Affiliation:
1. Division of Gastroenterology, Hepatology and Nutrition, Department of Pediatrics University of Oklahoma Health Sciences Center Oklahoma City Oklahoma USA
2. Division of Gastroenterology, Hepatology and Nutrition, Department of Pediatrics Texas Children's Hospital, Baylor College of Medicine Houston Texas USA
Abstract
AbstractUp to 90% of people with CF (pwCF) will have some form of hepatobiliary involvement. This manuscript aims to explore the different endovascular, endoscopic, radiological and surgical procedures available to diagnose and manage the most severe form of CF hepatobiliary involvement (CFHBI) known as advanced cystic fibrosis liver disease (aCFLD), seen in 10% of pwCF. These procedures and interventions include liver biopsy, hepatic venous pressure gradient measurement, gastrostomy tube placement to optimize nutrition, paracentesis, endoscopic variceal control of bleeding and portosystemic shunting before liver transplantation. By utilizing advanced diagnostic or surgical techniques, healthcare professionals of pwCF can more effectively manage patients with CFHBI and aCFLD and potentially improve patient outcomes.