Cited 9 times in
Long-term Developmental Trends of Pediatric Mitochondrial Diseases: The Five Stages of Developmental Decline
DC Field | Value | Language |
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dc.contributor.author | 이영목 | - |
dc.date.accessioned | 2018-07-20T07:39:01Z | - |
dc.date.available | 2018-07-20T07:39:01Z | - |
dc.date.issued | 2017 | - |
dc.identifier.uri | https://ir.ymlib.yonsei.ac.kr/handle/22282913/160379 | - |
dc.description.abstract | Mitochondrial diseases (MDs) are a heterogeneous group of progressive multisystem disorders caused by impaired mitochondrial function. This study aimed to evaluate the clinical course and long-term development of 53 pediatric patients with MDs. Developmental function was evaluated at nine time points (two pre-diagnosis, one at diagnosis, and six post-diagnosis), with the developmental quotient (DQ) from the Korean infant and child development test (KICDT) assessing a child's developmental age (rather than chronological age). Additionally, disease-related clinical variables were reviewed, and clinical progress was determined through observation. Subgroup analyses by epilepsy severity, syndromic diagnosis, diffuse brain atrophy, and clinical rating were performed. The pre- and post-diagnosis results were compared by the paired t-test and Bonferroni correction. The pre-diagnostic, diagnostic, and post-diagnostic evaluations were compared using repeated measures ANOVA. Patients with diffuse brain atrophy at the first pre-diagnostic and second post-diagnostic evaluations showed lower DQs. Compared with patients with a mildly or severely deteriorating clinical course, those with an improving or static clinical course presented higher DQs at the pre-diagnostic and diagnostic evaluations. The age at onset of the first symptom correlated positively with the DQ post-diagnosis. Follow-up revealed consistent patterns of significant developmental deterioration during the lead time to diagnosis, with no significant decline post-diagnosis. The DQ is a feasible predictor and a measure of long-term functional development in children with MD. Early initiation of treatment may minimize developmental regression. | - |
dc.description.statementOfResponsibility | open | - |
dc.language | English | - |
dc.publisher | Frontiers Research Foundation | - |
dc.relation.isPartOf | FRONTIERS IN NEUROLOGY | - |
dc.rights | CC BY-NC-ND 2.0 KR | - |
dc.rights | https://creativecommons.org/licenses/by-nc-nd/2.0/kr/ | - |
dc.title | Long-term Developmental Trends of Pediatric Mitochondrial Diseases: The Five Stages of Developmental Decline | - |
dc.type | Article | - |
dc.contributor.college | College of Medicine | - |
dc.contributor.department | Dept. of Pediatrics | - |
dc.contributor.googleauthor | Soyong Eom | - |
dc.contributor.googleauthor | Young-Mock Lee | - |
dc.identifier.doi | 10.3389/fneur.2017.00208 | - |
dc.contributor.localId | A02955 | - |
dc.relation.journalcode | J02996 | - |
dc.identifier.eissn | 1664-2295 | - |
dc.identifier.pmid | 28567029 | - |
dc.subject.keyword | brain atrophy | - |
dc.subject.keyword | developmental quotient | - |
dc.subject.keyword | metabolic disease | - |
dc.subject.keyword | mitochondrial disease | - |
dc.subject.keyword | pediatric epilepsy | - |
dc.contributor.alternativeName | Lee, Young Mock | - |
dc.contributor.affiliatedAuthor | Lee, Young Mock | - |
dc.citation.volume | 8 | - |
dc.citation.startPage | 208 | - |
dc.identifier.bibliographicCitation | FRONTIERS IN NEUROLOGY, Vol.8 : 208, 2017 | - |
dc.identifier.rimsid | 43538 | - |
dc.type.rims | ART | - |
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