Whole-Exome Sequencing Reveals CLCNKB Mutations in a Case of Sudden Unexpected Infant Death

Pediatr Dev Pathol. 2015 Jul-Aug;18(4):324-6. doi: 10.2350/14-08-1543-CR.1. Epub 2015 Apr 29.

Abstract

Cases of sudden unexpected infant death (SUID) leave many families devastated, especially in those without an identified cause of death. Here, we describe the case of an apparently healthy 15-day-old infant who died suddenly and unexpectedly. Through whole-exome sequencing, the infant was posthumously found to have 2 mutations in the CLCNKB gene, leading to a molecular diagnosis of Bartter syndrome type III, the likely cause of death. This case illustrates the potential utility of exome sequencing in cases of SUID to suggest a diagnosis, with important implications for families, allowing them to come to closure over the cause of death, informing their future reproductive decisions, and minimizing the risk of recurrence.

Keywords: Bartter syndrome; CLCNKB; SIDS; SUID; whole-exome sequencing.

Publication types

  • Case Reports

MeSH terms

  • Autopsy
  • Bartter Syndrome / genetics*
  • Cause of Death
  • Chloride Channels / genetics*
  • DNA Mutational Analysis*
  • Exome*
  • Fatal Outcome
  • Genetic Predisposition to Disease
  • Humans
  • Infant, Newborn
  • Male
  • Mutation*
  • Predictive Value of Tests
  • Sudden Infant Death / genetics*

Substances

  • CLCNKB protein, human
  • Chloride Channels

Supplementary concepts

  • Bartter syndrome, type 3