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      Calciphylaxis and its diagnosis: A review

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          Abstract

          Calciphylaxis also known as Calcific uremic arteriolopathy (CUA), is a rare fatal complication usually associated with end-stage renal disease (ESRD). It is characterized by skin ulceration and necrosis leading to significant pain. The disease calciphylaxis is pathological state resulting in accumulation of calcium content in medial wall of small blood vessels along with the fibrotic changes in intima. The aetiopathogenesis of this disease, small vessel vasculopathy, remains complicated, and unclear. It is believed that development of calciphylaxis depends on medial calcification, intimal fibrosis of arterioles and thrombotic occlusion. The disease is rare, life-threatening medical condition that occurs mostly in population with kidney disease or in patients on dialysis. Skin biopsy and radiographic features are helpful in the diagnosis of calciphylaxis, but negative results do not necessarily exclude the diagnosis. This article highlights steps undertaking in the diagnosis of calciphylaxis.

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          Most cited references33

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          The serum protein alpha 2-Heremans-Schmid glycoprotein/fetuin-A is a systemically acting inhibitor of ectopic calcification.

          Ectopic calcification is a frequent complication of many degenerative diseases. Here we identify the serum protein alpha2-Heremans-Schmid glycoprotein (Ahsg, also known as fetuin-A) as an important inhibitor of ectopic calcification acting on the systemic level. Ahsg-deficient mice are phenotypically normal, but develop severe calcification of various organs on a mineral and vitamin D-rich diet and on a normal diet when the deficiency is combined with a DBA/2 genetic background. This phenotype is not associated with apparent changes in calcium and phosphate homeostasis, but with a decreased inhibitory activity of the Ahsg-deficient extracellular fluid on mineral formation. The same underlying principle may contribute to many calcifying disorders including calciphylaxis, a syndrome of severe systemic calcification in patients with chronic renal failure. Taken together, our data demonstrate a critical role of Ahsg as an inhibitor of unwanted mineralization and provide a novel therapeutic concept to prevent ectopic calcification accompanying various diseases.
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            Mechanisms of vascular calcification in chronic kidney disease.

            Vascular calcification is common in chronic kidney disease and associated with increased morbidity and mortality. Its mechanism is multifactorial and incompletely understood. Patients with chronic kidney disease are at risk for vascular calcification because of multiple risk factors that induce vascular smooth muscle cells to change into a chondrocyte or osteoblast-like cell; high total body burden of calcium and phosphorus due to abnormal bone metabolism; low levels of circulating and locally produced inhibitors; impaired renal excretion; and current therapies. Together these factors increase risk and complicate the management of vascular calcification.
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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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                Author and article information

                Journal
                J Family Med Prim Care
                J Family Med Prim Care
                JFMPC
                Journal of Family Medicine and Primary Care
                Wolters Kluwer - Medknow (India )
                2249-4863
                2278-7135
                September 2019
                30 September 2019
                : 8
                : 9
                : 2763-2767
                Affiliations
                [1 ] Department of Conservative Dentistry and Endodontics, P.S.M Dental College and Research Centre, Akkikavu, Thrissur, Kerala, India
                [2 ] Department of Pediatric and Preventive Dentistry, Saraswati Dental College, Lucknow, Uttar Pradesh, India
                [3 ] Department of Prosthodontics, Rajasthan Dental College and Hospital, Jaipur, Rajasthan, India
                [4 ] Department of Oral Medicine and Radiology, Institute of Dental Sciences, Siksha ‘O’ Anusandhan (Deemed to be University), Bhubaneswar, Odisha, India
                [5 ] Department of Oral and Maxillofacial Pathology, Hi-Tech Dental College and Hospital, Bhubaneswar, Odisha, India
                [6 ] Department of Periodontics, Private Practitioner, Chandigarh, India
                Author notes
                Address for correspondence: Dr. Shivangi Gupta, Private Practitioner, Chandigarh, India. E-mail: shivangigupta69@ 123456gmail.com
                Article
                JFMPC-8-2763
                10.4103/jfmpc.jfmpc_588_19
                6820424
                31681640
                ec600966-8ba9-4072-81c9-d1b9b9980c18
                Copyright: © 2019 Journal of Family Medicine and Primary Care

                This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.

                History
                : 26 July 2019
                : 20 August 2019
                : 05 September 2019
                Categories
                Review Article

                calciphylaxis,calcium,small blood vessels
                calciphylaxis, calcium, small blood vessels

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