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The Yanomamo Indians live in the tropical equatorial rain forests of northern Brazil and southern Venezuela. They essentially eat a salt free diet. As such, they have been demonstrated to have minimal 24 h urine sodium excretion, markedly elevated serum renin and aldosterone levels, and low to normal blood pressure (BP) throughout their lives. We present an unusual case of a 75-year-old woman, with chronic kidney disease (CKD), stage 1 hypertension (HTN), and a partial colectomy who maintained a strict low salt diet while using a significant amount of a stimulant laxative (bisacodyl) on a daily basis.

ABSTRACT Background Humoral responses to coronavirus disease 2019 (COVID-19) vaccines in hemodialysis (HD) patients can direct vaccination policy. Methods We compared 409 COVID-19-naïve HD patients from 13 HD units in Israel to 148 non-dialysis-dependent COVID-19-naïve controls. Twenty-four previously infected (antinucleocapsid positive) HD patients were analysed separately. Blood samples were obtained ≥14 days post-vaccination (BNT162b2, Pfizer/BioNTech) to assess seroconversion rates and titers of anti-spike (anti-S) and neutralizing antibodies. Results The median time from vaccination to blood sample collection was 82 days [interquartile range (IAR) 64–87] and 89 days (IQR 68–96) for HD patients and controls, respectively. Seroconversion rates were lower in HD patients compared with controls for both anti-S and neutralizing antibodies (89% and 77% versus 99.3%, respectively; P < 0.0001). Antibody titers were also significantly lower in HD patients compared with controls {median 69.6 [IQR 33.2–120] versus 196.5 [IQR 118.5–246], P < 0.0001; geometric mean titer [GMT] 23.3 [95% confidence interval (CI) 18.7–29.1] versus 222.7 [95% CI 174–284], P < 0.0001, for anti-S and neutralizing antibodies, respectively}. Multivariate analysis demonstrated dialysis dependence to be strongly associated with lower antibody responses and antibody titers waning with time. Age, low serum albumin and low lymphocyte count were also associated with lower seroconversion rates and antibody titers. HD patients previously infected with sudden acute respiratory syndrome coronavirus 2 (SARS-CoV-2) had no difference in their seroconversion rates or antibody titers compared with COVID-19-naïve patients. Conclusion This study demonstrates diminished and waning humoral responses following COVID-19 vaccination in a large and diverse cohort of HD patients, including those previously infected with SARS-CoV-2. Considering these results and reduced vaccine effectiveness against variants of concern, in addition to continued social distancing precautions, a third booster dose should be considered in this population. Graphical Abstract Graphical Abstract

Effective systemic therapies suppress toxic light chain production leading to an increased proportion of patients with light chain (AL) amyloidosis who survive longer albeit with end-stage renal disease. There is a critical need to identify patients in this population who benefit from renal transplantation. This multicenter, observational study from five countries includes 237 patients with AL amyloidosis who underwent renal transplantation between 1987 and 2020. With a median follow-up of 8.5 years, the median overall survival from renal transplantation was 8.6 years and was significantly longer in patients with complete and very good partial hematologic responses (CR + VGPR) compared to less than VGPR (9 versus 6.8 years; HR: 1.5, P = 0.04 [95% CI: 1–2.1]) at renal transplantation. Median graft survival was 7.8 years and was better in the CR + VGPR group (8.3 vs 5.7 years, HR: 1.4, P = 0.05 [95% CI: 1–2]). The frequency and time to amyloid recurrence in the graft was also lower (16% vs 37%, p = 0.01) and longer (median time not achieved vs 10 years, p = 0.001) in the CR + VGPR group. Comparing CR vs. VGPR there was no difference in overall or graft survival. Although 69 patients (29%) experienced hematologic relapse, treatment effectively prevented graft loss in the majority (87%). Renal transplantation in selected AL amyloidosis patients is associated with extended overall and renal graft survival. Patients with hematologic CR or VGPR have the most favorable outcomes, and these patients should be considered for renal transplantation.

Background Chronic kidney disease (CKD) manifested as reduced GFR and/or albuminuria, has been known to accelerate arterial stiffness and early vascular aging (EVA). Diabetes, hypertension, and glomerular disorders are the leading causes of CKD and renal failure. The question which etiology contributes more to this vascular phenomenon-hypertensive and diabetic CKD or CKD secondary to immune-mediated glomerulonephritis—remained unclear. Objective To compare pulse wave velocity (PWV), a marker of arterial stiffness, between CKD patients of different etiologies: hypertensive and diabetic nephrosclerosis. vs. CKD secondary to glomerular disorders. Methods Clinical data were collected on 56 patients followed at the Nephrology and Hypertension Institute in Samson Assuta Ashdod University Hospital. All patients had at least one visit at our Nephrology clinics prior to recruitment. All patients with a glomerular disorder had a clinical-pathological diagnosis based on a recent kidney biopsy. Pulse wave velocity (PWV) was measured using a validated Sphygmocor XCEL ® device. Univariate and multivariate analyses were performed to compare PWV between hypertensive/diabetic CKD and CKD secondary to glomerular disorders. Results PWV was significantly higher in the hypertensive/diabetic CKD group, compared to the CKD-GN group, with an average of. 12.2 m/s vs 8.3 m/s, respectively (p < 0.001). In a multivariate linear regression model, having CKD secondary to glomerulonephritis was associated with a significantly lower PWV (B = − 3.262, p < 0.001), compared with CKD secondary to hypertension and diabetes, with adjustment of age, creatinine, and comorbidities. Conclusion CKD Patients secondary to glomerulonephritis, have lower PWV when compared to CKD patients with diabetes and/or hypertension, even after adjusting for age, renal function, and the presence of comorbidities. It is intriguing to further study the possible protective role of immunosuppression on the arterial properties of CKD patients.

The long-term risk associated with resistant hypertension compared to other phenotypes of hypertension is still unclear. We aimed to assess cardiovascular and renal outcomes over 10 years of follow-up of patients with uncontrolled resistant hypertension (uRH) compared to a similarly treated (≥ 3 medication classes including a diuretic) and adherent group whose blood pressure is under control. This retrospective cohort study utilized the computerized database of Maccabi Healthcare Services, a state-mandated health provider covering 25% of the Israeli population. Clinical outcomes were assessed using Cox regression multivariable analyses. A total of 1487 patients (50% males, mean age at baseline = 68.3 ± 10.4 years) were included in the uRH cohort and 1343 patients (50% males, 66.2 ± 10.6 years) in the controlled hypertension reference group (Controlled hypertension on multi drug regimen- CH-MDR). After adjusting for age, sex, BMI and patients’ comorbidities, uRH was associated with a Hazard Ratio of 1.35 (95% CI: 1.08–1.69) for incidence of ischemic heart disease, 1.51 (1.06–2.16) for secondary cardiovascular events, and 1.36 (1.00–1.86) for risk of stroke or transient ischemic attack compared to the reference group. Patients with uRH were found to have more hospitalization days (mean, 4.2 vs. 3 days per year, p < 0.001), and more emergency room visits (83.3% vs. 77%, p < 0.001). Overall, uRH was associated with a 19% (95% CI 11% to 29%) increase in direct healthcare expenditures during the first year of follow-up. uRH is associated with a substantial increased risk of both cardiovascular and cerebrovascular events, when compared to similarly treated hypertensive patients whose blood pressure is under control.

Background Genetic kidney diseases contribute a significant portion of kidney diseases in children and young adults. Nephrogenetics is a rapidly evolving subspecialty; however, in the clinical setting, increased use of genetic testing poses implementation challenges. Consequently, we established a national nephrogenetics clinic to apply a multidisciplinary model. Methods Patients were referred from different pediatric or adult nephrology units across the country if their primary nephrologist suspected an undiagnosed genetic kidney disease. We determined the diagnostic rate and observed the effect of diagnosis on medical care. We also discuss the requirements of a nephrogenetics clinic in terms of logistics, recommended indications for referral, and building a multidisciplinary team. Results Over 24 months, genetic evaluation was completed for a total of 74 unrelated probands, with an age range of 10 days to 72 years. The most common phenotypes included congenital anomalies of the kidneys and urinary tract, nephrotic syndrome or unexplained proteinuria, nephrocalcinosis/nephrolithiasis, tubulopathies, and unexplained kidney failure. Over 80% of patients were referred due to clinical suspicion of an undetermined underlying genetic diagnosis. A molecular diagnosis was reached in 42/74 probands, yielding a diagnostic rate of 57%. Of these, over 71% of diagnoses were made via next generation sequencing (gene panel or exome sequencing). Conclusions We identified a substantial fraction of genetic kidney etiologies among previously undiagnosed individuals which influenced subsequent clinical management. Our results support that nephrogenetics, a rapidly evolving field, may benefit from well-defined multidisciplinary co-management administered by a designated team of nephrologist, geneticist, and bioinformatician. Graphical abstract A higher resolution version of the Graphical abstract is available as Supplementary information