Hepatic Necrosis Mimicking Infiltrative Masses in Acute Budd-Chiari Syndrome With Hereditary Protein C Deficiency

We report the case of a patient with an unusual acute Budd-Chiari syndrome (BCS). The patient presented with high-grade fever and right upper quadrant pain. Infiltrative lesions at the right hepatic lobe and segment IVB with intrahepatic inferior vena cava and right hepatic vein thrombus appeared on...

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Autores principales: Ananchuensook, Prooksa, Karuehardsuwan, Julalak, Sanpawat, Anapat, Wisedopas, Naruemon, Treeprasertsuk, Sombat, Komolmit, Piyawat, Thanapirom, Kessarin
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer 2022
Materias:
Case Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9232359/
https://www.ncbi.nlm.nih.gov/pubmed/35765684
http://dx.doi.org/10.14309/crj.0000000000000802
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author Ananchuensook, Prooksa
Karuehardsuwan, Julalak
Sanpawat, Anapat
Wisedopas, Naruemon
Treeprasertsuk, Sombat
Komolmit, Piyawat
Thanapirom, Kessarin
author_facet Ananchuensook, Prooksa
Karuehardsuwan, Julalak
Sanpawat, Anapat
Wisedopas, Naruemon
Treeprasertsuk, Sombat
Komolmit, Piyawat
Thanapirom, Kessarin
author_sort Ananchuensook, Prooksa
collection PubMed
description We report the case of a patient with an unusual acute Budd-Chiari syndrome (BCS). The patient presented with high-grade fever and right upper quadrant pain. Infiltrative lesions at the right hepatic lobe and segment IVB with intrahepatic inferior vena cava and right hepatic vein thrombus appeared on abdominal imaging. Liver biopsy revealed hepatic infarction compatible with acute BCS. Thrombophilia work-up demonstrated low protein C activity with the -1657C/T mutation of the PROC gene. Necrotic liver mass with acute BCS related to congenital protein C deficiency was diagnosed. Patient symptoms and necrotic masses improved after anticoagulant treatment for 4 months.
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spelling pubmed-92323592022-06-27 Hepatic Necrosis Mimicking Infiltrative Masses in Acute Budd-Chiari Syndrome With Hereditary Protein C Deficiency Ananchuensook, Prooksa Karuehardsuwan, Julalak Sanpawat, Anapat Wisedopas, Naruemon Treeprasertsuk, Sombat Komolmit, Piyawat Thanapirom, Kessarin ACG Case Rep J Case Report We report the case of a patient with an unusual acute Budd-Chiari syndrome (BCS). The patient presented with high-grade fever and right upper quadrant pain. Infiltrative lesions at the right hepatic lobe and segment IVB with intrahepatic inferior vena cava and right hepatic vein thrombus appeared on abdominal imaging. Liver biopsy revealed hepatic infarction compatible with acute BCS. Thrombophilia work-up demonstrated low protein C activity with the -1657C/T mutation of the PROC gene. Necrotic liver mass with acute BCS related to congenital protein C deficiency was diagnosed. Patient symptoms and necrotic masses improved after anticoagulant treatment for 4 months. Wolters Kluwer 2022-06-24 /pmc/articles/PMC9232359/ /pubmed/35765684 http://dx.doi.org/10.14309/crj.0000000000000802 Text en © 2022 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of The American College of Gastroenterology. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND) (https://creativecommons.org/licenses/by-nc-nd/4.0/) , where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.
spellingShingle Case Report
Ananchuensook, Prooksa
Karuehardsuwan, Julalak
Sanpawat, Anapat
Wisedopas, Naruemon
Treeprasertsuk, Sombat
Komolmit, Piyawat
Thanapirom, Kessarin
Hepatic Necrosis Mimicking Infiltrative Masses in Acute Budd-Chiari Syndrome With Hereditary Protein C Deficiency
title Hepatic Necrosis Mimicking Infiltrative Masses in Acute Budd-Chiari Syndrome With Hereditary Protein C Deficiency
title_full Hepatic Necrosis Mimicking Infiltrative Masses in Acute Budd-Chiari Syndrome With Hereditary Protein C Deficiency
title_fullStr Hepatic Necrosis Mimicking Infiltrative Masses in Acute Budd-Chiari Syndrome With Hereditary Protein C Deficiency
title_full_unstemmed Hepatic Necrosis Mimicking Infiltrative Masses in Acute Budd-Chiari Syndrome With Hereditary Protein C Deficiency
title_short Hepatic Necrosis Mimicking Infiltrative Masses in Acute Budd-Chiari Syndrome With Hereditary Protein C Deficiency
title_sort hepatic necrosis mimicking infiltrative masses in acute budd-chiari syndrome with hereditary protein c deficiency
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9232359/
https://www.ncbi.nlm.nih.gov/pubmed/35765684
http://dx.doi.org/10.14309/crj.0000000000000802
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