A Study On The Deficiency Of Plasma PC Proteins And AT-Ⅲ In Chinese Patients With Budd-Chiari Syndrome

Background and Objective: Budd-Chiari syndrome (B-CS) presents the partial or complete outlet obstruction of major hepatic veins (MHVs) and/or hepatic inferior vena cava (HIVC) by various factors, which results in portal hypertension (PHT) and (or) IVC hypertension syndrome. Since Budd described this disease firstly in 1845, almost 170 years had past. B-CS is a serious disease which seriously harm the health of people. the nature prognosis is very poor. Thanks to the increasing recognition to the syndrome and development of the diagnostic imaging technology inthe past decade, a large amount of cases were diagnosed and reported. The incidence of B-CS is about 4~6 per 100 000 in China. Most of the casesdistributed in the middle and lower reach of yellow river, such as Henan, Shandong and Anhui etc. Xu peiqin et al. had treated more than 1400 cases since 1983 in the General Surgery Department of the First Affiliated Hospital of Zhengzhou University. The exact etiology of the syndrome is not clearly identified. According to the etiology, we can divide the syndrome into secondary or primary. The secondary B-CS usually results from definite disease such as reported frequently, myeloproliferative disorders — including polycythemia vera and polyplastocytosis, antiphospholipid antibodies, paroxysmal nocturnal hemoglobinuria, systemic lupus erithematosus, Bechet' s disease, coeliac disease andcarcinoma Oral contraceptive usage and pregnancy are cooperative facts may play a role in the pathogenesis of B-CS.As to the primary B-CS, there are congenital membrane theory and acquired thrombosis theory to illustrate the cause. The secondary thrombosis organized and shaped the membrane obstruction. The later is proved by more and more object i ve ev i dences. Present I y, the research about the acquired thrombosis theory focus on the blood hypercoagulable states resulted from various hereditary ant i coagu I at i on deficiencies such as F V Leiden gene mutation, protein C, antithrombin III and protein S deficiency and FIIG20210A gene mutation. There are some papers reporting the relations of B-CS to protein C and antithrombin III deficiency in western country, nevertheless, the corresponding report is rarely in china. The incidence of protein C and antithrombin III deficiency in B-CS is 6. 8%~9. 2% according to reports of western country. The Statistics is based histopathoIogy of their B-CS. However, the major histopathoIogy of B-CS in Chinese and other oriental countries is the membrane obstruction.In order to explore whether the Chinese B-CS patients have relations to protein C and antithrombin III deficiency or not, we measured the activity level of plasma protein C > antithrombin III and VD factor by Coamate and first stage method with 30 hepatic cirrhosis secondary to B virus and 30 health controls, therefore , we can we discuss the roles of protein C .. antithrombin III activity deficiency played in the pathogenesis of Chinese B-CS.Data and methods: Chinese B-CS group, male, 18; female, 12. hepatic cirrhosis secondary to B virus group, male, 17; female, 13. Seventy contro I s were hea I thy b I ood donors i n Henan BI ood Center, f ema I e, 16; ma I e, 14. All subjects were Chinese people and excluded injury, postoperation, infection, myeIodyspIastic syndrome, antiphosphol ipid antibody syndrome, paroxysmal nocturnal hemoglobinuria, systemic lupus erithematosus andBechet' s disease, and no use of anticoagulants at least 3 months recent Iy. femaIe cases weren' t in pregnancy or using oral contraceptive recently, at the same time, also excluded other system diseases.Five mi 11iIiters venous blood were drawn from each patient and control and reserved after anticoagulant process on 4°C. Plasma frozen on -20°C. Thawing for 10min by 37"C water-path before measurement. The activity level measurement of PC and AT—III all by Coamate method. The activity level measurement of VII factor by first stage method. Draw standard cure, and obtain relative results. After that, judge the result. Use SPSS10.0 and SAS software package to classify and analyze the statistic dataResults: Plasma activity levels of PC and AT—III in the B-CS group and hepatic cirrhosis secondary to B virus group were significantly lower than the healthy individuals groups (PC (86. 6%±11. 5%), (76. 4%±10. 5%),(102. 8%±12. 0%), AT-III (82. 9%±10. 4%), (73. 2%±9. 0%), (103. 7%±12. 4%), all P<0.05)); Plasma activity levels of PC ^ AT-III and VD factor in the hepatic cirrhosis group secondary to B vi rus were significantly lower than in B-CS group (PC (76. 4%±10. 5%), (86. 6X±11. 5%); AT-III (73. 2%±9. 0%),(82.9%±10.4%), VD (67. 7%±11.1%) , (76. 8%±10. 5%) .PiSKO. 05) . al I P <0. 05) ); The regression analysis of I iver function(W factor level) and levels of AT-ITJ , PC, PS suggests that there is no significant d i ff erent i at i on in the B-CS groups and the hea I thy i nd i v i dua I s groups ( bAT-,? (0.92, 0.93), bpc (0.96, 0.94), all P<0.05)Conclusions: 1. Through this research, we can speculate there have no relations of Chinese B-CS patients to PC and AT-III activity. PC and AT-III activity lower than health group is caused by liver function damage secondary to B-CS itself.2. The secondary liver function damage in B-CS is weaker than in hepatic cirrhosis secondary to B virus patient.3. Combined with the research abroad, it also prove that there have ethnic >. geographic and histopathologic type differences in PC and AT-III activity deficiency.4. Whether congenital PC and AT—III activity deficiency play roles in the pathogenesis of Chinese B-CS or not, still need enlarge samples and improve measurement method for further step research.