Faculty Collaboration Database

Growth characteristics in individuals with osteogenesis imperfecta in North America: results from a multicenter study. Genet Med 2019 Feb;21(2):275-283

Date

07/05/2018

Pubmed ID

29970925

Pubmed Central ID

PMC6320321

DOI

10.1038/s41436-018-0045-1

Scopus ID

2-s2.0-85049515167 (requires institutional sign-in at Scopus site)   32 Citations

Abstract

PURPOSE: Osteogenesis imperfecta (OI) predisposes people to recurrent fractures, bone deformities, and short stature. There is a lack of large-scale systematic studies that have investigated growth parameters in OI.

METHODS: Using data from the Linked Clinical Research Centers, we compared height, growth velocity, weight, and body mass index (BMI) in 552 individuals with OI. Height, weight, and BMI were plotted on Centers for Disease Control and Prevention normative curves.

RESULTS: In children, the median z-scores for height in OI types I, III, and IV were -0.66, -6.91, and -2.79, respectively. Growth velocity was diminished in OI types III and IV. The median z-score for weight in children with OI type III was -4.55. The median z-scores for BMI in children with OI types I, III, and IV were 0.10, 0.91, and 0.67, respectively. Generalized linear model analyses demonstrated that the height z-score was positively correlated with the severity of the OI subtype (P < 0.001), age, bisphosphonate use, and rodding (P < 0.05).

CONCLUSION: From the largest cohort of individuals with OI, we provide median values for height, weight, and BMI z-scores that can aid the evaluation of overall growth in the clinic setting. This study is an important first step in the generation of OI-specific growth curves.

Author List

Jain M, Tam A, Shapiro JR, Steiner RD, Smith PA, Bober MB, Hart T, Cuthbertson D, Krischer J, Mullins M, Bellur S, Byers PH, Pepin M, Durigova M, Glorieux FH, Rauch F, Lee B, Sutton VR, , Members of the Brittle Bone Disorders Consortium*,, Nagamani SCS

Author

Gerald Harris PhD Director in the Orthopaedic Research Engineering Center (OREC) department at Marquette University

MESH terms used to index this publication - Major topics in bold

Adolescent
Adult
Body Height
Body Mass Index
Body Weight
Child
Child, Preschool
Diphosphonates
Female
Humans
Male
North America
Osteogenesis Imperfecta
Young Adult