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October 1983

Dietary Management of Oculocutaneous Tyrosinemia in an 11-Year-Old Child

Author Affiliations

From the Department of Pediatrics (Drs Schneider, Kelts, and Nyhan and Ms Bay) and General Clinical Research Center (Mss Ney and Bay), University of California—San Diego Medical Center.

Am J Dis Child. 1983;137(10):995-1000. doi:10.1001/archpedi.1983.02140360055018

• An 11-year-old girl with keratitis and plantar keratosis had tyrosinemia. The concentration of tyrosine in the plasma was 16.5 mg/dL. Dietary intake of phenylalanine and tyrosine was systematically varied, and the plasma concentrations of tyrosine and nitrogen balance were studied. It was necessary to achieve a total intake of phenylalanine and tyrosine less than 100 mg/kg/day to obtain plasma concentrations of tyrosine of less than 10 mg/dL. After dietary therapy was started, the keratitis resolved promptly, and the patient remained asymptomatic during a period of 16 months in which the mean plasma concentration of tyrosine was 11.1 mg/dL. The dietary management of a child at this age presents a different problem from that of a young infant. It can be successfully pursued at home, as well as in the carefully regulated environment of a clinical research center.

(Am J Dis Child 1983;137:995-1000).