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April 21, 2016PLoS ONEOpen Access

Calmodulin 2 Mutation N98S Is Associated with Unexplained Cardiac Arrest in Infants Due to Low Clinical Penetrance Electrical Disorders

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Population

2 unrelated children in southern Spain with apparently unexplained cardiac arrest/death and normal baseline…

Design

Case_series

Follow-up

2 years (for the surviving proband)

Key result

The CALM2 p.Asn98Ser mutation is associated with sudden cardiac death in childhood, presenting with variable clinical penetrance suggestive of CPVT and LQTS.

Authors

JJJuan Jiménez‐JáimezJDJulián Palomino DozaAOA. Revenga Ortega

Discussion

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Overview

Warrants CALM2 screening in unexplained pediatric SCD; hypothesis-generating for CPVT/LQTS overlap and requires validation.

Study Design

Type

Case Report (n=2)

Multicenter

No

Structured PICO

P
Population
Two unrelated children, aged 4 and 7, who experienced sudden cardiac arrest and were found to carry the CALM2 p.Asn98Ser mutation.
O
Outcome
Detection of pathogenic genetic mutations and description of clinical phenotypes

The CALM2 p.Asn98Ser mutation is associated with unexplained cardiac arrest in infants due to low clinical penetrance electrical disorders such as CPVT and LQTS.

Limitations

  • Very small sample size (two cases)
  • No functional analysis of the mutation was performed
  • Parents of proband B refused genetic testing
  • Very small sample size

Cite This Study

Jiménez‐Jáimez et al. (2016) conducted a case report in Unexplained cardiac arrest (n=2). CALM2 p.Asn98Ser mutation was evaluated on Identification of genetic mutation causing unexplained cardiac arrest. The CALM2 p.Asn98Ser mutation is associated with sudden cardiac death in childhood, presenting with variable clinical penetrance suggestive of CPVT and LQTS.

synapsesocial.com/papers/6a8848825ee6e03ae3cf1b1fhttps://doi.org/10.1371/journal.pone.0153851
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