In:
Journal of Medicine and Life, S.C. JURNALUL PENTRU MEDICINA SI VIATA S.R.L, Vol. 15, No. 4 ( 2022-4), p. 579-586
Kurzfassung:
Mucopolysaccharidosis VI is a genetic disorder affecting multiple organs with sundry clinical presentations. The main etiological factor reflects the disturbances in mucopolysaccharide metabolism leading to deposition of acid mucopolysaccharide in various tissues. The pathognomonic features of the disease include a large head, short neck, corneal opacity, open mouth associated with an enlarged tongue, enlargement of the skull, and long anteroposterior dimension with unerupted dentition, dentigerous cyst-like follicles, condylar defects, and gingival hyperplasia. An 18-year-old boy with Maroteaux-Lamy syndrome (mucopolysaccharidosis type VI) is described in this article, emphasizing the oral manifestations and radiographic illustration of lesions in the jaws. It also emphasizes the essential role of cone-beam computed tomography to identify and analyze multicentric pathologies in the jaws.
Materialart:
Online-Ressource
ISSN:
1844-122X
,
1844-3117
DOI:
10.25122/jml-2021-0288
Sprache:
Englisch
Verlag:
S.C. JURNALUL PENTRU MEDICINA SI VIATA S.R.L
Publikationsdatum:
2022
ZDB Id:
2559353-5