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http://dx.doi.org/10.5933/JKAPD.2019.46.4.416

Dental Management in a Patient with Congenital Insensitivity to Pain with Anhidrosis : A Case Report  

Bae, Cheolhyeon (Department of Pediatric Dentistry, Jeonbuk National University Dental Hospital)
Lee, Daewoo (Department of Pediatric Dentistry, Jeonbuk National University Dental Hospital)
Kim, Jaegon (Department of Pediatric Dentistry, Jeonbuk National University Dental Hospital)
Yang, Yeonmi (Department of Pediatric Dentistry, Jeonbuk National University Dental Hospital)
Publication Information
Journal of the korean academy of Pediatric Dentistry / v.46, no.4, 2019 , pp. 416-421 More about this Journal
Abstract
Congenital insensitivity to pain with anhidrosis (CIPA) is a rare, autosomal recessive disorder; affected patients are characterized by inability to feel pain and to sweat over the entire body, as well as by mental retardation. Because, in the oral examination, no specific findings on soft or hard tissue may be found except possible lesions due to self-mutilation, early recognition and diagnosis are essential for these patients. Pediatric dentists must be aware of the clinical manifestations and treatment considerations related to uncontrolled body temperature, tactile hyperesthesia and lack of pain reflex. In this case report, dental management of CIPA was suggested by presenting a 6-year follow-up of young patient.
Keywords
CIPA; Pain insensitivity; Anhidrosis; Self-mutilation;
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1 Langer J, Goebel HH, Veit S : Eccrine sweat glands are not innervated in hereditary sensory neuropathy type IV. An electron-microscopic study. Acta Neuropathol , 54:199-202, 1981.   DOI
2 Indo Y : Genetics of congenital insensitivity to pain with anhidrosis (CIPA) or hereditary sensory and autonomic neuropathy type IV. Clinical, biological and molecular aspects of mutations in TRKA(NTRK1) gene encoding the receptor tyrosine kinase for nerve growth factor. Clin Auton Res , 12:I20-I32, 2002.   DOI
3 Swanson AG : Congenital insensitivity to pain with anhydrosis. A unique syndrome in two male siblings. Arch Neurol , 8:299-306, 1963.   DOI
4 Pinsky L, DiGeorge AM : Congenital familial sensory neuropathy with anhidrosis. J Pediatr, 68:1-13, 1966.   DOI
5 Haga N, Kubota M, Miwa Z : Epidemiology of hereditary sensory and autonomic neuropathy type IV and V in Japan. Am J Med Genet A , 161:871-874, 2013.   DOI
6 Shatzky S, Moses S, Parvari R, et al.: Congenital insensitivity to pain with anhidrosis (CIPA) in Israeli-Bedouins: genetic heterogeneity, novel mutations in the TrkA/NGF receptor gene, clinical findings, and results of nerve conduction studies. Am J Med Genet, 92:353-360, 2000.   DOI
7 Indo Y, Tsuruta M, Matsuda I, et al.: Mutations in the TRKA/NGF receptor gene in patients with congenital insensitivity to pain with anhidrosis. Nat Genet, 13:485-488, 1996.   DOI
8 Indo Y : Nerve growth factor and the physiology of pain: lessons from congenital insensitivity to pain with anhidrosis. Clin Genet, 82:341-350, 2012.   DOI
9 Bodner L, Woldenberg Y, Pinsk V, Levy J : Orofacial manifestations of congenital insensitivity to pain with anhidrosis: a report of 24 cases. ASDC J Dent Child, 69:293-296, 2002.
10 Kouvelas N, Terzoglou C : Congenital insensitivity to pain with anhidrosis: case report. Pediatr Dent , 11:47-51, 1989.
11 Limeres J, Feijoo JF, Diz P, et al.: Oral self-injury: an update. Dent Traumatol, 29:8-14, 2013.   DOI
12 Nguyen KV, Naviaux RK, Nyhan WL : Novel mutation in the human HPRT1 gene and the Lesch-Nyhan disease. Nucleosides Nucleotides Nucleic Acids , 36:704-711, 2017.   DOI
13 Dyck PJ, Mellinger JF, Lambert EH, et al.: Not 'indifference to pain' but varieties of hereditary sensory and autonomic neuropathy. Brain , 106:373-390, 1983.   DOI
14 Rotthier A, Baets J, Timmerman V, Janssens K : Mechanisms of disease in hereditary sensory and autonomic neuropathies. Nat Rev Neurol , 8:73-85, 2012.   DOI
15 Harris JC : Lesch-Nyhan syndrome and its variants: examining the behavioral and neurocognitive phenotype. Curr Opin Psychiatry , 31:96-102, 2018.   DOI
16 Tomioka T, Awaya Y, Hanaoka K, et al.: Anesthesia for patients with congenital insensitivity to pain and anhidrosis: a questionnaire study in Japan. Anesth Analg , 94:271-274, 2002.   DOI
17 Zlotnik A, Natanel D, Bodner L, et al.: Anesthetic management of patients with congenital insensitivity to pain with anhidrosis: a retrospective analysis of 358 procedures performed under general anesthesia. Anesth Analg , 121:1316- 1320, 2015.   DOI
18 Rozentsveig V, Katz A, Gurman GM, et al.: The anaesthetic management of patients with congenital insensitivity to pain with anhidrosis. Paediatr Anaesth , 14:344-348, 2004.   DOI
19 Rosemberg S, Marie SK, Kliemann S : Congenital insensitivity to pain with anhidrosis (hereditary sensory and autonomic neuropathy type IV). Pediatr Neurol, 11:50-56, 1994.   DOI