title: Hepatopulmonary Syndrome in Patients With Cystic Fibrosis and Liver Disease.
abstract: Hepatopulmonary 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.
authors: Pediatric Pulmonology Unit and CF Center, Hadassah-Hebrew University Medical Center, Jerusalem, Israel.
authors: Oded
authors: O
authors: Breuer
authors: Pediatric Gastroenterology Unit, Hadassah-Hebrew University Medical Center, Jerusalem, Israel.
authors: Eyal
authors: E
authors: Shteyer
authors: Pediatric Gastroenterology Unit, Hadassah-Hebrew University Medical Center, Jerusalem, Israel.
authors: Michael
authors: M
authors: Wilschanski
authors: Pediatric Cardiology Unit, Hadassah-Hebrew University Medical Center, Jerusalem, Israel.
authors: Zeev
authors: Z
authors: Perles
authors: Pediatric Pulmonology Unit and CF Center, Hadassah-Hebrew University Medical Center, Jerusalem, Israel.
authors: Malena
authors: M
authors: Cohen-Cymberknoh
authors: Pediatric Pulmonology Unit and CF Center, Hadassah-Hebrew University Medical Center, Jerusalem, Israel.
authors: Eitan
authors: E
authors: Kerem
authors: Pediatric Pulmonology Unit and CF Center, Hadassah-Hebrew University Medical Center, Jerusalem, Israel. Electronic address: shoseyov@hadassah.org.il.
authors: David
authors: D
authors: Shoseyov
mesh_terms: D000293:Adolescent; D003550:Cystic Fibrosis / Q000150:complications / Q000175:diagnosis*; D003937:Diagnosis, Differential; D005541:Forced Expiratory Volume; D020065:Hepatopulmonary Syndrome / Q000175:diagnosis* / Q000209:etiology; D006801:Humans; D008103:Liver Cirrhosis / Q000175:diagnosis* / Q000209:etiology; D008297:Male; D014057:Tomography, X-Ray Computed
keywords: hypoxia; intrapulmonary vascular shunt; liver disease; portal hypertension