Cystic fibrosis with liver involvement in adults has a benign course: Results from a tertiary referral center cohort

• Almudena Marinero Martínez-Lázaro ^[1] ; Rosa María Girón Moreno ^[2] ; Fernando Casals Seoane ^[2] ; Óscar Cano-Valderrama ^[3] ; Luisa García-Buey ^[2]
  1. [1] Hospital Clínico San Carlos de Madrid

     Hospital Clínico San Carlos de Madrid

     Madrid, España

     
  2. [2] Hospital Universitario de la Princesa

     Hospital Universitario de la Princesa

     Madrid, España

     
  3. [3] Complexo Hospitalario Universitario de Vigo

     Complexo Hospitalario Universitario de Vigo

     Vigo, España

     

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• Localización: Revista Española de Enfermedades Digestivas, ISSN-e 2340-4167, ISSN 1130-0108, Vol. 115, Nº. 6, 2023, págs. 301-305
• Idioma: inglés
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• Resumen

  • Background: cystic fibrosis liver disease is a poorly under- stood entity, especially in adults, in terms of its real preva- lence, natural history and diagnostic criteria, despite being the most important extrapulmonary cause of mortality.

    The aim was to evaluate the prevalence, characteristics and potential risk factors of liver disease in adults with cystic fibrosis, according to two diagnostic criteria accepted in the scientific literature.

    Methods: patients were recruited in a tertiary referral hos- pital, and laboratory, ultrasound, non-invasive liver fibrosis tests (AST to Platelet Ratio Index [APRI]; Fibrosis-4 Index [FIB- 4]) and transient elastography (Fibroscan® ) were performed.

    The proportion of patients with liver disease according to the Debray and Koh criteria were evaluated.

    Results: ninety-five patients were included, 48 (50.5 %) fe- males, with a mean age of 30.4 (28.6-32.2) years. According to the Debray criteria, six (6.3 %) patients presented liver disease. According to the Koh criteria, prevalence increased up to 8.4 %, being statistically different from the 25 % val- ue described in other published series (p = 0.005). Seven (7.5 %) presented ultrasonographic chronic liver disease.

    Eleven (13 %) presented liver fibrosis according to the APRI score; 95 (100 %) had a normal FIB-4 value. Mean liver stiff- ness value was 4.4 (4.1-4.7) kPa. FEV1 (OR = 0.16, p 0.05), meconium ileus (OR = 14.16, p 0.002), platelets (Pearson co- efficient -0.25, p 0.05) and younger age (Pearson coefficient -0.19, p 0.05) were risk factors.

    Conclusions: prevalence and severity of liver disease in adult cystic fibrosis patients were lower than expected. Meconium ileus, platelets, age and respiratory function were confirmed as risk factors associated to cystic fibrosis liver disease