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Background Aim Methods Results Conclusion Fabry disease is a rare X‐linked multisystem disease, with progressive proteinuric kidney disease contributing significantly to morbidity and mortality of these patients. Evidence shows that sodium‐glucose cotransporter 2 inhibitors (SGLT2Is) can reduce proteinuria and slow progression to end‐stage kidney disease in both diabetic and non‐diabetic kidney disease.Evaluate the effects of SGLT2I on kidney function and albuminuria in patients with Fabry disease.Single‐centre real‐world case series reviewing electronic medical records of patients with Fabry disease who initiated therapy with dapagliflozin or empagliflozin (n = 11). Changes in urine albumin–creatinine ratio (uACR) and creatinine before and after treatment with SGLT2I were analysed using Wilcoxon signed‐rank test. Two‐tailed P‐values <0.05 were considered significant.Eleven patients were followed for up to 19 months after commencement of SGLT2I. An overall significant reduction in albuminuria (P = 0.05) was seen with SGLT2I use in the Fabry cohort. Median uACR before SGLT2I was 76 mg/mmol (interquartile range (IQR) 47–141) and after SGLT2I was 39 mg/mmol (IQR 18–95) (P = 0.05). All patients with uACR >100 mg/mmol had reduction in albuminuria over the study period. SGLT2Is were well tolerated overall, with only one case resulting in cessation of treatment due to adverse effects.These results suggest SGLT2Is can significantly reduce albuminuria in a portion of patients with Fabry‐related kidney disease and offer additional treatment for Fabry nephropathy. Given the nature of the study design and small case numbers, further long‐term controlled studies are required to evaluate the long‐term efficacy of this medication class in both cardiac and renal outcomes in Fabry disease. [ABSTRACT FROM AUTHOR]

The article in the Journal of Magnetic Resonance Imaging discusses the reliability and agreement of manual region of interest (ROI) segmentation methods in tissue sodium magnetic resonance imaging (23Na MRI). The study found good to excellent inter-rater and intrarater reliability for skin, leg muscle, tibial bone, and whole leg tissue segmentation. However, there was a clinically unacceptable bias in mean sodium concentration when comparing different skin segmentation methods. The research highlights the importance of establishing standards in ROI segmentation methods for wider clinical use of 23Na MRI technology. [Extracted from the article]

Background Calciprotein particles (CPP) are colloidal aggregates of calcium phosphate and the mineral-binding protein fetuin-A, and are potential mediators of cardiovascular disease in chronic kidney disease (CKD). Emerging evidence suggests non-calcium-containing phosphate binders may reduce serum CPP in patients with kidney failure who require dialysis; however, it is unclear whether similar interventions are effective in patients with earlier stages of CKD. Methods The IMpact of Phosphate Reduction On Vascular End-points in CKD (IMPROVE-CKD) was a multi-centre, placebo-controlled, randomized trial of lanthanum carbonate on cardiovascular markers in 278 participants with stage 3b/4 CKD. In this pre-specified exploratory analysis, primary (CPP-I) and secondary CPP (CPP-II) were measured in a sub-cohort of participants over 96 weeks. Treatment groups were compared using linear mixed-effects models and the relationship between serum CPP and pulse wave velocity (PWV) and abdominal aortic calcification (AAC) was examined. Results A total of 253 participants had CPP data for baseline and at least one follow-up timepoint and were included in this analysis. The mean age was 62.4 ± 12.6 years, 32.0% were female and the mean estimated glomerular filtration rate (eGFR) was 26.6 ± 8.3 mL/min/1.73 m2. Baseline median serum CPP-I was 14.9 × 104 particles/mL [interquartile range (IQR) 4.6–49.3] and median CPP-II was 3.3 × 103 particles/mL (IQR 1.4–5.4). There was no significant difference between treatment groups at 96 weeks in CPP-I [22.8% (95% confidence interval −39.2, 36.4), P = 0.65] or CPP-II [−18.3% (95% confidence interval −40.0, 11.2), P = 0.20] compared with a placebo. Serum CPP were not correlated with baseline PWV or AAC, or with the progression of either marker. Conclusions Lanthanum carbonate was not associated with a reduction of CPP at 96 weeks when compared with a placebo in a CKD cohort. [ABSTRACT FROM AUTHOR]

Background: Indigenous Australians are disproportionately affected by end stage kidney disease. Despite this, they face significant delays being assessed and waitlisted for kidney transplant. Aims: To examine the kidney transplant waitlisting process in our region, to compare the workup process between Indigenous Australians and non‐Indigenous patients, and identify major sources of delay. Methods: We analysed the records of all patients being treated by our service who were on the kidney transplant waitlist between January 2017 and June 2018. Between‐group differences were used to compare the time between commencement of dialysis and completion of each component of assessment. Patients who had more than 1 year between commencement of dialysis and waitlisting were further analysed for major sources of delay. Results: Twenty‐five patients were included (20 Indigenous Australians and 5 non‐Indigenous). The median time to waitlisting for transplant after commencing dialysis was significantly longer in the Indigenous group (1215 vs 264 days, P = 0.032). Indigenous Australian patients waited longer before commencing the transplant assessment process and before completing dental assessment, tissue typing and review by the transplant nephrologist and surgeon. Five patients (two Indigenous Australians, three non‐Indigenous) were waitlisted within 1 year of commencing dialysis. Among the remaining 20 patients, cardiac and systems issues were the two most common major sources of delay. Conclusion: Indigenous Australian patients face significant delays accessing the kidney transplant waitlist. Cardiac assessment and systems issues are prominent sources of delay and efforts to address these areas may help to improve equity of access to kidney transplantation. [ABSTRACT FROM AUTHOR]