Obliterated pulp cavities in the Sanfilippo syndrome (mucopolysaccharidosis III)

doi:10.1016/0030-4220(77)90058-5

Oral Surgery, Oral Medicine, Oral Pathology

Volume 43, Issue 5, May 1977, Pages 734-738

Oral Surgery, Oral M...

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Abstract

Generalized obliteration of the pulp chambers and canals by irregular secondary dentin was observed in an individual with the Sanfilippo syndrome. Heretofore unreported, this dental finding may represent a manifestation of this mucopolysaccharide disorder.

References (11)

V.A. McKusick
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R.A. Cawson
The Oral Changes in Gargoylism
D.G. Gardner
The Oral Manifestations of Hurler's Syndrome
Oral Surg
(1971)

There are more references available in the full text version of this article.

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Occurrence of multiple dentigerous cysts in a patient with the Maroteaux-Lamy syndrome (mucopolysaccharidosis, type VI)
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The mucopolysaccharidoses (MPS) are a group of genetic lysosomal storage diseases. These diseases result from a defect in specific lysosomal enzymes required for the degradation of specific mucopolysaccharides. These incompletely degraded saccharides accumulate in tissues and are excreted in the urine. A general characteristic of these diseases is dysostosis multiplex. Dental complications can be severe and include unerupted dentition, dentigerous cystlike follicles, malocclusions, condylar defects, and gingival hyperplasia. This report examines multiple dentigerous cysts in a patient with a deficiency in N-acetylgalactosamine-4-sulfatase, Maroteaux-Lamy syndrome (MPS VI). The inability to hydrolyze the sulfate group from N-acetylgalactosamine-4-sulfate residue of dermatan sulfate due to a deficiency in this enzyme results in the accumulation of dermatan sulfate in tissues and its excretion in the urine. Examination of dentigerous cyst fluid revealed glycosaminoglycan content of 397 μg per milliliter. Compositional analyses revealed 60% hyaluronic acid, 30% chondroitin 4- and -6-sulfate, and only 10% dermatan sulfate. This was consistent with dentigerous cyst fluid derived from persons without mucopolysaccharide-storage disorders but distinctly different from glycosaminoglycans assayed from other body fluids of this patient.
Clinical, genetic, and histologic features of the trichoonychodental (TOD) syndrome
1978, Oral Surgery, Oral Medicine, Oral Pathology
A syndrome of scanty, fine, curled hair, thin dysplastic nails, taurodontic molars, hypoplastic-hypomature enamel, dysplasia of dentin, and hypohidrosis segregating as an autosomal dominant trait is described in a Japanese family. The patients received repeated doses of tetracycline during odontogenesis which fortuitously assisted in interpreting the pathogenesis of the dentin defect. The condition was previously described as familial congenital ectodermal dysplasia by Westerholm¹ and as odontogenesis imperfecta by Siirilä and Heikinheimo.² It is suggested that the name trichoonychodental (TOD) syndrome is a better descriptive term for the condition.
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Short communication & case reportObliterated pulp cavities in the Sanfilippo syndrome (mucopolysaccharidosis III)

Abstract

Heritable Diseases of Connective Tissue

The Face in Genetic Disorders

Clinical Definition of Hurler-Hunter Prototypes

Am. J. Dis. Child

The Oral Changes in Gargoylism

The Oral Manifestations of Hurler's Syndrome

Oral Surg

Short communication & case report
Obliterated pulp cavities in the Sanfilippo syndrome (mucopolysaccharidosis III)