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Oral Management of Hereditary Sensory and Autonomic Neuropathy Type V: A Rare Case Report
Abstract
Raja Belgacem, Yamina Elelmi, Imed Ouni, Farah chouchène and Hichem Ghedira
Hereditary sensory and autonomic neuropathies (HSAN) are rare diseases. Five different types are described. Hereditary sensory and autonomic neuropathy type V, also known as congenital insensitivity to pain, is a rare autosomal recessive disease seen in early childhood. Self-mutilation is an invariable feature of this disorder involving the teeth, tongue, lips, and fingers. This report described the case of a 2-year-old baby boy who had self-mutilating injuries to her tongue and hands, caused by biting. Protective splints were given to the patient to prevent further self-mutilation.