Colombo et al identified risk factors associated with liver disease in patients with cystic fibrosis. These can help to identify patients who may benefit from screening and closer monitoring. The authors are from the University of Milan.
Patient selection: patients with cystic fibrosis
The onset of liver disease was often before age 10 years of age, after which there was a sharp decline.
Risk factors for CFALD:
(1) male gender
(2) history of meconium ileus
(3) a severe genotype
where:
• Fustick et al reported severe mutations as: DeltaF508, G542X, N1303K, CFTRdel.21Kb, 1811+1GàC, and Y1092X.
Some patients have a rapid progression to cirrhosis and require liver transplant.
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