Biliary atresia—a fifteen-year review of clinical and pathologic factors associated with liver transplantation

Baerg, Joanne; Zuppan, Craig W.; Klooster, M.

doi:10.1016/j.jpedsurg.2004.02.020

Cited by 37 publications

(27 citation statements)

References 7 publications

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“…Various attempts have been made to develop29 and improve unique scoring systems such as the Pediatric End‐Stage Liver Disease model30, 31 to set the indication for LTx and to determine the multiple factors influencing the outcome of children with BA 12, 32, 33. The integration of Vfib may be another element to improve the predictive value of those scoring systems.…”

Section: Discussionmentioning

confidence: 99%

Prognostic value of computerized quantification of liver fibrosis in children with biliary atresia

et al. 2009

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Biliary atresia (BA) is the leading indication for liver transplantation (LTx) during childhood. Predictive markers for progression are lacking. We investigated the correlation between quantified fibrosis of the liver at the time of Kasai hepatic portoenterostomy (KPE) and the clinical course. Liver tissue was obtained at the time of KPE in 53 children and stained with Picrosirius red. The mean volume of fibrosis per number of periportal fields (Vfib) and the Ishak score were calculated. Vfib was 3.9%. Transplant-free survival was significantly higher in those patients with Vfib Ͻ 2.5% (P Ͻ 0.05, Kaplan-Meier analysis). Vfib Ͻ 2.5% predicted transplant-free survival with a sensitivity of 100% and a specificity of 48% (positive predictive value: 100%, negative predictive value: 17%). Vfib was correlated with bilirubin 6 months after KPE (Bili6m). The negative predictive value could be enhanced to 23% if Vfib Ͻ 2.5% was combined with Bili6m Ͻ 20 mol/L. The Ishak score showed no correlation with transplant-free survival or Vfib. Detection of Vfib at the time of KPE is a valid marker in predicting transplant-free survival in children with BA. This method should be considered an integral part of scoring systems predicting the indication for LTx. Liver Transpl 15:876-882, 2009.

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Section: Discussionmentioning

confidence: 99%

Prognostic value of computerized quantification of liver fibrosis in children with biliary atresia

et al. 2009

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“…Ductal diameter less than 100 microns was a feature identified with children requiring liver transplantation[61]. Whereas, when ductal size was more than 150 microns, in combination with a columnar lined epithelium, it was predictive of good prognosis after surgical management[62].…”

Section: Investigationsmentioning

confidence: 99%

Biliary atresia: Where do we stand now?

Govindarajan

2016

WJH

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The pathway from clinical suspicion to establishing the diagnosis of biliary atresia in a child with jaundice is a daunting task. However, investigations available help to point towards the correct diagnosis in reasonable time frame. Imaging by Sonography has identified several parameters which can be of utility in the diagnostic work up. Comparison of Sonography with imaging by Nuclear medicine can bring out the significant differences and also help in appropriate imaging. The battery of Biochemical tests, available currently, enable better understanding of the line-up of investigations in a given child with neonatal cholestasis. Management protocols enable standardized care with optimal outcome. The place of surgical management in biliary atresia is undisputed, although Kasai procedure and primary liver transplantation have been pitted against each other. This article functions as a platform to bring forth the various dimensions of biliary atresia.

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“…In the Kasai procedure, an anastomosis between the small intestine and the portal rim of the liver is performed; and therefore, it has been considered that the most important factor determining the bile drainage would be the portal plate anatomy. Some studies have shown no relationship [7][8][9][10][11][12][13], and yet others have shown that there is one [14][15][16][17][18][19][20][21][22][23][24][25][26][27][28]. Yet others have postulated that the intrahepatic lesions determine the prognosis [15].…”

mentioning

confidence: 99%

Histologic features of the portal plate in extrahepatic biliary atresia and their impact on prognosis—a Danish study

Mirza

Kvist

Petersen

2009

Journal of Pediatric Surgery

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Biliary atresia—a fifteen-year review of clinical and pathologic factors associated with liver transplantation

Cited by 37 publications

References 7 publications

Prognostic value of computerized quantification of liver fibrosis in children with biliary atresia

Prognostic value of computerized quantification of liver fibrosis in children with biliary atresia

Biliary atresia: Where do we stand now?

Histologic features of the portal plate in extrahepatic biliary atresia and their impact on prognosis—a Danish study

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