Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association
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Nephrol. Dial. Transplant. · Mar 2010
End-stage renal disease in patients with Fabry disease: natural history data from the Fabry Registry.
Fabry disease, an X-linked lysosomal storage disorder caused by deficiency of alpha-galactosidase activity, is associated with progressive loss of kidney function. This study was undertaken to characterize Fabry disease among patients who reached end-stage renal disease. ⋯ While all Fabry patients are at risk of cardiovascular events and strokes, patients with Fabry nephropathy who develop kidney failure appear to have concurrent involvement of other major organ systems. It is important that Fabry patients are diagnosed early and that their renal function is monitored carefully.
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Nephrol. Dial. Transplant. · Jan 2010
Comparative StudyComparison of methods for estimating glomerular filtration rate in critically ill patients with acute kidney injury.
In critically ill patients with acute kidney injury, estimates of kidney function are used to modify drug dosing, adjust nutritional therapy and provide dialytic support. However, estimating glomerular filtration rate is challenging due to fluctuations in kidney function, creatinine production and fluid balance. We hypothesized that commonly used glomerular filtration rate prediction equations overestimate kidney function in patients with acute kidney injury and that improved estimates could be obtained by methods incorporating changes in creatinine generation and fluid balance. ⋯ In patients with acute kidney injury, glomerular filtration rate estimating equations can be improved by incorporating data on creatinine generation and fluid balance. A better assessment of glomerular filtration rate in acute kidney injury could improve evaluation and management and guide interventions.
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Nephrol. Dial. Transplant. · Jan 2010
Effect of the cGMP pathway on AQP2 expression and translocation: potential implications for nephrogenic diabetes insipidus.
Arginine vasopressin (AVP) binding to the V2 receptor (V2R) in renal collecting duct principal cells induces a cAMP signalling cascade resulting in the activation of protein kinase A (PKA), translocation of aquaporin-2 (AQP2) to the apical membrane and an increase in AQP2 expression. Consequently, concentration of urine is initiated. X-linked nephrogenic diabetes insipidus (NDI), characterized by the inability to concentrate urine in response to AVP, is caused by mutations in the V2R gene. Initiation of AQP2 translocation, while circumventing the V2R-cAMP-PKA pathway has been suggested as a putative therapy for these patients. In this respect, the activation of a cAMP-independent and cGMP-dependent pathway for AQP2 membrane insertion by different cyclic guanosine monophosphate (cGMP) pathway activators, such as atrial natriuretic peptide (ANP), l-arginine and 8-bromoguanosine 3',5'-cyclic monophosphate (8-Br-cGMP), has been put forward. However, it is unclear whether they can increase AQP2 expression. ⋯ Our results suggest that while activators of the cGMP pathway are likely beneficial in the treatment of X-linked NDI, their ability to relieve NDI in the patients may be improved when combined with agents stimulating AQP2 expression.
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Nephrol. Dial. Transplant. · Jan 2010
Multicenter StudyPreferences for dialysis withdrawal and engagement in advance care planning within a diverse sample of dialysis patients.
Rates of dialysis withdrawal are higher among the elderly and lower among Blacks, yet it is unknown whether preferences for withdrawal and engagement in advance care planning also vary by age and race or ethnicity. DESIGN, SETTING, PARTICIPANTS AND METHODS: We recruited 61 participants from two dialysis clinics to complete questionnaires regarding dialysis withdrawal preferences in five different health states. Engagement in advance care planning (end-of-life discussions), completion of advance directives and 'do not resuscitate' or 'do not intubate' (DNR/DNI) orders were ascertained by a questionnaire and from dialysis unit records. ⋯ Preferences for withdrawal vary by race/ ethnicity, whereas the pattern of engagement in advance care planning varies by age and race/ethnicity. Knowledge of these differences may be useful for improving communication about end-of-life preferences and in implementing effective advance care planning strategies among diverse haemodialysis patients.
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Nephrol. Dial. Transplant. · Jan 2010
Comparative StudyOutcome definitions in non-dialysis intervention and prevention trials in acute kidney injury (AKI).
The risk, injury, failure, loss-of-function, end-stage-renal-failure (RIFLE) and acute kidney injury network (AKIN) consensus definitions of acute kidney injury (AKI) were established in part to facilitate comparison of trials. Contrast-induced nephropathy (CIN) has traditionally used a less demanding definition. ⋯ Considerable heterogeneity remains in outcome variables of AKI and CIN clinical trials. Even when the RIFLE or AKIN criteria were used, they were not applied consistently. There is a need for further consensus on surrogate outcome variables.