Mortality and Causes of Death in Patients With Osteogenesis Imperfecta: A Register‐Based Nationwide Cohort Study

@article{Folkestad2016MortalityAC,
  title={Mortality and Causes of Death in Patients With Osteogenesis Imperfecta: A Register‐Based Nationwide Cohort Study},
  author={Lars Folkestad and Jannie Dahl Hald and Vladimir Canudas-Romo and Jeppe Gram and Anne Pernille Hermann and Bente Lomholt Langdahl and Bo Abrahamsen and Kim Brixen},
  journal={Journal of Bone and Mineral Research},
  year={2016},
  volume={31}
}
Osteogenesis imperfecta (OI) is a hereditary connective tissue disease that causes frequent fractures. Little is known about causes of death and length of survival in OI. The objective of this work was to calculate the risk and cause of death, and the median survival time in patients with OI. This study was a Danish nationwide, population‐based and register‐based cohort study. We used National Patient Register data from 1977 until 2013 with complete long‐term follow‐up. Participants comprised… 
Fracture Rates and Fracture Sites in Patients With Osteogenesis Imperfecta: A Nationwide Register‐Based Cohort Study
  • L. Folkestad, J. Hald, K. Brixen
  • Medicine
    Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research
  • 2017
TLDR
The risk of fractures seems largest in the childhood and adolescent years, and the relative risk of fracture declines with age in patients with OI compared to the general population, in a Danish nationwide, population‐based, cohort study.
Prevalence and Hospital Admissions in Patients With Osteogenesis Imperfecta in The Netherlands: A Nationwide Registry Study
TLDR
This OI nationwide registry study shows that the life expectancy of OI patients is adversely affected by the disease, and patients with OI had a 2.9 times higher hospitalization rate compared to the general Dutch population.
Mortality and morbidity in patients with osteogenesis imperfecta in Denmark.
TLDR
It is hypothesized that patients with OI will have increased prevalence and risk of fractures throughout life, lower bone mineral density (BMD), impaired bone microstructure and bone geometry and increased risk of cardiovascular diseasesthus increasedrisk of all cause mortality compared to the general population.
Hospital incidence, management and direct cost of osteogenesis imperfecta in Spain: a retrospective database analysis
TLDR
Analysis of admission records from patients admitted with OI in specialized care settings in Spain between 2000 and 2017 provides data that should be taken into account for the development of improved and more efficient treatment protocols, and in reducing the burden of OI at the healthcare system level.
Cardiopulmonary Status in Adults with Osteogenesis Imperfecta: Intrinsic Lung Disease May Contribute More Than Scoliosis.
TLDR
The lack of a relationship between decreased pulmonary function and the severity of scoliosis suggests that restrictive lung physiology in this population is likely because of factors intrinsic to OI and not entirely because of thoracic cage deformities.
Scoliosis and Cardiopulmonary Outcomes in Osteogenesis Imperfecta Patients.
TLDR
Pulmonary function is not significantly correlated with scoliosis, supporting the hypothesis that decreased pulmonary function is intrinsic to OI and/or chest wall deformities, rather than secondary to scolium.
Impact of fracture characteristics and disease-specific complications on health-related quality of life in osteogenesis imperfecta
TLDR
Physical function was decreased in OI patients who had fractures before 2 years old, especially in lower extremity, and Appropriate medical managements for cardiopulmonary insufficiency are required not only to maintain physical function but also to decrease mortality.
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