Radiological assessment of the hand and wrist in phenylketonuria and hyperphenylalaninaemia

A radiograph of the left hand and wrist was taken in 141 children and young adults with phenylketonuria and hyperphenylalaninaemia. Ten (7.1%) had a bony abnormality and, of these, six (4.3%) showed evidence of reduced bone density. Only one patient had evidence of spiculation of the lower ulnar met...

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Bibliographic Details
Published in:Pediatric radiology 1995-07, Vol.25 (5), p.353-355
Main Authors: GREEVES, L. G, THOMAS, P. S, CARSON, D. J
Format: Article
Language:English
Subjects:
Adolescent
Adult
Age Determination by Skeleton
Aminoacid disorders
Biological and medical sciences
Bone and Bones - diagnostic imaging
Bone Density
Bone Development
Child
Child, Preschool
Errors of metabolism
Female
Hand - diagnostic imaging
Humans
Infant
Male
Medical sciences
Metabolic diseases
Phenylalanine - blood
Phenylketonurias - blood
Phenylketonurias - diagnostic imaging
Phenylketonurias - diet therapy
Phenylketonurias - physiopathology
Wrist Joint - diagnostic imaging
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Summary:A radiograph of the left hand and wrist was taken in 141 children and young adults with phenylketonuria and hyperphenylalaninaemia. Ten (7.1%) had a bony abnormality and, of these, six (4.3%) showed evidence of reduced bone density. Only one patient had evidence of spiculation of the lower ulnar metaphysis previously described in phenylketonuria. Bone maturity was also assessed in 130 patients by the Tanner-Whitehouse (TW2) method. In 28 patients (21.5%) bone age was less than chronological age by 1 year or more. Bone age delay was greater in younger children on a more restricted diet with a lower intake of natural protein.
ISSN:0301-0449
1432-1998
DOI:10.1007/BF02021701