title: Nitric oxide for post-liver-transplantation hypoxemia in pediatric hepatopulmonary syndrome: case report and review.
abstract: HPS is rare in the pediatric population. Liver transplantation is the ultimate treatment for severe HPS. There are only a few case reports and one series of children in whom HPS was the main indication for liver transplantation. Outcome was good in most of them, with full regression of the pulmonary process. However, hypoxemia in the early post-operative course can have severe consequences, and effective treatment modalities are needed. There are rare instances of the use of iNO for the treatment of post-operative hypoxemia. We describe a 10.5-yr-old boy with severe HPS owing to chronic liver disease after bone marrow transplantation. Liver transplantation from a living related donor (the same sister who donated the bone marrow) was complicated by severe hypoxemia on POD 2. iNO was administered via the ventilator circuit and, after extubation, through nasal prongs. It was slowly tapered down and stopped on POD 10. The child had an otherwise uneventful course and was discharged home on POD 21 with normal oxygen saturation. Liver transplantation should be offered to children with severe HPS. iNO can reverse the hypoxemia that may occur after the operation.
authors.affiliation: Pediatric Intensive Care Unit, Schneider Children's Medical Center of Israel, Petah Tiqwa 49202, Israel. schillero@gmail.com
authors.forename: Ofer
authors.initials: O
authors.lastname: Schiller
authors.forename: Yaron
authors.initials: Y
authors.lastname: Avitzur
authors.forename: Gili
authors.initials: G
authors.lastname: Kadmon
authors.forename: Elchanan
authors.initials: E
authors.lastname: Nahum
authors.forename: Ran M
authors.initials: RM
authors.lastname: Steinberg
authors.forename: Vered
authors.initials: V
authors.lastname: Nachmias
authors.forename: Tommy
authors.initials: T
authors.lastname: Schonfeld
mesh_terms: D000792:Angiography / Q000379:methods; D016026:Bone Marrow Transplantation / Q000009:adverse effects; D002648:Child; D004452:Echocardiography / Q000379:methods; D058625:End Stage Liver Disease / Q000150:complications; D020065:Hepatopulmonary Syndrome / Q000628:therapy*; D006801:Humans; D006965:Hyperplasia / Q000473:pathology; D000860:Hypoxia / Q000150:complications / Q000378:metabolism / Q000473:pathology; D016031:Liver Transplantation / Q000379:methods*; D008168:Lung / Q000473:pathology; D008297:Male; D009569:Nitric Oxide / Q000378:metabolism*; D010100:Oxygen / Q000737:chemistry; D011184:Postoperative Period; D014057:Tomography, X-Ray Computed / Q000379:methods; D016896:Treatment Outcome
chemical_list: D009569:Nitric Oxide; D010100:Oxygen