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Early cardiac evaluation in children with non-specific mitochondrial disease with isolated mitochondrial respiratory chain complex I defect.

DC FieldValueLanguage
dc.contributor.author강훈철-
dc.contributor.author김흥동-
dc.contributor.author이영목-
dc.contributor.author이준수-
dc.date.accessioned2014-12-19T17:14:07Z-
dc.date.available2014-12-19T17:14:07Z-
dc.date.issued2012-
dc.identifier.issn1034-4810-
dc.identifier.urihttps://ir.ymlib.yonsei.ac.kr/handle/22282913/91031-
dc.description.abstractAIMS: We evaluated echocardiography and electrocardiography (ECG) results in children with non-specific mitochondrial disease (MD) in order to study early cardiac involvement, a well-known complication of the disease. METHODS: Among non-specific MD children whose isolated mitochondrial respiratory chain complex I defect was confirmed by muscle biopsy and satisfied the criteria of MD, 27 who had no cardiac symptoms were evaluated by echocardiography and ECG. RESULTS: Three (11.1%) out of the 27 non-specific MD patients had left ventricular ejection fraction of less than 55% and two of them (7.4%) had fractional shortening of less than 26%. ECG abnormalities were observed in 16 of the non-specific MD patients (59.3%). Prolongation of heart rate-corrected QT interval was seen in 11 (40.7%) and widening of the QRS interval in eight (29.6%). Left ventricular ejection fraction and fractional shortening of the patients were significantly decreased compared with those in the control group while heart rate-corrected QT interval was prolonged in the former group. QRS interval was more widened in non-specific MD patients, but without statistical significance. CONCLUSION: The potentially severe cardiac involvement observed in our subjects suggests that early cardiac evaluation after confirming the diagnosis of MD and regular follow-up tests should be strongly recommended in children even in cases without typical cardiac manifestations.-
dc.description.statementOfResponsibilityopen-
dc.relation.isPartOfJOURNAL OF PAEDIATRICS AND CHILD HEALTH-
dc.rightsCC BY-NC-ND 2.0 KR-
dc.rights.urihttps://creativecommons.org/licenses/by-nc-nd/2.0/kr/-
dc.subject.MESHArrhythmias, Cardiac/diagnostic imaging-
dc.subject.MESHArrhythmias, Cardiac/etiology-
dc.subject.MESHChild-
dc.subject.MESHChild, Preschool-
dc.subject.MESHEarly Diagnosis-
dc.subject.MESHEchocardiography-
dc.subject.MESHElectrocardiography-
dc.subject.MESHElectron Transport Complex I*/metabolism-
dc.subject.MESHFemale-
dc.subject.MESHHumans-
dc.subject.MESHMale-
dc.subject.MESHMitochondrial Diseases/complications-
dc.subject.MESHMitochondrial Diseases/diagnostic imaging-
dc.subject.MESHMitochondrial Diseases/physiopathology*-
dc.subject.MESHMyocardial Contraction/physiology-
dc.subject.MESHRetrospective Studies-
dc.subject.MESHVentricular Dysfunction, Left/diagnostic imaging-
dc.subject.MESHVentricular Dysfunction, Left/etiology-
dc.titleEarly cardiac evaluation in children with non-specific mitochondrial disease with isolated mitochondrial respiratory chain complex I defect.-
dc.typeArticle-
dc.contributor.collegeCollege of Medicine (의과대학)-
dc.contributor.departmentDept. of Pediatrics (소아과학)-
dc.contributor.googleauthorRan Baik-
dc.contributor.googleauthorRita Yu-
dc.contributor.googleauthorYoung Mock Lee-
dc.contributor.googleauthorHoon Chul Kang-
dc.contributor.googleauthorJoon Soo Lee-
dc.contributor.googleauthorHeung Dong Kim-
dc.identifier.doi23038991-
dc.admin.authorfalse-
dc.admin.mappingfalse-
dc.contributor.localIdA00102-
dc.contributor.localIdA01208-
dc.contributor.localIdA02955-
dc.contributor.localIdA03177-
dc.relation.journalcodeJ01675-
dc.identifier.eissn1440-1754-
dc.identifier.pmid23038991-
dc.identifier.urlhttp://onlinelibrary.wiley.com/doi/10.1111/j.1440-1754.2012.02585.x/abstract-
dc.subject.keywordArrhythmias, Cardiac/diagnostic imaging-
dc.subject.keywordArrhythmias, Cardiac/etiology-
dc.subject.keywordChild-
dc.subject.keywordChild, Preschool-
dc.subject.keywordEarly Diagnosis-
dc.subject.keywordEchocardiography-
dc.subject.keywordElectrocardiography-
dc.subject.keywordElectron Transport Complex I*/metabolism-
dc.subject.keywordFemale-
dc.subject.keywordHumans-
dc.subject.keywordMale-
dc.subject.keywordMitochondrial Diseases/complications-
dc.subject.keywordMitochondrial Diseases/diagnostic imaging-
dc.subject.keywordMitochondrial Diseases/physiopathology*-
dc.subject.keywordMyocardial Contraction/physiology-
dc.subject.keywordRetrospective Studies-
dc.subject.keywordVentricular Dysfunction, Left/diagnostic imaging-
dc.subject.keywordVentricular Dysfunction, Left/etiology-
dc.contributor.alternativeNameKang, Hoon Chul-
dc.contributor.alternativeNameKim, Heung Dong-
dc.contributor.alternativeNameLee, Young Mock-
dc.contributor.alternativeNameLee, Joon Soo-
dc.contributor.affiliatedAuthorKang, Hoon Chul-
dc.contributor.affiliatedAuthorKim, Heung Dong-
dc.contributor.affiliatedAuthorLee, Young Mock-
dc.contributor.affiliatedAuthorLee, Joon Soo-
dc.citation.volume48-
dc.citation.number11-
dc.citation.startPage1016-
dc.citation.endPage1020-
dc.identifier.bibliographicCitationJOURNAL OF PAEDIATRICS AND CHILD HEALTH, Vol.48(11) : 1016-1020, 2012-
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Pediatrics (소아청소년과학교실) > 1. Journal Papers

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