Literature DB >> 15065031

The outcome of the older (> or =100 days) infant with biliary atresia.

M Davenport1, V Puricelli, P Farrant, N Hadzic, G Mieli-Vergani, B Portmann, E R Howard.   

Abstract

BACKGROUND: There is a detrimental effect of increasing age on the results of the Kasai portoenterostomy for biliary atresia (BA), and some centers routinely advocate primary liver transplantation for the older infant, irrespective of other criteria. This perception that such infants are indeed irretrievable was tested by retrospective analysis.
METHODS: All infants who had undergone surgery for BA during the period 1980 through 2000 aged > or =100 days were reviewed. Actuarial survival was calculated using 2 end-points (death and transplantation). A retrospective review of their ultrasonography (n = 12) and preoperative liver histology (n = 22) was also undertaken to ascertain possible predictive criteria.
RESULTS: A total of 422 infants had BA diagnosed during this period, of which 35 (8.2%) were > or =100 days at surgery (median [interquartile range], 133 [range, 108 to 180] days). Surgery included portoenterostomy (n = 26), hepaticojejunostomy (n = 7), and a resection and end-to-end anastomosis (n = 1). A laparotomy only was performed in 1. Five- and 10-year actuarial survival rate with native liver was 45% and 40%, respectively. Currently, 12 (35%) patients are alive with their native liver (8 are anicteric), 9 (28%) have undergone transplantation, and 13 have died. Although there were some survival advantages for types 1 or 2 BA and "noncirrhosis" at time of surgery, neither reached statistical significance. Individual histologic features (eg, degrees of fibrosis, giant cell transformation, bile duct destruction) in the retrospective review of available material were not discriminatory. The finding of a "heterogeneous" parenchyma on ultrasonography was predictive of poor outcome but lacked sensitivity.
CONCLUSIONS: The potential for reasonable medium-term survival is present in about one third of infants 100 days or older coming to primary corrective surgery. In the absence of accurate discrimination, the authors continue to favor this option rather than subject all to transplant simply on the basis of age.

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Year:  2004        PMID: 15065031     DOI: 10.1016/j.jpedsurg.2003.12.014

Source DB:  PubMed          Journal:  J Pediatr Surg        ISSN: 0022-3468            Impact factor:   2.545


  25 in total

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4.  Inflammation, Active Fibroplasia, and End-stage Fibrosis in 172 Biliary Atresia Remnants Correlate Poorly With Age at Kasai Portoenterostomy, Visceral Heterotaxy, and Outcome.

Authors:  Kevin E Bove; Andrew D Thrasher; Robert Anders; Catherine T Chung; Oscar W Cummings; Milton J Finegold; Laura Finn; Sarangarajan Ranganathan; Grace E Kim; Mark Lovell; Margret S Magid; Hector Melin-Aldana; Pierre Russo; Bahig Shehata; Larry Wang; Francis White; Zhen Chen; Catherine Spino; John C Magee
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6.  [Predictive parameters in children with biliary atresia].

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8.  Diagnostic and Prognostic Significance of Various Histopathological Features in Extrahepatic Biliary Atresia.

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9.  PELD score and age as a prognostic index of biliary atresia patients undergoing Kasai portoenterostomy.

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10.  Biliary atresia.

Authors:  C K Sinha; Mark Davenport
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