Adult Medical Issues in Cystinosis--William Gahl, M.D.
Adult Medical Issues in Cystinosis--William Gahl, M.D.
Adult Medical Issues in Cystinosis--William Gahl, M.D.
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Nephrogenic Fibros<strong>in</strong>gDermopathy• Entity first reported <strong>in</strong> 1997• 15 patients on chronic dialysis developed scleroderma-like disease• Most rapidly progressive and debilitat<strong>in</strong>g -- no apparentbenefit to range of anti-<strong>in</strong>flammatory and immunosuppressive drugs• Cause unknown:? Dialysate or dialyzer tox<strong>in</strong>? Erythropoiet<strong>in</strong>? Occult <strong>in</strong>fection? Autoimmunity