Dental management of a patient with hemophagocytic lymphohistiocytosis under general anesthesia: A case report
신현지, 김현태, 송지수, 신터전, 김영재, 김정욱, 장기택, 현홍근,
소속 상세정보
신현지 ( Shin Hyun-Ji ) - Seoul National University School of Dentistry Department of Pediatric Dentistry
김현태 ( Kim Hyun-Tae ) - Seoul National University School of Dentistry Department of Pediatric Dentistry
송지수 ( Song Ji-Soo ) - Seoul National University School of Dentistry Department of Pediatric Dentistry
신터전 ( Shin Teo-Jeon ) - Seoul National University School of Dentistry Department of Pediatric Dentistry
김영재 ( Kim Young-Jae ) - Seoul National University School of Dentistry Department of Pediatric Dentistry
김정욱 ( Kim Jung-Wook ) - Seoul National University School of Dentistry Department of Pediatric Dentistry
장기택 ( Jang Ki-Taeg ) - Seoul National University School of Dentistry Department of Pediatric Dentistry
현홍근 ( Hyun Hong-Keun ) - Seoul National University School of Dentistry Department of Pediatric Dentistry
Abstract
Hemophagocytic lymphohistiocytosis (HLH) is an aggressive and life-threatening syndrome of excessive immune activation. It is characterized by mutations in genes encoding the signaling system related to immune system inactivation such as the PRF1 gene encoding perforin. HLH-2004, a treatment protocol designed for patients with HLH, is an immuno-cancer chemotherapy based on administration of etoposide and early administration of immunosuppressants. Reported oral manifestations of chemotherapy are microdontia and hypodontia, and recognized oral complications due to immunosuppressive therapies are mucositis, salivary gland dysfunction, and gingival overgrowth. In this report, we describe the dental treatment of a child with HLH under general anesthesia, who presented with multiple caries in primary and permanent teeth and also seemed to have hypodontia and microdontia of permanent teeth. Because of a prescription for the immunosuppressant prednisolone, the patient was given hydrocortisone before and during general anesthesia. The patient was treated by extraction and composite resin restoration. Intensive oral hygiene such as plaque control, diet consultation, and topical fluoride application with periodic panorama x-ray are essential for oral health to reduce the risk of dental caries in patients with HLH. To understand the medical status of patients and avoid the side effects of a drug, dentists should properly communicate with pediatricians.
키워드
Hemophagocytic lymphohistiocytosis; Immuno-cancer chemotherapy; Dental treatment
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