FSGS permeability factor-associated nephrotic syndrome: remission after oral galactose therapy.

Some cases of nephrotic syndrome in focal and segmental glomerulosclerosis (FSGS) are associated with a circulating factor, the FSGS permeability factor (FSPF). Galactose has a high affinity for FSPF, and experimental data suggest that it could reduce its activity. We describe the case of a 48-year-old male with a nephrotic syndrome found to be resistant to corticosteroids, immunosuppression and plasmaphaeresis. The patient was given oral galactose as a last resort treatment, which was followed by a remission of his nephrotic syndrome that correlated with a reduction of FSPF activity. This case is the first report of a long-standing remission of an FSPF-associated nephrotic syndrome on oral galactose therapy.

Measurement of the international normalized ratio (INR) in hemodialysis patients with heparin-locked central venous catheters: evaluation of a novel blood sampling method.

BACKGROUND: Accurate measurement of the international normalized ratio (INR) may be difficult in hemodialysis (HD) patients with heparin-locked central catheters. Blood contamination with locking solutions may interfere with INR measurement when samples are collected directly from the catheter. METHODS: The aim of our study was to evaluate a novel sampling method for the measurement of INR in HD patients with heparin-locked central catheters. This novel method consists of measuring the INR directly from the dialysis circuit (arterial bloodline sample port) after 1 hr of treatment regardless of heparin administration during dialysis. We compared this method to the gold standard (peripheral venipuncture prior to dialysis) using the paired t-test. We included 30 patients (23 with warfarin therapy and 7 without). RESULTS: INRs obtained using the novel sampling method were only minimally overestimated compared to venipuncture values (mean INR overestimation: 0.2 +/- 0.2). This overestimation was not clinically significant and did not lead to changes in warfarin prescription for any of the patients. Correlation tests confirmed the influence of heparin administration on INR overestimation (R=0.4; p=0.05). This influence was present mostly among patients receiving more than 100 Units/kg of heparin during their treatment. CONCLUSION: This novel sampling technique provides a convenient and simple method of monitoring INR among HD patients.

A practice-related risk score (PRS): a DOPPS-derived aggregate quality index for haemodialysis facilities.

BACKGROUND: The Dialysis Outcomes and Practice Patterns Study (DOPPS) database was used to develop and validate a practice-related risk score (PRS) based on modifiable practices to help facilities assess potential areas for improving patient care. METHODS: Relative risks (RRs) from a multivariable Cox mortality model, based on observational haemodialysis (HD) patient data from DOPPS I (1996-2001, seven countries), were used. The four practices were the percent of patients with Kt/V > or =1.2, haemoglobin > or =11 g/dl (110 g/l), albumin > or =4.0 g/dl (40g/l) and catheter use, and were significantly related to mortality when modelled together. DOPPS II data (2002-2004, 12 countries) were used to evaluate the relationship between PRS and mortality risk using Cox regression. RESULTS: For facilities in DOPPS I and II, changes in PRS over time were significantly correlated with changes in the standardized mortality ratio (SMR). The PRS ranged from 1.0 to 2.1. Overall, the adjusted RR of death was 1.05 per 0.1 points higher PRS (P < 0.0001). For facilities in both DOPPS I and II (N = 119), a 0.2 decrease in PRS was associated with a 0.19 decrease in SMR (P = 0.005). On average, facilities that improved PRS practices showed significantly reduced mortality over the same time frame. CONCLUSIONS: The PRS assesses modifiable HD practices that are linked to improved patient survival. Further refinements might lead to improvements in the PRS and will address regional variations in the PRS/mortality relationship.

[Nephrogenic systemic fibrosis: more hard times for renal failure patients]. / La fibrose systémique néphrogénique: nouveau coup dur pour les insuffisants rénaux.

To date, more than 200 cases of nephrogenic systemic fibrosis have been documented worldwide. All patients have had renal failure, most of them requiring dialysis. We herein describe the course of a hemodialyzed patient who developed nephrogenic systemic fibrosis in the months following magnetic resonance angiography of the lower extremities. The disease is characterized by skin thickening and tendon fibrosis leading to joint contractures that can quickly confine the patient to a wheelchair. Systemic involvement may occur, leading to cardiomyopathy, pulmonary fibrosis, pulmonary hypertension or even death. No consistently effective therapy has been reported. An association between gadolinium exposure and the development of the disease has been found, although no causal link has yet been proven. In a patient with renal failure, magnetic resonance imaging with gadolinium enhancement should be done only after having seriously considered the risk/benefit ratio. Implications concerning the choice of imaging methods when searching for ischemic nephropathy or aorto-iliac disease before renal transplantation are discussed.

Extracorporeal treatments in a dapsone overdose: a case report.

INTRODUCTION: Intentional dapsone intoxication can be life-threatening. There is limited data on the clinical effect of extracorporeal treatments (ECTRs) on dapsone elimination. We describe a case of severe dapsone toxicity treated with different ECTRs. CASE DETAILS: A 23-year-old woman was admitted 2.5 h after ingesting 2.2 g of dapsone. She developed methemoglobinemia (39.9%) and showed signs of toxicity (hemodynamic instability and altered mental status) despite multiple-activated charcoal, methylene blue, vasopressors and endotracheal intubation. Continuous venovenous hemofiltration (CVVH) was then initiated for 5 h, followed by intermittent hemodialysis with hemoperfusion (IHD-HP) for 4 h, and CVVH for another 48 h. The platelet count decreased to 32 × 109/L 3 h after IHD-HP. The elimination half-life of dapsone was 2.0 h during IHD-HP, and 14.2 h during CVVH. Mean dapsone clearance with IHD was 62 mL/min versus 22 mL/min with CVVH. IHD removed 95.3 mg, and CVVH removed 67.8 mg over 3.8 h. No rebound occurred following ECTR cessation. The toxicokinetics of dapsone metabolites were also accelerated during ECTR. The patient was extubated after 3.5 days and discharged without sequelae after 7 days. DISCUSSION: Dapsone clearance was enhanced by ECTR, especially by IHD-HP. However, HP was associated with severe asymptomatic thrombocytopenia.

Biofeedback regulation of ultrafiltration and dialysate conductivity for the prevention of hypotension during hemodialysis.

Intradialytic hypotension remains a frequent complication of dialysis, occurring in up to 33% of patients. We tested a fully integrated biofeedback system (the Hemocontrol system) that monitors and regulates blood volume contraction during hemodialysis. Seven hypotension prone patients were selected for the study. We conducted a prospective crossover study alternating dialysis sessions using the blood volume regulation system and standard dialysis sessions. Event free sessions were defined as dialysis sessions not requiring any therapeutic intervention for hypotension related signs or symptoms. There was a significant improvement in the number of event free sessions with blood volume regulation compared with standard dialysis (50.8% of sessions vs. 29.2%; p < 0.01). Percentages of event free sessions and mean postdialysis systolic blood pressure improved progressively over the course of the study, indicating improved hemodynamic stability over the study period. Therefore, the use of a biofeedback system to monitor and regulate blood volume during dialysis was helpful in restoring cardiovascular stability in a population of hypotension prone hemodialysis patients. Further studies are needed to confirm these preliminary results and to establish the role of blood volume regulation systems in reducing the incidence of hypotension during hemodialysis.

Lymphopenia and treatment-related infectious complications in ANCA-associated vasculitis.

BACKGROUND AND OBJECTIVES: ANCA-associated vasculitis (AAV) is treated with potent immunosuppressive regimens. This study sought to determine risk factors associated with infections during first-intention therapy. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: This retrospective study involved two separate cohorts of consecutive cases of AAV seen from 2004 to 2011 at two university hospitals. The following were assessed: vasculitis severity; therapy; and periods with no, moderate (lymphocyte count, 0.3-1.0× 10(9)/L), or severe (lymphocyte count ≤ 0.3×10(9)/L) lymphopenia and neutropenia (neutrophil count ≤ 1.5×10(9)/L). RESULTS: One hundred patients had a mean age of 57±15 years and a Birmingham vasculitis activity score of 7.7±3.6. Therapy consisted of pulse methylprednisolone (59%), cyclophosphamide (85%), methotrexate (6%), and plasmapheresis (25%) in addition to oral corticosteroids. During follow-up, 53% of patients experienced infection and 28% were hospitalized for infection (severe infection). Only 18% experienced neutropenia, but 72% and 36% presented moderate and severe lymphopenia for a total duration of <0.1%, 73%, and 8% of the treatment follow-up, respectively. Lower initial estimated GFR, longer duration of corticosteroid use, and presence of lymphopenia were risk factors of infections. The rate was 2.23 events/person-year in the presence of severe lymphopenia compared with 0.41 and 0.19 during periods with moderate or no lymphopenia (P<0.001). Similarly, the rate of severe infections was 1.00 event/person-year with severe lymphopenia and 0.08 and 0.10 with moderate and no lymphopenia (P<0.001). This association remained independent of other risk factors. CONCLUSIONS: Lymphopenia is frequent during the treatment of AAV, and its severity is associated with the risk of infectious complications.

Impact of hemocontrol on hypertension, nursing interventions, and quality of life: a randomized, controlled trial.

BACKGROUND: Volume overload contributes to the pathogenesis of hypertension in hemodialysis (HD) patients. DESIGN, SETTING, PARTICIPANTS, AND MEASUREMENTS: The Hemocontrol (HC) system (Gambro), which automatically adjusts ultrafiltration rate and dialysate conductivity during dialysis, has been suggested to improve hemodynamic tolerance and thereby facilitate fluid removal. A 6-mo randomized, controlled trial was performed to test the hypothesis that the addition of the HC system to a systematic BP management protocol may lower home BP in comparison with standard HD as primary end point. Secondary end points were the number of nursing interventions during dialysis and health-related quality of life. RESULTS: Complete BP data were available for 36 of the 44 patients who completed the trial. There was a statistically significant overall decrease in systolic BP during the study period (P = 0.005). However, the difference between the HC group and the standard HD group was NS (HC: from 147.8 +/- 21.7 to 139.8 +/- 16.2 mmHg; standard HD: from 141.9 +/- 19.2 to 135.2 +/- 9.9 mmHg). The number of HD sessions that required nursing interventions decreased in the HC group, whereas it increased in the standard HD group (HC: 42.9% reduction; standard HD: 35.7% increase; P = 0.04). There was also a significant improvement in health-related quality of life in the HC group but not in the standard HD group. CONCLUSIONS: These results suggest that the addition of the HC system to a systematic BP management protocol provides no additional benefit with regard to BP reduction. However, the HC system may improve the patient tolerability to dialysis.