Cystic fibrosis liver disease in Newfoundland and Labrador: a prevalence study.

Background: Liver disease in patients with cystic fibrosis (CF) is an understudied and increasingly common concern. The prevalence of cystic fibrosis liver disease (CFLD) in Canada has not been clearly established, although it is now the third leading cause of death among patients with CF. The current literature identifies a range in prevalence from 4% to 65%, which implies the need for further research. This study aimed to determine the prevalence of CFLD among adult patients with CF in Newfoundland and Labrador. Methods: Charts of patients with CF from the St. John's, Newfoundland, CF clinics were reviewed retrospectively for the presence of elevated liver enzymes, imaging or biopsy of the liver, and other etiologies of liver disease. Prevalence was determined for patients meeting the criteria for CFLD in the population as a whole and for those who had undergone all pertinent investigations. Results: The diagnostic guidelines for CFLD were met in 14 of 57 cases (24.6% prevalence). Severe CFLD was present in 9 patients (15.8%). Among all patients with CF, 33 (57.9%) had isolated liver enzyme elevation. Eleven patients had not had the requisite imaging performed for accurate diagnosis. Among the subset who had undergone imaging, the prevalence of CFLD was 30%. Conclusions: This study attempted to fill the gap in Canadian CFLD prevalence data by examining the population with CF in Newfoundland and Labrador. The prevalence of CFLD was found to be between 25% and 30%. More accurate determination of prevalence could be done with future cross-sectional or prospective studies.