Melnick M, Eastman J R, Goldblatt L I, Michaud M, Bixler D
Oral Surg Oral Med Oral Pathol. 1977 Oct;44(4):592-9. doi: 10.1016/0030-4220(77)90303-6.
Dentin dysplasia, Type II, is a rare autosomal dominant disorder. The primary teeth are amber and translucent and the pulp chambers are obliterated. The permanent teeth have a normal to brown-gray coloration and a thistle-tube pulp configuration with multiple true denticles. To date, only five families with this disorder have been reported. This article presents two additional families. Light and scanning electron microscopy of an affected primary incisor showed the dentin, including the mantle layer, to be highly disorganized throughout. Possible pathogenic events associated with the phenotype are discussed.
II型牙本质发育异常是一种罕见的常染色体显性疾病。乳牙呈琥珀色且半透明，牙髓腔闭塞。恒牙颜色正常至棕灰色，牙髓呈蓟管状结构并伴有多个真性牙本质小体。迄今为止，仅有五个患有该疾病的家族被报道。本文介绍另外两个家族。对一颗受累乳牙进行光镜和扫描电镜检查显示，包括罩牙本质层在内的牙本质整体高度紊乱。文中讨论了与该表型相关的可能致病因素。
