title: Dental manifestations of a pediatric patient with hyperimmunoglobulin e syndrome: a case report.
abstract: A 7-year-old girl with hyperimmunoglobulin E syndrome (HIES) presented to our clinic with tooth agenesis in both primary and permanent dentitions. The patient's serum immunoglobulin E level was elevated at 17,091 IU/ml, and her medical history indicated the occurrence of HIES, numerous  skin abscesses, and recurrent infection by bacteria and/or fungi such as Candida from birth. She also suffered from heart disease. Dental manifestations included extensive caries, impaired root resorption of primary mandibular central incisors and absence of primary mandibular canines and  permanent mandibular lateral incisors. Intraoral phenotypes in HIES patients have already been reported in detail, but no previous report has described abnormalities in the number of primary teeth in HIES patients. The purpose of this report was to describe the dental manifestations in the  primary dentition of a hyperimmunoglobulin E syndrome patient and to emphasize the importance of preventive dental management from early childhood in compromised children, such as those with HIES.
authors: Department of Pediatric Dentistry, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan. ichiro@nagasaki-u.ac.jp
authors: Yoko
authors: Y
authors: Kamasaki
authors: Kiyoshi
authors: K
authors: Hidaka
authors: Miyuki
authors: M
authors: Nishiguchi
authors: Taku
authors: T
authors: Fujiwara
mesh_terms: D002648:Child; D003731:Dental Caries / Q000209:etiology* / Q000628:therapy*; D005260:Female; D006801:Humans; D016867:Immunocompromised Host; D007589:Job Syndrome / Q000150:complications*; D010641:Phenotype; D011862:Radiography, Panoramic; D012391:Root Resorption; D014071:Tooth Abnormalities / Q000209:etiology* / Q000628:therapy*; D014081:Tooth Extraction