Lower limb weakness may contribute to limitations in mobility in people with OI Type IV despite multidisciplinary treatment.
![Understanding the Functional Consequences of Osteogenesis Imperfecta Type IV Understanding the Functional Consequences of Osteogenesis Imperfecta Type IV](https://images.medindia.net/health-images/1200_1000/bone6.jpg)
‘The characteristic features of osteogenesis imperfecta vary greatly from person to person, even among people with the same type of OI, and even within the same family.’
![pinterest](https://images.medindia.net/icons/news/social/pinterest.png)
To date, muscle function, and in particular that of the lower extremity, in OI type IV has not been investigated systematically. A new study in the journal ’Calcified Tissue international’ has now assessed upper and lower extremity muscle function in 27 children and adolescents aged 7-21 years with OI type IV, compared with age-and sex-matched controls. The researchers found that the patients, all with a clinical diagnosis of OI type IV and known mutations in the COLIA1 and COLIA2 genes, had: ![twitter](https://images.medindia.net/icons/news/social/twitter.png)
![facebook](https://images.medindia.net/icons/news/social/facebook.png)
![whatsapp](https://images.medindia.net/icons/news/social/whatsapp.png)
![linkedin](https://images.medindia.net/icons/news/social/linkedin.png)
![pinterest](https://images.medindia.net/icons/news/social/pinterest.png)
Approximately 30% lower-limb peak force and 50% peak power deficits compared to healthy age- and sex-matched controls.
At the lower limb level, a 50% lower peak power than age- and sex- matched patients with OI type 1 (the type of OI with the mildest form of bone fragility).
Normal upper-limb muscle force, but a muscle function deficit at the lower-limb level.
Among the potential determinants of lower-limb muscle function in OI type IV, only fracture history emerged as a significant negative predictor. The number of fractures is likely to explain periods of inactivity which contributes to lower muscle function. Muscle function may also be impaired by a direct effect of collagen type 1 mutations on tendons, ligaments and intramuscular connective tissue, although more studies are needed to clarify this aspect.
Advertisement
Source-Eurekalert