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Budd-Chiari syndrome in small hepatic veins: A diagnostic and therapeutic challenge in a low resource setting

INTRODUCTION: Budd-Chiari syndrome (BCS) is a rare condition affecting one in a million adults. BCS involves outflow obstruction in the hepatic venous system, which can occur anywhere between the small hepatic veins and the atrio-caval junction and cannot be due to heart, pericardial, or hepatic ven...

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Autores principales: Sultana, Emiko, Hasan, M. Tasdik, Mahmood, Hassan Rushekh, Ansar, Adnan, Podder, Vivek, Emrul, Khan, Ali, Md. Yusuf
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer - Medknow 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8483139/
https://www.ncbi.nlm.nih.gov/pubmed/34660462
http://dx.doi.org/10.4103/jfmpc.jfmpc_2213_20
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author Sultana, Emiko
Hasan, M. Tasdik
Mahmood, Hassan Rushekh
Ansar, Adnan
Podder, Vivek
Emrul, Khan
Ali, Md. Yusuf
author_facet Sultana, Emiko
Hasan, M. Tasdik
Mahmood, Hassan Rushekh
Ansar, Adnan
Podder, Vivek
Emrul, Khan
Ali, Md. Yusuf
author_sort Sultana, Emiko
collection PubMed
description INTRODUCTION: Budd-Chiari syndrome (BCS) is a rare condition affecting one in a million adults. BCS involves outflow obstruction in the hepatic venous system, which can occur anywhere between the small hepatic veins and the atrio-caval junction and cannot be due to heart, pericardial, or hepatic veno-occlusive disease. CASE PRESENTATION: We report an exceedingly rare form of BCS with less common initial clinical features in a young poor adult male patient which ignited a diagnostic uncertainty and a therapeutic challenge. The presence of the classical triad of BCS in the absence of major hepatic vein obstruction prompted the medical team to consider that the patient had a rare form of BCS. In this case, the financial condition of the patient and limited resources available restricted our ability to advance into the specific investigations. However, the patient was given symptomatic medical treatment and was followed up monthly. We also provided the patient with a statement that reaffirmed our inability to provide affordable surgical management options and called for an optimized national clinical guideline that could help the physicians deal with the challenges. CONCLUSION: An uncommon form of BCS in this patient provided a diagnostic challenge and therapeutic uncertainty in the low-resource settings. Primary care physicians should commence evidenced medical management based on clinical suspicion acknowledging the fact that obstruction of small hepatic veins is often not detected on an ultrasound.
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spelling pubmed-84831392021-10-14 Budd-Chiari syndrome in small hepatic veins: A diagnostic and therapeutic challenge in a low resource setting Sultana, Emiko Hasan, M. Tasdik Mahmood, Hassan Rushekh Ansar, Adnan Podder, Vivek Emrul, Khan Ali, Md. Yusuf J Family Med Prim Care Case Report INTRODUCTION: Budd-Chiari syndrome (BCS) is a rare condition affecting one in a million adults. BCS involves outflow obstruction in the hepatic venous system, which can occur anywhere between the small hepatic veins and the atrio-caval junction and cannot be due to heart, pericardial, or hepatic veno-occlusive disease. CASE PRESENTATION: We report an exceedingly rare form of BCS with less common initial clinical features in a young poor adult male patient which ignited a diagnostic uncertainty and a therapeutic challenge. The presence of the classical triad of BCS in the absence of major hepatic vein obstruction prompted the medical team to consider that the patient had a rare form of BCS. In this case, the financial condition of the patient and limited resources available restricted our ability to advance into the specific investigations. However, the patient was given symptomatic medical treatment and was followed up monthly. We also provided the patient with a statement that reaffirmed our inability to provide affordable surgical management options and called for an optimized national clinical guideline that could help the physicians deal with the challenges. CONCLUSION: An uncommon form of BCS in this patient provided a diagnostic challenge and therapeutic uncertainty in the low-resource settings. Primary care physicians should commence evidenced medical management based on clinical suspicion acknowledging the fact that obstruction of small hepatic veins is often not detected on an ultrasound. Wolters Kluwer - Medknow 2021-08 2021-08-27 /pmc/articles/PMC8483139/ /pubmed/34660462 http://dx.doi.org/10.4103/jfmpc.jfmpc_2213_20 Text en Copyright: © 2021 Journal of Family Medicine and Primary Care https://creativecommons.org/licenses/by-nc-sa/4.0/This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.
spellingShingle Case Report
Sultana, Emiko
Hasan, M. Tasdik
Mahmood, Hassan Rushekh
Ansar, Adnan
Podder, Vivek
Emrul, Khan
Ali, Md. Yusuf
Budd-Chiari syndrome in small hepatic veins: A diagnostic and therapeutic challenge in a low resource setting
title Budd-Chiari syndrome in small hepatic veins: A diagnostic and therapeutic challenge in a low resource setting
title_full Budd-Chiari syndrome in small hepatic veins: A diagnostic and therapeutic challenge in a low resource setting
title_fullStr Budd-Chiari syndrome in small hepatic veins: A diagnostic and therapeutic challenge in a low resource setting
title_full_unstemmed Budd-Chiari syndrome in small hepatic veins: A diagnostic and therapeutic challenge in a low resource setting
title_short Budd-Chiari syndrome in small hepatic veins: A diagnostic and therapeutic challenge in a low resource setting
title_sort budd-chiari syndrome in small hepatic veins: a diagnostic and therapeutic challenge in a low resource setting
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8483139/
https://www.ncbi.nlm.nih.gov/pubmed/34660462
http://dx.doi.org/10.4103/jfmpc.jfmpc_2213_20
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