Post-mortem genetic investigation of cardiac disease-associated genes in sudden infant death syndrome (SIDS) cases.


Journal

International journal of legal medicine
ISSN: 1437-1596
Titre abrégé: Int J Legal Med
Pays: Germany
ID NLM: 9101456

Informations de publication

Date de publication:
Jan 2021
Historique:
received: 10 06 2020
accepted: 03 08 2020
pubmed: 14 8 2020
medline: 3 9 2021
entrez: 14 8 2020
Statut: ppublish

Résumé

The sudden infant death syndrome (SIDS) is one of the leading causes of postneonatal infant death. It has been shown that there exists a complex relationship between SIDS and inherited cardiac disease. Next-generation sequencing and surveillance of cardiac channelopathy and cardiomyopathy genes represent an important tool for investigating the cause of death in SIDS cases. In the present study, targeted sequencing of 80 genes associated with genetic heart diseases in a cohort of 31 SIDS cases was performed. To determine the spectrum and prevalence of genetic heart disease associated mutations as a potential monogenic basis for SIDS, a stringent variant classification was applied and the percentage of rare (minor allele frequency ≤ 0.2%) and ultra-rare variants (minor allele frequency ≤ 0.005%) in these genes was assessed. With a minor allele frequency of ≤ 0.005%, about 20% of the SIDS cases exhibited a variant of uncertain significance (VUS), but in only 6% of these cases, gene variants proved to be "potentially informative." The present study shows the importance of careful variant interpretation. Applying stringent criteria misinterpretations are avoided, as the results of genetic analyses may have an important impact of the family members involved.

Identifiants

pubmed: 32789579
doi: 10.1007/s00414-020-02394-x
pii: 10.1007/s00414-020-02394-x
pmc: PMC7782403
doi:

Substances chimiques

KCNJ2 protein, human 0
MYH7 protein, human 0
NAV1.5 Voltage-Gated Sodium Channel 0
Potassium Channels, Inwardly Rectifying 0
SCN5A protein, human 0
Cardiac Myosins EC 3.6.1.-
Myosin Heavy Chains EC 3.6.4.1

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

207-212

Références

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Auteurs

Jasmin Köffer (J)

Institute of Legal Medicine, University of Frankfurt, Kennedyallee 104, 60596, Frankfurt/Main, Germany.

Stefanie Scheiper-Welling (S)

Institute of Legal Medicine, University of Frankfurt, Kennedyallee 104, 60596, Frankfurt/Main, Germany.

Marcel A Verhoff (MA)

Institute of Legal Medicine, University of Frankfurt, Kennedyallee 104, 60596, Frankfurt/Main, Germany.

Thomas Bajanowski (T)

Institute of Legal Medicine, University of Essen, Hufelandstraße 55, 45122, Essen, Germany.

Silke Kauferstein (S)

Institute of Legal Medicine, University of Frankfurt, Kennedyallee 104, 60596, Frankfurt/Main, Germany. kauferstein@em.uni-frankfurt.de.

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