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Cause of Death in Children With Mitochondrial Diseases

Authors
 Soyong Eom  ;  Ha Neul Lee  ;  Sunho Lee  ;  Hoon-Chul Kang  ;  Joon Soo Lee  ;  Heung Dong Kim  ;  Young-Mock Lee 
Citation
 Pediatric Neurology, Vol.66 : 82-88, 2017 
Journal Title
 Pediatric Neurology 
ISSN
 0887-8994 
Issue Date
2017
MeSH
Age of Onset ; Cause of Death ; Child ; Child, Preschool ; Female ; Follow-Up Studies ; Humans ; Male ; Mitochondrial Diseases/mortality*
Keywords
cause of death ; children ; mitochondrial disease ; mortality ; pediatric ; risk factor
Abstract
BACKGROUND: We investigated the clinical characteristics that represent risk factors for death in pediatric patients with mitochondrial diseases. METHODS: The medical records of mitochondrial disease pediatric patients attended between 2006 and 2015 (n = 221) were reviewed for clinical characteristics, diagnosis, hospitalization, follow-up, survival, and cause of death. RESULTS: The global mortality rate in the cohort was 14% (average age at death, six years). By syndromic diagnosis, the mortality rates were as follows: Leigh syndrome, 17% (15 of 88); mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes, 50% (two of four); and nonspecific mitochondrial disease, 11% (14 of 129). Data regarding 31 patients (17 males) were included in the analysis of cause of death. The age at symptom onset, lead time to diagnosis, duration of illness, and duration of life were 1.8 ± 2.0, 1.7 ± 1.5, 4.3 ± 2.7, and 6.1 ± 2.9 years, respectively. The most common causes of death were sepsis, pneumonia, disseminated intravascular coagulation, and sudden unexpected death (55%, 42%, 29%, and 29%, respectively). Early death (age six years or younger) was associated with lesions in the thalamus, number of organs involved, and Leigh syndrome. CONCLUSIONS: Careful monitoring of the medical condition and early intervention are key to improving survival in pediatric patients with mitochondrial disease.
Full Text
http://www.sciencedirect.com/science/article/pii/S0887899416306737
DOI
10.1016/j.pediatrneurol.2016.10.006
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Pediatrics (소아청소년과학교실) > 1. Journal Papers
Yonsei Authors
강훈철(Kang, Hoon Chul) ORCID logo https://orcid.org/0000-0002-3659-8847
김흥동(Kim, Heung Dong) ORCID logo https://orcid.org/0000-0002-8031-7336
이선호(Lee, Sun Ho)
이영목(Lee, Young Mock) ORCID logo https://orcid.org/0000-0002-5838-249X
이준수(Lee, Joon Soo) ORCID logo https://orcid.org/0000-0001-9036-9343
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URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/154312
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