Cystic Fibrosis: A Current Review

Department of Pediatric Dentistry

Lutheran Medical Center

 

Resident’s Name:  Chad Abby                                    Date: 5/29/2009

Article title:  Cystic Fibrosis:  A Current Review

Author(s): Gerald Fernald, Michael Roberts, Thomas Boat

Journal:  Pediatric Dentistry

 Volume (number): Vol. 12, No. 2

Month, Year:  1990

Major topic:  Cystic Fibrosis

 Minor topic(s:  Cystic Fibrosis

Type of Article:  Theme article

Main Purpose:  To gain a better understanding of and the oral implications of cystic fibrosis

Findings:  Oral implications associated with Cystic fibrosis include enamel hypoplasia and tooth discoloration, salivary glands involvement, reduced incidence of dental caries, reservoir for potentially pathogenic respiratory bacteria, mouth breathing and anterior open bite associated with nasal and sinus obstruction.  Patients with CF require routine dental care similar to that of the general population.  Unless acutely ill, the CF patient can and should receive regular dental care.  Often children with CF do not manifest symptoms for several years, although now 70% are identified by age two.  Most infants are diagnosed because of persistent loose, bulky, oily stools, failure to thrive, and or recurrent pneumonia.  CF patients are often taking antibiotics for treatment of their lung infection and it is important to know that patients of CF are not immune deficient.  The frequency of sugar-containing food consumption generally is greater in children with CF to maintain elevated caloric and salt intake however the caries incidence is reportedly lower.  It is also observed that there is less dental plaque and gingivitis in CF patients.  The reduced caries incidence may be due to the effects of long term antibiotic and pancreatic enzyme replacement therapy on the oral microbiota.  The elevated calcium content and buffering capacity of the whole saliva may also play a role, which favors an increased prevalence of dental calculus on the teeth.  Dental appointments should be kept short and the patient may prefer to be maintained in an upright sitting position while being treated.  The use of any agent that interferes with pulmonary function, such as narcotic analgesics and sedatives, should be avoided.  Nitrous also is contraindicated in patients exhibiting evidence of emphysema and should be used only after consultation and concurrence by the patient’s physician.  Regular professional care and good home oral hygiene habits are extremely important in CF patient management. 

Key points/Summary :  Cystic fibrosis is the most common of the severe genetic disorders seen in Caucasians.  Defective exocrine gland secretions due to abnormal water and electrolyte transport across epithelial cells, result in chronic disease of the respiratory and gastrointestinal systems.  CF is an autosomal recessive disorder ranging from 1:1700 in incidence.  The classic CF phenotype includes chronic progressive pulmonary disease, pancreatic insufficiency with steatorrhea and failure to thrive, excess sweat electrolyte content, male sterility and decreased female fertility.  Most of the clinical manifestations of CF are produced by abnormal secretions causing sticky or thickened mucous secretions that can lead to respiratory malfunction or obstruction.  Inadequate hydration is partly responsible for problems with clearance of mucous from airways.  Respiratory failure is the most common cause of death.  The diagnostic criteria for CF include a sweat chloride level grater than 60 meq/L, chronic obstructive pulmonary disease, exocrine pancreatic insufficiency, and a familial history of CF.  At least two of these criteria are necessary for diagnosis. 

Assessment of article:   Great overview of cystic fibrosis