Cartilage-hair hypoplasia (CHH) is one of the most common hereditary primary immunodeficiency syndromes in Finland. CHH is characterized by short stature and hypoplastic hair. The patients suffer from anemia, impaired immunity, and increased risk for malignancies. An increased risk for prolonged human papilloma virus (HPV) infection has also been found. The aim of this study is to explore craniofacial growth and dental development in patients with CHH, as well as the association between impaired immunity and oral health. Due to the immunological defects and increased incidence of cancer associated with CHH, it is important to increase our knowledge on oral health of these patients in order to minimize oral infections, and to establish a follow-up system for the patients with prolonged oral HPV infection.

2020

2022