Abstract
Cystic fibrosis (CF) is an inherited multisystemic disorder that results in a generalized dysfunction of exocrine glands. Besides chronic obstructive pulmonary diseases, chronic sinusitis, nasal polyposis and hypertrophy of the inferior turbinates with nasal airway obstruction are typical signs. Tissue samples of the inferior turbinates and nasal polyps were taken during nasal surgery from 21 children, ranging from 3 to 16 years of age. Light- and electron microscopical examination were carried out. Furthermore, specimens of nasal mucosa of patients without chronic inflammation as controls and specimens of duodenal mucosa of patients with CF were investigated. Under a thick layer of respiratory epithelium with a high proportion of goblet cells, the seromucous glands display abnormal morphological structures with wide mucous cells and cystic dilatation. The glandular cells show inhomogeneous glandular droplets in the supranuclear cell portion. The nucleus contains dispersed chromatin as a sign of increased activity and the structures of the Golgi apparatus are clearly detectable. Apart from investigations concerning nasal polyps in CF, studies on the different morphological changes of nasal mucosa at the electron microscopic level are rare. This histological study focuses on various morphological changes of nasal glands at the ultrastructural level in correlation with typical symptoms in CF. In addition, a comparison with electron microscopic findings of CF-enteropathies is proposed. These findings could help to bring information concerning new morphological aspects in the pathophysiology of patients with CF.
Similar content being viewed by others
References
Agha-Mir-Salim P, Merker HJ, Jahnke V, Berghaus A (1998) Glands of the human nasal mucosa—electron microscopic and immunohistochemical investigations. Laryngo Rhino Otol 77:322–327
Beju D, Meek WD, Kramer JC (2004) The ultrastructure of the nasal polyps in patients with and without cystic fibrosis. J Submicrosc Cytol Pathol 36(2):155–165
Bergoin C, Gosset P, Lamblin C, Bolard F, Turck D, Tonnel AB, Wallaert B (2002) Cell and cytokine profile in nasal secretions in cystic fibrosis. J Cyst Fibros 1(3):110–115
Crockett DM, McGill TJ, Healy GB, Friedman EM, Salkeld LJ (1987) Nasal and paranasal sinus surgery in children with cystic fibrosis. Ann Otol Rhinol Laryngol 96:367–372
Deitmer TH (1992) Current understanding of mucociliary transport. HNO 40:459–463
Drake-Lee AB, Morgan DW (1989) Nasal polyps and sinusitis in children with cystic fibrosis. J Laryngol Otol 103:753–755
Irving RM, McMahon R, Clark R, Jones NS (1997) Cystic fibrosis transmembrane conductance regulator gene mutations in severe nasal polyposis. Clin Otolaryngol 22:519–521
Henderson WR, Chi EY (1992) Degranulation of cystic fibrosis nasal polyp mast cells. J Pathol 166(4):395–404
Hui Y, Gaffney R, Crysdale WS (1995) Sinusitis in patients with cystic fibrosis. Eur Arch Otorhinolaryngol 252:191–196
Jahnke V, Theopold HM (1977) Fine structure of nasal mucosa in mucociscidosis, with special regard to polyposis. Laryngo Rhinol 56:773–781
Jahnke V, Merker HJ (1998) Electron microscopy of the human vomeronasale organ. HNO 46:502–506
Knipping St, Riederer A, Mir-Salim PA, Berghaus A (2000) Ultrastructural innervation pattern of nasal mucosal glands. Laryngo Rhino Otol 79:146–150
Markmann HU, Matthus J (1990) Das primäre ziliare Dyskinesiesyndrom. Ultrastrukturelle Beobachtungen bei einem Zwillingspaar. Pathologe 11:80–84
Reiß M (1997) Current clinical concepts regarding the diagnosis and therapy of nasal polyposis. Wien Klein Wochenschr 109/20:820–825
Rulon JT, Brown H, Logan GB (1963) Nasal polyps and Cystic Fibrosis of the pancreas. Arch Otorhinolaryngol 78:192–199
Schwachman H, Kulczycki LL, Mueller HL, Flake CG (1962) Nasal polyposis in patients with cystic fibrosis. Pedriatics 30:389–401
Tandon R, Derkay C (2003) Contemporary management of rhinosinusitis and cystic fibrosis. Curr Opin Otolaryngol Head Neck Surg 11:41–44
Tos M, Morgensen C, Thomsen J (1977) Nasal polyps in cystic fibrosis. J Laryngol Otol 91:827–835
Yung MW, Gould J, Upton GJG (2002) Nasal polyposis in children with cystic fibrosis: a long follow-up study. Ann Otol Rhino Laryngol 111(12):1081–1086
Acknowledgments
Financial support was provided by the German Research Society (DFG, KN 501/1-2). In particular, we would like to thank the medical laboratory assistants Ms. C. Fischer and Ms. K. Linde for their collaboration.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Knipping, S., Holzhausen, H.J., Riederer, A. et al. Cystic fibrosis: ultrastructural changes of nasal mucosa. Eur Arch Otorhinolaryngol 264, 1413–1418 (2007). https://doi.org/10.1007/s00405-007-0393-y
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00405-007-0393-y