• Title/Summary/Keyword: 뮤코다당증

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Experience of Patients with Mucopolysaccharide Receiving Enzyme Replacement Therapy (효소보충치료(ERT)를 받고 있는 뮤코다당증 환자의 경험)

  • Kwon, Eun Kyung;Jung, So-Young;Choi, Eu Jin;Cho, Sung Yoon;Jin, Dong Kyu
    • Journal of Industrial Convergence
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    • v.19 no.4
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    • pp.37-48
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    • 2021

  • The purpose of this study was to explore the meaning of the experience of Mucopolysaccharidosis patients receiving Enzyme Replacement Therapy(ERT). A qualitative research design was adopted. The participants were 7 patients diagnosed with MPS who is receiving ERT for several years. Data were collected through in-depth interviews from 1 July 2018 to 22 July 2018 for seven adult patients with MPS receiving ERT. Individual interviews were recorded, and transcribed data were analyzed using the inductive method of content analysis. The final 4 categories and 15 subcategories were identified. The 4 categories of the experience of Mucopolysaccharidosis patients receiving ERT were "Physical and psychological changes through ERT", "Continuous tunnel called ERT", "Accepting the reality" and "Cautious hope". This study provides deep insight into the experience of Mucopolysaccharidosis patients receiving ERT. Medical staff including nurses and related organizations should concern their distress during ERT as well as physical symptoms.

  • https://doi.org/10.22678/JIC.2021.19.4.037 인용 PDF

GINGIVAL HYPERPLASIA IN A MUCOPOLYSACCHARIDOSIS' PATIENT : A CASE REPORT (뮤코다당증(Mucopolysaccharidosis)환아의 치은 증식)

  • Song, Ju-Hyun;Jang, Chul-Ho;Kim, Young-Jae;Hahn, Se-Hyun;Lee, Sang-Hoon
    • Journal of the korean academy of Pediatric Dentistry
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    • v.34 no.1
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    • pp.150-155
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    • 2007

  • Mucopolysaccharidosis (MPS) is a disorder of storage in which there is excessive accumulation of glycosaminoglycans (GAGs) from lysosomal enzyme defect. Lysosomal accumulation of GAGs eventually results in cell, tissue and organ dysfunction. This patient may manifest mental retardation and physical disorders. This clinical report presents a girl with MPS having severe gingival hyperplasia. Gingivectomy was performed under general anesthesia. The pediatric dentist must be aware of oral manifestations present in the MPS. The approach to dental management will require teamwork between the dentist and the patient's physician.

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DENTAL MANAGEMENT OF PATIENT WITH HUNTER SYNDROME (MUCOPOLYSACCHARIDOSIS TYPE II) : A CASE REPORT (Hunter 증후군 환아의 치과적 관리: 증례보고)

  • Lee, Min-Jeong;Kim, Jae-Gon;Yang, Yeon-Mi;Baik, Byeong-Ju;Song, Hee-Jeong
    • Journal of the korean academy of Pediatric Dentistry
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    • v.39 no.4
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    • pp.412-417
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    • 2012

  • Mucopolysaccharidosis (MPS) is a disorder which is caused by the defect of the lysosomal enzyme that is essentially needed for resolution of glycosaminoglycans (GAGs). Metabolite of GAGs will accumulate in the lysosome of cells and will result in the dysfunction of cells, tissues, and organs. Eventually, patients will manifest both mental retardation and physical disorders. In worst cases, mucopolysaccharidosis can cause premature death. The current clinical types have been classified as MPS from type I to type IX according to the defect of certain enzyme. The dental complications have been reported as delay of eruption, enamel hypoplasia, microdontia, malocclusion, condylar defects, gingival hyperplasia and dentigerous cystlike follicle. This clinical report presents the case of a boy with MPS type II, Hunter Syndrome which has various dental complications.

  • https://doi.org/10.5933/JKAPD.2012.39.4.412 인용 PDF KSCI