Clinical nephrology最新文献

Background: Fibronectin glomerulopathy (FGP), also known as fibronectin deposition glomerulopathy (GFND), is a rare hereditary autosomal dominant glomerular disease. Its clinical manifestations are proteinuria, hematuria, hypertension, and hyperkalemic distal renal tubular acidosis, which often progresses slowly to end-stage renal disease.

Case description: We report a 21-year-old woman with fibronectin glomerulopathy who underwent renal puncture at the age of 10. The pathology was considered to be thrombotic microangiopathy, and it was not treated regularly. This time, renal puncture was performed again due to proteinuria combined with elevated serum creatinine. Light microscopy showed severe mesangial matrix hyperplasia of glomeruli with dense deposition and foam cell aggregation in capillary loops. Fibrinogen immunostaining was positive. Electron microscope showed severe hyperplasia of mesangial matrix, and a large amount of electron-dense matter deposited in mesangial area. Perfect genetic testing suggested that the FN1 gene was heterozygous for NM_212482.4 (c.2918A>G), that is, Y973C mutation. Therefore, she was diagnosed with fibronectin glomerulopathy and was given sacubitril valsartan sodium tablets 200 mg b.i.d. orally.

Conclusion: We report a case of a patient with fibronectin glomerulopathy and review the literature of this disease. The disease often has insidious onset, and fibronectin deposition is a typical pathological change that can result. The disease slowly progresses to end-stage renal disease. At present, there is no specific treatment. It is advocated to use reninangiotensin-aldosterone system blockers to strictly control blood pressure and proteinuria, and the overall prognosis is poor. Genetic testing techniques may be helpful in early diagnosis of the disease.

Objective: The safety and efficacy of denosumab in patients undergoing hemodialysis for osteoporosis remain underexplored. Therefore, guidelines are unclear regarding whether denosumab can be safely used in these patients. This study aimed to present the experience of denosumab treatment in a small cohort of patients for up to 3 years.

Materials and methods: This study evaluated the effects of denosumab on bone metabolism in 12 patients with end-stage kidney disease (ESKD) and osteoporosis undergoing long-term hemodialysis in an observational cohort setting. These patients were maintained on high doses of calcium carbonate (1,250 mg/day) and vitamin D (1,000 IU/day) supplementation during denosumab treatment to prevent hypocalcemia. Eleven patients with ESKD undergoing hemodialysis who did not receive osteoporosis treatment were followed for the same period as a control group. Changes in biochemical markers, bone mineral density (BMD), and clinical outcomes were analyzed over a 3-year follow-up period.

Results: The results revealed no significant differences in lumbar spine BMD but indicated a trend toward high femoral BMD values in the denosumab treatment group at the 2- and 3-year follow-up points. However, 2 patients experienced severe hypocalcemia. Most cases of denosumab discontinuation were due to a lack of BMD improvement.

Conclusion: These findings highlight the potential of denosumab in managing osteoporosis in patients undergoing hemodialysis but also underscore the need for careful monitoring of calcium levels to mitigate adverse effects. Therefore, adequate calcium replacement is required to prevent severe hypocalcemia. This study provides valuable real-world evidence to guide therapeutic strategies for improving bone health in patients with ESKD.

Objective: The aim of this study is to assess renal outcomes and long-term prognosis associated with different therapeutic regimens, including supportive care and immunosuppressive therapy, in patients with IgA nephropathy (IgAN) presenting with partial crescent formation. The objective is to provide evidence-based recommendations for the management of early-stage crescentic IgAN.

Materials and methods: A single-center retrospective cohort study was conducted, involving 134 adults diagnosed with biopsy-proven IgAN and partial crescents. Participants were categorized into three groups: the renin-angiotensin system inhibitors (RASI) group, the prednisone (P) group, and the P plus cyclophosphamide (CTX) group. Data were extracted from hospitalization records and outpatient follow-ups over a 2-year period. The primary outcomes measured were the decline in estimated glomerular filtration rate (eGFR) and reduction in proteinuria.

Results: Patients in the P+CTX group had comparatively worse baseline clinical parameters than the other two groups - for example, higher proteinuria, lower serum albumin, and poorer renal function. Nevertheless, following 6 months of immunosuppressive therapy, 60.00% of patients in the P+CTX group achieved effective remission. Urinary protein levels in this group decreased from 1,534.00 mg/day at baseline to 289.50 mg/day at the 24-month follow-up. Renal function remained stable within the P+CTX group throughout the follow-up period. Although the effective remission rate was comparable among all groups after the sixth month, no significant differences were observed in the incidence of endpoint events across the three groups. Thus, the P+CTX group presented significant improvements in proteinuria.

Conclusion: Despite more severe baseline disease, the P+CTX regimen was associated with a greater reduction in proteinuria in IgAN patients with minor crescent formation. It is important to note, however, that causal inference was limited by residual confounding. Despite adequate short-term safety, extended follow-up is mandatory to fully evaluate the long-term complications of CTX.

Atypical anti-glomerular basement membrane (GBM) nephritis is a rare autoimmune disease characterized by the linear deposition of immunoglobulin G (IgG) detected in the GBM without circulating anti-GBM antibodies or lung involvement. Atypical anti-GBM disease is distinguished from typical anti-GBM disease in both clinical and pathological features. Herein, we report 2 patients who developed mild proteinuria, hematuria, and elevated serum creatinine levels following coronavirus disease 2019 (COVID-19) vaccination. Renal biopsy found bright linear IgG deposition along the GBM, but anti-GBM antibodies were seronegative. Atypical anti-GBM nephritis was determined. The female patient improved with the treatment of valsartan. For the male patient, serum creatinine levels significantly decreased through two plasma exchange sessions, cyclophosphamide, and glucocorticoid. Atypical and typical anti-GBM nephritis are different clinical entities. They target different antigen epitopes and, therefore, atypical anti-GBM disease is not the early stage of typical anti-GBM disease. In atypical anti-GBM nephritis, monoclonal IgG deposition in the glomeruli is not equal to plasma cell dyscrasias but still needs close monitoring for hematologic diseases. The relationship between the disease and COVID-19 vaccination is uncertain and needs further exploration.

Aim: The safety and efficacy of warfarin vs. those of direct oral anticoagulants (DOACs) in kidney transplant (KT) recipients are unclear. Therefore, anticoagulation therapy outcomes in KT recipients were compared.

Materials and methods: This retrospective study included 57 KT recipients on outpatient anticoagulation therapy at Ulsan University Hospital between July 1998 and May 2021. Major bleeding, thromboembolic events, and mortality were compared between warfarin and DOAC groups. Kaplan-Meier plot and Cox regression were used to assess between-group differences.

Results: Median follow-up was 140 months for the warfarin group and 92.5 months for the DOAC group. No significant differences were observed for baseline characteristics between the warfarin (n = 33) and DOAC (n = 24) groups, except for atrial fibrillation or flutter. No significant differences were observed in major bleeding, thromboembolic events, or mortality. Kaplan-Meier analysis also showed no group differences in these outcomes. In multivariable Cox regression, age and sex were significantly associated with major bleeding, whereas thromboembolic events were higher in the DOAC group.

Conclusion: Major bleeding and mortality rates were comparable; however, thromboembolic events were more frequent in the DOAC group. Further research on DOAC subtypes, drug interactions, and dosing is warranted.

Introduction: Limited information exists regarding acute kidney injury (AKI) and kidney replacement therapy (KRT) in pediatric trauma in South Africa. This study aims to summarize the management of AKI and KRT in the pediatric population (<  13 years) admitted to a level 1 Trauma and Burns intensive care unit between January 2018 and September 2023.

Materials and methods: A retrospective chart review from the Trauma Registry at Inkosi Albert Luthuli Central Hospital in Durban, South Africa, examined demographics, mechanism of injury, injury severity score, management, outcome, and imaging. Patients with AKI were classified according to the Kidney Disease Improving Global Outcomes (KDIGO) classification adapted for children.

Results: 51 (17.5%) of 291 patients were classified as having AKI. Ten patients were classified as stage 1 (19.6%), 15 as stage 2 (29.4%), and 26 as stage 3 (51%). Of these 51, only 9 (17.7%) patients were treated with KRT, with 1 survivor (11.1%). 24 patients were considered to have a poor prognosis and did not receive KRT (47.1%), while 18 (35.3%) patients recovered without the need for KRT. The average length of stay was 14 days. Six (66.7%) of the 9 KRT patients were diagnosed with AKI on day 1, and 1 (1.1%) patient each on day 2, day 4, and day 5. Overall renal recovery was 35.3% for all patients.

Conclusion: In this South African pediatric cohort, most young children who presented with AKI in the post-trauma or burns group either recovered without KRT or died from multi-organ dysfunction. A small percentage required KRT, and of these, few survived.

IgA nephropathy (IgAN) is the most common primary glomerular disease in the world; it is associated with the intestinal microbiota, diet, genetics, etc., and is mainly diagnosed by kidney biopsy. Patients with IgAN may develop end-stage renal disease (ESRD) within decades of diagnosis, placing an enormous burden on patients and society. Therefore, early prediction and effective measures are needed to prevent disease progression. To date, a large number of studies have explored biomarkers of IgAN progression. In this paper, IgAN biomarkers are discussed to guide the early diagnosis, prevention, and treatment of this disease.

Background and aims: Minor lower-limb amputations are limb and potentially life-saving procedures. However, they are associated with serious adverse events, including acute kidney injury (AKI).

Materials and methods: We conducted a single-center retrospective study to determine the incidence of AKI after these procedures, identify risk factors, and assess impact on patient survival.

Results: We included 201 patients. AKI occurred in 18.9% using AKIN criteria, and 24.9% using KDIGO criteria. Only 1 patient required temporary dialysis. Patients with AKI were older (73.0 ± 10.4 vs. 68.5 ± 11.8 years, p = 0.033), had a higher incidence of chronic kidney disease (CKD); estimated glomerular filtration rate (eGFR) < 45 mL/min/1.73m² (39.5 vs. 14.7%, p = 0.001), and/or chronic obstructive pulmonary disease (COPD) (28.9 vs. 13.5% p = 0.028), and higher use of diuretics (68.4 vs. 49.1%, p = 0.049), fluoroquinolones (71.1 vs. 52.8% p = 0.047), and/or carbapenems (10.5 vs. 2.5%, p = 0.043) compared to patients without AKI. eGFR < 45 mL/min/1.73m² (OR: 3.24, CI: 1.40 - 7.52, p = 0.006), use of fluoroquinolones (OR: 3.19, CI: 1.30 - 7.82, p = 0.012), and day-1 C-reactive protein (CRP) (OR: 1.01, CI: 1.00 - 1.01, p = 0.009) were established as independent risk factors for AKI. Cumulative survival was not significantly lower in patients with AKI (log rank: 0.02, p = 0.88).

Conclusion: AKI is a potential complication following minor lower-limb amputations. Age, COPD, diuretics, fluoroquinolones, and carbapenems were associated with increased incidence of AKI. An eGFR < 45 mL/min/1.73m², day-1 C-reactive protein, and fluoroquinolone use were identified as independent risk factors for AKI.

Introduction: This study is a retrospective analysis of patients undergoing maintenance hemodialysis (MHD) at our institution. The objective is to assess bone density and the prevalence of osteoporosis among these patients, as well as to analyze associated risk factors.

Materials and methods: A total of 131 MHD patients undergoing regular dialysis treatment at our hemodialysis center from September 2022 to December 2023 were included, and bone mineral density (BMD) values of lumbar spine (L1 - L3) of the patients were measured using QCT. Data were analyzed using the SPSS computer software version 26.0 to assess the relationship between BMD and clinical biochemical parameters in end-stage renal disease patients.

Results: We found that of the 131 patients included, 82 were males and 49 were females, with a male to female ratio of 1.67 : 1, age 56.92 ± 13.37 years, and dialysis age of 24 (12 - 60) months. In the overall population, 25 cases (19.1%) were osteoporotic, 45 cases (34.4%) had low bone mass, and 61 cases (46.56%) had normal bone mass. Regarding risk factors, BMD was significantly negatively correlated with age (β = -1.788, p < 0.001), hypertension (β = -21.605, p = 0.018) and significantly positively correlated with total iron-binding capacity (β = 0.803, p = 0.01). Further logistics regression showed that age, decreased total iron binding, calcium-phosphorus product, and intact parathyroid hormone (iPTH) ≥ 300 pg/mL were independent risk factors for osteoporosis in MHD patients.

Conclusion: Abnormal BMD is prevalent in patients with MHD. Age, decreased total iron binding capacity, calcium-phosphorus product, and iPTH ≥ 300 pg/mL are independent risk factors for the development of osteoporosis in patients undergoing maintenance hemodialysis.

Introduction: The prevalence of hypertension in lupus nephritis varies according to studies and can be as high as 74%. The aim of this study was to determine the prevalence of hypertension in lupus nephritis and to search for factors associated with hypertension and the occurrence of major adverse cardiovascular events (MACE).

Materials and methods: This was a multicenter, retrospective, descriptive, and analytical study over a 10-year period from January 1, 2012, to December 31, 2022. It targeted patients followed for lupus nephritis confirmed by anatomo-pathological examination in three nephrology departments in Dakar. We compared hypertensive and non-hypertensive patients to identify hypertension-associated factors.

Results: During the study period, 73 cases of lupus nephritis were collected. In the study population, the mean age was 33.90 years, with a sex ratio of 0.30. The prevalence of hypertension was 40.1%. 23 patients were class III, 25 class IV, and 19 class V. Among hypertensive patients, mean creatinine was 33.7 mg/L, and renal failure was present in 56.66% of patients. Mean proteinuria was 5.42 g/24h. Hypertension-associated factors were age (OR = 1.15, 95% CI: 1.05 - 2.25; p = 0.001), renal failure (OR = 12.872, 95% CI: 2.23 - 74.28; p = 0.004), and proliferative class (OR = 18.83, 95% CI: 1.91 - 185.25; p = 0.012). For the cardiovascular events, there were 3 cases of stroke, 0 cases of heart attack, and 0 cardiovascular deaths.

Conclusion: Hypertension in lupus nephritis is common in our setting. Hypertension-associated factors were related to advanced age and severity of lupus nephritis. Long-term follow-up would be necessary to better detect cardiovascular events.