Hepatopulmonary syndrome from liver disease associated with autosomal recessive polycystic kidney disease.

Hepatopulmonary syndrome (HPS) is a life-threatening complication of chronic liver disease (CLD) that currently can be managed only by liver transplant. Though uncommon, some children with kidney disease have coexistent CLD and hence are at risk of developing HPS. Paediatric cases of HPS are rarely described in the nephrology literature. We present a patient with autosomal recessive polycystic kidney disease (ARPKD) and persistent hypoxaemia diagnosed as HPS and successfully managed with a liver transplant. We suggest that nephrologists caring for children with kidney and concomitant CLD consider routine screening for HPS with pulse oximetry.