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Related Experiment Video

Updated: Jul 16, 2026

Bile Duct Ligation in Mice: Induction of Inflammatory Liver Injury and Fibrosis by Obstructive Cholestasis
08:56

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Cholestasis and liver failure with lambda-AL amyloidosis.

F Konikoff1, C Mor, S Stern

  • 1Department of Internal Medicine E, Beilinson Medical Center, Petah Tiqva, Israel.

Gut
|July 1, 1987
PubMed
Summary

Systemic amyloidosis rarely causes significant liver issues, but this case showed severe cholestasis leading to liver failure. This unique presentation involved only Lambda light chains and did not respond to standard treatments or dimethyl sulfoxide.

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Area of Science:

  • Hepatology
  • Nephrology
  • Oncology

Background:

  • Systemic amyloidosis is a rare condition characterized by amyloid protein deposition in organs.
  • Liver involvement in systemic amyloidosis, particularly with cholestasis, is uncommon.
  • Primary amyloidosis often involves light chain deposition, but specific patterns can vary.

Observation:

  • A rare case of primary amyloidosis presented with severe intrahepatic cholestasis.
  • The cholestasis progressed to terminal liver failure.
  • The amyloid deposition was exclusively associated with Lambda light chains.

Findings:

  • This is the first reported case of primary amyloidosis with cholestasis solely due to Lambda light chains.
  • Conventional therapies for amyloidosis were ineffective in this patient.
  • A trial of dimethyl sulfoxide (DMSO) also failed to improve the liver condition.

Implications:

  • Highlights the potential for rare presentations of systemic amyloidosis impacting liver function.
  • Suggests that Lambda light chain-associated amyloidosis may have unique clinical manifestations and treatment challenges.
  • Underscores the need for further research into novel therapeutic strategies for advanced hepatic amyloidosis.