; P=0.04), respectively. @*Conclusion@#BAM is a relatively good salvage method with tolerable patency. However, the risk factors for patency and the outcomes of BAM differ between RC and BC AVFs.

Intravascular Fasciitis in the Femoral Vein with Hypermetabolic Signals Mimicking a Sarcoma:The Role of Preoperative Imaging Studies with Review of Literature

Intravascular fasciitis (IVF) is a very rare disease that is difficult to diagnose preoperatively. Frequently, it can be misdiagnosed as a malignancy or deep vein thrombosis. A 26-year-old man presented with a 6-month history of intermittent cramping pain in the right calf. Duplex ultrasonography, computed tomography, magnetic resonance imaging, and positron emission tomography were performed in various hospitals. The work-up revealed a hypermetabolic mass in the femoral vein, suggestive of a malignancy, such as leiomyosarcoma. The tumor was located inside the femoral vein with no invasion, and the mass was resected en bloc with the vein wall. Intraoperative frozen section biopsy revealed no malignancy, and the final pathological diagnosis was IVF. Herein, we report a case of IVF and discuss the role of imaging studies in its preoperative diagnosis, with an extensive literature review.

Risk of Graft Failure in Kidney Recipients with Cured Post-Transplant Cancer

Background@#Post-transplant cancer (PTC) is a critical complication after kidney transplantation. However, whether successfully cured PTC affects the long-term graft outcome remains unclear. @*Methods@#We retrospectively reviewed 1,629 kidney transplant recipients from 1995 to 2017 after excluding patients with post-transplant hematologic or advanced non-curable cancers and who underwent allograft nephrectomy because of cancer. Cured PTCs were defined as cancers treated with curative methods and/or adjuvant therapy without recurrence during ≥ 2 years. Propensity score matching was performed to match cured PTC patients with cancer-naïve patients (i.e., non-PTC group). @*Results@#During the median period of 7 years (maximum, 23 years), 70 patients (4.3%) had cured PTCs. The PTC group showed significantly higher risks of death-censored graft failure (adjusted hazard ratio [HR], 2.56 [1.05–6.23]), class II donor-specific antibodies (adjusted HRs, 3.37 [1.30–8.71]), estimated glomerular filtration rate 1 g (adjusted HR, 3.61 [1.92–6.79]) compared to non-PTC group. However, the risk of mortality was not different between the PTC and non-PTC groups. According to the cancer type, only urogenital cancer had a significant association with graft failure (adjusted HR, 4.26 [1.19–15.22]) and the gastrointestinal cancer showed elevated risk of T cell mediated rejection compared to non-PTC (adjusted HR, 20.44 [6.02–69.39]). @*Conclusion@#Appropriate monitoring of graft function is necessary in patients with cured PTCs.

Klotho as a potential predictor of deceased donor kidney transplantation outcomes

Purpose@#Klotho is an antiaging factor mainly produced by renal tubular cells. Klotho is reportedly decreased in an animal model of acute kidney injury and patients with chronic kidney disease. However, information on Klotho expression after kidney transplantation is limited. We analyzed the correlation between donor Klotho expression and clinical outcomes of kidney transplantation. @*Methods@#Sixty patients who underwent deceased donor kidney transplantation between March 2015 and October 2017 were enrolled. Serum and tissue Klotho expression levels were measured by enzyme-linked immunosorbent assay and immunohistochemistry, respectively. Graft function was assessed by estimated glomerular filtration rate (eGFR). @*Results@#Patients were divided into 2 groups according to donor Klotho expression in renal tissues. A greater improvement in eGFR was observed at 1 week after transplantation in patients receiving kidneys with higher Klotho expression (47.5 ± 21.9 mL/min/1.73 m2 vs. 63.9 ± 28.2 mL/min/1.73 m2, P = 0.030). Patients were also classified into 2 groups according to donor serum Klotho level. There was a tendency for a higher eGFR at 12 months after transplantation in patients receiving kidneys from donors with a higher Klotho level (51.0 ± 18.0 mL/min/1.73 m2 vs. 61.2 ± 16.5 mL/min/1.73 m2, P = 0.059). When subgrouped into patients with or without biopsy-proven acute rejection, 12-month eGFR remained higher in patients receiving kidneys from donors with higher serum Klotho. @*Conclusion@#Our data demonstrated that donor tissue expression of Klotho correlated with early recovery of eGFR after kidney transplantation. Donor serum Klotho level tended to be associated with posttransplant 12-month eGFR. Donor Klotho expression might be a new predictor for deceased donor kidney transplantation outcome.

Erratum: Post-Transplant Lymphoproliferative Diseases in Pediatric Kidney Allograft Recipients with Epstein-Barr Virus Viremia

The authors regret that there were errors in (Text/Table 1). This notice corrects on page 4 and page 5.

Erratum: Post-Transplant Lymphoproliferative Diseases in Pediatric Kidney Allograft Recipients with Epstein-Barr Virus Viremia

Erratum: Post-Transplant Lymphoproliferative Diseases in Pediatric Kidney Allograft Recipients with Epstein-Barr Virus Viremia

The authors regret that there were errors in (Text/Table 1). This notice corrects on page 4 and page 5.

Predictive Value of Abnormal and Borderline Ankle-Brachial Index for Coronary Re-Intervention and Mortality in Patients with Coronary Artery Disease: An Observational Cohort Study

Purpose@#This study aimed to investigate the abnormal and borderline ABIs for predicting coronary re-intervention and mortality in patients with coronary artery disease (CAD). @*Materials and Methods@#Data from a previous study were obtained and used to investigate the prevalence of peripheral arterial disease among Korean patients with CAD (n=285) in 2010. All patients underwent follow-up coronary angiography as scheduled (asymptomatic: 2-, 5-, and 7-month intervals) or as clinically indicated (symptomatic). @*Results@#In total, 33 patients had an abnormal ABI (ab-ABI: 1.4), and 252 had a normal ABI (nl-ABI: 1.0≤ABI≤1.4). The mean follow-up was 47 months. The mortality was significantly higher in the ab-ABI group than in the nl-ABI group (18.2% vs. 6.7%, P=0.0233). MACEs were significantly more common in the ab-ABI group (60.6% vs. 34.5%, P=0. 0036). Moreover, the ab-ABI group had a greater CAD progression than the nl-ABI group (48.5% vs. 31.3%, P=0.0496). The incidence of clinically indicated coronary re-intervention was significantly higher in the ab-ABI group than in the nl-ABI group (33.3% vs. 13.1%, P=0.0025). After adjusting for age, diabetes, dyslipidemia, dialysis, smoking, and obesity, the incidence of clinically indicated re-intervention was significantly higher in the ab-ABI group than in the nl-ABI group (HR, 2.80; 95% CI, 1.24 to 6.34). @*Conclusion@#Abnormal and borderline ABI significantly increased the incidence of clinically indicated coronary revascularization and all-cause mortality during a 4-year follow-up among patients with CAD. Hence, ABI could be used to stratify extremely high-risk patients with CAD who may require aggressive surveillance or treatment.

Directional Atherectomy for Treating In-Stent Restenosis of the Superficial Femoral Artery

Purpose@#The optimal treatment for in-stent restenosis (ISR) of the superficial femoral artery (SFA) is still in debate. This study aimed to evaluate the safety and effectiveness of directional atherectomy (DA) as a primary treatment modality for ISR in SFA. @*Materials and Methods@#A retrospective single-center analysis was conducted. In total, 617 stents were deployed in 242 limbs for SFA diseases during the study period. ISR was identified in 29 limbs (12.0%); 14 limbs were treated with DA and 15 limbs with balloon angioplasty (BAP) alone. Technical success rate, target lesion revascularization (TLR) and patency rates (PRs) at 12 months, and any complications were evaluated. @*Results@#DA group included complete occlusions in 50% of patients and BAP group included in 40%. Mean improvement in the ankle-brachial index was 0.29 and 0.32, respectively (P=0.638). Technical success was achieved in all patients.The procedural success rates were 85.7% and 73.3%, respectively (P=0.651). There was no significant difference regarding residual stenosis, distal embolization, or flow-limiting dissection. Primary PRs at 1 year were 85.7% and 73.3%, secondary PRs were 100.0% and 93.3%, and TLR rates were 14.3% and 20.0% (P=0.411, 0.326, and 0.684, respectively). @*Conclusion@#Short-term outcomes after DA for ISR were not different from those after BAP but showed a tendency of better primary PR and TLR. Larger multicenter prospective studies are needed to define the role of DA in ISR treatment.

Klotho as a potential predictor of deceased donor kidney transplantation outcomes

Purpose@#Klotho is an antiaging factor mainly produced by renal tubular cells. Klotho is reportedly decreased in an animal model of acute kidney injury and patients with chronic kidney disease. However, information on Klotho expression after kidney transplantation is limited. We analyzed the correlation between donor Klotho expression and clinical outcomes of kidney transplantation. @*Methods@#Sixty patients who underwent deceased donor kidney transplantation between March 2015 and October 2017 were enrolled. Serum and tissue Klotho expression levels were measured by enzyme-linked immunosorbent assay and immunohistochemistry, respectively. Graft function was assessed by estimated glomerular filtration rate (eGFR). @*Results@#Patients were divided into 2 groups according to donor Klotho expression in renal tissues. A greater improvement in eGFR was observed at 1 week after transplantation in patients receiving kidneys with higher Klotho expression (47.5 ± 21.9 mL/min/1.73 m2 vs. 63.9 ± 28.2 mL/min/1.73 m2, P = 0.030). Patients were also classified into 2 groups according to donor serum Klotho level. There was a tendency for a higher eGFR at 12 months after transplantation in patients receiving kidneys from donors with a higher Klotho level (51.0 ± 18.0 mL/min/1.73 m2 vs. 61.2 ± 16.5 mL/min/1.73 m2, P = 0.059). When subgrouped into patients with or without biopsy-proven acute rejection, 12-month eGFR remained higher in patients receiving kidneys from donors with higher serum Klotho. @*Conclusion@#Our data demonstrated that donor tissue expression of Klotho correlated with early recovery of eGFR after kidney transplantation. Donor serum Klotho level tended to be associated with posttransplant 12-month eGFR. Donor Klotho expression might be a new predictor for deceased donor kidney transplantation outcome.

Nationwide Epidemiologic Study of Abdominal Aortic Aneurysms in Korea: A Cross-Sectional Study Using National Health Insurance Review and Assessment Service Data

PURPOSE: The prevalence and treatment patterns of abdominal aortic aneurysm (AAA) vary according to ethnicity and region. This study analyzed nationwide data on the epidemiology, practice patterns, and mortality rates of AAA in Korea.MATERIALS AND METHODS: Data from patients treated for AAA from 2012 to 2016 were extracted from the Korean Health Insurance Review and Assessment (HIRA) database.RESULTS: A total of 30,766 patients in Korea had treatment codes for AAA and 2,618 patients were treated for ruptured AAA. Of the 6,356 patients treated surgically, 1,849 and 4,507 underwent open surgical aneurysmal repairs (OSAR) or endovascular aneurysmal repairs (EVAR), respectively. The number of surgical treatments performed annually for AAA increased from 1,129 cases in 2012 to 1,501 cases in 2016. The number of EVAR cases increased from 753 to 1,109 during these five years, while the number of OSAR cases remained similar, at 376 and 392, respectively. The 30-day mortality rates after EVAR and OSAR were 4.2% and 10.6%, respectively. The mortality rates were significantly higher in patients with hypertension, dyslipidemia, chronic renal disease, diabetes mellitus, and congestive heart failure. There were significant differences in the prevalence, proportion of EVAR, and mortality rates according to the regional area.CONCLUSION: The prevalence of AAA and the proportion of EVAR in Korea increased in the past 5 years, while the rupture rate and the proportion of OSAR remained similar. To minimize mortality and regional discrepancies, nationwide registry and treatment standardization are needed.

Post-Transplant Lymphoproliferative Diseases in Pediatric Kidney Allograft Recipients with Epstein-Barr Virus Viremia

BACKGROUND: Post-transplant lymphoproliferative disease (PTLD) is one of the major complications of organ transplantation, especially in children with Epstein-Barr virus (EBV) viremia (EV). We performed a retrospective study to evaluate risk factors for PTLD in children with EV. METHODS: Among 199 pediatric kidney transplantation (KT) recipients at our center from January 2001 to October 2015, records of those with EBV viral loads of > 1,000 copies/mL and/or PTLD were reviewed. RESULTS: Diagnosis of PTLD was made in seven patients (PTLD group), and 39 patients had EV only (EV only group). The median time from KT to EV and PTLD diagnosis was 6.7 (range 0.4–47.8) months and 8.2 (range, 2.8–98.9) months, respectively. There were no significant differences between the groups in terms of sex, age at transplantation, donor type, EBV viral load, or EV-free duration after KT. Higher tacrolimus level before EV (hazard ratio, 44.5; P = 0.003) was an independent risk factor for PTLD in multivariate Cox regression analysis. Six patients with a high EBV load (median 171,639 copies/mL) were treated with preemptive rituximab (RTX) therapy, resulting in transient reduction of EBV load. None of these patients developed PTLD (median follow-up 51.5 months); however, two had neutropenia and two developed infection requiring hospital admission. CONCLUSION: In pediatric KT recipients, higher tacrolimus levels were associated with a higher incidence of PTLD. Conversely, those who received preemptive RTX for EV did not develop PTLD.

Disseminated adenovirus infection in a 10-year-old renal allograft recipient

Disseminated adenovirus infection can result in high mortality and morbidity in immunocompromised patients. Here, we report the case of a 10-year-old renal allograft recipient who presented with hematuria and dysuria. Adenovirus was isolated from his urine. His urinary symptoms decreased after intravenous hydration and reduction of immunosuppressants. However, 2 weeks later he presented with general weakness and laboratory tests indicated renal failure necessitating emergency hemodialysis. Adenovirus was detected in his sputum; therefore, intravenous ganciclovir and immunoglobulin therapy were initiated. Renal biopsy revealed diffuse necrotizing granulomatous tubulointerstitial nephritis compatible with renal involvement of the viral infection. Adenovirus was detected in his serum. Despite cidofovir administration for 2 weeks, adenovirus was also detected in the cerebrospinal fluid, resulting in generalized tonic-clonic seizure. The patient died 7 weeks after the onset of urinary symptoms. Adenovirus should be considered in screening tests for post-renal transplantation patients who present with hemorrhagic cystitis.

Kidney Transplantation in Patients with Atypical Hemolytic Uremic Syndrome due to Complement Factor H Deficiency: Impact of Liver Transplantation

BACKGROUND: Atypical hemolytic uremic syndrome (aHUS) is a rare disease that is often associated with genetic defects. Mutations of complement factor H (CFH) are the most common genetic defects that cause aHUS and often result in end-stage renal disease. Since CFH is mainly produced in the liver, liver transplantation (LT) has been performed in patients with defective CFH. METHODS: The clinical courses of four kidney allograft recipients who lost their native kidney functions due to aHUS associated with a CFH mutation were reviewed. RESULTS: Subject A underwent kidney transplantation (KT) twice, aHUS recurred and the allograft kidney failed within a few years. Subject B received a KT and soon experienced a recurrence of aHUS coinciding with infection. Her allograft kidney function has worsened, and she remains on plasma infusion therapy. Subject C underwent LT followed by KT. She is doing well without plasma infusion therapy after combined LT-KT for 3 years. Subject D received KT following LT and is now recurrence-free from aHUS. CONCLUSION: In patients with aHUS associated with a CFH mutation, KT without LT was complicated with a recurrence of aHUS, which might lead to allograft loss. Conversely, LT was successful in preventing the recurrence of aHUS and thus might be another option for a recurrence-free life for aHUS patients associated with CFH mutation.