Managing upper respiratory tract complications of primary ciliary dyskinesia in children

Current Opinion in Allergy and Clinical Immunology

Volumn 12, Issue 1, 2012, Pages 32-38

  Campbell, Raewyn ^a  

^a PRINCE OF WALES HOSPITAL   (Australia)

Author keywords

Chronic rhinosinusitis; Kartagener's syndrome; Otitis media with effusion; Primary ciliary dyskinesia; Ventilation tubes

Indexed keywords

MACROLIDE;

ANTIBIOTIC THERAPY; CHILDHOOD DISEASE; CILIARY DYSKINESIA; ENDOSCOPIC SURGERY; HEARING LOSS; HUMAN; MUCOCILIARY CLEARANCE; NOSE SURGERY; OTORRHEA; PHARMACOGENETICS; PRIORITY JOURNAL; RARE DISEASE; REVIEW; RHINOSINUSITIS; SECRETORY OTITIS MEDIA; UPPER RESPIRATORY TRACT;

ADOLESCENT; CHILD; FEMALE; HUMANS; KARTAGENER SYNDROME; MALE; MUCOCILIARY CLEARANCE; OTITIS MEDIA; SINUSITIS;

EID: 84857062817     PISSN: 15284050     EISSN: 14736322     Source Type: Journal    
DOI: 10.1097/ACI.0b013e32834eccc6     Document Type: Review

References (125)

1. Long SS, Pickering LK, Prober G. Long: principles and practice of pediatric infectious diseases. 2nd ed. Philadelphia: Churchill Livingstone; 2003.
2. Kumar V, Abbas A, Fausto N, et al. Pathologic basis of disease. 7th ed. Philadelphia: Elsevier Saunders; 2005.
3. Yoo Y, Koh YY. Current treatment of primary ciliary dyskinesia conditions. Expert Opin Pharmacother 2004; 5:369-377. (Pubitemid 38219863)
4. Behrman RE, Kliegman RM, Jenson HB. Nelson textbook of pediatrics. 17th ed. Philadelphia: Saunders; 2004.
5. Moore A, Escudier E, Roger G, et al. RPGR is mutated in patients with complete X-linked phenotype combining primary ciliary dyskinesia and retinitis pigmentosa. J Med Genet 2006; 43:326-333.
6. Katsuhara K, Kawamoto S, Wakabayashi T, et al. Situs inversus totalis and Kartagener's syndrome in a Japanese population. Chest 1972; 61:56-61.
7. Verra F, Escudier E, Bignon J, et al. Inherited factors in diffuse bronchiectasis in the adult: a prospective study. Eur Respir J 1991; 4:937-944.
8. Coren ME, Meeks M, Morrison I, et al. Primary ciliary dyskinesia: age at diagnosis and symptom history. Acta Paediatr 2002; 91:667-669. (Pubitemid 34779328)
9. Hadfield PJ, Rowe-Jones JM, Bush A, Mackay IS. Treatment of otitis media with effusion in children with primary ciliary dyskinesia. Clin Otolaryngol Allied Sci 1997; 22:302-306. (Pubitemid 28346966)
10. Brueckner M. Cilia propel the embryo in the right direction. Am J Med Genet 2001; 101:339-344. (Pubitemid 32591425)
11. Raman R, Al-Ali SY, Poole CA, et al. Isomerism of the right atrial appendages: clinical, anatomical and microscopic study of a long surviving case with asplenia and ciliary abnormalities. Clin Anat 2003; 16:269-276. (Pubitemid 36444153)
12. Bush A, Chodhari R, Collins N, et al. Primary ciliary dyskinesia: current state of the art. Arch Dis Child 2007; 92:1136-1140. (Pubitemid 350221463)
13. Badano JL, Mitsuma N, Beales PL, et al. The ciliopathies: an emerging class of human genetic disorders. Annu Rev Genomics Hum Genet 2006; 7: 125-148. (Pubitemid 44627925)
14. Bisgrove BW, Yost HJ. The roles of cilia in developmental disorders and disease. Development 2006; 133:4131-4143. (Pubitemid 44830657)
15. Gemou Engesaeth V, Warner JO, Bush A. New associations of primary ciliary dyskinesia syndrome. Pediatr Pulmonol 1993; 16:9-12.
16. Budny B, Chen W, Omran H, et al. A novel X-linked recessive mental retardation syndrome comprising macrocephaly and ciliary dysfunction is allelic to oral-facial-digital type 1 syndrome. Hum Genet 2006; 120: 171-178. (Pubitemid 44204005)
17. Pruliere-Escabasse V, Coste A, Chauvin P, et al. Otologic features in children with primary ciliary dyskinesia. Arch Otolaryngol Head Neck Surg 2010; 136:1121-1126. This study is the first to correlate age and type of ciliary defect in PCD with the characteristics of the otologic features of the disease.
18. Sommer JU, Schafer K, Omran H, et al. ENT manifestations in patients with primary ciliary dyskinesia: prevalence and significance of otorhinolaryngologic co-morbidities. Eur Arch Otorhinolaryngol 2011; 268:383-388. This study highlights the delayed diagnosis in children with PCD and the prevalence of upper respiratory tract symptoms and encourages an increased awareness of PCD among ENT specialists.
19. Barbato A, Frischer T, Kuehni CE, et al. Primary ciliary dyskinesia: a consensus statement on diagnostic and treatment approaches in children. Eur Respir J 2009; 34:1264-1276.
20. O'Callaghan C, Chilvers M, Hogg C, et al. Diagnosing primary ciliary dyskinesia. Thorax 2007; 62:656-657. (Pubitemid 47235001)
21. Ellerman A, Bisgaard H. Longitudinal study of lung function in a cohort of primary ciliary dyskinesia. Eur Respir J 1997; 10:2376-2379. (Pubitemid 27479564)
22. Tamalet A, Clement A, Roudot-Thoraval F, et al. Abnormal central complex is a marker of severity in the presence of partial ciliary defect. Pediatrics 2001; 108:E86.
23. El-Sayed Y, Al-Sarhani A, Al-Essa AR. Otological manifestations of primary ciliary dyskinesia. Clin Otolaryngol Allied Sci 1997; 22:266-270. (Pubitemid 28344981)
24. van der Baan S. Primary ciliary dyskinesia and the middle ear. Laryngoscope 1991; 101:751-754.
25. Mygind N, Pedersen M. Nose-, sinus- and ear-symptoms in 27 patients with primary ciliary dyskinesia. Eur J Respir Dis Suppl 1983; 127:96-101. (Pubitemid 13036870)
26. Majithia A, Fong J, Hariri M, Harcourt J. Hearing outcomes in children with primary ciliary dyskinesia: a longitudinal study. Int J Pediatr Otorhinolaryngol 2005; 69:1061-1064. (Pubitemid 40956982)
27. Ernstson S, Afzelius BA, Mossberg B. Otologic manifestations of the immotile cilia syndrome. Acta Otolaryngol (Stockh) 1984; 97:83-92. (Pubitemid 14215099)
28. Turner JA, Corkey C, Lee JVC, et al. Clinical expressions of immotile cilia syndrome. Pediatrics 1981; 67:805-810. (Pubitemid 11005260)
29. Lous J, Burton LJ, Felding JU, et al. Grommets (ventilation tubes) for hearing loss associated with otitis media with effusion in children. Cochrane Database Syst Rev 2005:CD001801.
30. Rovers MM, Black N, Browning GG, et al. Grommets in otitis media with effusion: an individual patient data meta-analysis. Arch Dis Childhood 2005; 90:480-485. (Pubitemid 40627443)
31. MRC Multicentre Otitis Media Study Group. Speech reception in noise: an indicator of benefit from otitis media with effusion surgery. Clin Otolaryngol 2004; 29:497-504.
32. MRC Multicentre Otitis Media Study Group. The role of ventilation tube status in the hearing levels in children managed for bilateral persistent otitis media with effusion. Clin Otolarygol 2003; 28:146-153.
33. Diacova S, McDonald TJ. A comparison of outcomes following tympanostomy tube placement or conservative measures for management of otitis media with effusion. Ear Nose Throat J 2007; 86:552-554. (Pubitemid 351268046)
34. Welch D, Dawes P. No negative outcomes of childhood middle ear disease in adulthood. Laryngoscope 2007; 117:466-469. (Pubitemid 46407755)
35. Simpson SA, Thomas CL, van der Linden MK, et al. Identification of children in the first four years of life for early treatment for otitis media with effusion. Cochrane Database Syst Rev 2007; (1):CD004163.
36. Bennett KE, Haggard MP, Silva PA, Stewart IA. Behaviour and developmental effects of otitis media with effusion into the teens. Arch Dis Child 2001; 85:91-95. (Pubitemid 32701673)
37. Gravel JS, Wallace IF. Language, speech and educational outcomes of otitis media. J Otolaryngol 1998; 27 (Suppl 2):17-25. (Pubitemid 28517039)
38. Minter KR, Roberts JE, Hooper SR, et al. Early childhood otitis media in relation to children's attention-related behaviour in the first six years of life. Pediatrics 2001; 107:1037-1042. (Pubitemid 32451781)
39. Rosenfeld RM, Bhaya MH, Bower CM, et al. Impact of tympanostomy tubes on child quality of life. Arch Otolaryngol Head Neck Surg 2000; 126: 585-592. (Pubitemid 30252930)
40. Sade J. Ciliary activity and middle ear clearance. Arch Otolaryngol 1967; 86:128-135.
41. Greenstone M, Rutman A, Dewar A, et al. Primary ciliary dyskinesia: cytological and clinical features. Q J Med 1988; 67:405-430.
42. Vaile L, Williamson T, Waddell A, Taylor G. Interventions for ear discharge associated with grommets (ventilation tubes). Cochrane Database Syst Rev 2006; (2):CD001933.
43. Debruyne F, Degroote M. One-year follow-up after tympanostomy tube insertion for recurrent acute otitis media. ORL J Otorhinolaryngol Relat Spec 1993; 55:226-229. (Pubitemid 23207186)
44. Bush A, Cole P, Hariri M, et al. Primary ciliary dyskinesia: diagnosis and standards of care. Eur Respir J 1998; 12:982-988. (Pubitemid 28491678)
45. Samuelson A, Freijd A, Rynnel-Dagoo B. Treatment failure in otitis-prone children with prophylactic tympanostomy tubes is correlated with nasopharyngeal Haemophilus influenzae colonization. Acta Otolaryngol 1991; 111:1090-1096.
46. Ingels K, Rovers MM, van der Wilt GJ, Zielhuis GA. Ventilation tubes in infants increase the risk of otorrhoea and antibiotic usage. B-ENT 2005; 1: 173-176. (Pubitemid 43779970)
47. Nawasreh OO, Al-Wedyan IA. Prophylactic ciprofloxacin drops after tympanostomy tube insertion. Saudi Med J 2004; 25:38-40. (Pubitemid 38262506)
48. Corkey CW, Levison H, Turner JA. The immotile cilia syndrome. A longitudinal survey. Am Rev Respir Dis 1981; 124:544-548. (Pubitemid 12236675)
49. Hellinckx J, Demedts M, De Boeck K. Primary ciliary dyskinesia: evolution of pulmonary function. Eur J Pediatr 1998; 157:422-426. (Pubitemid 28225448)
50. Campbell RG, Birman CS, Morgan L. Management of otitis media with effusion in children with primary ciliary dyskinesia: a literature review. Int J Pediatr Otorhinolaryngol 2009; 73:1630-1638.
51. Macfayden CA, Acuin JM, Gamble C. Systemic antibiotics versus topical treatments for chronically discharging ears with underlying eardrum perforations. Cochrane Database Syst Rev 2006:CD005608. doi: 10.1002/14651858.CD005608.
52. Bouchet V, Hood DW, Li J, et al. Host-derived sialic acid is incorporated into Haemophilus influenzae lipopolysaccharide and is a major virulence factor in experimental otitis media. Proc Natl Acad Sci U S A 2003; 100:8898-8903. (Pubitemid 36899195)
53. Malaty J, Antonelli PJ. Effect of blood and mucus on tympanostomy tube biofilm formation. Laryngoscope 2008; 118:867-870. (Pubitemid 351631300)
54. Kocaturk S, Yardimci S, Yildirim A, Incesulu A. Preventive therapy for postoperative purulent otorrhea after ventilation tube insertion. Am J Otolaryngol 2005; 26:123-127. (Pubitemid 40312074)
55. Krespi YP, Stoodley P, Hall-Stoodley L. Laser disruption of biofilm. Laryngoscope 2008; 118:1168-1173.
56. Ma ZX, Chen XH, Li M, et al. Histo-morphological changes of Eustachian tube of guinea pigs with effusion after being treated by pulmonary surfactant. Chung Hua Erh Pi Yen Hou Ko Tsa Chih 2003; 38:445-447.
57. Williamson IG, Dunleavey J, Bain J, Robinson D. The natural history of otitis media with effusion: a three-year study of the incidence and prevalence of abnormal tympanograms in four South West Hampshire infant schools. J Laryngol Otol 1994; 108:930-934. (Pubitemid 24350768)
58. Woodbury K, Ferguson BJ. Recalcitrant chronic rhinosinusitis: investigation and management. Curr Opin Otolaryngol Head Neck Surg 2011; 19:1-5. This article evaluates alternative diagnoses for recalcitrant CRS and provides an update on treatment options for difficult CRS.
59. Leigh MW. Primary ciliary dyskinesia. Semin Respir Crit Care Med 2003; 24:653-662. (Pubitemid 38036760)
60. Min YG, Shin JS, Choi SH, et al. Primary ciliary dyskinesia: ultrastructural defects and clinical features. Rhinology 1995; 33:189-193.
61. Pifferi M, Bush A, Caramella D, et al. Agenesis of paranasal sinuses and nasal nitric oxide in primary ciliary dyskinesia. Eur Respir J 2011; 37:566-571. This study reports on an inverse correlation between frontal and sphenoidal sinus hypoplasia/aplasia and nasal nitric oxide levels in PCD and suggests the finding of aplasia/hypoplasia of these sinuses should prompt exclusion of PCD.
62. Sato I, Sunohara M, Mikami A, et al. Immunocytochemical study of the maxilla and maxillary sinus during human fetal development. Okajimas Folia Anat Jpn 1998; 75:205-216. (Pubitemid 128529132)
63. Ellington JK, Elhofy A, Bost KL, et al. Involvement of mitogen-activated protein kinase pathways in Staphylococcus aureus invasion of normal osteoblasts. Infect Immun 2001; 69:5235-5242. (Pubitemid 32786453)
64. Berlucchi M, Maroldi R, Aga A, et al. Ethmoid mucocele: a new feature of primary ciliary dyskinesia. Pediatr Pulmonol 2010; 45:197-201.
65. Greenstone MA, Jones RW, Dewar A, et al. Hydrocephalus and primary ciliary dyskinesia. Arch Dis Child 1984; 59:481.
66. Picco P, Leveratto L, Cama A, et al. Immotile cilia syndrome associated with hydrocephalus and precocious puberty: a case report. Eur J Pediatr Surg 1993; 3 (Suppl 1):20.
67. Parsons DS, Greene BA. A treatment for primary ciliary dyskinesia: efficacy of functional endoscopic sinus surgery. Laryngoscope 1993; 103: 1269-1272. (Pubitemid 23335858)
68. Hassan JA, Saadiah S, Roslan H, Zainudin B. Bronchodilator response to inhaled beta-2 agonist and anticholinergic drugs in patients with bronchiectasis. Respirology 1999; 4:423-426. (Pubitemid 30016295)
69. Garg A, Wadhera R, Gulati SP, et al. Primary ciliary dyskinesia: an underdiagnosed entity. J Assoc Phys India 2010; 58:704-706.
70. Schidlow DV. Primary ciliary dyskinesia (the immotile cilia syndrome). Ann Allergy 1994; 73:457-469.
71. Bush A, Payne D, Pike S, et al. Mucus properties in children with primary ciliary dyskinesia. Comparison with cystic fibrosis. Chest 2006; 129: 118-123. (Pubitemid 43265251)
72. DeMarcantonio MA, Han J. Nasal polyps: pathogenesis and treatment implications. Otolaryngol Clin North Am 2011; 44:685-695. An excellent review and update on the pathogenesis and treatment of nasal polyposis.
73. Robertson JM, Friedman EM, Rubin BK. Nasal and sinus disease in cystic fibrosis. Paediatr Respir Rev 2008; 9:213-219.
74. Claeys S, Van Hoecke H, Holtappels G, et al. Nasal polyps in patients with and without cystic fibrosis: a differentiation by innate markers and inflammatory mediators. Clin Exp Allergy 2005; 35:467-472. (Pubitemid 40577239)
75. Barker AF, Couch I, Fiel SB, et al. Tobramycin solution for inhalation reduces sputum Pseudomonas aeruginosa density in bronchiectasis. Am J Respir Crit Care Med 2000; 162:481-485. (Pubitemid 30647187)
76. Couch IA. Treatment with tobramycin solution for inhalation in bronchiectasis patients with Pseudomonas aeruginosa. Chest 2001; 120:114-117.
77. Bilton D. Update on noncystic fibrosis bronchiectasis. Curr Opin Pulm Med 2008; 14:595-599.
78. Hand WL, Hand DL, King-Thompson NL. Antibiotic inhibition of the respiratory burst response in human polymorphonuclear leukocytes. Antimicrob Agents Chemother 1990; 34:863-870. (Pubitemid 20142414)
79. Braga PC, Maci S, DalSasso M, et al. Effects of rokitamycin on phagocytosis and release of oxidant radicals of human polymorphonuclear leukocytes. Chemotherapy 1997; 43:190-197. (Pubitemid 27168357)
80. Inamura K, Ohta N, Fukase S, et al. The effects of erythromycin on human peripheral neutrophil apoptosis. Rhinology 2000; 38:124-129.
81. Aoshiba K, Nagai A, Konno K. Erythromycin shortens neutrophil survival by accelerating apoptosis. Antimicrob Agents Chemother 1995; 39:872-877.
82. MacLeod CM, Hamid QA, Cameron L, et al. Anti-inflammatory activity of clarithromycin in adults with chronically inflamed sinus mucosa. Adv Ther 2001; 18:75-82. (Pubitemid 32601204)
83. Oishi K, Sonoda F, Kobayashi S, et al. Role of interleukin-8 (IL-8) and an inhibitory effect of erythromycin on IL-8 release in the airways of patients with chronic airway diseases. Infect Immun 1994; 62:4145-4152. (Pubitemid 24301936)
84. Suzuki H, Shimomura A, Ikeda K, et al. Inhibitory effect of macrolides on interleukin- 8 secretion from cultured human nasal epithelial cells. Laryngoscope 1997; 107 (12 Pt 1):1661-1666. (Pubitemid 27520179)
85. Hirakata Y, Kaku M, Mizukane R, et al. Potential effects of erythromycin on host defense systems and virulence of Pseudomonas aeruginosa. Antimicrob Agents Chemother 1992; 36:1922-1927.
86. Wozniak DJ, Keyser R. Effects of subinhibitory concentrations of macrolide antibiotics on Pseudomonas aeruginosa. Chest 2004; 125 (2 Suppl): 62S-69S. (Pubitemid 38200962)
87. Nakano T, Ohashi Y, Tanaka A, et al. Roxythromycin reinforces epithelial defence function in rabbit trachea. Acta Otolaryngol Suppl 1998; 538: 233-238. (Pubitemid 28563349)
88. Hamid Q, Cameron L, MacLeod C, et al. Anti-inflammatory activity of clarithromycin in the treatment of adults with chronically-inflamed sinus mucosa. In: Abstracts of the European Congress of Chemotherapy; 2000; Madrid.
89. Nishi K, Mizuguchi M, Tachibana H, et al. Effect of clarithromycin on symptoms and mucociliary transport in patients with sino-bronchial syndrome. Nippon Kyobu Shikkan Gakkai Zasshi 1995; 33:1392-1400. (Pubitemid 26051126)
90. Tamaoki J, Takeyama K, Yamawaki I, et al. Lipopolysaccharide-induced gobletcell hyper-secretion in the guinea pig trachea: inhibition by macrolides. Am J Physiol 1997; 272 (1 Pt 1):L15-L19.
91. Rhee CS, Majima Y, Arima S, et al. Effects of clarithromycin on rheological properties of nasal mucus in patients with chronic sinusitis. Ann Otol Rhinol Laryngol 2000; 109:484-487. (Pubitemid 30318261)
92. Yoshioka D, Sakamoto N, Ishimatsu Y, et al. Primary ciliary dyskinesia that responded to long-term, low-dose clarithromycin. Intern Med 2010; 49:1437-1440. This study is the only study to report on the response of sinonasal symptoms in PCD to macrolide therapy.
93. Ishiguro T, Takayanagi N, Hijikata N, et al. Primary ciliary dyskinesia. A case report and comparison with 4 previous cases. Nihon Kokyuki Gakkai Zasshi 2009; 47:242-248.
94. Itoh M, Kishi K, Nakamura H, et al. A case of immotile dyskinetic cilia syndrome responding to clenbuterol hydrochloride and azithromycin. Nihon Kokyuki Gakkai Zasshi 2002; 40:617-621.
95. Homma S, Kawabat M, Kishi K, et al. Bronchiolitis in Kartagener's syndrome. Eur Resp J 1999; 14:1332-1339. (Pubitemid 30019529)
96. Noone PG, Leigh MW, Sannuti A, et al. Primary ciliary dyskinesia: diagnostic and phenotypic features. Am J Respir Crit Care Med 2004; 169:459-467. (Pubitemid 38187117)
97. Karadag B, James AJ, Gultekin E, et al. Nasal and lower airway level of nitric oxide in children with primary ciliary dyskinesia. Eur Respir J 1999; 13:1402-1405. (Pubitemid 29357160)
98. Lundberg JO, Weitzberg E, Nordvall SL, et al. Primarily nasal origin of exhaled nitric oxide and absence in Kartagener's syndrome. Eur Respir J 1994; 7:1501-1504. (Pubitemid 24268339)
99. Grasemann H, Gartig SS, Wiesemann HG, et al. Effect of L-arginine infusion on airway NO in cystic fibrosis and primary ciliary dyskinesia syndrome. Eur Respir J 1999; 13:114-118. (Pubitemid 29079230)
100. Lindberg S, Cervin A, Runer T. Low levels of nasal nitric oxide(NO) correlate to impaired mucociliary function in the upper airways. Acta Otolaryngol (Stockh) 1997; 117:728-734. (Pubitemid 27418204)
101. Rossaint R, Falke KJ, Lopez F, et al. Inhaled nitric oxide for the adult respiratory distress syndrome. N Engl J Med 1993; 328:399-405. (Pubitemid 23042288)
102. Lundberg JO, Settergren G, Gelinder S, et al. Inhalation of nasally derived nitric oxide modulates pulmonary function in humans. Acta Physiol Scand 1996; 158:343-347. (Pubitemid 26426964)
103. Mancinelli RL, McKay CP. Effects of nitric oxide and nitrogen dioxide on bacterial growth. Appl Environ Microbiol 1983; 46:198-202. (Pubitemid 13045860)
104. Loukides S, Kharitonov S, Wodehouse T, et al. Effect of arginine on mucociliary function in primary ciliary dyskinesia. Lancet 1998; 352:371-372. (Pubitemid 28352208)
105. Lusby PE, Coombes AL, Wilkinson JM. Bactericidal activity of different honeys against pathogenic bacteria. Arch Med Res 2005; 36:464-467. (Pubitemid 41140049)
106. Alandejani T, Marsan J, Ferris W, et al. Effectiveness of honey on Staphylococcus aureus and Pseudomonas aeruginosa biofilms. Otolaryngol Head Neck Surg 2009; 141:114-118.
107. Potter JL, Spector S, Matthew JW, Lemm J. Studies on pulmonary secretions. 3: The nucleic acids in whole pulmonary secretions from patients with cytsic fibrosis, bronchiectasis, and laryngectomy. Am Rev Respir Dis 1969; 99:909-916.
108. Mainz JG, Schiller I, Ritschel C, et al. Sinonasal inhalation of dornase alfa in CF: a double-blind placebo-controlled cross-over pilot trial. Auris Nasus Larynx 2011; 38:220-227. This is the first study to evaluate the use of dornas alfa for the treatment of the sinonasal symptoms in CF patients.
109. Ten Berge M, Brinkhorst G, Kroon AA, de Jongste JC. Dnase treatment in primary ciliary dyskinesia: assessment by nocturnal pulse oximetry. Pediatr Pulmonol 1999; 27:59-61. (Pubitemid 29063803)
110. Desai M, Weller PH, Spencer DA. Clinical benefit from nebulised human recombinant Dnase in Kartagener's syndrome. Pediatr Pulmonol 1995; 20:307-308.
111. Phipps RJ, Nadel JA, Davis B. Effect of alpha-adrenergic stimulation on mucus secretion and on ion transport in cat trachea in vitro. Am Rev Respir Dis 1920; 121:359-365.
112. Phipps RJ, Williams IP, Richardson PS, et al. Sympathomimetic drugs stimulate the output of secretory glycoproteins from human bronchi in vitro. Clin Sci (Lond) 1982; 63:23-28. (Pubitemid 13227732)
113. Bennett WD. Effect of B-adrenergic agonists on mucociliary clearance. J Allergy Clin Immunol 2002; 110 (6 Suppl):S291-S297. (Pubitemid 35471718)
114. King M, Dasgupta B, Tomkiewicz RP, Brown NE. Rheology of cystic fibrosis sputum after in vitro treatment with hypertonic saline alone and in combination with recombinant human deoxyribonuclease I. Am J Respir Crit Care Med 1997; 156:173-177. (Pubitemid 27293184)
115. Robinson M, Regnis JA, Bailey DL, et al. Effect of hypertonic saline, amiloride and cough on mucociliary clearance in patients with cystic fibrosis. Am J Respir Crit Care Med 1996; 153:1503-1509. (Pubitemid 26141332)
116. Daviskas E, Anderson SD, Eberl S, et al. Inhalation of dry-powder mannitol increases mucociliary clearance in patients with bronchiectasis. Am J Respir Crit Care Med 1999; 159:1843-1848. (Pubitemid 29275053)
117. Knowles MR, Clarke LL, Boucher RC. Activation by extracellular nucleotides of chloride secretion in the airway epithelia of patients with cystic fibrosis. N Engl J Med 1991; 325:533-538.
118. Noone PG, Bennett WD, Regnis JA, et al. Effect of aerosolised uridine-50-triphosphate on airway clearance with cough in patients with primary ciliary dyskinesia. Am J Respir Crit Care Med 1999; 160:144-149. (Pubitemid 29326080)
119. Pedersen H, Rebbe H. Absence of arms in the axoneme of immobile human spermatozoa. Biol Reprod 1975; 12:541-544.
120. American Academy of Pediatrics. Subcommittee on Management of Sinusitis and Committee on Quality Improvement Clinical Practice Guideline: management of sinusitis. Pediatrics 2001; 108:798-808.
121. Jarrett WA, Militsakh O, Anstad M, Manaligod J. Endoscopic sinus surgery in cystic fibrosis: effects on pulmonary function and ideal body weight. Ear Nose Throat J 2004; 83:118-121. (Pubitemid 38667551)
122. Osborn AJ, Leung R, Ratjen F, James AL. Effect of endoscopic sinus surgery on pulmonary function and microbial pathogens in a pediatric population with cystic fibrosis. Arch Otolaryngol Head Neck Surg 2011; 137:542-547.
123. Rosbe KW, Jones DT, Rahbar R, et al. Endoscopic sinus surgery in cystic fibrosis: do patients benefit from surgery? Int J Pediatr Otorhinolaryngol 2001; 61:113-119. (Pubitemid 32895687)
124. Chiu A, Palmer JN, Woodworth BA, et al. Baby shampoo nasal irrigations for the symptomatic postfunctional endoscopic sinus surgery patient. Am J Rhinol 2008; 22:34-37. (Pubitemid 351310950)
125. Amirav I, Cohen-Cymberknoh M, Shoseyov D, Kerem E. Primary ciliary dyskinesia: prospects for new therapies, building on the experience in cystic fibrosis. Paediatr Respir Rev 2009; 10:58-62.