The most efficient therapeutic approach for immunoglobulin light chain amyloidosis (AL) is autologous stem cell transplantation (ASCT); however, the toxicity of ASCT limits its feasibility to a minority of patients. Patients ineligible for ASCT are usually treated with standard oral melphalan and prednisone, but the response rate to this regimen is unsatisfactory, and time to response is long. Highdose dexamethasone provides a rapid response time in patients with AL. We evaluated the combination of oral melphalan and high-dose dexamethasone (MDex) in 46 patients with AL ineligible for ASCT. Thirty-one (67%) achieved a hematologic response and 15 (33%) a complete remission. In 22 (48%) of the responsive patients functional improvement of the organs involved was observed. Five patients (11%) experienced severe adverse events, 3 required hospitalization, and no treatment-related deaths were observed. M-Dex represents a feasible and effective therapeutic option for patients with advanced AL who are ineligible for ASCT.
Iron deficiency and anaemia seem to play no part in HD-related pruritus, whereas lower serum transferrin and albumin levels and higher ferritin values are consistent with the possible role of inflammation in the development and severity of pruritus.
The peritoneal equilibration test (PET) with 3.86% glucose concentration (3.86%-PET) has been suggested to be more useful than the standard 2.27%-PET in peritoneal dialysis (PD), but no longitudinal data for 3.86%-PET are currently available. A total of 242 3.86%-PETs were performed in 95 incident PD patients, who underwent the first test during the first year of treatment and then once a year. The classical parameters of peritoneal transport, such as peritoneal ultrafiltration (UF), D/D(0), and D/P(Creat), were analyzed. In addition, the absolute dip of dialysate sodium concentration (DeltaD(Na)), as an expression of sodium sieving, was studied. D/D(0) was stable, and a progressive decrease in UF was observed after the second PET, whereas D/P(Creat) firstly increased and then stabilized. DeltaD(Na) was the only parameter showing a progressive decrease over time. On univariate analysis, D/D(0) and DeltaD(Na) were found to be significantly associated with the risk of developing UF failure (risk ratio (RR) 0.987 (0.973-0.999), P=0.04, and RR 0.768 (0.624-0.933), P=0.007, respectively), but on multivariate analysis only DeltaD(Na) showed an independent association with the risk of developing UF failure (RR 0.797 (0.649-0.965), P=0.020). UF, D/D(0), and D/P(Creat) changed only in those patients developing UF failure, reflecting increased membrane permeability, whereas DeltaD(Na) significantly decreased in all patients. The 3.86%-PET allows a more complete study of peritoneal membrane transport than the standard 2.27%-PET. DeltaD(Na) shows a constant and significant reduction over time and is the only factor independently predicting the risk of developing UF failure in PD patients.
In 32 noncirrhotic patients on peritoneal dialysis, mean serum β2-microglobulin (sβ2M) was 26.58 ± 12.32 mg/l (9.7-63.5). We found a significant correlation between sβ2M and serum creatinine (sCr; r = 0.760), blood urea nitrogen (BUN; r = 0.573), total creatinine and BUN clearance (r = 0.623 and 0.599, respectively), 24-hour Kt/V (r = 0.638), glomerular filtration rate (r = 0.623), 24-hour urine output (r = 0.669), serum total protein (r = 0.584) (p < 0.01 for all the above r values); β2M peritoneal clearance and mass transfer (r = 0.414 and 0.427, respectively; p < 0.05). Our data demonstrate and confirm the contribution of residual renal function in determining sβ2M levels and it is seemingly more important than β2M peritoneal clearance.
Ultrafiltration (UF) failure is one of the most important causes of long-term peritoneal dialysis (PD) failure in patients. Osmotic forces acting across small and ultra-small pores generate a UF with solutes through the small pore and free water transport (FWT) through the ultra-small pore. The ability of glucose to exert an osmotic pressure sufficient to cause UF is the so-called 'osmotic conductance to glucose' (OCG) of the peritoneal membrane. Our study proposes a simple method to determine both the OCG and FWT. In 50 patients on PD, a Double Mini-Peritoneal Equilibration Test (Double Mini-PET), consisting of two Mini-PET, was performed consecutively. A solution of 1.36% glucose was used for the first test, whereas a solution of 3.86% glucose was used for the second test. The sodium removal values and the differences in UF between the two tests were used to calculate FWT and the OCG. Patients with UF failure showed significant reductions not only in the OCG and the FWT but also of UF of small pores. The Double Mini-PET is simple, fast, and could become useful to evaluate patients on PD in everyday clinical practice.
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