Literature DB >> 9828617

Morquio's syndrome. A case report and review of clinical findings.

J Fitzgerald, S J Verveniotis.   

Abstract

Morquio's syndrome is a genetic disorder caused by the lack of the enzyme responsible for the breakdown of the mucopolysaccharide keratin sulfate. The resulting buildup of keratin sulfate causes a specific pattern of abnormalities, including short stature, spinal deformity, cardiac abnormalities, corneal opacities, deafness and dental abnormalities. The specific dental findings in the patient described here are as follows: the posterior teeth were tapered and had pointed. cusps; the maxillary anterior teeth were spaced and flared, and on radiographs, the enamel was less than 25 percent of normal thickness but of normal radiodensity. Despite these findings, the patient exhibited a low rate of caries. This paper is a review of medical and dental findings in the Morquio's patient and how this may affect dental management.

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Year:  1998        PMID: 9828617

Source DB:  PubMed          Journal:  N Y State Dent J        ISSN: 0028-7571


  5 in total

Review 1.  Review of clinical presentation and diagnosis of mucopolysaccharidosis IVA.

Authors:  C J Hendriksz; P Harmatz; M Beck; S Jones; T Wood; R Lachman; C G Gravance; T Orii; S Tomatsu
Journal:  Mol Genet Metab       Date:  2013-04-10       Impact factor: 4.797

Review 2.  Physiopathological function of hematoside (GM3 ganglioside).

Authors:  Jin-ichi Inokuchi
Journal:  Proc Jpn Acad Ser B Phys Biol Sci       Date:  2011       Impact factor: 3.493

3.  Scintigraphic features of Morquio's syndrome: a case report.

Authors:  Bjoern Kitzing; Kevin C Allman
Journal:  J Med Case Rep       Date:  2011-01-28

4.  Morquio's Syndrome: A Case Report of Two Siblings.

Authors:  Sathish Muthukumar Ramalingam; Daya Srinivasan; Sandhya ArunKumar; Joe Louis ChiriyanKandath; Sriram Kaliamoorthy
Journal:  Case Rep Dent       Date:  2017-01-16

5.  Audiometric evaluation in individuals with mucopolysaccharidosis.

Authors:  Marcela Rosana Maia da Silveira; Ana Karina Lima Buriti; Ana Maria Martins; Daniela Gil; Marisa Frasson de Azevedo
Journal:  Clinics (Sao Paulo)       Date:  2018-12-03       Impact factor: 2.365

  5 in total

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