Cystic fibrosis (CF) is the most common severe genetic disorder seen in Caucasians. Defective exocrine gland secretions result in chronic diseases of the respiratory and gastrointestinal systems. However, the CF gene recently has been located and cloned. Currently, genetic technology allows identification of sibling carriers and antenatal diagnosis within families. Oral implications associated with CF include enamel hypoplasia and tooth discoloration, salivary gland involvement, reduced incidence of dental caries, reservoir for potentially pathogenic respiratory bacteria, mouth breathing, and anterior open bite associated with nasal and sinus obstruction. Continued efforts to improve early diagnosis and treatment of CF should increase life expectancy. Affected patients are expected to seek regular dental care more frequently as they learn to view the disease as manageable.
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