|
KMID : 0386119980380020367
|
|
Journal of the Korean Radiological Society
1998 Volume.38 No. 2 p.367 ~ p.370
|
|
|
Bone Changes in Phenylketonuria
|
|
Hong Hyun-Sook
Lee Hae-Kyung
Kwon Kui-Hyang
Choi Deuk-Lin
Lee Dong-Hwan
|
|
|
Abstract
|
|
|
Purpose : While treating 14 phenylketonuria(PKU) patients, we evaluated bone density, changes in bone age, and bony changes such as spiculation or metaphyseal widening.
Materials and Methods :A total of 14 PKU patients aged between 1 month and 14 years(mean, 6.4 years) were under dietary treatment. Eight and eleven patients underwent patients underwent radiography of the left hand and wrist and bone
densitometry (BMD) of the lumbar spine, respectively. The results were assigned to either the early or late treatment group, depending on whether or not dietary theraphy was started before 3 months of age. Those in whom a blood phenylalanine level of under 10 mg/dl was maintained were assigned to the ’good control’ group: others were classified as ’variable cintrol’. The findings of radiographs of the left hand and lumbar BMD were evaluated in relation to the time of dietary therapy, and adequacy of treatment.
Results : On lumbar BMD, four of 11 patients (36%) showed reduced bone density of more than 1 S.D. None of the 11 who underwent radiography of the left hand showed bony abnormalities such as spiculation or metaphyseal widening. In four of the 11, bone age was less than chronological age by at least one year. According tl Fisher’s exact test there was no relation between delayed bone age, osteoporosis and the time and adequacy of dietary therapy (p >0.05).
Conclusion : None of the 14 PKU patients who underwent dietary therapy had bony abnormalities such as spiculation or metaphyseal widening. In four of the 11, bone age was at lest one year less than chronological age, and on lumbar BMD, osteoporosis was seen. For the evaluation of bone change in PKU patients, plain radiography and BMD are thus complementary. (J Korean Radiol Soc 1998; 38:367-370)
|
|
|
KEYWORD
|
|
|
Children, skeletal system, Bones, growth and development, Bones, mineralization, Phenylketonuria, Metabolism,
|
|
|
FullTexts / Linksout information
|
|
|
|
|
Listed journal information
|
|
 
|
|