Journal of Nephrology最新文献

Background: Anemia in children on maintenance hemodialysis (HD) leads to poor quality of life. Our study aimed to assess and monitor anemia and iron status management in children on maintenance HD over 18 months using key performance indicators.

Methods: Key performance indicators, formulated as the percentage of patients achieving the KDIGO (2012) guideline-recommended targets for hemoglobin (Hb) (11-12 g/dl), transferrin saturation (TSAT) (20-40%) and serum ferritin (200-500 ng/ml), were reported quarterly over the 18-month-period of this study.

Results: This study was carried out over an 18 month-period, from April 1st, 2020, till October 31st, 2021. A total of 78 patients (45 males and 33 females) were included; mean age 12.16 ± 3.3 years and HD duration range 3.0-140.88 months, median 16.51 months. The three most common primary causes of CKD were Congenital Anomalies of the Kidney and Urinary Tract (CAKUT) (29.5%), unknown cause (24.4%), and chronic glomerular diseases (20.5%). The quarterly reported percentages of patients achieving the recommended targets for Hb, TSAT, and serum ferritin ranges were 18.2-35.7%, 38.8-57.1%, and 11.9-26.6%, respectively.

Conclusion: Although the mean Hb trend was nearing the KDIGO (2012) target, the key performance indicators showed that only a small percentage of our HD patients were achieving the targets for Hb, TSAT, & serum ferritin, thus alerting us to the need to revise our protocol for the management of anemia and iron status.

Background: Kidney transplantation is the best mode of kidney replacement therapy. However, the shortage of organ donations has been a major challenge globally. Relatives of patients with end-stage kidney disease (ESKD) are potential kidney donors. We explored their perspectives about kidney donation, kidney commercialisation, and barriers to kidney donation.

Methods: In-depth interviews were conducted among 28 relatives of ESKD patients across the six geopolitical zones and Federal Capital Territory of Nigeria. The interview focused on potential sources of kidney donors, kidney commercialisation and barriers to kidney donation. ATLAS.ti version 9.0.22.0 was used for data analysis.

Results: Mean age of the study participants was 41.57 ± 14.55 years; 54% were females, 60.7% were married, 93% had tertiary education and 75% were first degree relatives of ESKD patients. There were 7 themes and 28 subthemes generated in this study. The potential sources of kidney donors identified by the study participants included commercial, hospital, family and non-family member donors. While some opined that a family member is the best choice as a kidney donor, others preferred a commercial donor. The majority of those interviewed do not believe that it is wrong to purchase a kidney, and would be willing to do so. Identified factors that promote kidney commercialisation were unwillingness of a family member to donate, having the financial capacity to purchase a kidney, non-fitness of family members to donate. Identified barriers to kidney donation were age, poor health status, polygamy, perceived poor expertise of the medical team, perceived risk of the procedure, parental influence and religious beliefs.

Conclusions: The majority of participants lacked correct information about kidney donation. Implementation of educational program policies and laws regulating and reinforcing ethical principles of kidney donation and transplantation should be ensured.

Background: Steroid-resistant nephrotic syndrome (SRNS) is the second most frequent cause of chronic kidney disease in children and young adults. The most severe form of steroid-resistant nephrotic syndrome is congenital nephrotic syndrome Finnish type (CNSF), caused by biallelic loss-of-function variants in NPHS1, encoding nephrin. Since each of the 68 monogenic causes of steroid-resistant nephrotic syndrome represents a rare cause of the disease, tailoring therapeutic interventions to multiple molecular targets remains challenging, suggesting gene replacement therapy (GRT) as a viable alternative. To set the ground for a gene replacement study in vivo, we established rigorous, quantifiable, and reproducible phenotypic assessment of a conditional Nphs1 knockout mouse model.

Methods: By breeding a floxed Nphs1^fl/- mouse (Nphs1^tm1Afrn/J) previously studied for pancreatic β-cell survival with a podocin promoter-driven Cre recombinase mouse model (Tg(NPHS2-Cre)^295Lbh/J), we generated mice with podocyte-specific nephrin deficiency (Nphs1^fl/fl NPHS2-Cre +).

Results: We observed a median survival to postnatal day P5 in nephrin-deficient mice, whereas heterozygous control mice and wild type (WT) control group showed 90% and 100% survival, respectively (at P50 days). Light microscopy analysis showed a significantly higher number of renal-tubular microcysts per kidney section in nephrin-deficient mice compared to the control groups (P < 0.0022). Transmission electron microscopy demonstrated reduced foot process (FP) density in nephrin-deficient mice compared to controls (P < 0.0001). Additionally, proteinuria quantitation using urine albumin-to-creatinine ratio (UACR) was significantly higher in nephrin-deficient mice compared to controls.

Conclusions: This study represents the first comprehensive description of the kidney phenotype in a nephrin-deficient mouse model, laying the foundation for future gene replacement therapy endeavors.

Background: Peritoneal dialysis (PD) is a widely-used renal replacement therapy while low ultrafiltration volume usually results in technique failure. Retroperitoneal leakage has been reported to be one of the causes of acquired decline in ultrafiltration. The present study investigated retroperitoneal leakage in PD patients and explored related risk factors.

Methods: This study was designed as a prospective, observational study. A total of 420 PD patients regularly followed up at our center were enrolled from May 2011 to July 2021 and followed until December 2021. Retroperitoneal leakage was determined by magnetic resonance peritoneography and was used as the endpoint. Patients with retroperitoneal leakage were given intermittent PD or temporary hemodialysis (HD) as therapy. Cox regression models were used to identify risk factors for retroperitoneal leakage.

Results: The cohort was followed up for up to 125.0 months (median: 46.4 months; interquartile range: 16.6 months). During the follow-up, 68 patients developed retroperitoneal leakage, with 31 (45.6%) cases occurring within the first year after PD initiation. A total of 62 (91.2%) patients recovered from retroperitoneal leakage and resumed their original PD regimen. Multivariate Cox regression analysis revealed that age and gender were independent predictors for retroperitoneal leakage. Younger males were more likely to develop retroperitoneal leakage. In females, waistline and body mass index (BMI) were found to be risk factors for retroperitoneal leakage.

Conclusions: Retroperitoneal leakage was common in PD patients with ultrafiltration insufficiency and was usually reversible after appropriate treatment. Age and gender were independent risk factors for retroperitoneal leakage.

Background: The aim of this study was to evaluate the impact on the national health system of COVID-19 infection in vaccinated patients undergoing haemodialysis.

Methods: From the cohort of vaccinated dialysis patients enrolled in 118 dialysis centres, we calculated hospitalisation incidence in COVID-19-infected subjects. COVID-19-related hospitalisations and ICU admissions were analysed over two time periods (prior to administration of the third dose and following administration of the third dose of vaccine) and adjusted for several co-variates. Using the general population as the reference, we then calculated the Standardized Incidence Ratio (SIR) of hospitalisation.

Results: Eighty-two subjects out of 1096 infected patients were hospitalised (7.5%) and sixty-four hospitalisations occurred among the 824 infected persons after the third dose. Age ≥ 60 years (Adj RR 2.91; 95% CI 1.34-6.30) and lung disease (Adj RR = 2.45; 95% CI 1.32-4.54) were the only risk factors associated with hospitalisation. The risk of ICU admission in the second time period (Time 2) was reduced by 86% (RR = 0.14; 95% CI 0.03-0.71) compared to the first time period (Time 1). The SIR of hospitalisation (SIR 14.51; 95% CI 11.37-17.65) and ICU admission (SIR 14.58; 95% CI 2.91-26.24) showed an increase in the number of events in dialysis patients compared to the general population.

Conclusions: Our analysis revealed that while the second variant of the virus increased infection rates, it was concurrently associated with mitigated severity of infections. Dialysis patients exhibited a higher susceptibility to both COVID-19 hospitalisation and ICU admission than the general population throughout the pandemic.