Cystic Fibrosis (CF) is an inherited disease that affects multiple organs in the body. CF is caused by a disruption in the body's ability to transport salt across cell membranes, leading to thicker and healthier mucus in the lungs, intestines, pancreas, and liver. This can eventually result in tissue damage. The most well-known impact is on the lungs, which is accompanied by susceptibility to recurring infections. Liver involvement manifests as thickening of the bile fluid, which can lead to blockages in the bile ducts and cause damage to liver tissue. For reasons not fully understood, CF-related liver disease develops in only about a third of patients. When liver involvement occurs, it can range from mild to potentially progressing to liver failure.
Diagnosis of CF-related liver disease is typically made in children known to have CF through routine evaluations. If the treating physician suspects liver disease, an abdominal ultrasound will be performed for a better assessment, and in some cases, a liver biopsy or a specialized magnetic resonance cholangiopancreatography (MRCP) scan might also be needed.
Treatment primarily involves supportive care, focusing on improving nutrition and managing elevated liver pressure. In specific circumstances, a medication called ursodeoxycholic acid might be used to improve bile flow. In rare cases of severe liver disease associated with cystic fibrosis, liver transplantation might be necessary.