Document Type

Journal Article

Publication Date

6-2015

Journal

Molecular Genetics and Metabolism Reports

Volume

Volume 3

Inclusive Pages

47-54

DOI

10.1016/j.ymgmr.2015.03.007

Abstract

We describe a young girl with dilated cardiomyopathy, long QT syndrome, and possible energy deficiency. Two major sequence changes were identified by whole exome sequencing (WES) and mitochondrial DNA analysis that were interpreted as potentially causative. Changes were identified in the KCNH2 gene and mitochondrial tRNA for cysteine. A variation was also seen in MYPBC3. Since the launch of WES as a clinically available technology in 2010, there has been concern regarding the identification of variants unrelated to the patient's phenotype. However, in cases where targeted sequencing fails to explain the clinical presentation, the underlying etiology could be more complex than anticipated. In this situation, the extensive reach of this tool helped explain both her phenotype and family history.

Comments

Reproduced with permission of Elsevier B.V. Molecular Genetics and Metabolism Reports.

Creative Commons License

Creative Commons License
This work is licensed under a Creative Commons Attribution-Noncommercial-No Derivative Works 4.0 License.

Peer Reviewed

1

Open Access

1

Included in

Pediatrics Commons

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