Saudi Journal of Kidney Diseases and Transplantation最新文献

The data available on immunoglobulin A (IgA) deposition disease indicate an inherited predisposition to the disease with autoimmune triggering. Hence, we prospectively evaluated the role of a new autoimmune regimen in the treatment of severe nephrotic or nephritic flares associated with noncrescentic nephritis in adult patients. Thirty-six patients were included, and the regimen consisted of an initial 3-month induction phase of prednisone and mycophenolate mofetil (MMF), followed by a maintenance phase of MMF alone for 21 months. Complete remission (CR) (normalization of creatinine clearance [CrCl] and a decrease in protein output to <500 mg/day) was achieved in 29 of 36 patients, and a partial response (no further decline in CrCl and a decrease in proteinuria to <50%) was seen in seven patients. CrCl was maintained in patients with CR but was mildly reduced in partially responsive ones. Our study showed the short- and longterm safety and efficacy of this autoimmune regimen directed toward the autoimmune triggering factors in severe forms of noncrescentic IgA nephritis.

Hyperphosphatemia is an electrolyte disorder highly prevalent in patients with chronic kidney disease undergoing hemodialysis (HD) that usually requires treatment with oral phosphate binders (PBs). Sucroferric oxyhydroxide (SO) is a calcium-free, iron-based PB indicated for the control of serum phosphorus. In the real-world setting, SO has shown clinical effectiveness with a lower pill burden and has also been associated with reduced hospital admission rates. This study aims to assess the potential economic benefits resulting from the introduction of SO to the health-care setting of the Kingdom of Saudi Arabia (KSA). An economic analysis using data from a retrospective real-world study that compared HD patients with uninterrupted SO prescriptions with patients who discontinued SO and switched to other PBs (oPBs). Annual drug costs for the estimated PB-eligible population in KSA were quantified. Costs per responder were estimated for all treatments. Hospital admissions' incidence rates were converted into annual inpatient cost savings and were deducted from drug costs to estimate the annual economic effect of SO versus oPBs. Sensitivity and breakeven analyses were also conducted. The eligible population for PB therapy in KSA was estimated at n = 14,748. Treating therapy-eligible populations exclusively with SO was estimated to generate annual inpatient cost-savings of SAR 107.4-119.4 million compared to treating the population with oPBs. The estimated economic effect signified overall annual savings ranging from SAR 82.8 to SAR 94.8 million when the population is treated with SO. Sensitivity analyses showed persistent cost savings. The estimated benefit-cost ratios showed that for every SAR 1 spent on SO, the expected return on investment was SAR 4.4-4.9. SO is an effective therapy that may result in substantial cost savings from reducing hospital admission costs that are attributable to hyperphosphatemia among HD patients.

According to the current guidelines, renal biopsies are performed in systemic lupus erythematosus (SLE) patients for proteinuria of 0.5 g/24 h or higher. Renal pathology may be present in patients with lower-level proteinuria (<0.5 g/24 h). We aimed to review the renal histopathology in SLE patients, with lower levels of proteinuria. In this retrospective study, we retrieved SLE patients' data, including 24-h urinary protein excretion and renal histopathology results. We compared various parameters in different lupus nephritis (LN) classes and in different levels of proteinuria (urinary protein <0.5 g, 0.5 to <1 g, and ≥1 g per 24 h). Out of 476 patients, 274 (57.6%) had proteinuria of <0.5 g, 44 (9.2%) had 0.5 to <1 g, and 158 (33.2%) had ≥1 g per 24 h. SLE patients with proteinuria of <0.5 g/24 h were found to have LN, including the proliferative classes. Of the 299 LN cases confirmed by a renal biopsy, low-level proteinuria (<0.5 g) was found in 39.8% of all LN patients, in 50% of patients with Class III LN, 33.3% of those with Class IV LN, 31.4% of those with Class V LN, and 41.4% of those with other LN classes (II/V, III/V, and IV/V). Overall, 35.9% (87/242) of patients with the proliferative LN classes (III, IV, V, II/V, III/V and IV/V) had low-level proteinuria of <0.5 g/24 h. SLE patients with low-level proteinuria had significant renal pathology. Our study suggests there is a need to perform renal biopsies at lower levels of proteinuria.

Management of renal malignancies in Von Hippel-Lindau (VHL) is challenging. We present six patients [mean age = 35.1 years (range: 24-54), males = 5] with VHL syndrome with multiple bilateral renal malignancies and the lessons learned during their management. The number of tumors at the time of presentation ranged from 1 to 6, while the number of new lesions varied from 1 to 3. Different combinations of radical nephrectomy (n = 2), partial nephrectomy (n = 7), and focal therapy (n = 6) were used appropriately. Median follow-up was 36 months (range: 12-72). Two patients developed new lesions which were managed with focal therapy. Nephron-sparing approaches are successful even in bilateral, multifocal, large, and recurring renal tumors associated with VHL. Awareness about the availability of efficacious surgical and minimally invasive measures would reduce psychosocial problems faced by patients and their families due to the social stigma associated with malignancies running in a family and burden of renal replacement therapy.

In patients with chronic kidney disease (CKD), restricting dietary salt is recommended to prevent fluid retention. Rapid weight loss is often observed when CKD patients with a high salt intake are hospitalized and started on a low-salt diet. We investigated the effects of 7-day dietary salt restriction on weight loss in hospitalized patients with CKD. During the 7-day hospitalization, a low-salt (6 g/day) and low-protein (0.6-0.8 g/kg/day ideal body weight) diet was served to all patients. Urine samples were collected for the first 24 h after admission, and patients were divided into quartiles (Q1-Q4) by urinary salt excretion. Body weight was measured on days 1 and 7. Weight loss after admission was compared among the groups. Factors associated with weight loss were evaluated by multivariate logistic regression. The mean age of the patients was 70.3 ± 11.7 years, and 73% were male. Mean weight loss was 1.6 ± 1.7 kg on day 7. Weight loss was significantly greater in Q3 and Q4 than in Q1 (P = 0.009 and P <0.001, respectively). In the univariate analysis, weight loss correlated positively with 24-h urinary salt excretion on admission (γ2 = 0.146) and body mass index (γ2 = 0.223). The 24-h urinary salt excretion on admission and BMI were independently associated with weight loss of >2 kg. The adjusted odds ratios (95% confidence interval) were 1.24 (1.13-1.36) and 1.15 (1.07-1.22), respectively. Twenty-four-hour urinary salt excretion on admission is useful for predicting significant weight loss with short-term dietary salt restriction.

Intradialytic hypertension (IDH) is an important emerging complication in hemodialysis patients. No study has examined the diagnostic markers of various risk factors for the occurrence of IDH in chronic hemodialysis patients. Therefore, our study aimed to assess the use of nitric oxide (NO) as a marker of IDH among end-stage renal disease patients. The patients were divided into two groups: Group I (40 patients) with IDH and Group II (40 patients) without IDH. For all participants, a full medical history was taken, followed by laboratory examinations to measure the level of NO and a clinical examination. The dose of erythropoietin per week, the level of intact parathyroid hormone, and platelet count were significantly higher in Group I than in Group II, whereas the mean level of NO (2.10 ± 1.23 pmol/L) was highly significantly lower in patients with IDH (P < 0.001). Multivariate analysis showed that hypertension (odds ratio: 1.824, 95% confidence interval: 1.273-2.982) and the level of NO (odds ratio: 1.68, 95% confidence interval: 1.13-2.97) were independent risk factors for IDH. The receiver operating characteristic curve showed that the cutoff point of NO was 2.52 μmol/L to differentiate between cases with and without IDH (area under the curve = 0.844). Our findings support previous research regarding the involvement of endothelial dysfunction and a higher sodium level in the pathogenesis of IDH. We also found that the NO level had a good diagnostic value for the occurrence of IDH at a cutoff of 2.52 μmol/L.

The prevalence of glomerulonephritis (GN), especially membranous GN (MGN), changes from time to time. This change may be due to genetic predisposition, environmental factors race, age, and indications for a renal biopsy. This study was conducted to evaluate the distribution and changing patterns of GN by further assessing the prevalence of MGN. A 1000, 123 biopsies were performed from January 2012 to October 2019 in Hospital Serdang and Hospital Kuala Lumpur. Electron microscopy, immunohistochemistry, and clinical presentations were used to differentiate primary and secondary MGN, from which 611 and 457 primary and secondary subjects were diagnosed with primary and secondary GN, respectively. Primary MGN accounts for 13% of all the primary GN, while lupus nephritis (LN) accounts for 44.2% of all secondary GN followed by diabetes mellitus (25.6%). The proportions of primary and secondary MGN were 64.8% and 35.2%, respectively, with a male-to-female ratio of 1:1.1 in favor of females. The renal biopsy obtained from the registry of two prominent hospitals in Malaysia provided valuable prevalence and demonstrated changes in the prevalence of GN in Malaysia. Notwithstanding, immunoglobulin A nephropathy and LN remain the most common causes of primary and secondary GN in Malaysia.

Information on the genetic profile of congenital nephrotic syndrome (CNS) from India is scarce. The management of CNS is largely supportive of the setting of developing countries, mainly via the administration of intravenous albumin infusions, angiotensin-converting enzyme inhibitors, and levothyroxine. Inadequate infrastructure and management facilities, including genetic analyses, further hamper the outcome. These infants may progress to end-stage renal disease, and mortality is high in infancy. Here, we report a case series of four infants (aged 14-60 days) with CNS from our center with genetic mutations (including mutations in the NPHS1 and LAMB2 genes) that were not described in previous reports from India. Although responsiveness to enalapril has been documented in anecdotal reports of NPHS1 mutations, our case series of four infants did not exhibit any response to enalapril. Our case series adds to the existing literature regarding the genetic profile of CNS in India.

Acquired perforating dermatosis (APD) is an adult skin disease characterized by an umbilicated papulonodular rash with transepidermal elimination of dermal components such as collagen and/or elastin. It is frequently associated with multiple medications and diseases such as diabetes and chronic renal failure. It is a disabling disease with severe pruritus in 83.3% of cases and generalized ulcerating lesions that are associated with infections and scarring. Nearly 10% of renal patients are affected. Supportive measurements of disease activity and previous medications failed to halt its natural progression. In our study, we documented significant improvements in the severity of the disease as measured by the eczema area and severity index (EASI), in 32 patients with the renal disease through the use of mycophenolate mofetil (MMF), with EASI decreasing from 31 [interquartile range (IQR) = 4] to 3 (IQR = 4) by the 3rd month. Moreover, such changes persisted for up to 2 years despite a decrease in the dose of MMF to half after 1 year. In conclusion, our study showed that MMF is a safe and effective immunosuppressive drug for short- and intermediate-term therapy of severe APD and confirmed its autoimmune etiology.

The prevalence of hypertension (HTN) among youngsters has increased recently. Often, it is underrecognized owing to a lack of routine blood pressure measurement in many health centers, partly due to the unavailability of instruments and possibly because of this perception that it is not the foremost problem in children. There is less information about the parental perception of childhood HTN from Bangladesh. We aimed to conduct this survey among parents to see the perception and knowledge about this childhood HTN, which can be served as a baseline for future reference. This cross-sectional study was carried out in Dr. MR Khan Shishu Hospital and ICH from April 2021 to September 2021 over 6 months. Parents who visited our hospital, both in outpatient and inpatient, for their children aged (3 years-17 years) were included in this study. Parents who did not give permission were excluded. Purposive sampling was done during the study period. Parents were interviewed with a structured questionnaire. Parents' educational status and the idea about HTN in children were recorded. The data were analyzed and expressed as frequency and percentage. A total of 352 respondents were interviewed with a mean age of 28 ± 11 years. Among these respondents, 187 (53.2%) were male, and 165 (46.8%) were female. In this study, most of the participant parents are literate; only a negligible proportion, 5.6%, are illiterate. Eighty-nine percent of participants know high blood pressure is HTN and 83.5% know HTN can be detected by measuring BP. Among the participants, 83% think children do not develop HTN, and only 17% responded that children could develop HTN. This study offers insight into parents' lack of awareness of childhood HTN. Hence, proper knowledge and understanding of pediatric HTN play a crucial role in early detection and management.