Dilawari J B, Bambery P, Chawla Y, Kaur U, Bhusnurmath S R, Malhotra H S, Sood G K, Mitra S K, Khanna S K, Walia B S
Department of Hepatology, Postgraduate Institute of Medical Education and Research, Chandigarh, India.
Medicine (Baltimore). 1994 Jan;73(1):21-36. doi: 10.1097/00005792-199401000-00003.
Budd-Chiari syndrome (BCS) may not be as uncommon as was once believed. Our study has substantiated the existence of 2 major clinical forms. The acute syndrome is invariably associated with extensive blockage of the major hepatic veins, resulting in congestive liver cell necrosis. In a small, but significant, number of patients the inferior vena cava (IVC) is also occluded. The important etiologic factors are related to hypercoagulability of blood. Immediate placement of a shunt improves survival. The chronic syndrome is characterized by portal hypertension and is associated with a variable abnormal vascular anatomy. The causes of the chronic syndrome are not clear, but a substantial number of cases are related to the presence of an IVC membrane. Shunt surgery is effective but procedures aimed at the primary pathology are likely to be even more so. The natural history of BCS should be viewed over a long period of time. The very long survival of several patients urges a more cautious approach to surgical remedies. Budd-Chiari syndrome probably represents a spectrum of disease caused primarily by a hypercoagulable state and having a varied presentation depending on the balance between rate of formation and the extent of the thrombosis and the body's own rate of thrombolysis and recanalization. The extent and efficacy of the individual's collateral circulation and the rate of development of liver fibrosis are other determinants. It is thus possible to view BCS as a continuum of a single pathogenetic spectrum. Pregnancy-related BCS in India probably has strong social determinants, and is usually acute and fulminant. We have, however, documented a chronic form not described earlier. Children usually do not have acute BCS, but chronic BCS in children and adolescents is similar to that in adults. Membranous obstruction of the inferior vena cava (MOVC) is common and was found even at a young age. The association of MOVC with hepatocellular carcinoma, however, did not appear to be as clear as was previously believed. There has been a wide geographical variability in the causes and manifestations of BCS. Our study has clearly shown that--Kipling's categorical statement to the contrary--East and West do meet in India, in the Budd-Chiari syndrome.
布加综合征（BCS）可能并不像人们曾经认为的那样罕见。我们的研究证实了其存在两种主要临床类型。急性综合征总是与主要肝静脉的广泛阻塞相关，导致充血性肝细胞坏死。在少数但数量可观的患者中，下腔静脉（IVC）也会被阻塞。重要的病因与血液高凝状态有关。立即进行分流术可提高生存率。慢性综合征的特征是门静脉高压，且与多种异常血管解剖结构相关。慢性综合征的病因尚不清楚，但相当一部分病例与下腔静脉膜的存在有关。分流手术有效，但针对原发性病变的手术可能效果更佳。布加综合征的自然病程应从长期角度来看待。几位患者的长期存活促使我们对手术治疗采取更为谨慎的态度。布加综合征可能代表了一系列主要由高凝状态引起的疾病，其表现因血栓形成速率与血栓范围之间的平衡以及机体自身的溶栓和再通速率而异。个体侧支循环的范围和效能以及肝纤维化的发展速率是其他决定因素。因此，可以将布加综合征视为单一发病机制谱的连续统一体。印度与妊娠相关的布加综合征可能有很强的社会决定因素，通常为急性和暴发性。然而，我们记录了一种此前未描述过的慢性类型。儿童通常不会患急性布加综合征，但儿童和青少年的慢性布加综合征与成人相似。下腔静脉膜性阻塞（MOVC）很常见，甚至在年轻时就能发现。然而，MOVC与肝细胞癌的关联似乎并不像之前认为的那样明确。布加综合征的病因和表现存在广泛的地域差异。我们的研究清楚地表明——与吉卜林的绝对论断相反——在印度，在布加综合征方面，东方和西方确实有交集。
