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Volume 348:1722-1724 April 24, 2003 Number 17
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Tetrahydrobiopterin and Mild Phenylketonuria

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by Muntau, A. C.
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To the Editor: The concept that phenylketonuria may be cured by tetrahydrobiopterin, the cofactor of phenylalanine hydroxylase, rather than by dietary protein restriction, has generated a number of therapeutic attempts. Muntau et al. (Dec. 26 issue)1 offer a more extensive study and claim that the majority of mild cases of phenylalanine hydroxylase deficiency are variably responsive to tetrahydrobiopterin. Major issues concerning the selection of patients and the experimental design, however, weaken the significance of their findings. Patients were classified according to their pretreatment plasma phenylalanine concentration instead of their phenylalanine tolerance, but only the latter permits the definite identification of . . . [Full Text of this Article]



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