Abstract

Cystic fibrosis is a genetic disorder in which the mutation of the Cystis Fibrosis Transmembrane Conductance Regulator (CFTR) gene that codes the protein forming a chloride channel of epithelial cells results in its distorted functioning. The manifestations of the disorder are mainly observed in the respiratory and digestive system. Accumulation of sticky and thick mucus is the dominant clinical symptom; it leads to chronic infections and gradual tissue destruction. Although cystic fibrosis remains incurable, it is currently feasible to extend patients' life expectancy thanks to modern therapy possibilities. As cystic fibrosis is no longer the domain of pediatricians, health care to CF patients needs to be provided by doctors of various specializations. The multidisciplinary team of doctors should include a dentist aware of specific prevention and treatment needs of this group of patients. It results from the fact that in the course of cystic fibrosis it is possible to observe a variety of changes in the oral cavity environment. The study presents dental issues observed in CF patients and reported in literature. Particular attention was paid to dental caries, mineralization disorders of hard dental tissues, gingivitis and the change in the content and properties of saliva; moreover, prevention and treatment options regarding oral cavity health is this group of patients were taken into consideration.

Highlights

  • IntroductionCystic fibrosis (lat. mucoviscidosis) is the most frequent genetically conditioned total exocrine dysfunction among Caucasians

  • Cystic fibrosis is the most frequent genetically conditioned total exocrine dysfunction among Caucasians

  • This study presents dental problems experienced by cystic fibrosis patients and reported in the literature

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Summary

Introduction

Cystic fibrosis (lat. mucoviscidosis) is the most frequent genetically conditioned total exocrine dysfunction among Caucasians. Mucoviscidosis) is the most frequent genetically conditioned total exocrine dysfunction among Caucasians. It is inherited in an autosomal recessive manner and the frequency of its occurrence (1:2000 births) is similar for both sexes.[1,2,3] Since 2006, due to the high population frequency of the disease, two-stage newborn screening for cystic fibrosis has been conducted in Poland. The level of trypsin in the blood is determined, and a molecular test is conducted. Immunoreactive tripsin (ITR) is measured in a blood sample drawn on a piece of absorbent paper between the 3rd and the 6th day of life. An increased level of ITR may indicate cystic fibrosis; the initial diagnosis is later confirmed with a molecular DNA analysis. With the use of this test, it is possible to establish an early diagnosis and implement professional treatment

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