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Small hepatic veins Budd-Chiari syndrome and paroxysmal nocturnal hemoglobinuria - The association of two rare entities: a case report

Small hepatic veins Budd-Chiari syndrome is a rare disorder characterized by hepatic venous outflow obstruction limited to the small intrahepatic veins, with normal appearance of the large hepatic veins at imaging. In this case only a liver biopsy can demonstrate the presence of a small vessels outf...

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Autores principales: Gioia, Stefania, De Santis, Emanuela, Cerbelli, Bruna, Nardelli, Silvia, Ridola, Lorenzo, De Santis, Adriano, d’Amati, Giulia, Riggio, Oliviero
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Pacini Editore srl 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7931566/
https://www.ncbi.nlm.nih.gov/pubmed/32760053
http://dx.doi.org/10.32074/1591-951X-4-20
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author Gioia, Stefania
De Santis, Emanuela
Cerbelli, Bruna
Nardelli, Silvia
Ridola, Lorenzo
De Santis, Adriano
d’Amati, Giulia
Riggio, Oliviero
author_facet Gioia, Stefania
De Santis, Emanuela
Cerbelli, Bruna
Nardelli, Silvia
Ridola, Lorenzo
De Santis, Adriano
d’Amati, Giulia
Riggio, Oliviero
author_sort Gioia, Stefania
collection PubMed
description Small hepatic veins Budd-Chiari syndrome is a rare disorder characterized by hepatic venous outflow obstruction limited to the small intrahepatic veins, with normal appearance of the large hepatic veins at imaging. In this case only a liver biopsy can demonstrate the presence of a small vessels outflow block. Paroxysmal nocturnal haemoglobinuria (PNH) is one of the most severe acquired thrombophilic state and represents one of the main aetiological factors of Budd-Chiari syndrome. In patient affected by PNH with liver impairment and/or ascites, Budd-Chiari syndrome must be always taken into consideration and, if necessary, a liver biopsy performed to exclude the small hepatic veins involvement. We report a case of small hepatic veins Budd–Chiari syndrome secondary to paroxysmal nocturnal haemoglobinuria.
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spelling pubmed-79315662021-07-08 Small hepatic veins Budd-Chiari syndrome and paroxysmal nocturnal hemoglobinuria - The association of two rare entities: a case report Gioia, Stefania De Santis, Emanuela Cerbelli, Bruna Nardelli, Silvia Ridola, Lorenzo De Santis, Adriano d’Amati, Giulia Riggio, Oliviero Pathologica Case Report Small hepatic veins Budd-Chiari syndrome is a rare disorder characterized by hepatic venous outflow obstruction limited to the small intrahepatic veins, with normal appearance of the large hepatic veins at imaging. In this case only a liver biopsy can demonstrate the presence of a small vessels outflow block. Paroxysmal nocturnal haemoglobinuria (PNH) is one of the most severe acquired thrombophilic state and represents one of the main aetiological factors of Budd-Chiari syndrome. In patient affected by PNH with liver impairment and/or ascites, Budd-Chiari syndrome must be always taken into consideration and, if necessary, a liver biopsy performed to exclude the small hepatic veins involvement. We report a case of small hepatic veins Budd–Chiari syndrome secondary to paroxysmal nocturnal haemoglobinuria. Pacini Editore srl 2020-06-01 /pmc/articles/PMC7931566/ /pubmed/32760053 http://dx.doi.org/10.32074/1591-951X-4-20 Text en © 2020 Copyright by Società Italiana di Anatomia Patologica e Citopatologia Diagnostica, Divisione Italiana della International Academy of Pathology https://creativecommons.org/licenses/by-nc-nd/4.0/deed.en This is an open access article distributed in accordance with the CC-BY-NC-ND (Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International) license. The article can be used by giving appropriate credit and mentioning the license, but only for non-commercial purposes and only in the original version. For further information: https://creativecommons.org/licenses/by-nc-nd/4.0/deed.en
spellingShingle Case Report
Gioia, Stefania
De Santis, Emanuela
Cerbelli, Bruna
Nardelli, Silvia
Ridola, Lorenzo
De Santis, Adriano
d’Amati, Giulia
Riggio, Oliviero
Small hepatic veins Budd-Chiari syndrome and paroxysmal nocturnal hemoglobinuria - The association of two rare entities: a case report
title Small hepatic veins Budd-Chiari syndrome and paroxysmal nocturnal hemoglobinuria - The association of two rare entities: a case report
title_full Small hepatic veins Budd-Chiari syndrome and paroxysmal nocturnal hemoglobinuria - The association of two rare entities: a case report
title_fullStr Small hepatic veins Budd-Chiari syndrome and paroxysmal nocturnal hemoglobinuria - The association of two rare entities: a case report
title_full_unstemmed Small hepatic veins Budd-Chiari syndrome and paroxysmal nocturnal hemoglobinuria - The association of two rare entities: a case report
title_short Small hepatic veins Budd-Chiari syndrome and paroxysmal nocturnal hemoglobinuria - The association of two rare entities: a case report
title_sort small hepatic veins budd-chiari syndrome and paroxysmal nocturnal hemoglobinuria - the association of two rare entities: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7931566/
https://www.ncbi.nlm.nih.gov/pubmed/32760053
http://dx.doi.org/10.32074/1591-951X-4-20
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