Natural history, risk factors, and outcome of hepatopulmonary syndrome in pediatric liver diseases.

BACKGROUND

Limited pediatric literature is available regarding hepatopulmonary syndrome (HPS) especially in subjects with biliary atresia (BA) despite its proven prognostic significance. Thus, we aimed to study the natural history, risk factors, and outcome of HPS in BA and other chronic liver disease (CLD) subjects.

METHODS

All children (BA and other non-BA CLDs) older than 6 months of age were included in the study. HPS was diagnosed on the basis of standard international criteria. Also, fractional exhaled nitric oxide (FeNO) was measured at baseline.

RESULTS

During the study period from January 2017 to December 2018, there were 42 children in BA and 62 in the CLD group. The overall prevalence of HPS was 42.3%: 57.1% in the BA group and 32.2% in the CLD group. Median age at HPS diagnosis was 14.4 months and 90 months in the BA and non-BA CLD groups, respectively. By the end of study period, the prevalence of HPS in the BA group further increased to 73.8% at 0.7% per month. Lower serum albumin (p < 0.05) in BA and higher splenic Z scores (p 0.013) in other CLDs were found to be significant risk factors for HPS. FeNO measurement did not reach diagnostic significance.

CONCLUSION

Prevalence of HPS is higher and also develops at an earlier age in the BA group compared to other CLDs. Also, risk of HPS development increases with increasing disease duration in BA. Lower serum albumin in BA and higher splenic Z scores in other CLDs may predict risk for HPS development.