Background: Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency (21-OHD) is reported inadequate growth and impairment of the final height (FH).
Objective: We present the results of near FH in 55 patients with classic 21-OHD followed up for approximately 11.6 years(the longest 25.3 years)in a single institution, and the variables related to NFH.
Method: Patients with classic 21-OHD followed up at our clinic, who had achieved the FH by 2014 were evaluated. FH was characterized by growth velocity less than 0.5 cm/year, and bone age greater than 15 for girls and 17 years for boys. The diagnosis was based on the clinical history, clinical status, and hormonal criteria. We compare the z-score for FH (FHZ) with the standard height for the population, the target height and the hydrocortisone treatment schedule.
Results: 55 patients studied: 43 (78.2%) were female, 18 (32.7%) were salt-wasters, 14 (25.5%) were untreated. Median age at the beginning of follow-up was 4.67 years (ranging from newborn to 28.3 years). Mean follow-up until the FH was 11.6 years. Mean FH (FHZ) of the whole group was (151.88±7.41) cm (−2.0±1.2). Of the 55 patients, 27of them reached FH within the normal population range (≧−2 S.D.). The treated patients were significantly taller than those untreated (FH (FHZ): (153.62±7.35) cm) (−1.9±1.2) vs (146.74±4.91) cm (−2.6±0.9)).When corrected for the genetic potential, 48 of the 54 patients had NFH below their target height, and 15 patients had NFH even below −2 S.D. of the target range. A better height outcome was observed in patients with good compliance, salt-wasters and non-overdose hydrocortisone administration.
Conclusion: We conclude that growth in classic 21-OHD children is below expectation, as compared with both the reference population and the target height. Careful treatment adjustments and good compliance have a major influence on growth of children with classic 21-OHD.
01 - 03 Oct 2015
European Society for Paediatric Endocrinology