Calciphylaxis.

J Tenn Med Assoc

Vanderbilt University Medical Center, Nashville, USA.

Published: October 1995

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Calciphylaxis is a rare but potentially life-threatening disease that is not yet completely understood. It occurs mainly in patients with chronic kidney disease termed calcific uremic arteriolopathy (CUA) but also affects patients with normal renal function. Although this disease's pathogenesis is unclear, it is associated with the dysregulation of calcium and phosphate and subsequent calcification of peripheral arterioles.

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Background: Vascular calcification is highly prevalent and associated with mortality in hemodialysis patients. However, extreme splanchnic arterial calcification in calciphylaxis with poor prognosis raises questions regarding the reliability of previous vascular calcification scoring methods. Therefore, this study aimed to examine the distribution characteristics of abdominal aortic branch calcification and identify a more reliable predictor of mortality in hemodialysis patients.

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Calciphylaxis is a rare and serious disorder almost exclusively seen in patients on dialysis or those with advanced chronic kidney disease (CKD) not on dialysis and is associated with very high mortality. We present the case of a 50-year-old male with a background of end-stage renal disease (ESRD) compliant with dialysis, parathyroid adenoma, secondary hyperparathyroidism, and high body mass index (BMI). Whilst receiving 31 doses of intravenous sodium thiosulphate (STS) over an 11-week period, the patient underwent surgical debridement of multiple painful ulcerative lesions in his lower abdomen and left thigh and then subsequently a subtotal parathyroidectomy at 70 days from admission.

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Calciphylaxis, also known as calcific uremic arteriolopathy (CUA), is a rare disorder with many unknown treatment and diagnostic aspects. It is characterized by calcification and thrombosis of small blood vessels. This disease leads to progressive skin calcification, necrotizing ulcers, and infections and is associated with a high mortality rate.

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