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July 1966

Hemodialysis in a Patient With Cystinosis: Effects on Amino Acid and Bone Metabolism

Author Affiliations

From the Department of Pediatrics, University of Washington, Seattle, and departments of biochemistry and research, Sacred Heart Hospital, Spokane, Wash.

Am J Dis Child. 1966;112(1):65-71. doi:10.1001/archpedi.1966.02090100101015

THE PATHOGENESIS of cystine crystal formation in cystinosis (Lignac-Fanconi disease) is unknown.1-3 Baar and Bickel proposed that cystinosis is a disorder of intracellular protein metabolism, and cystine crystalizes intracellularly at the site of its formation.2 More recently, Worthen and Good3 postulated that cystine crystal formation is also due to an elevated blood concentration of cystine,4 leading to a gradual deposition of supersaturating amounts of cystine in the cornea and renal tubule and to phagocytosis by the cells of the reticuloendothelial system. Woods et al, in a study of the effects of hemodialysis on uremic patients, reported a 22% to 29% removal of free plasma amino acids with each passage of blood through the artificial kidney during a six-hour dialysis.5 In order to see if hemodialysis would result in a greater removal of cystine than other amino acids, as would be expected if a large miscible