De B K, De K K, Sen S, Biswas P K, Das T K, Das S, Hazra B
Department of Medicine and Cardiology, Institute of Post Graduate Medical Education and Research, 244, Acharya JC Bose Road, Calcutta 700 020.
J Assoc Physicians India. 2000 Aug;48(8):800-3.
Diagnosis of Budd-Chiari syndrome (BCS) is often missed unless its possibility has been kept in mind. Obstruction of inferior vena cava (IVC) is reportedly the most frequent cause of BCS in Afro-Asian variety.
An attempt was made to classify BCS (in an eastern Indian population) etiopathologically.
Thirty consecutive cases of BCS presenting over a period of five years were included. Following a thorough physical examination, necessary investigations (including coagulation profile, ultrasonography (with Doppler study) of hepatobiliary tract, hepatic vein and IVC angiography (n = 22) and liver biopsy (n = 26, including autopsy in two cases) were performed.
Mean age at presentation was 32.7 +/- 10.36 years (range 12-60 years) with M:F = 21:9. Clinical presentations included, hepatomegaly in 28 (93.3%), ascites in 27 (90%), splenomegaly in 15 (50%), pain in abdomen in 26 (86.6%), jaundice in 10 (33.3%), back veins in 20 (66.6%) and gastrointestinal bleeding in three (10%) cases. Amongst the total of 30 patients, four, six and 20 cases presented as fulminant, acute and chronic BCS respectively. Twenty four cases of BCS could be diagnosed by ultrasonography alone, while the remainder required angiography for diagnosis. IVC and hepatic vein angiography revealed membranous obstruction in nine, partial stricture of IVC in six, and IVC and/or hepatic vein block in others. The etiopathological nature in 30 cases were as follows: idiopathic membranous obstruction in nine (30%), hepatocellular carcinoma in six (20%), idiopathic stricture in six (20%) cases and one case (3.3%) each of the following: cholangiocarcinoma, renal cell carcinoma, chronic pancreatitis, hydatid cyst in liver, protein S deficiency, oral contraceptive use, nephrotic syndrome (with antithrombin III deficiency), polycythemia rubra vera and chronic lymphatic leukemia.
Idiopathic membranous obstruction and stricture of IVC are the commonest cause of BCS in the eastern part of India. Hepatocellular carcinoma is also a common cause, presenting in the fulminant form. Ultrasonography may be a helpful screening test for BCS, but IVC and hepatic vein catheterisation is essential for a complete work up of these patients.
布加综合征（BCS）的诊断常常被漏诊，除非临床医生时刻牢记其可能性。据报道，下腔静脉（IVC）梗阻是亚非地区BCS最常见的病因。
尝试对（印度东部人群中的）BCS进行病因病理分类。
纳入连续5年收治的30例BCS患者。经过全面体格检查后，进行了必要的检查（包括凝血指标、肝胆道超声检查（包括多普勒检查）、肝静脉和IVC血管造影（n = 22）以及肝活检（n = 26，其中2例为尸检））。
患者的平均就诊年龄为32.7±10.36岁（范围12 - 60岁），男:女 = 21:9。临床表现包括肝肿大28例（93.3%）、腹水27例（90%）、脾肿大15例（50%）、腹痛26例（86.6%）、黄疸10例（33.3%）、腹壁静脉曲张20例（66.6%）以及胃肠道出血3例（10%）。在30例患者中，分别有4例、6例和20例表现为暴发性、急性和慢性BCS。仅通过超声检查即可诊断24例BCS，其余患者则需要血管造影来确诊。IVC和肝静脉血管造影显示9例为膜性梗阻，6例为IVC部分狭窄，其余患者为IVC和/或肝静脉阻塞。30例患者的病因病理情况如下：特发性膜性梗阻9例（30%）、肝细胞癌6例（20%）、特发性狭窄6例（20%），以及以下各1例（3.3%）：胆管癌、肾细胞癌、慢性胰腺炎、肝包虫囊肿、蛋白S缺乏症、口服避孕药使用、肾病综合征（伴抗凝血酶III缺乏）、真性红细胞增多症和慢性淋巴细胞白血病。
特发性膜性梗阻和IVC狭窄是印度东部BCS最常见的病因。肝细胞癌也是常见病因，多表现为暴发性。超声检查可能是BCS的一项有用筛查手段，但IVC和肝静脉插管对于这些患者的全面评估至关重要。
