Colomba Besa-Witto, Ana Ortega-Pinto, Sebastián Véliz, Marco Cornejo, Ignacia Fuentes, Francis Palisson, Susanne Krämer
{"title":"大疱性结缔组织表皮松解所致无淀粉性不全患者冠吸收的发生率。","authors":"Colomba Besa-Witto, Ana Ortega-Pinto, Sebastián Véliz, Marco Cornejo, Ignacia Fuentes, Francis Palisson, Susanne Krämer","doi":"10.1111/odi.15250","DOIUrl":null,"url":null,"abstract":"<p><strong>Introduction: </strong>Junctional epidermolysis bullosa (JEB) is a rare genetic disease manifesting with skin and mucosal blistering. As part of the JEB, patients present with syndromic amelogenesis imperfecta (AI). Reports have described external crown resorption (ECR) in the teeth of patients with JEB, but its prevalence is unknown.</p><p><strong>Objective: </strong>To determine the prevalence of ECR in patients with JEB.</p><p><strong>Methods: </strong>A longitudinal retrospective cohort study was performed at the Special Care Dentistry Clinic, University of Chile. Clinical records of patients with JEB between 2005 and 2024 were analysed. Prevalence of ECR per patient, per type of tooth and per tooth was calculated.</p><p><strong>Results: </strong>Of the 20 patients examined, 90% presented ECR in at least one tooth, with an average of 4.8 primary and 6.8 permanent teeth affected. The most affected type of teeth were the incisors. 57.5% of primary incisors and 68% of permanent incisors had resorption. The most affected tooth was #82 in primary dentition (75%) and #32 in the permanent dentition (88.9%).</p><p><strong>Conclusions: </strong>The prevalence of ECR in patients with AI due to JEB caused by variants in LAMB3 was 90%. Establishing clinical and radiographic dental protocols for the early detection of ECR is essential to prevent extensive tooth destruction.</p>","PeriodicalId":19615,"journal":{"name":"Oral diseases","volume":" ","pages":""},"PeriodicalIF":2.9000,"publicationDate":"2025-01-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Prevalence of Crown Resorption in Amelogenesis Imperfecta due to Junctional Epidermolysis Bullosa.\",\"authors\":\"Colomba Besa-Witto, Ana Ortega-Pinto, Sebastián Véliz, Marco Cornejo, Ignacia Fuentes, Francis Palisson, Susanne Krämer\",\"doi\":\"10.1111/odi.15250\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Introduction: </strong>Junctional epidermolysis bullosa (JEB) is a rare genetic disease manifesting with skin and mucosal blistering. As part of the JEB, patients present with syndromic amelogenesis imperfecta (AI). Reports have described external crown resorption (ECR) in the teeth of patients with JEB, but its prevalence is unknown.</p><p><strong>Objective: </strong>To determine the prevalence of ECR in patients with JEB.</p><p><strong>Methods: </strong>A longitudinal retrospective cohort study was performed at the Special Care Dentistry Clinic, University of Chile. Clinical records of patients with JEB between 2005 and 2024 were analysed. Prevalence of ECR per patient, per type of tooth and per tooth was calculated.</p><p><strong>Results: </strong>Of the 20 patients examined, 90% presented ECR in at least one tooth, with an average of 4.8 primary and 6.8 permanent teeth affected. The most affected type of teeth were the incisors. 57.5% of primary incisors and 68% of permanent incisors had resorption. The most affected tooth was #82 in primary dentition (75%) and #32 in the permanent dentition (88.9%).</p><p><strong>Conclusions: </strong>The prevalence of ECR in patients with AI due to JEB caused by variants in LAMB3 was 90%. Establishing clinical and radiographic dental protocols for the early detection of ECR is essential to prevent extensive tooth destruction.</p>\",\"PeriodicalId\":19615,\"journal\":{\"name\":\"Oral diseases\",\"volume\":\" \",\"pages\":\"\"},\"PeriodicalIF\":2.9000,\"publicationDate\":\"2025-01-08\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Oral diseases\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1111/odi.15250\",\"RegionNum\":3,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q1\",\"JCRName\":\"DENTISTRY, ORAL SURGERY & MEDICINE\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Oral diseases","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1111/odi.15250","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q1","JCRName":"DENTISTRY, ORAL SURGERY & MEDICINE","Score":null,"Total":0}
Prevalence of Crown Resorption in Amelogenesis Imperfecta due to Junctional Epidermolysis Bullosa.
Introduction: Junctional epidermolysis bullosa (JEB) is a rare genetic disease manifesting with skin and mucosal blistering. As part of the JEB, patients present with syndromic amelogenesis imperfecta (AI). Reports have described external crown resorption (ECR) in the teeth of patients with JEB, but its prevalence is unknown.
Objective: To determine the prevalence of ECR in patients with JEB.
Methods: A longitudinal retrospective cohort study was performed at the Special Care Dentistry Clinic, University of Chile. Clinical records of patients with JEB between 2005 and 2024 were analysed. Prevalence of ECR per patient, per type of tooth and per tooth was calculated.
Results: Of the 20 patients examined, 90% presented ECR in at least one tooth, with an average of 4.8 primary and 6.8 permanent teeth affected. The most affected type of teeth were the incisors. 57.5% of primary incisors and 68% of permanent incisors had resorption. The most affected tooth was #82 in primary dentition (75%) and #32 in the permanent dentition (88.9%).
Conclusions: The prevalence of ECR in patients with AI due to JEB caused by variants in LAMB3 was 90%. Establishing clinical and radiographic dental protocols for the early detection of ECR is essential to prevent extensive tooth destruction.
期刊介绍：
Oral Diseases is a multidisciplinary and international journal with a focus on head and neck disorders, edited by leaders in the field, Professor Giovanni Lodi (Editor-in-Chief, Milan, Italy), Professor Stefano Petti (Deputy Editor, Rome, Italy) and Associate Professor Gulshan Sunavala-Dossabhoy (Deputy Editor, Shreveport, LA, USA). The journal is pre-eminent in oral medicine. Oral Diseases specifically strives to link often-isolated areas of dentistry and medicine through broad-based scholarship that includes well-designed and controlled clinical research, analytical epidemiology, and the translation of basic science in pre-clinical studies. The journal typically publishes articles relevant to many related medical specialties including especially dermatology, gastroenterology, hematology, immunology, infectious diseases, neuropsychiatry, oncology and otolaryngology. The essential requirement is that all submitted research is hypothesis-driven, with significant positive and negative results both welcomed. Equal publication emphasis is placed on etiology, pathogenesis, diagnosis, prevention and treatment.
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