title: [Diffuse lung disease: cause of persistent pulmonary hypertension before one year of age].
abstract: Pulmonary vascular disease in children is multifactorial and heterogeneous. While it shares some features with pulmonary hypertension in adults, there are differences in the associated comorbidities and conditions, the coexistence of genetic or developmental diseases. Interstitial lung diseases may be responsible for this entity. One is alveolar capillary dysplasia with misalignment of pulmonary veins, a rare pathology but with a mortality rate of 100%, characterized by a failure in the formation of lung tissue that eventually results in impaired gas diffusion. We present a 5-month-old patient studied due to suspected congenital heart disease with persistent hypoxemia; diagnosis was made through lung biopsy.
authors.affiliation: Servicio de Neumonología, Hospital de Pediatría "Prof. Dr. Juan P. Garrahan", Ciudad Autónoma de Buenos Aires, Argentina. manu_dice@hotmail.com.
authors.forename: Manuela
authors.initials: M
authors.lastname: Dicembrino
authors.affiliation: Servicio de Neumonología, Hospital de Pediatría "Prof. Dr. Juan P. Garrahan", Ciudad Autónoma de Buenos Aires, Argentina.
authors.forename: Dora
authors.initials: D
authors.lastname: Haag
authors.affiliation: Servicio de Anatomía Patológica, Hospital de Pediatría "Prof. Dr. Juan P. Garrahan", Ciudad Autónoma de Buenos Aires, Argentina.
authors.forename: Mariana
authors.initials: M
authors.lastname: Álvarez
authors.affiliation: Servicio de Neumonología, Hospital de Pediatría "Prof. Dr. Juan P. Garrahan", Ciudad Autónoma de Buenos Aires, Argentina.
authors.forename: Agustina
authors.initials: A
authors.lastname: Díaz Cazaux
authors.affiliation: Servicio de Neumonología, Hospital de Pediatría "Prof. Dr. Juan P. Garrahan", Ciudad Autónoma de Buenos Aires, Argentina.
authors.forename: Claudio
authors.initials: C
authors.lastname: Castaños
mesh_terms: D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology*; D007223:Infant; D017563:Lung Diseases, Interstitial / Q000150:complications*
keywords: Alveolar capillary dysplasia with misalignment of pulmonary veins; Diffuse parenchymal lung disease; Pulmonary hypertension