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J Pediatr. 2008 Mar;152(3):429-33. doi: 10.1016/j.jpeds.2007.10.010.

Diffuse lymphoplasmacytic bronchiolitis in cartilage-hair hypoplasia.

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  • 1Universit√© Paris Descartes, Lyon, France.


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.

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