IMPE2023 Poster Presentations Growth and Syndromes (15 abstracts)
Bone age in children with achondroplasia: findings from a cohort of children participating in the PROPEL studies
Ravi Savarirayan 1 , Josep Maria De Bergua 2 , Paul Arundel 3 , Jean Pierre Salles 4 , Antonio Leiva-Gea 5 , Melita Irving 6 , Vrinda Saraff 7 , Helen McDevitt 8 , Maria Salcedo 9 , Marc Nicolino 10 , Valerie Cormier-Daire 11 , Peter Kannu 12 , Mars Skae 13 , Michael B. Bober 14 , John Phillips III 15 , Toby Candler 16 , Paul Harmatz 17 , Howard Saal 18 , Julie Hoover-Fong 19 , Elena Muslimova 20 , Terry Cho 20 , Richard Weng 20 & Daniela Rogoff 20
1Murdoch Children’s Research Institute, Melbourne, Australia. 2Hospital Vithas San José, Vitoria-Gasteiz, Spain. 3Sheffield Children’s NHS Foundation Trust, Sheffield, United Kingdom. 4Hôpital des Enfants – Toulouse, Toulouse, France. 5Hospital Universitario Virgen de la Victoria, Malaga, Spain. 6Guy’s and Saint Thomas’ NHS Foundation Trust, London, United Kingdom. 7Birmingham Women’s and Children’s NHS Foundation Trust, Birmingham, United Kingdom. 8NHS Greater Glasgow and Clyde, Glasgow, United Kingdom. 9Hospital Universitario La Paz, Madrid, Spain. 10Hôpital Femme Mère Enfant, Lyon, France. 11Hôpital Necker-Enfants Malades, Paris, France. 12University of Alberta – Stollery Children’s Hospital, Edmonton, Canada. 13Manchester University NHS Foundation Trust, Manchester, United Kingdom. 14Nemours Children’s Hospital, Wilmington, USA. 15Vanderbilt University Medical Center, Nashville, USA. 16University Hospitals Bristol and Weston NHS Foundation Trust, Bristol, United Kingdom. 17Benioff Children’s Hospital, Oakland, USA. 18Cincinnati Children’s Hospital Medical Center, Cincinnati, USA. 19Johns Hopkins University School of Medicine, Baltimore, USA. 20QED Therapeutics, Inc. (an affiliate of BridgeBio Pharma), San Francisco, USA
Background: Bone age (BA) is commonly used in pediatrics to define skeletal maturity for medical and non-medical purposes. Normal range is represented by 2 standard deviations (SDs) ± the mean. A BA > ±2 SDs from the chronological age (CA) is considered abnormal. BA in achondroplasia (ACH) has not been fully characterized; calculation is challenging given difficulties in comparing x-rays with standard radiographs if using the Greulich-Pyle (G&P) method and complexity of using the Tanner Whitehouse method. Few publications have described delays in BA in children with ACH. One study showed a delay of 1.4 years for males and 1.2 years for females, with a difference between CA and RUS (radius, ulna, short bones) BA of 0.9±1.1 for children <10 years and 1.6±0.9 for those >10 years of age. Another study described a mean delay in BA of 11.6 months for boys and 8.2 months for girls during early childhood. Here we describe BA at baseline in a group of children participating in the phase 2 dose-finding PROPEL 2 study evaluating preliminary safety and efficacy of infigratinib in children with ACH.
Methods: Left-hand and wrist radiographs of 56 children with ACH (age 7.6±2.2 years; female n=34; Tanner stage 1) were evaluated for BA by a single reader using the RUS method (TW2). BA relation to CA was expressed as BA/CA overall and by sex. SD score (SDS) was calculated using SD data from the G&P atlas.
Results: Mean±SD BA was 7.6±2.9 years (no difference vs CA; P=0.9). BA/CA was 0.99±0.28. Mean±SD BA/CA was 1.1±0.29 in females and 0.87±0.22 in males, a statistically significant difference (P=0.005). Nine children (16%; 8 females, 1 male) had a BA >+2 SDs for age and sex, indicating an advanced BA compared with CA. Twelve children (21%; 3 female, 9 male) had a BA that was delayed compared with CA (≥ –2 SDs for age and sex). No correlation between BA and BMI was found overall or by sex.
Conclusion: This analysis did not confirm previous findings suggesting a delay in BA in pre-pubertal children with ACH. BA was more advanced in females than in males in this study, but was within the expected variability for the age group evaluated. This work suggests that BA estimation in children with ACH can be employed for the same purposes as in children without skeletal dysplasia.
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