대한약학회:학술대회논문집 (Proceedings of the PSK Conference)
- 대한약학회 2002년도 Proceedings of the Convention of the Pharmaceutical Society of Korea Vol.2
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- Pages.431.2-432
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- 2002
Quality of Life in Pediatric Patients with Mucopolysaccharidosis
- Na, Jong-Cheon (Graduate School of Clinical Pharmacy, Sookmyung University) ;
- Jin, Dong-Kyu (Samsung Medical Center) ;
- Kwon, Eun-Kyung (Samsung Medical Center) ;
- Lee, Suk-Hyang (Samsung Medical Center.)
- 발행 : 2002.10.01
초록
Mucopolysaccharidosis (MPS) is a genetic disorder with deficiency of Iysomal enzymes needed for the degradation of glycosaminoglycans(GAGs). This storage disease is characterized by intra-lysosomal accumulation of GAGs. progressive mental and physical deterioration. multi-organ failure and premature death. Quality of life (QOL) is very low in MPS patients. The MOS 36-ltem Short Form Health Survey (SF-36) was designed to measure the eight (8) dimensions of health in clinical and general population settings. (omitted)
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