Figure 2From: Pathognomonic oral profile of Enamel Renal Syndrome (ERS) caused by recessive FAM20A mutationsTooth phenotype of ERS patients. (A, B) Oral and radiograph view show intra pulpal calcification and absence of enamel (C) H&E staining of a sagittal section from an extracted deciduous molar. (D) Magnified boxed area from C showing intra-pulpal calcification (black arrow). (E) Magnified view of the surface of the tooth presented in C showing a sparse thin hypoplastic enamel layer. (F) Sagittal section of a deciduous molar observed via optical microscopy (zoom ×840). Note normal tubular dentine, columnar arrangements close to the dentine indicating enamel rods contrasting with a generally disorganized enamel surface. Note the abnormal dentino-enamel junction which is less scalloped than normal (dashed line). (G) Magnified view of the area in F with scanning electron microscopy (zoom ×900). Note absence of normal enamel rod architecture and increased porosity in the enamel. (H) Magnified view of box in F (zoom ×10,000). e: enamel, d: dentin, p: dental pulp, DEJ: Dentin-enamel junction (dashed line).Back to article page