論文

査読有り 国際誌
2020年8月17日

Advanced pathological study for definite diagnosis of mitochondrial cardiomyopathy.

Journal of clinical pathology
  • Atsuhito Takeda
  • Kei Murayama
  • Yasushi Okazaki
  • Atsuko Imai-Okazaki
  • Akira Ohtake
  • Emi Takakuwa
  • Hirokuni Yamazawa
  • Gaku Izumi
  • Jiro Abe
  • Ayako Nagai
  • Kota Taniguchi
  • Daisuke Sasaki
  • Takao Tsujioka
  • John M Basgen
  • 全て表示

記述言語
英語
掲載種別
研究論文(学術雑誌)
DOI
10.1136/jclinpath-2020-206801

AIMS: Mitochondrial cardiomyopathy (MCM) is difficult to make a definite diagnosis because of various cardiovascular phenotypes and no diagnostic criteria in the pathology examination. We aim to add myocardial pathology to the diagnostic criteria for mitochondrial respiratory chain disorders. METHODS: Quantitative analysis of mitochondria using electron microscopy and immunohistopathological analysis with respiratory chain enzyme antibodies were performed in 11 patients with hypertrophic or restrictive cardiomyopathy who underwent endomyocardial biopsy for possible MCM . Respiratory chain enzymatic assay in biopsied myocardium and genetic studies were also performed in all the subjects to define MCM. RESULTS: Four patients were diagnosed with MCM according to the recent criteria of mitochondrial respiratory chain disorders. Using electron microscopy with quantitative analysis, the volume density of mitochondria within cardiac muscle cells was significantly increased in the MCM group compared with the non-MCM group (p=0.007). Immunohistopathological results were compatible with the result of the respiratory chain enzymatic assay. CONCLUSIONS: Pathological diagnosis of MCM could be confirmed by a quantitative study of electron microscopy and immunohistopathological analysis using the mitochondrial respiratory chain enzyme subunit antibody.

リンク情報
DOI
https://doi.org/10.1136/jclinpath-2020-206801
PubMed
https://www.ncbi.nlm.nih.gov/pubmed/32817174
ID情報
  • DOI : 10.1136/jclinpath-2020-206801
  • PubMed ID : 32817174

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