EVALUATION OF THE EFFECTIVENESS OF DENTAL REHABILITATION OF CHILDREN WITH ORPHAN DISEASES AND DISORDERS OF PHOSPHORUS-CALCIUM METABOLISM BASED ON QUALITY OF LIFE CRITERIA
Abstract and keywords
Abstract:
In recent decades, quality of life assessment has become an integral part of a comprehensive approach to managing patients with rare diseases. An integrated assessment of the impact of dental health on key areas of life is becoming a component of comprehensive rehabilitation for children with rare diseases, aimed at restoring quality of life. Objective: To study the impact of dental rehabilitation on the dynamics of quality of life criteria in children with rare phosphorus-calcium metabolism disorders using the OHRQoL questionnaire. Materials and Methods A clinical and sociological study was conducted in groups of children (6–17 years) with the genetic diseases X-linked hypophosphatemic rickets (X-HPR), hypophosphatasia (HPP), and osteogenesis imperfecta (OI). The effects of dental rehabilitation on the condition of dental tissues in children were studied according to clinical indices (OHI-S, CAST) and the dynamics of quality of life criteria using the Oral Health-Related Quality of Life (OHRQoL) parental questionnaire. The questionnaire (OHRQoL) is a tool for assessing the impact of dental problems on various areas of daily life, reflecting physical discomfort in the oral cavity; the child's communication difficulties; support and resolution of dental problems by legal representatives. Results. Dental rehabilitation in groups of children with X-linked hypophosphatemic rickets (X-HPR), hypophosphatasia (HPP), and osteogenesis imperfecta (OI) demonstrated significant clinical efficacy and a reduction in physical discomfort in the oral cavity, as evidenced by a reduction in the functional domain scores of the OHRQoL questionnaire. Furthermore, it was found that parents of children with orphan diseases showed increased adherence to dental treatment and preventive measures.

Keywords:
children with orphan diseases, dental status, quality of life assessment, Oral Health-Related Quality of Life (OHRQoL) questionnaire, dental rehabilitation
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