Giornale italiano di nefrologia : organo ufficiale della Società italiana di nefrologia
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Renal involvement in Fabry's disease in males starts at an early age with microalbuminuria and proteinuria and progresses rapidly towards end-stage renal disease requiring dialysis or renal transplantation. Renal involvement, together with cardiac and cerebral damage, is responsible for the severe morbidity and mortality in patients with Fabry's disease. ⋯ Considering the relevance of renal damage in the prognosis of Fabry's disease, it is mandatory to point out the diagnostic criteria of Fabry's nephropathy and the modalities of follow-up of patients with renal involvement. The aim of this study is also to provide recommendations regarding the diagnosis, follow-up and indication for enzyme replacement therapy in patients with Fabry's disease.
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Pruritus is a common and unpleasant symptom in the dialysis setting, affecting about half of all hemodialysis and peritoneal dialysis patients. It has a great impact on patients' quality of life and is also associated with increased mortality. The pathogenesis of uremic pruritus (UP) is clearly multifactorial and still poorly understood. ⋯ The second step may be local therapy with skin emollients and capsaicin creams. More specific treatments that appear promising but have not been proven to be definitively efficacious include UVB light, gabapentin and the novel k-opioid-agonist nalfurafine. Nephrologists, who still tend to neglect this disabling symptom, need to be aware that UP is associated with poorer patient outcomes and that a stepwise therapeutic approach is now available.
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It is well known that the presence of alloantibodies against human HLA class I (A, B, C) and class II (DR, DQ) antigens in transplant recipients waiting for a first or subsequent kidney transplant has a significant negative impact on graft outcome, with increased acute and chronic rejection rates. HLA antibodies, present in hyperimmunized patients (PRA > 80%) as a result of pregnancies, blood transfusions and previous failed grafts, once thought to be a formidable barrier to renal transplantation, can now be overcome with excellent results by means of desensitization protocols in kidney transplant recipients from living or cadaver donors. Such pretransplant desensitization protocols consist of high-dose intravenous immunoglobulin infusions (IVIg-HD), plasmapheresis associated with low-dose IVIg (IVIg-LD) and immunoabsorption by protein-A sepharose or Ig-sepharose columns. ⋯ Similar desensitization protocols have been used for non-A2 AB0-incompatible living donor kidney transplants. These techniques have allowed successful transplantation in this high-risk patient category by providing live donor kidneys that function promptly with minimal risk of early loss, and have consequently increased the organ donor pool. Long-term follow- up of these patients and the application on a wider scale of these techniques, which for many patients may represent the only realistic chance of a successful transplant, will provide the definitive answers about their real efficacy.
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In the last 10-15 years, user-friendly continuous renal replacement therapy (CRRT) machines have played a major role in increasing the popularity of these techniques in intensive care settings. At present it is not clear which modality of renal replacement therapy (RRT) is optimal for critically ill patients with acute kidney injury (AKI). The choice between different modalities should therefore not be based on unproven ''outcome'' advantages but on evaluation of the clinical picture and logistical circumstances. ⋯ Data from several studies comparing the costs of different RRT modalities showed that CRRT is more expensive than IHD or SLED. However, the costs related to SLED can fluctuate within a wide range and in particular settings the higher costs of CRRT could be partially justified by logistical advantages. Further improvements in CRRT device characteristics, anticoagulation protocols, and adaptation of dialysis/replacement fluids to clinical needs will possibly contribute to maintaining, in the coming years, the key role of CRRT in the treatment of hemodynamically unstable critically ill patients requiring RRT.
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Comparative Study
[Continuous vs intermittent renal replacement therapies in acute renal failure: toward an agreement?].
Many studies have addressed the question of renal replacement therapy (RRT) modalities in patients with acute kidney injury (AKI) in the intensive care setting. There is no definite evidence of the superiority of one RRT modality over another. ⋯ The ideal RRT modality for patients with AKI in the ICU probably does not exist, and a more rational approach should be based on the judicious utilization of all the modalities currently available in the ICU, tailoring RRT on the basis of the changing needs of the patients along their clinical course. An important improvement in the approach to RRT in the critical care setting could be the so-called hybrid or prolonged intermittent RRT techniques (e.g., sustained low-efficiency dialysis or SLED), that seem to share most of the advantages of both classical (i.e., short-duration) intermittent and continuous modalities, without their shortcomings.