AIM: Despite the fact that ectodermal dysplasia (ED) is a rare disease, it is often seen in a tertiary clinic. ED affects ectodermal tissues such as skin, hair, teeth, nails, and sweat glands. Patients usually have sparse light hair, deformed nails, and dry skin. They suffer from dental abnormalities such as oligodontia (absence of 6 or more teeth) or complete anodontia; salivation can also be affected. The absence of teeth can be the overriding problem for both patients and their parents, and lead to substantial social ostracisation. This study aims to summarise the facts about the disease, especially dental treatment options based on data drawn from a representative Czech cohort. MATERIALS: The present article summarises the facts about ectodermal dysplasia (ED) in a cohort of 13 patients, where the following were evaluated: clinical manifestations of ED, pathogenic variants detected in selected candidate genes and dental treatment options from child removable dentures to fixed crowns and implants insertion. Three cases are described in detail and demonstrate approaches for different age groups. CONCLUSION: Early diagnosis and active cooperation between the geneticist and dentist will facilitate cooperation with parents and patients and assure secondary prevention. It is preferable that the geneticist understands dental treatment options and can discuss these with patients/parents.

• MeSH
• anodoncie * terapie   MeSH
• dítě MeSH
• ektodermální dysplazie * komplikace   terapie   MeSH
• lidé MeSH
• stomatologická péče MeSH
• Check Tag
• dítě MeSH
• lidé MeSH
• Publikační typ
• časopisecké články MeSH

AIM: This study investigates the influence of early childhood caries (ECC) on the occurence of developmental defects of enamel (DDE) on permanent teeth. METHODS: Study design: This prospective original study was conducted to monitor the relationship between ECC in primary dentition and DDE as their consequences on permanent successors. One hundred and forty children were recruited and included in the study group with diagnosed ECC (ECCG) (N=60) or the control group (CG) (N=80). Deciduous upper central incisors were assessed for existing dental caries according to the dmft index and the modified pufa score and permanent incisors were examined using the modified DDE index. RESULTS: Prevalence of DDE was 19.3% in the whole sample; in ECCG it was 24.2% and 12.6% in CG. The most common defect was diffuse opacity with a prevalence of 44.8% in ECCG and 72% in CG. Prevalence of hypoplasia was 13.8% in ECCG, in CG it was present as a combination defect with a prevalence of 4%. The extent of less than 1/3 of labial aspect was in 69% of defects in ECCG,in CG it was 52%. The location of defects in the incisal third was 48.3% in ECCG and 32% in controls. CONCLUSION: A statistically significant relationship between ECC and DDE was not observed. The results were compared with studies about the relationship between ECC and DDE.

• MeSH
• dítě MeSH
• hypoplazie zubní skloviny * epidemiologie   MeSH
• lidé MeSH
• longitudinální studie MeSH
• náchylnost k zubnímu kazu MeSH
• předškolní dítě MeSH
• prevalence MeSH
• prospektivní studie MeSH
• zubní kaz * epidemiologie   MeSH
• zubní sklovina MeSH
• zuby mléčné MeSH
• Check Tag
• dítě MeSH
• lidé MeSH
• předškolní dítě MeSH
• Publikační typ
• časopisecké články MeSH
• Geografické názvy
• Česká republika MeSH