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Survival analysis of spinal muscular atrophy type I.

Authors
 Hyun Bin Park  ;  Soon Min Lee  ;  Jin Sung Lee  ;  Min Soo Park  ;  Kook In Park  ;  Ran Namgung  ;  Chul Lee 
Citation
 Korean Journal of Pediatrics, Vol.53(11) : 965-970, 2010 
Journal Title
 Korean Journal of Pediatrics 
ISSN
 1738-1061 
Issue Date
2010
Keywords
Spinal muscular atrophy ; Survival ; Survival analysis
Abstract
PURPOSE: 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
Files in This Item:
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DOI
10.3345/kjp.2010.53.11.965
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Pediatrics (소아청소년과학교실) > 1. Journal Papers
Yonsei Authors
Namgung, Ran(남궁란) ORCID logo https://orcid.org/0000-0001-7182-9535
Park, Kook In(박국인) ORCID logo https://orcid.org/0000-0001-8499-9293
Park, Min Soo(박민수) ORCID logo https://orcid.org/0000-0002-4395-9938
Park, Hyun Bin(박현빈)
Lee, Soon Min(이순민) ORCID logo https://orcid.org/0000-0003-0174-1065
Lee, Jin Sung(이진성) ORCID logo https://orcid.org/0000-0002-1262-8597
Lee, Chul(이철)
URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/103291
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