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Biomedical subjects

L S Levin

Publications and source records attributed to L S Levin.

9 recordsLinked to original sources

Adenomatoid odontogenic tumor: ultrastructural demonstration of two cell types and amyloid.

A typical adenomatoid odontogenic tumor removed from a 13-year-old female was studied by light and electron microscopy. The tumor was composed of two types of epithelial cells: Type I cells were cuboidal and occurred in nests or formed ductlike structures and Type II cells were smaller and spindle shaped. The formation of extracellular masses of amyloid was found in association with Type I epithelial cells, and amyloid formation was not observed in association with Type II cells. Results suggest that the lesion is of enamel organ origin, derived from cells of the inner enamel epithelium at the pre-ameloblastic stage, stellate reticulum and stratum intermedium. The origin of this amyloid material is unknown; however, it may be of enamel protein origin which, like amyloid, may have a beta-protein conformation.

Adolescent

The Rieger syndrome.

Fourteen patients with hypodontia and the ocular features of the Rieger syndrome were examined for the presence of systemic anomalies. A periumbilical defect that consisted of failure of the periumbilical skin to involute was seen in ten of the thirteen evaluated for the defect. Three others had scars over the umbilical area and had a history of surgery for herniation. In addition, four males in one family and one male from another family had hypospadias. None of several other anomalies reported to be components of the Rieger syndrome by other authors was detected in the fourteen patients. The mode of inheritance in the familial cases studied was compatible with autosomal dominance. The results of this study indicate that the Rieger syndrome is an autosomal dominant syndrome whose cardinal features are hypodontia, goniodysgenesis, and failure of the periumbilical skin to involute properly.

Abnormalities, Multiple

Hearing patterns in Morquio's syndrome (mucopolysaccharidosis IV).

The hearing status of 18 patients with Morquio's syndrome (mucopolysaccharidosis IV) was evaluated. All three patients under age 8 years had a conductive hearing loss. Fourteen of 15 patients 8 years of age and older had a mixed or sensorineural hearing loss; one had normal hearing. Six patients wore hearing aids. By the end of the first decade of life, most persons with Morquio's syndrome may be expected to have either a mixed or sensorineural hearing loss. Amplification may be required.

Adolescent

A postaxial polydactyly-dental-vertebral syndrome.

Three patients with postaxial polydactyly and other abnormalities of the hands and feet, hypoplasia and fusion of the vertebral bodies, and dental abnormalities are reported. Two were sisters born to normal unrelated parents; the other patient was the male offspring of a consanguineous marriage. We suggest that this constellation of abnormalities represents a recessively inherited syndrome.

Abnormalities, Multiple