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BCS 预后
TIPSS术后的预后可以用Child Pugh 或MELD（Model for end-stage liver disease）评分系统评估。MELD评分系统初始设计是TIPSS（预防食道静脉曲张出血和顽固性腹水）术后预期3个月死亡的评估方法【50】。它包括胆红素、INR和肌酐，这些作为BCS死亡的其它预测因子。慢性进行性疾病的BCS患者死亡由MELD评分预测。但是对于所有BCS患者，MELD并非是一个好的预测因子，因为BCS患者的肝功能情况非常不同【51】。
这是为爆发性肝功能衰竭的BCS患者提出要履行标准的特殊情况。在此种情况下，一种MELD例外已经建议列为器官共享网络（United Network for Organ Sharing status，UNOS）1A【52】。
回顾性分析1970~1992年间120例患者，发现1、5、10年生存率分别为77±4%、64±5% 和 57±6%。120例患者中，82例为外科分流术，38例仅接受内科治疗。两组中发现四个因素与生存相关，年龄、腹水对利尿剂反应、Child-Pugh评分和血清肌酐【4-36】。1984~2001年间237例患者的国际多中心研究显示1、5、10年生存率分别是82% (95% CI: 77–87)、69% (95% CI: 62–76) 和 62% (95% CI: 54–70)【33】。
专门制定用于BCS的4中评分方式见下表。
Prognostic indices for Budd–Chiari syndrome
|
Study |
Description |
Patients
(n) |
Formula |
Actuarial survival
according to
prognostic index |
|
Clichy【30】 |
Multivariate retrospective
analysis from 1970 to
1992. Assessment of
factors affecting survival
in patients undergoing
medical therapy (n = 38)
or surgical shunts (n = 82) |
120 |
(Ascites score × 0.75) + (Pugh score × 0.28) +
(age × 0.037) + (creatinine × 0.0036). (Ascites
score [1]: absent with free sodium intake and
no diuretic agents; [2] easy to control with
sodium restriction or diuretic agents; and [3]
resistant to this treatment because of
hyponatremia or functional renal failure) |
Score >5.4: 1 year
survival ~75%, 5 year
survival ~65%; Score
<5.4: 1 year survival
100%, 5 year survival
~95% |
|
New Clichy【53】 |
Multivariate retrospective
analysis on 69 patients,
then on these 69 and 54
previous patients all
diagnosed since 1985 |
123 |
(0.95 × ascites) + (0.35 × Pugh score) +
(0.047 × age) + (0.0045 × creatinine) + (2.2 ×
Type III) – 2.6. Type III is a binary variable
coded as ‘1’ for patients with acute and
chronic features and ‘0’ if no acute or chronic
features |
Score >5.1: 1-year
survival 80%, 5-year
survival 65%; score <5.1
1- and 5-year survival
100% |
Rotterdam
score【33】 |
Multivariate retrospective
analysis from 1984 to
2001 |
237 |
(1.27 × encephalopathy) + (1.04 × ascites) +
(0.72 × prothrombin time) + (0.004 ×
bilirubin). Ascites and hepatic encephalopathy
were scored as present (1) or absent (0) and
prothrombin time as higher (1) or lower (0)
than INR of 2.3 |
5-year survival rate: 89%
(95% CI: 79–99) for class
I, 74% (95% CI: 65–83)
for class II, 42% (95% CI:
28–56) for class III, (class
I [0–1.1], class II [1.1–1.5],
class II [>1.5]) |
|
BCS-TIPSS【47】 |
Multivariate retrospective
analysis from 1993 to
2006. Assessment of
factors affecting survival
in BCS patients
undergoing TIPSS |
124 |
PI = (age × 0.08) + (bilirubin [mg/dl] × 0.16) +
(INR × 0.63) |
114 patients had a
BCS- TIPSS PI <7 and
8 patients >7. This cut-off
had a sensitivity of 58%,
a specificity of 99%, a
positive predictive value
of 88%, and a negative
predictive value of 96%
for death or OLT 1 year
after TIPSS |
BCS: Budd–Chiari syndrome; INR: International normalized ratio; OLT: Orthotopic liver transplantation; PI: Prognostic Index; TIPSS: Transjugular intrahepatic portosystemic stent–shunt.
用多因素分析，Rotterdam 评分首创【33-19】。腹水和肝性脑病的存在或缺乏被纳入评分因素，凝血酶原时间高或低于INR2.3也被纳入评分因素。病人被分为3类：基于评分结果的分类1（预后好），5年生存率为89%（95% CI：79-99）；分类2（预后中等）5年生存率74%（95% CI：65-83）和分类3（预后差）5年生存率42%（95% CI：28-56）。
第二个多因素分析123例病人发现，年龄，Child-Pugh评分、腹水和血清肌酐与生存率成反比[53-80]。这一研究形成Clichy预后评分的基础。它们也包括如果BCS是急性、慢性或兼具两种的特征。应用这一暂时性分类的证据是缺乏的，并没有得到专家们的支持[3]。Clichy 预后指数得到修改，被称为新Clichy 预后指数【53-80】。
TIPSS特定指数（BCS-TIPSS）也经建立用于评估BCS病人进行TIPSS的预后评分。评分＞7分的8例患者中的7例患者或死亡，或进行肝移植；比较＜7分的114例患者仅为5例。
1995年-2005年诊断BCS的96例患者用另一种预后指数并与Clichy、Rotterdam BCS指数、New Clichy 和BCS-TIPSS 【54-81】。作者得出结论：当预后指数合适于计算肝移植和介入治疗无病生存，其预测精度用于个体化患者是不精确的。四种预测因子总结在上表。
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