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Characteristics of Growth in Children With Classic Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency During Adrenarche and Beyond

CONTEXT: Patients with classic congenital adrenal hyperplasia (CAH) often do not achieve their full growth potential. Adrenarche may accelerate bone maturation and thereby result in decreased growth in CAH. OBJECTIVE: The study aimed to analyze the impact of growth during adrenarche on final height...

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Autores principales: Troger, Tobias, Sommer, Grit, Lang-Muritano, Mariarosaria, Konrad, Daniel, Kuhlmann, Beatrice, Zumsteg, Urs, Flück, Christa E
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8764343/
https://www.ncbi.nlm.nih.gov/pubmed/34599587
http://dx.doi.org/10.1210/clinem/dgab701
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author Troger, Tobias
Sommer, Grit
Lang-Muritano, Mariarosaria
Konrad, Daniel
Kuhlmann, Beatrice
Zumsteg, Urs
Flück, Christa E
author_facet Troger, Tobias
Sommer, Grit
Lang-Muritano, Mariarosaria
Konrad, Daniel
Kuhlmann, Beatrice
Zumsteg, Urs
Flück, Christa E
author_sort Troger, Tobias
collection PubMed
description CONTEXT: Patients with classic congenital adrenal hyperplasia (CAH) often do not achieve their full growth potential. Adrenarche may accelerate bone maturation and thereby result in decreased growth in CAH. OBJECTIVE: The study aimed to analyze the impact of growth during adrenarche on final height of adequately treated classic CAH patients. METHODS: This retrospective, multicenter study (4 academic pediatric endocrinology centers) included 41 patients with classical CAH, born 1990-2012. We assessed skeletal maturation (bone age), growth velocity, and (projected) adult height outcomes, and analyzed potential influencing factors, such as sex, genotype, and glucocorticoid therapy. RESULTS: Patients with classic CAH were shorter than peers (−0.4 SDS ± 0.8 SD) and their parents (corrected final height −0.6 SDS ± 1.0 SD). Analysis of growth during adrenarche revealed 2 different growth patterns: patients with accelerating bone age (49%), and patients with nonaccelerating bone age relative to chronological age (BA-CA). Patients with accelerating BA-CA were taller than the normal population during adrenarche years (P = 0.001) and were predicted to achieve lower adult height SDS (−0.9 SDS [95% CI, −1.3; −0.5]) than nonaccelerating patients when assessed during adrenarche (0.2 SDS [95% CI, −0.3; 0.8]). Final adult height was similarly reduced in both accelerating and nonaccelerating BA-CA groups (−0.4 SDS [95% CI, −0.9; 0.1] vs −0.3 SDS [95% CI, [−0.8; 0.1]). CONCLUSION: Patients with and without significant bone age advancement, and thus differing height prediction during adrenarche, showed similar (predicted) final height when reassessed during pubertal years. Bone age alone should not be used during adrenarche as clinical marker for metabolic control in CAH treatment.
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spelling pubmed-87643432022-01-19 Characteristics of Growth in Children With Classic Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency During Adrenarche and Beyond Troger, Tobias Sommer, Grit Lang-Muritano, Mariarosaria Konrad, Daniel Kuhlmann, Beatrice Zumsteg, Urs Flück, Christa E J Clin Endocrinol Metab Online Only Articles CONTEXT: Patients with classic congenital adrenal hyperplasia (CAH) often do not achieve their full growth potential. Adrenarche may accelerate bone maturation and thereby result in decreased growth in CAH. OBJECTIVE: The study aimed to analyze the impact of growth during adrenarche on final height of adequately treated classic CAH patients. METHODS: This retrospective, multicenter study (4 academic pediatric endocrinology centers) included 41 patients with classical CAH, born 1990-2012. We assessed skeletal maturation (bone age), growth velocity, and (projected) adult height outcomes, and analyzed potential influencing factors, such as sex, genotype, and glucocorticoid therapy. RESULTS: Patients with classic CAH were shorter than peers (−0.4 SDS ± 0.8 SD) and their parents (corrected final height −0.6 SDS ± 1.0 SD). Analysis of growth during adrenarche revealed 2 different growth patterns: patients with accelerating bone age (49%), and patients with nonaccelerating bone age relative to chronological age (BA-CA). Patients with accelerating BA-CA were taller than the normal population during adrenarche years (P = 0.001) and were predicted to achieve lower adult height SDS (−0.9 SDS [95% CI, −1.3; −0.5]) than nonaccelerating patients when assessed during adrenarche (0.2 SDS [95% CI, −0.3; 0.8]). Final adult height was similarly reduced in both accelerating and nonaccelerating BA-CA groups (−0.4 SDS [95% CI, −0.9; 0.1] vs −0.3 SDS [95% CI, [−0.8; 0.1]). CONCLUSION: Patients with and without significant bone age advancement, and thus differing height prediction during adrenarche, showed similar (predicted) final height when reassessed during pubertal years. Bone age alone should not be used during adrenarche as clinical marker for metabolic control in CAH treatment. Oxford University Press 2021-10-02 /pmc/articles/PMC8764343/ /pubmed/34599587 http://dx.doi.org/10.1210/clinem/dgab701 Text en © The Author(s) 2021. Published by Oxford University Press on behalf of the Endocrine Society. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an Open Access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs licence (https://creativecommons.org/licenses/by-nc-nd/4.0/), which permits non-commercial reproduction and distribution of the work, in any medium, provided the original work is not altered or transformed in any way, and that the work is properly cited. For commercial re-use, please contact journals.permissions@oup.com
spellingShingle Online Only Articles
Troger, Tobias
Sommer, Grit
Lang-Muritano, Mariarosaria
Konrad, Daniel
Kuhlmann, Beatrice
Zumsteg, Urs
Flück, Christa E
Characteristics of Growth in Children With Classic Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency During Adrenarche and Beyond
title Characteristics of Growth in Children With Classic Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency During Adrenarche and Beyond
title_full Characteristics of Growth in Children With Classic Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency During Adrenarche and Beyond
title_fullStr Characteristics of Growth in Children With Classic Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency During Adrenarche and Beyond
title_full_unstemmed Characteristics of Growth in Children With Classic Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency During Adrenarche and Beyond
title_short Characteristics of Growth in Children With Classic Congenital Adrenal Hyperplasia due to 21-Hydroxylase Deficiency During Adrenarche and Beyond
title_sort characteristics of growth in children with classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency during adrenarche and beyond
topic Online Only Articles
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8764343/
https://www.ncbi.nlm.nih.gov/pubmed/34599587
http://dx.doi.org/10.1210/clinem/dgab701
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