Cargando…
Primary Systemic Amyloidosis: A Case Report
Primary systemic amyloidosis is a systemic disease characterised by the deposition of misfolded proteins extracellularly in different organs without any known cause in the background, eventually leading to multiorgan dysfunction and death. The incidence of primary amyloidosis is estimated at 5.1-12....
Autores principales: | , , , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Journal of the Nepal Medical Association
2023
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10579773/ http://dx.doi.org/10.31729/jnma.8297 |
_version_ | 1785121798612844544 |
---|---|
author | Oli, Saurav Sen Jha, Abhishek Karki, Anisha Sapkota, Shova Adhikari, Laxman |
author_facet | Oli, Saurav Sen Jha, Abhishek Karki, Anisha Sapkota, Shova Adhikari, Laxman |
author_sort | Oli, Saurav Sen |
collection | PubMed |
description | Primary systemic amyloidosis is a systemic disease characterised by the deposition of misfolded proteins extracellularly in different organs without any known cause in the background, eventually leading to multiorgan dysfunction and death. The incidence of primary amyloidosis is estimated at 5.1-12.8 cases per million, with a poor prognosis. We report a case of a 69-year male with lower back pain, shortness of breath, and anasarca diagnosed as primary systemic amyloidosis by serumfree light chain assay and kidney needle biopsy. He was started on intravenous bortezomib and dexamethasone. Though he adhered to his medications, with time he developed renal insufficiency marked by azotemia following which hemodialysis was performed. Primary systemic amyloidosis is a rare clinical condition with a very poor prognosis. Further studies are needed to understand the proper pathophysiology and treatment of the disease. |
format | Online Article Text |
id | pubmed-10579773 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | Journal of the Nepal Medical Association |
record_format | MEDLINE/PubMed |
spelling | pubmed-105797732023-10-18 Primary Systemic Amyloidosis: A Case Report Oli, Saurav Sen Jha, Abhishek Karki, Anisha Sapkota, Shova Adhikari, Laxman JNMA J Nepal Med Assoc Case Report Primary systemic amyloidosis is a systemic disease characterised by the deposition of misfolded proteins extracellularly in different organs without any known cause in the background, eventually leading to multiorgan dysfunction and death. The incidence of primary amyloidosis is estimated at 5.1-12.8 cases per million, with a poor prognosis. We report a case of a 69-year male with lower back pain, shortness of breath, and anasarca diagnosed as primary systemic amyloidosis by serumfree light chain assay and kidney needle biopsy. He was started on intravenous bortezomib and dexamethasone. Though he adhered to his medications, with time he developed renal insufficiency marked by azotemia following which hemodialysis was performed. Primary systemic amyloidosis is a rare clinical condition with a very poor prognosis. Further studies are needed to understand the proper pathophysiology and treatment of the disease. Journal of the Nepal Medical Association 2023-10 2023-10-31 /pmc/articles/PMC10579773/ http://dx.doi.org/10.31729/jnma.8297 Text en © The Author(s) 2018. https://creativecommons.org/licenses/by/4.0/This is an Open-Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0) which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Case Report Oli, Saurav Sen Jha, Abhishek Karki, Anisha Sapkota, Shova Adhikari, Laxman Primary Systemic Amyloidosis: A Case Report |
title | Primary Systemic Amyloidosis: A Case Report |
title_full | Primary Systemic Amyloidosis: A Case Report |
title_fullStr | Primary Systemic Amyloidosis: A Case Report |
title_full_unstemmed | Primary Systemic Amyloidosis: A Case Report |
title_short | Primary Systemic Amyloidosis: A Case Report |
title_sort | primary systemic amyloidosis: a case report |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10579773/ http://dx.doi.org/10.31729/jnma.8297 |
work_keys_str_mv | AT olisauravsen primarysystemicamyloidosisacasereport AT jhaabhishek primarysystemicamyloidosisacasereport AT karkianisha primarysystemicamyloidosisacasereport AT sapkotashova primarysystemicamyloidosisacasereport AT adhikarilaxman primarysystemicamyloidosisacasereport |