Literature DB >> 30600267

Genomic Sequencing Expansion and Incomplete Penetrance.

Joseph T C Shieh1.   

Abstract

BACKGROUND: Genetic data have the potential to impact patient care significantly. In primary care and in the ICU, patients are undergoing genetic testing. Genetics is also transforming cancer care and undiagnosed diseases. Optimal personalized medicine relies on the understanding of disease penetrance. In this article, I examine the complexity of penetrance.
METHODS: In this article, I assess how variable penetrance can be seen with many diseases, including those of different modes of inheritance, and how genomic testing is being applied effectively for many diseases. In this article, I also identify challenges in the field, including the interpretation of gene variants.
RESULTS: Using advancing bioinformatics and detailed phenotypic assessment, we can increase the yield of genomic testing, particularly for highly penetrant conditions. The technologies are useful and applicable to different medical situations.
CONCLUSIONS: There are now effective genome diagnostics for many diseases. However, the best personalized application of these data still requires skilled interpretation.
Copyright © 2019 by the American Academy of Pediatrics.

Entities:  

Mesh:

Year:  2019        PMID: 30600267      PMCID: PMC7185999          DOI: 10.1542/peds.2018-1099E

Source DB:  PubMed          Journal:  Pediatrics        ISSN: 0031-4005            Impact factor:   7.124


  33 in total

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Journal:  Mol Genet Metab       Date:  2017-07-08       Impact factor: 4.797

2.  Evaluating the Clinical Validity of Gene-Disease Associations: An Evidence-Based Framework Developed by the Clinical Genome Resource.

Authors:  Natasha T Strande; Erin Rooney Riggs; Adam H Buchanan; Ozge Ceyhan-Birsoy; Marina DiStefano; Selina S Dwight; Jenny Goldstein; Rajarshi Ghosh; Bryce A Seifert; Tam P Sneddon; Matt W Wright; Laura V Milko; J Michael Cherry; Monica A Giovanni; Michael F Murray; Julianne M O'Daniel; Erin M Ramos; Avni B Santani; Alan F Scott; Sharon E Plon; Heidi L Rehm; Christa L Martin; Jonathan S Berg
Journal:  Am J Hum Genet       Date:  2017-05-25       Impact factor: 11.025

3.  Direct-to-Consumer Medical Testing in the Era of Value-Based Care.

Authors:  Kimberly Lovett Rockwell
Journal:  JAMA       Date:  2017-06-27       Impact factor: 56.272

4.  Molecular spectrum of SLC22A5 (OCTN2) gene mutations detected in 143 subjects evaluated for systemic carnitine deficiency.

Authors:  Fang-Yuan Li; Ayman W El-Hattab; Erawati V Bawle; Richard G Boles; Eric S Schmitt; Fernando Scaglia; Lee-Jun Wong
Journal:  Hum Mutat       Date:  2010-08       Impact factor: 4.878

5.  Diagnoses of newborns and mothers with carnitine uptake defects through newborn screening.

Authors:  Ni-Chung Lee; Nelson Leung-Sang Tang; Yin-Hsiu Chien; Chun-An Chen; Sho-Juan Lin; Pao-Chin Chiu; Ai-Chu Huang; Wuh-Liang Hwu
Journal:  Mol Genet Metab       Date:  2009-12-28       Impact factor: 4.797

6.  Carnitine-associated encephalopathy caused by long-term treatment with an antibiotic containing pivalic acid.

Authors:  Yasuko Makino; Tokio Sugiura; Tetsuya Ito; Naruji Sugiyama; Norihisa Koyama
Journal:  Pediatrics       Date:  2007-08-27       Impact factor: 7.124

7.  Developmental outcomes with early orthotopic liver transplantation for infants with neonatal-onset urea cycle defects and a female patient with late-onset ornithine transcarbamylase deficiency.

Authors:  Kim L McBride; Geoffrey Miller; Susan Carter; Saul Karpen; John Goss; Brendan Lee
Journal:  Pediatrics       Date:  2004-10       Impact factor: 7.124

8.  Standards and guidelines for the interpretation of sequence variants: a joint consensus recommendation of the American College of Medical Genetics and Genomics and the Association for Molecular Pathology.

Authors:  Sue Richards; Nazneen Aziz; Sherri Bale; David Bick; Soma Das; Julie Gastier-Foster; Wayne W Grody; Madhuri Hegde; Elaine Lyon; Elaine Spector; Karl Voelkerding; Heidi L Rehm
Journal:  Genet Med       Date:  2015-03-05       Impact factor: 8.822

9.  International Cooperation to Enable the Diagnosis of All Rare Genetic Diseases.

Authors:  Kym M Boycott; Ana Rath; Jessica X Chong; Taila Hartley; Fowzan S Alkuraya; Gareth Baynam; Anthony J Brookes; Michael Brudno; Angel Carracedo; Johan T den Dunnen; Stephanie O M Dyke; Xavier Estivill; Jack Goldblatt; Catherine Gonthier; Stephen C Groft; Ivo Gut; Ada Hamosh; Philip Hieter; Sophie Höhn; Matthew E Hurles; Petra Kaufmann; Bartha M Knoppers; Jeffrey P Krischer; Milan Macek; Gert Matthijs; Annie Olry; Samantha Parker; Justin Paschall; Anthony A Philippakis; Heidi L Rehm; Peter N Robinson; Pak-Chung Sham; Rumen Stefanov; Domenica Taruscio; Divya Unni; Megan R Vanstone; Feng Zhang; Han Brunner; Michael J Bamshad; Hanns Lochmüller
Journal:  Am J Hum Genet       Date:  2017-05-04       Impact factor: 11.025

10.  ACMG Practice Guideline: lack of evidence for MTHFR polymorphism testing.

Authors:  Scott E Hickey; Cynthia J Curry; Helga V Toriello
Journal:  Genet Med       Date:  2013-01-03       Impact factor: 8.822

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  4 in total

1.  Nonlinear gene expression-phenotype relationships contribute to variation and clefting in the A/WySn mouse.

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Journal:  Dev Dyn       Date:  2019-09-14       Impact factor: 3.780

2.  Genomic Sequencing for Newborn Screening: Results of the NC NEXUS Project.

Authors:  Tamara S Roman; Stephanie B Crowley; Myra I Roche; Ann Katherine M Foreman; Julianne M O'Daniel; Bryce A Seifert; Kristy Lee; Alicia Brandt; Chelsea Gustafson; Daniela M DeCristo; Natasha T Strande; Lori Ramkissoon; Laura V Milko; Phillips Owen; Sayanty Roy; Mai Xiong; Ryan S Paquin; Rita M Butterfield; Megan A Lewis; Katherine J Souris; Donald B Bailey; Christine Rini; Jessica K Booker; Bradford C Powell; Karen E Weck; Cynthia M Powell; Jonathan S Berg
Journal:  Am J Hum Genet       Date:  2020-08-26       Impact factor: 11.025

Review 3.  Application of magnetic nanoparticles in nucleic acid detection.

Authors:  Congli Tang; Ziyu He; Hongmei Liu; Yuyue Xu; Hao Huang; Gaojian Yang; Ziqi Xiao; Song Li; Hongna Liu; Yan Deng; Zhu Chen; Hui Chen; Nongyue He
Journal:  J Nanobiotechnology       Date:  2020-04-21       Impact factor: 10.435

Review 4.  Incomplete Penetrance and Variable Expressivity: From Clinical Studies to Population Cohorts.

Authors:  Rebecca Kingdom; Caroline F Wright
Journal:  Front Genet       Date:  2022-07-25       Impact factor: 4.772

  4 in total

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