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Abstract
Cystic fibrosis liver disease (CFLD) is the third leading cause of death among patients with CF. Although the majority of patients with CFLD are pediatric patients, recent research suggests a “second wave” of liver damage in adulthood.
In addition to biliary cirrhosis, many patients with CF may have hepatic steatosis. For a long time, hepatic steatosis in patients with CF was associated with nutritional deficiencies, in particular essential fatty acids. Recently, it was found out that steatosis in patients with CF is a multifactorial phenomenon and follows a similar pathogenesis to that observed in the general population (metabolic-associated fatty liver disease and alcohol-induced liver disease). In the UK, a questionnaire-based study revealed that 83% of CF patients consume alcohol.
Another significant condition leading to chronic liver damage in patients with CF is lithogenesis, due to changes in the composition of bile and its stagnation. This can cause biliary strictures and sclerosing cholangitis, which in turn may lead to secondary biliary cirrhosis.
In addition to laboratory markers and imaging techniques, other non-invasive indices of liver health aid in the diagnosis of CFLD. Two laboratory markers, the AST-platelet ratio index (APRI) and the fibrosis marker FIB-4, correlate with the diagnosis of CFLD through a double liver biopsy.
Therapy goals for CFLD include addressing the sequelae associated with each specific manifestation, particularly portal hypertension, and slowing the progression of the disease. All patients with CFLD should be monitored every 3–6 months for liver enzymes and markers related to liver synthetic function, including a coagulation profile.
Unfortunately, there are still no drug treatments to slow the progression or improve the course of CFLD. Traditionally, doctors administer ursodeoxycholic acid to patients after diagnosing CFLD. Nutrition is important as well. It is necessary to pay attention to the treatment of portal hypertension (drug, endoscopic, and surgical). In some cases, it becomes necessary to perform liver transplantation simultaneously with lung transplantation.
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