Abstract
Background:
In congenital adrenal hyperplasia (CAH), adjusting hydrocortisone dose during childhood avoids reduced adult height. However, there are currently no CAH-specific charts to monitor growth during treatment. Our objective was to elaborate growth reference charts and bone maturation data for CAH patients.
Methods:
We conducted a retrospective observational cohort study, in 34 French CAH centers. Patients were 496 children born 1970–1991 with genetically proven 21-hydroxylase deficiency. Their growth and bone maturation data were collected until age 18 together with adult height, puberty onset, parental height, and treatment. The mean (SD) heights were modeled from birth to adulthood. The median±1 SD and ±2 SDs model-generated curves were compared with the French references. A linear model for bone maturation and a logistic regression model for the probability of short adult height were built.
Results:
Growth charts were built by sex for salt wasting (SW) and simple virilizing (SV) children treated before 1 year of age. In girls and boys, growth was close to that of the general French population up to puberty onset. There was almost no pubertal spurt and the mean adult height was shorter than that of the general population in girls (−1.2 SD, 156.7 cm) and boys (−1.0 SD, 168.8 cm). Advanced bone age at 8 years had a strong impact on the risk of short adult height (OR: 4.5 per year advance).
Conclusions:
The 8-year bone age is a strong predictor of adult height. It will help monitoring the growth of CAH-affected children.
Acknowledgments
We express our gratitude to all patients and their parents as well as to all investigators involved in the Opale Study. The authors thank Jean Iwaz (Hospices Civils de Lyon) for the thorough editing of the final versions of the manuscript.
Author contributions: All the authors have accepted responsibility for the entire content of this submitted manuscript and approved submission. Contributors: Clinical Investigation Center: E. Brocard, S. Gaillard, C. Cornu, B. Kassaï, A. Delahaye, C. Desprez, M. Pelosse, C. Eloy. Biostatistics: L. Remontet, B. Riche, P. Roy. The Collaborative CAH Growth Study Group: Régis Coutant, Anne-Marie Bertrand, Chantal Metz, Guy-André Loeuille, Philippe Garnier, Anne Lienhardt-Roussie, Gilbert Simonin, Rachel Reynaud, Charles Sultan, Claire Jeandel, Bruno Leheup, Kathy Wagner, Paul Czernichow, Juliane Leger-Metoudi, Odile Richard, Sylvie Soskin, Maité Tauber, Catherine Pienkowski, Michel Polak, Graziella Pinto, Claire Nihoul-Fekete, Yves Le Bouc, Sylvie Cabrol, Marie-Charles Raux-Demay, Hélène Carla, Marc De Kerdanet, Candace Ben Signore, François Despert, Eric Mallet, Claudine Lecointre, Véronique Sulmont, Pierre-François Souchon, Hubert Ythier, Paola Adiceam, Pierre-François Bougneres, Claire Bouvattier, Sabine Baron, Pascal Barat, Michel Colle, Elisabeth Baechler, Raja Brauner, Jacques Weill, Maryse Gatigny, Marc Jullien.
Research funding: The study was funded by the Scientific Council of “Association Surrénales” and by Hospices Civils de Lyon (“Jeune chercheur” and interrégional PHRC calls).
Employment or leadership: None declared.
Honorarium: None declared.
Competing interests: The funding organization(s) played no role in the study design; in the collection, analysis, and interpretation of data; in the writing of the report; or in the decision to submit the report for publication.
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