Mass E, Sarnat H, Ram D, Gadoth N
Department of Pediatric Dentistry, Maurice and Gabriela Goldschleger School of Dental Medicine, Tel Aviv University, Israel.
Oral Surg Oral Med Oral Pathol. 1992 Sep;74(3):305-11. doi: 10.1016/0030-4220(92)90064-w.
Familial dysautonomia is an inherited autosomal recessive disease found almost exclusively in Ashkenazi Jews. It is characterized by selective damage to the sensory, motor, and autonomic peripheral nervous system. The main clinical features include decreased pain sensation, impaired temperature and blood pressure regulation, lack of tearing, absent tendon reflexes, and fungiform papillae on the tongue. The purpose of this study was to explore in depth and to verify the oral and dental status in familial dysautonomia. Twenty-two patients and 44 match-paired healthy persons of Ashkenazi descent were examined. Patients and parents had only little concern for their oral condition. Caries prevalence was lower than normal and plaque accumulation increased in all patients. Dental trauma was found in 59% of the patients, and 32% showed orodental self-mutilation. Dental age was within normal range, and dental arch measurements implied proportionally small jaws and little crowding. The low caries rate may be related to the known "hypersalivation" in familial dysautonomia and/or a possible change in the salivary composition and content, caused by chronic autonomic denervation.
家族性自主神经功能障碍是一种几乎仅在阿什肯纳兹犹太人中发现的常染色体隐性遗传病。其特征是感觉、运动和自主神经外周神经系统受到选择性损伤。主要临床特征包括痛觉减退、体温和血压调节受损、无泪、腱反射消失以及舌上蕈状乳头缺失。本研究的目的是深入探究并验证家族性自主神经功能障碍患者的口腔和牙齿状况。对22名患者和44名配对的阿什肯纳兹血统健康人进行了检查。患者及其父母对自身口腔状况几乎没有关注。龋齿患病率低于正常水平，所有患者的牙菌斑积聚均有所增加。59%的患者存在牙齿外伤，32%的患者表现出口腔自残行为。牙齿年龄在正常范围内，牙弓测量表明颌骨比例较小且拥挤程度较低。低龋齿率可能与家族性自主神经功能障碍中已知的“流涎过多”和/或慢性自主神经去神经支配导致的唾液成分和含量可能变化有关。
