WS1.1 Liver disease in cystic fibrosis

Idiosyncratic reactions are the most common cause of abnormal liver function tests in patients with cystic fibrosis

“…This may relate to the nature of CFLD being predominantly a disease leading to complications of portal hypertension rather than that of defects of hepatic synthetic function. 17 Notably, male sex and lower mean leucocyte count were significantly associated with abnormal LFT, but these variables only explained 4.2% of the variance. Existing literature states gender as a risk factor for betalactam-associated liver injury; however, this is dependent on the antibiotic agent (e.g.…”

Cystic Fibrosis Liver Disease: Outcomes and Risk Factors in a Large Cohort of French Patients

“…(33,34) So far, the literature is inconclusive about the impact of CFLD on CF evolution. While some studies have shown that patients with CFLD were likely to have a more severe CF phenotype with altered nutrition and lung function statuses, (5,(35)(36)(37)(38) others did not observe any association. (19,20,34,39,40) We were able to confirm in this large cohort that patients with CFLD had more severe lung disease, with the worst sufferers being patients with severe CFLD complications.…”