What is Nephrology - What is the definition of calciphylaxis?
Calcific uremic arteriolopathy (CUA), commonly known as calciphylaxis, is a painful condition characterized by purpuric plaques and nodules under the skin. In severe disease, these nodules may necrose. Despite the severe skin manifestations, it's best to conceive of it as a systemic disease that needs to be treated promptly due to its high fatality rate. When the extremities are implicated, the lesions are commonly described as a mottled or violaceous discoloration with a reticular pattern, comparable to livedo reticularis (seen in atheroembolic kidney disease, antiphospholipid syndrome, and cryoglobulinemia) and are bilateral and symmetric in distribution. It's worth noting that those with proximal lesions (trunk, buttocks, and thighs) have a worse prognosis than those with more distal lesions (forearms and fingers; calves and toes). Poorly treated secondary hyperparathyroidism, uncontrolled diabetes mellitus, obesity, female sex, duration of renal replacement treatment, history of skin damage, and use of warfarin are all known risk factors for CUA. Increased expression of osteopontin and bone morphogenic protein 4 suggests that inducers of vascular calcification play a key role in the disease's etiology. Suspicion is essential for early detection. When cancer is detected early on (nonulcerative), treatment interventions have been found to enhance prognosis. The key to managing CUA is prevention. Controlling secondary hyperparathyroidism aggressively is critical. One of the treatments for established CUA is sodium thiosulfate. It is thought to work through two mechanisms: 1. Removes calcium from soft tissues by chelating it. 2. Antioxidant, enhancing local blood flow and soft tissue oxygenation by stimulating endothelial nitric oxide synthesis. 5 to 25 g of intravenous (IV) Na thiosulfate delivered toward the end of dialysis for several weeks to months is a standard treatment regimen. Although there is minimal data, bisphosphonates (IV pamidronate and ibandronate, and PO etidronate) may be useful in modifying ectopic calcium phosphate deposition and directly suppressing calcification via the nuclear factor F06BF06BB cascade. Hyperbaric oxygen therapy enhances oxygen supply to damaged tissues by increasing oxygen partial pressure; it also aids wound healing by promoting phagocytosis and angiogenesis while reducing tissue edema.
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