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Osteogenesis imperfecta increases mortality risk
There is an increased risk for death due to respiratory and gastrointestinal diseases and trauma in people with osteogenesis imperfecta compared with those without the genetic bone disorder, study data show.
“Better knowledge about life expectancy and the primary causes of death in patients with [osteogenesis imperfecta] will give insight into diseases that need attention in the care for patients with [osteogenesis imperfecta],” the researchers wrote.
Lars Folkestad, MD, of the department of endocrinology at Odense University Hospital, Endocrine Elite Research Centre in Denmark, and colleagues evaluated data from the National Patient Register from 1977 to 2013 on 687 people with a diagnosis of osteogenesis imperfecta (379 female) and a reference population of 3,435 people (1,895 female) to determine the risk and cause of death as well as median survival among the study populations.
Only one cause of death was registered for 34% of the reference group compared with 13.4% of the osteogenesis imperfecta group. The osteogenesis imperfecta group had a significantly higher Charlson comorbidity index compared with the reference group (P = .003).
Median survival time was greater in men in the reference group (81.9 years) compared with the osteogenesis imperfecta group (72.4 years; P < .001). The same was true for women (84.5 years vs. 77.4 years; P < .001).
Overall, all-cause mortality was greater in the osteogenesis imperfect group compared with the reference group (HR = 2.9; 95% CI, 2.3-3.6); the HRs in the osteogenesis imperfecta group were 2.4 (95% CI, 1.8-3.3) for women and 3.7 (95% CI, 2.6-5.2) for men.
The primary causes of death in both groups were cardiovascular diseases and neoplasms. Compared with the reference group, the osteogenesis imperfecta group had increased risks for deaths caused by respiratory diseases (sub-HR = 3.1; 95% CI, 1.4-6.9), deaths caused by a digestive illness (sub-HR = 4.2; 95% CI, 1.6-10.8) and deaths due to external forces of morbidity and mortality (sub-HR = 4.7; 95% CI, 1.4-16.3).
“We conclude that patients with [osteogenesis imperfecta] have increased all-cause mortality rates, with a higher risk of death due to respiratory disease, gastrointestinal disease and trauma (bone fractures),” the researchers wrote. “The shorter survival of patients with [osteogenesis imperfecta] may be due to increased risk of neonatal death associated with more severe [osteogenesis imperfecta] phenotypes and to increased risk of death throughout life.” – by Amber Cox
Disclosure: Folkestad reports receiving speaker fees from AstraZeneca and Genzyme, a Sanofi Company. Please see the full study of all other author’ relevant financial disclosures.