{"title":"牙科方法儿科患者先天性疼痛不敏感和无汗症(CIPA)综合征:一个病例报告","authors":"Uğur Akdağ , Kamile Nur Tozar","doi":"10.1016/j.pdj.2024.11.006","DOIUrl":null,"url":null,"abstract":"<div><div>Congenital pain insensitivity syndrome with anhidrosis (CIPA, MIM <span><span>256800</span><svg><path></path></svg></span>) or hereditary sensory and autonomic neuropathy (HSAN) type IV is a rare autosomal recessive disease. CIPA has characteristic symptoms such as failure to respond to painful stimuli, lack of thermal sensitivity, decreased or absent sweating (anhidrosis), varying degrees of reduced intellectual disability, musculoskeletal fractures, and joint deformities. A 4-year-old female patient diagnosed with CIPA syndrome presented to our clinic with the complaint of self-harm with her anterior teeth. In the clinical examination, sores were detected on the child's hands, fingers, mouth, and tongue. The front teeth of the patient, which caused the trauma, were extracted. In the 6-month follow-up, the patient's wounds healed. In this case report, the treatment of a patient with CIPA syndrome who was severely self-harming is described.</div></div>","PeriodicalId":19977,"journal":{"name":"Pediatric Dental Journal","volume":"35 1","pages":"Article 100335"},"PeriodicalIF":0.6000,"publicationDate":"2024-12-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Dental approach to a pediatric patient with congenital insensitivity to pain and anhidrosis (CIPA) syndrome: A case report\",\"authors\":\"Uğur Akdağ , Kamile Nur Tozar\",\"doi\":\"10.1016/j.pdj.2024.11.006\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<div><div>Congenital pain insensitivity syndrome with anhidrosis (CIPA, MIM <span><span>256800</span><svg><path></path></svg></span>) or hereditary sensory and autonomic neuropathy (HSAN) type IV is a rare autosomal recessive disease. CIPA has characteristic symptoms such as failure to respond to painful stimuli, lack of thermal sensitivity, decreased or absent sweating (anhidrosis), varying degrees of reduced intellectual disability, musculoskeletal fractures, and joint deformities. A 4-year-old female patient diagnosed with CIPA syndrome presented to our clinic with the complaint of self-harm with her anterior teeth. In the clinical examination, sores were detected on the child's hands, fingers, mouth, and tongue. The front teeth of the patient, which caused the trauma, were extracted. In the 6-month follow-up, the patient's wounds healed. In this case report, the treatment of a patient with CIPA syndrome who was severely self-harming is described.</div></div>\",\"PeriodicalId\":19977,\"journal\":{\"name\":\"Pediatric Dental Journal\",\"volume\":\"35 1\",\"pages\":\"Article 100335\"},\"PeriodicalIF\":0.6000,\"publicationDate\":\"2024-12-14\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Pediatric Dental Journal\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://www.sciencedirect.com/science/article/pii/S0917239424000454\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q4\",\"JCRName\":\"DENTISTRY, ORAL SURGERY & MEDICINE\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Pediatric Dental Journal","FirstCategoryId":"1085","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S0917239424000454","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"DENTISTRY, ORAL SURGERY & MEDICINE","Score":null,"Total":0}
Dental approach to a pediatric patient with congenital insensitivity to pain and anhidrosis (CIPA) syndrome: A case report
Congenital pain insensitivity syndrome with anhidrosis (CIPA, MIM 256800) or hereditary sensory and autonomic neuropathy (HSAN) type IV is a rare autosomal recessive disease. CIPA has characteristic symptoms such as failure to respond to painful stimuli, lack of thermal sensitivity, decreased or absent sweating (anhidrosis), varying degrees of reduced intellectual disability, musculoskeletal fractures, and joint deformities. A 4-year-old female patient diagnosed with CIPA syndrome presented to our clinic with the complaint of self-harm with her anterior teeth. In the clinical examination, sores were detected on the child's hands, fingers, mouth, and tongue. The front teeth of the patient, which caused the trauma, were extracted. In the 6-month follow-up, the patient's wounds healed. In this case report, the treatment of a patient with CIPA syndrome who was severely self-harming is described.