AI Article Synopsis
- Pulmonary Langerhans cell histiocytosis is an interstitial lung disease characterized by the buildup of specific cells in the lungs, which can lead to issues like spontaneous pneumothorax due to lung tissue damage and cyst formation.
- A case study of a 2-year-old boy showed recurrent bilateral spontaneous pneumothorax with CT scans revealing significant lung damage, fibrosis, and cysts, leading to a confirmed diagnosis.
- The child was treated with prednisone and etoposide, resulting in a positive clinical response and improvements noted in a follow-up CT scan.
Article 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.
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| http://dx.doi.org/10.5546/aap.2014.e113 | DOI Listing |
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