The mutation of cystic fibrosis transmembrane conductance regulator (CFTR) can modify the physical and chemical properties of saliva, which in turn can affect the oral microflora and oral health in patients with cystic fibrosis (CF). The aim of the study was to examine oral health status, salivary properties, and total oral bacteria count in CF adults. Dental status was assessed using the decayed missing filled surfaces (DMF-S) index, and oral clearness using the approximal plaque index (API). The Saliva-Check BUFFER test was used to assess saliva, and real-time polymerase chain reaction (PCR) test to determine the total oral bacteria count. CF patients in comparison to healthy controls showed a higher level of examined clinical indices, higher total oral bacteria count, lower salivary flow rate, lower salivary pH, and increased viscosity. Conclusions: In CF patients, saliva properties, accompanied by insufficient dental care, might be an essential dental caries risk factor. In CF patients, among the etiological factors for dental caries, the bacterial agent seems to be less significant. The frequent and long-term infectious pharmacotherapy can probably explain that. A great deal of the information collected on the oral environment in CF patients, which has helped us understand the etiological conditions for inflammation and infection in this area of the body, indicates that proper dental care can mostly counteract these pathologies.

Papillon-Lefëvre syndrome (PLS), classified as ectodermal dysplasia, is an autosomal recessive condition related to the cathepsin C (CTSC) gene mutation. The first clinical symptoms, occurring most commonly between the ages of 1 and 4, are palmoplantar hyperkeratosis and also periodontitis...

This study aimed to assess the asymmetry of the lower face and motor dysfunction of the masticatory system resulting from unilateral or asymmetrical bilateral temporomandibular joint (TMJ) involvement in juvenile idiopathic arthritis (JIA) patients. The study consisted of clinical examination and cone beam computed tomography (CBCT) of TMJs. Statistical analysis showed correlations between several factors: facial asymmetry and mandibular lateral deviation; the direction of mandibular deviation and the degree of radiological deformities in TMJs; the child’s age at the time of the onset and the range of lateral movement towards the healthy or less destructed joint. In addition, there was a significant difference in ranges of lateral movements; a significantly smaller range was observed for the joint with fewer condylar abnormalities compared to the range in the opposite direction. In JIA children, among the clinical markers of unilateral or asymmetrical TMJ involvement, the asymmetry of the lower face, deviation of the mandible on opening, and an uneven range of mandibular lateral movements deserve attention. The obtained results do not show a relationship between the degree of condylar changes and the asymmetry of the lower face and the presence and degree of mandibular motor dysfunction.

Background: Primary ciliary dyskinesia (PCD) is a rare, ciliopathic disorder. In many ciliopathies, dental anomalies are observed alongside other symptoms of the disease. To date, there are no published reports concerning the dental developmental problems that are associated with ciliary defects in PCD patients. Methods: Patients suffering from PCD underwent dental clinical examination, which included the assessment of developmental disorders regarding the number and morphological structure of the teeth (size and shape) as well as developmental disorders of mineralised dental tissues. Then, three-dimensional radiographic examination was performed utilising Cone Beam Computed Tomography (CBCT). Results: Four PCD patients, aged 31-54, agreed to enter the study. Dental examinations showed the presence of dental developmental disorders in three of them. Additionally, CBCT showed abnormalities in those patients. Conclusions: 1. The dental phenotype in PCD patients seems to be heterogeneous. Tooth developmental disorders resulting from abnormal odontogenesis may be a symptom of PCD that is concomitant with other developmental abnormalities resulting from malfunctioning primary cilia. 2. Patients with ciliopathies are likely to develop dental developmental defects. Therefore, beginning in early childhood, they should be included in a targeted specialised dental programme to enable early diagnosis and to ensure dedicated preventive and therapeutic measures.