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IntroductionAnti-glomerular basement membrane (anti-GBM) disease is a severe entity with few therapeutic options including plasma exchange and immunosuppressive agents. The aim of this study was to analyze the clinical and pathological features that predict the evolution of end-stage kidney disease (ESKD) and the kidney survival in a cohort of patients with anti-GBM disease with renal involvement in real life.MethodsA retrospective multicentre observational study including 72 patients from 18 nephrology departments with biopsy-proven anti-GBM disease from 1999 to 2019 was performed. Progression to ESKD in relation to clinical and histological variables was evaluated.ResultsCreatinine at admission was 8.6 (± 4) mg/dL and 61 patients (84.7%) required dialysis. Sixty-five patients (90.3%) underwent plasma exchange. Twenty-two patients (30.6%) presented pulmonary hemorrhage. Kidney survival was worse in patients with creatinine levels > 4.7 mg/dL (3 vs. 44% p < 0.01) and in patients with > 50% crescents (6 vs. 49%; p = 0.03). Dialysis dependence at admission and creatinine levels > 4.7 mg/dL remained independent significant predictors of ESKD in the multivariable analysis [HR (hazard ratio) 3.13 (1.25–7.84); HR 3 (1.01–9.14); p < 0.01]. The discrimination value for a creatinine level > 4.7 mg/dL and 50.5% crescents had an area under the curve (AUC) of 0.9 (95% CI 0.82–0.97; p < 0.001) and 0.77 (95% CI 0.56–0.98; p = 0.008), respectively. Kidney survival at 1 and 2 years was 13.5 and 11%, respectively. Patient survival at 5 years was 81%.ConclusionIn real life, patients with severe anti-GBM disease (creatinine > 4.7 mg/dL and > 50% crescents) remained with devastating renal prognosis despite plasma exchange and immunosuppressive treatment. New therapies for the treatment of this rare renal disease are urgently needed.

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Introduction: Retrospective observational study to evaluate the technique of cannulation guided by ultrasound of the left internal jugular vein (LIJV) using a lateral oblique axis (LOAX) approach with variable angulation in the placement of tunneled central venous catheters (CVC) for hemodialysis. Methods: Seventy-one patients with 77 LIJV vascular accesses aged 16 or older who needed CVC for hemodialysis were evaluated. The catheters were inserted, guided by LOAX ultrasound with variable angulation, depending on the angulation of the left brachiocephalic trunk. The success rate, additional instrumentation needs, and number of immediate and late complications were analyzed. Findings: Central venous catheters placement was possible in all cases and none of the peelable introducers folded. A placement guide was needed in only eight patients, whose brachiocephalic trunk elongation and angulation was 90. We found no major complications, and only five cases of minor complications (6.5%): four periprocedural and one displacement of the catheter a week after placement. Discussion: Tunneled CVC percutaneous cannulation in LIJV guided by ultrasound with the LOAX approach with variable angulation provides very good results, allows visualization of the needle and the vascular structures at the same time, and reduces the number of manoeuvers required for placement and complications that might arise.

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