Growth patterns and final height in congenital adrenal hyperplasia due to classical 21-hydroxylase deficiency results of a multicenter study

Gábor Hargitai, J. Sólyom, Tadej Battelino, Jan Lebl, Zuzanna Pribilincová, Roland Hauspie, József Kovács, Franz Waldhauser, Herwig Frisch

Research output: Contribution to journalArticle

56 Citations (Scopus)

Abstract

Background: Longitudinal growth and bone age (BA) development are the most important clinical parameters for monitoring adequate glucocorticoid replacement in children with congenital adrenal hyperplasia (CAH). Aim of the Study: To analyze the growth pattern of patients treated for CAH of the salt wasting (SW) and simple virilizing (SV) clinical forms; to evaluate final height as compared to reference data and individual target height; to evaluate the course of BA development. Patients and Methods: A large database of 598 patients with CAH was created in 5 Central European countries and growth data of 341 treated patients with 21-hydroxylase deficiency were analyzed retrospectively. The patients were of Caucasian origin. Centiles were constructed in a cross-sectional manner and an additional longitudinal analysis was performed in order to evaluate the pubertal growth spurt by applying particular statistical methods (Preece-Baines model). Results: The growth of SW CAH patients was impaired in infancy and early childhood (0-3 years of age), but followed normal patterns in childhood until puberty. In contrast, children with SV CAH had normal patterns of growth in infancy and early childhood and were considerably taller than healthy references during childhood. In the longitudinal study, peak height velocity in both boys and girls was normal, but it occurred at an earlier age than in the standard population. The final height of patients with CAH was reduced in comparison to both the reference and the individual target height. No correlations were found between final height and age at the start of the therapy in SV patients or between final height and year of birth. BA was advanced in both types of CAH, but more accelerated in SV patients. Conclusion: Characteristic growth patterns for treated SV and SW CAH children were identified, with a normal pubertal growth spurt and reduced final height being observed.

Original languageEnglish
Pages (from-to)161-171
Number of pages11
JournalHormone Research
Volume55
Issue number4
DOIs
Publication statusPublished - 2001

Fingerprint

Congenital Adrenal Hyperplasia
Multicenter Studies
Growth
Salts
Bone Development
Congenital adrenal hyperplasia due to 21 hydroxylase deficiency
Puberty
Glucocorticoids
Longitudinal Studies
Parturition
Databases
Bone and Bones

Keywords

  • Body height percentiles
  • Congenital adrenal hyperplasia
  • Final height
  • Growth velocity
  • Pubertal growth spurt

ASJC Scopus subject areas

  • Endocrinology

Cite this

Growth patterns and final height in congenital adrenal hyperplasia due to classical 21-hydroxylase deficiency results of a multicenter study. / Hargitai, Gábor; Sólyom, J.; Battelino, Tadej; Lebl, Jan; Pribilincová, Zuzanna; Hauspie, Roland; Kovács, József; Waldhauser, Franz; Frisch, Herwig.

In: Hormone Research, Vol. 55, No. 4, 2001, p. 161-171.

Research output: Contribution to journalArticle

Hargitai, G, Sólyom, J, Battelino, T, Lebl, J, Pribilincová, Z, Hauspie, R, Kovács, J, Waldhauser, F & Frisch, H 2001, 'Growth patterns and final height in congenital adrenal hyperplasia due to classical 21-hydroxylase deficiency results of a multicenter study', Hormone Research, vol. 55, no. 4, pp. 161-171. https://doi.org/10.1159/000049990
Hargitai, Gábor ; Sólyom, J. ; Battelino, Tadej ; Lebl, Jan ; Pribilincová, Zuzanna ; Hauspie, Roland ; Kovács, József ; Waldhauser, Franz ; Frisch, Herwig. / Growth patterns and final height in congenital adrenal hyperplasia due to classical 21-hydroxylase deficiency results of a multicenter study. In: Hormone Research. 2001 ; Vol. 55, No. 4. pp. 161-171.
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AU - Hargitai, Gábor

AU - Sólyom, J.

AU - Battelino, Tadej

AU - Lebl, Jan

AU - Pribilincová, Zuzanna

AU - Hauspie, Roland

AU - Kovács, József

AU - Waldhauser, Franz

AU - Frisch, Herwig

PY - 2001

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N2 - Background: Longitudinal growth and bone age (BA) development are the most important clinical parameters for monitoring adequate glucocorticoid replacement in children with congenital adrenal hyperplasia (CAH). Aim of the Study: To analyze the growth pattern of patients treated for CAH of the salt wasting (SW) and simple virilizing (SV) clinical forms; to evaluate final height as compared to reference data and individual target height; to evaluate the course of BA development. Patients and Methods: A large database of 598 patients with CAH was created in 5 Central European countries and growth data of 341 treated patients with 21-hydroxylase deficiency were analyzed retrospectively. The patients were of Caucasian origin. Centiles were constructed in a cross-sectional manner and an additional longitudinal analysis was performed in order to evaluate the pubertal growth spurt by applying particular statistical methods (Preece-Baines model). Results: The growth of SW CAH patients was impaired in infancy and early childhood (0-3 years of age), but followed normal patterns in childhood until puberty. In contrast, children with SV CAH had normal patterns of growth in infancy and early childhood and were considerably taller than healthy references during childhood. In the longitudinal study, peak height velocity in both boys and girls was normal, but it occurred at an earlier age than in the standard population. The final height of patients with CAH was reduced in comparison to both the reference and the individual target height. No correlations were found between final height and age at the start of the therapy in SV patients or between final height and year of birth. BA was advanced in both types of CAH, but more accelerated in SV patients. Conclusion: Characteristic growth patterns for treated SV and SW CAH children were identified, with a normal pubertal growth spurt and reduced final height being observed.

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