Twenty-Year Transplant-Free Survival Rate Among Patients With Biliary Atresia
Twenty-Year Transplant-Free Survival Rate Among Patients With Biliary Atresia
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DOI：
10.1016/j.cgh.2011.07.024
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发表时间：
2011-12-01
影响因子：
12.6
通讯作者：
Verkade, Henkjan J.
中科院分区：
文献类型：
--
作者：
de Vries, Willemien;Homan-Van der Veen, Jenneke;Verkade, Henkjan J.
背景与目的：尽管大多数患者最终需要肝移植，但 Kasai 门肠造口术的手术治疗改善了胆道闭锁患者的预后。具有长期、无移植生存的良好描述的患者很少；我们评估了治疗后存活 20 年的荷兰患者的肝脏状况和健康认知，并调查了无移植存活率是否随着时间的推移而增加。方法：通过使用荷兰国家胆道闭锁数据库，我们识别了 104 名 1977 年至 1988 年间出生的患者。我们收集了所有 20 岁时存活的未移植患者的临床特征、肝脏生化和超声检查数据（n = 28；占患者的 27%）。一般健康认知数据 (RAND-36) 在上次检查时收集。结果：20 年无移植生存率从 1977 年至 1982 年队列中的 20%（49 例中的 10 例）增加到 1983 年至 1988 年队列中的 32%（55 例中的 18 例）（P = .03）。 21% 的长期幸存者（28 人中的 6 人）的肝脏生化测试结果正常，并且没有肝硬化的临床或超声检查迹象。与对照组相比，女性患者的总体健康认知较低，男性患者则不然（RAND-36 评分，54 +/- 14 vs 74 +/- 18；P = .005）。结论：在荷兰，超过 25% 的胆道闭锁患者在没有进行肝移植的情况下存活了至少 20 年。与对照患者相比，患有胆道闭锁的女性对其健康状况的认知度较低。百分之二十的长期幸存者没有任何症状，没有肝硬化或门静脉高压的临床或超声检查迹象。
BACKGROUND & AIMS: Surgical treatment with Kasai portoenterostomy has improved the prognosis for patients with biliary atresia, although most patients ultimately require liver transplantation. Well-described patients with long-term, transplant-free survival are scarce; we assessed liver status and health perception among Dutch patients who survived 20 years after therapy and investigated whether the rate of transplant-free survival increases with time. METHODS: By using the Dutch national database for biliary atresia, we identified 104 patients, born between 1977 and 1988. We collected data on clinical characteristics, liver biochemistry, and ultrasonography from all transplant-free patients who were alive at age 20 years (n = 28; 27% of the patients). General health perception data (RAND-36) were collected at the last examination. RESULTS: The 20-year transplant-free survival rate increased from 20% (10 of 49) in the 1977 to 1982 cohort to 32% (18 of 55) in the 1983 to 1988 cohort (P = .03). Twenty-one percent of the long-term survivors (6 of 28) had normal liver biochemistry test results and no clinical or ultrasonographic signs of cirrhosis. The general health perception of female, but not male, patients, was lower, compared with controls (RAND-36 score, 54 +/- 14 vs 74 +/- 18; P = .005). CONCLUSIONS: More than 25% of patients with biliary atresia survive at least 20 years without liver transplantation in The Netherlands. Women with biliary atresia have a reduced perception of their health, compared with control patients. Twenty percent of long-term survivors are symptom-free, without clinical or ultrasonographic signs of cirrhosis or portal hypertension.