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Diseases of the Liver and Gallbladder01:26

Diseases of the Liver and Gallbladder

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Liver and gallbladder diseases are a significant health concern, with prominent conditions including cirrhosis, hepatitis, non-alcoholic fatty liver disease (NAFLD), and gallstones. Jaundice is a common manifestation of liver and biliary disease.
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The microscopic anatomy of the liver is a complex and intricate system that comprises numerous structural units known as liver lobules, each of which is comparable in size to a sesame seed. These hexagonal structures consist of plates of liver cells or hepatocytes, which are characterized by their versatility and abundance of cellular apparatus like rough and smooth ER, Golgi apparatus, peroxisomes, and mitochondria.
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Liver Physiology01:30

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The liver, an essential organ in the human body, performs over 200 vital functions that can be broadly categorized into metabolic, hematological, endocrine regulation, and bile production.
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The liver is an important organ in vertebrates that plays an essential role in metabolism. It is also responsible for storing and redistributing nutrients such as carbohydrates, fats, and vitamins in the body. Additionally, the liver releases bile salts which are critical for digesting food and eliminating toxic metabolites from the body.
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Author Spotlight: Advancing Liver Regeneration Research through ALPPS Mouse Model
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Fontan-Associated Liver Disease: A Review.

Gabriel Heering1, Nachum Lebovics1,2, Raksheeth Agarwal1,3

  • 1From the Department of Medicine, Westchester Medical Center/New York Medical College, Valhalla, NY.

Cardiology in Review
|March 13, 2024
PubMed
Summary
This summary is machine-generated.

Fontan-associated liver disease (FALD) is a serious complication for adults with single-ventricle circulation after the Fontan procedure. Current diagnostic and treatment strategies for FALD face significant challenges, necessitating further research.

Keywords:
Fontan procedureFontan-associated liver diseasecongenital heart diseasesingle-ventricle congenital heart disease

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Area of Science:

  • Cardiology
  • Hepatology
  • Pediatric Surgery

Background:

  • Fontan-associated liver disease (FALD) is a progressive complication in adults following the Fontan procedure for congenital heart disease.
  • The growing Fontan population faces significant morbidity and mortality due to FALD, encompassing fibrosis, cirrhosis, and hepatocellular carcinoma.
  • FALD pathophysiology involves complex hemodynamic and inflammatory factors unique to single-ventricle physiology.

Purpose of the Study:

  • To review the current literature on Fontan-associated liver disease (FALD).
  • To highlight challenges in diagnosing and managing FALD, including unreliable noninvasive tests and inconsistent biopsy results.
  • To discuss current treatment controversies and identify knowledge gaps for future research.

Main Methods:

  • Comprehensive literature review of epidemiology, pathophysiology, diagnosis, and management of FALD.
  • Analysis of challenges in current diagnostic modalities, including noninvasive markers and invasive sampling.
  • Examination of treatment options for end-stage FALD, including transplantation strategies.

Main Results:

  • FALD presents a spectrum of liver abnormalities, from fibrosis to cancer, posing diagnostic difficulties due to congestion and patchy fibrosis.
  • Noninvasive tests for liver stiffness are unreliable in FALD, and tissue sampling is inconsistent.
  • Liver and heart transplantation are treatment options, with combined heart-liver transplantation increasingly favored in the US.

Conclusions:

  • FALD is a major concern for the growing adult Fontan population, requiring improved diagnostic and surveillance protocols.
  • Current diagnostic methods for FALD are inadequate, necessitating the development of validated approaches.
  • Further research is crucial to address knowledge gaps in FALD management and optimize patient outcomes.