Background and objectives: Calciphylaxis, or calcific uremic arteriolopathy, is a well-described entity in end-stage kidney disease and renal transplant patients; however, little systematic information is available on calciphylaxis from nonuremic causes. This systematic review was designed to characterize etiologies, clinical features, laboratory abnormalities, and prognosis of nonuremic calciphylaxis.Design, setting, participants, & measurements: A systematic review of literature for case reports and case series of nonuremic calciphylaxis was performed. Cases included met the operational definition of nonuremic calciphylaxis-histopathologic diagnosis of calciphylaxis in the absence of end-stage kidney disease, renal transplantation, or acute kidney injury requiring renal replacement therapy.Results: We found 36 cases (75% women, 63% Caucasian, aged 15 to 82 yr) of nonuremic calciphylaxis. Primary hyperparathyroidism, malignancy, alcoholic liver disease, and connective tissue disease were the most common reported causes. Preceding corticosteroid use was reported for 61% patients. Protein C and S deficiencies were seen in 11% of patients. Skin lesions were morphologically similar to calcific uremic arteriolopathy. Mortality rate was 52%, with sepsis being the leading cause of death.Conclusion: Calciphylaxis should be considered while evaluating skin lesions in patients with predisposing conditions even in the absence of end-stage kidney disease and renal transplantation. Nonuremic calciphylaxis is reported most often in white women. Mineral abnormalities that are invoked as potential causes in calcific uremic arteriolopathy are often absent, suggesting that heterogeneous mechanisms may contribute to its pathogenesis. Nonuremic calciphylaxis is associated with high mortality, and there is no known effective treatment.
Background: Most healthy people exhibit a decrease in systolic blood pressure (SBP) at night. A drop of less than 10% from mean daytime values (nondipping) is associated with chronic kidney disease, insulin resistance, and cardiovascular events. Whether nondipping precedes a decline in renal function remains unclear. We hypothesized that nondipping would predict a decline in the glomerular filtration rate (GFR) over time.Methods: Consecutive patients referred for ambulatory blood pressure monitoring were included in our retrospective cohort if they had a serum creatinine level noted at the time of their ambulatory blood pressure recording and a follow-up creatinine level recorded at least 1 year later. Mean day and night SBPs were compared (daytime SBP-nighttime SBP ratio). We defined nondipping as a daytime SBP-nighttime SBP ratio higher than 0.90. The GFR was calculated using the Modification of Diet in Renal Disease 4-variable equation.Results: Of 322 patients included, 137 were dippers and 185 were nondippers; their mean baseline GFRs were 80.5 mL/min per 1.73 m 2 and 76.4 mL/min per 1.73 m 2 , respectively. During a median follow-up of 3.2 years, the GFRs remained stable among dippers (mean change, 1.3%) but declined among nondippers (mean change, −15.9%) (PϽ.001). The creatinine levels increased by more than 50% in 2 dippers (1.5%) and in 32 nondippers (17.3%) (PϽ.001). These findings persisted after adjustment for other predictors of GFR decline. Conclusion:Blunted diurnal blood pressure variation is associated with a subsequent deterioration in renal function that is independent of SBP load and other risk factors for renal impairment.
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