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a Department of Paediatrics, John Radcliffe Hospital,
Oxford, b Department of Paediatrics,
Royal Brompton Hospital, London, c Department of Medicine, Bristol Royal
Infirmary, Bristol
Correspondence to: Dr D B Dunger, Department of Paediatrics, John Radcliffe Hospital, Oxford OX3 9DU.
Accepted 26 October 1996
OBJECTIVES
Despite improved nutrition and
intensive treatment, subjects with cystic fibrosis have difficulty in
maintaining anabolism during intercurrent infections, which can result
in reduced body mass index and impaired skeletal growth. Insulin-like
growth factor-I (IGF-I) and its binding protein IGFBP3 are sensitive to
changes in nutritional status. The aim of this study was to determine the relation between circulating concentrations of these peptides, body
mass index, and clinical status in cystic fibrosis.
METHODS
Serum concentrations of IGF-I and IGFBP3
were measured in 197 subjects (108 males, 89 females; mean age 9.69 years, range 0.41-17.9 years) and these data were analysed with respect
to body mass index, pubertal stage, and clinical status as assessed by
Shwachman score and forced expiratory volume in one second (FEV1 ).
RESULTS
The mean height SD score of the children
studied was
0.2 (SD 1.14) and the body mass index SD score
0.26
(1.4). The body mass index SD score declined with increasing age
(r=
0.18) and paralleled changes in IGF-I
concentrations, which also declined. The IGF-I SD score (calculated
from control data) correlated with age (r=
0.53). The
abnormalities were most obvious during late puberty, when IGF-I and
IGFBP3 concentrations were significantly reduced compared with those in
control subjects matched for pubertal stage. The IGF-I SD score
correlated with height SD score (r=0.14) and the decline
in IGF-I concentrations with the fall in body mass index SD score
(r=0.42). IGF-I SD scores also correlated with the
Shwachman score (r=0.33) and FEV1
(r=0.17).
CONCLUSIONS
The close relation between declining
IGF-I and IGFBP3 concentrations and body mass index in patients with
cystic fibrosis may simply reflect poor nutritional status and insulin
hyposecretion. Nevertheless, IGF-I deficiency could also contribute
towards the catabolism observed in these patients, and IGF-I SD scores
correlated with other measures of clinical status such as the Shwachman
score and FEV1.
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