Oral Health-Related Quality of Life in People with Rare Hereditary Connective Tissue Disorders: Marfan Syndrome

Marcel Hanisch, Sabrina Wiemann, Susanne Jung, Johannes Kleinheinz and Lauren Bohner
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Marcel Hanisch: Department of Cranio-Maxillofacial Surgery, Research Unit Rare Diseases with Orofacial, Manifestations (RDOM), University Hospital Münster, Albert-Schweitzer-Campus 1, Building W 30, D-48149 Münster, Germany
Sabrina Wiemann: Department of Cranio-Maxillofacial Surgery, Research Unit Rare Diseases with Orofacial, Manifestations (RDOM), University Hospital Münster, Albert-Schweitzer-Campus 1, Building W 30, D-48149 Münster, Germany
Susanne Jung: Department of Cranio-Maxillofacial Surgery, Research Unit Rare Diseases with Orofacial, Manifestations (RDOM), University Hospital Münster, Albert-Schweitzer-Campus 1, Building W 30, D-48149 Münster, Germany
Johannes Kleinheinz: Department of Cranio-Maxillofacial Surgery, Research Unit Rare Diseases with Orofacial, Manifestations (RDOM), University Hospital Münster, Albert-Schweitzer-Campus 1, Building W 30, D-48149 Münster, Germany
Lauren Bohner: Department of Cranio-Maxillofacial Surgery, Research Unit Rare Diseases with Orofacial, Manifestations (RDOM), University Hospital Münster, Albert-Schweitzer-Campus 1, Building W 30, D-48149 Münster, Germany

IJERPH, 2018, vol. 15, issue 11, 1-8

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.

Keywords: rare diseases; oral health-related quality of life; OHRQoL; Marfan; patient reported outcome; OHIP-14 (search for similar items in EconPapers)
JEL-codes: I I1 I3 Q Q5 (search for similar items in EconPapers)
Date: 2018
References: View references in EconPapers View complete reference list from CitEc
Citations: View citations in EconPapers (3)

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