| Background and objectivesBudd-Chiari Syndrome(BCS) refers to the inferior vena caval hypertension and post hepatic portal hypertension(PHT) complexes caused by obstruction of hepatic segments or post hepatic segments of inferior vena cava(IVC), including obstruction of hepatic vena outflow tract without IVC reflux barrier. Currently, in Henan, Shandong, Jiangsu and Anhui Provinces, BCS has become a common clinical disease, which is difficult for medical treatment, and has unfavorable prognosis, which has seriously affected the labor capacity of patients and their living quality.The local pathologies of BCS are complicated, and the location and degrees of disorder are different. However the most fundamental change is the formation of membrane on the post hepatic segment of IVC and secondary thrombus. Study has shown that membrane has played a significant role in the incidence of BCS and the recurrence of BCS after treatment.The formation mechanism of membrane organism is still not quite clear now. Study of histology has discovered that there are large amount of collagenous fiber contained in BCS membrane organism and skin scar, which indicates that there may be similar mechanism in the formation of the two. It has been proved that transforming growthfactor β (TGF β ), platelet derived growth factor(PDGF), fibroblast growth factor(FGF) and endothelin-l(ET-1) have played important roles in the excessive growth of skin scars, which help to bring about new progress on prevention and treatment of scar tissue. Inspired by these findings, we infer that these factors might have expression and play some part in the formation of membrane organism. However, there are few current research on the role of cytokine in the formation of BCS membrane organism at home and abroad, which are mostly focused on some growth factors. It is the viewpoint of immunology that the function of cytokines must be combined with their corresponding receptors. Thus we plan to make a study of the expression of transforming growth factor β receptor(TGF β R), platelet derived growth factor receptor(PDGFR), fibroblast growth factor receptor(FGFR) and ET-1 in both BCS membrane organism and normal IVC by using immunohistochemical method, to explore the immune mechanism of BCS membrane organism, which has no relevant report in China. Meanwhile, the pathologic changes of BCS membrane organism and whether there is correlation between it and the sex, age, course of disease and hepatic reserve function are observed and analysed in this study, so as to have further understanding of the pathogenesis of BCS membrane organism.Materials and methodSamples of membrane organism came from the prepared wax blocks of removed organisms from BCS patients in our hospital from 1988 to 2000, 34 cases in all, and were arranged as an experiment group, of which there were 24 males, 10 females, aged from 17 to 67, the average age was 31, the course of disease ranged from 6 weeks to 37 years, 19 cases with grade A hepatic reserve function, 12 cases with grade B and 3 cases with grade C hepatic reserve function. In addition, inferior vena caval organisms are removed from16 doners of renal transplantation, solidified, dewatered and covered with wax, which are set as a controlled group.Immune histochemical stain was conducted according to the instructions on the immune histochemical kit of TGF β R, PDGFR, FGFR and ET-1, nucleus re-dyed and the microscopic manifestation was observed. Positive criteria was worked out andexpression of the above indexes in the membrane organism observed, the rates of expression were calculated respectively. By using the SPSS 10.0 statistical software package, we compared expressions of the above indexes in BCS membrane organism with that in normal IVC, and studied the relationship between the pathologic changes and the sex, courses of disease and grade of hepatic function.Results1. Under microscope, it can be seen that BCS membrane organism has varying degrees of connective fiber proliferation, hyaline degeneration and muciform degeneration. The chronic inflammatory cell infiltration can be seen in 22/34 (64.71%), mainly the plasma cell and lymphocyte cells.2. There are expression of TGF β R, PDGFR, FGFR and ET-1 in both groups but the amount is different. The rate of expression of TGFR, PDGFR and FGFR in normal blood vessel and BCS membrane organism is 0.21 ±0.07 and 0.38 ± 0.14, 0.30 ±0.08 and 0.52 ±0.07, 0.24 ±0.06 and 0.50 ±0.11 respectively; The rate of expression of ET-1 in BCS membrane organism and normal blood vessel is 0.24 ± 0.08 and 0.54 ± 0.12 respectively; That means all the expression are significantly higher in the membrane organism of BCS than in normal blood vessels(p<0.05).3. The correlative coefficient between the age of patients and expression of the TGF 3 R, PDGFR, FGFR and ET-1 in BCS membrane organism is 0.3 80, 0.020,-0.801 and -0.206 respectively. Only the significantly inverse proportion of age to FGFR was observed(P<0.05).4. The correlative coefficient between course of disease of BCS patients and expression of TGF β R, PDGFR, FGFR and ET-1 in membrane organism is respectively 0.181, 0.002 , -0.917 , -0.302, which is only in inverse proportion to FGFR, and are irrelevant with other three indexes.5. The expression of TGF β R, PDGFR and FGFR has no obvious relationship with the patients' grade of hepatic function(p>0.05); while the patients with different grade of hepatic function has marked difference in the expression of ET-1 (p<0.05), the expression of ET-1 in patients with fine hepatic function reserve is higher thanthose with poor hepatic function reserve.Conclusions1. Inflammation reaction participates in the formation of BCS membrane organism in some degree.2. There are significant differences of expression of TGF & R, PDGFR, FGFR and ET-1 between BCS membrane organism and normal blood vessel, which indicate that the differences may play an important part in the formation of BCS membrane organism.3. No relationship is found between the expression of TGF β R, PDGFR, ET-1 and sex or age ; there is an inverse proportion to age and course of disease for the expression of FGFR.4. Expression of ET-1 is related with different grade levels of hepatic function of BCS patients, expression of ET-1 in patients with fine hepatic function reserve is higher than those with poor hepatic function reserve. |
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