• Oded Breuer, Eyal Shteyer, Michael Wilschanski, Zeev Perles, Malena Cohen-Cymberknoh, Eitan Kerem, and David Shoseyov.
• Pediatric Pulmonology Unit and CF Center, Hadassah-Hebrew University Medical Center, Jerusalem, Israel.
• Chest. 2016 Feb 1; 149 (2): e35-e38.

AbstractHepatopulmonary syndrome (HPS) is a liver-induced lung disorder defined as a triad of liver disease, pulmonary vascular dilatation, and a defect in oxygenation. It can complicate chronic liver disease of any etiology, but is most commonly associated with portal hypertension. Severe liver disease with portal hypertension is present in 2% to 8% of patients with cystic fibrosis (CF), but to date, to our knowledge, only one patient with CF has been reported to suffer from HPS. Here, we describe two patients with CF diagnosed with HPS, one subsequent to unresolved hypoxemia and the other following screening for HPS performed in our center. We speculate that HPS is underdiagnosed in patients with CF because of their coexisting respiratory morbidity, and we advocate routine screening for every patient with CF who has liver disease and portal hypertension.Copyright © 2016 The Authors. Published by Elsevier Inc. All rights reserved.

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