Diffuse lymphoplasmacytic bronchiolitis in cartilage-hair hypoplasia.
case_report · Level V
Where this comes from
- Record sourced from PubMed, PMID 18280854.
- Also identified by DOI 10.1016/j.jpeds.2007.10.010.
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Abstract
Three children with cartilage-hair hypoplasia presented with chronic obstructive symptoms and bronchiolar wall thickening on high-resolution computed tomography scanning. In all children, surgical lung biopsy demonstrated diffuse dilated lymphoplasmacytic bronchiolitis. The bronchiolar wall was infiltrated by a lymphocyte sheath with plasma cell differentiation and dispersed secondary follicles. Clarithromycin substantially improved respiratory symptoms and pulmonary function, allowing children to return home.
Medical subject headings
- Abnormalities, Multiple
- Bronchiolitis
- Hair
- Lung
- Lymphopenia
- Pulmonary Disease, Chronic Obstructive