Oral Health-Related Quality of Life in Italian Children and Adolescents Living with Bone Dysplasia: A Cross-Sectional Study
Abstract
:1. Introduction
2. Materials and Methods
2.1. Data Collection
2.2. Statistical Analysis
3. Results
3.1. Demographic and Medical Features of the Sample
3.2. OHRQoL Questionnaires
4. Discussion
5. Conclusions
Supplementary Materials
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Conflicts of Interest
References
- Savarirayan, R.; Tunkel, D.E.; Sterni, L.M.; Bober, M.B.; Cho, T.J.; Goldberg, M.J.; Hoover-Fong, J.; Irving, M.; Kamps, S.E.; Mackenzie, W.G.; et al. Best practice guidelines in managing the craniofacial aspects of skeletal dysplasia. Orphanet J. Rare Dis. 2021, 14, 31. [Google Scholar] [CrossRef] [PubMed]
- Walleczek, N.K.; Förster, K.; Seyr, M.; Kadrnoska, N.; Kolar, J.; Wasinger-Brandweiner, V.; Vodopiutz, J. Rare skeletal disorders: A multidisciplinary postnatal approach to diagnosis and management. Wien Med. Wochenschr. 2021, 171, 94–101. [Google Scholar] [CrossRef]
- Handa, A.; Grigelioniene, G.; Nishimura, G. Skeletal dysplasia families: A stepwise approach to diagnosis. Radiographics 2023, 43, 220067. [Google Scholar] [CrossRef] [PubMed]
- Rothenbuhler, A.; Schnabel, D.; Högler, W.; Linglart, A. Diagnosis, treatment-monitoring and follow-up of children and adolescents with X-linked hypophosphatemia (XLH). Metabolism 2020, 103, 153892. [Google Scholar] [CrossRef] [PubMed]
- Cho, T.J.; Ko, J.M.; Kim, H.; Shin, H.I.; Yoo, W.J.; Shin, C.H. Management of osteogenesis imperfecta: A multidisciplinary comprehensive approach. Clin. Orthop. Surg. 2020, 12, 417–429. [Google Scholar] [CrossRef] [PubMed]
- Najirad, M.; Ma, M.S.; Rauch, F.; Sutton, V.R.; Lee, B.; Retrouvey, J.M.; Members of the BBD; Esfandiari, S. Oral health-related quality of life in children and adolescents with osteogenesis imperfecta: Cross-sectional study. Orphanet J. Rare Dis. 2018, 25, 187. [Google Scholar] [CrossRef] [PubMed]
- Robinson, C.; Collins, M.T.; Boyce, A.M. Fibrous Dysplasia/McCune-Albright Syndrome: Clinical and translational perspectives. Curr. Osteoporos. Rep. 2016, 14, 178–186. [Google Scholar] [CrossRef] [PubMed]
- Online Mendeline Inheritance of Man. Available online: https://www.omim.org (accessed on 12 January 2024).
- Jackowski, J.; Hanisch, M. Orofacial manifestations in 2006 rare diseases—A preliminary systematic review of the literature. J. Ger. Soc. Dent. Oral Med. 2012, 67, D10. [Google Scholar]
- Hanisch, M.; Hanisch, L.; Kleinheinz, J.; Danesh, G.; Benz, K.; Jackowski, J. Orthodontically relevant manifestations in people with rare diseases. Med. Princ. Pract. 2019, 28, 216–221. [Google Scholar] [CrossRef]
- Hanisch, M.; Jung, S.; Kleinheinz, J. Oral health-related quality of life in rare diseases with oral manifestations. Gesundheitswesen 2019, 81, 902–906. [Google Scholar] [CrossRef]
- Bennadi, D.; Reddy, C.V.K. Oral health related quality of life. J. Int. Soc. Prev. Community Dent. 2013, 3, 1–6. [Google Scholar] [CrossRef]
- Wang, Y.W.; Carnevale, F.A.; Ezcurra, M.; Chougui, K.; Bilodeau, C.; Siedlikowski, S.; Tsimicalis, A. The moral experiences of children with osteogenesis imperfecta. Nurs. Ethics 2022, 29, 1773–1791. [Google Scholar] [CrossRef]
- Sischo, L.; Broder, H.L. Oral health-related quality of life: What, why, how, and future implications. J. Dent. Res. 2011, 90, 1264–1270. [Google Scholar] [CrossRef]
- Nguyen, C.; Celestin, E.; Chambolle, D.; Linglart, A.; Biosse Duplan, M.; Chaussain, C.; Friedlander, L. Oral health-related quality of life in patients with X-linked hypophosphatemia: A qualitative exploration. Endocr. Connect. 2022, 28, 11. [Google Scholar] [CrossRef]
- Gjørup, H.; Beck-Nielsn, S.S.; Hald, J.D.; Haubek, D. Oral health-related quality of life in X-linked hypophosphataemia and osteogenesis imperfecta. J. Oral Rehabil. 2021, 48, 160–168. [Google Scholar] [CrossRef]
- Cachia Mintoff, J.M.; Riddington, M.; Parekh, S. Oral health-related quality of life in children with osteogenesis imperfecta. Eur. Arch. Paediatr. Dent. 2022, 23, 261–270. [Google Scholar] [CrossRef] [PubMed]
- Corridore, D.; Campus, G.; Guerra, F.; Ripari, F.; Sale, S.; Ottolenghi, L. Validation of the Italian version of the Oral Health Impact Profile-14 (IOHIP-14). Ann. Stomatol. 2014, 4, 239–243. [Google Scholar]
- Broder, H.L.; Wilson-Genderson, M. Reliability and convergent and discriminant validity of the Child Oral Health Impact Profile (COHIP Child’s version). Community Dent. Oral Epidemiol. 2007, 35, 20–31. [Google Scholar] [CrossRef]
- Jokovic, A.; Locker, D.; Guyatt, G. Short forms of the Child Perceptions Questionnaire for 11–14-year-old children (CPQ11-14): Development and initial evaluation. Health Qual. Life Outcomes 2006, 19, 4. [Google Scholar] [CrossRef]
- John, M.T.; Rener-Sitar, K.; Baba, K.; Čelebić, A.; Larsson, P.; Szabo, G.; Norton, W.E.; Reissmann, D.R. Patterns of impaired oral health-related quality of life dimensions. J. Oral Rehabil. 2016, 43, 519–527. [Google Scholar] [CrossRef] [PubMed]
- Carr, A.J.; Gibson, B.; Robinson, P.G. Measuring quality of life: Is quality of life determined by expectations or experience? BMJ 2001, 322, 1240–1243. [Google Scholar] [CrossRef]
- Dahan-Oliel, N.; Oliel, S.; Tsimicalis, A.; Montpetit, K.; Rauch, F.; Dogba, M.J. Quality of life in osteogenesis imperfecta: A mixed-methods systematic review. Am. J. Med. Genet A 2016, 170, 62–76. [Google Scholar] [CrossRef] [PubMed]
- Ierardo, G.; Calcagnile, F.; Luzzi, V.; Ladniak, B.; Bossu, M.; Celli, M.; Zambrano, A.; Franchi, L.; Polimeni, A. Osteogenesis imperfecta and rapid maxillary expansion: Report of 3 patients. Am. J. Orthod. Dentofac. Orthop. 2015, 148, 130–137. [Google Scholar] [CrossRef] [PubMed]
- Kok, D.H.J.; Sakkers, R.J.B.; Janse, A.J.; Pruijs, H.E.H.; Verbout, A.J.; Castelein, R.M.; Engelbert, R.H.H. Quality of life in children with osteogenesis imperfecta treated with oral bisphosphonates (olpadronate): A 2-year randomized placebo-controlled trial. Eur. J. Pediatr. 2007, 166, 1155–1161. [Google Scholar] [CrossRef] [PubMed]
- Oliveira, T.C.; Bradaschia-Correa, V.; Castro, J.R.; Simões, A.; Arana-Chavez, V.E. Ultrastructural and biochemical analysis of the effects of alendronate on salivary glands of young rats. Arch. Oral Biol. 2014, 59, 1307–1311. [Google Scholar] [CrossRef]
- Tessaris, D.; Matarazzo, P.; Lala, R.; Defabianis, P. Odontoiatric perspectives and osteonecrosis of the jaw as a possible adverse effect of bisphosphonates therapy in fibrous dysplasia and McCune-Albright syndrome. J. Pediatr. Endocrinol. Metab. 2016, 29, 333–336. [Google Scholar] [CrossRef]
Variables | Bone Dysplasia (n = 40) | Controls (n = 40) | p-Value |
---|---|---|---|
Age (years), mean ± SD | 11.1 ± 2.5 | 10.8 ± 2.2 | 0.538 |
Gender, n (%) | NA | ||
Female | 19 (47.5) | 19 (47.5) | |
Male | 21 (52.5) | 21 (52.5) | |
Race, n (%) | 0.494 | ||
White | 38 (95.0) | 40 (100) | |
Black or other | 2 (5.0) | 0 (0.0) | |
Parents’ education n, (%) | 0.581 | ||
High | 8 (20.0) | 12 (30.0) | |
Medium | 20 (50.0) | 18 (45.0) | |
Low | 12 (30.0) | 10 (25.0) | |
BD diagnosis, n (%) | |||
OI | 24 (60.0) | - | |
XHL | 5 (12.5) | - | |
MAS | 2 (5.0) | - | |
FD | 3 (7.5) | - | |
PHP | 6 (15.0) | - | |
Familiarity for BD, n (%) | |||
Mother line | 15 (37.5) | - | |
Father line | 5 (12.5) | - | |
Both parents | 4 (10.0) | - | |
No familiarity | 16 (40.0) | - |
OI (n = 24) | XHL (n = 5) | PHP (n = 6) | MAS (n = 2) | FD (n = 3) | Total (n = 40) | |
---|---|---|---|---|---|---|
No pain | 15 (62.5) | 4 (80.0) | 3 (50.0) | 2 (100) | 3 (100) | 27 (67.5) |
Localized pain | 5 (20.8) | 1 (20.0) | 2 (33.33) | 0 (0.0) | 0 (0.0) | 8 (20.0) |
Diffuse pain | 3 (12.5) | 0 (0.0) | 1 (16.7) | 0 (0.0) | 0 (0.0) | 4 (10.0) |
Occasional pain | 1 (4.2) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 0 (0.0) | 1 (2.5) |
Questionnaires | Group | p Value | |||
---|---|---|---|---|---|
OHIP-14 domains (maximum possible score) | Bone dysplasia patients (n = 40) | Controls (n = 40) | |||
Mean ± SD | Median (min/max) | Mean ± SD | Median (min/max) | ||
Overall OHIP (56) | 5.82 ± 6.69 | 3.0 (0/22) | 2.40 ± 3.90 | 0.0 (0/21) | 0.011 |
Functional limitation (8) | 0.45 ± 1.19 | 0.0 (0/6) | 0.50 ± 0.93 | 0.0 (0/4) | 0.272 |
Physical pain (8) | 1.85 ± 1.77 | 2.0 (0/6) | 1.05 ± 1.58 | 0.0 (0/5) | 0.025 |
Phycological discomfort (8) | 1.12 ± 1.92 | 0.0 (0/8) | 0.17 ± 0.54 | 0.0 (0/2) | 0.003 |
Physical disability (8) | 0.62 ± 1.54 | 0.0 (0/7) | 0.32 ± 0.97 | 0.0 (0/5) | 0.504 |
Psychosocial disability (8) | 1.10 ± 1.67 | 0.0 (0/5) | 0.25 ± 0.54 | 0.0 (0/2) | 0.026 |
Social disability (8) | 0.40 ± 0.95 | 0.0 (0/4) | 0.075 ± 0.47 | 0.0 (0/3) | 0.015 |
Social handicap (8) | 0.27 ± 0.78 | 0.0 (0/3) | 0.02 ± 0.15 | 0.0 (0/1) | 0.084 |
SF-CPQ domains (maximum possible score) | Bone dysplasia patients (n = 40) | Controls (n = 40) | |||
Mean ± SD | Median (min/max) | Mean ± SD | Median (min/max) | ||
Overall SF-CPQ (16) | 7.40 ± 6.40 | 6.0 (0/32) | 1.50 ± 2.80 | 0.0 (0/12) | <0.001 |
Oral symptoms (4) | 2.85 ± 1.91 | 3.0 (0/8) | 0.62 ± 1.07 | 0.0 (0/4) | <0.001 |
Functional well-being (4) | 1.77 ± 2.24 | 1.0 (0/8) | 0.40 ± 1.13 | 0.0 (0/4) | <0.001 |
Emotional well-being (4) | 1.52 ± 1.85 | 1.0 (0/8) | 0.15 ± 0.48 | 0.0 (0/2) | <0.001 |
Social well-being (4) | 1.27 ± 1.83 | 0.0 (0/8) | 0.22 ± 0.69 | 0.0 (0/3) | 0.002 |
COHIP domains * (maximum possible score) | Bone dysplasia patients (n = 40) | Controls (n = 40) | |||
Mean ± SD | Median (min/max) | Mean ± SD | Median (min/max) | ||
Overall COHIP (136) | 108.37 ± 12.80 | 110.5 (82/135) | 126.25 ± 8.08 | 128.0 (106/136) | <0.001 |
Oral symptoms (40) | 30.17 ± 5.95 | 30.5 (15/39) | 36.6 ± 3.84 | 37.0 (26/40) | <0.001 |
Functional well-being (24) | 18.60 ± 3.56 | 19.0 (9/24) | 22.65 ± 1.95 | 23.0 (16/24) | <0.001 |
Emotional well-being (32) | 27.50 ± 4.41 | 28.5 (13/32) | 31.82 ± 0.67 | 32.0 (28/32) | <0.001 |
School environment (16) | 14.92 ± 1.43 | 16.0 (12/16) | 15.32 ± 1.20 | 16.0 (12/16) | 0.159 |
Peer interaction (24) | 17.17 ± 5.12 | 17.5 (6/24) | 19.85 ± 3.41 | 20.0 (14/24) | 0.020 |
Group | OHIP-14 Score [Mean ± SD, Median, Range)] | |||||||
---|---|---|---|---|---|---|---|---|
Overall | Functional Limitation | Physical Pain | Phycological Discomfort | Physical Disability | Psychosocial Disability | Social Disability | Social Handicap | |
Gender | ||||||||
Female (n = 19) | 6.9 ± 7.9 4.0 (0/22) | 0.21 ± 0.71 0.0 (0/3) | 1.78 ± 1.98 1.0 (0/6) | 1.42 ± 1.98 0.0 (0/6) | 1.05 ± 1.98 0.0 (0/7) | 1.42 ± 1.80 0.0 (0/5) | 0.57 ± 0.16 0.0 (0/4) | 0.47 ± 1.02 0.0 (0/3) |
Male (n = 21) | 4.8 ± 5.3 3.0 (0/19) | 0.60 ± 1.49 0.0 (0/6) | 1.90 ± 1.60 2.0 (0/6) | 0.85 ± 1.87 0.0 (0/8) | 0.23 ± 0.88 0.0 (0/4) | 0.8 ± 1.47 0.0 (0/4) | 0.23 ± 0.7 0.0 (0/3) | 0.89 ± 0.43 0.0 (0/2) |
p-Value | 0.830 | 0.469 | 0.688 | 0.307 | 0.226 | 0.282 | 0.486 | 0.376 |
Age | ||||||||
8–10 yrs (n = 18) | 5.8 ± 6.5 3.5 (0/20) | 0.22 ± 0.64 0.0 (0/2) | 2.16 ± 1.82 2.0 (0/6) | 0.83 ± 1.54 0.0 (0/6) | 1.16 ± 2.09 0.0 (0/7) | 0.83 ± 1.42 0.0 (0/5) | 0.44 ± 1.04 0.0 (0/4) | 0.22 ± 0.73 0.0 (0/3) |
11–14 yrs (n = 22) | 5.7 ± 6.96 2.5 (0/22) | 0.63 ± 1.49 0.0 (0/6) | 1.59 ± 1.73 1.5 (0/6) | 1.36 ± 2.19 0.0 (0/8) | 0.18 ± 0.66 0.0 (0/3) | 1.31 ± 1.86 0.0 (0/5) | 0.36 ± 0.99 0.0 (0/3) | 0.31 ± 0.83 0.0 (0/3) |
p-Value | 0.717 | 0.527 | 0.325 | 0.563 | 0.172 | 0.563 | 0.840 | 0.882 |
Group | SF-CPQ Scores [Mean ± SD, Median, (Range)] | ||||
---|---|---|---|---|---|
Overall SF-CPQ | SF-CPQ Oral Symptoms | SF-CPQ Functional | SF-CPQ Emotional Well-Being | SF-CPQ Social Well-Being | |
Gender | |||||
Female (n = 19) | 9.2 ± 8.0 7.0 (0/32) | 3.47 ± 2.11 3.0 (0/8) | 2.36 ± 2.81 1.0 (0/8) | 1.78 ± 2.07 1.0 (0/8) | 1.57 ± 2.26 1.0 (0/8) |
Male (n = 21) | 5.8 ± 4.13 5.0 (0/14) | 2.28 ± 1.55 2.0 (0/5) | 1.23 ± 1.44 1.0 (0/5) | 1.28 ± 1.64 0.0 (0/4) | 1.00 ± 1.34 0.0 (0/4) |
p-Value | 0.226 | 0.083 | 0.307 | 0.405 | 0.573 |
Age | |||||
8–10 yrs (n = 18) | 5.88 ± 5.1 4.5 (0/18) | 2.72 ± 1.90 3.0 (0/8) | 1.66 ± 2.30 0.5 (0/8) | 0.83 ± 1.24 0.0 (0/4) | 0.66 ± 1.23 0.0 (0/4) |
11–14 yrs (n = 22) | 8.68 ± 7.20 8.0 (0/32) | 2.95 ± 1.96 3.0 (0/8) | 1.86 ± 2.25 2.0 (0/8) | 2.09 ± 2.09 2.0 (0/8) | 1.77 ± 2.11 2.0 (0/8) |
p-Value | 0.163 | 0.717 | 0.563 | 0.042 | 0.045 |
Group | COHIP Scores [Mean ± SD, Median, (Range)] * | |||||
---|---|---|---|---|---|---|
Overall COHIP | Oral Symptoms | Functional Well-Being | Social- Emotional Well-Being | School Environment | Peer Interaction | |
Gender | ||||||
Female (n = 19) | 106.21± 14.68 110.0 (82/135) | 30.00 ± 6.61 31.0 (15/39) | 17.89 ± 4.70 18.0 (9/24) | 26.63 ± 4.58 28.0 (13/32) | 14.94 ± 1.54 16.0 (12/16) | 16.73 ± 5.98 17.0 (6/24) |
Male (n = 21) | 110.33 ± 10.82 111.0 (88/129) | 30.3 ± 5.45 30.0 (18/38) | 19.24 ± 3.18 20.0 (11/24) | 28.29 ± 4.09 29.0 (19/32) | 14.90 ± 1.37 16.0 (12/16) | 17.57 ± 4.31 18.0 (9/24) |
p-Value | 0.376 | 1.000 | 0.145 | 0.169 | 0.810 | 0.768 |
Age | ||||||
8–10 yrs (n = 18) | 111.44 ± 11.90 113.5 (82/129) | 30.05 ± 6.23 30.5 (15/38) | 18.72 ± 3.22 19.5 (9/23) | 28.11 ± 3.22 28.5 (21/32) | 15.11 ± 1.40 16.0 (12/16) | 19.44 ± 4.21 20.0 (12/24) |
11–14 yrs (n = 22) | 105.86 ± 13.22 109.0 (82/135) | 30.27 ± 5.86 30.5 (18/39) | 18.5 ± 3.81 19.0 (11/24) | 27.0 ± 5.16 28.5 (13/32) | 14.77 ± 1.47 15.5 (12/16) | 15.31 ± 5.13 15.0 (6/24) |
p-Value | 0.100 | 1.000 | 0.798 | 0.840 | 0.476 | 0.011 |
BONE DYSPLASIA | OHIP-14 | SF-CPQ | COHIP * |
---|---|---|---|
OI (n = 24) | 5.87 ± 5.75 4.0 (0/20) | 6.83 ± 7.09 5.5 (0/32) | 110.21 ± 13.28 112.50 (82/135) |
XLH (n = 5) | 4.80 ± 6.42 2.0 (0/16) | 11.6 ± 8.29 12.0 (4/24) | 105.20 ± 13.10 110.00 (82/114) |
MAS (n = 2) | 0.00 ± 0.00 0.0 (0.0/0.0) | 3.5 ± 0.71 3.5 (3/4) | 109.50 ± 0.71 109.50 (109/110) |
FD (n = 3) | 1.00 ± 1.00 1.0 (0/2) | 7.00 ± 2.65 8.0 (4/9) | 109.67 ± 11.93 106.00 (100/123) |
PHP (n = 6) | 10.83 ± 10.26 12.0 (0/22) | 7.83 ± 2.64 8.0 (4/11) | 102.67 ± 14.36 101.50 (84/124) |
Disclaimer/Publisher’s Note: The statements, opinions and data contained in all publications are solely those of the individual author(s) and contributor(s) and not of MDPI and/or the editor(s). MDPI and/or the editor(s) disclaim responsibility for any injury to people or property resulting from any ideas, methods, instructions or products referred to in the content. |
© 2024 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
Share and Cite
Defabianis, P.; Ninivaggi, R.; Tessaris, D.; Bocca, N.; Romano, F. Oral Health-Related Quality of Life in Italian Children and Adolescents Living with Bone Dysplasia: A Cross-Sectional Study. Int. J. Environ. Res. Public Health 2024, 21, 306. https://doi.org/10.3390/ijerph21030306
Defabianis P, Ninivaggi R, Tessaris D, Bocca N, Romano F. Oral Health-Related Quality of Life in Italian Children and Adolescents Living with Bone Dysplasia: A Cross-Sectional Study. International Journal of Environmental Research and Public Health. 2024; 21(3):306. https://doi.org/10.3390/ijerph21030306
Chicago/Turabian StyleDefabianis, Patrizia, Rossella Ninivaggi, Daniele Tessaris, Norma Bocca, and Federica Romano. 2024. "Oral Health-Related Quality of Life in Italian Children and Adolescents Living with Bone Dysplasia: A Cross-Sectional Study" International Journal of Environmental Research and Public Health 21, no. 3: 306. https://doi.org/10.3390/ijerph21030306
APA StyleDefabianis, P., Ninivaggi, R., Tessaris, D., Bocca, N., & Romano, F. (2024). Oral Health-Related Quality of Life in Italian Children and Adolescents Living with Bone Dysplasia: A Cross-Sectional Study. International Journal of Environmental Research and Public Health, 21(3), 306. https://doi.org/10.3390/ijerph21030306