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POEMS syndrome, calciphylaxis and focal segmental glomerulosclerosis – VEGF as a possible link
BACKGROUND: Polyneuropathy organomegaly endocrinopathy M-protein skin changes (POEMS) syndrome is a rare cause of polyneuropathy. Calciphylaxis, a severe disease leading to necrotic ulcers of the skin, is associated with POEMS syndrome and also with renal disease. This case report describes a patien...
Autores principales: | , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
BioMed Central
2014
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4226878/ https://www.ncbi.nlm.nih.gov/pubmed/25369758 http://dx.doi.org/10.1186/s12883-014-0210-3 |
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author | Heck, Dorothee Mergen, Miriam Ganner, Athina Pelisek, Jaroslav Mader, Irina Weiller, Cornelius Niesen, Wolf-Dirk |
author_facet | Heck, Dorothee Mergen, Miriam Ganner, Athina Pelisek, Jaroslav Mader, Irina Weiller, Cornelius Niesen, Wolf-Dirk |
author_sort | Heck, Dorothee |
collection | PubMed |
description | BACKGROUND: Polyneuropathy organomegaly endocrinopathy M-protein skin changes (POEMS) syndrome is a rare cause of polyneuropathy. Calciphylaxis, a severe disease leading to necrotic ulcers of the skin, is associated with POEMS syndrome and also with renal disease. This case report describes a patient with POEMS syndrome plus primary focal segmental glomerulosclerosis. CASE PRESENTATION: A 27-year-old Caucasian woman with chronic renal insufficiency due to focal segmental glomerulosclerosis and calciphylaxis presented to our institution with polyneuropathy and encephalopathy. An extensive diagnostic workup revealed POEMS syndrome. Serum concentrations of vascular endothelial growth factor (VEGF) were highly elevated, consistent with POEMS syndrome. CONCLUSION: To our knowledge, this is the first report of a patient with POEMS syndrome and primary focal segmental glomerulosclerosis. The combination of POEMS syndrome, calciphylaxis and primary focal segmental glomerulosclerosis may be coincidental, suggesting the need for additional studies to confirm or exclude this association. VEGF may be an important pathogenetic link, suggesting that treatment with antiangiogenic agents may improve patient outcomes. |
format | Online Article Text |
id | pubmed-4226878 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2014 |
publisher | BioMed Central |
record_format | MEDLINE/PubMed |
spelling | pubmed-42268782014-11-12 POEMS syndrome, calciphylaxis and focal segmental glomerulosclerosis – VEGF as a possible link Heck, Dorothee Mergen, Miriam Ganner, Athina Pelisek, Jaroslav Mader, Irina Weiller, Cornelius Niesen, Wolf-Dirk BMC Neurol Case Report BACKGROUND: Polyneuropathy organomegaly endocrinopathy M-protein skin changes (POEMS) syndrome is a rare cause of polyneuropathy. Calciphylaxis, a severe disease leading to necrotic ulcers of the skin, is associated with POEMS syndrome and also with renal disease. This case report describes a patient with POEMS syndrome plus primary focal segmental glomerulosclerosis. CASE PRESENTATION: A 27-year-old Caucasian woman with chronic renal insufficiency due to focal segmental glomerulosclerosis and calciphylaxis presented to our institution with polyneuropathy and encephalopathy. An extensive diagnostic workup revealed POEMS syndrome. Serum concentrations of vascular endothelial growth factor (VEGF) were highly elevated, consistent with POEMS syndrome. CONCLUSION: To our knowledge, this is the first report of a patient with POEMS syndrome and primary focal segmental glomerulosclerosis. The combination of POEMS syndrome, calciphylaxis and primary focal segmental glomerulosclerosis may be coincidental, suggesting the need for additional studies to confirm or exclude this association. VEGF may be an important pathogenetic link, suggesting that treatment with antiangiogenic agents may improve patient outcomes. BioMed Central 2014-11-05 /pmc/articles/PMC4226878/ /pubmed/25369758 http://dx.doi.org/10.1186/s12883-014-0210-3 Text en © Heck et al.; licensee BioMed Central Ltd. 2014 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 credited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated. |
spellingShingle | Case Report Heck, Dorothee Mergen, Miriam Ganner, Athina Pelisek, Jaroslav Mader, Irina Weiller, Cornelius Niesen, Wolf-Dirk POEMS syndrome, calciphylaxis and focal segmental glomerulosclerosis – VEGF as a possible link |
title | POEMS syndrome, calciphylaxis and focal segmental glomerulosclerosis – VEGF as a possible link |
title_full | POEMS syndrome, calciphylaxis and focal segmental glomerulosclerosis – VEGF as a possible link |
title_fullStr | POEMS syndrome, calciphylaxis and focal segmental glomerulosclerosis – VEGF as a possible link |
title_full_unstemmed | POEMS syndrome, calciphylaxis and focal segmental glomerulosclerosis – VEGF as a possible link |
title_short | POEMS syndrome, calciphylaxis and focal segmental glomerulosclerosis – VEGF as a possible link |
title_sort | poems syndrome, calciphylaxis and focal segmental glomerulosclerosis – vegf as a possible link |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4226878/ https://www.ncbi.nlm.nih.gov/pubmed/25369758 http://dx.doi.org/10.1186/s12883-014-0210-3 |
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