Epidemiological and clinical features of Budd-Chiari syndrome in Japan

doi:10.1016/0168-8278(95)80252-5

Journal of Hepatology

Volume 22, Issue 1, January 1995, Pages 1-9

https://doi.org/10.1016/0168-8278(95)80252-5 Get rights and content

Abstract

The Japanese Ministry of Health and Welfare Research Committee on Aberrant Portal Blood Flow carried out an epidemiological survey and clinical study on Budd-Chiari syndrome in 1990. In the primary survey for determining the prevalence of the disease, a questionnaire was sent to all major hospitals throughout Japan and 160 cases seen in 1989 were compiled. More epidemiological details were obtained in 87 of these 160 cases. The number of patients with Budd-Chiari syndrome in this country was estimated to be about 300 (prevalence of 2.4/million) with about 20 new cases occurring every year. In the clinical study, 157 authentic cases of Budd-Chiari syndrome studied in 15 years (1975–89) were analyzed. There were 87 males (average age, 36.4 years) and 70 females (46.5 years), and the average period from the likely onset to the first medical consultation was 6.6 years, suggesting that these patients were mostly chronic cases. The main clinical features were hepatomegaly, leg edema, ascites and venous dilatation over the trunk. Abdominal pain was recorded in only four (2.5%). There were 16 (10.2%) with known identifiable etiologies. Of the patients 93% showed an obstructing lesion of varius thickness in the hepatic portion of the inferior vena cava. Only nine (5.7%) had hepatic vein obstruction without caval lesions. Thus, the majority of Budd-Chiari syndrome patients in Japan are idiopathic, having an obstructing lesion in the inferior vena cava. The main causes of 33 deaths (21%) were liver failure, variceal bleeding and hepatocellular carcinoma. Hepatocellular carcinoma occurred in 10 (6.4%) in the 15-year period. However, the incidence of Budd-Chiari syndrome among all cases of hepatocellular carcinoma was less than 1% in the survey made by the Liver Cancer Study Group of Japan.

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Epidemiological and clinical features of Budd-Chiari syndrome in Japan

Abstract

Gastroenterology

Am J Med

Gastroenterology

Am J Med

Gastroenterology

Gastroenterology

Gastroenterology

Disorders of the hepatic veins and venules

Budd-Chiari syndrome

Multiple venous thromboses and membranous obstruction of inferior vena cava in association with hereditary protein C deficiency: a case report

J Gastroenterol Hepatol

Pathology of hepatocellular carcinoma in Japan: 232 consecutive cases autopsied in ten years

Cancer

Primary occlusion of the ostia of the hepatic veins

Q J Med

Occlusion of the hepatic veins in man

Medicine

Membranous obstruction of the hepatic segment of the inferior vena cava

Br J Surg

Congenital membranous obstruction of the inferior vena cava: report of a case and review of the literature

J Cardiovasc Surg

Congenital obstruction of inferior vena cava

Acta Chir Scand

The role of membranous obstruction of the inferior vena cava in the etiology of hepatocellular carcinoma in Southern African blacks

Hepatology

Clinical spectrum of Budd-Chiari syndrome in Chandigarh with particular reference to obstruction of intrahepatic portion of inferior vena cava

Ind J Med Res