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Hurler syndrome: a case report

  • S.Thomas1
  • S.Tandon1,*,

1Department of Pedodontics and Preventive Dentistry, College of Dental Surgery, Manipal 5761 19, India

DOI: 10.17796/jcpd.24.4.ku653u75nv5vt735 Vol.24,Issue 4,July 2000 pp.335-338

Published: 01 July 2000

*Corresponding Author(s): S.Tandon E-mail: none

Abstract

Hurler syndrome is an inherited disorder of mucopolysaceharide metabolism, which is caused by a defect in genetically controlled pathways of lysosomal degradation. It represents the classical prototype of mucopolysaccharide disorder. An interesting case of a three and a half-year old boy with a rare combination of skeletal, neurological, ophthalmologic, and dental findings is presented. It is a rare syndrome with a very low prevalence of 1:100,000 births and as such the clinician should be aware of this syndrome.


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S.Thomas,S.Tandon. Hurler syndrome: a case report. Journal of Clinical Pediatric Dentistry. 2000. 24(4);335-338.

References

1. Scott HS, Litiens T, Hopwood JJ, Morris CP. A common mutation for mucopolysaccharidosis type I associated with a severe Hurler syndrome phenotype. Hum Hut 1: 103-108, 1992.

2. Jones JH, Mason DK. Oral manifestations of systemic disease. WB Saunders, London, 1st edition, 37, 1980.

3. Lowry RB, Renwick DHG. Relative frequency of the Hurler and Hunter syndrome. N Engl J Med 284: 221-222, 1971. Cited in Gorlin RJ, Pindborg JJ, Cohen MM. Syndromes of the head and neck. McGraw Hill Company, USA, 2nd edition, 476, 1976.

4. Nelson WE. Textbook of Pediatrics. WB Saunders Company, Philadelphia, 15th edition, 398-399, 1996.

5. Silveri CP, Kaplan FS, Fallon MD, Bayever E, August CS. Hurler syndrome with special reference to histologic abnormalities of the growth plate. Clinc Orthop 69: 305-311, 1991.

6. Gorlin RJ, Pindborg JJ, Cohen MM. Syndromes of the head and neck. McGraw Hill Company, USA, 2nd edition, 476, 1976.

7. Gardner D.G. Metachromatic cells in the gingiva in Hurler’s syndrome. Oral Surgery 26: 782-789, 1968. Cited in Gorlin RJ, Pindborg JJ, Cohen MM. Syndromes of the head and neck. McGraw Hill Company, USA, 2ndedition, 476, 1976.

8. Bhaskar SN. Synopsis of oral pathology, CV Mosby, St. Louis, 5th edition, 625, 1977.

9. Whitley CB, Belani KG, Chang PN, Summers CG, Blazar BR, Tsai MY, et al. Long term outcome of Hurler syndrome following bone marrow transplantation. Am J Med Genetics 46: 209-218, 1993.

10. Neville BW, Damm DD, Allen CM, Bouquot JE. Oral and maxillofacial pathology. WB Saunders Co, Philadelphia, 1st edition, 595, 1995.


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