title: Rehabilitating patients with hepatopulmonary syndrome using living-related orthotopic liver transplant: a case report.
abstract: The objective of this study was to rehabilitate a patient with hepatopulmonary syndrome (HPS) who underwent living-related orthotopic liver transplantation (LT). HPS is rare; it presents severe complication in patients with liver disease. A 17-year-old woman with HPS developed portal hypertension after undergoing Kasai's surgery for congenital biliary atresia and underwent a living-related orthotopic LT. After LT, her allograft functioned well, but she continued to have hypoxemia and orthodeoxia. She was referred for rehabilitation for disuse atrophy, contracture of hip and shoulder joints, left common peroneal nerve palsy, and rehabilitation for respiratory dysfunction. By day 106 after LT, her orthodeoxia and disuse atrophy had improved because of daily exercise training and active joint range of motion exercises. Patients with HPS have orthodeoxia and poor responsiveness to oxygen therapy, and correction of hypoxemia after LT may be delayed. Therefore, rehabilitation approaches for patients with HPS should be based on the pathophysiology and characteristics of HPS.
authors.affiliation: Department of Internal Medicine & Rehabilitation Science, Tohoku University Graduate School of Medicine, Sendai, Japan.
authors.forename: M
authors.initials: M
authors.lastname: Kohzuki
authors.forename: T
authors.initials: T
authors.lastname: Abo
authors.forename: M
authors.initials: M
authors.lastname: Watanabe
authors.forename: Y
authors.initials: Y
authors.lastname: Goto
authors.forename: N
authors.initials: N
authors.lastname: Ohkohchi
authors.forename: S
authors.initials: S
authors.lastname: Satomi
authors.forename: T
authors.initials: T
authors.lastname: Sato
mesh_terms: D000293:Adolescent; D000792:Angiography; D001164:Arteriovenous Fistula / Q000150:complications / Q000000981:diagnostic imaging; D001784:Blood Gas Analysis; D005081:Exercise Therapy; D017079:Exercise Tolerance; D020065:Hepatopulmonary Syndrome / Q000150:complications / Q000175:diagnosis / Q000534:rehabilitation* / Q000601:surgery*; D006801:Humans; D006975:Hypertension, Portal / Q000209:etiology; D000860:Hypoxia / Q000209:etiology / Q000628:therapy; D008111:Liver Function Tests; D016031:Liver Transplantation / Q000534:rehabilitation*; D008297:Male; D010102:Oxygen Inhalation Therapy; D011187:Posture; D011651:Pulmonary Artery / Q000002:abnormalities / Q000000981:diagnostic imaging; D012129:Respiratory Function Tests; D016896:Treatment Outcome; D016138:Walking