Cystic Fibroses

Reports from the MHC data base

Report from the Observation Chart (PDF)

Synonyms

  • Pancreatic cystic fibrosis
  • Mucoviscidosis

Codes

ICD-10: E84.8
ORPHA: 586

Estimated occurrence

60:1,000,000 people, i.e. more than 500 people in Sweden. 15-20 diagnosed children are born annually.

Etiology

Damage or mutation in a gene on chromosome 7, which leads to a reduced ability of the cells to bind water. The disease is inherited autosomal recessively.

General symptoms

There is a wide variation in severity and symptoms in people with cystic fibrosis (CF). CF progressively deteriorates over the years, but the consequences of the disease can be delayed. The mucus producing glands in the body do not function normally, resulting in viscous mucus that is difficult to remove. The lungs and gastrointestinal tract are especially affected. This causes coughing, breathing difficulties, proneness to infections in the lungs, as well as difficulties to digest food. The most common sign in young children is that they do not gain weight. The disease is usually discovered during the first year of life. Another sign of CF is exceptionally salty sweat.

Orofacial/odontological symptoms

Persons with the diagnosis may have difficulties in breathing through the nose and mouth breathing increases the risk of dry mouth. Some people may suffer from gastroesophageal reflux (acid reflux) which in time may increase the risk of enamel damage. Prolonged treatment with antibiotics likely explains why children with cystic fibrosis have fewer cavities than might be expected. Prolonged antibiotic treatment is commonly associated with fungal infection of the mouth.

Orofacial/odontological treatment

  • Many of these children and adults require extra prophylactic dental care.
  • In case of fungal infection in the mouth, there are effective prescription drugs available.

Sources

  • The rare disease database of the Swedish National Board of Health and Welfare.
  • The MHC database - The Mun-H-Center database on oral health and orofacial function in rare diseases.
  • The Documentation from the Ågrenska national competence centre for rare diseases.
Updated: 2017-09-12 14:00