ORAL MANIFESTATIONS AND PROSTHETIC REHABILITATION IN HEREDITARY SENSORY AND AUTONOMIC NEUROPATHY (HSAN)TYPE IV:A CASE REPORT*

Autor: Elif Yaman, Zeynep Aytepe, Nazlı Altin, Elif Ince, Duygu Ofluoglu, Hakkı Tanyeri
Jazyk: angličtina
Rok vydání: 2016
Předmět:
Zdroj: Journal of Istanbul University Faculty of Dentistry, Vol 50, Iss 2, Pp 49-53 (2016)
Volume: 50, Issue: 2 49-53
Journal of Istanbul University Faculty of Dentistry
ISSN: 2149-4592
2149-2352
Popis: Hereditary sensory and autonomic neuropathies (HSAN) are rare genetic syndromes of unknown etiology. They are seen in early childhood and are categorized into six different types by their symptoms. HSAN type 4 demonstrates autosomal recessive transmission pattern, with such major characteristics as loss of sense of pain, self-mutilation, anhydrosis and mental retardation. Sympathetic innervations are deficient despite the existence of sweat glands. Sufferers are hypotonic without any tendon reflexes, and neuro-motor development is retarded. In some cases tactile sensation and vibration may be intact. Biting injuries due to lack of pain sensation cause laceration, ulceration and scarring of the tongue, lips and other parts of oral mucosa. Tooth luxation and severe dental attrition have been observed. This case report presents oral and dental findings, surgical treatments and prosthetic rehabilitation of an 11- year-old boy with HSAN type 4.
Databáze: OpenAIRE