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Fabry Disease The Kidney

Over time, the accumulation of GL-3 in the blood vessels of the kidneys causes problems with kidney function and may eventually lead to kidney failure. Kidney problems may be accompanied by hypertension (high blood pressure). People with a mild loss of kidney function may be helped with a low-salt, low-protein diet. Severe kidney problems or kidney failure can be treated with dialysis (a procedure that performs the kidneys' job of removing waste products from the blood) or transplantation (surgery to replace one or both kidneys). Kidney failure is the most common cause of death in Fabry disease.

Monitoring
Kidney function can be monitored with periodic testing of the blood (creatinine measurement) and the urine (urinalysis). People with kidney problems often have a high creatinine level in their blood and/or protein or blood in their urine. In addition, people with Fabry disease may have clusters of fat in their urine, called Maltese crosses because of their shape. Hypertension can be monitored with periodic blood pressure checks.

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