International Urology and Nephrology最新文献

Purpose: We described our experience in the management of PUV at UNIOSUN Teaching Hospital, Osogbo, Osun State.

Methods: This was a retrospective analysis of patients with diagnosis of PUV carried out over a 4 year period (2019-2023). Demographic characteristics, clinical presentation, diagnosis and mode of therapeutic interventions were the variables analysed.

Results: A total of 17 patients with PUV were managed during the study period. Median age at presentation and surgery were 10 months (range 3.0-48.0) and 13 months (range 3.0-49.5), respectively. Most common presentation was poor urinary stream, 11 (64.7%). Mean PCV was 34.42%. Klebsiella aerogens was the predominant 9 (52.9%) organism isolated. A patient had prenatal USS diagnosis suggestive of PUV, majority (52.9%) had bilateral grade 1 V hydronephrosis at presentation. Voiding cystogram was diagnostic in 14 patients, (82.4%) while urethrocystoscopy was done in 14 (82.4%) patients. Median creatinine level were 116, 76.5 and 51.0 (micromol/l) pre- and post-catheterization and 1 month post-surgery, respectively. There was positive correlation between the age and post- surgery creatinine but a negative correlation between the PCV and grade of hydronephrosis. All patients had Mohans valvotomy. We had mortality in a patient from urosepsis. At 6 months-1 year follow-up, 15 patients had good urine stream with stable renal function.

Conclusion: Early intervention assist in optimizing renal and bladder function and minimize risk of urosepsis. Where there is no facility for endoscopic valve ablation, Mohans valvotomy remains a viable treatment option.

Purpose: We performed the study to investigate the association between heart rate (HR) non-dipping pattern and target organ damage in patients with chronic kidney disease (CKD) and hypertension.

Methods: In this cross-sectional study, 447 patients with CKD and hypertension were enrolled. 24 h ambulatory blood pressure monitoring was conducted. Linear regression and logistic regression analysis were conducted to investigate the association between HR non-dipping pattern and target organ damage, including estimated glomerular filtration rate (eGFR), left ventricular mass index (LVMI), and left ventricular hypertrophy (LVH).

Results: Overall, 261 patients (58.4%) followed non-dipping patterns of HR. HR non-dipping pattern remained to be significantly associated with reduced eGFR (β: -0.384; 95% CI: -0.719 to -0.050; p = 0.025) and the higher prevalence of CKD stages 4-5 (OR: 2.141; 95% CI: 1.153 to 3.977; p = 0.016). Meanwhile, HR non-dipping pattern was independently associated with LVMI (β: 0.021; 95% CI: 0.000 to 0.041; p = 0.049) and LVH (OR: 1.78; 95% CI: 1.07 to 2.96; p = 0.027) after adjusting for confounding factors.

Conclusions: HR non-dipping pattern was independently associated with impaired renal function and cardiac damage. Non-dipping HR deserves further attention and needs to be detected and treated during the management of CKD patients.

Fabry Disease (FD) is a genetic disease caused by a deficiency in the activity of lysosomal galactosidase A (α-GalA), an enzyme responsible for the catabolism of globotriaosylceramide (Gb3). Since lysosomes are present throughout the body and play a crucial role in catabolism and recycling of cytosolic compounds, FD can affect multiple organs and result in various symptoms, including renal, cardiovascular, neurological, cutaneous, and ophthalmic manifestations. Due to the nonspecific symptoms and the rarity of FD, it is often diagnosed late in life. However, introducing targeted therapies such as enzyme replacement therapy (ERT) and chaperone therapy has significantly improved FD's natural history and prognosis by restoring α-GalA enzyme activity. Despite the advancements, there are limitations to the currently available therapies, which has prompted research into new potential treatments for FD, including alternative forms of enzyme replacement therapy, substrate reduction therapy, mRNA therapy, and genetic therapy. In this review, we analyze the epidemiology, pathophysiology, and treatment of FD, with particular emphasis on promising therapeutic opportunities that could shift the treatment of this rare disease from a standardized to a personalized approach soon.

Purpose: In patients with chronic kidney disease (CKD), renal osteodystrophy may be associated with a progressive bone mass loss that increases fracture risk. Denosumab, a monoclonal antibody inhibiting osteoclast activity, is an antiresorptive medication used for the treatment osteoporosis.

Methods: Its efficacy and safety were initially established in the FREEDOM study, showing a significant reduction in incident fractures in osteoporotic women treated with denosumab. Subsequent post hoc analyses showed its efficacy in patients stratified by kidney function, but these analyses did not include patients with advanced stages of CKD. The capability of denosumab in improving bone mineral density in uremic patients was evaluated in 12 studies including 461 dialysis patients with low bone mineral density. The improvement of bone mineral density was the final end point in these studies assessed during a follow-up of 6-60 months. Nine of these studies did not have hyperparathyroidism among criteria for patient inclusion and their participants may have low-turnover bone disease. Despite current recommendations, no patients underwent bone biopsy before denosumab therapy.

Results: Overall, findings in these studies suggest that denosumab is a viable option for promoting bone mass recovery in patients with advanced stages of CKD having either high or low serum levels of PTH. However, the increase of bone mineral density was lower in patients with low serum markers of bone turnover at baseline. These studies also highlighted the need for calcium and vitamin D supplementation to prevent hypocalcemia that remains a serious concern.

Conclusions: Denosumab emerges as a potentially safe and effective option for enhancing bone health in CKD patients.

Background: This study aimed to investigate the role of uric acid (UA) in diabetic nephropathy (DN) from epidemiological and genetic perspectives.

Methods: We used data from the 2007-2016 National Health and Nutrition Examination Survey to evaluate the relationship between UA and DN risk using weighted multivariate-adjusted logistic regression. Subsequently, a two-sample Mendelian randomization study was conducted using genome-wide association study summary statistics. The main inverse variance weighting (IVW) method and supplementary MR method were used to verify the causal relationship between UA and DN, and sensitivity analysis was conducted to confirm the credibility of the results.

Results: Our observational study enrolled 4363 participants with diabetes mellitus from NHANES, among them, 2682 (61.4%) participants were identified as DN. The multivariate logistic regression model showed that compared with those without hyperuricemia, the DN risk of the hyperuricemia population was significantly increased (P < 0.05). The MR results suggest a direct causal effect of hyperuricemia on DN (IVW odds ratio (OR): 1.37 (95% confidence interval 1.07-1.76); P = 0.01), which is consistent with findings from other MR methods.

Conclusion: The evidence from observational studies indicates a positive correlation between HUA and the onset of DN. And the causal effects of HUA on DN were supported by the MR analysis.

Objective: The aim of this study is to determine whether there is a relationship between peritoneal membrane permeability and dyspepsia in peritoneal dialysis patients.

Patients and methods: This study included 95 peritoneal dialysis patients aged 18 and older. The presence of dyspepsia in patients was recorded according to the 2016 ROME-IV Functional Dyspepsia Diagnostic Criteria. Subsequently, the Glasgow Dyspepsia Severity Score questionnaire was administered to assess the severity of dyspepsia. Endoscopy was performed for those who agreed to exclude organic pathology, or the results of endoscopy conducted within the last 2 years were recorded. Furthermore, stool samples were examined for H. pylori to exclude organic causes of dyspepsia. PET (peritoneal equilibration test) and Kt/V values of patients were calculated using the "PD Adequest" computer software. PET values were categorized as low and low-normal for low permeability and high and high-normal for high permeability.

Results: Dyspepsia was detected in 51.6% of all peritoneal dialysis patients. H. pylori was found positive in 11.6% of all patients and 12.2% of those with dyspeptic symptoms. There was no significant difference in the rate of H. pylori occurrence between low and high permeability groups. The Glasgow Dyspepsia Severity Score did not differ significantly between H. pylori-positive and -negative patients. Dyspepsia was more frequent and severe in the low permeability group. Dyspepsia in the low permeability group was mostly considered as functional dyspepsia due to the predominance of normal endoscopic findings.

Conclusion: Dyspepsia is a common health problem in approximately half of peritoneal dialysis patients. Dyspepsia observed in those with low peritoneal membrane permeability is generally of functional origin. Furthermore, the frequency and severity of dyspepsia are higher in individuals with low permeability. When planning peritoneal dialysis for these patients, the current status should be taken into consideration, and patients should be informed about necessary precautions and recommendations.

Objective: High serum IgA and low serum C3 levels resulting from lectin and alternative pathway activation might be related to IgA nephropathy (IgAN) progression and exacerbation. This study examined whether the serum IgA/C3 ratio can serve as an IgAN progression marker.

Methods: (1) This nationwide multicenter retrospective study in Japan included 718 patients with biopsy-confirmed IgAN. The patients whose serum creatinine levels at the time of renal biopsy had doubled were defined as having disease progression. (2) Furthermore, to investigate the pathological significance of a reduction in serum IgA/C3 ratio, we reviewed 63 patients whose serum IgA and C3 data at the end of the observation period were obtained.

Results: (1) A Kaplan-Meier analysis of the patients with IgAN revealed that the group with a high serum IgA/C3 (≥ 3.3) had a significantly worse renal outcome. In a multivariate analysis of eGFR ≥ 60 mL/min per 1.73m² at the time of biopsy, poor renal outcome was significantly predicted by a serum IgA/C3 ratio of ≥ 3.3. (2) A 15% reduction in the change of serum IgA/C3 ratio was associated with a significantly higher percentage of complete remission of proteinuria. Among the four groups divided by treatment, both the serum IgA/C3 ratio and proteinuria were reduced only in the tonsillectomy and steroid pulse group.

Conclusion: The serum IgA/C3 level might reflect the disease activity and be a potent surrogate marker of therapeutic efficacy in patients with IgAN.