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January 1, 2011Circulation Journal28 citationsOpen Access

Genetic Screening and Double Mutation in Japanese Patients With Hypertrophic Cardiomyopathy

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Why the study?

What is the prevalence and clinical significance of multiple sarcomere gene mutations in Japanese patients with hypertrophic cardiomyopathy?

Population

93 unrelated Japanese patients with Hypertrophic Cardiomyopathy (HCM)

Design

Cross-sectional

Key result

Comprehensive genetic screening of 5 sarcomere genes in 93 Japanese patients with hypertrophic cardiomyopathy identified mutations in 28 patients, including the first report of double mutations in 2 probands.

Authors

TKToru KuboHKHiroaki KitaokaMOMakoto Okawa

Discussion

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Overview

Double mutations may associate with severe HCM phenotypes; leaves open prevalence and screening utility in Japanese patients.

Study Design

Type

Observational (n=93)

Multicenter

No

Structured PICO

What is the prevalence and clinical significance of multiple sarcomere gene mutations in Japanese patients with hypertrophic cardiomyopathy?

P
Population
93 unrelated Japanese patients with familial or sporadic hypertrophic cardiomyopathy who underwent comprehensive genetic analysis of 5 sarcomere genes.
E
Exposure
Comprehensive genetic analysis of 5 sarcomere genes (MYH7, MYBPC3, TNNT2, TPM1, TNNI3)
O
Outcome
Identification of sarcomere gene mutations and their clinical implicationssurrogate

Double mutations in sarcomere genes occur in Japanese HCM patients and may be associated with a more severe phenotype, such as earlier onset and greater wall thickness.

Limitations

  • Effects of R869C and E1049D in MYH7 are not clear due to lack of family survey data for single variant carriers.
  • Comprehensive genetic analysis was limited to only 5 sarcomere genes.
  • Only 1 patient with a double pathological mutation was found, requiring further analysis with larger cohorts to clarify phenotypic effects.
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Cite This Study

Kubo et al. (2011) conducted an observational in Hypertrophic cardiomyopathy (n=93). Genetic screening of 5 sarcomere genes was evaluated on Identification of sarcomere gene mutations. Comprehensive genetic screening of 5 sarcomere genes in 93 Japanese patients with hypertrophic cardiomyopathy identified mutations in 28 patients, including the first report of double mutations in 2 probands.

synapsesocial.com/papers/6a207b4314cb6ef00ff48e93https://doi.org/10.1253/circj.cj-10-1314
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Also Consider

Synapse has enriched 5 closely related papers on similar clinical questions. Consider them for comparative context:

  1. 1Prevalence and Distribution of Sarcomeric Gene Mutations in Japanese Patients With Familial Hypertrophic Cardiomyopathy2011 · 91 citations
  2. 2Spectrum and clinical manifestations of mutations in genes responsible for hypertrophic cardiomyopathy2012 · 28 citations
  3. 3Compound and double mutations in patients with hypertrophic cardiomyopathy: implications for genetic testing and counselling2005 · 389 citations
  4. 4Mutation spectrum in a large cohort of unrelated consecutive patients with hypertrophic cardiomyopathy2003 · 228 citations
  5. 5Mutations in the cardiac troponin T gene show various prognoses in Japanese patients with hypertrophic cardiomyopathy2013 · 20 citations