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Absence of Increased Height Velocity in the First Year of Life in Untreated Children with Simple Virilizing Congenital Adrenal Hyperplasia

Departments of Metabolic and Endocrine Diseases (H.L.C.-v.d.G., K.N., B.J.O.) and Epidemiology and Biostatistics (G.F.B.), Radboud University Nijmegen Medical Centre, 6500 HB Nijmegen, The Netherlands

Address all correspondence and requests for reprints to: H. L. Claahsen-van der Grinten, M.D., Radboud University Nijmegen Medical Centre, Department of Metabolic and Endocrine Diseases (435), P.O. Box 9101, 6500 HB Nijmegen, The Netherlands. E-mail: h.claahsen{at}cukz.umcn.nl.

Context: In congenital adrenal hyperplasia (CAH), elevation of adrenal androgens leads to accelerated growth and bone maturation with compromised adult height.

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

Setting: The study was conducted at Radboud University Nijmegen Medical Centre.

Interventions: 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_.

Main Outcome Measures: 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.