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Iron and Protein Sufficiency and Red Cell Indices in Phenylketonuria

Source: www.jacn.org
Topic: Phenylketonuria
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Sort Desciption: The dietary management of phenylketonuria (PKU) includes severe restriction of phenylalanine through the limitation of dietary protein and the addition of amino acids, vitamins and ...

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The dietary management of phenylketonuria (PKU) includes severe restriction of phenylalanine through the limitation of dietary protein and the addition of amino acids, vitamins and other nutrients through medical food (commonly known as "formula"). The loss of natural nutrients including heme iron, protein, vitamins and trace metals potentially affects hematopoiesis, and both iron and protein deficiency previously have been reported in PKU 1â 6 . However, there is a paucity of information on the potential effects of the recommended PKU diet on red cell counts and indices in children with PKU. One prior study identified lower hemoglobin and higher mean corpuscular hemoglobin in children with treated PKU compared to children without PKU, although mean values were within the normal range 2 . Iron deficiency without anemia was apparent in several subjects in that study, but the role of protein sufficiency in hematopoiesis was not addressed. In contrast, another study did not identify any differences in hematopoiesis 5 . We reviewed the hemoglobin, hematocrit and red cell count and indices in relationship to the iron and protein status of our patients with PKU to identify the extent to which iron or protein insufficiency might contribute to impaired hematopoiesis.

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