Author | Year | CF subjects | Structures studied | Main findings |
Gilljam18 | 1987 | Adults (n=7) | Epithelium | Loss of microvilli, otherwise no abnormalities of the epithelium |
Durieu19 | 1998 | Children and adults, age 10–30 years (n=6) | RBM, subepithelium | Thinning of the basal lamina, subepithelial fibrosis, degradation of the extracellular matrix |
Wojnarowski20 | 1999 | Children, age 6–17 years (n=16) | Epithelium, subepithelium | Lymphocytic infiltrate in stable disease, neutrophilic infiltrate in exacerbation; high expression of TGF-β1 and IFN-γ associated with mild disease |
Hauber21 | 2004 | Adults (n=10) | Epithelium | Increased PAS staining in the epithelium; increased expression of IL-9, IL-9R and hCLCA1 in mucus-producing cells |
Hays22 | 2005 | Adults (n=7) | Smooth muscle | Hyperplasia without hypertrophy of airway smooth muscle |
Hays23 | 2006 | Adults (n=7) | Goblet cells, mucus-secreting glands | Increased goblet cell size but normal number; hypertrophy of submucosal glands, with normal proportion of mucous and serous acini |
Hilliard24 | 2007 | Children, age 0–17 years (n=43) | RBM | RBM thickening related to TGF-β1 in BAL but independent of other markers of inflammation |
Regamey25 | 2008 | Children, age 6–17 years (n=27) | Smooth muscle | Increase in airway smooth muscle (both hyperplasia and hypertrophy) |
hCLCA, human calcium-activated chloride channel; IFN, interferon; IL, interleukin; PAS, periodic acid-Schiff; RBM, reticular basement membrane; TGF, transforming growth factor.