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Article |

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

Denise Ney, RD; Carolyn Bay, MS; Jerry A. Schneider, MD; Drew Kelts, MD; William L. Nyhan, MD, PhD
Am J Dis Child. 1983;137(10):995-1000. doi:10.1001/archpedi.1983.02140360055018.
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• 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).


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