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Calciphylaxis.

ABSTRACT: Calciphylaxis remains a poorly understood vascular calcification disorder with predilection for patients with end-stage renal disease (ESRD). Recent data from large patient registries and databases have begun to provide information regarding incidence, risk factors, and outcomes in patients with calciphylaxis.The most recent estimate places the incidence of calciphylaxis at 3.5 new cases/1000 patient-years among the patients with ESRD on chronic hemodialysis. It is possible that misdiagnosis or subclinical disease may attribute to lower than the true incidence. There is a suggestion that the incidence is higher in peritoneal dialysis patients compared with patients with hemodialysis. Recent studies have identified a number of risk factors and point to the effects of vitamin K deficiency mediated impairment in Matrix Gla Protein carboxylation as one of the likely pathogenic mechanisms. The outcomes in calciphylaxis patients remain poor with mortality approaching 30% at 6 months and 50% at 12 months.The present review describes recent literature in the field of calciphylaxis. Calciphylaxis registries and specimen biorepositories promise to provide insights into the pathogenesis of calciphylaxis and will pave the way for much needed clinical trials.

SUBMITTER: Nigwekar SU

PROVIDER: S-EPMC5907931 | biostudies-literature | 2017 Jul

REPOSITORIES: biostudies-literature

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Calciphylaxis.

Nigwekar Sagar U SU

Current opinion in nephrology and hypertension 20170701 4

<h4>Purpose of review</h4>Calciphylaxis remains a poorly understood vascular calcification disorder with predilection for patients with end-stage renal disease (ESRD). Recent data from large patient registries and databases have begun to provide information regarding incidence, risk factors, and outcomes in patients with calciphylaxis.<h4>Recent findings</h4>The most recent estimate places the incidence of calciphylaxis at 3.5 new cases/1000 patient-years among the patients with ESRD on chronic ...[more]

PMID: 28375870

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Project description:Importance:Classic calciphylaxis associated with renal failure is a life-threatening disease. Warfarin-associated calciphylaxis without renal injury has been described, but whether it is a subset of classic calciphylaxis or a different entity remains unknown. We describe 1 case of warfarin-associated calciphylaxis, present data from 2 others from our institution, and review all cases of warfarin-associated calciphylaxis available in the literature. Our review indicates that warfarin-associated calciphylaxis is clinically and pathophysiologically distinct from classic calciphylaxis. Objective:To review warfarin-associated calciphylaxis and determine its relationship to classic calciphylaxis. Design, Setting, and Participants:We searched MEDLINE and Ovid without language or date restrictions for case reports of calciphylaxis from the inpatient setting using the terms "calciphylaxis and warfarin," "non-uremic calciphylaxis," and "nonuremic calciphylaxis." We defined nonuremic calciphylaxis as a histopathologic diagnosis of calciphylaxis without severe kidney disease (serum creatinine level >3 mg/dL; glomerular filtration rate <15 mL/min; acute kidney injury requiring dialysis; and renal transplantation). Exposures:Each patient had been exposed to warfarin before the onset of calciphylaxis. Main Outcomes and Measures:Patient data were abstracted from published reports. Original patient medical records were requested and reviewed when possible. Results:We identified 18 patients with nonuremic calciphylaxis, 15 from the literature, and 3 from our institution. Patients were predominantly female (15 of 18 [83%]) with ages ranging from 19 to 86 years. Duration of warfarin therapy prior to calciphylaxis onset averaged 32 months. Lesions were usually located below the knees (in 12 of 18 [67%]). No cases reported elevated calcium-phosphate products (0 of 17 [0%]). Calcifications were most often noted in the tunica media (n?=?8 [44%]) or in the vessel lumen and tunica intima (n?=?7 [39%]). The most common treatments included substitution of heparin or low-molecular weight heparin for warfarin (n?=?13 [72%]), intravenous sodium thiosulfate (n?=?9 [50%]), and hyperbaric oxygen (n?=?3 [17%]). The survival rate on hospital discharge was remarkably high, with 15 cases (83%) reporting full recovery and 3 cases ending in death. Conclusions and Relevance:Warfarin-associated calciphylaxis is distinct from classic calciphylaxis in pathogenesis, course, and, particularly, outcome. This finding should influence clinical management of the disease and informs targeted treatment of the disease.

Project description:Background and aimsCalciphylaxis is a rare condition associated with very high mortality in patients with end-stage kidney disease. Data from country-based registries have been an invaluable resource for a better understanding of the natural history and management for this condition. This study aimed to investigate the current management strategies and outcomes of patients enrolled in the United Kingdom Calciphylaxis study (UKCS).MethodsThe study was conducted on 89 patients registered in the UKCS since 2012. The initial analysis included a description of the baseline characteristics, management strategies and outcomes on follow-up until May 2020. Further analysis included a comparison of the mortality outcome of the UKCS patients who were receiving haemodialysis with a propensity score matched cohort of haemodialysis patients from the Chronic Renal Insufficiency Standards Implementation Study- Haemodialysis (CRISIS-HD).ResultsMedian age of the cohort was 59 years, with a predominance of females (61%) and Caucasian (95%) ethnicity. About 54% of the patients were diabetic and 70% were receiving haemodialysis at study entry. The skin lesions were mostly distributed in the lower extremities (48%). Sodium thiosulphate and calcimimetic were the most widely used management strategies. The mortality rate was 72 deaths per hundred patient-years (50 deaths observed in 69.5 patient years). Complete wound healing was noted in 17% and bacteraemia was reported in 26% of patients. In a comparative analysis of the matched haemodialysis patients, the presence of calciphylaxis in 62 patients showed a strong association with all-cause mortality (HR 6.96; p < 0.001), with annual mortality 67% versus 10.2% in haemodialysis patients without calciphylaxis.ConclusionsThis UK wide study strengthens the evidence that calciphylaxis is a strong and independent risk factor associated with all-cause mortality; no significant benefit was shown with any individual treatment modality. Until further evidence becomes available, a multifaceted approach would be the appropriate treatment strategy in the management of this extremely serious condition.

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Calciphylaxis.

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Calciphylaxis.

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