Growth Trajectory and Adult Height in Children with Nonclassical Congenital Adrenal Hyperplasia.

Abstract

BACKGROUND:

Children with nonclassical congenital adrenal hyperplasia (NCCAH) often present increased growth velocity secondary to elevation of adrenal androgens that accelerates bone maturation and might compromise adult height (AH).

OBJECTIVE:

The aim of the study was to analyze prognostic factors affecting growth trajectory (GT) and AH in children with NCCAH.

METHODS:

The study was a retrospective, multicentric study. The study population consisted of 192 children with a confirmed molecular diagnosis of NCCAH, followed by pediatric endocrinology centers from diagnosis up to AH. Clinical records were collected and analyzed. AH (standard deviation score; SDS), pubertal growth (PG) (cm), GT from diagnosis to AH (SDS), and AH adjusted to target height (TH) (AH-TH SDS) were evaluated as outcome indicators using stepwise linear regression models.

RESULTS:

The stepwise linear regression analysis showed that AH and AH-TH were significantly related to chronological age (CA) (p = 0.008 and 0.016), bone age (BA)/CA ratio (p = 0.004 and 0.001), height (H) (p < 0.001 for both parameters) at NCCAH diagnosis, and TH (p = 0.013 and <0.001). PG was higher in males than in females (22.59 ± 5.74 vs. 20.72 ± 17.4 cm, p = 0.002), as physiologically observed, and was positively related to height (p = 0.027), negatively to BMI (p = 0.001) and BA/CA ratio (p = 0.001) at NCCAH diagnosis. Gender, genotype, biochemical data, and hydrocortisone treatment did not significantly impair height outcomes of these NCCAH children.

CONCLUSIONS:

The results of this study suggest that AH and GT of NCCAH patients are mainly affected by the severity of phenotype (CA, BA/CA ratio, and H) at the time of diagnosis.

• Authors: Malgorzata Gabriela Wasniewska, Letteria Anna Morabito, Federico Baronio, Silvia Einaudi, Mariacarolina Salerno, Carla Bizzarri, Gianni Russo, Mariangela Chiarito, Anna Grandone, Laura Guazzarotti, Antonietta Spinuzza, Domenico Corica, Rita Ortolano, Antonio Balsamo, Enrica Abrigo, Barbara Baldini Ferroli, Angela Alibrandi, Donatella Capalbo, Tommaso Aversa, Maria Felicia Faienza, Adrenal Diseases Working Group of the Italian Society for Pediatric Endocrinology and Diabetology
• Journal: Hormone research in paediatrics (Horm Res Paediatr) Vol. 93 Issue 3 Pg. 173-181 ( 2020) ISSN: 1663-2826 [Electronic] Switzerland
• PMID: 32810858 (Publication Type: Clinical Trial, Journal Article, Multicenter Study)
• Copyright: © 2020 S. Karger AG, Basel.

Chemical References

• Hydrocortisone

Topics

• Adrenal Hyperplasia, Congenital (drug therapy, pathology, physiopathology)
• Adult
• Body Height
• Child
• Child, Preschool
• Female
• Humans
• Hydrocortisone (administration & dosage)
• Male
• Models, Biological
• Retrospective Studies