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Canadian Journal of Anesthesia, Vol 40, 518-520, Copyright © 1993 by Canadian Anesthesiologists' Society

ARTICLES

Anaesthetic implications of cystinosis

JD Tobias
Division of Pediatric Anesthesiology/Critical Care Medicine, Vanderbilt University, Nashville, Tennessee 37232.

The author presents and discusses the anaesthetic care of a 12-yr-old boy with cystinosis. Cystinosis is a recessively inherited disorder of amino acid metabolism resulting in the abnormal intracellular accumulation of cystine. Anaesthetic care may be affected by variable end-organ involvement, most notably progressive renal deterioration beginning with the development of Fanconi syndrome and progressing to overt renal failure during the first decade of life. Additional organ system involvement may lead to cirrhosis with portal hypertension, diabetes mellitus, and hypothyroidism. Identification of the extent of end-organ involvement during the preoperative evaluation will help in the provision of safe anaesthetic care for such patients.




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Copyright © 1993 by the Canadian Anesthesiologists' Society.