title: Diffuse lymphoplasmacytic bronchiolitis in cartilage-hair hypoplasia.
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.
authors.affiliation: Université Paris Descartes, Lyon, France.
authors.forename: Céline
authors.initials: C
authors.lastname: Bailly-Botuha
authors.forename: Francis
authors.initials: F
authors.lastname: Jaubert
authors.forename: Rola Abou
authors.initials: RA
authors.lastname: Taam
authors.forename: Louise
authors.initials: L
authors.lastname: Galmiche
authors.forename: Capucine
authors.initials: C
authors.lastname: Picard
authors.forename: Gabriel
authors.initials: G
authors.lastname: Bellon
authors.forename: Jacques
authors.initials: J
authors.lastname: de Blic
mesh_terms: D000015:Abnormalities, Multiple / Q000175:diagnosis*; D001707:Biopsy, Needle; D001988:Bronchiolitis / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology*; D001992:Bronchoalveolar Lavage Fluid / Q000166:cytology; D001999:Bronchoscopy / Q000379:methods; D002675:Child, Preschool; D002806:Chondrodysplasia Punctata; D017291:Clarithromycin / Q000627:therapeutic use; D004392:Dwarfism; D005260:Female; D005500:Follow-Up Studies; D006197:Hair / Q000002:abnormalities*; D006801:Humans; D007039:Hypotrichosis / Q000175:diagnosis; D007150:Immunohistochemistry; D008168:Lung / Q000473:pathology*; D008231:Lymphopenia / Q000175:diagnosis* / Q000188:drug therapy; D008297:Male; D010950:Plasma Cells / Q000473:pathology; D029424:Pulmonary Disease, Chronic Obstructive / Q000175:diagnosis*; D011659:Pulmonary Gas Exchange; D012129:Respiratory Function Tests; D018570:Risk Assessment; D012720:Severity of Illness Index; D013577:Syndrome; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
chemical_list: D017291:Clarithromycin