Osteogenesis imperfecta(OI) is a hereditary disease characterized by abnormally brittle, easily fractured bones. OI is caused by a genetic mutation leading to the production of defective Type I collagen. Collagen is an important protein and without it the bones are weak and break very easily. Fractures usually occur once the child begins to walk, however, some children may have fractures at birth. Most children suffer from severe growth retardation. The treatment of osteogenesis imperfects is provided by medical management, physical rehabilitation, surgical intervention, gene therapy and bone marrow transplantation. The goal of this study is to make information available on osteogenesis imperfecta and the population it affects.
Methods:
The Healthcare Cost and Utilization Projects (HCUP) Kids' Inpatient Database (KID) is a multi-institutional, multi-State database created to analyze pediatric hospital utilization. Information on children that were discharged was collected from hospitals in 27 HCUP partner states. A definition for osteogenesis imperfecta was established and the database was analyzed. Some of the information collected from the patients included demographics (ie: age, gender, race, ect.), where the patient was admitted from (ie: the emergency department, another hospital, another health facility), the type of birth (complicated or uncomplicated), and the type of payment expected (Medicaid, private insurance, self-pay, no charge, or other payment). Information was also collected on the hospital the patient was admitted to. This included the type of hospital (children's general, children's specialty, children's unit in a general hospital or not identified), bed size (the number of patients at that hospital), and the location of the hospital.
Results:
More than half the patients were male, and White, with an average age at admission of 7 years old.
The majority of the hospitals were not identified as Children's Hospitals.
Half of the hospitals were considered large.
A small number of the hospitals were non-teaching hospitals located in rural areas, and the majority of the hospitals were urban teaching hospitals.
Most of the hospitals in the sample were located in the South or West.
Female patients were 2.56 times more likely to die than male patients, while controlling for all other factors.
Black patients were 3.27 times more likely to die than patients of all other races, while controlling for all other factors.
The patients that were admitted from another hospital were 9.67 times more likely to die that patient from all other sources, while controlling for other factors.
Patients admitted urgently and as newborns were significantly more likely to die than patients with all other types of admission.
Patients who were planning on self-paying for their hospital stay were 5.08 times more likely to die than patients with all other types of payment.
Patients were .66 times as likely to die with each additional year of age.
Patients admitted to a children's unit in a general hospital were .27 times as likely to die than patients in all other types of hospitals.
Patients admitted to hospitals with a large number of beds were 4.09 times as likely to die than patients admitted to hospitals with a small number of beds.
Those patients visiting hospitals in the south were 2.13 times as likely to die than patients in all other regions.
Conclusion:
Our study is unique in that it focuses on looking back at a large population of patients with osteogenesis imperfecta. We analyze not only the general demographic information of the patients, but also the characteristics of the hospitals they stay in. From this we can correlate both hospital characteristics and patient characteristics with death during an impatient admission.
In terms of patient demographics, our study sample had an even distribution between genders, and over half of the patients were white. Female gender and Black race were significant predictors of death. Although there are no specific characteristics of females or blacks illustrated in literature to explain why these groups are more likely to die than others, it is possible that once the genetic mutation occurs, a more severe form may come about leading to death at an earlier age. Also, it has been established that other diseases occur more frequently within one race, such as cystic fibrosis in Whites and sickle cell anemia in Blacks.
When analyzing the results retrieved for admission source it is not surprising that the majority of patients were admitted after birth, since bony deformities are visible during fetal ultrasound and patients requiring frequent admission for surgery may be admitted via routine means. The next most frequent source of admission was the emergency department, which follows the theory that children who had a mild form of OI would only be diagnosed and admitted after a fracture once they begin walking or playing sports. Interestingly, every single patient with OI suffered from a complicated in-hospital birth. This leads us to believe that although every patient was not able to be diagnosed in utero, some form of trouble occurred during delivery, probably a fracture. Over half of the patients were insured privately while over one third of the patients were insured by Medicaid. Combining this insurance information with that of the average household income, we can conclude that the OI population does not come from an extremely poor background.
It was not surprising that most of the patients were seen at large urban teaching hospitals as parents would want to seek the best care for their children. Larger urban teaching hospitals have more physicians that have more specialized training than smaller, rural non-teaching hospitals.
The demographics indicated that almost 70% of the OI patients were admitted in the South West regions of the United States, which suggests that the population of those regions may have the genetic mutation in a higher frequency than other regions. Patients admitted from another hospital were much more likely to die following admission, which is due to the fact that the majority of patients who are transferred to another hospital require a level of care that the previous hospital was unable to provide. Since these children are sicker than children admitted only to one hospital, it is not surprising that they are more likely to die. It is also interesting to note that no children admitted to a children's specialty hospital died during admission. This may indicated that at these specialty hospitals there is a higher level of care with physicians who are better trained to handle cases of OI.
Introduction: