title: Long survival of congenital alveolar capillary dysplasia patient with NO inhalation and epoprostenol: effect of sildenafil, beraprost and bosentan.
abstract: The case is described herein of a patient with alveolar capillary dysplasia with double-outlet right ventricle and duodenal atresia who survived for a remarkably long time. The newborn girl was born at a gestational age of 36 weeks and weighed 1926 g. One min after delivery the Apgar score was 4. The patient had persistent pulmonary hypertension (PH) and needed nitric oxide inhalation and i.v. epoprostenol all through her life. Although other oral medications for PH were tried, they could not be used in practice because of gastrointestinal complications. The patient died on the 237 th day of life as a result of worsening PH associated with infection.
authors.affiliation: Department of Neonatology, Japanese Red Cross Medical Center, Tokyo, Japan. kodamayoshihiko@gmail.com
authors.forename: Yoshihiko
authors.initials: Y
authors.lastname: Kodama
authors.forename: Katsuo
authors.initials: K
authors.lastname: Tao
authors.forename: Fumihiko
authors.initials: F
authors.lastname: Ishida
authors.forename: Tadashi
authors.initials: T
authors.lastname: Kawakami
authors.forename: Keiji
authors.initials: K
authors.lastname: Tsuchiya
authors.forename: Kazuo
authors.initials: K
authors.lastname: Ishida
authors.forename: Tamiko
authors.initials: T
authors.lastname: Takemura
authors.forename: Atsuko
authors.initials: A
authors.lastname: Nakazawa
authors.forename: Kentaro
authors.initials: K
authors.lastname: Matsuoka
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Yoda
mesh_terms: D000280:Administration, Inhalation; D000077300:Bosentan; D001993:Bronchodilator Agents / Q000008:administration & dosage; D004305:Dose-Response Relationship, Drug; D004359:Drug Therapy, Combination; D011464:Epoprostenol / Q000008:administration & dosage / Q000031:analogs & derivatives* / Q000627:therapeutic use*; D017809:Fatal Outcome; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007231:Infant, Newborn; D009569:Nitric Oxide / Q000008:administration & dosage*; D010547:Persistent Fetal Circulation Syndrome / Q000175:diagnosis / Q000188:drug therapy* / Q000503:physiopathology; D010879:Piperazines / Q000008:administration & dosage / Q000627:therapeutic use*; D010975:Platelet Aggregation Inhibitors / Q000008:administration & dosage / Q000627:therapeutic use; D011650:Pulmonary Alveoli / Q000002:abnormalities* / Q000503:physiopathology; D011687:Purines / Q000008:administration & dosage / Q000627:therapeutic use; D000068677:Sildenafil Citrate; D013449:Sulfonamides / Q000008:administration & dosage / Q000627:therapeutic use*; D013450:Sulfones / Q000008:administration & dosage / Q000627:therapeutic use*; D013997:Time Factors; D014665:Vasodilator Agents / Q000008:administration & dosage / Q000627:therapeutic use
chemical_list: D001993:Bronchodilator Agents; D010879:Piperazines; D010975:Platelet Aggregation Inhibitors; D011687:Purines; D013449:Sulfonamides; D013450:Sulfones; D014665:Vasodilator Agents; D009569:Nitric Oxide; C048081:beraprost; D000068677:Sildenafil Citrate; D011464:Epoprostenol; D000077300:Bosentan