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Survival analysis of spinal muscular atrophy type I.

DC Field Value Language
dc.contributor.author이철-
dc.contributor.author남궁란-
dc.contributor.author박국인-
dc.contributor.author박민수-
dc.contributor.author박현빈-
dc.contributor.author이순민-
dc.contributor.author이진성-
dc.date.accessioned2015-04-23T17:52:09Z-
dc.date.available2015-04-23T17:52:09Z-
dc.date.issued2010-
dc.identifier.issn1738-1061-
dc.identifier.urihttps://ir.ymlib.yonsei.ac.kr/handle/22282913/103291-
dc.description.abstractPURPOSE: The life expectancy of patients with spinal muscular atrophy (SMA) type I is generally considered to be less than 2 years. Recently, with the introduction of proactive treatments, a longer survival and an improved survival rate have been reported. In this study, we analyzed the natural courses and survival statistics of SMA type I patients and compared the clinical characteristics of the patients based on their survival periods. METHODS: We reviewed the medical records of 14 pediatric patients diagnosed with SMA type I during a 9-year period. We examined the demographic and clinical characteristics of these patients, calculated their survival probabilities, and plotted survival curves as on the censoring date, January 1, 2010. We also compared the characteristics of the patients who died before the age of 24 months (early-death, ED group) and those who survived for 24 months or longer (long-survival, LS group). RESULTS: The mean survival time was 22.8+/-2.0 months. The survival probabilities at 6 months, 12 months, 18 months, 24 months, and 30 months were 92.9%, 92.9%, 76.0%, 76.0%, and 65.1%, respectively. Birth weight was the only factor that showed a statistically significant difference between the ED and LS groups (P=0.048). CONCLUSION: In this study, the survival probabilities at 2 years were far greater than expected. Because of the limited number of patients and information in this study, the contribution of improved supportive care on longer survival could not be clarified; this may be elucidated in larger cohort studies-
dc.description.statementOfResponsibilityopen-
dc.format.extent965~970-
dc.languageKorean-
dc.publisher대한소아과학회-
dc.relation.isPartOfKorean Journal of Pediatrics-
dc.rightsCC BY-NC-ND 2.0 KR-
dc.rights.urihttps://creativecommons.org/licenses/by-nc-nd/2.0/kr/-
dc.titleSurvival analysis of spinal muscular atrophy type I.-
dc.typeArticle-
dc.contributor.collegeCollege of Medicine (의과대학)-
dc.contributor.departmentDept. of Pediatrics (소아과학)-
dc.contributor.googleauthorHyun Bin Park-
dc.contributor.googleauthorSoon Min Lee-
dc.contributor.googleauthorJin Sung Lee-
dc.contributor.googleauthorMin Soo Park-
dc.contributor.googleauthorKook In Park-
dc.contributor.googleauthorRan Namgung-
dc.contributor.googleauthorChul Lee-
dc.identifier.doi10.3345/kjp.2010.53.11.965-
dc.admin.authorfalse-
dc.admin.mappingfalse-
dc.contributor.localIdA03227-
dc.contributor.localIdA03253-
dc.contributor.localIdA01241-
dc.contributor.localIdA01438-
dc.contributor.localIdA01468-
dc.contributor.localIdA01738-
dc.contributor.localIdA02905-
dc.relation.journalcodeJ02100-
dc.identifier.eissn2092-7258-
dc.identifier.pmid21218019-
dc.subject.keywordSpinal muscular atrophy-
dc.subject.keywordSurvival-
dc.subject.keywordSurvival analysis-
dc.contributor.alternativeNameLee, Chul-
dc.contributor.alternativeNameNamgung, Ran-
dc.contributor.alternativeNamePark, Kook In-
dc.contributor.alternativeNamePark, Min Soo-
dc.contributor.alternativeNamePark, Hyun Bin-
dc.contributor.alternativeNameLee, Soon Min-
dc.contributor.alternativeNameLee, Jin Sung-
dc.contributor.affiliatedAuthorLee, Jin Sung-
dc.contributor.affiliatedAuthorLee, Chul-
dc.contributor.affiliatedAuthorNamgung, Ran-
dc.contributor.affiliatedAuthorPark, Kook In-
dc.contributor.affiliatedAuthorPark, Min Soo-
dc.contributor.affiliatedAuthorPark, Hyun Bin-
dc.contributor.affiliatedAuthorLee, Soon Min-
dc.citation.volume53-
dc.citation.number11-
dc.citation.startPage965-
dc.citation.endPage970-
dc.identifier.bibliographicCitationKorean Journal of Pediatrics, Vol.53(11) : 965-970, 2010-
dc.identifier.rimsid37253-
dc.type.rimsART-
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Pediatrics (소아과학교실) > 1. Journal Papers

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