| Literature DB >> 30086531 |
Oscar Campuzano1, Pilar Beltramo2, Anna Fernandez3, Anna Iglesias4, Laura García2, Catarina Allegue3, Georgia Sarquella-Brugada5, Monica Coll3, Alexandra Perez-Serra4, Irene Mademont-Soler4, Jesus Mates3, Bernat Del Olmo3, Ángeles Rodríguez2, Natalia Maciel2, Marta Puigmulé3, Ferran Pico3, Sergi Cesar5, Josep Brugada6, Alejandro Cuesta7, Carmen Gutierrez8, Ramon Brugada9.
Abstract
Sudden infant death syndrome is the leading cause of death during the first year of life. A large part of cases remains without a conclusive cause of death after complete autopsy. In these situations, cardiac arrhythmia of genetic origin is suspected as the most plausible cause of death. Our aim was to ascertain whether genetic variants associated with sudden cardiac death might be the cause of death in a cohort of infants died suddenly. We analyzed 108 genes associated with sudden cardiac death in 44 post-mortem samples of infants less than 1 year old of age who died at rest. Definite cause of death was not conclusive in any case after a complete autopsy. Genetic analysis identified at least one rare variant in 90.90% of samples. A total of 121 rare genetic variants were identified. Of them, 33.05% were novel and 39.66% were located in genes encoding ion channels or associated proteins. A comprehensive genetic analysis in infants who died suddenly enables the unraveling of potentially causative cardiac variants in 2045% of cases. Molecular autopsy should be included in forensic protocols when no conclusive cause of death is identified. Large part genetic variants remain of uncertain significance, reinforcing the crucial role of genetic interpretation before clinical translation but also in early identification of relatives at risk.Entities:
Keywords: Genetics; Molecular autopsy; Sudden infant death
Mesh:
Year: 2018 PMID: 30086531 DOI: 10.1016/j.fsigen.2018.07.023
Source DB: PubMed Journal: Forensic Sci Int Genet ISSN: 1872-4973 Impact factor: 4.882