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Journals / Journal of Istanbul University Faculty of Dentistry / 2016 / Cilt: 50 - Sayı: 1

Herediter Duyusal ve Otonomik Nöropati Tip IV Hastasının Oral Bulguları ve Protetik Tedavileri: Bir Olgu Nedeniyle

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

Elif YAMAN
Hakkı TANYERİ
Zeynep AYTEPE
Nazlı ALTIN
Duygu OFLUOĞLU
Elif Bahar TUNA-İNCE
Pages
49–53
Publication Date
2022-10-18
DOI
—
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AI Summary & Key Takeaways

AI Insight
⚡ TL;DR(Too Long; Didn't Read)

This case report presents oral and dental findings, surgical treatments and prosthetic rehabilitation of an 11year-old boy with HSAN type 4

📌 3 Key Takeaways

🎯
Objective & Hypothesis:Hereditary sensory and autonomic neuropathies (HSAN) are rare genetic syndromes of unknown etiology.
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Key Findings & Data:HSAN type 4 demonstrates autosomal recessive transmission pattern, with such major characteristics as loss of sense of pain, self-mutilation, anhydrosis and mental retardation.
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Conclusion & Impact:This case report presents oral and dental findings, surgical treatments and prosthetic rehabilitation of an 11year-old boy with HSAN type 4
🔬Design:Research Article
📍Scope:Comprehensive Sample
⚙️Variables:Domain Parameters

Abstract

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 11year-old boy with HSAN type 4

Özet

Herediter duyusal ve otonomik nöropatiler, etyolojileri bilinmeyen, nadir görülen genetik sendromlardır. Genellikle erken çocukluk döneminde görülürler ve semptomlarına göre 6 farklı tipi tanımlanmıştır. Otozomal çekinik geçiş gösterirler ve ağrı hissinin kaybolması, kendine zarar verme, terlemenin azalması ve mental gerilik karakteristik bulgularıdır. Ter bezleri mevcut olmasına rağmen sempatik inervasyon yetersizdir. Hastalar tendon refleksleri olmaksızın hipotoniklerdir ve nöro-motor gelişimde gerilik vardır, bazı vakalarda dokunma ve titreşim hissi zarar görmemiş olabilir. Ağrı hissinin olmaması nedeniyle ısırma sonucu dilde, dudaklarda ve oral mukozanın diğer bölgelerinde laserasyon, ülserasyon ve skar oluşumu görülebilir. Dişlerde sallanmalar ve şiddetli atrisyonlar oluşabilir. Bu vaka raporunda, HSAN Tip 4 teşhisi konmuş olan, 11 yaşındaki erkek hastanın oral ve dental bulguları, cerrahi ve protetik tedavileri sunulmuştur

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Cite This Article

YAMAN, E., TANYERİ, H., AYTEPE, Z., ALTIN, N., OFLUOĞLU, D., TUNA-İNCE, E. B. (2022). Herediter Duyusal ve Otonomik Nöropati Tip IV Hastasının Oral Bulguları ve Protetik Tedavileri: Bir Olgu Nedeniyle. Journal of Istanbul University Faculty of Dentistry, Cilt: 50 - Sayı: 1, 49–53. https://ajindex.com/articles/563794

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