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Association between joint hypermobility, scoliosis, and cranial base anomalies in paediatric Osteogenesis imperfecta patients: a retrospective cross-sectional study.

Arponen H, Mäkitie O, Waltimo-Sirén J - BMC Musculoskelet Disord (2014)

Bottom Line: Joint hypermobility was found in 70% of the patients.The presence of spinal complications was independent of the bisphosphonate treatment status and joint hypermobility.Scoliosis and craniovertebral anomalies are strongly associated in paediatric OI patients.

View Article: PubMed Central - PubMed

Affiliation: Department of Orthodontics, Institute of Dentistry, University of Helsinki, PO Box 41, FI-00014 Helsinki, Finland. heidi.arponen@fimnet.fi.

ABSTRACT

Background: Joint hypermobility is a common clinical characteristic of patients with Osteogenesis imperfecta (OI), a disorder with serious comorbidities of scoliosis and cranial base anomalies. This study aimed at evaluating how prevalent joint hypermobility is in paediatric OI patients, and to find out whether it serves as a potential predictive marker of the different spinal complications; scoliosis and craniovertebral anomalies (basilar impression and basilar invagination).

Methods: In this cross-sectional one-center study we analysed retrospectively clinical patient records and radiographs of 47 OI patients, aged 1-19 years, some of whom were treated with bisphosphonates. Presence of joint hypermobility, scoliosis, and craniovertebral anomalies was recorded and possible connections between the phenomena were explored with correlation analysis.

Results: Joint hypermobility was found in 70% of the patients. Scoliosis and cranial base anomalies had developed in 26%. The presence of spinal complications was independent of the bisphosphonate treatment status and joint hypermobility.

Conclusions: Scoliosis and craniovertebral anomalies are strongly associated in paediatric OI patients. Joint hypermobility that is much more common appears, however, to be a poor predictor.

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Related in: MedlinePlus

Data on the bisphosphonate-treated patients (n = 30); green color indicates mild OI type, purple color moderate type, and blue color severe OI type.
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Fig1: Data on the bisphosphonate-treated patients (n = 30); green color indicates mild OI type, purple color moderate type, and blue color severe OI type.

Mentions: Of the 47 patients, 30 (64%) had been treated with bisphosphonates. Most of the patients were treated with intravenous bisphosphonates (pamidronate and zoledronic acid) and only 2 patients received an oral preparation (risedronate). The average treatment time was 4.3 years (range 0.2 – 10.8 years) prior to cranial imaging. Characteristics of the patients treated with bisphosphonates are depicted in Figure 1. In this subgroup of patients, the results of the phi coefficient analysis indicated similarly no association between joint hypermobility and scoliosis (Phi = -0.04, p = 0.81, n = 30), or joint hypermobility and cranial anomalies (Phi = -0.005, p = 0.98). A significant positive association was again detected between scoliosis and the presence of a cranial anomaly (Phi = 0.92, p = 0.00).Table 1


Association between joint hypermobility, scoliosis, and cranial base anomalies in paediatric Osteogenesis imperfecta patients: a retrospective cross-sectional study.

Arponen H, Mäkitie O, Waltimo-Sirén J - BMC Musculoskelet Disord (2014)

Data on the bisphosphonate-treated patients (n = 30); green color indicates mild OI type, purple color moderate type, and blue color severe OI type.
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4300610&req=5

Fig1: Data on the bisphosphonate-treated patients (n = 30); green color indicates mild OI type, purple color moderate type, and blue color severe OI type.
Mentions: Of the 47 patients, 30 (64%) had been treated with bisphosphonates. Most of the patients were treated with intravenous bisphosphonates (pamidronate and zoledronic acid) and only 2 patients received an oral preparation (risedronate). The average treatment time was 4.3 years (range 0.2 – 10.8 years) prior to cranial imaging. Characteristics of the patients treated with bisphosphonates are depicted in Figure 1. In this subgroup of patients, the results of the phi coefficient analysis indicated similarly no association between joint hypermobility and scoliosis (Phi = -0.04, p = 0.81, n = 30), or joint hypermobility and cranial anomalies (Phi = -0.005, p = 0.98). A significant positive association was again detected between scoliosis and the presence of a cranial anomaly (Phi = 0.92, p = 0.00).Table 1

Bottom Line: Joint hypermobility was found in 70% of the patients.The presence of spinal complications was independent of the bisphosphonate treatment status and joint hypermobility.Scoliosis and craniovertebral anomalies are strongly associated in paediatric OI patients.

View Article: PubMed Central - PubMed

Affiliation: Department of Orthodontics, Institute of Dentistry, University of Helsinki, PO Box 41, FI-00014 Helsinki, Finland. heidi.arponen@fimnet.fi.

ABSTRACT

Background: Joint hypermobility is a common clinical characteristic of patients with Osteogenesis imperfecta (OI), a disorder with serious comorbidities of scoliosis and cranial base anomalies. This study aimed at evaluating how prevalent joint hypermobility is in paediatric OI patients, and to find out whether it serves as a potential predictive marker of the different spinal complications; scoliosis and craniovertebral anomalies (basilar impression and basilar invagination).

Methods: In this cross-sectional one-center study we analysed retrospectively clinical patient records and radiographs of 47 OI patients, aged 1-19 years, some of whom were treated with bisphosphonates. Presence of joint hypermobility, scoliosis, and craniovertebral anomalies was recorded and possible connections between the phenomena were explored with correlation analysis.

Results: Joint hypermobility was found in 70% of the patients. Scoliosis and cranial base anomalies had developed in 26%. The presence of spinal complications was independent of the bisphosphonate treatment status and joint hypermobility.

Conclusions: Scoliosis and craniovertebral anomalies are strongly associated in paediatric OI patients. Joint hypermobility that is much more common appears, however, to be a poor predictor.

Show MeSH
Related in: MedlinePlus