4.3 Article

Compromised adult height in females with non-classical congenital adrenal hyperplasia diagnosed in childhood

Journal

HORMONE RESEARCH IN PAEDIATRICS
Volume -, Issue -, Pages -

Publisher

KARGER
DOI: 10.1159/000529772

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Data on adult height in individuals with non-classical congenital adrenal hyperplasia (NCCAH) are inconsistent. A retrospective study found that adult height of females diagnosed with NCCAH in childhood was lower than their target height, regardless of glucocorticoid treatment. Pubertal status at diagnosis and the presence of central precocious puberty or early puberty were not correlated with adult height.
IntroductionData on adult height (AHt) in individuals with non-classical congenital adrenal hyperplasia (NCCAH) are inconsistent. MethodsWe conducted a retrospective study of 109 females diagnosed with NCCAH at age <18 years who reached AHt. We studied AHt compared to target height (THt), and the correlation of AHt with clinical parameters. Results The mean age at diagnosis was 9.7 +/- 4.4 years, the mean follow-up was 10.9 +/- 6.3 years. Hydrocortisone treatment (11.0 +/- 5.0 mg/m2) was initiated at age 9.7 +/- 4.0 years. Bone age was more advanced in girls who presented with central precocious puberty or early puberty (CPP/EP) (n=43) than with timely puberty. AHt-SDS was lower than Ht-SDS at diagnosis (-0.8 +/- 1.0 vs. +0.2 +/- 1.3; P<0.001), and -0.3 SDS shorter than THt (P<0.001). Height, weight and BMI-SDS at last visits were similar between patients treated with glucocorticoids (n=92) and those never treated (n=17). AHt was comparable between patients with timely puberty and with CPP/EP, with no difference between those treated or not by GnRH analogue. AHt was similar between patients who were fully pubertal, prepubertal and pubertal at diagnosis (158.0 +/- 7.6, 158.1 +/- 6.1 and 157.5 +/- 6.5, respectively; P=0.9). AHt-SDS was correlated with THt (R=0.67, P<0.001) and Ht-SDS at diagnosis (R=0.7; P<0.001), but not with age at diagnosis (R=-0.05, P=0.6), the extent of bone age advancement (R=-0.04, P=0.72), or glucocorticoid treatment duration (R=-0.15, P=0.17) or dose (R=-0.16, P=0.15).ConclusionAHt of females diagnosed with NCCAH in childhood was lower than their THt, irrespective of glucocorticoid treatment. Neither pubertal status at diagnosis nor having CPP or EP was correlated with AHt.

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