Unlabelled: The general features of Neurofibromatosis-1 are outlined and the oral and maxillofacial features described. Two case reports of patients treated in the Department of Sedation and Special Care Dentistry at King's College London Dental Institute are presented, and the dental management of patients with Neurofibromatosis discussed.
Clinical Relevance: Neurofibromatosis-1 is a neurocutaneous genetic disease with a prevalence of one in 4000-5000. Dentists may be presented with these patients in their surgery and this article will help them to understand the condition and explain how to manage these patients.
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Neurofibromatosis (NF) is an autosomal genetic disorder with three types, including NF1, NF2, and schwannomatosis. It is characterized by bulging and deforming masses arising from multiple nerves involving skin folds and connective tissues. Prompt diagnosis and provision of care for NF1 patients by clinicians aware of the diverse clinical features of this disorder are needed for optimum patient care and management.
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Neurofibromatosis type 1 (NF1) conveys significant disease morbidity and lower quality of life compared to the general population. Research has shown that decreased positive health outcomes are directly correlated with inadequate development of health-related self-management skills among similar patient populations, and among these populations a healthcare transition (HCT) intervention improves provision of care and health outcomes. Thus, HCT intervention may improve care and outcomes in NF1.
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Children and adolescents with Neurofibromatosis type 1 (NF1) are at increased risk for wide-ranging behavioral, developmental, and cognitive impairments and decreased quality of life. To date, no psychosocial screening tool has been developed to quickly assess the symptoms that 1) can be addressed during routine medical appointments in children with NF1, 2) can produce interpretable and actionable results, 3) can be integrated into medical care, and 4) can quickly identify patients at risk in order to better address that the provision of appropriate care are available. This study was conducted to test the overall usability of the Distress Thermometer (DT) and symptom checklist and concordance of DT ratings between pediatric patients, their caregivers and medical providers.
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Lifelong health monitoring is recommended in neurofibromatosis type 1 (NF1) because of the progressive and unpredictable range of disabling and potentially life-threatening symptoms that arise. In Australia, strategies for NF1 health surveillance are less well developed for adults than they are for children, resulting in inequalities between pediatric and adult care. The aims of this study were to determine the uptake of health monitoring and capacity of adults with NF1 to self-manage their health.
View Article and Find Full Text PDFNeurofibromatosis-1 and the provision of dental care.
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Oxfordshire Salaried Primary Dental Care Service, East Oxford Dental Access Centre, Manzil Way, Oxford.
Unlabelled: The general features of Neurofibromatosis-1 are outlined and the oral and maxillofacial features described. Two case reports of patients treated in the Department of Sedation and Special Care Dentistry at King's College London Dental Institute are presented, and the dental management of patients with Neurofibromatosis discussed.
Clinical Relevance: Neurofibromatosis-1 is a neurocutaneous genetic disease with a prevalence of one in 4000-5000.
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