Cause of Death in Children With Mitochondrial Diseases

Soyong Eom, Ha Neul Lee, Sunho Lee, hoonchul kang, Joon Soo Lee, HeungDong Kim, Young Mock Lee

Research output: Contribution to journalArticle

13 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)82-88
Number of pages7
JournalPediatric Neurology
Volume66
DOIs
Publication statusPublished - 2017 Jan 1

Fingerprint

Mitochondrial Diseases
Cause of Death
Leigh Disease
Pediatrics
Early Medical Intervention
MELAS Syndrome
Survival
Mortality
Disseminated Intravascular Coagulation
Sudden Death
Thalamus
Age of Onset
Medical Records
Sepsis
Pneumonia
Hospitalization

All Science Journal Classification (ASJC) codes

  • Pediatrics, Perinatology, and Child Health
  • Neurology
  • Developmental Neuroscience
  • Clinical Neurology

Cite this

Eom, Soyong ; Lee, Ha Neul ; Lee, Sunho ; kang, hoonchul ; Lee, Joon Soo ; Kim, HeungDong ; Lee, Young Mock. / Cause of Death in Children With Mitochondrial Diseases. In: Pediatric Neurology. 2017 ; Vol. 66. pp. 82-88.
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Cause of Death in Children With Mitochondrial Diseases. / Eom, Soyong; Lee, Ha Neul; Lee, Sunho; kang, hoonchul; Lee, Joon Soo; Kim, HeungDong; Lee, Young Mock.

In: Pediatric Neurology, Vol. 66, 01.01.2017, p. 82-88.

Research output: Contribution to journalArticle

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N2 - 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.

AB - 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.

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