Oral Comfort Therapy For Children With Cipa

Congenital painless anhidrosis(CIPA) is a rare autosomal recessive genetic disease with less than 60 cases worldwide. This paper discusses the necessity of multidisciplinary collaboration through oral comfort treatment of a 10-year-old child with congenital painless anhidrosis. The patient had a complex history, including mental retardation, recurrent high fever, history of self-harm, and joint dislocation. The oral examination revealed that several teeth needed treatment, and finally pulp protection and minimally invasive extraction were performed under general anesthesia. During the anesthesia process, the temperature was strictly controlled, and the oral midazolam solution was 0.5mg/Kg (Yichang Renfu Pharmaceutical Co., LTD.), propofol (1mg/Kg) was injected intravitously (Xi 'an Libon Pharmaceutical Co., LTD.), and 3% sevoflurane was inhaled to maintain anesthesia (Hubei Xinkang Pharmaceutical Chemical Co., LTD.). After the operation, the patient went home safely without complications. 1. Psychological medicine and humanistic care can alleviate the anxiety of children; 2. Body temperature control is related to physiological disciplines to prevent the risk of high fever; 3. The use of narcotic drugs requires attention to the differences in drug metabolism of patients with rare diseases and their effects on autonomic nervous system, which involves multidisciplinary studies such as pharmacology and neurology. The treatment of rare diseases needs the collaboration of stomatology, anesthesiology, psychology and other disciplines. In the future, interdisciplinary research should be strengthened to optimize the treatment plan and provide reference for other rare diseases.