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Introduction Recurrence of atypical hemolytic uremic syndrome (aHUS) in renal allografts is common, leading to dialysis and graft failure. Pretransplant versus posttransplant initiation of eculizumab treatment in patients with aHUS has not been rigorously investigated. We hypothesized eculizumab pretransplant would reduce dialysis incidence posttransplant. Methods Of patients enrolled in the Global aHUS Registry ( n = 1549), 344 had ≥1 kidney transplant. Of these, 188 had received eculizumab. Eighty-eight patients (47%) were diagnosed with aHUS and received eculizumab before, and during, their most recent transplant (group 1). A total of 100 patients (53%; group 2) initiated eculizumab posttransplantation. This second group was subdivided into those diagnosed with aHUS before ( n = 52; group 2a) or after ( n = 48; group 2b) their most recent transplant. Results Within 5 years of transplantation, 47 patients required dialysis; the risk of dialysis after transplantation was significantly increased in group 2b (hazard ratio [HR] 4.6; confidence interval [CI] 1.7–12.4) but not 2a (HR 2.3; CI 0.9–6.2). Graft function within 6 months of transplantation was significantly better in group 1 (median estimated glomerular filtration rate of 60.6 ml/min per 1.73 m 2 ) compared with 31.5 and 9.6 ml/min per 1.73 m 2 in groups 2a ( P = 0.004) and 2b ( P = 0.0001), respectively. One meningococcal infection (resolved with treatment) and 3 deaths (deemed unrelated to eculizumab) were reported. Conclusions Outcomes for transplant patients with aHUS treated with eculizumab were improved compared with previous reports of patients with aHUS not treated with eculizumab. Our findings suggest delayed aHUS diagnosis and therefore treatment is associated with an increased risk of dialysis posttransplantation and reduced allograft function.

Background: Over the past decade, the management of chronic kidney disease-mineral and bone disorder has changed substantially, altering the pattern of bone disease in chronic kidney disease. We aimed to evaluate the natural history of kidney bone disease in contemporary kidney transplant recipients and dialysis patients. Design, settings, participants, and measurement: Sixty-one dialysis patients referred to kidney transplantation participated in this prospective cohort study during November 2009 and December 2010. We performed baseline bone biopsies while the patients were on dialysis and repeated the procedure in 56 patients two years after kidney transplantation or two years after baseline if transplantation was not performed. Measurements of mineral metabolism and bone turnover as well as dual energy X-ray absorptiometry scans were obtained concurrently. Results: 37 of 56 participants received a kidney transplant, of which 27 underwent successful repeat bone biopsy. The proportion of patients with high bone turnover declined from 63% at baseline to 19% two years after kidney transplantation, while the proportion of those with low bone turnover increased from 26% to 52%. Of 19 participants remaining on dialysis after two years, 13 underwent successful repeat biopsy. The proportion of patients remaining on dialysis with high bone turnover decreased from 69% to 31%, and low bone turnover increased from 8% to 38%. Abnormal bone mineralization increased in transplant recipients from 33% to 44%, but decreased in patients remaining on dialysis from 46% to 15%. Trabecular bone volume showed little change after transplantation, but low bone volume increased in patients remaining on dialysis. Bone mineral density did not correlate with histomorphometric findings. Conclusions: Bone turnover decreased over time both in patients remaining on dialysis and in kidney transplant recipients. Bone mineral density and bone biomarkers were not associated with bone metabolism changes detected in bone biopsy.

Background: Bone and mineral disorders may contribute to extraosseous ossifications and cardiovascular disease (CVD) in end-stage renal disease (ESRD) patients. We have investigated the relationship between bone mineral density (BMD) and inflammation, wasting, CVD and mortality in ESRD patients. Methods: BMD (dual energy X-ray absorptiometry) and biochemical, nutritional and inflammatory markers were assessed in 277 incident ESRD patients (GFR 7.1 ± 0.2 ml/min) who were then followed prospectively for a mean of 27 (range 1–60) months. Carotid plaques were determined in 103 patients. Results: Patients with carotid plaques, clinical manifestation of CVD and wasting (assessed by subjective global assessment) had significantly lower BMD than their counterparts. Low BMD was associated with high all-cause and cardiovascular mortality. Even after adjustment for several confounders and risk factors, all-cause (HR = 2.1, CI: 1.1–3.9, p = 0.02) and cardiovascular (HR = 2.8, CI: 1.2–6.3, p = 0.02) mortality remained significantly associated with low BMD. Conclusions: Low BMD is associated with wasting and CVD, and is an independent predictor of all-cause and cardiovascular mortality in ESRD patients.