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Patients with Wilson disease have poorer dental and periodontal health and a have lower oral quality of life than control patients. Patients with a neurological form would also more frequently present limitations in the function of the masticatory apparatus. Systemic treatments for Wilson disease are associated with lesions of the oral mucosa. Analysis of copper level in saliva could testify to the effectiveness of copper depletion in treated patients The main objective is to compare the state of dental health between: patients with Wilson disease in the hepatic form and patients with the neurological form, and a population of controls.
The comparaison between the three groups is based on evaluation criteria wich is CAOD index of caries severity (developed by Klein and Palmer in 1940) counting the number of permanent teeth with caries (evolving to include non-cavitary caries) (C), absent due to caries (A) and filled (O) in an individual. The maximum score is 28 (third molars are not taken into account). The index is obtained from clinical examination and panoramic dental imaging.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| Cases | patients with hepatic form of Wilson disease and those with neurological form |
| |
| controls | control population consulting in routine dental with panoramic dental imaging, outside of an emergency context |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Saliva samples | Other | A saliva sample is added by research for all patients, as well as an oral quality of life questionnaire. For the witnesses, participation also entails the completion of the standardized oral examination (potentially longer than the assessment that would have been carried out as part of the treatment) and the administration of the oral quality of life questionnaire. |
| Measure | Description | Time Frame |
|---|---|---|
| CAOD index of the severity of the caries impairment counting the number of permanent teeth decayed in an individual. The maximum score is 28 (third molars are not taken into account) | The index is obtained from clinical examination and panoramic dental imaging | inclusion visit |
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Inclusion Criteria:
Exclusion Criteria:
Patient benefiting from a legal protection measure
Pregnant or breastfeeding women
Severe psychiatric disorders with behavioral disorders
For cases:
For witnesses:
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The recruitment of Wilson patients will be done in the coordinating center of the CRMR Wilson at the Fondation A de Rothschild Hospital. Control patients matched in age and sex will be identified among patients consulting in the oral medicine department of Charles Foix Hospital. Posters will be placed in the waiting room to inform patients of the existence of the study and promote recruitment.
| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Amélie YAVCHITZ | Contact | 01 48 03 64 54 | ayavhitz@for.paris | |
| Aurélia POUJOIS | Contact | 01 48 30 66 56 | apoujois@for.paris |
| Name | Affiliation | Role |
|---|---|---|
| Marjolaine GOSSET | Hopital Charles Foix | Study Director |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| Hopital Charles Foix | Active, not recruiting | Ivry-sur-Seine | 94200 | France | ||
| Hôpital Charles Foix |
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| ID | Term |
|---|---|
| D006527 | Hepatolenticular Degeneration |
| ID | Term |
|---|---|
| D008107 | Liver Diseases |
| D004066 | Digestive System Diseases |
| D001480 | Basal Ganglia Diseases |
| D001927 | Brain Diseases |
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|
| Questionnaire OHIP-14 | Other | A saliva sample is added by research for all patients, as well as an oral quality of life questionnaire. For the witnesses, participation also entails the completion of the standardized oral examination (potentially longer than the assessment that would have been carried out as part of the treatment) and the administration of the oral quality of life questionnaire. |
|
| Thorough dental examination | Other | A saliva sample is added by research for all patients, as well as an oral quality of life questionnaire. For the witnesses, participation also entails the completion of the standardized oral examination (potentially longer than the assessment that would have been carried out as part of the treatment) and the administration of the oral quality of life questionnaire. |
|
| Active, not recruiting |
| Ivry-sur-Seine |
| 94200 |
| France |
| Fondation Adolphe de Rothschild | Recruiting | Paris | 75019 | France |
|
| D002493 |
| Central Nervous System Diseases |
| D009422 | Nervous System Diseases |
| D020739 | Brain Diseases, Metabolic, Inborn |
| D001928 | Brain Diseases, Metabolic |
| D009069 | Movement Disorders |
| D020271 | Heredodegenerative Disorders, Nervous System |
| D019636 | Neurodegenerative Diseases |
| D030342 | Genetic Diseases, Inborn |
| D009358 | Congenital, Hereditary, and Neonatal Diseases and Abnormalities |
| D008661 | Metabolism, Inborn Errors |
| D008664 | Metal Metabolism, Inborn Errors |
| D008659 | Metabolic Diseases |
| D009750 | Nutritional and Metabolic Diseases |