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Optimized LC-MS/MS Method for the High-throughput Analysis of Clinical Samples of Ivacaftor, Its Major Metabolites, and Lumacaftor in Biological Fluids of Cystic Fibrosis Patients
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Advanced cystic fibrosis liver disease: Endovascular, endoscopic, radiologic, and surgical considerations

Sirish K Palle1, Daniel H Leung2

  • 1Division of Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma, USA.

Pediatric Pulmonology
|August 6, 2024
PubMed

Insights

Cystic fibrosis patients often develop liver issues. This review covers advanced procedures for diagnosing and managing severe cystic fibrosis liver disease, aiming to improve patient outcomes.

Area of Science:

  • Hepatology
  • Gastroenterology
  • Transplant Surgery

Background:

  • Hepatobiliary involvement affects up to 90% of people with cystic fibrosis (pwCF).
  • Advanced cystic fibrosis liver disease (aCFLD) impacts approximately 10% of pwCF.
  • Effective management of CFHBI is crucial for patient prognosis.

Purpose of the Study:

  • To explore diagnostic and management procedures for severe CF hepatobiliary involvement (CFHBI).
  • To detail interventions for advanced cystic fibrosis liver disease (aCFLD).
  • To highlight the role of advanced techniques in improving outcomes for pwCF with liver disease.

Main Methods:

  • Review of endovascular, endoscopic, radiological, and surgical procedures.
  • Discussion of liver biopsy and hepatic venous pressure gradient measurement.
  • Exploration of interventions including gastrostomy, paracentesis, variceal control, and shunting.

Main Results:

  • Identification of key diagnostic tools for CFHBI.
  • Outline of critical management strategies for aCFLD.
  • Emphasis on the necessity of specialized interventions prior to liver transplantation.

Conclusions:

  • Advanced diagnostic and surgical techniques are vital for managing CFHBI and aCFLD.
  • Effective application of these procedures can lead to improved patient outcomes.
  • Multidisciplinary care is essential for optimizing the health of pwCF with liver complications.

Keywords:
cystic fibrosis (CF)

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