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Variegate porphyria complicated by systemic AA amyloidosis: a case report
We report a Japanese woman with variegate porphyria accompanied by amyloid A (AA) amyloidosis. Arthropathy involving multiple joints occurred at 35 years old and persisted. C-reactive protein was 4.0 mg/dL, but rheumatoid factor was negative. Radiographs did not reveal any loss or narrowing of the j...
Autores principales: | , , , , , , , , , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Informa UK Ltd.
2013
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3836393/ https://www.ncbi.nlm.nih.gov/pubmed/24131077 http://dx.doi.org/10.3109/13506129.2013.837390 |
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author | Tsuchiya, Yoshiki Hoshino, Junichi Suwabe, Tatsuya Sumida, Keiichi Hiramatsu, Rikako Mise, Koki Hasegawa, Eiko Yamanouchi, Masayuki Hayami, Noriko Sawa, Naoki Arizono, Kenji Hara, Shigeko Takaichi, Kenmei Fujii, Takeshi Ubara, Yoshifumi |
author_facet | Tsuchiya, Yoshiki Hoshino, Junichi Suwabe, Tatsuya Sumida, Keiichi Hiramatsu, Rikako Mise, Koki Hasegawa, Eiko Yamanouchi, Masayuki Hayami, Noriko Sawa, Naoki Arizono, Kenji Hara, Shigeko Takaichi, Kenmei Fujii, Takeshi Ubara, Yoshifumi |
author_sort | Tsuchiya, Yoshiki |
collection | PubMed |
description | We report a Japanese woman with variegate porphyria accompanied by amyloid A (AA) amyloidosis. Arthropathy involving multiple joints occurred at 35 years old and persisted. C-reactive protein was 4.0 mg/dL, but rheumatoid factor was negative. Radiographs did not reveal any loss or narrowing of the joint spaces. Two years later, blister formation after sun exposure and reddish urine were first noted. At the age of 45 years, she developed abdominal pain, nausea, vomiting and seizures. After administration of phenobarbital, reddish urine was noted and muscular weakness progressed to atonic quadraparesis. Porphyria attack was diagnosed from high urinary levels of ∂ aminolevulinic acid and porphobilinogen. At the age of 47 years, hemodialysis was started. At the age of 49 years, progression of her gastrointestinal event resulted in death. Autopsy showed massive deposits of AA amyloidosis in various organs, including the kidneys and digestive tract. Thus, amyloid deposition may have contributed to both end-stage renal failure and her gastrointestinal symptoms. This is the first report about the coexistence of porphyria and AA amyloidosis. Chronic inflammation related to this patient’s seronegative arthropathy, although atypical for porphyria, might have contributed to the development of AA amyloidosis. |
format | Online Article Text |
id | pubmed-3836393 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2013 |
publisher | Informa UK Ltd. |
record_format | MEDLINE/PubMed |
spelling | pubmed-38363932013-11-22 Variegate porphyria complicated by systemic AA amyloidosis: a case report Tsuchiya, Yoshiki Hoshino, Junichi Suwabe, Tatsuya Sumida, Keiichi Hiramatsu, Rikako Mise, Koki Hasegawa, Eiko Yamanouchi, Masayuki Hayami, Noriko Sawa, Naoki Arizono, Kenji Hara, Shigeko Takaichi, Kenmei Fujii, Takeshi Ubara, Yoshifumi Amyloid Case Report We report a Japanese woman with variegate porphyria accompanied by amyloid A (AA) amyloidosis. Arthropathy involving multiple joints occurred at 35 years old and persisted. C-reactive protein was 4.0 mg/dL, but rheumatoid factor was negative. Radiographs did not reveal any loss or narrowing of the joint spaces. Two years later, blister formation after sun exposure and reddish urine were first noted. At the age of 45 years, she developed abdominal pain, nausea, vomiting and seizures. After administration of phenobarbital, reddish urine was noted and muscular weakness progressed to atonic quadraparesis. Porphyria attack was diagnosed from high urinary levels of ∂ aminolevulinic acid and porphobilinogen. At the age of 47 years, hemodialysis was started. At the age of 49 years, progression of her gastrointestinal event resulted in death. Autopsy showed massive deposits of AA amyloidosis in various organs, including the kidneys and digestive tract. Thus, amyloid deposition may have contributed to both end-stage renal failure and her gastrointestinal symptoms. This is the first report about the coexistence of porphyria and AA amyloidosis. Chronic inflammation related to this patient’s seronegative arthropathy, although atypical for porphyria, might have contributed to the development of AA amyloidosis. Informa UK Ltd. 2013-12 2013-10-16 /pmc/articles/PMC3836393/ /pubmed/24131077 http://dx.doi.org/10.3109/13506129.2013.837390 Text en © 2013 Informa UK Ltd. All rights reserved: reproduction in whole or part not permitted http://creativecommons.org/licenses/by-nc/3.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution Noncommercial License which permits any noncommercial use, distribution, and reproduction in any medium, provided the source is credited. |
spellingShingle | Case Report Tsuchiya, Yoshiki Hoshino, Junichi Suwabe, Tatsuya Sumida, Keiichi Hiramatsu, Rikako Mise, Koki Hasegawa, Eiko Yamanouchi, Masayuki Hayami, Noriko Sawa, Naoki Arizono, Kenji Hara, Shigeko Takaichi, Kenmei Fujii, Takeshi Ubara, Yoshifumi Variegate porphyria complicated by systemic AA amyloidosis: a case report |
title | Variegate porphyria complicated by systemic AA amyloidosis: a case report |
title_full | Variegate porphyria complicated by systemic AA amyloidosis: a case report |
title_fullStr | Variegate porphyria complicated by systemic AA amyloidosis: a case report |
title_full_unstemmed | Variegate porphyria complicated by systemic AA amyloidosis: a case report |
title_short | Variegate porphyria complicated by systemic AA amyloidosis: a case report |
title_sort | variegate porphyria complicated by systemic aa amyloidosis: a case report |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3836393/ https://www.ncbi.nlm.nih.gov/pubmed/24131077 http://dx.doi.org/10.3109/13506129.2013.837390 |
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