Clinical journal of the American Society of Nephrology : CJASN
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Clin J Am Soc Nephrol · Feb 2010
ReviewDialysis and transplantation in Fabry disease: indications for enzyme replacement therapy.
ESRD is a major cause of morbidity and premature mortality in Fabry disease, particularly in classically affected males. The decline of renal function in Fabry nephropathy is adversely affected by male gender, advanced chronic kidney disease (CKD), and severe proteinuria. The diagnosis of Fabry nephropathy may be missed if not specifically addressed in progressive CKD and patients have been first identified in screening programs of dialysis patients. ⋯ Whether the excess mortality risk of Fabry patients on RRT can be prevented by ERT is unknown. Despite observational reports of symptomatic improvement, the available evidence supporting ERT for such patients is not compelling enough. To clarify this issue, studies are needed to test the effectiveness of agalsidases in preventing cardiac and cerebrovascular complications in Fabry patients with ESRD.
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Clin J Am Soc Nephrol · Feb 2010
Randomized Controlled TrialUltrasound-guided femoral dialysis access placement: a single-center randomized trial.
Insertion of dialysis catheters (DCs) is a prerequisite for successful initiation of hemodialysis. We attempted to determine if ultrasonography-guided (USG) insertion was superior and safer than the anatomical landmark-guided technique (ALT) for the femoral vein (FV). ⋯ USG significantly improves success rate, reduces number of attempts, and decreases the incidence of complications related to FV DC insertion.
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Clin J Am Soc Nephrol · Feb 2010
Neutrophil gelatinase-associated lipocalin and acute kidney injury after cardiac surgery: the effect of baseline renal function on diagnostic performance.
Neutrophil gelatinase-associated lipocalin (NGAL) is rapidly released by renal tubules after injury, potentially allowing early identification of acute kidney injury (AKI) after cardiac surgery. However, the diagnostic performance of NGAL has varied widely in clinical studies, and it remains unknown what factors modify the relationship between NGAL and AKI. We hypothesized the relationship between urinary NGAL and AKI would vary with baseline renal function, allowing a stratified analysis to improve diagnostic performance of this novel biomarker. ⋯ The relationship between urinary NGAL and AKI after cardiac surgery varies with baseline renal function, with optimal discriminatory performance in patients with normal preoperative function.
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Clin J Am Soc Nephrol · Feb 2010
Development, implementation, and results of the ASN in-training examination for fellows.
The American Society of Nephrology and the fellowship training program directors in conjunction with the National Board of Medical Examiners developed a comprehensive assessment of medical knowledge for nephrology fellows in-training. This in-training examination (ITE) consisted of 150 multiple-choice items covering 11 broad content areas in a blueprint similar to the American Board of Internal Medicine certifying examination for nephrology. ⋯ The first examination was given in April 2009 to 682 fellows and six training program directors. Examinees felt that the examination was well structured and relevant to their training experience Longitudinal performance on the examination will be helpful in allowing training programs to utilize results from content areas in identifying deficits in medical knowledge as well as assessing the results of any curriculum changes.
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Involvement of the kidneys in Fabry disease ("nephropathy") occurs in male and female individuals. The majority of patients with progressive nephropathy will have significant proteinuria and develop progressive loss of kidney function, leading to ESRD. All too often, treating physicians may ignore "normal" serum creatinine levels or "minimal" proteinuria and fail to assess properly the severity of kidney involvement and institute appropriate management. ⋯ Antiproteinuric therapy can be dosed to a prespecified urine protein target rather than a specific BP goal, with the proviso that successful therapy will usually lower the BP below the goal of 130/80 mmHg that is used for other forms of kidney disease. The overall goal for treating Fabry nephropathy is to reduce the rate of loss of GFR to -1 ml/min per 1.73 m(2)/yr, which is that seen in the normal adult population. A systematic approach is presented for reaching this goal in the individual patient.