Clinical nephrology最新文献

Cardiorenal syndrome (CRS) is a term defined as complex interactions between concomitant cardiac and renal dysfunction in which disease of one organ initiates, perpetuates, and/or accelerates the decline in the other. It accounts for a third of presentations with heart failure and is associated with poor clinical outcomes. Polypharmacy (defined as using five or more medications) is common in CRS patients and is associated with worst clinical outcomes. The risk for polypharmacy increases to several fold with associated comorbidities, poses risks to the overall health of the patient, and enhances non-compliance to essential medications. Deprescribing non-essential medications, coordination between multiple specialties to mitigate the risk of polypharmacy, pharmacist- and nurse-led clinics to improve adherence to medications, use of polypills and telemonitoring are various methods to reduce polypharmacy. In this paper, we highlight different strategies to prevent polypharmacy and improve compliance and adherence to essential medications.

Autosomal dominant polycystic kidney disease (ADPKD) is the most common heritable multifocal cystic disease encountered in clinical practice, and it is usually diagnosed in patients with family history by the evidence of markedly enlarged kidneys with multiple bilateral cysts at ultrasound (U.S.), computed tomography (CT) scan, or magnetic resonance imaging (MRI). In most cases, genetic testing is not required. Though ADPKD diagnosis is often straightforward, misdiagnosis is possible. Here we present a case of ADPKD misdiagnosis, followed by a review of the most important kidney heritable multifocal cystic diseases. Our case report demonstrates that ADPKD can be erroneously diagnosed when other kidney heritable multifocal cystic diseases occur without their distinguishing manifestations and when there is no genetic characterization among the relatives. A proper diagnosis of heritable diseases is crucial, as it allows an appropriate management of family members who carry disease allele, apart from patient management. Therefore, we suggest a careful differential diagnosis with possible molecular genetic analysis in presentations with familial cystic kidneys and suspicious clinical and radiological features.

Objective: To evaluate the effectiveness of a video-based exercise program on nutritional status and quality of life (QOL) of peritoneal dialysis (PD) patients.

Materials and methods: Participants satisfying the inclusion and exclusion criteria were recruited and divided into the intervention group and the control group. The intervention group received video-based exercise based on a manual advocated by the National Kidney Foundation, while the control group received routine exercise guidance. Their nutritional indicators and the Quality of Life Scale for Peritoneal Dialysis patients (QLSPD) scores were collected and analyzed.

Results: No adverse events occurred during the study. Baseline data between the two groups were comparable, and there were no significant differences in body mass index (BMI) (t = 0.214, p = 0.831), triceps skin fold thickness (TSF) (t = 0.407, p = 0.685), arm muscle circumference (AMC) (t = 0.310, p = 0.757), grip strength (Gs) (t = 0.557, p = 0.579), calf circumference (Cc) (t = 0.441, p = 0.660), serum albumin (Alb) (t = 0.261, p = 0.795), prealbumin (PA) (t = 0.218, p = 0.828), hemoglobin (Hb) (t = 0.114, p = 0.909), triglyceride (TG) (t = 0.192, p = 0.848), total cholesterol (TC) (t = 0.092, p = 0.927), low-density lipoprotein (LDL) (t = 0.124, p = 0.902), high-density lipoprotein (HDL) (t = 0.265, p = 0.792), blood urea nitrogen (BUN) (t = 0.540, p = 0.590), serum creatinine (SCr) (t = 0.255, p = 0.799), total urea clearance index (Kt/V) (t = 0.958, p = 0.340), total creatinine clearance rate (Ccr) (t = 0.166, p = 0.868), protein equivalent of nitrogen appearance (nPNA) (t = 0.240, p = 0.811), and QLSPD scores (p > 0.05) between the two groups at the baseline measures. However, after 12 weeks of intervention, the AMC (t = 2.993, p = 0.003) and Cc (t = 2.811, p = 0.006) of the intervention group improved significantly compared with the control group. The BMI (t = 2.068, p = 0.041) and TSF (t = 2.578, p = 0.011) showed a trend of improvement compared with baseline. And the intervention group scored less in body physiology function (t = 7.084, p < 0.001), disease factors (t = 13.336, p < 0.001), mental state (t = 9.555, p < 0.001), and social role (t = 5.156, p < 0.001) components, while their satisfaction hardly changed (t = 0.240, p = 0.811).

Conclusion: The video-based exercise intervention is safe and can remarkably improve the nutritional status and QOL of PD patients.

Objective: This is a study on the demographics and clinical outcomes including the response to therapy of patients with focal segmental glomerulosclerosis (FSGS) over the past decade.

Materials and methods: All histologically proven FSGS cases diagnosed between 2008 and 2018 were analyzed for their clinical, laboratory, and histological characteristics including treatment that could influence the disease progression and renal outcome of these patients. We used the Columbia Classification for FSGS for the renal biopsy.

Results: There were two subgroups of FSGS patients; those with nephrotic syndrome and those without nephrotic syndrome. Patients with FSGS with non-nephrotic syndrome had poorer survival rates compared to the nephrotic group. For those without nephrotic syndrome, the indices responsible for progression involved more tubular and blood vessel lesions in addition to glomerular pathology compared to those with nephrotic syndrome. Patients with FSGS with nephrotic syndrome responded to immunosuppressants more favorably compared to the non-nephrotic group, though both groups responded with decreasing proteinuria. The nephrotic group had a better 10-year long-term survival rate of 92 vs. 72% for the non-nephrotic group (log-rank 0.002). The 10-year survival for the whole group of FSGS patients was 64%.

Conclusion: Our data suggest that in FSGS, one of the significant components of the disease is the vascular and tubular damage, apart from the underlying glomerular pathology, resulting in varying responses to therapy, and the difference is reflected in inherently poorer response to immunosuppressant therapy in those without nephrotic syndrome as opposed to those with nephrotic syndrome, who responded to immunosuppressant therapy (IST) with stabilization of renal function and had less blood vessel and tubular lesions.

Lecithin-cholesterol acyltransferase (LCAT) deficiency is an autosomal recessive disorder that can reveal two different diseases: a very interesting nephrological picture of complete enzyme deficiency characterized by the association of dyslipidemia, corneal opacities, anemia, and progressive nephropathy; and a partial form (fish-eye disease) with dyslipidemia and progressive corneal opacities only. We report herein the case of a 35-year-old man who presented hypertension, renal symptomatology of rapidly progressive glomerulonephritis associates: nephrotic proteinuria, severe renal failure, in combination with annular corneal opacities, anemia, and dyslipidemia. The diagnosis of familial LCAT deficiency was confirmed by clinical examination, characteristic dyslipidemia, undetectable LCAT levels in plasma, and positive family history.

Background: There is little published information on the natural history and the treatment of immune complex membrano-proliferative glomerulonephritis (IC-MPGN) of unknown cause in the transplanted kidney.

Materials and methods: From 01/2004 to 12/2018, 41 patients had the diagnosis of post-transplant idiopathic IC-MPGN and were included in the study.

Results: The mean age of the cohort at the time of transplant was 50 ± 13 years. The most common presentation was increased proteinuria, followed by kidney dysfunction. Fewer than 50% of patients had hematuria at presentation. 25 patients (61%) had no change in their baseline immunosuppression after the diagnosis of idiopathic IC-MPGN. Eight patients (19.5%) received steroids alone, and 8 patients (19.5%) received rituximab with (7) or without (1) steroids. The patients who received rituximab had better uncensored graft survival than the patients who received no treatment (p = 0.02), but the benefit of steroids compared to no treatment did not reach statistical significance (p = 0.05). The multivariate analysis retained eGFR < 30 mL/min/1.73m² at time of diagnosis (HR = 3.30, p = 0.02; 95% Cl 1.15 - 9.46) as a significant predictor of graft loss. In this analysis, treatment of idiopathic IC-MPGN was associated with lower graft loss (HR = 0.22, p = 0.02; 95% Cl 0.06 - 0.78).

Conclusion: To the best of our knowledge, this is the largest clinic-pathological series of post-transplant idiopathic IC-MPGN. Treatment of idiopathic IC-MPGN may be associated with better graft outcomes.

Background: Recently, evidence has emerged that the ubiquitin system, which is involved in extracellular protein degradation, is most susceptible to damage in podocytes in cases of podocytopathies. We studied anti-ubiquitin carboxyl-terminal hydrolase L1 (UCHL1) antibodies in glomerulopathies with proteinuria.

Materials and methods: 71 patients with glomerulopathy and 11 healthy subjects were included in our study. 44 patients had nephrotic syndrome, and 27 did not. Serum levels of anti-UCHL1 antibodies were measured by ELISA.

Results: The levels of anti-UCHL1 antibodies were significantly higher in focal segmental glomerulosclerosis (FSGS) patients than in minimal change disease (MCD), IgA nephropathy, membranous nephropathy, or membranoproliferative glomerulonephritis patients and control group. The levels of UCHL1 antibodies in serum did not correlate with 24-hour proteinuria, blood pressure, glomerulosclerosis percentage, or area of tubulointerstitial fibrosis, but did correlate with serum creatinine and estimated glomerular filtration rate (eGFR). During the development of the ROC curve (AUC = 0.766 (95% CI 0.634 - 0.897)) for FSGS vs. other forms of glomerulopathies, a readjustment of the sensitivity of 75% and specificity of 61% were established. A former increase in anti-UCHL1 antibody levels above 1.93 ng/mL may be a marker of FSGS OR 3.617 (95% CI 1.051 - 12.447), p = 0.041.

Conclusion: An increase in the level of anti-UCHL1 antibodies in the serum was noted in FSGS, which suggests that these antibodies could be a potential biomarker for FSGS patients.

Objective: To evaluate the effectiveness of a video-based exercise intervention on depression and sleep conditions of peritoneal dialysis (PD) patients.

Materials and methods: Participants satisfying the inclusion and exclusion criteria were recruited from the Peritoneal Dialysis Center, Department of Nephrology of the First Affiliated Hospital of Guangxi Medical University, and they were divided into an intervention group and a control group. The intervention group received video-based exercise intervention while the control group received routine care. Then their BDI-II and PSQI scores were collected and analyzed before and after the intervention.

Results: There were no significant differences in BDI-II score and the seven components of PSQI score between the two groups at the baseline measures (p > 0.05). However, after 12 weeks of intervention, the intervention group had a significantly lower score in BDI-II (p < 0.05) and in six components of PSQI (p < 0.05), with the exception of the "use of sleep medications" subscale (p > 0.05). Their depression level also improved significantly after intervention (p < 0.05), while that of the control group did not change significantly(p > 0.05).

Conclusion: The video-based exercise intervention is safe and can remarkably improve the depression and sleep conditions of PD patients and is thus worthwhile to be applied widely.

Introduction: Patients with end-stage renal disease (ESRD) on dialysis and COVID-19 infection have an increased risk of in-hospital mortality, but whether these patients have a higher long-term mortality risk is unknown.

Materials and methods: Retrospective chart review of 958 patients admitted with COVID-19 infection or those with ESRD admitted for any other reason between February 2020 and August 2020. We collected data on demographics, comorbidities, laboratory tests, and mortality. The primary outcome was all-cause 1-year mortality. The secondary outcome was in-hospital mortality. We used primarily logistic regression models to assess the mortality risk.

Results: In total, 651 patients without ESRD with COVID-19 (COVID+ESRD-), 259 with ESRD without COVID-19 (ESRD+COVID-), and 48 with ESRD with COVID-19 (COVID+ESRD+) were hospitalized between February 2020 and August 2020. Patients were followed after discharge until September 2021. The all-cause 1-year mortality rates were 24% in patients with COVID+ESRD-, 22% in ESRD+COVID- patients, and 40% in those with COVID+ESRD+ (p < 0.05). Compared to the COVID+ESRD- group, the unadjusted and adjusted odds ratio (OR) for all-cause 1-year mortality in the COVID+ESRD+ group was 2.13 (95% confidence interval (CI), 1.16 - 3.91) and 2.15 (95% CI,1.12 - 4.14), respectively. The unadjusted and adjusted OR for all-cause in-hospital mortality in the COVID+ESRD+ group was 1.79 (95% CI, 0.92 - 3.49); and 1.79 (95% CI, 0.88 - 3.65), respectively. We found no statistically significant difference between the COVID+ESRD- and ESRD+COVID- groups for both in-hospital and 1-year mortality (p > 0.05).

Conclusion: Patients with COVID+ESRD+ have significantly higher odds for all-cause 1-year mortality compared to COVID+ESRD- patients. Future studies should investigate the mechanisms of long-term mortality risk in ESRD patients with COVID-19 infection.

Background: We aimed to investigate the role of red blood cell distribution width (RDW)-to-platelet ratio (RPR) and mean platelet volume (MPV) in evaluating the disease activity of microscopic polyangiitis (MPA).

Materials and methods: A total of 73 newly diagnosed MPA patients and 57 healthy controls were enrolled in this study. The hematologic and biochemical indexes of two groups were assessed. The RPR was calculated as the ratio of RDW and platelet counts, and Birmingham Vasculitis Activity Score (BVAS) was used to evaluate the disease activity.

Results: Compared with the healthy controls, RPR and RDW were significantly increased, and MPV was significantly decreased in MPA patients. In the MPA group, RPR was positively correlated with BVAS (p = 0.032), but negatively correlated with lymphocyte, hemoglobin, and complement 3 (all p < 0.05). MPV was negatively correlated with white blood cell (p = 0.045). Patients with BVAS > 15 had significantly higher RPR than patients with BVAS ≤ 15 (p = 0.011). A cut-off level of 0.066 for RPR had 47.9% sensitivity and 90.4% specificity in predicting MPA, and the combination of RPR and MPV had 75.3% sensitivity and 78.9% specificity in differentiating MPA patients from healthy controls.

Conclusion: The study suggests that RPR may be a potential marker of diagnosis and disease activity in newly diagnosed MPA patients. Additionally, a higher predictive value in monitoring and evaluation of MPA was found when RPR and MPV were combined.