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Genetic Analysis of Hereditary Transthyretin Ala97Ser Related Amyloidosis
Published on: June 9, 2018
Fatal Subacute Hepatic Failure in a Patient with AA-Type Amyloidosis: Case Report
Ibrahim Altraif1, Fayaz A Handoo, Khaled O Alsaad
1Department of Hepatobiliary and Liver Transplantation, King Abdulaziz Medical City, P.O. Box 22490, Riyadh 11426, Saudi Arabia.
Abstract:
Although systemic amyloidosis of amyloid-associated protein (AA) type (secondary or reactive amyloidosis) frequently involves the liver, it rarely causes clinically apparent liver disease. Mild elevation of alkaline phosphatase and hepatomegaly are the most common biochemical and clinical findings, respectively. We report a case of systemic amyloidosis of AA type, which clinically presented as subacute hepatic failure and resulted in a fatal clinical course in a 69-year-old man. To the best of our knowledge, this is the fifth case of hepatic amyloidosis of AA type that clinically presented as fatal subacute hepatic failure, an unusual clinical presentation for hepatic involvement by systemic AA-type amyloid.
Insights
Systemic amyloidosis of amyloid-associated protein (AA) type rarely causes severe liver disease. This case highlights a rare presentation of fatal subacute hepatic failure due to AA-type amyloidosis.
Area of Science:
- Medicine
- Pathology
- Hepatology
Background:
- Systemic amyloidosis of amyloid-associated protein (AA) type, also known as secondary or reactive amyloidosis, commonly affects the liver.
- However, clinically apparent liver disease is an infrequent manifestation of AA amyloidosis.
Observation:
- A 69-year-old man presented with subacute hepatic failure.
- This clinical presentation led to a fatal outcome.
Findings:
- The patient was diagnosed with systemic amyloidosis of AA type.
- Hepatic involvement presented unusually as fatal subacute hepatic failure, a rare clinical manifestation.
Implications:
- This case underscores the importance of considering AA-type amyloidosis in the differential diagnosis of unexplained hepatic failure.
- Recognizing this rare presentation is crucial for timely diagnosis and management of hepatic amyloidosis.
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