Histologic characteristics and mucin immunohistochemistry of cystic fibrosis sinus mucosa.

OBJECTIVES

To evaluate the histologic characteristics of paranasal sinus mucosa of a disease control population and children with chronic rhinosinusitis and cystic fibrosis (CRS/CF) (1) to determine whether goblet cell (GC) hyperplasia and/or submucosal gland (SMG) hyperplasia occur in pediatric CRS/CF and (2) to compare expression and localization of MUC5AC and MUC5B mucins in the sinus mucosa of both cohorts.

DESIGN

Histologic and morphometric analyses of paranasal sinus mucosa were used to quantify the number of GCs and mucin-expressing cells. Digital imaging was used to evaluate the SMG area. Immunohistochemistry was performed to identify the cellular localization of MUC5AC and MUC5B mucins, and confocal microscopy was used to determine whether MUC5AC and MUC5B mucins were expressed in the same secretory cells.

SETTING

Children's National Medical Center, Washington, DC.

PARTICIPANTS

Twenty-one children with CRS/CF who underwent endoscopic sinus surgical procedures and 18 children who underwent craniofacial resection or neurosurgical procedures for abnormalities other than sinusitis.

RESULTS

A statistically significant increased area (4.4-fold) of SMGs was detected in the sinus mucosa of patients with CRS/CF compared with the controls (P = .02). Neither GC hyperplasia nor increased expression of MUC5AC was observed in the CRS/CF group. MUC5AC was expressed only in a subpopulation of GCs in both cohorts, and MUC5B was expressed in a subpopulation of GCs as well as in SMGs. There was a positive trend toward increased glandular MUC5B expression in the CRS/CF cohort. Colocalization of MUC5AC and MUC5B expression was observed in a subset of GCs.

CONCLUSIONS

Significant SMG hyperplasia and a trend toward increased glandular MUC5B expression exist in children with CRS/CF. This suggests that SMG hyperplasia and glandular MUC5B mucin contribute to mucus overproduction in the sinus mucosa of this population.