KMID : 1144020220180020048
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Journal of Korean Association for Disability and Oral Health 2022 Volume.18 No. 2 p.48 ~ p.54
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Dental management of a patient with hemophagocytic lymphohistiocytosis under general anesthesia: A case report
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Shin Hyun-Ji
Kim Hyun-Tae Song Ji-Soo Shin Teo-Jeon Kim Young-Jae Kim Jung-Wook Jang Ki-Taeg Hyun Hong-Keun
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Abstract
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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.
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KEYWORD
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Hemophagocytic lymphohistiocytosis, Immuno-cancer chemotherapy, Dental treatment
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