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Management of Calciphylaxis A Case Study and Literature Review

1 August 2007


Context: Calciphylaxis (Cal-Cee-Fah-Lax-Is) is a rare but deliberating disease that affects mostly patients with End-Stage Renal Disease (ESRD) on Dialysis or have had recent renal transplant. The disease has however been seen in people without ESRD. Calciphylaxis has been reported in patients with clinical diagnosis of breast carcinoma treated with chemotherapy, alcoholic liver cirrhosis, cholangiocarcinoma, Crohn's disease, rheumatoid arthritis, and systemic lupus erythematosus with or without chronic kidney disease.

Objective: To promote understanding ofthe condition by developing a report that blends a current literature review with a case study, to assist medical personnel to identify the disease early and to best manage it. Calciphylaxis is mainly cutaneous but can systemic. This study focuses on the cutaneous calciphylaxis.

Data Sources, Study Selection and Data Extraction: The patient's chart was reviewed. The MEDLINE and OVID databases were searched for articles published between 1976 and June 2007 using database specific keywords. Bibliographies of the retrieved articles were searched, along with Cochrane Library and relevant websites. Reference textbooks were searched for further background and supporting information. The retrieved literature was reviewed for pertinent information.

Data Synthesis: Calciphylaxis affects 1-4% of the people with ESRD, it is characterized by small vessel mural calcification with or without endovascular fibrosis, extravascular calcification and vascular thrombosis, leading to tissue ischemia. The pathogenesis of calciphylaxis is poorly understood, making diagnosis difficult especially in non-ESRD patients. At present there is no clear first line treatment recommendation. However a few medications have shown promising results including Sodium Thiosulfate, Cinacalcet, Pamidronate, Etidronate disodiurn, Tissue Plasminogen Antigen and prednisone. Parathyroidectomy has also been used. On the other hand this is a disease with a high mortality rate, 60-80%.

Conclusion: Calciphylaxis is a rare but deliberating disease that can be managed with early recognition and treatment. Treatment is directed at the underlying cause. Pain management and wound care remains the cornerstone of patient care. Sodium Thiosulfate has been used successively in several patients with and without a high calcium/phosphate product.


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