Diffuse lymphoplasmacytic bronchiolitis in cartilage-hair hypoplasia.
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.
Bailly-Botuha
Céline
C
Université Paris Descartes, Lyon, France.
Jaubert
Francis
F
Taam
Rola Abou
RA
Galmiche
Louise
L
Picard
Capucine
C
Bellon
Gabriel
G
de Blic
Jacques
J
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
D017291:Clarithromycin