Genetics in Medicine - 2014

175 articles | Last updated: 2025-12-03 14:12:56
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T A T A T A T A
Genomics education for Veterans Affairs nurses
News Briefs
Erratum: Whole-exome sequencing identifies rare pathogenic variants in new predisposition genes for familial colorectal cancer
Reviewer Acknowledgment 2014
In This Issue
In This Issue
News Briefs
Diagnosis and management of glycogen storage disease type I: a practice guideline of the American College of Medical Genetics and Genomics
American College of Medical Genetics and Genomics Standards and Guidelines for Clinical Genetics Laboratories, 2014 edition: technical standards and guidelines for Huntington disease
In This Issue
News Briefs
Genetic counseling for women referred for advanced maternal age: a telegenetic approach
Interpreting noninvasive prenatal paternity tests
Response to Drábek and Cereda
Regulatory changes raise troubling questions for genomic testing
News Briefs
In This Issue
Vascular Ehlers–Danlos syndrome, pixels, and high-definition clinical genomics
Michael J. Fox Foundation LRRK2 Consortium: geographical differences in returning genetic research data to study participants
Response to Pyeritz et al.
Loeys–Dietz syndrome is a specific phenotype and not a concomitant of any mutation in a gene involved in TGF-β signaling
In this Issue
News Briefs
Utility before business
The business of genomic testing: a survey of early adopters
Panel testing for inherited susceptibility to breast, ovarian, and colorectal cancer
Correction: Corrigendum: Mutations in NGLY1 cause an inherited disorder of the endoplasmic reticulum–associated degradation pathway
News Briefs
In this Issue
Pregnancy-related deaths and complications in women with vascular Ehlers–Danlos syndrome
Survival is affected by mutation type and molecular mechanism in vascular Ehlers–Danlos syndrome (EDS type IV)
The usefulness of whole-exome sequencing in routine clinical practice
Accurate mitochondrial DNA sequencing using off-target reads provides a single test to identify pathogenic point mutations
In This Issue
News Briefs
How do research participants perceive “uncertainty” in genome sequencing?
Postanalytical tools improve performance of newborn screening by tandem mass spectrometry
Ethnic effect on FMR1 carrier rate and AGG repeat interruptions among Ashkenazi women Ethnic; Ashkenazi women
Translational research is a key to nongeneticist physicians’ genomics education
Targeted next-generation sequencing of deafness genes in hearing-impaired individuals uncovers informative mutations
Public preferences for the return of research results in genetic research: a conjoint analysis
Comparison of risk management strategies between women testing positive for a BRCA variant of unknown significance and women with known BRCA deleterious mutations
Multifactorial beliefs about the role of genetics and behavior in common health conditions: prevalence and associations with participant characteristics and engagement in health behaviors
Exploring pathways to trust: a tribal perspective on data sharing
Noninvasive prenatal testing for autosomal recessive conditions by maternal plasma sequencing in a case of congenital deafness
Genetic factors affecting statin concentrations and subsequent myopathy: a HuGENet systematic review
Genome-wide karyomapping accurately identifies the inheritance of single-gene defects in human preimplantation embryos in vitro
Comprehensive and quantitative multilocus methylation analysis reveals the susceptibility of specific imprinted differentially methylated regions to aberrant methylation in Beckwith–Wiedemann syndrome
In This Issue
News Briefs
Michael Kaback: people and places
Direct-to-patient disclosure of results of mismatch repair screening for Lynch syndrome via electronic personal health record: a feasibility study
The implications of familial incidental findings from exome sequencing: the NIH Undiagnosed Diseases Program experience
Framework for development of physician competencies in genomic medicine: report of the Competencies Working Group of the Inter-Society Coordinating Committee for Physician Education in Genomics
Genetic legacy and the genomic future
Unaffected family members report improvements in daily routine sun protection 2 years following melanoma genetic testing
Underutilization of specimens in biobanks: an ethical as well as a practical concern?
A germline missense mutation in COQ6 is associated with susceptibility to familial schwannomatosis
Utilization of multigene panels in hereditary cancer predisposition testing: analysis of more than 2,000 patients
In This Issue
Correction: Corrigendum: Phenylalanine hydroxylase deficiency: diagnosis and management guideline
News Briefs
The natural history of MPS I: global perspectives from the MPS I Registry
Deep sequencing with intronic capture enables identification of an APC exon 10 inversion in a patient with polyposis
A single center’s experience with noninvasive prenatal testing
The shifting model in clinical diagnostics: how next-generation sequencing and families are altering the way rare diseases are discovered, studied, and treated
Clinical course of patients with Fabry disease who were switched from agalsidase-β to agalsidase-α
Comparing universal Lynch syndrome tumor-screening programs to evaluate associations between implementation strategies and patient follow-through
American College of Medical Genetics and Genomics guideline for the clinical evaluation and etiologic diagnosis of hearing loss
Mutations in NGLY1 cause an inherited disorder of the endoplasmic reticulum–associated degradation pathway
Acute care utilization for inherited metabolic diseases among children identified through newborn screening in New York state
Genetics and the Veterans Health Administration
Identity-by-descent–guided mutation analysis and exome sequencing in consanguineous families reveals unusual clinical and molecular findings in retinal dystrophy
The impact of chromosomal microarray on clinical management: a retrospective analysis
Social and behavioral research in genomic sequencing: approaches from the Clinical Sequencing Exploratory Research Consortium Outcomes and Measures Working Group
News Briefs
In This Issue
Integration of targeted sequencing and NIPT into clinical practice in a Chinese family with maple syrup urine disease
Autosomal recessive POLR1D mutation with decrease of TCOF1 mRNA is responsible for Treacher Collins syndrome
A clinical scoring system to identify patients with sebaceous neoplasms at risk for the Muir–Torre variant of Lynch syndrome
Involvement of DNMT3B in the pathogenesis of Hirschsprung disease and its possible role as a regulator of neurogenesis in the human enteric nervous system
Loeys–Dietz syndrome: a primer for diagnosis and management
Results of the College of American Pathology/American College of Medical Genetics and Genomics external proficiency testing from 2006 to 2013 for three conditions prevalent in the Ashkenazi Jewish pop Ashkenazi Jewish population
Genetic, cell biological, and clinical interrogation of theCFTR mutation c.3700 A>G (p.Ile1234Val) informs strategies for future medical intervention
PALB2 sequencing in Italian familial breast cancer cases reveals a high-risk mutation recurrent in the province of Bergamo
Nicotinic acetylcholine receptors in human genetic disease
Clinical whole-exome sequencing: are we there yet?
Fetoplacental mosaicism: potential implications for false-positive and false-negative noninvasive prenatal screening results
Appreciating the broad clinical features of SMAD4 mutation carriers: a multicenter chart review
The landscape of genetic variation in dilated cardiomyopathy as surveyed by clinical DNA sequencing
Delivery of clinical genetic consultative services in the Veterans Health Administration
The growing role of professional societies in educating clinicians in genomics
Erratum: Exonic duplication CNV of NDRG1 associated with autosomal-recessive HMSN-Lom/CMT4D
News Briefs
Erratum: Processes and preliminary outputs for identification of actionable genes as incidental findings in genomic sequence data in the Clinical Sequencing Exploratory Research Consortium
Announcement
In This Issue
Prostate cancer incidence in males with Lynch syndrome
Attitudes of parents toward the return of targeted and incidental genomic research findings in children
Correction: Corrigendum: Single-nucleotide polymorphism array genotyping is equivalent to metaphase cytogenetics for diagnosis of Turner syndrome
News Briefs
In This Issue
Noninvasive prenatal diagnosis in a fetus at risk for methylmalonic acidemia
Horizon scanning for translational genomic research beyond bench to bedside
Guidelines for return of research results from pediatric genomic studies: deliberations of the Boston Children’s Hospital Gene Partnership Informed Cohort Oversight Board
Predictive genetic testing experience for myocilin primary open-angle glaucoma using the Australian and New Zealand Registry of Advanced Glaucoma
Assessing the necessity of confirmatory testing for exome-sequencing results in a clinical molecular diagnostic laboratory
Recommendations for the nutrition management of phenylalanine hydroxylase deficiency
Phenylalanine hydroxylase deficiency: diagnosis and management guideline
Mining the human genome after Association for Molecular Pathology v. Myriad Genetics
Cystic fibrosis carrier screening in a North American population
Large numbers of individuals are required to classify and define risk for rare variants in known cancer risk genes
The Angelina effect: immediate reach, grasp, and impact of going public
Angelina Jolie’s faulty gene: newspaper coverage of a celebrity’s preventive bilateral mastectomy in Canada, the United States, and the United Kingdom
Developing a public health-tracking system for follow-up of newborn screening metabolic conditions: a four-state pilot project structure and initial findings
ACMG technical standards and guidelines for genetic testing for inherited colorectal cancer (Lynch syndrome, familial adenomatous polyposis, and MYH-associated polyposis)
Preferences for results delivery from exome sequencing/genome sequencing
A multicenter open-label treatment protocol (HGT-GCB-058) of velaglucerase alfa enzyme replacement therapy in patients with Gaucher disease type 1: safety and tolerability
In silico tools for splicing defect prediction: a survey from the viewpoint of end users
Jaffe–Campanacci syndrome, revisited: detailed clinical and molecular analyses determine whether patients have neurofibromatosis type 1, coincidental manifestations, or a distinct disorder
The economic value of personalized medicine tests: what we know and what we need to know
Economic analyses of genetic tests in personalized medicine: clinical utility first, then cost utility
A multicenter, open-label study evaluating safety and clinical outcomes in children (1.4–7.5 years) with Hunter syndrome receiving idursulfase enzyme replacement therapy
Public opinion on policy issues in genetics and genomics
Parents’ perceptions of genetics services for congenital heart disease: the role of demographic, clinical, and psychological factors in determining service attendance
Reduction in newborn screening metabolic false-positive results following a new collection protocol
A prospective study of the clinical utility of prenatal chromosomal microarray analysis in fetuses with ultrasound abnormalities and an exploration of a framework for reporting unclassified variants a
Impact of prenatal technologies on the sex ratio in India: an overview
Prenatal technologies and the sex ratio
Informed consent for return of incidental findings in genomic research
Phenotypic consequences in black South African Fanconi anemia patients homozygous for a founder mutation
Opportunities to improve recruitment into medical genetics residency programs: survey results of program directors and medical genetics residents
22q13.2q13.32 genomic regions associated with severity of speech delay, developmental delay, and physical features in Phelan–McDermid syndrome
Exonic duplication CNV of NDRG1 associated with autosomal-recessive HMSN-Lom/CMT4D
The integration of next-generation sequencing panels in the clinical cancer genetics practice: an institutional experience
Similar prevalence of expanded CGG repeat lengths in the fragile X mental retardation I gene among infertile women and among women with proven fertility: a prospective study
Patient decisions for disclosure of secondary findings among the first 200 individuals undergoing clinical diagnostic exome sequencing
Determining pathogenicity of genetic variants in hypertrophic cardiomyopathy: importance of periodic reassessment
Communication of genetic test results to family and health-care providers following disclosure of research results
Maternal plasma DNA testing for aneuploidy in pregnancies achieved by assisted reproductive technologies
Familial incidence and associated symptoms in a population of individuals with nonsyndromic craniosynostosis
Recommendations from the EGAPP Working Group: does PCA3 testing for the diagnosis and management of prostate cancer improve patient health outcomes?
Processes and factors involved in decisions regarding return of incidental genomic findings in research
Factors affecting maternal participation in the genetic component of the National Birth Defects Prevention Study—United States, 1997–2007
“Is a cure in my sight?” Multi-stakeholder perspectives on phase I choroideremia gene transfer clinical trials
Mutation spectrum and differential gene expression in cystic and solid vestibular schwannoma
Population-based carrier screening for cystic fibrosis: a systematic review of 23 years of research
Effectiveness of a Web-based genomics training for health educators in Texas
Risk estimates for complex disorders: comparing personal genome testing and family history
Study of phenotype evolution during childhood in Marfan syndrome to improve clinical recognition
Noninvasive prenatal testing: limitations and unanswered questions
Promoting the participant–researcher partnership
Factors influencing organizational adoption and implementation of clinical genetic services
Frequency of Prader–Willi syndrome in births conceived via assisted reproductive technology
The utility of the traditional medical genetics diagnostic evaluation in the context of next-generation sequencing for undiagnosed genetic disorders
Decision-making process for conditions nominated to the Recommended Uniform Screening Panel: statement of the US Department of Health and Human Services Secretary’s Advisory Committee on Heritable Dis
Patient management and the association of less common familial Mediterranean fever symptoms with other disorders
Subjectivity in chromosome band–level estimation: a multicenter study
Medical management adherence as an outcome of genetic counseling in a pediatric setting
Understanding of informed consent by parents of children enrolled in a genetic biobank
Variations in predicted risks in personal genome testing for common complex diseases
An Ashkenazi Jewish SMN1 haplotype specific to duplication alleles improves pan-ethnic carrier screening for spinal muscular atrophy Ashkenazi Jewish
Neonatal hypocalcemia, neonatal seizures, and intellectual disability in 22q11.2 deletion syndrome
A cancer genetics toolkit improves access to genetic services through documentation and use of the family history by primary-care clinicians
Diagnostic yield of array comparative genomic hybridization in adults with autism spectrum disorders
Next-generation carrier screening
The recurrent distal 22q11.2 microdeletions are often de novo and do not represent a single clinical entity: a proposed categorization system
Parents’ interest in whole-genome sequencing of newborns
Single-nucleotide polymorphism array genotyping is equivalent to metaphase cytogenetics for diagnosis of Turner syndrome
Endoscopic evaluation of gastrointestinal tract in patients with hereditary hemorrhagic telangiectasia and correlation with their genotypes
Effectiveness of oncogenetics training on general practitioners’ consultation skills: a randomized controlled trial
Three-year experience of a CAP/ACMG methods-based external proficiency testing program for laboratories offering DNA sequencing for rare inherited disorders
Population-based estimates of the prevalence of FMR1 expansion mutations in women with early menopause and primary ovarian insufficiency
The use of US health insurance data for surveillance of rare disorders: hereditary hemorrhagic telangiectasia
Public preferences regarding informed consent models for participation in population-based genomic research