Leukocyte chemotactic factor 2 amyloidosis (ALECT2) is a recently described form of amyloidosis that most frequently manifests clinically with progressive renal failure. In a series of 414 cases of amyloidosis, there were 40 cases of ALECT2: the second most common type of renal amyloidosis in this series. This was particularly common in Hispanic patients in the Southwest United States, where more than half of amyloidosis cases were ALECT2. It is possible that this represents a familial amyloidosis as there were two brothers with ALECT2 in our study. Morphologically, there was consistent amyloid deposition in the renal cortex with medullary involvement in only about a third of cases. There were no mutations detected in the LECT2 gene, although all patients tested were homozygous for the G nucleotide in a non-synonymous SNP at position 172. Most patients presented with chronic kidney disease and, on follow-up, showed progression with an average deterioration in renal function of 0.5 ml/min/1.73 m(2) per month. Unfortunately, the etiology of ALECT2 is currently unknown and there is currently no efficacious treatment of the disease.

Background and objectives: A bicarbonate dialysate acidified with citrate (CD) has been reported to have local anticoagulant effect. This study examines the effect of CD on dialysis efficiency, measured as eKt/Vurea, and predialysis concentrations of BUN, creatinine, phosphate, and ␤-2 microglobulin in chronic dialysis units.Design, settings, participants, & measurements: Three outpatient chronic hemodialysis units with 142 patients were switched to CD for 6 mo. Using each patient's prior 6 mo on regular bicarbonate dialysate acidified by acetate (AD) as control, eKt/Vurea was compared with that of CD. Follow-up data for 7 mo after the study were collected from about one-half of the participants remaining on CD and the others returned to AD.Results: eKt/Vurea, increased (P < 0.0001) from pre-CD value of 1.51 ؎ 0.01 to 1.57 ؎ 0.01 with CD. During CD use ␤-2 microglobulin levels declined (P ‫؍‬ 0.0001) from 28.1 ؎ 10.0 to 25.9 ؎ 10.0. Similarly, the concentrations of BUN, creatinine, and phosphate also decreased on CD (P < 0.008). In the poststudy period, eKt/Vurea for the patients staying on CD remained unchanged at 1.60 ؎ 0.17 versus 1.59 ؎ 0.18 (P ‫؍‬ NS), whereas in those returning to AD the eKt/Vurea decreased from 1.55 ؎ 0.20 to 1.52 ؎ 0.17 (P < 0.0001).Conclusions: Data suggest that CD use is associated with increased solute removal.

Objective: The high pill burden of many phosphate binders (PBs) may contribute to increased prevalence of hyperphosphatemia and poor nutritional status observed among patients undergoing maintenance hemodialysis therapy. We examined the real-world effectiveness of sucroferric oxyhydroxide (SO), a PB with low pill burden, in managing serum phosphorus in patients with prevalent hemodialysis over a 1-year period.Design: Historical cohort analyses of de-identified electronic medical records. Subjects: In-center hemodialysis patients switched from another PB to SO therapy as part of routine care with 12 months of uninterrupted SO prescriptions recorded, and documented serum phosphorus levels were eligible for inclusion. Clinical data were extracted from a pharmacy service, FreseniusRx, database and Fresenius Kidney Care clinical data warehouse.Main outcome measures: Comparisons were made between the 91-day period before SO initiation (i.e., baseline) and the 4 consecutive 91-day intervals of SO treatment (Q1-Q4). Clinical measures included achievement of target phosphorus levels (#5.5 mg/dL) and mean number of PB pills/day.Results: Among 530 analyzed patients, the proportion achieving target serum phosphorus levels increased by .100% 1 year after switching to SO therapy, that is, from 17.7% at baseline to 24.5%, 30.5%, 36.4%, and 36.0% at Q1 through Q4, respectively (P , .0001 for all). Reductions in serum phosphorus were observed at all follow-up timepoints (P , .0001), irrespective of baseline PB. From a mean baseline PB pill burden of 8.5 pills/day, patients experienced an average 50% pill burden reduction during SO treatment (P , .0001). Phosphorus-attuned albumin and phosphorus-attuned protein intake (normalized protein catabolic rate) improved significantly after transition to SO (P , .0001). The effectiveness of SO was evident in prespecified subgroups of interest (i.e., black/African-American patients, Hispanic/Latino patients, and women).Conclusion: Among patients on hemodialysis, switching to SO resulted in a 2-fold greater likelihood of achieving target phosphorus levels while halving daily PB pill burden. Increases in phosphorus-attuned albumin and protein intake suggest improved nutritional status.

Aims: Hyperphosphatemia has been associated with an increased risk of mortality in patients with end-stage renal disease. We sought to assess the real-world effectiveness of sucroferric oxyhydroxide (SO), an iron-based phosphate binder (PB), in control of serum phosphorus levels, and to determine the associated pill burden in hemodialysis patients. Materials and methods: Adult, in-center hemodialysis patients first prescribed SO through a renal pharmacy service as part of routine clinical care between April 1, 2014 and March 31, 2015 were included in the analysis. The proportion of patients with phosphorus levels ≤ 5.5 mg/dL and the mean prescribed PB pills/day were compared between baseline (3 months prior to SO) and SO follow-up at 3 (SO 1 – 3) and 6 months (SO 4 – 6). Mineral bone disease markers, hemoglobin, iron indices, and erythropoiesis-stimulating agents and intravenous iron use were assessed. Results: At baseline, all patients (n = 1,029) were prescribed PB, and 13.9% had mean serum phosphorus ≤ 5.5 mg/dL. Comparing baseline to SO 1 – 3, the mean prescribed PB pills/day declined from 9.6 to 3.8 pills/day (p < 0.001), and the proportion of patients with serum phosphorus ≤ 5.5 mg/dL increased from 13.9 to 26.1% (+88%). Comparing baseline to SO 4 – 6 (n = 424), the mean prescribed PB pills/day declined from 9.7 to 4.0 pills/day (p < 0.001), and the proportion of patients with serum phosphorus ≤ 5.5 mg/dL increased from 15.6 to 30.4% (+95%). Conclusions: Prescription of SO was associated with an increase in the proportion of patients achieving serum phosphorus levels ≤ 5.5 mg/dL along with fewer prescribed PB pills/day.

Dialysis dose is an important determinant of clinical outcomes in patients with end stage renal disease on maintenance dialysis. In clinical practice dialysis dose is monitored at least monthly by urea clearance based on Urea Kinetic Modeling. Online clearance monitoring using effective ionic dialysance (EID) of sodium (Na 1 ) is available on some hemodialysis machines. This paper reviews the background, methodology, additional applications, and potential risks associated with EID. Effective ionic dialysance provides a reliable, real-time, noninvasive, and inexpensive measurement of dialysis dose during an ongoing hemodialysis (HD) session to allow interventions and assess the impact of these changes on clearance. Surveillance of vascular access flow rates can be used to screen for access dysfunction and refer for interventions. There is a concern that EID measurements may cause Na 1 loading because of high dialysate Na 1 used during these measurements, however, mathematical models, in vitro experiments, and clinical studies in patients on maintenance HD do not show any evidence of Na 1 loading during EID measurements. We cannot rule out the possibility of nonosmotic Na 1 accumulation in the skin because no published literature exists on this topic as it pertains to clearance measurements based on EID of Na 1 .