Autor: |
Nagaveni N, Suma R, Shashikiran N, Subba Reddy V |
Jazyk: |
angličtina |
Rok vydání: |
2008 |
Předmět: |
|
Zdroj: |
Journal of Indian Society of Pedodontics and Preventive Dentistry, Vol 26, Iss 2, Pp 78-81 (2008) |
Druh dokumentu: |
article |
ISSN: |
0970-4388 |
Popis: |
Papillon-Lefevre syndrome is a very rare syndrome of autosomal recessive inheritance characterized by palmar-plantar hyperkeratosis and early onset of a severe destructive periodontitis, leading to premature loss of both primary and permanent dentitions. Various etiopathogenic factors are associated with the syndrome but a recent report has suggested that the condition is linked to mutations of the cathepsin C gene. Two cases of Papillon-Lefevre syndrome in the same family, having all of the characteristic features, are presented. An 11-year-old girl and a 9-year-old boy presented with the complaints of loose teeth. Both expressed hyperkeratosis of palms, soles, and knees. Severe generalized periodontal destruction, with mobility of teeth, was evident on intraoral examination; orthopantomograph examination showed severe generalized loss of alveolar bone in both the patients. |
Databáze: |
Directory of Open Access Journals |
Externí odkaz: |
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