Abstract
Background: The aim of this study was to analyze data on oral health-related quality of life (OHRQoL) in people with Marfan syndrome and to obtain information on the diagnosis period, orthodontic treatment, and oral symptoms. Methods: A questionnaire was developed consisting of open questions and the standardized German version of the OHIP-14 (Oral Health Impact Profile) questionnaire for the evaluation of OHRQoL. The age of diagnosis, time period from the first signs of the disease to diagnosis, and OHIP-values were compared between male and female participants. Additionally, the OHIP-values between participants who were orthodontically treated and those who were not treated were assessed. The statistical analysis was performed using the Mann–Whitney test with a significance level at p = 0.05. Results: A total of 51 questionnaires were evaluated, which included 34 female and 17 male participants. Overall, 84% of respondents reported oral symptoms. Male respondents tended to diagnose the disease earlier (p = 0.00), with a smaller period between the first symptom and the diagnosis (p = 0.04). The OHIP-14 score was gender-neutral at 13.65 ± 13.53 points. Conclusion: In Marfan syndrome, many years (12.01 ± 11.61) elapse between the onset of first symptoms and correct diagnosis of the disease. People with Marfan syndrome have a worse OHRQoL than do the general population.
Highlights
IntroductionMarfan syndrome is a genetic systemic connective tissue disorder with varied and combined symptoms of the heart, circulation, muscles, skeleton, eyes, and lungs [2]
In the European Union, a disease is considered “rare” if it affects less than one in 2000 people [1].Hereditary connective tissue diseases such as Marfan syndrome are classified as rare diseases.The prevalence of Marfan syndrome is 1:5000 for both genders [2,3].Marfan syndrome is a genetic systemic connective tissue disorder with varied and combined symptoms of the heart, circulation, muscles, skeleton, eyes, and lungs [2]
The study was designed as an anonymous epidemiological survey to be carried out amongst people with Marfan syndrome to evaluate their oral health-related quality of life (OHRQoL)
Summary
Marfan syndrome is a genetic systemic connective tissue disorder with varied and combined symptoms of the heart, circulation, muscles, skeleton, eyes, and lungs [2]. The aim of this study was to analyze data on oral health-related quality of life (OHRQoL) in people with Marfan syndrome and to obtain information on the diagnosis period, orthodontic treatment, and oral symptoms. The age of diagnosis, time period from the first signs of the disease to diagnosis, and OHIP-values were compared between male and female participants. Male respondents tended to diagnose the disease earlier (p = 0.00), with a smaller period between the first symptom and the diagnosis (p = 0.04).
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