Absence of increased height velocity in the first year of life in untreated children with simple virilizing congenital adrenal hyperplasia.
BACKGROUND
In congenital adrenal hyperplasia (CAH), elevation of adrenal androgens leads to accelerated growth and bone maturation with compromised adult height.


OBJECTIVE
The objective of the study was to analyze retrospectively early growth and bone maturation in 17 untreated simple virilizing (SV) CAH patients.


METHODS
The study was conducted at Radboud University Nijmegen Medical Centre.


METHODS
Growth data were collected until time of diagnosis. Height was expressed as height sd score and corrected for target height. Bone maturation was determined and expressed as bone age acceleration.


METHODS
Growth pattern and bone maturation were measured before the diagnosis.


RESULTS
In the term group (n = 11), there was no increase in height sd score and corrected for target height in the first year of life [-0.1 sd/yr; 95% confidence interval (CI) -0.5, 0.3] with a consecutive significant (P < 0.001) increase up to 0.9 sd/yr (95% CI 0.7, 1.0). In the premature group (n = 3), there was a catch-up growth of 1.6 sd/yr (95% CI 0.9, 2.3) in the first year followed by a growth of 1.1 sd/yr (95% CI 0.9, 1.5) in the following years. There was a positive linear correlation between bone age acceleration and age of diagnosis (r = 0.8).


CONCLUSIONS
Height velocity and bone maturation are not increased in untreated children with mild forms of SV CAH in the first year of life. After this period there is a progressive increase in height velocity and bone maturation in strong relation to the duration of androgen exposition. This observation has implications for the dose of glucocorticoids to be used in SV CAH patients in the first year of life.
Claahsen-van der Grinten
Hedi L
HL
Department of Metabolic and Endocrine Diseases (435), Radboud University Nijmegen Medical Centre, P.O. Box 9101, 6500 HB Nijmegen, The Netherlands. h.claahsen@cukz.umcn.nl
Noordam
Kees
K
Borm
George F
GF
Otten
Barto J
BJ
D000305:Adrenal Cortex Hormones / Q000097:blood; D000312:Adrenal Hyperplasia, Congenital / Q000175:diagnosis / Q000473:pathology*; D000728:Androgens / Q000378:metabolism; D001724:Birth Weight / Q000502:physiology; D001827:Body Height / Q000502:physiology*; D001846:Bone Development / Q000502:physiology; D002648:Child; D002675:Child, Preschool; D004252:DNA Mutational Analysis; D005260:Female; D006128:Growth / Q000502:physiology*; D006801:Humans; D007223:Infant; D016014:Linear Models; D008297:Male; D012189:Retrospective Studies
D000305:Adrenal Cortex Hormones; D000728:Androgens