Advanced cystic fibrosis liver disease: Endovascular, endoscopic, radiologic, and surgical considerations

Up 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 adv...

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Bibliographic Details
Published in:Pediatric pulmonology 2024-09, Vol.59 (S1), p.S115-S122
Main Authors: Palle, Sirish K., Leung, Daniel H.
Format: Article
Language:English
Subjects:
cystic fibrosis (CF)
Cystic Fibrosis - complications
Cystic Fibrosis - diagnostic imaging
Cystic Fibrosis - surgery
Endovascular Procedures - methods
Gastrostomy - methods
Humans
Liver Diseases - diagnostic imaging
Liver Diseases - surgery
Liver Transplantation
Paracentesis - methods
Portasystemic Shunt, Surgical - methods
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Summary:Up 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.
ISSN:8755-6863
1099-0496
1099-0496
DOI:10.1002/ppul.27174