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Fibrous dysplasia is a non‐neoplastic developmental process that affects the craniofacial bones, characterized by painless enlargement as a result of bone substitution by abnormal fibrous tissue. Postzygotic somatic activating mutations in the GNAS1 gene cause fibrous dysplasia and have been extensively investigated, as well as being helpful in the differential diagnosis of the disease. Fibrous dysplasia may involve one (monostotic) or multiple bones (polyostotic), sporadically or in association with McCune‐Albright syndrome, Jeffe‐Lichenstein syndrome, or Mazabreud syndrome. This review summarizes the current knowledge on fibrous dysplasia, emphasizing the value of integrating the understanding of its molecular pathogenesis with the clinical, radiological, and histopathological features. In addition, we address important aspects related to the differential diagnosis and patient management.  相似文献   
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Background. A persistent patent ductus arteriosus (PDA) may delay closure of a coexisting atrial septal defect (ASD) due to volume loading and enlargement of the left atrium. The purpose of this study was to investigate the natural history of ASD size in patients with a PDA following transcatheter PDA occlusion. Methods. All patients with an ASD and a PDA who underwent transcatheter PDA occlusion at Texas Children’s Hospital were identified. Patients with ASD diameter <3 mm, or additional cardiac defects were excluded. Eight patients (7 females) with small‐ to moderate‐sized ASDs and a PDA were identified. Patient demographics, echocardiographic data, and cardiac catheterization data were recorded. Data were analyzed by 1‐tailed t‐test. Results. Following PDA occlusion, ASD diameter decreased in 6 of 8 patients by a mean of 3.8 mm (±2.3 mm), including 2 that closed. The median duration of follow‐up was 689 days. One ASD remained unchanged and 1 increased in size. The mean maximum ASD diameter decreased from 6.4 mm (±2.2 mm) to 3.9 mm (±3.4 mm) (P = .03). Two patients underwent subsequent transcatheter ASD occlusion. Conclusion. Following transcatheter PDA occlusion, small‐ to moderate‐sized ASDs have significant probability to decrease in size, and possibly close. In infants and children, we recommend transcatheter PDA occlusion, and serial follow‐up of the size of the ASD. This will allow many small‐ to moderate‐sized ASDs to either close, or become smaller, obviating the need for future intervention.  相似文献   
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BACKGROUND: Oral hairy leukoplakia (OHL) may be an indicator of the progression of Human Immunodeficiency Virus (HIV)-induced immuno-depression, and the evaluation of risk factors leading to OHL is important in the management of these HIV-infected patients. However, there are few studies that analyze risk factors leading to OHL in the Brazilian population. The aim of this case-control study is to present data about prevalence rates and risk factors leading to OHL in a sample of HIV-infected adults in Brazil. METHODS: This case-control study included 111 HIV-infected patients treated at a clinic for sexually transmitted diseases and HIV. In the initial examinations with dentists, variables were collected from all patients. Diagnosis of OHL was performed in accordance with the International Classification System and cytological features. The Fisher and the chi-squared tests were used for statistical analysis. The proportional prevalence and odds ratio were estimated. RESULTS: Outcome presented a positive, statistically significant association among the presence of OHL and viral load of 3000 copies/mul or greater (P = 0.0001; odds ratio (OR) = 5.8), presence of oral candidiasis (P = 0.0000; OR = 11.1), previous use of fluconazole (P = 0.0000; OR = 24.6), and use of systemic acyclovir (P = 0.032; OR = 4.3). Antiretroviral medication presented a negative, statistically significant association with the presence of OHL (P = 0.002; OR = 8.4). CONCLUSIONS: Prevalence of OHL was 28.8%. Viral load, oral candidiasis, previous use of fluconazole, and systemic acyclovir were determined to be risk factors for OHL. Antiretroviral medication proved to be protective against the development of OHL.  相似文献   
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