Frontiers in nephrology最新文献

Cystinosis is a rare autosomal recessive lysosomal storage disease caused by a defective lysosomal cystine carrier protein, cystinosin, resulting in formation and deposition of cystine crystals throughout the body. The renal manifestations of the disease have long been studied, but the musculoskeletal consequences of the disease are generally less well understood. Limb deformities, scoliosis, myopathy and low bone mineral density are associated with cystinosis and can lead to pain, fragility fractures, bone deformity, and difficulty ambulating. Although potentially exacerbated by renal disease and post-transplant medications, it has been found that the musculoskeletal manifestations of cystinosis are also due to inherent dysfunction caused by the mutation of cystinosin. Surgical intervention can provide solutions to the bony symptoms of cystinosis. Early referral to an orthopedic surgeon and evaluation for corrective scoliosis surgery, guided growth for growing children with lower extremity deformity and formal osteotomies for deformity correction in skeletally mature individuals may improve physical function and decrease pain. Standard principles of operative treatment of scoliosis and of bone deformity correction utilized for the treatment of bone deformity in other metabolic bone disease may be applied to patients with cystinosis in the absence of cystinosis-specific studies of the efficacy and outcomes of orthopedic surgery.

Aim: Inflammation is very common among dialysis patients and can lead to an increase in morbidity and mortality. Monocyte-to-lymphocyte ratio (MLR) can serve as a reliable predictor of long-term survival in hemodialysis patients. However, few studies have addressed the role of MLR in patients initially receiving hemodialysis (within 3 months). In this study, we aimed to examine the association between MLR and the risk of cardiovascular and all-cause mortality in patients initially receiving hemodialysis.

Methods: In this study, a total of 216 patients newly receiving hemodialysis for at least 3 months were recruited. The associations between MLR and cardiovascular diseases (CVD) and all-cause mortality were assessed by multivariable Cox models.

Results: A total of 216 patients were included (mean age 57.65 ± 15.68 years, 42.13% male patients). Patients were divided into the low MLR group (<0.49) and the high MLR group (≥0.49). The levels of neutrophil and serum iron and the number of deaths were significantly higher in the high MLR group (P < 0.05). Spearman's analysis showed that MLR was positively correlated with BUN (R = 0.210, P = 0.002), WBC (R = 0.178, P = 0.009), and neutrophil (R = 0.237, P < 0.001). Kaplan-Meier analysis showed that patients in the low MLR group present longer survival (64.08 ± 2.30 vs. 51.07 ± 3.12 months, P < 0.001). Multivariate Cox regression analysis showed that age, diabetes, and MLR (all P < 0.05) were factors significantly associated with a higher risk of CVD and all-cause mortality.

Conclusions: Our results showed that high MLR values are an independent risk factor for CVD and all-cause mortality in patients initially receiving hemodialysis, especially in the elderly and those with a history of diabetes.

Background: Hemorrhage represents the primary complication associated with kidney biopsy, with post-biopsy bleeding occurring in up to 14% of cases. Some clinicians routinely administer hemostatic agents, such as desmopressin, prior to kidney biopsy to mitigate the risk of significant bleeding. However, the efficacy of this practice remains contentious. Consequently, this meta-analysis was undertaken to assess existing studies regarding the efficacy and safety of desmopressin used before kidney biopsy.

Methods: This systematic review and meta-analysis incorporated both randomized controlled trials and observational studies that examined the outcomes of desmopressin administration prior to percutaneous renal biopsy. Efficacy was measured by the incidence of bleeding events, while safety was assessed through the rate of hyponatremia. A comprehensive search of multiple databases was performed, and the risk of bias was evaluated, and statistical analyses were conducted using appropriate models.

Results: Twelve studies were included. The primary meta-analysis showed no significant reduction in overall bleeding risk with desmopressin (pooled OR 0.71, 95% CI: 0.47 - 1.09; I² = 79%; p = 0.12).Statistically significant differences were observed in the intranasal administration group (pooled OR 0.41;95% CI: 0.28 to 0.60; I ² = 20%; p < 0.0001)(Fixed effect), the RCT group (pooled OR 0.30; 95% CI: 0.17 to 0.53; I ² = 0%; p < 0.0001)(Fixed effect), the low bias group (pooled OR 0.53; 95% CI: 0.32 to 0.87; I ² = 74%; p = 0.01)(Random effect). We conducted statistical analysis on six studies with specific data on hyponatremia, and the pooled OR used fixed model was 2.14 (95% CI: 1.51 to 3.03; I ² = 28%) (Fixed effect), indicating there was a statistical difference between the two groups (p < 0.0001).

Conclusion: Desmopressin did not significantly reduce overall bleeding risk after kidney biopsy. While intranasal administration, RCT only and low bias group showed efficacy in subgroup analyses, it carried a significant hyponatremia risk. Route-specific protocols warrant further study.

Systematic review registration: https://www.crd.york.ac.uk/PROSPERO/, identifier CRD42023391915.

Background: Type 2 diabetes mellitus (T2DM) is an increasing global pandemic, frequently complicated by diabetic kidney disease, that may result in end stage kidney disease and increased cardiovascular morbidity and mortality. The 2020 KDIGO guidelines recommend SGLT2 inhibitors and GLP1RAs for cardio-renal protection in patients with T2DM and kidney disease. This study aimed to evaluate the implementation of the 2020 KDIGO guidelines among adult diabetic patients receiving nephrology care.

Material and methods: This retrospective study included 587 patients with T2DM and chronic kidney disease treated in a single nephrology clinic between 1 May 2021 and 31 May 2022. Demographic, diabetes related, and CKD-related data was assessed. The utilization of the 2020 KDIGO recommended medications was analyzed during the study period, along with factors influencing treatment decisions.

Results: The findings revealed a low initial utilization of recommended medications, with only 12.9% and 10.4% of patients treated with SGLT2i and GLP1RA, respectively. Only a modest, but significant, increase in SGLT2i usage was observed by the end of the study period. Factors associated with underutilization of SGLT2i and GLP1RA included older age and decreased kidney function. The study also highlights a significant gap between the recommendations given by nephrologists during the study period and the actual use of recommended medications in the last clinic visit.

Conclusions: In conclusion, the study provides insights into the challenges of implementing KDIGO guidelines in real-world nephrology clinical setting. Further research is needed to explore the reasons behind low adherence to guidelines and strategies to improve compliance, ultimately enhancing patient outcomes in the management of kidney disease in T2DM.

Introduction: Recent studies in Europe have reported a rising incidence in anti-glomerular basement membrane (anti-GBM) disease, potentially linked to demographic shifts or environmental factors. This study aimed to assess temporal trends in incidence, clinical presentation, and outcomes of anti-GBM disease in two urban areas of Madrid over the past two decades.

Materials and methods: We conducted a retrospective observational study of patients diagnosed with anti-GBM disease between 2006 and 2022 at two urban areas covering 884,000 residents in Madrid. Inclusion required confirmed anti-GBM antibodies with clinical manifestations. Incidence was calculated per 1,000,000 person-years. Data were analyzed across six time periods and compared pre- and post-COVID-19 onset.

Results: A total of 26 cases were identified (mean age 52 ± 26 years; 54% female). Incidence increased from 1.13 cases per million persons-year before 2020, to 4.53 cases per million persons-year after 2020 (p<0.001). No differences were observed in demographic data or environmental exposures over time. Post-COVID-19 cases had lower serum creatinine at presentation (5.09 ± 4 vs. 8.7 ± 3.9 mg/dL, p=0.037), more pulmonary involvement (83.3% vs. 35.7%, p=0.039), and better 1-year renal survival (50% vs. 14.3%, p=0.049). Overall patient survival did not differ between groups.

Conclusions: Incidence of anti-GBM disease has increased in Madrid, particularly after the COVID-19 pandemic. Improved renal survival appears linked to earlier diagnosis and management, rather than changes in environmental exposure. These findings highlight the importance of heightened clinical awareness for early detection and treatment of this aggressive disease.

Background: The risk of infection-related death is high in patients undergoing dialysis. This study aimed to identify the modifiable risk factors for PD-related infections in patients undergoing peritoneal dialysis.

Methods: This was a population-based retrospective cohort study conducted in Skåne, South Sweden, which included all patients receiving peritoneal dialysis (PD) between 2011 and 2020. The primary outcome was PD-related peritonitis, and the secondary outcome was a composite of PD-related infections, that is, peritonitis, exit site, or tunnel infections. Time-to-event frailty models, unadjusted and adjusted for age at PD start, sex and Charleson comorbidity index, were used to investigate potentially modifiable risk factors for PD-related infections. Cox regression models were subsequently used to analyze the relationship between PD-related infection episodes and all-cause mortality during the study period.

Results: In total, 545 patients were included in the study, of whom 212 (39%) patients had at least one episode of peritonitis during a median follow-up time of 1.6 years. We found that BMI ≥ 30 may be associated with a clinically relevant increased risk for PD-related infection (aHR 1.45, 95% CI 1.08-1.93, p-value 0.012, n_events = 486), but not for peritonitis alone (adjusted Hazard Ratio, aHR, 1.34, 95% CI 0.95- 1.91; p = 0.099; n_events = 365). Patients with >3 peritonitis episodes had an almost three-fold increased risk of all-cause mortality (aHR, 2.66; 95% CI 1.56-4.52, p < 0.001).

Conclusion: We found that a BMI ≥ 30 may be a modifiable risk factor for peritoneal dialysis-related infections and that multiple episodes of infectious complications of peritoneal dialysis are associated with increased all-cause mortality.

Background: Although medical guidelines for chronic kidney disease (CKD) clearly recommend measures such as blood pressure control, dietary changes, regular physical activity, and consistent medication adherence, individuals frequently encounter challenges in implementing these behavioral modifications. In medical practices, there is a lack of time and resources to comprehensively support CKD patients and low-threshold (digital) interventions aimed at enhancing patient activation are needed. This paper analyzes the acceptance and usage intention (Study 1) and the contribution to health literacy and behavioral change (Study 2) of a m-health program for CKD ("Oska"). The Oska program combines personal counseling via video calls with app-based support and is theoretically grounded in the Health Action Process Approach (HAPA), with a strong emphasis on fostering self-efficacy and promoting implementation in daily routines.

Method: Study 1: An online survey was conducted with N = 401 individuals with CKD and/or hypertension, obesity, type 2 diabetes, or coronary heart disease (age: 50-89 years, M = 64.1, 49% female). Participants were recruited via the provider Appinio and presented with a vignette illustrating the Oska program and answered questionnaires on usage intention, desired support, compatible health benefits, health literacy, and perceived usefulness. Study 2: N = 109 participants with CKD, who already took part in the Oska program for an average of 4.7 months (age: 29-84 years, M = 62.3, 64% female, BMI: M = 29.6), completed established questionnaires on working alliance, kidney-specific health literacy, and behavior change. The analysis was conducted using structural equation models and linear regression analyses.

Results: Acceptance and usage intention in study 1 were high and predominantly explained by compatible health benefits, health literacy, and perceived usefulness, but largely independent of sociodemographic factors and health-related variables. In study 2, higher health literacy was primarily fostered by longer program participation and, most notably, by a positive trust relationship (working alliance) (R²_adj = .48) Successful behavior change (across all guideline areas) was primarily attributed to a positively evaluated working alliance and Oska's contribution to health literacy, rather than sociodemographic factors or the number and type of diagnoses (R²_adj = .14).

Discussion: Digitally delivered coaching combined with app-based support is not only acceptable but may be particularly effective for CKD patients with low health literacy and multiple comorbidities. Relevant determinants include a trusting coaching relationship and a focus on health literacy as well as self-efficacy in implementing measures in everyday life.

Anti-glomerular basement membrane (anti-GBM) disease is a rare autoimmune disorder characterized by circulating autoantibodies targeting type IV collagen, leading to rapidly progressive glomerulonephritis. We report a case of a 44-year-old African American female with a history of hypertension who presented with acute kidney injury, hematuria, and shortness of breath. She tested positive for COVID-19 and received antiviral therapy; however, her renal function rapidly deteriorated, with serum creatinine rising from 3.4 to 10 mg/dL. Serologic testing ruled out common autoimmune conditions, but elevated CH50 levels suggested ongoing immune activation. Renal biopsy demonstrated diffuse necrotizing crescentic glomerulonephritis with linear IgG staining, consistent with anti-GBM disease. Despite aggressive therapy, including plasmapheresis, corticosteroids, and dialysis, renal recovery was not achieved. Immunosuppressive therapy was deferred in light of her active COVID-19 infection and the risk of immunosuppression-related complications. This case highlights a potential association between COVID-19 and anti-GBM disease, suggesting viral-induced endothelial injury and aberrant immune activation as possible mechanisms. Given emerging reports of autoimmune kidney diseases following COVID-19, further research is needed to clarify this relationship and guide optimal management. This is particularly important for patients who present with severe renal dysfunction in the context of an active infection.

Introduction: Myonecrosis is a rare but serious complication of diabetes, particularly in patients with end-stage kidney disease (ESKD), characterized by ischemic necrosis of the skeletal muscles. Its diagnosis is often delayed due to overlapping presentations with cellulitis or deep vein thrombosis. Treatment is traditionally limited to supportive measures such as rest and pain control, which remains the cornerstone. The role of corticosteroids remains controversial in this condition as its effectiveness and utility are not widely understood. This case highlights the unconventional use of corticosteroids in the management of refractory diabetic myonecrosis, emphasizing their potential in mitigating inflammation and promoting recovery.

Case report: We present a 31-year-old woman with ESKD on hemodialysis and a history of type 1 diabetes who presented with recurrent, debilitating pain and swelling in the right lower extremity. Despite a comprehensive workup, including MRI and a muscle biopsy confirming myonecrosis, the patient's symptoms persisted despite conventional supportive care. Following a multidisciplinary discussion, corticosteroid therapy was initiated, resulting in dramatic symptom resolution within 48 h. The patient experienced significant pain reduction, improved mobility, and decreased swelling, allowing for discharge on a tapered steroid regimen. Notably, a subsequent recurrence of myonecrosis in a different muscle group also responded favorably to corticosteroid treatment, further underscoring its therapeutic potential in the management of patients with this condition.

Discussion/conclusion: This case underscores the importance of considering corticosteroids as an adjunctive therapy in refractory diabetic myonecrosis, particularly in patients who fail to respond to standard care. A detailed workup, a high degree of suspicion, distinct clinical findings, and imaging such as MRI, along with muscle biopsy, can accurately diagnose this condition. While corticosteroids are not routinely used due to their potential risks, their dramatic effect in this patient highlights the need for further research to better understand their role and to refine treatment strategies. By expanding the therapeutic approach to diabetic myonecrosis, this case provides valuable insights for improving outcomes in this rare and challenging condition. This case opens the door for the exploration of corticosteroids as an adjunctive therapy in similar diabetic patients with ESKD and refractory myonecrosis.