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author Lunke, Sebastian
Bouffler, Sophie E.
Patel, Chirag V.
Sandaradura, Sarah A.
Wilson, Meredith
Pinner, Jason
Hunter, Matthew F.
Barnett, Christopher P.
Wallis, Mathew
Kamien, Benjamin
Tan, Tiong Y.
Freckmann, Mary-Louise
Chong, Belinda
Phelan, Dean
Francis, David
Kassahn, Karin S.
Ha, Thuong
Gao, Song
Arts, Peer
Jackson, Matilda R.
Scott, Hamish S.
Eggers, Stefanie
Rowley, Simone
Boggs, Kirsten
Rakonjac, Ana
Brett, Gemma R.
de Silva, Michelle G.
Springer, Amanda
Ward, Michelle
Stallard, Kirsty
Simons, Cas
Conway, Thomas
Halman, Andreas
Van Bergen, Nicole J.
Sikora, Tim
Semcesen, Liana N.
Stroud, David A.
Compton, Alison G.
Thorburn, David R.
Bell, Katrina M.
Sadedin, Simon
North, Kathryn N.
Christodoulou, John
Stark, Zornitza
author_facet Lunke, Sebastian
Bouffler, Sophie E.
Patel, Chirag V.
Sandaradura, Sarah A.
Wilson, Meredith
Pinner, Jason
Hunter, Matthew F.
Barnett, Christopher P.
Wallis, Mathew
Kamien, Benjamin
Tan, Tiong Y.
Freckmann, Mary-Louise
Chong, Belinda
Phelan, Dean
Francis, David
Kassahn, Karin S.
Ha, Thuong
Gao, Song
Arts, Peer
Jackson, Matilda R.
Scott, Hamish S.
Eggers, Stefanie
Rowley, Simone
Boggs, Kirsten
Rakonjac, Ana
Brett, Gemma R.
de Silva, Michelle G.
Springer, Amanda
Ward, Michelle
Stallard, Kirsty
Simons, Cas
Conway, Thomas
Halman, Andreas
Van Bergen, Nicole J.
Sikora, Tim
Semcesen, Liana N.
Stroud, David A.
Compton, Alison G.
Thorburn, David R.
Bell, Katrina M.
Sadedin, Simon
North, Kathryn N.
Christodoulou, John
Stark, Zornitza
author_sort Lunke, Sebastian
collection PubMed
description Critically ill infants and children with rare diseases need equitable access to rapid and accurate diagnosis to direct clinical management. Over 2 years, the Acute Care Genomics program provided whole-genome sequencing to 290 families whose critically ill infants and children were admitted to hospitals throughout Australia with suspected genetic conditions. The average time to result was 2.9 d and diagnostic yield was 47%. We performed additional bioinformatic analyses and transcriptome sequencing in all patients who remained undiagnosed. Long-read sequencing and functional assays, ranging from clinically accredited enzyme analysis to bespoke quantitative proteomics, were deployed in selected cases. This resulted in an additional 19 diagnoses and an overall diagnostic yield of 54%. Diagnostic variants ranged from structural chromosomal abnormalities through to an intronic retrotransposon, disrupting splicing. Critical care management changed in 120 diagnosed patients (77%). This included major impacts, such as informing precision treatments, surgical and transplant decisions and palliation, in 94 patients (60%). Our results provide preliminary evidence of the clinical utility of integrating multi-omic approaches into mainstream diagnostic practice to fully realize the potential of rare disease genomic testing in a timely manner.
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spelling pubmed-103539362023-07-20 Integrated multi-omics for rapid rare disease diagnosis on a national scale Lunke, Sebastian Bouffler, Sophie E. Patel, Chirag V. Sandaradura, Sarah A. Wilson, Meredith Pinner, Jason Hunter, Matthew F. Barnett, Christopher P. Wallis, Mathew Kamien, Benjamin Tan, Tiong Y. Freckmann, Mary-Louise Chong, Belinda Phelan, Dean Francis, David Kassahn, Karin S. Ha, Thuong Gao, Song Arts, Peer Jackson, Matilda R. Scott, Hamish S. Eggers, Stefanie Rowley, Simone Boggs, Kirsten Rakonjac, Ana Brett, Gemma R. de Silva, Michelle G. Springer, Amanda Ward, Michelle Stallard, Kirsty Simons, Cas Conway, Thomas Halman, Andreas Van Bergen, Nicole J. Sikora, Tim Semcesen, Liana N. Stroud, David A. Compton, Alison G. Thorburn, David R. Bell, Katrina M. Sadedin, Simon North, Kathryn N. Christodoulou, John Stark, Zornitza Nat Med Article Critically ill infants and children with rare diseases need equitable access to rapid and accurate diagnosis to direct clinical management. Over 2 years, the Acute Care Genomics program provided whole-genome sequencing to 290 families whose critically ill infants and children were admitted to hospitals throughout Australia with suspected genetic conditions. The average time to result was 2.9 d and diagnostic yield was 47%. We performed additional bioinformatic analyses and transcriptome sequencing in all patients who remained undiagnosed. Long-read sequencing and functional assays, ranging from clinically accredited enzyme analysis to bespoke quantitative proteomics, were deployed in selected cases. This resulted in an additional 19 diagnoses and an overall diagnostic yield of 54%. Diagnostic variants ranged from structural chromosomal abnormalities through to an intronic retrotransposon, disrupting splicing. Critical care management changed in 120 diagnosed patients (77%). This included major impacts, such as informing precision treatments, surgical and transplant decisions and palliation, in 94 patients (60%). Our results provide preliminary evidence of the clinical utility of integrating multi-omic approaches into mainstream diagnostic practice to fully realize the potential of rare disease genomic testing in a timely manner. Nature Publishing Group US 2023-06-08 2023 /pmc/articles/PMC10353936/ /pubmed/37291213 http://dx.doi.org/10.1038/s41591-023-02401-9 Text en © The Author(s) 2023 https://creativecommons.org/licenses/by/4.0/Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons license and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this license, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) .
spellingShingle Article
Lunke, Sebastian
Bouffler, Sophie E.
Patel, Chirag V.
Sandaradura, Sarah A.
Wilson, Meredith
Pinner, Jason
Hunter, Matthew F.
Barnett, Christopher P.
Wallis, Mathew
Kamien, Benjamin
Tan, Tiong Y.
Freckmann, Mary-Louise
Chong, Belinda
Phelan, Dean
Francis, David
Kassahn, Karin S.
Ha, Thuong
Gao, Song
Arts, Peer
Jackson, Matilda R.
Scott, Hamish S.
Eggers, Stefanie
Rowley, Simone
Boggs, Kirsten
Rakonjac, Ana
Brett, Gemma R.
de Silva, Michelle G.
Springer, Amanda
Ward, Michelle
Stallard, Kirsty
Simons, Cas
Conway, Thomas
Halman, Andreas
Van Bergen, Nicole J.
Sikora, Tim
Semcesen, Liana N.
Stroud, David A.
Compton, Alison G.
Thorburn, David R.
Bell, Katrina M.
Sadedin, Simon
North, Kathryn N.
Christodoulou, John
Stark, Zornitza
Integrated multi-omics for rapid rare disease diagnosis on a national scale
title Integrated multi-omics for rapid rare disease diagnosis on a national scale
title_full Integrated multi-omics for rapid rare disease diagnosis on a national scale
title_fullStr Integrated multi-omics for rapid rare disease diagnosis on a national scale
title_full_unstemmed Integrated multi-omics for rapid rare disease diagnosis on a national scale
title_short Integrated multi-omics for rapid rare disease diagnosis on a national scale
title_sort integrated multi-omics for rapid rare disease diagnosis on a national scale
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10353936/
https://www.ncbi.nlm.nih.gov/pubmed/37291213
http://dx.doi.org/10.1038/s41591-023-02401-9
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