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Integration of Non-invasive Screening for Cystic Fibrosis Related Liver Disease in the Regular Follow-Up for Cystic

Diana Coman1, Marc Bilodeau1, Catherine Vincent1

  • 1Liver Unit, Centre Hospitalier de l'Université de Montréal, Montreal, Canada.

Digestive Diseases and Sciences
|December 13, 2024
PubMed

Insights

Cystic Fibrosis-Related Liver Disease (CFLD) affects 13% of patients, with nodular liver linked to higher mortality. Non-invasive screening using transient elastography shows promise for early detection and management.

Area of Science:

  • Hepatology
  • Pulmonology
  • Gastroenterology

Background:

  • Cystic Fibrosis-Related Liver Disease (CFLD) affects up to 40% of patients with cystic fibrosis (CF) and is a leading cause of mortality.
  • Early identification and management of CFLD are crucial to prevent severe complications like cirrhosis and portal hypertension.

Purpose of the Study:

  • To determine the prevalence of CFLD in a CF cohort at a tertiary university center.
  • To characterize the types of liver involvement in CFLD.
  • To assess the utility of non-invasive screening methods for CFLD detection.

Main Methods:

  • Retrospective review of patient files from a CF clinic (2020-2022).
  • Defined CFLD using NIH criteria: one major (abnormal imaging) or two minor criteria (abnormal labs, hepatosplenomegaly, transient elastography [TE] ≥ 7 kPa).

Main Results:

  • CFLD was identified in 13% (46/357) of patients.
  • Transient elastography (TE) demonstrated high performance for non-invasive CFLD assessment (AUROC 0.80).
  • A nodular liver was observed in 7% of patients, associated with increased fibrosis markers and short-term mortality (14.8% vs. 1.5%).

Conclusions:

  • Non-invasive methods, particularly abdominal imaging and TE, show promise for early CFLD detection.
  • Prompt recognition of CFLD can help prevent progression to cirrhosis and portal hypertension.
  • Integrating these non-invasive tools into clinical practice is recommended for optimal patient care.
Abstract

Keywords:
Cystic fibrosis (CF)Cystic fibrosis liver disease (CFLD)Hepatic involvementNon-invasive testsTransient elastography

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