Osteogenesis Imperfecta

Abstract

Osteogenesis imperfecta is a heritable disease that may result in bone fragility, increased joint laxity, decreased muscle tone, thinning of the skin, a bluish appearance of the sclerae, and scoliosis in as many as 60% of cases. The purpose of this study was to examine the impact of patient and hospital characteristics on mortality rate during inpatient stays. Data was collected retrospectively from the Healthcare Cost and Utilization Project Kids' Inpatient Database, a resource designed to analyze pediatric hospital usage. Data were collected from 1793 patients in the 27 states. Overall, 3% of this population died during hospitalization. Self-pay patients, patients in hospitals with small bed sizes, patients in non pediatric hospitals, and younger patients all had higher mortality rates than did their counterparts. In addition, black patients were 3.7 times more likely to die than did patients of any other race, and women were more likely to die than did men, although more than half of the number of patients were classified as white and 52% were men. Although these trends suggest that the mortality of younger patients may be reduced by admittance to children's hospitals, the children who are hospitalized younger tend to have more severe forms of the disease and are therefore more deformed and more difficult to treat. Overall, the results of this study indicate that children with osteogenesis imperfecta who need hospitalization may benefit from being referred to a large children's hospital, and that there is further research needed into the significant differences in the mortality of black patients and female patients.