The First Case of TEMPI Syndrome in Japan

TEMPI syndrome, a disease entity comprising telangiectasia, erythrocytosis with high erythropoietin, monoclonal gammopathy, perinephric fluid collection, and intrapulmonary shunting, was first described by Sykes et al. in 2011. To our knowledge, only 15 cases have been reported worldwide, none of wh...

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Autores principales: Shizuku, Tatsunori, Matsui, Keiji, Yagi, Shinya, Iwabuchi, Shogo
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Japanese Society of Internal Medicine 2020
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7434545/
https://www.ncbi.nlm.nih.gov/pubmed/32295999
http://dx.doi.org/10.2169/internalmedicine.3547-19
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author Shizuku, Tatsunori
Matsui, Keiji
Yagi, Shinya
Iwabuchi, Shogo
author_facet Shizuku, Tatsunori
Matsui, Keiji
Yagi, Shinya
Iwabuchi, Shogo
author_sort Shizuku, Tatsunori
collection PubMed
description TEMPI syndrome, a disease entity comprising telangiectasia, erythrocytosis with high erythropoietin, monoclonal gammopathy, perinephric fluid collection, and intrapulmonary shunting, was first described by Sykes et al. in 2011. To our knowledge, only 15 cases have been reported worldwide, none of which were in Japan. We herein report a 47-year-old man who had intractable ascites for 2 and a half years and was referred to our department for a peritoneovenous shunt. In addition to ascites, he had telangiectasia, high erythropoietin, monoclonal gammopathy, and perinephric fluid collection. Thus, this is the first case of TEMPI syndrome in Japan.
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spelling pubmed-74345452020-08-27 The First Case of TEMPI Syndrome in Japan Shizuku, Tatsunori Matsui, Keiji Yagi, Shinya Iwabuchi, Shogo Intern Med Case Report TEMPI syndrome, a disease entity comprising telangiectasia, erythrocytosis with high erythropoietin, monoclonal gammopathy, perinephric fluid collection, and intrapulmonary shunting, was first described by Sykes et al. in 2011. To our knowledge, only 15 cases have been reported worldwide, none of which were in Japan. We herein report a 47-year-old man who had intractable ascites for 2 and a half years and was referred to our department for a peritoneovenous shunt. In addition to ascites, he had telangiectasia, high erythropoietin, monoclonal gammopathy, and perinephric fluid collection. Thus, this is the first case of TEMPI syndrome in Japan. The Japanese Society of Internal Medicine 2020-04-16 2020-07-15 /pmc/articles/PMC7434545/ /pubmed/32295999 http://dx.doi.org/10.2169/internalmedicine.3547-19 Text en Copyright © 2020 by The Japanese Society of Internal Medicine https://creativecommons.org/licenses/by-nc-nd/4.0/ The Internal Medicine is an Open Access journal distributed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License. To view the details of this license, please visit (https://creativecommons.org/licenses/by-nc-nd/4.0/).
spellingShingle Case Report
Shizuku, Tatsunori
Matsui, Keiji
Yagi, Shinya
Iwabuchi, Shogo
The First Case of TEMPI Syndrome in Japan
title The First Case of TEMPI Syndrome in Japan
title_full The First Case of TEMPI Syndrome in Japan
title_fullStr The First Case of TEMPI Syndrome in Japan
title_full_unstemmed The First Case of TEMPI Syndrome in Japan
title_short The First Case of TEMPI Syndrome in Japan
title_sort first case of tempi syndrome in japan
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7434545/
https://www.ncbi.nlm.nih.gov/pubmed/32295999
http://dx.doi.org/10.2169/internalmedicine.3547-19
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