Our research initiatives focus on describing the natural history and pathophysiology of HPS through clinical and basic science studies, establishing prognostic indicators, studying methods to optimize transplant outcomes, and establishing and testing medical therapies.
Research is an integral part of the Toronto HPS clinic and is made possible largely through donations to the Rare Lung Disease Research Program at St. Michael’s Hospital. Current research studies include:
HPS patient database: Clinical information is collected on each clinic visit on all patients from our program who agree to be included in the HPS database . The database is used to study disease patterns and clinical outcomes over time.
EPC study: Animal models suggest that HPS may be caused by impaired function of endothelial progenitor cells (EPCs). We are now studying these cells in humans with HPS to determine if they behave differently under various conditions, when compared to EPCs from patients without HPS.
Recently Completed Research
Inhaled nitric oxide improves the hepatopulmonary syndrome: a physiologic analysis.
Development and Evaluation of an Online Resource for Hepatopulmonary Syndrome Patients and Caregivers: WWW.HPSCARE.COM
Alternative designs for clinical trials in rare diseases
Variability of Oxygenation in Possible Hepatopulmonary Syndrome: Effects of Requiring Two Abnormal Arterial Blood Gas Results for Diagnosis
Defining and Characterizing Severe Hypoxemia After Liver Transplantation in the Hepatopulmonary Syndrome
A Framework For Applying Unfamiliar Trial Designs In Studies Of Rare Diseases
Norfloxacin therapy for hepatopulmonary syndrome: a pilot randomized controlled trial
A Case of Unusual Pulmonary and Neurological Complications of Sarcoidosis
Proposed Management Algorithm for Severe Hypoxemia After Liver Transplantation in the Hepatopulmonary Syndrome
Rare Diseases: Canada’s Research Orphans
The Dilemma of Rare Lung Disease Research: Strategies for Identification and Recruitment of Research Participants
Improved Survival After Liver Transplantation in Patients with Hepatopulmonary Syndrome
CT Scan Does Not Differentiate Patients with Hepatopulmonary Syndrome from Other Patients with Liver Disease
The Canadian HPS Program is open to new research collaborations. We invite anyone interested in contributing, from across Canada and around the world, to contact us about any research initiatives that may help patients with HPS. Our goal is to improve the understanding and treatment of this disease in order to further improve the lives of people with this rare disease. Please contact the HPS research coordinator, Stephanie Segovia at [email protected] for more information.