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      Bilateral breast calciphylaxis in a patient who survived earlier extensive tissue necrosis 5 years previously: A case report

      case-report

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          Highlights

          • Consider calciphylaxis in patients with renal disease developing skin necrosis.

          • A multidisciplinary treatment approach is recommended.

          • The role of surgery and hyperbaric oxygen for tissue necrosis is limited.

          Abstract

          Introduction

          Calciphylaxis is a rare condition including patchy dermal necrosis that mostly affects chronic hemodialysis patients. The syndrome usually heralds impending death although patients may survive following a set of measures including an adapted dialysis regimen. The present case is a unique patient who recovered from an earlier episode of upper leg calciphylaxis 5 years previously but developed fatal bilateral breast necrosis.

          Presentation of case

          A 69 year old Caucasian woman with a history of atrial fibrillation, hypertension, CVA, hyperparathyroidectomy for secondary hyperparathyroidism and end stage renal disease with hemodialysis recovered in 2012 from extensive symptomatic left upper leg necrosis due to calciphylaxis. In 2017, she developed painful, necrotic ulcers on both breasts, again due to calciphylaxis. She had no history of anticoagulants use but she did use prednisolone 5mg/day. She received adequate wound care, pain medication, antibiotics and dialysis frequency was increased with an addition of sodium thiosulfate. A bilateral ablation was discussed but she decided to stop all treatment following pulmonary aspiration and passed away one week later.

          Discussion

          Calciphylaxis is a rare diagnosis that should be considered in patients with renal insufficiency developing painful patches of skin necrosis.

          Conclusion

          A multidisciplinary treatment approach including hyperparathyroidectomy, modified hemodialysis and wound treatment is recommended. There is limited evidence for surgical intervention.

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          Most cited references17

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          Calciphylaxis: risk factors, diagnosis, and treatment.

          Calciphylaxis is a rare but devastating condition that has continued to challenge the medical community since its early descriptions in the scientific literature many decades ago. It is predominantly seen in patients with chronic kidney failure treated with dialysis (uremic calciphylaxis) but is also described in patients with earlier stages of chronic kidney disease and with normal kidney function. In this review, we discuss the available medical literature regarding risk factors, diagnosis, and treatment of both uremic and nonuremic calciphylaxis. High-quality evidence for the evaluation and management of calciphylaxis is lacking at this time due to its rare incidence and poorly understood pathogenesis and the relative paucity of collaborative research efforts. We hereby provide a summary of recommendations developed by a multidisciplinary team for patients with calciphylaxis.
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            Risk factors and mortality associated with calciphylaxis in end-stage renal disease.

            We conducted a case control study to determine risk factors and mortality associated with calciphylaxis in end-stage renal disease. Cases of calciphylaxis diagnosed between December 1989 and January 2000 were identified. Three controls were identified for each hemodialysis patient, with calciphylaxis matched to the date of initiation of hemodialysis. Laboratory data and medication doses were recorded during the 12 months prior to the date of diagnosis and at the time of diagnosis of calciphylaxis. Conditional logistic regression was used to identify risk factors for calciphylaxis. Cox proportional hazards models were used to estimate the risk of death associated with calciphylaxis. Nineteen cases and 54 controls were identified. Eighteen patients were hemodialysis patients, and one had a functioning renal allograft. Diagnosis was confirmed by skin biopsy in 16 cases. Women were at a sixfold higher risk of developing calciphylaxis (OR = 6.04, 95% CI 1.62 to 22.6, P = 0.007). There was a 21% lower risk of calciphylaxis associated with each 0.1 g/dL increase in the mean serum albumin during the year prior to diagnosis and at the time of diagnosis of calciphylaxis (OR = 0.79, 95% CI, 0.64 to 0.99, P = 0.037, and OR = 0.80, 95% CI, 0.67 to 0.96, P = 0.019, respectively). There was a 3.51-fold increase in the risk of calciphylaxis associated with each mg/dL increase in the mean serum phosphate during the year prior to diagnosis (95% CI, 0.99 to 12.5, P = 0.052). At the time of diagnosis of calciphylaxis, for each 10 IU/L increment in alkaline phosphatase, the risk of calciphylaxis increased by 19% (OR = 1.19, 95% CI, 1.00 to 1.40, P = 0.045). Body mass index, diabetes, blood pressure, aluminum, and higher dosage of erythropoietin and iron dextran were not independent predictors of calciphylaxis. Calciphylaxis independently increased the risk of death by eightfold (OR = 8.58, 95% CI, 3.26 to 22.6, P < 0.001). Female gender, hyperphosphatemia, high alkaline phosphatase, and low serum albumin are risk factors for calciphylaxis. Calciphylaxis is associated with a very high mortality.
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              Sodium thiosulfate therapy for calcific uremic arteriolopathy.

              Calcific uremic arteriolopathy (CUA) is an often fatal condition with no effective treatment. Multiple case reports and case series have described intravenous sodium thiosulfate (STS) administration in CUA, but no studies have systematically evaluated this treatment.
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                Author and article information

                Contributors
                Journal
                Int J Surg Case Rep
                Int J Surg Case Rep
                International Journal of Surgery Case Reports
                Elsevier
                2210-2612
                07 May 2018
                2018
                07 May 2018
                : 48
                : 22-25
                Affiliations
                [0005]Departement of general surgery. Máxima Medisch Centrum, De Run 4600, 5504 DB Veldhoven, The Netherlands
                Author notes
                [* ]Corresponding author at: Máxima Medisch Centrum. De Run 4600, 5504 DB, Veldhoven, The Netherlands. emj.verstappen@ 123456student.maastrichtuniversity.nl
                Article
                S2210-2612(18)30161-5
                10.1016/j.ijscr.2018.04.035
                6043165
                29775967
                58d2136d-6cc1-4dfb-a331-6f071fe6d960
                © 2018 The Authors

                This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

                History
                : 1 February 2018
                : 6 April 2018
                : 28 April 2018
                Categories
                Article

                calciphylaxis,cua,calcific uremic arteriolopathy,breast
                calciphylaxis, cua, calcific uremic arteriolopathy, breast

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