What is Nephrology – What is Nephrogenic Systemic Fibrosis ?
NSF (nephrogenic systemic fibrosis) is a type of systemic fibrosis that affects the kidneys. NSF (nephrogenic fibrosing dermopathy) is characterized by gradual fibrosis and thickening of the skin (similar to scleroderma), which is very painful, as well as fibrosis in other organs (e.g., pleura, diaphragm). Plaques, papules, or nodules show as asymmetrically distributed plaques, papules, or nodules over the distal extremities. Deposition of gadolinium (a contrast substance used in magnetic resonance imaging) that is not removed in the presence of severe chronic renal disease or acute kidney damage is the etiology. The time between gadolinium exposure and the onset of NSF symptoms might range from 2 days to 18 months. This variation is due to gadolinium mobilization from bone over time. When opposed to macro cyclic gadolinium compounds, the risk of NSF appears to be higher with linear molecules (e.g., gadodiamide). Because NSF has no effective treatment and a significant mortality rate, prevention is the primary goal. In individuals with severe chronic kidney disease or acute kidney injury, gadolinium-enhanced scans should be avoided. In patients with advanced chronic renal disease, hemodialysis (HD; removes 92 percent of gadolinium after two HD sessions; 99 percent after three HD sessions) has been described as a preventative strategy when gadolinium-enhanced scans are required. Similarly, a high-intensity peritoneal dialysis (PD) program can remove gadolinium (90 percent of the gadolinium in two days with 10 to 15 PD exchanges each day). It is unclear whether these efforts are successful.
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