Portal Hypertension in Children and Young Adults With Biliary Atresia

Abstract

Objectives 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 (ChiLDREN) was utilized to perform a cross-sectional multi-centered analysis of PHT in children with BA. Methods BA subjects receiving medical management at a ChiLDREN site were enrolled. A priori, clinically evident PHT was defined as “definite” when there was either 1) history of a complication of PHT or 2) 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, while PHT was “absent” if none of the criteria were met. Results 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. ALT, GGTP, and sodium levels were similar, while there were significant differences in AST, AST/ALT, albumin, total bilirubin, PT, WBC, platelet count and AST/platelet between definite and absent PHT. Thirty-four percent of those with definite PHT had either PT > 15s or albumin < 3 g/L. 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.

Biliary Atresia - Clinical Series

“…Moreover, a portal hypertension development rate (44.4%) inferior to that registered in other studies [13,16,21] was documented in this series. These studies describe that over 2/3 of biliary atresia patients will present with this complication.…”

“…This difference is probably related to the short follow-up time of some of the studied patients, in whom portal hypertension may be documented in the future. However, since this diagnosis is often clinical and based on diagnostic tests -in the same way as it was defined in this series -portal hypertension prevalence in biliary atresia may be underestimated [21].…”

Comprehensive Predictors of Portal Pressure from Functional Liver Reserve in Patients Who Underwent Hepatectomy

“…Clinical features associated with the presence of portal hypertension include ascites, splenomegaly, and venous dilatation as caput medusa [20]. Thrombocytopenia is an important predictor of significant portal hypertension and the presence of esophagealgastric varices [21]. The ratio of platelet count to spleen size is thought to offer a surrogate marker [22].…”

A conceptual and practical approach to haemostasis in paediatric liver disease

“…Most clinical data regarding the effects of portal hypertension in children arise from studies in patients with biliary atresia. Approximately 20% of children with biliary atresia experience oesophageal variceal haemorrhage 46. Recurrent variceal bleeding is associated with a high mortality 47.…”