范可尼贫血的口腔和牙齿表现

IF 0.3 Q3 MEDICINE, GENERAL & INTERNAL Galician Medical Journal Pub Date : 2021-09-01 DOI:10.21802/gmj.2021.3.2
V. Ambarkova
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引用次数: 1

摘要

范科尼贫血是一种罕见的疾病,其特征是所有类型的血细胞产生减少。范科尼贫血是再生障碍性贫血最常见的遗传形式。先天性眼睛、耳朵和心脏异常,肾脏畸形或缺失,泌尿生殖系统受累是常见的。身体发育迟缓。范科尼贫血患者的智力通常是正常的。与范科尼贫血相关的最严重的问题包括骨髓疾病的逐渐发展。许多范科尼贫血患者会发展为白血病或骨髓增生异常综合征,以及其他肿瘤学疾病。范可尼贫血患者的口腔表现可分为牙龈炎、牙周炎、龋齿、牙齿异常、软组织病变、口腔癌症和舌头病变。凡科尼贫血患者易患头颈部鳞状细胞癌和口腔癌症。由医学和牙科专家组成的跨学科团队必须参与Fanconi贫血患者的医学和牙科治疗。为了对范科尼贫血患者进行适当的牙科护理,牙科专家的密切合作尤其重要,包括正畸医生、儿童牙科医生、修复师、口腔外科医生以及牙周病和口腔疾病专家。
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Oral and Dental Manifestations of Fanconi Anemia
Fanconi anemia is a rare disease, which is characterized by decreased production of all blood cell types. Fanconi anemia is the most common inherited form of aplastic anemia. Congenital abnormalities of the eyes, ears, and heart, malformed or absent kidney, urogenital system involvement are common. There is a delay in physical development. Intelligence in patients with Fanconi anemia is usually normal. The most serious problems associated with Fanconi anemia include the gradual development of bone marrow disorders. Many patients with Fanconi anemia develop leukemia or myelodysplastic syndrome, as well as other oncological diseases. Oral manifestations in patients with Fanconi anemia can be classified as gingivitis, periodontitis, dental caries, dental anomalies, soft tissue lesions, oral cancer, and lesions of the tongue. Patients with Fanconi anemia have increased predisposition to squamous cell carcinoma of the head and neck and oral cancer. The interdisciplinary team of medical and dental specialists must be included in the medical and dental treatment of patients with Fanconi anemia. For proper dental care of patients with Fanconi anemia, the close cooperation of dental specialists, including orthodontists, pedodontists, prosthetists, oral surgeons, as well as specialists in periodontology and oral diseases, is of particular importance.
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