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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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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

Park, Hye-Sook;Kim, Kwuy-Bun
- Child Health Nursing Research
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- v.17 no.1
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- pp.58-67
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- 2011
Purpose: This study was done to explore the meaning of joys and sorrows in mothers of children with Mucopolysaccharidosis (MPS). Methods: The participants were 9 mothers who each had a child with MPS. Data were gathered using in-depth interviews. The interviews were recorded and transcribed verbatim. Data were analyzed using Colaizzi method. Results: The significant results from analyzing the interviews can be grouped into 12 concept descriptions, and 5 theme clusters. The essential theme clusters for the experience of the mothers were 'hopelessness about this rare disease', 'guilt because of the disease being hereditary, 'endless courses of treatment', 'wounds in the relationship', and 'relative composure of mind'. Conclusion: The finding of this study offer profound information on joys and sorrows experienced by mothers of children with MPS and provide basic data for developing nursing intervention strategies for the mothers of children with rare diseases.
https://doi.org/10.4094/jkachn.2011.17.1.58 인용 PDF KSCI