1. Enamel-renal syndrome in 2 patients with a mutation in FAM20 A and atypical hypertrichosis and hearing loss phenotypes 
Sabina Pena B. Pêgo, DDS, PhD, Ricardo D. Coletta, DDS, PhD, Simona Dumitriu, MD, PhD, Daniela Iancu, MD, PhD, Saleh Albanyan, MD, Robert Kleta, MD, PhD, Maria Teresa Auricchio, PhD, Luis Antônio Santos, DDS, PhD, Breno Rocha, DDS, Hercílio Martelli-Júnior, DDS, PhD 
Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology 
Volume 123, Issue 2, Pages e2 (February 2017)
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2. Fig. 1
Clinical features of proband. A, Intraoral photographs of the proband. Teeth exhibited severe enamel hypoplasia, and the gingiva showed generalized but mild hyperplasia, which was of normal color and fibrous consistency. B, Panoramic radiography showed mixed dentition with generalized enamel hypoplasia, intrapulpal calcifications, and unerupted teeth with pericoronal radiolucent areas. C, Hypertrichosis was observed as thick eyebrows and on the forehead, lateral portions of the face, arms, and back close to the sacral region. D, Ultrasound revealed nephrocalcinosis involving both kidneys.
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3. Fig. 2
Pedigree of family affected with enamal-renal syndrome (ERS), showing an autosomal recessive trait. Affected individuals are indicated by blackened symbols. Circles denote females and squares denote males, a slash through a symbol denotes a deceased individual, and the arrow indicates the proband.
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4. Fig. 3
Sequencing chromatograms of FAM20 A exon 2. This homozygous nonsense mutation, charcaterized by the c.406 C>T transition in both alleles of FAM20 A, caused a premature termination at p.Arg136*. Electropherogram represents an unaffected family member (control) in (A) and the index case in (B).
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5. Fig. 4
Histologic features of the gingival overgrowth. Numerous concentric calcified particles in a psammomatoid pattern in close contact with the odontogenic epithelial rests were distributed in the dense fibrous connective tissue. (A, original magnification ×100, and B, original magnification ×200.)
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6. Fig. S1
Sagital non-enhanced computed tomography of the right ear, bone window, showing normal appearance of the vestibule and semicircular canals.
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7. Fig. S2
Axial non-enhanced computed tomography of the right ear, bone window, showing normoaereted mastoid cells and middle ear cavity. Middle ear ossicles and inner ear structures such as cochlea, vestibule and semicircular canals show no detectable abnormalities.
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8. Fig. S3
Axial non-enhanced computed tomography of the left ear, bone window, showing normoaereted mastoid cells and middle ear cavity. Middle ear ossicles and inner ear structures such as cochlea, vestibule and semicircular canals show no detectable abnormalities.
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9. Fig. S4
Coronal non-enhanced computed tomography, bone window. Notice normal configuration of inner ear structures.
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