title: Nitric oxide inhalation therapy for an infant with persistent pulmonary hypertension caused by misalignment of pulmonary veins with alveolar capillary dysplasia.
abstract: Misalignment of pulmonary veins with alveolar capillary dysplasia (MPV) has been reported to be a rare cause of persistent pulmonary hypertension of the newborn (PPHN) and to be fatal despite extracorporeal membrane oxygenation (ECMO). A full-term female neonate with PPHN was brought to the hospital for ECMO therapy at 2 days of age. On the 14th day of life, she was extubated early after the second run of ECMO, and underwent nitric oxide (NO) inhalation therapy in the incubator. She died of catheter-related sepsis on the 61st day of life. After autopsy findings revealed MPV, the longest survival with this disease was documented. NO inhalation therapy in the incubator may provide time for lung transplantation.
authors.affiliation: Department of Pediatric Surgery, Osaka University Medical School, Japan.
authors.forename: Y
authors.initials: Y
authors.lastname: Kitayama
authors.forename: S
authors.initials: S
authors.lastname: Kamata
authors.forename: H
authors.initials: H
authors.lastname: Okuyama
authors.forename: N
authors.initials: N
authors.lastname: Usui
authors.forename: T
authors.initials: T
authors.lastname: Sawai
authors.forename: T
authors.initials: T
authors.lastname: Kobayashi
authors.forename: Y
authors.initials: Y
authors.lastname: Fukui
authors.forename: A
authors.initials: A
authors.lastname: Okada
mesh_terms: D000280:Administration, Inhalation; D002196:Capillaries / Q000002:abnormalities; D002405:Catheterization, Central Venous / Q000009:adverse effects; D015199:Extracorporeal Membrane Oxygenation; D017809:Fatal Outcome; D005260:Female; D006612:High-Frequency Ventilation; D006801:Humans; D007185:Incubators; D007231:Infant, Newborn; D007442:Intubation, Intratracheal; D009569:Nitric Oxide / Q000008:administration & dosage / Q000627:therapeutic use*; D010547:Persistent Fetal Circulation Syndrome / Q000188:drug therapy* / Q000473:pathology; D011650:Pulmonary Alveoli / Q000098:blood supply*; D011667:Pulmonary Veins / Q000002:abnormalities*; D012138:Respiratory Therapy; D018805:Sepsis; D015996:Survival Rate
chemical_list: D009569:Nitric Oxide