Frontiers in nephrology最新文献

We report a case of a 55-year-old male patient with a medical history of cardiorenal syndrome and rectosigmoid colon adenocarcinoma, who started dialysis five years prior to presenting with unusual yellow discoloration of his dialyzer filter during his regular dialysis session. Following a regimen of standard chemotherapy, the patient was initiated on fruquintinib, 5 mg daily for 21 days, as an alternative treatment due to the intolerability of previous agents and failure of malignancy to respond. Shortly after starting fruquintinib, the patient developed hyperbilirubinemia and experienced significant yellow discoloration of the dialysis filter-a phenomenon not previously documented in association with this medication. The absence of dialyzer discoloration during five years of dialysis highlights the temporal relationship between the introduction of fruquintinib and the onset of filter discoloration. [removed some sentence] This case highlights the need for heightened awareness of potential adverse effects of fruquintinib, potentially detectable in patients undergoing dialysis, and aims to contribute to the growing body of literature on the medication's safety profile.

Background: BK virus-associated nephropathy (BKVAN) is a major complication in kidney transplantation caused by the reactivation of latent BK virus (BKV) under immunosuppression. BKVAN has been strongly associated with increased graft loss. Currently, there is no effective antiviral treatment for BKVAN. Additionally, the development of donor-specific antibodies (DSAs) and the risk of acute and chronic rejection complicate the reduction of immunosuppressive therapy (IS). This case report illustrates the management of BKVAN in a highly sensitized transplant recipient and explores the potential use of extracorporeal photopheresis (ECP) as an immunomodulatory tool.

Case: 44-year-old Caucasian woman with a history of failed prior transplant and multiple transfusions underwent a second kidney transplant. Due to a high panel-reactive antibody level, she received induction therapy with plasma exchange, thymoglobulin and steroids, followed by maintenance with tacrolimus, mycophenolate mofetil (MMF), and steroids. Initial graft function was good, and protocol biopsies showed no rejection. In year four, the patient developed an increasing BKV viremia (peak of 40,050 copies/mL) and MMF was reduced, which cleared BKV in six months. Two years later, DSAs reappeared, which led to an increase in MMF. In August 2020 the patient showed a decline of GFR, elevated BKV viremia (peak 162,000 copies/mL), and a graft biopsy was performed revealing BKVAN. IS was reduced (MMF was discontinued, and tacrolimus was tapered). After eight months, the viremia cleared up, but anti-DR53 DSAs (MFI 16000) levels increased significantly. As the patient was highly sensitized and had a thrombosis of arteriovenous fistula, mTOR inhibitors were not recommended. In order to modulate alloimmunity without further suppressing antiviral immunity, ECP was introduced. Over the next two years, the patient showed stable renal function (eGFR 30-40 mL/min), no recurrence of BKV viremia, and a gradual reduction in DSAs titers. No acute rejection episodes occurred.

Conclusions: In a highly sensitized patient with BKVAN and contraindications to standard therapies, ECP combined with a tailored immunosuppressive regimen proved effective in controlling viral replication, preserving graft function, and mitigating alloimmune risks. Considering the potential of ECP as an adjunctive therapy in complex BKVAN scenarios, further investigation is warranted.

Background: Managing osteoporosis (OP) in chronic kidney disease (CKD) presents significant challenges due to altered bone metabolism. Given the lack of robust clinical trial data and a notable knowledge gap exists among nephrologists regarding an optimal management in this population, an expert consensus is crucial for developing tailored management strategies. This study aimed to gather an expert opinion to bridge this gap and establish consensus recommendations on the diagnosis and management of osteoporosis in CKD patients.

Methods: A panel of 13 Indian and 1 international experts, including nephrologists and endocrinologists, participated in a structured survey and discussion process. Thirteen Indian experts provided their opinion on key clinical issues, including screening, diagnosis, and treatment strategies for osteoporosis in CKD. Consensus was achieved in a single round of voting, and recommendations were formulated based on the level of agreement among the panelists.

Results: The expert panel reached a strong consensus (80-100% agreement) on several critical recommendations. It was agreed that osteoporosis in CKD is often asymptomatic, with fragility fractures being less common, and thus, early screening using dual-energy X-ray absorptiometry (DXA) is essential. The panel emphasized the importance of evaluating bone turnover status using serum biomarkers such as bone-specific alkaline phosphatase (BSAP) and intact parathyroid hormone (iPTH) to guide treatment decisions. Tailored treatment strategies were recommended, with a judicious use of bisphosphonates and denosumab, depending on the patient's estimated glomerular filtration rate (eGFR) and bone turnover state. The management of renal osteodystrophy (ROD) was deemed necessary before addressing CKD-induced osteoporosis.

Conclusion: This expert consensus provides critical insights and guidance for the management of osteoporosis in CKD. The recommendations emphasize individualized treatment approaches, the importance of early screening, and the integration of multidisciplinary care. These findings aim to fill existing knowledge gaps and improve clinical outcomes for CKD patients with osteoporosis.

Lupus nephropathy is a common manifestation of systemic lupus erythematosus (SLE), with immune-mediated inflammatory damage to the glomerulus leading to acute kidney injury, chronic kidney disease, and end-stage renal disease. Occasionally, patients present with renal-limited lupus nephropathy with classic full-house staining on immunofluorescence and no signs of systemic lupus. Limited data are available on renal-limited "lupus-like nephropathy" in pregnancy. A 24-year-old G1P0 woman at 14 weeks of gestation was referred to nephrology for further evaluation of 8.4g proteinuria. She was found to be ANA negative with a decreased C1q level and a renal biopsy revealing membranous nephropathy. Immunofluorescence staining was positive for IgG, IgA, IgM, C3, and C1Q, consistent with full-house pattern. She was started on 500 mg pulse dose methylprednisolone for 3 days, which was gradually tapered to 5 mg daily, and cyclosporine 75 mg BID. She delivered a healthy baby via induction at 36 weeks. Six-month follow-up revealed 1g protein on 24-hour urine collection, normal C3/C4 levels, and no signs of SLE. This case report adds to the literature discussing renal-limited "lupus-like nephropathy" in pregnancy and helps guide further management of this condition.

Background: Vitamin C deficiency is an underrecognized yet prevalent concern in hemodialysis patients, driven by dietary restrictions, increased oxidative stress, and vitamin losses during dialysis. While supplementation could mitigate deficiency-related complications and reduce inflammation and oxidative damage, clinical implementation remains limited due to concerns about oxalosis and potential pro-oxidative effects.

Case presentation: We report the case of a 74-year-old female with End-Stage Kidney Disease (ESKD) secondary to diabetic nephropathy who developed scurvy after prolonged hemodialysis. She presented with unintended weight loss, gingival bleeding, and recurrent pulmonary edema. Physical examination revealed characteristic dermatological findings, including perifollicular erythema predominantly on the lower extremities. Laboratory testing confirmed severe vitamin C deficiency, with serum levels below the detection limit of 4 mg/L, along with hypoalbuminemia and elevated inflammatory markers. Nutritional assessment indicated adherence to standard hemodialysis dietary restrictions, likely exacerbating deficiency.

Intervention and outcomes: Oral vitamin C supplementation resulted in significant clinical improvement, including resolution of dermatological manifestations, cessation of gingival bleeding, improvement in cardiac function, and without recurrence of pulmonary edema episodes, with no adverse effects observed.

Conclusion: This case highlights the importance of considering scurvy in hemodialysis patients, particularly those with inflammation and restrictive dietary patterns. It underscores the clinical manifestations of vitamin C deficiency, its potential cardiovascular implications, and the need to revisit supplementation guidelines in this population. The findings support the safe and effective use of vitamin C supplementation in reversing deficiency-related complications while emphasizing the broader consideration of routine vitamin C supplementation in hemodialysis patients, even in the absence of overt clinical manifestations.

Background: Regular follow-up care after kidney transplantation is performed in transplant centers together with local nephrologist practices in Germany. Patients after kidney transplantation have to fulfill many tasks and manage their disease, follow a complex therapeutic regimen, communicate with the transplant center and home nephrologists, and coordinate doctor appointments. It has been shown that mHealth solutions such as mobile phone applications (apps) can support patients in their self-management. However, stand-alone apps have limitations and ideally, the mHealth solutions are embedded in a holistic treatment approach, including healthcare professionals.

Methods: We will conduct a 1-year, prospective, randomized, 2-armed, parallel group multicenter trial in three German Kidney Transplant Centers (KTCs) to demonstrate that additional and continuous interventional telemedical management will improve health after kidney transplantation in patients of all ages. Therefore, a composite endpoint of seven key outcome variables [fewer hospitalizations, shorter length of hospitalization, less development of de novo donor-specific antibody (DSA), better medication adherence, lower tacrolimus intra-patient variability, better blood pressure control, and better renal function after kidney transplantation]was defined. All the patients will receive the same routine post-transplant aftercare. The patients in the interventional arm will receive additional predefined telemedical management, including regular telemedicine visits and automatic bidirectional data transfer (e.g., vital signs, wellbeing, medication plan, and laboratory data together with a chat option) between the patient at home and the KTC through a certified smartphone app. If necessary, a home nephrologist can be included in the automatic data transfer. In the interventional arm, the iBox score will be used to better detect patients at risk for early graft failure and drug-drug interactions will be regularly checked with certified software.

Discussion: The study aims to prolong patient and graft survival through additional telemedical services in order to reduce avoidable hospitalizations, improve treatment of co-morbidities, and improve adherence through patient empowerment, which should result in lower health care costs, and better quality of life of patients after kidney transplantation.

Clinical trial registration: ClinicalTrials.gov, identifier NCT05897047.

Background: Artificial intelligence (AI) is increasingly recognized for its potential to enhance nephrology training and practice. However, the integration of AI into fellowship training remains inadequately explored. This study aimed to assess current AI utilization, perceptions, and educational needs among nephrology fellows at Mayo Clinic.

Methods: A structured online survey was administered to 23 fellows-including those specializing in kidney transplantation and onco-nephrology-across three Mayo Clinic sites (Minnesota, Arizona, and Florida). The survey addressed domains such as current AI usage, perceived relevance of AI in clinical practice, interest in formal AI training, self-assessed comfort with AI integration, and barriers to adopting AI technologies in nephrology education.

Results: A total of 21 fellows (91% response rate) participated in the survey. 76% of respondents rated AI as moderately to highly relevant to nephrology. Similarly, 76% indicated a moderate to very high interest in receiving targeted AI training. Despite these favorable perceptions, 76% had rarely or never used AI in their clinical or research activities, and none reported any formal AI education. Interactive workshops emerged as the preferred modality for AI training (52%), with limited knowledge cited as the primary barrier to adoption. Optimism was especially high regarding AI applications in predictive modeling (86%) and diagnostic imaging (81%), while confidence in AI for direct clinical decision-making remained cautious.

Conclusion: There is significant interest among nephrology fellows in AI, along with a critical need for formal education and training. The enthusiasm for AI's potential contrasts with a cautious perspective towards its current use in clinical decision-making. Our study highlights the necessity for educational initiatives that bridge the knowledge gap and foster confidence in the appropriate use of AI technologies in Nephrology fellowship.

Catastrophic antiphospholipid syndrome (CAPS) leads to organ dysfunction due to thrombotic microangiopathy (TMA). Complement may play a role in CAPS, and its blockade could prevent antiphospholipid syndrome (APS) complications after kidney transplantation (KT). Here, we report a case of APS recurrence after KT in a 38-year-old woman with early acute cortical kidney allograft necrosis despite preventive eculizumab treatment, probably because of insufficient complement blockade. The patient had recurrent but controlled CAPS for years with renal dysfunction, leading to preemptive KT. Anticoagulation and eculizumab were administered to prevent thrombosis and TMA after KT. She developed acute kidney injury (AKI) with incomplete biological TMA. Imaging revealed cortical necrosis in the renal allograft. In the absence of donor-specific anti-HLA antibodies, we concluded a relapse. Additional doses of eculizumab and plasma exchange allowed the normalization of biological tests and improvement of kidney allograft function. A retrospective complement analysis showed an incomplete blockade at the time of AKI. One year after KT, the renal allograft function was impaired. This suggests that inadequate complement blockade leads to a relapse of APS in the renal allograft with cortical necrosis and dysfunction. Our case highlights the importance of monitoring complement activity and adjusting the dose of eculizumab or ravulizumab.

Introduction: Renal vasculitis is a rare disease, the incidence of which increased markedly during the COVID-19 pandemic in our center. The aim of this study is to compare the incidence and the clinical and histopathological characteristics of anti-neutrophil cytoplasm antibodies (ANCA)-associated vasculitis patients before and during the COVID-19 pandemic.

Methods: A single-center observational retrospective analysis of 61 patients with ANCA-associated vasculitis who were divided into two groups according to date of diagnosis: pre-pandemic from 2008 to 2020 (n=37) and during the pandemic from 2020 to the middle of 2022 (n=24). The annual incidence rate was compared, as were characteristics such as age, gender, Birmingham Vasculitis Activity Score (BVAS) score, renal clinic, organ involvement, and ANCA serotype. Biopsy findings, such as optical microscopy glomerular characteristics, crescents, interstitium, immunofluorescence, and electron microscopy findings, were analyzed. Mortality and renal replacement therapy needs were also compared.

Results: The annual incidence rate was higher in the pandemic group compared to the pre-pandemic group, with 9.6 cases per year vs. 3.1 cases per year [incidence rate ratio (IRR)=3.11, 95% CI 1.86 to 5.20]. No significant differences between the groups were found for clinical characteristics, except for greater hemoptysis frequency in the pandemic group. Significant differences in immunofluorescence and electronic microscopy were observed, with a higher IgG deposit and C3 in the pandemic group (37.5% vs 8.1%, p=0.0064; 43.5% vs 10.8%, p=0.009, respectively), whereas the incidence of pauci-immune patterns was higher in the pre-pandemic group (81.1% vs 54.1%, p=0.016). Mortality and the need for renal replacement therapy were significant higher in the pandemic group (IRR=3.56, CI 95% 1.27-9.98 and IRR=4.24, CI 95% 2.08-8.65, respectively).

Conclusion: The incidence of ANCA vasculitis increased during the COVID-19 pandemic and was associated with higher rates of IgG deposit and C3 in the immunofluorescence findings and with higher rates of deaths and dialysis in the pandemic group compared with the pre-pandemic group.