Santhosh B P, Jethmalani Preeti
Reader, Department of Pedodontics and Preventive Dentistry Narsinhbhai Patel Dental College and Hospital, Visnagar, Gujarat India, e-mail:
J Contemp Dent Pract. 2013 May 1;14(3):544-7. doi: 10.5005/jp-journals-10024-1359.
Oral rehabilitation of a child with Johanson-Blizzard syndrome (JBS).
JBS is an extremely rare inherited disorder characterized by unusually small nose that appears 'beak shaped' due to the absence (aplasia) or underdevelopment (hypoplasia) of the nostrils (nasal alae), abnormally small, malformed primary (deciduous) teeth and misshapen or absent secondary (permanent) teeth, hearing disorder, hypothyroidism, dwarfism, malabsorption and mental retardation. It is sometimes described as a form of ectodermal dysplasia.
Oral findings in JBS are very obscure in the literature. The present report describes oral findings in an 8 years old boy with JBS and his oral rehabilitation.
JBS has an emotional consequence for the affected individuals at early ages. Oral rehabilitation in this case had a very positive impact on the child's mind.
Early identification and treatment of this disease is of great importance to rehabilitate the patient on functional, esthetic and psychological front.
对一名患有约汉森-布莱兹德综合征（JBS）的儿童进行口腔修复。
JBS是一种极为罕见的遗传性疾病，其特征为鼻子异常小，由于鼻孔（鼻翼）缺失（发育不全）或发育不良（发育不全）而呈“喙状”，乳牙异常小且畸形，恒牙畸形或缺失，听力障碍，甲状腺功能减退，侏儒症，吸收不良和智力迟钝。它有时被描述为外胚层发育不良的一种形式。
JBS的口腔表现在文献中非常模糊。本报告描述了一名患有JBS的8岁男孩的口腔表现及其口腔修复情况。
JBS在早期对受影响个体有情感方面的影响。该病例的口腔修复对孩子的心理有非常积极的影响。
早期识别和治疗这种疾病对于在功能、美学和心理方面使患者康复非常重要。
