Nordgarden H, Johannessen S, Storhaug K, Jensen J L
Department of Oral Surgery and Oral Medicine, Faculty of Dentistry, Oslo, Norway.
Oral Dis. 1998 Jun;4(2):152-4. doi: 10.1111/j.1601-0825.1998.tb00270.x.
Ectodermal dysplasias (EDs) are a group of developmental disorders (more than 100) mainly affecting ectodermal tissues and organs. The X-linked hypohidrotic ED (HED) is the most common form of EDs, involving defects in teeth, sweat glands, and hair. In a few reports, HED has been associated with reduced salivary function. In the present case report, a dramatically reduced salivary fluid and acidic proline rich protein production was identified in a 38-year-old man with HED. Computed tomography was performed, revealing that one submandibular gland and both parotid glands were hypoplastic, whereas the right submandibular gland seemed to be absent. These findings are in line with a general developmental disturbance also involving the salivary glands. As salivary tests are inexpensive and easy to perform, it is suggested to routinely evaluate salivary secretion in persons with HED, to prevent a possible negative impact on oral health.
外胚层发育不良（EDs）是一组主要影响外胚层组织和器官的发育障碍（超过100种）。X连锁隐性少汗型外胚层发育不良（HED）是最常见的EDs形式，涉及牙齿、汗腺和毛发缺陷。在一些报告中，HED与唾液功能降低有关。在本病例报告中，一名38岁的HED男性被发现唾液分泌量显著减少且富含脯氨酸的酸性蛋白生成减少。进行了计算机断层扫描，结果显示一侧下颌下腺和双侧腮腺发育不全，而右侧下颌下腺似乎缺失。这些发现与涉及唾液腺的一般发育障碍一致。由于唾液检测成本低廉且易于操作，建议对HED患者常规评估唾液分泌，以预防对口腔健康可能产生的负面影响。
