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Congenital Insensitivity to Pain with Anhidrosis: A Case with Self-Inflicted Oral Ulcerations

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Congenital insensitivity to pain with anhidrosis (CIPA), also known as hereditary sensory and autonomic neuropathy type IV, is a rare autosomal recessive condition. CIPA is caused by mutations in the NTRK1 gene, leading to the inability to feel pain and decreased or absent sweating (anhidrosis). The signs and symptoms of CIPA may not be easily diagnosed at birth, but repeated severe injuries or unintentional self-injurious behavior during infancy may prompt further investigation leading to a CIPA diagnosis. We present the case of an 18-month old child who was diagnosed with CIPA, after repeated visits to a hopsital's emergency department for serious tongue and finger biting, which prompted further investigation.
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Document Type: Case Report

Affiliations: 1: Dr. Soussou is an attending pediatric dentist, British Columbia Children's Hospital and a clinical assistant professor, Department of Oral and Health Sciences, Faculty of Dentistry, University of British Columbia, Canada 2: Dr. Cheung is interim chief of Dentistry, British Columbia Children's Hospital and clinical associate professor, Department of Oral and Health Sciences, Faculty of Dentistry, University of British Columbia, both in Vancouver, Canada;, Email: [email protected] 3: Dr. Campbell is an associate professor and director, Graduate Program in Pediatric Dentistry, Faculty of Dentistry, University of Toronto, Canada

Publication date: May 1, 2019

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  • Acquired after the merger between the American Society of Dentistry for Children and the American Academy of Pediatric Dentistry in 2002, the Journal of Dentistry for Children (JDC) is an internationally renowned journal whose publishing dates back to 1934. Published three times a year, JDC promotes the practice, education and research specifically related to the specialty of pediatric dentistry. It covers a wide range of topics related to the clinical care of children, from clinical techniques of daily importance to the practitioner, to studies on child behavior and growth and development. JDC also provides information on the physical, psychological and emotional conditions of children as they relate to and affect their dental health.
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