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Portal Hypertension in Children and Young Adults With Biliary Atresia

Shneider, Benjamin L.*; Abel, Bob; Haber, Barbara; Karpen, Saul J.§; Magee, John C.; Romero, Rene||; Schwarz, Kathleen; Bass, Lee M.#; Kerkar, Nanda**; Miethke, Alexander G.††; Rosenthal, Philip‡‡; Turmelle, Yumirle§§; Robuck, Patricia R.||||; Sokol, Ronald J.¶¶; for the Childhood Liver Disease Research and Education Network (ChiLDREN)

Journal of Pediatric Gastroenterology & Nutrition: November 2012 - Volume 55 - Issue 5 - p 567–573
doi: 10.1097/MPG.0b013e31826eb0cf
Original Articles: Hepatology and Nutrition

Objective: Biliary atresia (BA) frequently results in portal hypertension (PHT), complications of which lead to significant morbidity and mortality. The Childhood Liver Disease Research and Education Network was used to perform a cross-sectional multicentered analysis of PHT in children with BA.

Methods: Subjects with BA receiving medical management at a Childhood Liver Disease Research and Education Network site were enrolled. A priori, clinically evident PHT was defined as “definite” when there was either history of a complication of PHT or clinical findings consistent with PHT (both splenomegaly and thrombocytopenia). PHT was denoted as “possible” if one of the findings was present in the absence of a complication, whereas PHT was “absent” if none of the criteria were met.

Results: A total of 163 subjects were enrolled between May 2006 and December 2009. At baseline, definite PHT was present in 49%, possible in 17%, and absent in 34% of subjects. Demographics, growth, and anthropometrics were similar amongst the 3 PHT categories. Alanine aminotransferase, γ-glutamyl transpeptidase, and sodium levels were similar, whereas there were significant differences in aspartate aminotransferase (AST), AST/alanine aminotransferase, albumin, total bilirubin, prothrombin time, white blood cell count, platelet count, and AST/platelet count between definite and absent PHT. Thirty-four percent of those with definite PHT had either prothrombin time >15 seconds or albumin <3 g/dL.

Conclusions: Clinically definable PHT is present in two-thirds of North American long-term BA survivors with their native livers. The presence of PHT is associated with measures of hepatic injury and dysfunction, although in this selected cohort, the degree of hepatic dysfunction is relatively mild and growth is preserved.

*Children's Hospital Pittsburgh of UPMC, Pittsburgh, PA

University of Michigan, Ann Arbor, MI

Children's Hospital of Philadelphia, Philadelphia, PA

§Texas Children's Hospital, Houston, TX

||Emory University, Altanta, GA

Johns Hopkins University, Baltimore, MD

#Children's Memorial Hospital, Chicago, IL

**Mount Sinai School of Medicine, New York, NY

††Cincinnati Children's Hospital Medical Center, Cincinnati, OH

‡‡University of California San Francisco, San Francisco, CA

§§Washington University, St Louis, MO

||||National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD

¶¶University of Colorado and Children's Hospital Colorado, Aurora, CO.

Address correspondence and reprint requests to Dr Benjamin L. Shneider, Children's Hospital of Pittsburgh of UPMC, Division of Pediatric Gastoenterology, Hepatology and Nutrition, 4401 Penn Ave, Pittsburgh, PA 15224 (e-mail:

Received 5 June, 2012

Accepted 15 June, 2012

Supplemental digital content is available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal's Website ( registration number: NCT00061828.

This work was financially supported by the National Center for Research Resources (5M01 RR00069 [R.J.S.], UL1RR025780 [Colorado], UL1RR024153 [Pittsburgh], UL1RR024134 [Philadelphia], UL1RR024131 [San Francisco], UL1RR025005 [Baltimore], UL1RR025741 [Chicago]), UL1RR029877 [New York] and the National Institute of Diabetes, Digestive and Kidney Diseases (DK 62453 [R.J.S.], DK 62497 [A.G.M.], DK 62481 [B.A.H.], DK 62470 [S.J.K.], DK 62500 [P.R.], DK 62530 [K.S.], DK 62445 [N.K.], DK 62466 [B.L.S.], DK 62456 [J.C.M.], DK 62452 [Y.T.], DK 62436 [L.B.], DK 84585 [R.R.].

The authors report no conflicts of interest.

Copyright 2012 by ESPGHAN and NASPGHAN