Portal Hypertension in Children and Young Adults With Biliary Atresia

• Authors: Benjamin L. Shneider, Children's Hospital Pittsburgh; Bob Abel, University of Michigan; Barbara Haber, Children's Hospital of Philadelphia; Saul Karpen, Emory University; John C. Magee, University of Michigan; Rene Romero, Emory University; Kathleen Schwarz, Johns Hopkins University; Lee M. Bass, Children's Memorial Hospital; Nanda Kerkar, Mt Sinai School of Medicine; Alexander G. Miethke, Cincinnati Children's Hospital Medical Center; Philip Rosenthal, University of California, San Francisco; Yumirle Turmelle, Washington University; Patricia R. Robuck, National Institute of Diabetes and Digestive and Kidney Diseases,; Ronald J. Sokol, University of Colorado
• Journal Title: Journal of Pediatric Gastroenterology and Nutrition
• Volume: Volume 55, Number 5
• Publisher: Lippincott, Williams & Wilkins | 2012-11-01, Pages 567-573
• Type of Work: Article | Post-print: After Peer Review
• Publisher DOI: http://doi.org/10.1097/MPG.0b013e31826eb0cf
• Subjects: Health Sciences, Medicine and Surgery
• Keywords: Science & Technology; Life Sciences & Biomedicine; Gastroenterology & Hepatology; Nutrition & Dietetics; Pediatrics; ascites; hepatopulmonary syndrome; hypersplenism; pediatric; varices; EXPERT PEDIATRIC OPINION; PLATELET RATIO INDEX; KASAI OPERATION; VARICEAL HEMORRHAGE; CONSENSUS WORKSHOP; ESOPHAGEAL-VARICES; PROGNOSTIC VALUE; EXPERIENCE; DIAGNOSIS; FIBROSIS

Abstract

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.