2. Academic Validation
  3. Diffuse lymphoplasmacytic bronchiolitis in cartilage-hair hypoplasia

Diffuse lymphoplasmacytic bronchiolitis in cartilage-hair hypoplasia

  • J Pediatr. 2008 Mar;152(3):429-33. doi: 10.1016/j.jpeds.2007.10.010.
Céline Bailly-Botuha 1,  Francis Jaubert,  Rola Abou Taam,  Louise Galmiche,  Capucine Picard,  Gabriel Bellon,  Jacques de Blic
Affiliations

Affiliation

  • 1 Université Paris Descartes, Lyon, France.
PMID: 18280854 DOI: 10.1016/j.jpeds.2007.10.010
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.

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