A Budd-Chiari Syndrome Due to C Protein Deficiency: A Case Report at YaoundéGeneral Hospital (Cameroon)  

作　　者：Antonin Wilson Ndjitoyap Ndam Gilles Gael Aghoagni Gouajio Armel Awana Tenone Danah Larry Tangie Ngek Mathurin Kowo Firmin Andoulo Ankouane Elie Claude Ndjitoyap Ndam Antonin Wilson Ndjitoyap Ndam;Gilles Gael Aghoagni Gouajio;Armel Awana;Tenone Danah;Larry Tangie Ngek;Mathurin Kowo;Firmin Andoulo Ankouane;Elie Claude Ndjitoyap Ndam(Department of Internal Medicine, Faculty of Medicine and Biomedical Sciences, University of Yaoundé 1, Yaoundé, Came-roon;Department of Internal Medicine and Peadiatrics, Faculty of Health Sciences, University of Buéa, Buéa, Cameroon;Department of Biomedical Sciences, Faculty of Sciences, University of Ngaoundéré, Ngaoundéré, Cameroon)

机构地区：[1]Department of Internal Medicine, Faculty of Medicine and Biomedical Sciences, University of Yaoundé 1, Yaoundé, Came-roon [2]Department of Internal Medicine and Peadiatrics, Faculty of Health Sciences, University of Buéa, Buéa, Cameroon [3]Department of Biomedical Sciences, Faculty of Sciences, University of Ngaoundéré, Ngaoundéré, Cameroon

出　　处：《Open Journal of Gastroenterology》2024年第4期117-124,共8页肠胃病学期刊（英文）

摘　　要：Primary Budd-Chiari syndrome (BCS) is a spontaneously fatal disease characterized by an obstruction of the hepatic venous outflow tract due to thrombosis or a primary disease of the venous wall. The primary form of BCS is extremely rare. This is a disease mainly affecting young adults of both sexes. Clinical manifestations are variable;they can be asymptomatic, acute, or subacute but mostly chronic. Several causes have been identified, such as myeloproliferative syndrome, antiphospholipid syndrome, paroxysmal nocturnal hemoglobinuria, and inherited thrombotic disorders. Data on primary BCS in Sub-Saharan Africa is rare as most publications available are case reports. In these reports, the causes are unknown with poor prognosis in most cases often leading to patient death. We herein present a case report of a male patient diagnosed with a primary BCS at Yaoundé General Hospital (Cameroon) caused by a Protein C deficiency who presented with ascites decompensating liver cirrhosis. Treatment was based on anticoagulants, diuretics and laxatives administration. Two years after the diagnosis, the patient is alive with clinical and paraclinical improvement.Primary Budd-Chiari syndrome (BCS) is a spontaneously fatal disease characterized by an obstruction of the hepatic venous outflow tract due to thrombosis or a primary disease of the venous wall. The primary form of BCS is extremely rare. This is a disease mainly affecting young adults of both sexes. Clinical manifestations are variable;they can be asymptomatic, acute, or subacute but mostly chronic. Several causes have been identified, such as myeloproliferative syndrome, antiphospholipid syndrome, paroxysmal nocturnal hemoglobinuria, and inherited thrombotic disorders. Data on primary BCS in Sub-Saharan Africa is rare as most publications available are case reports. In these reports, the causes are unknown with poor prognosis in most cases often leading to patient death. We herein present a case report of a male patient diagnosed with a primary BCS at Yaoundé General Hospital (Cameroon) caused by a Protein C deficiency who presented with ascites decompensating liver cirrhosis. Treatment was based on anticoagulants, diuretics and laxatives administration. Two years after the diagnosis, the patient is alive with clinical and paraclinical improvement.

关 键 词：Budd-Chiari Syndrome Hepatic Veins Liver Cirrhosis Protein C Deficiency Cameroon 

分 类 号：R73[医药卫生—肿瘤]