title: [Alveolar capillary dysplasia as a cause of failure in treatment of a neonate with pulmonary persistent hypertension of the newborn - case report].
abstract: A patient with severe persistent pulmonary hypertension of the newborn (PPHN) due to alveolar capillary dysplasia, congenital (ACD), is presented. In the treatment, apart from standard methods, high frequency oscillatory ventilation (HFOV), inhaled nitric oxide and activated C protein have been applied. In spite of treatment the patient died and post-mortem diagnosis was based on lung histopathology examination. ACD occurs very rarely and is a congenital disease. Diagnosis is by pulmonary tissue histopathology examination. Pathological structure of the lungs leads to severe dysfunction of gas exchange as well as increasing pulmonary hypertension. No effective treatment is known and all so far described cases have ended up with death. The described case and literature data lead the authors to the following conclusions: 1. in case of PPHN resistant to treatment, ACD diagnosis should be taken into consideration, 2. histopathological examination determines the diagnosis, 3. limited capabilities of diagnosis are the reason for applying non-standard and expensive treatment methods which so far are doomed to failure, 4. in case of a patient with severe, persistent pulmonary hypertension and unclear aetiology, not reacting to nitrous oxide treatment, a diagnostic lung biopsy should be considered.
authors.affiliation: Oddział Intensywnej Terapii Dzieci, Wojewódzkie Centrum Medyczne, Al. W. Witosa 26, 45-418 Opole, Poland. dorota@mik.pl
authors.forename: Dorota
authors.initials: D
authors.lastname: Granatowska
authors.forename: Wojciech
authors.initials: W
authors.lastname: Walas
authors.forename: Elzbieta
authors.initials: E
authors.lastname: Jakuszewska
authors.forename: Jacek
authors.initials: J
authors.lastname: Masełko
authors.forename: Ewa
authors.initials: E
authors.lastname: Zembala-Nozyńska
authors.forename: Robert
authors.initials: R
authors.lastname: Smigiel
mesh_terms: D002196:Capillaries / Q000473:pathology; D005260:Female; D006612:High-Frequency Ventilation; D006801:Humans; D007231:Infant, Newborn; D010547:Persistent Fetal Circulation Syndrome / Q000473:pathology* / Q000628:therapy; D011650:Pulmonary Alveoli / Q000098:blood supply / Q000473:pathology*; D011659:Pulmonary Gas Exchange; D016896:Treatment Outcome