Hereditary sensory and autosomal peripheral neuropathy-type IV: case series and review of literature
Hereditary sensory and autonomic neuropathy (HSAN) IV is a rare autosomal recessive disorder which is characterized by a decrease in the number of myelinated and non-myelinated nerve fibers of peripheral nerves which causes diminished or absent pain sensation leading to increase in self-mutilative h...
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| Veröffentlicht in: | Oral and maxillofacial surgery 2015-06, Vol.19 (2), p.117-123 |
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| creator | Ashwin, D. P. Chandan, G. D. Jasleen, Handa Kaur Rajkumar, G. C. Rudresh, K. B. Prashanth, R. |
| description | Hereditary sensory and autonomic neuropathy (HSAN) IV is a rare autosomal recessive disorder which is characterized by a decrease in the number of myelinated and non-myelinated nerve fibers of peripheral nerves which causes diminished or absent pain sensation leading to increase in self-mutilative habits. A retrospective study of eight cases ranging from age group of 4 to 17 years for oral and digital signs and symptoms is presented. All the patients showed congenital insensitivity to pain and anhidrosis. Oral self-mutilations, such as autoextraction of teeth and severe bite injuries (with resultant scarring) of the finger tips and oral soft tissues (tongue, lip, and buccal mucosa) were found in most patients. Our study suggests that early diagnosis and specific treatment plan are important for prevention of characteristic of the oral as well as digital trauma associated with this disorder. |
| doi_str_mv | 10.1007/s10006-015-0486-5 |
| format | Article |
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All the patients showed congenital insensitivity to pain and anhidrosis. Oral self-mutilations, such as autoextraction of teeth and severe bite injuries (with resultant scarring) of the finger tips and oral soft tissues (tongue, lip, and buccal mucosa) were found in most patients. 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A retrospective study of eight cases ranging from age group of 4 to 17 years for oral and digital signs and symptoms is presented. All the patients showed congenital insensitivity to pain and anhidrosis. Oral self-mutilations, such as autoextraction of teeth and severe bite injuries (with resultant scarring) of the finger tips and oral soft tissues (tongue, lip, and buccal mucosa) were found in most patients. Our study suggests that early diagnosis and specific treatment plan are important for prevention of characteristic of the oral as well as digital trauma associated with this disorder.</abstract><cop>Berlin/Heidelberg</cop><pub>Springer Berlin Heidelberg</pub><pmid>25744033</pmid><doi>10.1007/s10006-015-0486-5</doi><tpages>7</tpages></addata></record> |
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| subjects | Adolescent Child Child, Preschool Chromosome Aberrations Dentistry Early Diagnosis Genes, Recessive - genetics Hereditary Sensory and Autonomic Neuropathies - diagnosis Hereditary Sensory and Autonomic Neuropathies - genetics Humans Medicine Medicine & Public Health Oral and Maxillofacial Surgery Pain Insensitivity, Congenital - diagnosis Pain Insensitivity, Congenital - genetics Peripheral Nerves - abnormalities Phenotype Retrospective Studies Review Article Self Mutilation - diagnosis Self Mutilation - genetics Self Mutilation - prevention & control |
| title | Hereditary sensory and autosomal peripheral neuropathy-type IV: case series and review of literature |
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