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Severe Obstructive Cholestasis and Hypercalcemia Caused by Light-Chain Amyloidosis: A Case Report

Gastrointestinal amyloidosis is a condition caused by the deposition of extracellular protein fragments. It can be associated with complex and diverse pathways and can have numerous manifestations and etiologies. Hepatic amyloid light-chain (AL) amyloidosis is a rare disorder characterized by the de...

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Autores principales: Davoudi, Zahra, Bidari, Farahnaz, Jamali, Elena, Nikpour, Shahriar
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Shiraz University of Medical Sciences 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8743367/
https://www.ncbi.nlm.nih.gov/pubmed/35017780
http://dx.doi.org/10.30476/ijms.2021.88694.1942
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author Davoudi, Zahra
Bidari, Farahnaz
Jamali, Elena
Nikpour, Shahriar
author_facet Davoudi, Zahra
Bidari, Farahnaz
Jamali, Elena
Nikpour, Shahriar
author_sort Davoudi, Zahra
collection PubMed
description Gastrointestinal amyloidosis is a condition caused by the deposition of extracellular protein fragments. It can be associated with complex and diverse pathways and can have numerous manifestations and etiologies. Hepatic amyloid light-chain (AL) amyloidosis is a rare disorder characterized by the deposition of the insoluble amyloid protein in the liver. The clinical presentations of AL amyloidosis are frequently non-specific. In this case report, we describe a patient with amyloidosis, who initially presented with an unusual case of severe intrahepatic cholestasis, which followed a rapidly progressive clinical course that was associated with the acute hypercalcemic crisis. The diagnosis of amyloidosis was made after the liver and bone biopsies were performed. Our findings revealed that AL amyloidosis should be considered, when a patient presents with cholestatic hepatitis, renal failure, and hypercalcemia.
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spelling pubmed-87433672022-01-10 Severe Obstructive Cholestasis and Hypercalcemia Caused by Light-Chain Amyloidosis: A Case Report Davoudi, Zahra Bidari, Farahnaz Jamali, Elena Nikpour, Shahriar Iran J Med Sci Case Report Gastrointestinal amyloidosis is a condition caused by the deposition of extracellular protein fragments. It can be associated with complex and diverse pathways and can have numerous manifestations and etiologies. Hepatic amyloid light-chain (AL) amyloidosis is a rare disorder characterized by the deposition of the insoluble amyloid protein in the liver. The clinical presentations of AL amyloidosis are frequently non-specific. In this case report, we describe a patient with amyloidosis, who initially presented with an unusual case of severe intrahepatic cholestasis, which followed a rapidly progressive clinical course that was associated with the acute hypercalcemic crisis. The diagnosis of amyloidosis was made after the liver and bone biopsies were performed. Our findings revealed that AL amyloidosis should be considered, when a patient presents with cholestatic hepatitis, renal failure, and hypercalcemia. Shiraz University of Medical Sciences 2022-01 /pmc/articles/PMC8743367/ /pubmed/35017780 http://dx.doi.org/10.30476/ijms.2021.88694.1942 Text en Copyright: © Iranian Journal of Medical Sciences https://creativecommons.org/licenses/by-nd/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 4.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Davoudi, Zahra
Bidari, Farahnaz
Jamali, Elena
Nikpour, Shahriar
Severe Obstructive Cholestasis and Hypercalcemia Caused by Light-Chain Amyloidosis: A Case Report
title Severe Obstructive Cholestasis and Hypercalcemia Caused by Light-Chain Amyloidosis: A Case Report
title_full Severe Obstructive Cholestasis and Hypercalcemia Caused by Light-Chain Amyloidosis: A Case Report
title_fullStr Severe Obstructive Cholestasis and Hypercalcemia Caused by Light-Chain Amyloidosis: A Case Report
title_full_unstemmed Severe Obstructive Cholestasis and Hypercalcemia Caused by Light-Chain Amyloidosis: A Case Report
title_short Severe Obstructive Cholestasis and Hypercalcemia Caused by Light-Chain Amyloidosis: A Case Report
title_sort severe obstructive cholestasis and hypercalcemia caused by light-chain amyloidosis: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8743367/
https://www.ncbi.nlm.nih.gov/pubmed/35017780
http://dx.doi.org/10.30476/ijms.2021.88694.1942
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