Biliary atresia: The King's College Hospital experience (1974-1995)

被引:135
作者
Davenport, M [1 ]
Kerkar, N [1 ]
MieliVergani, G [1 ]
Mowat, AP [1 ]
Howard, ER [1 ]
机构
[1] UNIV LONDON KINGS COLL HOSP,DEPT HEPATOL,LONDON SE5 9RS,ENGLAND
关键词
biliary atresia; portoenterostomy; hepaticojejunostomy;
D O I
10.1016/S0022-3468(97)90611-4
中图分类号
R72 [儿科学];
学科分类号
100202 ;
摘要
The survival experience of 338 infants born with biliary atresia between January 1973 and December 1995 was analyzed. All the infants had their initial surgery at a single UK centre. These infants were divided into three groups based on year of birth; group 1 (1970s, n = 38); group 2 (1980s, n = 182), and group 3 (1990s, n = 118), The data from group 1 were incomplete and selected, and comparisons with the remaining groups were therefore restricted, However, all infants who had been treated since 1980 underwent portoenterostomy or hepaticojejunostomy and were included. Results: In the whole cohort there were 89 deaths (26%), 79 children (23%) who underwent liver transplantation and 170 children (50%) who were alive at last follow-up, The 5- and 10-year actuarial survival for group 2 was 50% and 41%, respectively and the 5-year actuarial survival for group 3 was 60%. Overall, 57 children have survived to 10 years after surgery for biliary atresia. There has been a progressive fall in the age at surgery from a median of 77 days in group 1, through 69 days in group 2 to 56 days in group 3 (P < .0001). However, there was no significant difference in outcome to 5 years between the age cohorts (< 40 days, 41 to 60 days, 61 to 99 days, and greater than or equal to 100 days; P > .1) for the infants treated since 1980 (n = 200). Conclusions: Portoenterostomy is an effective long-term procedure for biliary atresia in about 40% to 50% of infants. The remaining 50% to 60% will require transplantation mostly within 2 years of age, although there is also a continuing need beyond 5 and 10 years, The age at surgery has limited usefulness as a predictor of survival after portoenterostomy and certainly should not be used to dictate primary treatment. Copyright (C) 1997 by W.B. Saunders Company.
引用
收藏
页码:479 / 485
页数:7
相关论文
共 43 条
  • [11] HEPATIC LYMPHATIC DRAINAGE TO JEJUNUM FOR CONGENITAL BILIARY ATRESIA
    FONKALSRUD, EW
    KITAGAWA, S
    LONGMIRE, WP
    [J]. AMERICAN JOURNAL OF SURGERY, 1966, 112 (02) : 188 - +
  • [12] GAUTIER F, 1996, P 6 INT S BIL ATR SE, P41
  • [13] PROGNOSIS OF EXTRAHEPATIC BILIARY ATRESIA
    HOUWEN, RHJ
    ZWIERSTRA, RP
    SEVERIJNEN, RSVM
    BOUQUET, J
    MADERN, G
    VOS, A
    BAX, NMA
    HEYMANS, HSA
    BIJLEVELD, CMA
    [J]. ARCHIVES OF DISEASE IN CHILDHOOD, 1989, 64 (02) : 214 - 218
  • [14] Howard Edward R., 1995, P551
  • [15] HOWARD ER, 1982, J ROY SOC MED, V7, P408
  • [16] HOWARD ER, 1988, SURGERY LIVER BILIAR, P707
  • [17] HOWARD ER, 1991, P 5 INT SEND S BIL A, P111
  • [18] JAUNDICE AT 14 DAYS OF AGE - EXCLUDE BILIARY ATRESIA
    HUSSEIN, M
    HOWARD, ER
    MIELIVERGANI, G
    MOWAT, AP
    [J]. ARCHIVES OF DISEASE IN CHILDHOOD, 1991, 66 (10) : 1177 - 1179
  • [19] KALAYOGLU M, 1993, SURGERY, V114, P711
  • [20] HEPATIC HISTOLOGY AND THE DEVELOPMENT OF ESOPHAGEAL-VARICES IN BILIARY ATRESIA
    KANG, N
    DAVENPORT, M
    DRIVER, M
    HOWARD, ER
    [J]. JOURNAL OF PEDIATRIC SURGERY, 1993, 28 (01) : 63 - 66