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Roentgenologic Bone Changes in PhenylketonuriaRelation to Dietary Phenylalanine and Serum Alkaline Phosphatase
MALCOLM M. MURDOCH, MD;
GERALD H. HOLMAN, MD
Am J Dis Child. 1964;107(5):523-532.
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Introduction
Recently, Feinberg and Fisch 1 described the presence of calcified spicules projecting into the epiphyseal area of the long bones in some patients with phenylketonuria. These spicules were described as dense, blunt projections, associated with early cupping of the metaphysial plate. With bone growth this cupping disappeared and the spicules shortened, broadened, and were incorporated into the metaphysial spongiosa. Ultimately, they were seen in the diaphysis. The changes differ from rickets in that they occur earlier (as early as 3 months of age), there is no dissolution or fraying of the metaphysial plate of the epiphysis, the metaphysis does not vanish, and the spicules are more coarse.
These authors described five patients who demonstrated "strongly positive" roentgenologic changes while receiving 15-30 mg/kg/day of phenylalanine and maintaining a serum phenylalanine level below 8 mg/100 ml. Four patients with "less positive" changes were either not on the diet or inadequately
. . . [Full Text PDF of this Article]
Author Affiliations
KANSAS CITY, KAN
Gerald H. Holman, University of Kansas Medical Center, 39th and Rainbow Blvd, Kansas City, Kan 06103.; John and Mary R. Markle Scholar in Academic Medicine (Dr. Holman).; Department of Pediatrics, Kansas University Medical
Footnotes
Received for publication Sept 24, 1963.
Aided in part by grant No. 06695-01, Division Arthritis and Metabolism, National Institute of Health, and in part by a grant from the Kansas State Health Department, Division of Maternal and Child Health.
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