[Bilateral spontaneous pneumothorax as a setting of Langerhans cell histiocytosis].

Abstract:

:Pulmonary Langerhans cell histiocytosis is an interstitial lung disease that results from the accumulation of specific histiocytic cells in the lung. Spontaneous pneumothorax is a recognized feature of pulmonary Langerhans cell histiocytosis and results from destruction of lung parenchyma with associated cystic changes. We report on a 2-year-old boy with recurrent bilateral spontaneous pneumothorax; a computed tomography scan showed marked interstitial changes, fibrosis, cystic spaces and bilateral bullae. The diagnosis was confirmed by the histology and the immunohistochemistry examination of the pulmonary biopsy with CD1 and S100 positive antibodies. The child was treated with prednisone and etoposide, and had a good clinical response and favorable changes in the second thoracic CT scan.

journal_name

Arch Argent Pediatr

authors

Martínez García JJ,Rios Osuna MG,Altamirano Álvarez E

doi

10.5546/aap.2014.e113

subject

Has Abstract

pub_date

2014-06-01 00:00:00

pages

e113-6

issue

3

eissn

0325-0075

issn

1668-3501

pii

S0325-00752014000300018

journal_volume

112

pub_type

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