ESPEYB19 5. Bone, Growth Plate and Mineral Metabolism Advances in clinical practice (5 abstracts)
5.8. Growth in achondroplasia including stature, weight, weight-for-height and head circumference from CLARITY: achondroplasia natural history study-a multi-center retrospective cohort study of achondroplasia in the US
Hoover-Fong JE , Schulze KJ , Alade AY , Bober MB , Gough E , Hashmi SS , Hecht JT , Legare JM , Little ME , Modaff P , Pauli RM , Rodriguez-Buritica DF , Serna ME , Smid C , Liu C & McGready J
Orphanet J Rare Dis. 2021 Dec 23;16(1):522.Abstract: https://pubmed-ncbi-nlm-nih-gov/34949201/
In Brief: Based on over 37,000 length/height, weight and head circumference measures from 1374 patients with achondroplasia in the USA, updated length/height-for-age, weight-for-age, head circumference-for-age (HC) and weight-for-height curves were generated for children up to 18 years of age (HC up to 5 years of age).
Commentary: Growth charts for achondroplasia have been available. However, those were derived from a mix of longitudinal, cross-sectional, retrospective and/or prospectively collected data from populations ranging in size from 23 to 466 subjects, collected as long ago as 1967 and as recently as 2019.
The Achondroplasia natural history study (CLARITY) collected all available retrospective anthropometry data including length/height, weight and head circumference from achondroplasia patients from 4 US skeletal dysplasia centres (Johns Hopkins University, AI DuPont Hospital for Children, McGovern Medical School University of Texas Health, University of Wisconsin School of Medicine and Public Health). Weight-for-age values beyond 3 SD above the mean were excluded from the weight-for-height and weight-for-age curves to create a stricter tool for weight assessment in this population. Over 37,000 length/height, weight and head circumference measures from 1374 patients with achondroplasia from birth through 75 years of age were compiled. Stature and weight data from birth through 18 years of age and head circumference from birth through 5 years of age were used to construct new length/height-for-age, weight-for-age, head circumference-for-age and weight-for-height curves. The cross-sectional presentation of height-for-age data did not demonstrate a substantial pubertal growth spurt in achondroplasia. Head circumference data indicated no difference from all available data and those derived from data excluding those undergoing base of skull decompression and/or ventricular shunting. This study also explored the possibility of secular trends in weight in children by age group and decade of birth and utilized weight-for-age data to screen for extremes in weight that were excluded from their novel, prescriptive weight-for-age and weight-for-length/height curves.
These curves could be utilized in the clinic and research venues to ascertain deviation from normal cranial growth which should prompt further investigation. These data will also enable to identify children with achondroplasia who carry excess weight for their stature. However, the data are from the USA only and even though all ethnic groups were included, similar country-specific studies will be required to ascertain genetic and environmental factors contributing to growth in achondroplasia patients.
Volume 19
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ESPE Yearbook of Paediatric Endocrinology
ISSN 1662-4009 (Online)
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