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Portal hypertension in children and young adults with biliary atresia
Benjamin L Shneider1, Bob Abel, Barbara Haber
1Children's Hospital Pittsburgh of UPMC, Division of Pediatric Gastoenterology, Hepatology and Nutrition, PA 15224. Benjamin.Shneider@chp.edu
Insights
Portal hypertension (PHT) affects two-thirds of children with biliary atresia (BA) who survive long-term. PHT is linked to liver injury but shows mild dysfunction and preserved growth in this BA cohort.
Area of Science:
- Pediatric Hepatology
- Gastroenterology
- Clinical Research
Background:
- Biliary atresia (BA) is a severe liver disease in infants.
- Portal hypertension (PHT) is a common complication of BA.
- PHT leads to significant morbidity and mortality in children with BA.
Purpose of the Study:
- To analyze the prevalence and characteristics of PHT in children with BA.
- To identify clinical factors associated with PHT in BA survivors.
Main Methods:
- Cross-sectional multicentered analysis of 163 children with BA.
- Defined PHT as definite (complications or splenomegaly/thrombocytopenia), possible (one sign), or absent.
- Collected demographic, growth, anthropometric, and laboratory data.
Main Results:
- Definite PHT was present in 49% of subjects, possible in 17%, and absent in 34%.
- Subjects with definite PHT showed significant differences in AST, albumin, bilirubin, prothrombin time, and platelet count compared to those without PHT.
- 34% of those with definite PHT had prolonged prothrombin time or low albumin.
Conclusions:
- Clinically definable PHT is present in two-thirds of North American BA survivors with native livers.
- PHT presence correlates with indicators of hepatic injury and dysfunction.
- In this cohort, hepatic dysfunction was mild, and growth was preserved.
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.
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