Related Experiment Video

Updated: Feb 24, 2026

Application of Hemostatic Devices in Laparoscopic Hepatectomy
04:23

Application of Hemostatic Devices in Laparoscopic Hepatectomy

Published on: April 19, 2022

2.9K

A Fatal Case of Erdheim-Chester Disease with Hepatic Involvement

Gokulakrishnan Balasubramanian1, Alexandra Modiri1, Marina Affi1

  • 1Division of Gastroenterology and Hepatology, Medical College of Wisconsin, Milwaukee, WI.

ACG Case Reports Journal
|August 12, 2017
PubMed

Insights

Erdheim-Chester disease (ECD), a rare histiocytosis, uncommonly affects the liver. This case highlights ECD presenting as cirrhosis, leading to fatal liver dysfunction despite initial treatment response.

Area of Science:

  • Histiocytosis
  • Gastroenterology
  • Oncology

Background:

  • Erdheim-Chester disease (ECD) is a rare systemic histiocytosis.
  • ECD typically involves bone, lungs, pituitary, heart, and brain.
  • Liver involvement in ECD is exceptionally rare.

Observation:

  • A 56-year-old woman presented with newly diagnosed cirrhosis.
  • Clinical signs suggested intra-abdominal malignancy, including omental caking and peritoneal thickening.
  • Liver biopsy revealed xanthogranulomatous infiltration consistent with ECD.

Findings:

  • The patient initially responded to interferon therapy.
  • Severe depression led to treatment discontinuation.
  • Progressive liver dysfunction resulted in hepatorenal syndrome and death.

Implications:

  • This case underscores the potential for Erdheim-Chester disease to manifest with severe liver dysfunction.
  • Highlights the diagnostic challenge of ECD presenting as cirrhosis.
  • Emphasizes the need for considering ECD in unexplained liver disease with systemic symptoms.

More Related Videos

Application of the En Bloc Concept Combined with Anatomic Resection in Laparoscopic Hepatectomy04:41

Application of the En Bloc Concept Combined with Anatomic Resection in Laparoscopic Hepatectomy

Published on: March 10, 2023

1.7K
Endoscopic Ultrasound-Guided Biliary Drainage: Endoscopic Ultrasound-Guided Hepaticogastrostomy in Malignant Biliary Obstruction07:44

Endoscopic Ultrasound-Guided Biliary Drainage: Endoscopic Ultrasound-Guided Hepaticogastrostomy in Malignant Biliary Obstruction

Published on: March 25, 2022

6.9K

Related Concept Videos

Effect of Hepatic Disease on Pharmacokinetics: Pathophysiologic Assessment and Liver Function Test01:22

Effect of Hepatic Disease on Pharmacokinetics: Pathophysiologic Assessment and Liver Function Test

In clinical practice, the direct measurement of hepatic blood flow to evaluate liver function presents significant challenges due to the intricate and specialized nature of the necessary techniques. Consequently, healthcare professionals often rely on empirical estimates derived from thorough patient examinations and liver function tests to gauge liver health. Among the tools at their disposal, the Child–Pugh and MELD scoring systems stand out for their ability to categorize and assess...
243
Esophageal Varices-II: Clinical Features and Management01:28

Esophageal Varices-II: Clinical Features and Management

Esophageal varices often manifest as gastrointestinal bleeding episodes, presenting symptoms like hematemesis (vomiting of blood), hematochezia (passing fresh blood via the rectum), and melena (black, tarry stools). Other signs can include weight loss, anorexia, abdominal discomfort, jaundice, pruritus, altered mental status, and muscle cramps.
In the initial assessment, a thorough review of the patient's medical history is vital to identify risk factors such as liver disease, alcohol...
664
Effect of Hepatic Disease on Pharmacokinetics: Drug Dosing and Hepatic Blood Flow01:26

Effect of Hepatic Disease on Pharmacokinetics: Drug Dosing and Hepatic Blood Flow

Chronic liver disease significantly impacts drug metabolism due to alterations in hepatic blood flow and enzyme accessibility. This disruption affects the body's pharmacokinetics—the movement and processing of drugs within the system. Key enzymes crucial for metabolizing medications become less accessible, changing how drugs are processed and utilized. Furthermore, liver disease influences the synthesis of plasma proteins, such as albumin and globulins, which play critical roles in drug...
294
Endocarditis II: Clinical Features of Infective Endocarditis01:25

Endocarditis II: Clinical Features of Infective Endocarditis

Endocarditis can present various clinical features depending on the causative organism and the patient's underlying health conditions. Initially, the clinical features of infective endocarditis develop gradually, presenting with nonspecific symptoms that can be easily mistaken for other illnesses.General SymptomsEarly symptoms of infective endocarditis are fever, chills, weakness, malaise, fatigue, and weight loss. These symptoms reflect the systemic nature of the infection and the body's...
605
Diseases of the Liver and Gallbladder01:26

Diseases of the Liver and Gallbladder

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.
Cirrhosis is characterized by the scarring of hepatic lobules in the liver, which are replaced by fibrous tissue, affecting the liver's normal functioning. NAFLD, on the other hand, is caused by an excessive build-up of fat in the liver, not...
2.1K
Effect of Hepatic Disease on Pharmacokinetics: Active Drug, Metabolite and Fraction of Metabolized Drug01:14

Effect of Hepatic Disease on Pharmacokinetics: Active Drug, Metabolite and Fraction of Metabolized Drug

In pharmacotherapy, monitoring drug concentrations is paramount, especially for drugs whose therapeutic effects hinge on both the active compound and its metabolite. Hepatic impairment profoundly influences drug potency by altering liver function. If the drug is more potent than its metabolite, impaired liver function amplifies drug activity due to elevated drug concentration levels. Conversely, if the metabolite holds greater potency, diminished liver function diminishes drug activity by...
266