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Oral Health-Related Quality of Life in Different Subtypes of Ehlers-Danlos Syndrome.

ABSTRACT: This study assessed differences in the oral health-related quality of life (OHRQoL) between subtypes of Ehlers-Danlos syndrome (EDS). For statistical analysis, participants were divided according to their subtype: classical EDS (cEDS), hypermobile EDS (hEDS), and vascular EDS (vEDS). All other subtypes were descriptively analyzed. Free-text questions and the German short form of the Oral Health Impact Profile (OHIP-14) were used. Finally, 295 questionnaires were included, representing 10 different EDS subtypes. The mean OHIP score of all participants was 19.6 points (standard derivation (SD) ± 12.3). The most predominant subtypes showed similar reduced OHRQoL, with 18.0 (cEDS, ±12.9), 19.5 (hEDS, ±12.0), and 15.2 (vEDS, ±11.6) OHIP points. For all other subtypes, the OHIP values varied. Participants waited an average of 21.8 years (±12.8) for their diagnosis. However, within the predominant subtypes, vEDS patients waited a noticeably shorter period of 13.3 years (±13.0; p = 0.004) compared to participants with hEDS. Additionally, this study showed no difference in OHRQoL for the predominant subtypes regardless of whether a participant was a self-help group member (18.8, ±12.0) or not (19.4, ±12.1; p = 0.327).

SUBMITTER: Balke J

PROVIDER: S-EPMC9916345 | biostudies-literature | 2023 Jan

REPOSITORIES: biostudies-literature

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Oral Health-Related Quality of Life in Different Subtypes of Ehlers-Danlos Syndrome.

Balke Julius J Bohner Lauren L Köppe Jeanette J Jackowski Jochen J Oelerich Ole O Hanisch Marcel M

International journal of environmental research and public health 20230126 3

This study assessed differences in the oral health-related quality of life (OHRQoL) between subtypes of Ehlers-Danlos syndrome (EDS). For statistical analysis, participants were divided according to their subtype: classical EDS (cEDS), hypermobile EDS (hEDS), and vascular EDS (vEDS). All other subtypes were descriptively analyzed. Free-text questions and the German short form of the Oral Health Impact Profile (OHIP-14) were used. Finally, 295 questionnaires were included, representing 10 differe ...[more]

PMID: 36767586

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Project description:Background and objective: To date, there have only been a few studies on oral health-related quality of life (OHRQoL) of people with Ehlers-Danlos syndromes (EDS) and oral conditions. The aim of this study was, therefore, to analyze the OHRQoL of people with EDS from their own point of view as well as obtain information about their age at the time of the diagnosis, the period of time until diagnosis, and the presence of oral conditions (if any) and their association with oral health quality. Methods: The study was designed as an anonymous questionnaire-based cross-sectional study. We conducted a descriptive analysis of the Oral Health Impact Profile-14 (OHIP-14) scores, age of the participants, age at diagnosis, and the time-period between the first signs of the disease and the diagnosis of EDS. To verify the differences in OHIP-14 scores between patients with and without oral conditions, a Mann-Whitney U test was performed. A multivariate quantile (median) regression analysis was performed to evaluate the effect of different general characteristics (gender, age, and the presence of oral conditions) on the OHIP 14 scores. Furthermore, using a Mann-Whitney U test, the influence of different oral conditions was verified by testing the differences between patients without any oral conditions and patients with a specific diagnosis. Results: A total of 79 evaluable questionnaires from 66 female (83.5%) and 13 male (16.5%) participants were analyzed. On average, after the first condition, it takes 18.36 years before EDS are correctly diagnosed. Oral conditions were described by 69.6% of the participants. The median (interquartile range) OHIP-14 score was eight (ten) points for patients without oral conditions and 19 (15) for patients with oral conditions. The multivariable quantile regression shows a statistical notable association between OHIP-14 score and oral conditions (p < 0.001). OHIP-14 scores for dysgnathia, periodontitis, TMD (Temporomandibular dysfunction), a high-arched palate, malocclusion, and the anomaly of tooth formation were statistical notably different between the participants with and the participants without oral conditions. Conclusions: Long diagnostic pathways seem to be a typical problem in patients with EDS. Oral conditions associated with the underlying disease occurred regularly and showed a negative correlation with OHRQoL.

Project description:Ehlers-Danlos syndrome type IV, the vascular type of Ehlers-Danlos syndromes (EDS), is an inherited connective tissue disorder defined by characteristic facial features (acrogeria) in most patients, translucent skin with highly visible subcutaneous vessels on the trunk and lower back, easy bruising, and severe arterial, digestive and uterine complications, which are rarely, if at all, observed in the other forms of EDS. The estimated prevalence for all EDS varies between 1/10,000 and 1/25,000, EDS type IV representing approximately 5 to 10% of cases. The vascular complications may affect all anatomical areas, with a tendency toward arteries of large and medium diameter. Dissections of the vertebral arteries and the carotids in their extra- and intra-cranial segments (carotid-cavernous fistulae) are typical. There is a high risk of recurrent colonic perforations. Pregnancy increases the likelihood of a uterine or vascular rupture. EDS type IV is inherited as an autosomal dominant trait that is caused by mutations in the COL3A1 gene coding for type III procollagen. Diagnosis is based on clinical signs, non-invasive imaging, and the identification of a mutation of the COL3A1 gene. In childhood, coagulation disorders and Silverman's syndrome are the main differential diagnoses; in adulthood, the differential diagnosis includes other Ehlers-Danlos syndromes, Marfan syndrome and Loeys-Dietz syndrome. Prenatal diagnosis can be considered in families where the mutation is known. Choriocentesis or amniocentesis, however, may entail risk for the pregnant woman. In the absence of specific treatment for EDS type IV, medical intervention should be focused on symptomatic treatment and prophylactic measures. Arterial, digestive or uterine complications require immediate hospitalisation, observation in an intensive care unit. Invasive imaging techniques are contraindicated. Conservative approach is usually recommended when caring for a vascular complication in a patient suffering from EDS type IV. Surgery may, however, be required urgently to treat potentially fatal complications.

Dataset Information

Oral Health-Related Quality of Life in Different Subtypes of Ehlers-Danlos Syndrome.

Publications

Oral Health-Related Quality of Life in Different Subtypes of Ehlers-Danlos Syndrome.

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