An analysis of the most common causes of hospitalization among patients with Angelman syndrome reveals that they differ based on age and gender.
The study, published in the Journal of Applied Research in Intellectual Disabilities, shows common healthcare needs among sub-groups of Angelman patients, which may help design tailored strategies to improve their care and quality of life.
As for many other rare diseases, little is known about the incidence, distribution, and management of Angelman syndrome. The scarce information available comes from small group analysis and a few population-based studies. This puts healthcare practitioners in a difficult position, unable to recognize all the features of the disorder and key patient needs.
In the study “Main causes of hospitalization in people with Angelman syndrome,” a Spanish research team evaluated the main causes of hospitalization for patients with Angelman syndrome in Madrid.
“Knowing the main causes of hospitalization may help to better understand the disease, and it will also be important to inform clinicians and policymakers trying to comprehend the healthcare needs of people with this condition,” the investigators wrote.
This study used information from the SIERMA database system, which included data from 49 patients with Angelman syndrome. Created in 2015, SIERMA gathers epidemiological information of patients with rare diseases in Madrid.
Between 2006 and 2014, 85.7 percent of the patients were hospitalized at least once, and a total of 97 hospitalizations were reported. The most frequent causes of hospitalization in this population were oral-dental care (28.9%), seizures (19.6%), orthopedic problems (14.4%), and acute respiratory disorders (12.4%).
But the team found that oral-dental care hospitalizations were significantly more frequent among women, whereas hospitalizations due to orthopedic problems were more frequent in men. These events occurred mainly during adolescence, with patients having median age of 15.5 years for oral-dental care events, and 13.0 years for orthopedic problems.
In contrast, hospitalizations due to acute respiratory disorders were more frequent in adults with a median age of 23.5 years, and seizures were more frequent in small children with a median age of 2.0 years.
This shows that the causes of hospitalization vary depending on a patient’s age and gender, which is information that “can be useful in planning for treatments and services that help people with AS to receive optimal health care,” the investigators wrote.
“Awareness of specific differences by sex and age in the main causes of hospitalizations can also help to guide healthcare planning,” the team added. “It may be a small first step in the urgent need for high-quality health services research to identify optimal health services for people with an intellectual disability and concurrent physical health problems.”