Background: Congenital adrenal hyperplasia (CAH), mostly caused by 21-hydroxylase deficiency, is autosomal recessive disorder characterized by impaired cortisol synthesis. It can be presented with a combination of aldosterone and cortisol deficiency and androgen excess. Therefore, excess production of androgen and glucocorticoid replacement can result to early bone maturation and ultimately diminished adult height (AH).
Objectives: The purpose of this study was to obtain objective data on AH with classic CAH patients and analyze the affecting factors on AH. Also we evaluated growth pattern during age increase.
Study Design: We retrospectively reviewed the longitudinal medical records of 40 children with classic CAH (male [n=19]: 9 salt-wasting (SW), 10 simple-virilizing (SV); female [n=21]: 8 SW, 13 SV) who reached AH at Pediatric endocrinology clinic of Severance hospital from 1977 to 2015. We also analyzed the affecting factors on AH, and assessed growth patterns with serial height standard deviation score (SDS) dividing into following stages of growth: early childhood (0-4.99 years), mid-childhood (5-9.99 years), and adolescence (10-15 years)
Results: AH (162.7 ± 9.72 cm) was significantly shorter than the midparental height (MPH) (172.5 ± 3.40 cm) in male patients (P <0.001), and similarly, AH (154.5 ± 6.45 cm) was significantly shorter than the MPH (158.7 ± 2.96) in female patients (P=0.002). Accordingly, the AH SDS was meaningfully lower than the MPH SDS in both sex (males: P <0.001, females: P =0.002). Considering subtypes, SV had tendency to attain shorter AH than SW. In addition, the affecting factors on AH were analyzed that they were not significantly associated with subtype, age at diagnosis, dose of steroid, except MPH. Height SDS for chronologic age showed gradual decrement during childhood to adolescence (males: 0.5 ± 2.51 at early childhood, 0.8 ± 2.26 at mid-childhood, 0.2 ± 1.62 at adolescence; females: -0.4 ±1.40, -0.2 ± 2.01, -0.3 ± 1.42 at those same periods). The final AH SDS was -1.6 ± 1.98 in males and -0.81 ± 1.45 in females.
Conclusion: Reduced AH was observed in children with classic CAH compared with their given parental height, regardless of sex and subtype. Furthermore, the height SDS tended to decrease in accordance with age increase, so this finding suggests that proper intervention about growth assessment is needed in children with CAH.
19 - 21 Sep 2019
European Society for Paediatric Endocrinology