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Start Over Author "Abrigo E" Topic adrenal hyperplasia, congenital
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1. Growth Trajectory and Adult Height in Children with Nonclassical Congenital Adrenal Hyperplasia.

Author

Wasniewska MG, Morabito LA, Baronio F, Einaudi S, Salerno M, Bizzarri C, Russo G, Chiarito M, Grandone A, Guazzarotti L, Spinuzza A, Corica D, Ortolano R, Balsamo A, Abrigo E, Baldini Ferroli B, Alibrandi A, Capalbo D, Aversa T, and Faienza MF

Subjects
Adrenal Hyperplasia, Congenital drug therapy, Adrenal Hyperplasia, Congenital pathology, Adult, Child, Child, Preschool, Female, Humans, Hydrocortisone administration & dosage, Male, Retrospective Studies, Adrenal Hyperplasia, Congenital physiopathology, Body Height, Models, Biological
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., (© 2020 S. Karger AG, Basel.)

Published
2020
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2. Growth Trajectory and Adult Height in Children with Nonclassical Congenital Adrenal Hyperplasia

Author

Carla Bizzarri, Rita Ortolano, Silvia Einaudi, Mariangela Chiarito, Tommaso Aversa, Mariacarolina Salerno, Federico Baronio, Laura Guazzarotti, Angela Alibrandi, Maria Felicia Faienza, Domenico Corica, Donatella Capalbo, Barbara Baldini Ferroli, Enrica Abrigo, Malgorzata Wasniewska, Anna Grandone, Antonio Balsamo, Antonietta Spinuzza, Gianni Russo, Letteria Morabito, Wasniewska, M. G., Morabito, L. A., Baronio, F., Einaudi, S., Salerno, M., Bizzarri, C., Russo, G., Chiarito, M., Grandone, A., Guazzarotti, L., Spinuzza, A., Corica, D., Ortolano, R., Balsamo, A., Abrigo, E., Baldini Ferroli, B., Alibrandi, A., Capalbo, D., Aversa, T., and Faienza, M. F.

Subjects
Adult, Male, medicine.medical_specialty, Hydrocortisone, Pediatric endocrinology, Endocrinology, Diabetes and Metabolism, 030209 endocrinology & metabolism, 21-hydroxylase deficiency, Models, Biological, Adult height, 03 medical and health sciences, 0302 clinical medicine, Endocrinology, Internal medicine, medicine, Humans, Congenital adrenal hyperplasia, Child, Retrospective Studies, 030219 obstetrics & reproductive medicine, Adrenal Hyperplasia, Congenital, business.industry, 21-hydroxylase deficiency, Adult height, Growth and puberty, Nonclassical 21-hydroxylase deficiency, Severity of phenotype, Bone age, Stepwise regression, medicine.disease, Body Height, Child, Preschool, Pediatrics, Perinatology and Child Health, Bone maturation, Nonclassical 21-hydroxylase deficiency, Population study, Growth and puberty, Female, business, Severity of phenotype, medicine.drug
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 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.

Published
2020

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