Difference of Budd-Chiari syndrome between West and China
Xingshun Qi, M.D.1,2, Xiaozhong Guo, M.D., PH.D.1*, Daiming Fan, M.D., PH.D.2
1
Department of Gastroenterology, General Hospital of Shenyang Military Area, Shenyang,
110840 China. 2
Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi’an, 710032
China.
*Correspondence to: Prof. Xiaozhong Guo, M.D., PH.D., Department of Gastroenterology, General Hospital of Shenyang Military Area, No. 83 Wenhua Road, Shenyang, 110840 China. Tel: 86-24-28897603; Fax: 86-24-28851113; Email: [email protected]
Conflict of interest: None
Funding: None
Word count: 500
References: 7
This article has been accepted for publication and undergone full peer review but has not been through the copyediting, typesetting, pagination and proofreading process which may lead to differences between this version and the Version of Record. Please cite this article as doi: 10.1002/hep.27628 This article is protected by copyright. All rights reserved.
Hepatology
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Page 2 of 4
To the Editor: We read with great interest the review by Wang and colleagues that comprehensively analyzed the similarity and discrepancy in the epidemiology and characteristics of liver diseases between China and Western countries (1). Several common liver diseases were discussed, such as viral hepatitis, alcoholic liver disease, nonalcoholic fatty liver diseases, liver cirrhosis, and hepatocellular carcinoma. Herein, we would like to express additional concern about Budd-Chiari syndrome (BCS) which is a vascular liver disease resulting in life-threatening liver failure and portal hypertension-related complications (2).
The sites of occlusion, clinical presentations, etiological distributions, and preferred treatment modalities of BCS may be different between Western and Chinese populations. First, the occlusion is often located at the hepatic veins in West. By comparison, a combined occlusion of hepatic vein and inferior vena cava is the most frequent type of BCS in China. Second, most of Western patients have a rapid disease course with progressive hepatic function impairment due to acute thrombosis within the hepatic veins. However, a majority of Chinese patients has a relatively long history of abdominal wall varices and lower limb edema due to chronic occlusion of the inferior vena cava (3). Third, approximately 80% of Western patients have at least one thrombotic risk factor (4), such as myeloproliferative neoplasms with or without JAK2 V617F mutation, factor V Leiden mutation, prothrombin G20210A mutation, and paroxysmal nocturnal hemoglobinuria, etc. In contrast, they are less frequently observed in Chinese patients (5-6). Fourth, anticoagulation and transjugular intrahepatic portosystemic shunt are the mainstay treatment options for Western patients. Contrarily, our recent survey of 23352
2
Hepatology This article is protected by copyright. All rights reserved.
Page 3 of 4
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Hepatology
Chinese BCS patients suggests that percutaneous recanalization is the most common treatment modality (7). This is because percutaneous recanalization alone can achieve an excellent long-term patency and survival in most of Chinese BCS patients.
Collectively, the difference in the characteristics of BCS between West and China should be clearly recognized by Chinese hepatologists.
References 1.
Wang FS, Fan JG, Zhang Z, Gao B, Wang HY. The global burden of liver disease: The
major impact of China. Hepatology 2014. 2.
DeLeve LD, Valla DC, Garcia-Tsao G. Vascular disorders of the liver. Hepatology
2009;49:1729-1764. 3.
Qi X, Han G. Images in clinical medicine. Abdominal-wall varices in the Budd-Chiari
syndrome. N Engl J Med 2014;370:1829. 4.
Darwish Murad S, Plessier A, Hernandez-Guerra M, Fabris F, Eapen CE, Bahr MJ,
Trebicka J, et al. Etiology, management, and outcome of the Budd-Chiari syndrome. Ann Intern Med 2009;151:167-175. 5.
Qi X, Wu F, Ren W, He C, Yin Z, Niu J, Bai M, et al. Thrombotic risk factors in Chinese
Budd-Chiari syndrome patients. An observational study with a systematic review of the literature. Thromb Haemost 2013;109:878-884. 6.
Wang H, Sun G, Zhang P, Zhang J, Gui E, Zu M, Jia E, et al. JAK2 V617F mutation and
46/1 haplotype in Chinese Budd-Chiari syndrome patients. J Gastroenterol Hepatol
3
Hepatology This article is protected by copyright. All rights reserved.
Hepatology
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 43 44 45 46 47 48 49 50 51 52 53 54 55 56 57 58 59 60
Page 4 of 4
2014;29:208-214. 7.
Qi XS, Ren WR, Fan DM, Han GH. Selection of treatment modalities for Budd-Chiari
Syndrome in China: a preliminary survey of published literature. World J Gastroenterol 2014;20:10628-10636.
4
Hepatology This article is protected by copyright. All rights reserved.
Xingshun Qi, M.D.1,2, Xiaozhong Guo, M.D., PH.D.1*, Daiming Fan, M.D., PH.D.2
1
Department of Gastroenterology, General Hospital of Shenyang Military Area, Shenyang,
110840 China. 2
Xijing Hospital of Digestive Diseases, Fourth Military Medical University, Xi’an, 710032
China.
*Correspondence to: Prof. Xiaozhong Guo, M.D., PH.D., Department of Gastroenterology, General Hospital of Shenyang Military Area, No. 83 Wenhua Road, Shenyang, 110840 China. Tel: 86-24-28897603; Fax: 86-24-28851113; Email: [email protected]
Conflict of interest: None
Funding: None
Word count: 500
References: 7
This article has been accepted for publication and undergone full peer review but has not been through the copyediting, typesetting, pagination and proofreading process which may lead to differences between this version and the Version of Record. Please cite this article as doi: 10.1002/hep.27628 This article is protected by copyright. All rights reserved.
Hepatology
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 43 44 45 46 47 48 49 50 51 52 53 54 55 56 57 58 59 60
Page 2 of 4
To the Editor: We read with great interest the review by Wang and colleagues that comprehensively analyzed the similarity and discrepancy in the epidemiology and characteristics of liver diseases between China and Western countries (1). Several common liver diseases were discussed, such as viral hepatitis, alcoholic liver disease, nonalcoholic fatty liver diseases, liver cirrhosis, and hepatocellular carcinoma. Herein, we would like to express additional concern about Budd-Chiari syndrome (BCS) which is a vascular liver disease resulting in life-threatening liver failure and portal hypertension-related complications (2).
The sites of occlusion, clinical presentations, etiological distributions, and preferred treatment modalities of BCS may be different between Western and Chinese populations. First, the occlusion is often located at the hepatic veins in West. By comparison, a combined occlusion of hepatic vein and inferior vena cava is the most frequent type of BCS in China. Second, most of Western patients have a rapid disease course with progressive hepatic function impairment due to acute thrombosis within the hepatic veins. However, a majority of Chinese patients has a relatively long history of abdominal wall varices and lower limb edema due to chronic occlusion of the inferior vena cava (3). Third, approximately 80% of Western patients have at least one thrombotic risk factor (4), such as myeloproliferative neoplasms with or without JAK2 V617F mutation, factor V Leiden mutation, prothrombin G20210A mutation, and paroxysmal nocturnal hemoglobinuria, etc. In contrast, they are less frequently observed in Chinese patients (5-6). Fourth, anticoagulation and transjugular intrahepatic portosystemic shunt are the mainstay treatment options for Western patients. Contrarily, our recent survey of 23352
2
Hepatology This article is protected by copyright. All rights reserved.
Page 3 of 4
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 43 44 45 46 47 48 49 50 51 52 53 54 55 56 57 58 59 60
Hepatology
Chinese BCS patients suggests that percutaneous recanalization is the most common treatment modality (7). This is because percutaneous recanalization alone can achieve an excellent long-term patency and survival in most of Chinese BCS patients.
Collectively, the difference in the characteristics of BCS between West and China should be clearly recognized by Chinese hepatologists.
References 1.
Wang FS, Fan JG, Zhang Z, Gao B, Wang HY. The global burden of liver disease: The
major impact of China. Hepatology 2014. 2.
DeLeve LD, Valla DC, Garcia-Tsao G. Vascular disorders of the liver. Hepatology
2009;49:1729-1764. 3.
Qi X, Han G. Images in clinical medicine. Abdominal-wall varices in the Budd-Chiari
syndrome. N Engl J Med 2014;370:1829. 4.
Darwish Murad S, Plessier A, Hernandez-Guerra M, Fabris F, Eapen CE, Bahr MJ,
Trebicka J, et al. Etiology, management, and outcome of the Budd-Chiari syndrome. Ann Intern Med 2009;151:167-175. 5.
Qi X, Wu F, Ren W, He C, Yin Z, Niu J, Bai M, et al. Thrombotic risk factors in Chinese
Budd-Chiari syndrome patients. An observational study with a systematic review of the literature. Thromb Haemost 2013;109:878-884. 6.
Wang H, Sun G, Zhang P, Zhang J, Gui E, Zu M, Jia E, et al. JAK2 V617F mutation and
46/1 haplotype in Chinese Budd-Chiari syndrome patients. J Gastroenterol Hepatol
3
Hepatology This article is protected by copyright. All rights reserved.
Hepatology
1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 43 44 45 46 47 48 49 50 51 52 53 54 55 56 57 58 59 60
Page 4 of 4
2014;29:208-214. 7.
Qi XS, Ren WR, Fan DM, Han GH. Selection of treatment modalities for Budd-Chiari
Syndrome in China: a preliminary survey of published literature. World J Gastroenterol 2014;20:10628-10636.
4
Hepatology This article is protected by copyright. All rights reserved.
