A198 CYSTIC FIBROSIS LIVER DISEASE IN NEWFOUNDLAND AND LABRADOR: A PREVALENCE STUDY

Liver disease in patients with cystic fibrosis (CF) is understudied and increasingly common. The prevalence of cystic fibrosis liver disease (CFLD) in Canada is not clearly established, though it is now the third leading cause of death among CF patients. The current literature suggests a broad range in prevalence from 4% to 65%, which implies the need for further research. An overall trend throughout the literature demonstrates that studies that included isolated liver enzyme elevation as prevalent cases had higher rates of CFLD, while older studies and those that relied heavily on examination alone had a lower reported prevalence. Assessment of liver size and liver enzymes comprise the current screening guidelines. Diagnostic criteria include evaluation of the liver via imaging and liver enzyme elevation, as outlined in the methods section. The primary aim of this study was to determine the prevalence of CFLD in adult patients with CF in Newfoundland and Labrador (NL). This was a descriptive cross sectional study of adult patients with CF in NL. Charts were reviewed retrospectively for: 1) hepatomegaly or splenomegaly on imaging, 2) ALT, AST, GGT >1.5x ULN for >6 months, 3) ultrasound showing coarseness, nodularity, portal HTN, or increased echogenicity of the liver, and 4) liver biopsy with cirrhosis. If ≥2 of the above criteria were satisfied, it counted as a case of CFLD. The prevalence of isolated liver enzyme elevation was also determined. Participants were included if they had CF and were being followed by the NL CF clinic in 2018. They were excluded if there were other etiologies of liver disease that were not accounted for by a hepatologist’s workup. Only 57 of the 60 adult patient’s charts were available to review. The population was comprised of 35 men (61.5%) and 22 women (38.5%). Prevalence was the not found to have a statistically significant difference across age groups or gender. The diagnostic guidelines of CFLD were met in 14 cases. This represents a prevalence of 24.6% in the total population; however, 11 patients had not had the requisite imaging performed to assess the diagnostic criteria. Of those patients, 4 (36.4%) had elevated liver enzymes. Among the subset who had imaging, the prevalence of CFLD was 30%. Additionally, 33 patients had isolated liver enzyme elevation (57.9%) In recent years, an increasing body of literature has developed around CFLD. As a result, many CF clinics have increased testing. However, prevalence data is lacking across Canada. This study has attempted to fill this gap by examining the prevalence of CFLD in NL. It was found to be between 25% and 30%, which is in line with the current data. Further imaging may be warranted to assess liver size in the subset of patients who have not yet been screened. Given the paucity of Canadian data, further study would be warranted in this area. None

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