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      Calcifilaxis

      case-report

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          Abstract

          La calcifilaxis es una vasculopatía caracterizada por isquemia y necrosis cutánea dolorosa debida a calcificación, fibroplastia de la íntima y trombosis de las arteriolas paniculares. Compromete más frecuentemente a pacientes con insuficiencia renal crónica terminal y tiene muy elevada mortalidad. La biopsia de las lesiones cutáneas se utiliza como método diagnóstico. No se han registrado hallazgos específicos de laboratorio. Las lesiones cutáneas generalmente comienzan en las extremidades a modo de moteado violáceo doloroso similar al livedo reticularis. La evolución natural es hacia úlceras y escaras. La primera línea de tratamiento consiste en el cuidado de las lesiones cutáneas y antibioticoterapia. El tiosulfato sódico se utiliza como tratamiento debido a su actividad como antioxidante y quelante. Se presentan dos casos clínicos.

          Translated abstract

          Calciphylaxis is vasculopathy characterized by ischemia and painful skin necrosis due to calcification and intimal fibroplasia of thrombosis of the panicular arterioles. It most frequently compromises patients with terminal chronic renal failure and has a high mortality rate. Biopsy of skin lesions is used as a diagnostic method. No specific laboratory findings have been recorded. Skin lesions usually begin in the extremities like a painful purplish mottling similar to "livedo reticularis". The natural evolution is to ulcers and bedsores. The first line of treatment involves the care of skin lesions and antibiotic therapy. Sodium thiosulfate is used as treatment due to its antioxidant activity and as a chelating. Two clinical cases are here reported.

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          Most cited references9

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          Calciphylaxis: diagnosis and clinical features.

          Calciphylaxis is a relatively rare disease, observed mainly in patients on dialysis, associated with high mortality rates, and characterized by painful skin ulceration. The pathogenesis of calciphylaxis is virtually unknown, although several risk factors, including warfarin therapy, hypoalbuminemia, and disturbances in calcium-phosphate metabolism, have been reported. The prevalence of calciphylaxis in Japan is likely to be less than 1:10,000 dialysis patients per year based on our nationwide survey in 2009. However, the results of the survey also showed that about 60 % of nephrologists in Japan are not familiar with the disease itself and it is highly likely that calciphylaxis is being overlooked. To facilitate recognition of calciphylaxis, we have proposed diagnostic criteria. At present, there is no specific therapy for calciphylaxis and general supportive measures, especially antibiotics for the accompanying infection and wound care, are important. Recently, sodium thiosulfate has been increasingly used to treat calciphylaxis and its efficacy should be evaluated by large clinical trials.
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            A case control study of proximal calciphylaxis.

            The purpose of this investigation was to describe the clinical presentation of nine patients with calciphylaxis involving the proximal lower extremities or trunk and to compare the clinical characteristics of these patients with those of 347 hemodialysis patients from the same geographic area. Patients were identified primarily through a computer search of pathology records, identifying patients with the term "calciphylaxis" in the biopsy report. All patients had pathologic specimens consistent with calciphylaxis. All the calciphylaxis patients were white and were markedly obese. While two patients had markedly elevated parathyroid hormone levels, most patients did not show severe derangements of calcium phosphate metabolism compared with other dialysis patients. A logistic regression model identified body mass index and low serum albumin 3 months before diagnosis as being highly associated with a diagnosis of calciphylaxis. Diabetes mellitus and parameters of calcium-phosphate metabolism were not significantly associated with proximal calciphylaxis. These findings suggest that white race, morbid obesity, and poor nutritional status are associated with proximal calciphylaxis in dialysis patients.
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              Determinants of survival in patients with calciphylaxis: a multivariate analysis.

              Our study aims to assess the factors affecting survival in patients with calciphylaxis. We identified 26 patients with biopsy-proven calciphylaxis treated between 1995 and 2007. Clinical and follow-up data were obtained from medical records. Cox proportional hazards models were used to assess the factors affecting survival. The study group consisted of 23 women and 3 men with a mean age of 56.4 +/- 12.9 years. All patients had multiple comorbidities/risk factors including coronary artery disease (58%), diabetes mellitus (58%), and peripheral vascular disease (23%). Mean laboratory values were: calcium, 9.0 mg/dL (range, 6.8-11.6); albumin, 2.8 mg/dL; phosphate, 4.5 mg/dL (range, 2.5-7.5); Ca *phosphate, 35.9; and parathyroid hormone, 320.9 pg/mL (range, 4.6-2,419). Parathyroidectomy was performed in 9 of 26 patients (35%). Of our patients, 19% underwent revascularization procedures and 58% underwent debridement. In multivariate analyses, factors associated with poor survival were female gender ( P = .01), increased weight ( P = .01), and need for vascular procedures ( P = .06). Improved survival was associated with operative debridement ( P = .01). Parathyroidectomy alone did not emerge as a determinant of patient survival, although there was a trend to improved survival when debridement and parathyroidectomy were combined ( P = .09). Rather than a single intervention such as parathyroidectomy, a multidisciplinary approach involving early diagnosis, aggressive medical management, operative debridement, and parathyroidectomy may improve survival in calciphylaxis.
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                Author and article information

                Contributors
                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Journal
                medba
                Medicina (Buenos Aires)
                Medicina (B. Aires)
                Fundación Revista Medicina (Ciudad Autónoma de Buenos Aires, , Argentina )
                0025-7680
                1669-9106
                August 2017
                : 77
                : 4
                : 331-333
                Affiliations
                [01] orgnameFundación Favaloro orgdiv1Hospital Universitario orgdiv2Departamento de Ortopedia y Traumatología
                Article
                S0025-76802017000400015
                7d1884a7-a694-4310-84f9-983b63846fba

                This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

                History
                : 11 July 2016
                : 03 April 2017
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 9, Pages: 3
                Product

                SciELO Argentina


                Sodium thiosulfate
                Sodium thiosulfate

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