PulseExploreJournal ClubResearchersJournals
Instagram
HomeJournal ClubExplore
Synapse
⌘+K
Synapse
July 17, 2009Circulation Cardiovascular GeneticsOpen Access

Prevalence of Sarcomere Protein Gene Mutations in Preadolescent Children With Hypertrophic Cardiomyopathy

View Full Paper
Ask AI
Bookmark
Share

Why the study?

What is the prevalence of sarcomere protein gene mutations in preadolescent children with hypertrophic cardiomyopathy?

Population

79 consecutive patients diagnosed with hypertrophic cardiomyopathy (HCM) aged 13 years or younger

Design

Cross-sectional

Key result

Genetic screening of preadolescent children with hypertrophic cardiomyopathy identified sarcomere protein gene mutations in 53.2% of patients, demonstrating familial disease is common.

Authors

JKJuan Pablo KaskiPSPetros SyrrisMTMaite Tome

Discussion

Loading...

Member takes

Overview

May support family screening in pediatric HCM; leaves open prospective validation of yield and outcomes.

Study Design

Type

Observational (n=79)

Structured PICO

What is the prevalence of sarcomere protein gene mutations in preadolescent children with hypertrophic cardiomyopathy?

P
Population
79 consecutive patients diagnosed with hypertrophic cardiomyopathy (HCM) aged 13 years or younger
I
Intervention
Genetic screening of 9 sarcomere protein genes (MYH7, MYBPC3, TNNI3, TNNT2, TPM1, MYL2, MYL3, ACTC, and TNNC1), DES, and PRKAG2
O
Outcome
Prevalence of sarcomere protein gene mutations

Familial disease caused by sarcomere protein gene mutations is a common cause of hypertrophic cardiomyopathy in infants and children, indicating that first-degree relatives should be offered screening.

Cite This Study

Kaski et al. (2009) conducted an observational in Hypertrophic cardiomyopathy (n=79). Genetic screening was evaluated on Prevalence of sarcomere protein gene mutations. Genetic screening of preadolescent children with hypertrophic cardiomyopathy identified sarcomere protein gene mutations in 53.2% of patients, demonstrating familial disease is common.

synapsesocial.com/papers/6a0a9060e7a7b397ee73785chttps://doi.org/10.1161/circgenetics.108.821314
View Full Paper
Ask AI
Bookmark
Share

Also Consider

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

  1. 1Sarcomere Protein Gene Mutations in Hypertrophic Cardiomyopathy of the Elderly2002 · 365 citations
  2. 2Development and Progression of Left Ventricular Hypertrophy in Children with Hypertrophic Cardiomyopathy1986 · 323 citations
  3. 3Shared Genetic Causes of Cardiac Hypertrophy in Children and Adults2008 · 413 citations
  4. 4Double heterozygosity for mutations in the β-myosin heavy chain and in the cardiac myosin binding protein C genes in a family with hypertrophic cardiomyopathy1999 · 70 citations
  5. 5Glycogen Storage Diseases Presenting as Hypertrophic Cardiomyopathy2005 · 652 citations