Skip to main content

Advertisement

SpringerLink
Log in

Dental treatment for people with cystic fibrosis

  • Original Scientific Article
  • Published:
European Archives of Paediatric Dentistry Aims and scope Submit manuscript

Abstract

Aim

To describe the nature and consequences of the multi-system genetic condition cystic fibrosis with a view to ensuring optimal dental treatment planning for these patients.

Methods

A literature search was conducted to identify the key medical and dental manifestations of cystic fibrosis. These findings are discussed and utilised to create recommendations for treatment planning in patients with cystic fibrosis for the practising dental practitioner.

Results

Cystic fibrosis is a complex, lethal, multisystem autosomal recessive disorder resulting from mutations on chromosome 7 which result in dysfunction of an ion channel that sits on epithelial surfaces. Respiratory disease remains the leading cause of mortality. Survival has greatly increased in recent decades secondary to improved treatment and specialist care. Specific dental manifestations of the disease may result from the condition itself or complications of treatment. Modification of patient management may be necessary to provide optimum patient care.

Conclusion

The pathophysiology and clinical manifestations are relevant to practicing dental practitioners and inform recommendations to be utilised to ensure optimal treatment planning for these patients.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2

Similar content being viewed by others

References

  • Alton EW, Armstrong DK, Ashby D, et al. Repeated nebulisation of non-viral CFTR gene therapy in patients with cystic fibrosis: a randomised, double-blind, placebo-controlled, phase 2b trial. Lancet Respir Med. 2015;3:684–91.

    Article  PubMed  PubMed Central  Google Scholar 

  • Andersen DH. Cystic fibrosis of the pancreas and its relation to celiac disease: a clinical and pathologic study. Am J Dis Child. 1938;56:344.

    Article  Google Scholar 

  • Aps JK, Van Maele GO, Claeys G, Martens LC. Mutans streptococci, lactobacilli and caries experience in cystic fibrosis homozygotes, heterozygotes and healthy controls. Caries Res. 2001;35:407–11.

    Article  PubMed  Google Scholar 

  • Azevedo TDPL, Feijó GCS, Bezerra ACB. Presence of developmental defects of enamel in cystic fibrosis patients. J Dent Child. 2006;73:159–63.

    Google Scholar 

  • Balfour-Lynn IM, Welch K. Inhaled corticosteroids for cystic fibrosis. Cochrane Database Syst Rev. 2014. doi:10.1002/14651858.CD001915.pub4.

  • Barry PJ, Ronan N, Plant BJ. Cystic fibrosis transmembrane conductance regulator modulators: the end of the beginning. Semin Respir Crit Care Med. 2015;36:287–98.

    Article  PubMed  Google Scholar 

  • Barry S, Fleming P, O’Connor M. Prescribing sugar-containing medicines for children—are we forgetting “primum non nocere?” Ir Med J. 2009;102:298–300.

    PubMed  Google Scholar 

  • Bear CE, Li CH, Kartner N, et al. Purification and functional reconstitution of the cystic fibrosis transmembrane conductance regulator (CFTR). Cell. 1992;68:809–18.

    Article  PubMed  Google Scholar 

  • Blacharsh C. Dental aspects of patients with cystic fibrosis: a preliminary clinical study. J Am Dent Assoc. 1977;95:106–10.

    Article  PubMed  Google Scholar 

  • Borowitz D, Baker RD, Stallings V. Consensus report on nutrition for pediatric patients with cystic fibrosis. J Pediatr Gastroenterol Nutr. 2002;35:246–59.

    Article  PubMed  Google Scholar 

  • Brennan AL, Beynon J. Clinical updates in cystic fibrosis-related diabetes. Semin Respir Crit Care Med. 2015;36:236–50.

    Article  PubMed  Google Scholar 

  • Brihaye P, Jorissen M, Clement PA. Chronic rhinosinusitis in cystic fibrosis (mucoviscidosis). Acta Otorhinolaryngol. 1997;51:323–37.

    Google Scholar 

  • Chi DL. Dental caries prevalence in children and adolescents with cystic fibrosis: a qualitative systematic review and recommendations for future research. Int J Paediatr Dent. 2013;23(5):376–86.

    Article  PubMed  PubMed Central  Google Scholar 

  • Colombo C. Liver disease in cystic fibrosis. Curr Opin Pulm Med. 2007;13:529–36.

    Article  PubMed  Google Scholar 

  • Conwell LS, Chang AB. Bisphosphonates for osteoporosis in people with cystic fibrosis. Cochrane Database Syst Rev. 2014. doi:10.1002/14651858.CD002010.pub3.

  • Dabrowska E, Błahuszewska K, Minarowska A, et al. Assessment of dental status and oral hygiene in the study population of cystic fibrosis patients in the Podlasie province. Adv Med Sci. 2006;51:100–3.

    PubMed  Google Scholar 

  • Davies JC, Alton EW, Bush A. Cystic fibrosis. Brit Med J. 2007;335:1255–9.

    Article  PubMed  PubMed Central  Google Scholar 

  • Di Sant’Agnese PA, Davis PB. Research in cystic fibrosis (first of three parts). N Engl J Med. 1976;295:481–5.

    Article  PubMed  Google Scholar 

  • de Oliveira AC, Maluta RP, Stella AE, et al. Isolation of Pseudomonas aeruginosa strains from dental office environments and units in Barretos, state of São Paulo, Brazil, and analysis of their susceptibility to antimicrobial drugs. Braz J Microbiol. 2008;39:579–84.

    Article  PubMed  PubMed Central  Google Scholar 

  • Döring G, Flume P, Heijerman H, Elborn JS, Consensus Study Group. Treatment of lung infection in patients with cystic fibrosis: current and future strategies. J Cyst Fibros. 2012;11:461–79.

    Article  PubMed  Google Scholar 

  • Drury D, Grey VL, Ferland G, Gundberg C, Lands LC. Efficacy of high dose phylloquinone in correcting vitamin K deficiency in cystic fibrosis. J Cyst Fibros. 2008;7:457–9.

    Article  PubMed  Google Scholar 

  • Farzal Z, Kou Y-F, St John R, Shah GB, Mitchell RB. The role of routine hearing screening in children with cystic fibrosis on aminoglycosides: a systematic review. Laryngoscope. 2016;126(1):228–35.

    Article  PubMed  Google Scholar 

  • Fernald GW, Roberts MW, Boat TF. Cystic fibrosis: a current review. Pediatr Dent. 1990;12:72–8.

    PubMed  Google Scholar 

  • Ferrazzano GF, Sangianantoni G, Cantile T, et al. Dental enamel defects in Italian children with cystic fibrosis: an observational study. Community Dent Health. 2012;29:106–9.

    PubMed  Google Scholar 

  • Fox PC. Differentiation of dry mouth etiology. Adv Dent Res. 1996;10:13–6.

    Article  PubMed  Google Scholar 

  • Gibson-Corley KN, Meyerholz DK, Engelhardt JF. Pancreatic pathophysiology in cystic fibrosis. J Pathol. 2016;238:311–20.

    Article  PubMed  Google Scholar 

  • Gysin C, Alothman GA, Papsin BC. Sinonasal disease in cystic fibrosis: clinical characteristics, diagnosis, and management. Pediatr Pulmonol. 2000;30:481–9.

    Article  PubMed  Google Scholar 

  • Huffmyer JL, Littlewood KE, Nemergut EC. Perioperative management of the adult with cystic fibrosis. Anesth Analg. 2009;109:1949–61.

    Article  PubMed  Google Scholar 

  • Jagels AE, Sweeney EA. Oral health of patients with cystic fibrosis and their siblings. J Dent Res. 1976;55:991–6.

    Article  PubMed  Google Scholar 

  • Jensen ET, Giwercman B, Ojeniyi B, et al. Epidemiology of Pseudomonas aeruginosa in cystic fibrosis and the possible role of contamination by dental equipment. J Hosp Infect. 1997;36:117–22.

    Article  PubMed  Google Scholar 

  • Jones AM, Govan JR, Doherty CJ, et al. Spread of a multiresistant strain of Pseudomonas aeruginosa in an adult cystic fibrosis clinic. Lancet. 2001;358:557–8.

    Article  PubMed  Google Scholar 

  • Kessler WR, Andersen DH. Heat prostration in fibrocystic disease of the pancreas and other conditions. Pediatrics. 1951;8:648–56.

    PubMed  Google Scholar 

  • Khasawneh FA, Moti DF, Zorek JA. Linezolid-induced black hairy tongue: a case report. J Med Case Rep. 2013;7:46.

    Article  PubMed  PubMed Central  Google Scholar 

  • Kinirons MJ. Increased salivary buffering in association with a low caries experience in children suffering from cystic fibrosis. J Dent Res. 1983;62:815–7.

    Article  PubMed  Google Scholar 

  • Kinirons MJ. Dental health of patients suffering from cystic fibrosis in Northern Ireland. Community Dent Health. 1989;6:113–20.

    PubMed  Google Scholar 

  • Kinirons MJ. The effect of antibiotic therapy on the oral health of cystic fibrosis children. Int J Paediatr Dent. 1992;2(3):139–43.

    Article  PubMed  Google Scholar 

  • Lewis PA, Morison S, Dodge JA, et al. Survival estimates for adults with cystic fibrosis born in the United Kingdom between 1947 and 1967. The UK Cystic Fibrosis Survey Management Committee. Thorax. 1999;54:420–2.

    Article  PubMed  PubMed Central  Google Scholar 

  • LiPuma JJ, Dasen SE, Nielson DW, Stern RC, Stull TL. Person-to-person transmission of Pseudomonas cepacia between patients with cystic fibrosis. Lancet. 1990;336:1094–6.

    Article  PubMed  Google Scholar 

  • MacDonald A. Nutritional management of cystic fibrosis. Arch Dis Child. 1996;74:81–7.

    Article  PubMed  PubMed Central  Google Scholar 

  • Mainz JG, Gerber A, Lorenz M, et al. Pseudomonas aeruginosa acquisition in cystic fibrosis patients in context of otorhinolaryngological surgery or dentist attendance: case series and discussion of preventive concepts. Case Rep Infect Dis. 2015. doi:10.1155/2015/438517.

  • Mall MA, Galietta LJV. Targeting ion channels in cystic fibrosis. J Cyst Fibros. 2015;14:561–70.

    Article  PubMed  Google Scholar 

  • Mandel ID, Kutscher A, Denning CR, Thompson RH, Zegarelli EV. Salivary studies in cystic fibrosis. Am J Dis Child. 1967;113:431–8.

    PubMed  Google Scholar 

  • Mangos JA, Benke PJ, McSherry NR. Salivary gland enlargement and functional changes during feeding of pancreatin to rats (possible relationship to pathophysiology of cystic fibrosis). J Pediatr. 1969;74:823.

    Article  PubMed  Google Scholar 

  • Mattsson A. Long-term physical illness in childhood: a challenge to psychosocial adaptation. Pediatrics. 1972;50:801–11.

    PubMed  Google Scholar 

  • McCallum TJ, Milunsky JM, Cunningham DL, et al. Fertility in men with cystic fibrosis: an update on current surgical practices and outcomes. Chest. 2000;118:1059–62.

    Article  PubMed  Google Scholar 

  • Mogayzel PJ, Naureckas ET, Robinson KA, et al. Cystic fibrosis pulmonary guidelines. Chronic medications for maintenance of lung health. Am J Respir Crit Care Med. 2013;187:680–9.

    Article  PubMed  Google Scholar 

  • Molloy L, Nichols K. Infectious diseases pharmacotherapy for children with cystic fibrosis. J Pediatr Health Care. 2015;29:565–78.

    Article  PubMed  Google Scholar 

  • Moursi AM, Fernandez JB, Daronch M, Zee L, Jones CL. Nutrition and oral health considerations in children with special health care needs: implications for oral health care providers. Pediatr Dent. 2010;32:333–42.

    PubMed  Google Scholar 

  • Narang A, Maguire A, Nunn JH, Bush A. Oral health and related factors in cystic fibrosis and other chronic respiratory disorders. Arch Dis Child. 2003;88:702–7.

    Article  PubMed  PubMed Central  Google Scholar 

  • O’Sullivan BP, Freedman SD. Cystic fibrosis. Lancet. 2009;373:1891–904.

    Article  PubMed  Google Scholar 

  • Plant BJ, Goss CH, Plant WD, Bell SC. Management of comorbidities in older patients with cystic fibrosis. Lancet Respir Med. 2013;1:164–74.

    Article  PubMed  Google Scholar 

  • Qvist T, Taylor-Robinson D, Waldmann E, et al. Comparing the harmful effects of nontuberculous mycobacteria and Gram negative bacteria on lung function in patients with cystic fibrosis. J Cyst Fibros. 2015. doi:10.1016/j.jcf.2015.09.007.

    PubMed Central  Google Scholar 

  • Ramsey BW, Davies J, McElvaney NG, et al. A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. N Engl J Med. 2011;365:1663–72.

    Article  PubMed  PubMed Central  Google Scholar 

  • Rashid M, Durie P, Andrew M, et al. Prevalence of vitamin K deficiency in cystic fibrosis. Am J Clin Nutr. 1999;70:378–82.

    PubMed  Google Scholar 

  • Riordan JR, Rommens JM, Kerem B, et al. Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science. 1989;245:1066–73.

    Article  PubMed  Google Scholar 

  • Schuster A, Shwachman H. The tetracyclines; applied pharmacology. Pediatr Clin North Am. 1956;3:295–303.

    Google Scholar 

  • Schwank G, Koo B-K, Sasselli V, et al. Functional repair of CFTR by CRISPR/Cas9 in intestinal stem cell organoids of cystic fibrosis patients. Cell Stem Cell. 2013;13:653–8.

    Article  PubMed  Google Scholar 

  • Scott RB, O’Loughlin EV, Gall DG. Gastroesophageal reflux in patients with cystic fibrosis. J Pediatr. 1985;106:223–7.

    Article  PubMed  Google Scholar 

  • Sosnay PR, Siklosi KR, Van Goor F, et al. Defining the disease liability of variants in the cystic fibrosis transmembrane conductance regulator gene. Nat Genet. 2013;45:1160–7.

    Article  PubMed  PubMed Central  Google Scholar 

  • Stephenson AL, Tom M, Berthiaume Y, et al. A contemporary survival analysis of individuals with cystic fibrosis: a cohort study. Eur Respir J. 2015;45:670–9.

    Article  PubMed  Google Scholar 

  • Sui W, Boyd C, Wright JT. Altered pH regulation during enamel development in the cystic fibrosis mouse incisor. J Dent Res. 2003;82:388–92.

    Article  PubMed  Google Scholar 

  • Sweeney EA, Shaw JH. The effect of dietary pancreatin supplements on dental caries and on the composition of saliva in caries-susceptible rats. J Dent Res. 1965;44:973–6.

    Article  PubMed  Google Scholar 

  • Van Goor F, Hadida S, Grootenhuis PDJ, et al. Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770. Proc Natl Acad Sci. 2009;106:18825–30.

    Article  PubMed  PubMed Central  Google Scholar 

  • Wainwright BJ, Scambler PJ, Schmidtke J, et al. Localization of cystic fibrosis locus to human chromosome 7cen-q22. Nature. 1985;318:384–5.

    Article  PubMed  Google Scholar 

  • Wiesmann UN, Boat TF, Di Sant’Agnese PA. Flow-rates and electrolytes in minor-salivary-gland saliva in normal subjects and patients with cystic fibrosis. Lancet. 1972;2:510–2.

    Article  PubMed  Google Scholar 

  • Wotman S, Mercadante J, Mandel ID, Goldman RS, Denning C. The occurrence of calculus in normal children, children with cystic fibrosis, and children with asthma. J Periodontol. 1973;44:278–80.

    Article  PubMed  Google Scholar 

Download references

Acknowledgments

The authors would like to acknowledge the kind contribution of the clinical image by Dr. Rowland Bright-Thomas, Manchester Adult CF Centre.

Author information

Authors and Affiliations

  1. Department of Paediatric Dentistry, University Dental Hospital Manchester, Higher Cambridge Street, Manchester, M15 6FH, UK

    N. Harrington & S. M. Barry

  2. University of Manchester, Manchester, UK

    S. M. Barry

  3. Manchester Adult Cystic Fibrosis, University Hospital of South Manchester, Wythenshawe, Manchester, UK

    P. J. Barry

  4. Institute of Inflammation and Repair, University of Manchester, Manchester, UK

    P. J. Barry

Authors
  1. N. Harrington
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. P. J. Barry
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. S. M. Barry
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to N. Harrington.

Ethics declarations

Conflict of interest

PB has received honoraria and financial support for educational activities from Vertex pharmaceuticals Ltd.

This article does not contain any studies with human participants or animals performed by any of the authors.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Harrington, N., Barry, P.J. & Barry, S.M. Dental treatment for people with cystic fibrosis. Eur Arch Paediatr Dent 17, 195–203 (2016). https://doi.org/10.1007/s40368-016-0229-9

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s40368-016-0229-9

Keywords

Search

Navigation