Hepatopulmonary syndrome in children: Literature review and clinical observation

Hepatopulmonary syndrome (HPS) is a syndrome characterized by arterial hypoxemia due to arteriovenous shunting in lung vessels with various liver diseases. HPS is diagnosed in 9-20% of sick children with the final stage of liver disease. Pathognomonic clinical signs of HPS are dyspnea increase on standing (platypnea) and oxygenation decrease in the standing position (orthodeoxy). To date, main methods of HPS treatment are oxygen therapy and liver transplantation. The article presents modern information on epidemiology, etiology, pathogenesis, study history, clinical picture, clinical and laboratory-instrumental diagnostics, therapy and disease prognosis on the basis of domestic and foreign literature and unique observations. Based on available publications and own observations of patients with HPS authors analyze data on demographic characteristics, etiology (based on liver biopsy results), computed tomography results, pathophysiology (blood oxygen saturation with room air breathing, intrapulmonary shunt ratio), therapy and disease outcome in 26 children and adolescents. The article provides clinical observation of late HPS diagnostics in a 7 year old girl hiding under the diagnosis of idiopathic pulmonary fibrosis. © 2017, Pediatria Ltd. All Rights Reserved.