Mobility in Osteogenesis Imperfecta: A Multicenter North American Study

Kruger, Karen M.; Caudill, Angela; Celin, Mercedes Rodríguez; Nagamani, Sandesh C.S.; Shapiro, Jay R.; Steiner, Robert D.; Bober, Michael B.; Hart, Tracy; Cuthbertson, David; Krischer, Jeff; Byers, Peter H.; Durigova, Michaela; Glorieux, Francis H.; Rauch, Frank; Sutton, V. Reid; Lee, Brendan; Rush, Eric T.; Smith, Peter A.; Harris, Gerald F.

doi:10.1101/378190

Cited by 4 publications

(11 citation statements)

References 45 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…In agreement with Kruger et al 19 , we found that the OI type was associated with mobility outcomes in the rodded group. Additionally, within the rodded group, a height deficit was associated with reduced mobility outcomes, agreeing with the literature in which height has been related to condition severity 19 , 21 , 22 . Although the use of BMI in populations with short stature is controversial because it may overestimate the proportion of obesity 24 , 37 , many studies found a high prevalence of elevated BMI, especially in individuals with OI Type III 33 , 38 .…”

Section: Discussionsupporting

confidence: 93%

Section: Discussionmentioning

confidence: 99%

“…Functional outcomes and quality of life have been reported to be related to condition severity 19 , 34 - 36 . In agreement with Kruger et al 19 , we found that the OI type was associated with mobility outcomes in the rodded group. Additionally, within the rodded group, a height deficit was associated with reduced mobility outcomes, agreeing with the literature in which height has been related to condition severity 19 , 21 , 22 .…”

Section: Discussionmentioning

confidence: 99%

“…Engelbert et al reported intramedullary rodding to be associated with greater OI severity, specifically interference with walking ability 18 . We previously reported that the phenotypic severity of OI has a significant influence on mobility outcomes 19 . Additionally, some studies have described the benefits of rodding with respect to mobility 14 , 20 .…”

mentioning

confidence: 99%

“…Additionally, some studies have described the benefits of rodding with respect to mobility 14 , 20 . Anthropometric measurements have also been related to the severity of the condition and mobility outcomes 19 , 21 , 22 .…”

mentioning

confidence: 99%

See 4 more Smart Citations

A Multicenter Study of Intramedullary Rodding in Osteogenesis Imperfecta

et al. 2020

Self Cite

View full text Add to dashboard Cite

Background: Osteogenesis imperfecta (OI), a heritable connective tissue disorder with wide clinical variability, predisposes to recurrent fractures and bone deformity. Management requires a multidisciplinary approach in which intramedullary rodding plays an important role, especially for moderate and severe forms. We investigated the patterns of surgical procedures in OI in order to establish the benefits of rodding. The main hypothesis that guided this study was that rodded participants with moderate and severe OI would have lower fracture rates and better mobility. Methods: With data from the Linked Clinical Research Centers, we analyzed rodding status in 558 individuals. Mobility and fracture data in OI Types III and IV were compared between rodded and non-rodded groups. Univariate regression analyses were used to test the association of mobility outcomes with various covariates pertinent to rodding. Results: Of the individuals with OI, 42.1% had undergone rodding (10.7% of those with Type I, 66.4% with Type III, and 67.3% with Type IV). Rodding was performed more frequently and at a younger age in femora compared with tibiae. Expanding intramedullary rods were used more frequently in femora. In Type III, the rate of fractures per year was significantly lower (p ≤ 0.05) for rodded bones. In Type III, the mean scores on the Gillette Functional Assessment Questionnaire (GFAQ) and Brief Assessment of Motor Function (BAMF) were higher in the rodded group. However, Type-IV non-rodded subjects had higher mean scores in nearly all mobility outcomes. OI type, the use of expanding rods in tibiae, and anthropometric measurements were associated with mobility outcomes scores. Conclusions: Current practice in 5 orthopaedic centers with extensive experience treating OI demonstrates that most individuals with moderate and severe types of OI undergo rodding procedures. Individuals with severe OI have improved mobility outcomes and lower fracture rates compared with their non-rodded peers, which suggests that early bilateral rodding benefits OI Type III. Our analysis showed a change in practice patterns in the final years of the study in the severe forms, with earlier and more simultaneous rodding procedures performed. Level of Evidence: Therapeutic Level III . See Instructions for Authors for a complete description of levels of evidence.

show abstract

Section: Discussionsupporting

confidence: 93%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

mentioning

confidence: 99%

See 3 more Smart Citations

A Multicenter Study of Intramedullary Rodding in Osteogenesis Imperfecta

et al. 2020

Self Cite

View full text Add to dashboard Cite

show abstract

A multicenter study to evaluate pain characteristics in osteogenesis imperfecta

Celin

Kruger

Caudill

et al. 2022

American J of Med Genetics Pt A

View full text Add to dashboard Cite

The objective was to describe pain characteristics and treatments used in individuals with varying severity of osteogenesis imperfecta (OI) and investigate pain‐associated variables. This work was derived from a multicenter, longitudinal, observational, natural history study of OI conducted at 12 clinical sites of the NIH Rare Diseases Clinical Research Network's Brittle Bone Disorders Consortium. Children and adults with a clinical, biochemical, or molecular diagnosis of OI were enrolled in the study. We did a cross‐sectional analysis of chronic pain prevalence, characteristics, and treatments used for pain relief and longitudinal analysis to find the predictors of chronic pain. We included 861 individuals with OI, in 41.8% chronic pain was present, with similar frequency across OI types. Back pain was the most frequent location. Nonsteroidal anti‐inflammatory drugs followed by bisphosphonates were the most common treatment used. Participants with chronic pain missed more days from school or work/year and performed worse in all mobility metrics than participants without chronic pain. The variables more significantly associated with chronic pain were age, sex, positive history of rodding surgery, scoliosis, other medical problems, assistive devices, lower standardized height, and higher body mass index. The predictors of chronic pain for all OI types were age, use of a wheelchair, and the number of fractures/year. Chronic pain is prevalent in OI across all OI types, affects mobility, and interferes with participation. Multiple covariates were associated with chronic pain.

show abstract

Prognostic factors for mobility in children with osteogenesis imperfecta

Sawamura¹,

Kitoh

Kaneko³

et al. 2022

Medicine

View full text Add to dashboard Cite

Osteogenesis imperfecta (OI) is a connective tissue disorder characterized by bone fragility. Although the disease severity is known to influence the ability to walk, little is known about how children with severe OI can achieve practical ambulation (PA). This study aimed to determine the early predictors of future mobility in children with OI. Thirty OI patients with an average age of 12.1 years were classified into the PA group (22 patients) and nonambulator group (NA group: 8 patients) on the basis of the Hoffer classification. Various clinical parameters related to mobility were compared between the PA and NA groups. Therapeutic interventions were also compared between the 2 groups. The mean age at diagnosis and initial fracture were significantly lower in the NA group than in the PA group. The height was significantly smaller in the NA group than in the PA group at all ages examined (at birth, 3 years, and 6 years). The number of patients with respiratory failure was significantly higher in the NA group than in the PA group. The age at initial corrective osteotomy of the lower extremities in the PA group was significantly lower than that in the NA group, although there was no significant difference in the disease severity in infancy between the groups. Height during infancy, age at initial fracture, and neonatal respiratory status could be prognostic factors for mobility in OI. Surgical interventions at an early age may influence walking ability in children with moderate OI.

show abstract

Mobility in Osteogenesis Imperfecta: A Multicenter North American Study

Cited by 4 publications

References 45 publications

A Multicenter Study of Intramedullary Rodding in Osteogenesis Imperfecta

A Multicenter Study of Intramedullary Rodding in Osteogenesis Imperfecta

A multicenter study to evaluate pain characteristics in osteogenesis imperfecta

Prognostic factors for mobility in children with osteogenesis imperfecta

Contact Info

Product

Resources

About