ABSTRACT
Primary ciliary dyskinesia (PCD) is a rare autosomal recessive disorder leading to chronic upper and lower airway disease. Fundamental data on epidemiology, clinical presentation, course and treatment strategies are lacking in PCD. We have established an international PCD registry to realise an unmet need for an international platform to systematically collect data on incidence, clinical presentation, treatment and disease course.The registry was launched in January 2014. We used internet technology to ensure easy online access using a web browser under www.pcdregistry.eu. Data from 201 patients have been collected so far. The database is comprised of a basic data form including demographic and diagnostic information, and visit forms designed to monitor the disease course.To establish a definite PCD diagnosis, we used strict diagnostic criteria, which required two to three diagnostic methods in addition to classical clinical symptoms. Preliminary analysis of lung function data demonstrated a mean annual decline of percentage predicted forced expiratory volume in 1â s of 0.59% (95% CI 0.98-0.22).Here, we present the development of an international PCD registry as a new promising tool to advance the understanding of this rare disorder, to recruit candidates for research studies and ultimately to improve PCD care.
Subject(s)
Kartagener Syndrome/diagnosis , Kartagener Syndrome/epidemiology , Registries , Adolescent , Adult , Age Distribution , Aged , Child , Child, Preschool , Disease Progression , Europe , Female , Forced Expiratory Volume , Humans , Incidence , Infant , Internet , Intersectoral Collaboration , Male , Middle Aged , North America , Patient Selection , Young AdultABSTRACT
OBJECTIVE: The aim of this project was to design and develop a personal electronic health record (EHR) in order to support patient empowerment and additionally to enhance their communication and information exchange with health professionals through this EHR. METHOD: The functionality of a personal Electronic Healthcare Record (EHR) may vary from a simple web-based interface for interactive data entry and data review up to a much more powerful system additionally supporting electronic data/document communication between clinical information systems of primary care practitioners or hospitals and even reminder based support for the empowered citizen, to actively take care of his health, based on relevant disease management programs. It is one means to support patient empowerment, additionally supported by tools for building a patient community. Since storage and communication of data in an EHR comprises sensible personal health data, each of those functions needs specific security and access management requirements to be considered and implemented. RESULT: Clinical pilot projects are already done or under development.
Subject(s)
Internet , Medical Records Systems, Computerized , Patient Advocacy , Physician-Patient Relations , Communication , Computer Security , Humans , Primary Health Care , Reminder Systems , User-Computer InterfaceABSTRACT
The functionality of a personal Electronic Healthcare Record (EHR) may vary from a simple web-based interface for interactive data entry and data review up to a much more powerful system additionally supporting electronic data/document communication between clinical information systems of primary care practitioners or hospitals and even reminder based support for the empowered citizen, to actively take care of his health, based on relevant disease management programs. It is one means to support patient empowerment. Since storage and communication of data in an EHR comprises sensible personal health data, each of those functions need specific security and access management requirements to be considered and implemented.
