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Arch Argent Pediatr. 2016 Jun 1;114(3):e175-8. doi: 10.5546/aap.2016.e175. Epub 2016 Jun 1.

[Diffuse lung disease: cause of persistent pulmonary hypertension before one year of age].

[Article in Spanish; Abstract available in Spanish from the publisher]

Author information

1
Servicio de Neumonología, Hospital de Pediatría "Prof. Dr. Juan P. Garrahan", Ciudad Autónoma de Buenos Aires, Argentina. manu_dice@hotmail.com.
2
Servicio de Neumonología, Hospital de Pediatría "Prof. Dr. Juan P. Garrahan", Ciudad Autónoma de Buenos Aires, Argentina.
3
Servicio de Anatomía Patológica, Hospital de Pediatría "Prof. Dr. Juan P. Garrahan", Ciudad Autónoma de Buenos Aires, Argentina.

Abstract

in English, Spanish

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.

KEYWORDS:

Alveolar capillary dysplasia with misalignment of pulmonary veins; Diffuse parenchymal lung disease; Pulmonary hypertension

PMID:
27164352
DOI:
10.5546/aap.2016.e175
[Indexed for MEDLINE]
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