کد مقاله | کد نشریه | سال انتشار | مقاله انگلیسی | نسخه تمام متن |
---|---|---|---|---|
3170089 | 1199515 | 2006 | 6 صفحه PDF | دانلود رایگان |
عنوان انگلیسی مقاله ISI
The oral manifestations of Maroteaux-Lamy syndrome (mucopolysaccharidosis VI): A case report
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موضوعات مرتبط
علوم پزشکی و سلامت
پزشکی و دندانپزشکی
دندانپزشکی، جراحی دهان و پزشکی
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![عکس صفحه اول مقاله: The oral manifestations of Maroteaux-Lamy syndrome (mucopolysaccharidosis VI): A case report The oral manifestations of Maroteaux-Lamy syndrome (mucopolysaccharidosis VI): A case report](/preview/png/3170089.png)
چکیده انگلیسی
Maroteaux-Lamy syndrome is one of the genetic disorders involving disturbances in mucopolysaccharide metabolism resulting in increased storage of acid mucopolysaccharide in various tissues. The basic defect in Maroteaux-Lamy syndrome is a deficiency of arylsulfatase B, which leads to accumulation of dermatan sulfate in tissues and their urinary excretion. The deposition of mucopolysaccharides leads to a progressive disorder involving multiple organs that often results in death in the second decade of life. This disease, which has several oral and dental manifestations, is first diagnosed on the basis of clinical findings. A large head, short neck, corneal opacity, open mouth associated with an enlarged tongue, enlargement of skull, and a long antero-posterior dimension are the main characteristic features. Dental complications can be severe and include unerupted dentition, dentigerous cystlike follicles, malocclusions, condylar defects, and gingival hyperplasia. An 11-year-old boy with Maroteaux-Lamy syndrome (mucopolysaccharidosis type VI) is described in this article, with special emphasis on the oral manifestations.
ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontology - Volume 101, Issue 5, May 2006, Pages 632-637
Journal: Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology, and Endodontology - Volume 101, Issue 5, May 2006, Pages 632-637
نویسندگان
Ali Rıza DDS, PhD, Mahmut MD, Elif DDS, Nazan Kocatas DDS, PhD, Damla MD, Otto P. PhD, Jan G.M. PhD,