What is the role of transplantation in the treatment of Fanconi syndrome?

Updated: Feb 09, 2018
  • Author: Sahar Fathallah-Shaykh, MD; Chief Editor: Craig B Langman, MD  more...
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Answer

Liver transplantation has been successfully used in patients with liver failure due to Wilson disease or tyrosinemia. Liver transplantation leads to the rapid disappearance of the renal tubular abnormalities.

Kidney transplantation has been performed in many patients with renal failure due to cystinosis. Cystine accumulates in the monocytes and interstitial cells of the transplanted kidney but not in proximal tubule cells. Consequently, the tubular transport abnormalities do not recur.


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