Infants with congenital nephrotic syndrome have comparable outcomes to infants with other renal diseases

BackgroundChildren with congenital nephrotic syndrome (CNS) commonly develop end stage renal failure in infancy and require dialysis, but little is known about the complications and outcomes of dialysis in these children.MethodsWe conducted a retrospective case note review across members of the European Society for Pediatric Nephrology Dialysis Working Group to evaluate dialysis management, complications of dialysis, and outcomes in children with CNS.ResultsEighty children (50% male) with CNS were identified form 17 centers over a 6-year period. Chronic dialysis was started in 44 (55%) children at a median age of 8 (interquartile range 4-14) months. Of these, 17 (39%) were on dialysis by the age of 6months, 30 (68%) by 1year, and 40 (91%) by 2years. Peritoneal dialysis (PD) was the modality of choice in 93%, but 34% switched to hemodialysis (HD), largely due to catheter malfunction (n=5) or peritonitis (n=4). The peritonitis rate was 0.77 per patient-year. Weight and height SDS remained static after 6months on dialysis. In the overall cohort, at final follow-up, 29 children were transplanted, 18 were still on dialysis (15 PD, 3 HD), 19 were in pre-dialysis chronic kidney disease (CKD), and there were 14 deaths (8 on dialysis). Median time on chronic dialysis until transplantation was 9 (6-18) months, and the median age at transplantation was 22 (14-28) months.ConclusionsInfants with CNS on dialysis have a comparable mortality, peritonitis rate, growth, and time to transplantation as infants with other primary renal diseases reported in international registry data.Peer reviewe

Lipid levels are inversely associated with infectious and all-cause mortality: international MONDO study results.

Cardiovascular (CV) events are increased 36-fold in patients with end-stage renal disease. However, randomized controlled trials to lower LDL cholesterol (LDL-C) and serum total cholesterol (TC) have not shown significant mortality improvements. An inverse association of TC and LDL-C with all-cause and CV mortality has been observed in patients on chronic dialysis. Lipoproteins also may protect against infectious diseases. We used data from 37,250 patients in the international Monitoring Dialysis Outcomes (MONDO) database to evaluate the association between lipids and infection-related or CV mortality. The study began on the first day of lipid measurement and continued for up to 4 years. We applied Cox proportional models with time-varying covariates to study associations of LDL-C, HDL cholesterol (HDL-C), and triglycerides (TGs) with all-cause, CV, infectious, and other causes of death. Overall, 6,147 patients died (19.2% from CV, 13.2% from infection, and 67.6% from other causes). After multivariable adjustment, higher LDL-C, HDL-C, and TGs were independently associated with lower all-cause death risk. Neither LDL-C nor TGs were associated with CV death, and HDL-C was associated with lower CV risk. Higher LDL-C and HDL-C were associated with a lower risk of death from infection or other non-CV causes. LDL-C was associated with reduced all-cause and infectious, but not CV mortality, which resulted in the inverse association with all-cause mortality

Renal replacement therapy for acute renal failure in children: European Guidelines

Acute renal failure (ARF) is uncommon in childhood and there is little consensus on the appropriate treatment modality when renal replacement therapy is required. Members of the European Pediatric Peritoneal Dialysis Working Group have produced the following guidelines in collaboration with nursing staff. Good practice requires early discussion of patients with ARF with pediatric nephrology staff and transfer for investigation and management in those with rapidly deteriorating renal function. Patients with ARF as part of multi-organ failure will be cared for in pediatric intensive care units where there should be access to pediatric nephrology support and advice. The choice of dialysis therapy will therefore depend upon the clinical circumstances, location of the patient, and expertise available. Peritoneal dialysis has generally been the preferred therapy for isolated failure of the kidney and is universally available. Intermittent hemodialysis is frequently used in renal units where nursing expertise is available and hemofiltration is increasingly employed in the intensive care situation. Practical guidelines for and the complications of each therapy are discussed

Effect of atorvastatin on inflammation and outcome in patients with type 2 diabetes mellitus on hemodialysis

Statins have multiple effects, including anti-inflammatory actions, lowering C-reactive protein levels, and reducing coronary events. We performed a post hoc analysis of the randomized placebo-controlled 4D Study that had evaluated the efficacy and safety of atorvastatin in 1255 patients with type 2 diabetes mellitus who were on maintenance hemodialysis. Here we determined the relationship between atorvastatin treatment, C-reactive protein, and the outcome of patients who had pre-specified and adjudicated endpoints of all-cause mortality, composite vascular endpoint, myocardial infarction, sudden death, and stroke. Atorvastatin had no significant effect on the risk of composite vascular endpoint or death relative to placebo in any quartile of baseline C-reactive protein. These baseline levels were not significantly different between the treated and placebo group and remained stable at 6 months on atorvastatin but significantly increased in those patients on placebo. All of the patients with baseline C-reactive protein in the fourth quartile had a significantly increased risk of deaths and in composite vascular endpoint compared to patients in the first quartile. The mean value of two consecutive C-reactive protein measurements was associated with significant increases in the risk of sudden death, stroke, all-cause mortality and composite vascular endpoint. Our results show that C-reactive protein was highly predictive of outcome, but atorvastatin treatment was not associated with reduced relative risks in the composite vascular endpoint or mortality in patients on hemodialysis with or without inflammation

Effect of dialysis membrane and patient's age on signs of dialysis-related amyloidosis

Effect of dialysis membrane and patient's age on signs of dialysis-related amyloidosis. This 12 center study was designed to assess factors affecting the development and progression of β2-microglobulin amyloidosis in long-term dialysis. A total of 221 patients who were on hemodialysis for more than five years, and who were treated the entire time only with AN69, a biocompatible, highly permeable membrane, or cuprophane, a less permeable, poorly biocompatible membrane (Cell) were evaluated for time on dialysis, development of carpal tunnel syndrome, and cystic bone lesions X-ray documentation was taken in a minimum of four of the six following joints: both hips, wrists and shoulders. The data demonstrate that patients treated solely by AN69 membranes display signs of bone amyloidosis less frequently than do those treated by Cell membranes. Age at onset of dialysis was found to have a striking correlation with the development of carpal tunnel syndrome and bone amyloidosis, while no significant influence was found for hyperparathyroidism, sex or year of first dialysis

Cycler adequacy and prescription data in a national cohort sample: The 1997 core indicators report

Cycler adequacy and prescription data in a national cohort sample: The 1997 core indicators report.BackgroundThe Health Care Financing Administration Peritoneal Dialysis Core Indicator Project obtains data yearly in four areas of patient care: dialysis adequacy, anemia, blood pressure, and nutrition.MethodsAdequacy and dialysis prescription data were obtained using a standardized data abstraction form from a random sample of adult U.S. peritoneal dialysis patients who were alive on December 31, 1996.ResultsFor the cohort receiving cycler dialysis, 22% were unable to meet the National Kidney Foundation Dialysis Outcome Quality Initiatives (NKF-DOQI) dialysis adequacy guidelines because they did not have at least one adequacy measure during the six-month period of observation. Thirty-six percent of patients met NKF-DOQI guidelines for weekly Kt/V urea, 33% met guidelines for weekly creatinine clearance (CCr), and 24% met guidelines for both urea and creatinine clearances. The mean weekly adequacy values were 2.24 ± 0.56 for Kt/V urea and 67.5 ± 24.4 liter/1.73m2 for CCr, and the median values were 2.20 and 62.25 liter/1.73m2, respectively. The mean prescribed 24-hour volume was 12,040 ± 3255 ml, and the median prescribed volume was 11,783ml. Only 60% of patients were prescribed at least one daytime dwell. By logistic regression analysis, risk factors for an inadequate dose of dialysis included being in the highest quartile of body surface area (odds ratio = 3.3 for CCr and 3.4 for Kt/V urea) and a duration of dialysis greater than two years (odds ratio = 4.2 for CCr and 2.1 for Kt/V urea).ConclusionThere is much room for improvement in providing an adequate dose of dialysis to cycler patients. Practitioners should be more aggressive in increasing dwell volumes, adding daytime dwells, and adjusting nighttime dwell times in order to compensate for the loss of residual renal function over time. These changes can only be accomplished if practitioners measure periodically the dose of dialysis as outlined in the NKF-DOQI guidelines

Depression as a predictor of mortality and hospitalization among hemodialysis patients in the United States and Europe

Depression as a predictor of mortality and hospitalization among hemodialysis patients in the United States and Europe.BackgroundDepression is not uncommon among patients with end-stage renal disease (ESRD) being treated by hemodialysis. We investigated whether risk of mortality and rate of hospitalization may be predicted from physician-diagnosed depression and patients' self-reports of depressive symptoms.MethodsData were analyzed from the Dialysis Outcomes and Practice Patterns Study (DOPPS) for randomly selected ESRD patients being treated by hemodialysis in the United States (142 facilities, 2855 patients) and five European countries (101 facilities, 2401 patients). The diagnosis of depression during the past year was abstracted from the medical records. In addition, the patients were asked to indicate how much of their time over the previous four weeks they had felt (1) “so down in the dumps that nothing could cheer you up” and (2) “downhearted and blue.” A response of “a good bit,”“most,” or “all” of the time were classified as depressed.ResultsThe prevalence of depression was nearly 20%. The relative risks of mortality and hospitalization among depressed (vs. non-depressed), adjusted for time on dialysis, age, race, socioeconomic status, comorbid indicators and country were, respectively: 1.23 and 1.11 for physician-diagnosed depression, 1.48 and 1.15 for the “so down in the dumps” question, and 1.35 and 1.11 for the “downhearted and blue” question (P < 0.05 for all six relative risks). These associations were not significantly different between US and European patients.ConclusionsSelf-reported depression by two simple questions was associated with increased risks of mortality and hospitalization for hemodialysis patients. Future research needs to assess whether early identification and treatment of depression may help to improve quality of life and survival in hemodialysis patients

Semiparametric Analysis of Correlated Recurrent and Terminal Events

In clinical and observational studies, recurrent event data (e.g., hospitalization) with a terminal event (e.g., death) are often encountered. In many instances, the terminal event is strongly correlated with the recurrent event process. In this article, we propose a semiparametric method to jointly model the recurrent and terminal event processes. The dependence is modeled by a shared gamma frailty that is included in both the recurrent event rate and terminal event hazard function. Marginal models are used to estimate the regression effects on the terminal and recurrent event processes, and a Poisson model is used to estimate the dispersion of the frailty variable. A sandwich estimator is used to achieve additional robustness. An analysis of hospitalization data for patients in the peritoneal dialysis study is presented to illustrate the proposed method.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/66008/1/j.1541-0420.2006.00677.x.pd

Serum Potassium and Mortality Risk in Hemodialysis Patients: A Cohort Study.

RATIONALE & OBJECTIVE: Both hypo- and hyperkalemia can cause fatal cardiac arrhythmias. Although predialysis serum potassium level is a known modifiable risk factor for death in patients receiving hemodialysis, especially for hypokalemia, this risk may be underestimated. Therefore, we investigated the relationship between predialysis serum potassium level and death in incident hemodialysis patients and whether there is an optimum level. STUDY DESIGN: Prospective multicenter cohort study. SETTING & PARTICIPANTS: 1,117 incident hemodialysis patients (aged >18 years) from the Netherlands Cooperative Study on the Adequacy of Dialysis-2 study were included and followed from their first hemodialysis treatment until death, transplantation, switch to peritoneal dialysis, or a maximum of 10 years. EXPOSURE: Predialysis serum potassium levels were obtained every 6 months and divided into 6 categories: ≤4.0 mmol/L, >4.0 mmol/L to ≤4.5 mmol/L, >4.5 mmol/L to ≤5.0 mmol/L, >5.0 mmol/L to ≤5.5 mmol/L (reference), >5.5 mmol/L to ≤6.0 mmol/L, and >6.0 mmol/L. OUTCOMES: 6-month all-cause mortality. ANALYTICAL APPROACH: Cox proportional hazards and restricted cubic spline analyses with time-dependent predialysis serum potassium levels were used to calculate the adjusted HRs for death. RESULTS: At baseline, the mean age of the patients was 63 years (standard deviation, 14 years), 58% were men, 26% smoked, 24% had diabetes, 32% had cardiovascular disease, the mean serum potassium level was 5.0 mmol/L (standard deviation, 0.8 mmol/L), 7% had a low subjective global assessment score, and the median residual kidney function was 3.5 mL/min/1.73 m2 (IQR, 1.4-4.8 mL/min/1.73 m2). During the 10-year follow-up, 555 (50%) deaths were observed. Multivariable adjusted HRs for death according to the 6 potassium categories were as follows: 1.42 (95% CI, 1.01-1.99), 1.09 (95% CI, 0.82-1.45), 1.21 (95% CI, 0.94-1.56), 1 (reference), 0.95 (95% CI, 0.71-1.28), and 1.32 (95% CI, 0.97-1.81). LIMITATIONS: Shorter intervals between potassium measurements would have allowed for more precise mortality risk estimations. CONCLUSIONS: We found a U-shaped relationship between serum potassium level and death in incident hemodialysis patients. A low predialysis serum potassium level was associated with a 1.4-fold stronger risk of death than the optimal level of approximately 5.1 mmol/L. These results may imply the cautious use of potassium-lowering therapy and a potassium-restricted diet in patients receiving hemodialysis