Department of Pediatric Gastroenterology and Hepatology, Beatrix Children's Hospital, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
Clin Gastroenterol Hepatol. 2011 Dec;9(12):1086-91. doi: 10.1016/j.cgh.2011.07.024. Epub 2011 Aug 4.
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.
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.
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).
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.
通过施行 Kasai 门腔分流术，胆道闭锁患者的预后得到了改善，尽管大多数患者最终仍需要进行肝移植。尽管有描述详尽的长期无移植生存患者，但此类患者仍较为少见；本研究评估了荷兰胆道闭锁治疗后 20 年存活患者的肝脏状态和健康感知，并探讨了无移植生存是否随时间推移而增加。
通过使用荷兰胆道闭锁国家数据库，我们确定了 104 名于 1977 年至 1988 年出生的患者。我们收集了所有在 20 岁时仍存活且无移植的患者（n=28；占患者总数的 27%）的临床特征、肝脏生化和超声数据。最后一次检查时，我们还收集了一般健康感知数据（RAND-36）。
20 年无移植生存率从 1977 年至 1982 年队列的 20%（49 例中的 10 例）上升至 1983 年至 1988 年队列的 32%（55 例中的 18 例）（P=.03）。21%的长期幸存者（28 例中的 6 例）肝脏生化检查结果正常，且无肝硬化的临床或超声征象。与对照组相比，女性（而非男性）患者的一般健康感知较低（RAND-36 评分，54±14 对 74±18；P=.005）。
在荷兰，超过 25%的胆道闭锁患者在无需肝移植的情况下至少存活 20 年。与对照患者相比，胆道闭锁女性患者的健康感知降低。20%的长期幸存者无症状，无肝硬化或门静脉高压的临床或超声征象。
