Genetics in Medicine - 2016

236 articles | Last updated: 2025-12-03 14:12:56
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T A T A T A T A
In This Issue
News Briefs
Correction: Corrigendum: Distributive justice, diversity, and inclusion in precision medicine: what will success look like?
Correction: Corrigendum: Prediction of CYP2D6 phenotype from genotype across world populations
In This Issue
News Briefs
In This Issue
Congenital heart defects in Noonan syndrome and RIT1 mutation
Correction: Corrigendum: The phenotypic spectrum of Schaaf-Yang syndrome: 18 new affected individuals from 14 families
Response to Calgani et al.
News Briefs
Neonates at risk of medium-chain acyl-CoA dehydrogenase deficiency: a perinatal protocol for use before population neonatal screening test results become available
Response to van Rijt et al.
Imprinting: the Achilles heel of trio-based exome sequencing
Epidemiology matters: peering inside the “black box” in economic evaluations of genetic testing
News Briefs
In This Issue
Long-term follow-up in newborn screening: the role of collaboration
A panoramic view of the accuracy of molecular genetic testing
News Briefs
In This Issue
Correction: Corrigendum: Comprehensive analysis via exome sequencing uncovers genetic etiology in autosomal recessive nonsyndromic deafness in a large multiethnic cohort
2016 ACMG Annual Meeting presidential address: the practice of medical genetics: myths and realities
Noninvasive prenatal screening for fetal aneuploidy, 2016 update: a position statement of the American College of Medical Genetics and Genomics
Response to Sierra-Delgado et al.
Use of bioinformatic tools in primer validation
Correction: Corrigendum: Phenotype-driven gene target definition in clinical genome-wide sequencing data interpretation
ADDENDUM: Technical standards and guidelines for spinal muscular atrophy testing
In This Issue
News Briefs
Erratum: Defective hepatic bicarbonate production due to carbonic anhydrase VA deficiency leads to early-onset life-threatening metabolic crisis
In This Issue
Correction: Corrigendum: Generating and evaluating evidence of the clinical utility of molecular diagnostic tests in oncology
A knowledge base for tracking the impact of genomics on population health
De novo mutations in autosomal recessive congenital malformations
What are people willing to pay for whole-genome sequencing information, and who decides what they receive?
Pharmacogenomic incidental findings in 308 families: The NIH Undiagnosed Diseases Program experience
Results from an external proficiency testing program: 11 years of molecular genetics testing for myotonic dystrophy type 1
Navigating highly homologous genes in a molecular diagnostic setting: a resource for clinical next-generation sequencing
Is it Fabry disease?
Inborn Errors of Metabolism Collaborative: large-scale collection of data on long-term follow-up for newborn-screened conditions
Erratum: Section E6.1–6.4 of the ACMG technical standards and guidelines: chromosome studies of neoplastic blood and bone marrow–acquired chromosomal abnormalities
Recommendations for the integration of genomics into clinical practice
Clinical outcomes of children with abnormal newborn screening results for Krabbe disease in New York State
Morbidity and mortality among exclusively breastfed neonates with medium-chain acyl-CoA dehydrogenase deficiency
CADD score has limited clinical validity for the identification of pathogenic variants in noncoding regions in a hereditary cancer panel
Family health history reporting is sensitive to small changes in wording
Correction: Corrigendum: Pathogenic and likely pathogenic variant prevalence among the first 10,000 patients referred for next-generation cancer panel testing
In This Issue
News Briefs
Highly accurate molecular genetic testing for HFE hereditary hemochromatosis: results from 10 years of blinded proficiency surveys by the College of American Pathologists
Comparative analysis of BRCA1 and BRCA2 variants of uncertain significance in patients with breast cancer: a multifactorial probability-based model versus ACMG standards and guidelines for interpretin
Section E6.1–6.4 of the ACMG technical standards and guidelines: chromosome studies of neoplastic blood and bone marrow–acquired chromosomal abnormalities
Clinical genomics can facilitate countrywide estimation of autosomal recessive disease burden
A standardized, evidence-based protocol to assess clinical actionability of genetic disorders associated with genomic variation
Section E6.5–6.8 of the ACMG technical standards and guidelines: chromosome studies of lymph node and solid tumor–acquired chromosomal abnormalities
Genotype and phenotype in patients with Noonan syndrome and a RIT1 mutation
Research participant interest in primary, secondary, and incidental genomic findings
Response to Patryn and Zagaja
Live births, natural losses, and elective terminations with Down syndrome in Massachusetts
Which BRCA genetic testing programs are ready for implementation in health care? A systematic review of economic evaluations
Incorporating truncating variants in PALB2, CHEK2, and ATM into the BOADICEA breast cancer risk model
It’s time to ramp up genetic counseling training
Designing babies through gene editing: science or science fiction?
Carrier screening in the era of expanding genetic technology
Biobanks and consent with a terminal clause
In This Issue
Phenotype-driven gene target definition in clinical genome-wide sequencing data interpretation
Erratum: Cystic fibrosis on the African continent
Low risk of solid tumors in persons with Down syndrome
News Briefs
Targeted next-generation sequencing makes new molecular diagnoses and expands genotype–phenotype relationship in Ehlers–Danlos syndrome
Parents’ experiences with requesting carrier testing for their unaffected children
Patient safety in genomic medicine: an exploratory study
The expanding clinical phenotype of Bosch-Boonstra-Schaaf optic atrophy syndrome: 20 new cases and possible genotype–phenotype correlations
Overcalling secondary findings
An openly available online tool for implementing the ACMG/AMP standards and guidelines for the interpretation of sequence variants
Novel mutations in LRP6 highlight the role of WNT signaling in tooth agenesis
Archibald E. Garrod: the father of precision medicine
Response to Cunningham et al.
Role of medical food in MMA
Response to Biesecker
Towards a more representative morphology: clinical and ethical considerations for including diverse populations in diagnostic genetic atlases
The complex behavioral phenotype of 15q13.3 microdeletion syndrome
Clinical utility of a Web-enabled risk-assessment and clinical decision support program
Expanded carrier screening in an infertile population: how often is clinical decision making affected?
An electronic atlas of human malformation syndromes in diverse populations
Cost-effectiveness of routine screening for Lynch syndrome in colorectal cancer patients up to 70 years of age
A prospective evaluation of whole-exome sequencing as a first-tier molecular test in infants with suspected monogenic disorders
Announcement
In this Issue
Erratum: Observed and expected frequencies of structural hemoglobin variants in newborn screening surveys in Africa and the Middle East: deviations from Hardy-Weinberg equilibrium
Contribution of genomic copy-number variations in prenatal oral clefts: a multicenter cohort study
Molecular diagnosis of glycogen storage disease and disorders with overlapping clinical symptoms by massive parallel sequencing
Defective hepatic bicarbonate production due to carbonic anhydrase VA deficiency leads to early-onset life-threatening metabolic crisis
Taking advantage of an old concept, “illegitimate transcription”, for a proposed novel method of genetic diagnosis of McArdle disease
Recontact in clinical practice: a survey of clinical genetics services in the United Kingdom
Implementation of the 21-gene recurrence score test in the United States in 2011
Establishing the precise evolutionary history of a gene improves prediction of disease-causing missense mutations
Patients with colorectal cancer associated with Lynch syndrome and MLH1 promoter hypermethylation have similar prognoses
The Geisinger MyCode community health initiative: an electronic health record–linked biobank for precision medicine research
Oncologists’ and cancer patients’ views on whole-exome sequencing and incidental findings: results from the CanSeq study
Effectiveness of whole-exome sequencing and costs of the traditional diagnostic trajectory in children with intellectual disability
New targets for monitoring and therapy in Barth syndrome
Should states adopt newborn screening for early infantile Krabbe disease?
Improving performance of multigene panels for genomic analysis of cancer predisposition
In this Issue
News Briefs
Adopting genetics: motivations and outcomes of personal genomic testing in adult adoptees
Evidence for an association between infant mortality and homozygosity for the arctic variant of carnitine palmitoyltransferase 1A
Low-pass whole-genome sequencing in clinical cytogenetics: a validated approach
Predictive genetic testing of minors: evidence and experience with families
Cardiovascular malformations caused by NOTCH1 mutations do not keep left: data on 428 probands with left-sided CHD and their families
Hypomorphic mutations identified in the candidate Leber congenital amaurosis gene CLUAP1
Response to Sahoo et al.
“You want the right amount of oversight”: interviews with data access committee members and experts on genomic data access
Newborn screening for Krabbe disease in New York State: the first eight years’ experience
Diagnostic exome sequencing provides a molecular diagnosis for a significant proportion of patients with epilepsy
Expanding noninvasive prenatal testing to include microdeletions and segmental aneuploidy: cause for concern?
Privacy-preserving genomic testing in the clinic: a model using HIV treatment
Expanding the clinical and mutational spectrum of the Ehlers–Danlos syndrome, dermatosparaxis type
Parental DNA sequence is critical family history in clinical genomics
ADDENDUM: Technical standards and guidelines for myotonic dystrophy type 1 testing
Correction: Corrigendum: The effect of genotypes and parent of origin on cancer risk and age of cancer development in PMS2 mutation carriers
Reviewer Acknowledgment 2015
News Briefs
Erratum: Novel mutations in genes causing hereditary spastic paraplegia and Charcot-Marie-Tooth neuropathy identified by an optimized protocol for homozygosity mapping based on whole-exome sequencing
ADDENDUM: Laboratory guideline for Turner syndrome
In this Issue
Expanding the genetic counseling workforce: program directors’ views on increasing the size of genetic counseling graduate programs
Identification of microsatellite markers <1 Mb from the FMR1 CGG repeat and development of a single-tube tetradecaplex PCR panel of highly polymorphic markers for preimplantation genetic diagnosis
The psychological impact of genetic information on children: a systematic review
Mosaic mutations in early-onset genetic diseases
Evaluation of the incremental prognostic value of the combination of CYP2C19 poor metabolizer status and ABCB1 3435 TT polymorphism over conventional risk factors for cardiovascular events after drug- East Asians
Pharmacogenomic biomarkers as inclusion criteria in clinical trials of oncology-targeted drugs: a mapping of ClinicalTrials.gov
VisCap: inference and visualization of germ-line copy-number variants from targeted clinical sequencing data
Exploring the landscape of pathogenic genetic variation in the ExAC population database: insights of relevance to variant classification
Characterization of individuals at high risk of developing melanoma in Latin America: bases for genetic counseling in melanoma
Pathogenic and likely pathogenic variant prevalence among the first 10,000 patients referred for next-generation cancer panel testing
Newborn testing and screening by whole-genome sequencing
Direct-to-consumer genetic testing: a revised position statement of the American College of Medical Genetics and Genomics
Gene discovery for Mendelian conditions via social networking: de novo variants in KDM1A cause developmental delay and distinctive facial features
Ethics of children’s participation in a Saudi biobank: an exploratory survey
Damaged goods?: an empirical cohort study of blood specimens collected 12 to 23 hours after birth in newborn screening in California
Cystic fibrosis on the African continent
Ashkenazi carrier screening for reproductive planning: is this what we planned for? Ashkenazi
Clinical utility of genetic and genomic services: context matters
Response to Zlotogora and Meiner
Accelerating matchmaking of novel dysmorphology syndromes through clinical and genomic characterization of a large cohort
Clinical application of whole-exome sequencing across clinical indications
Economic evaluation of whole-genome sequencing in healthy individuals: what can we learn from CEAs of whole-body CT screening?
Observed and expected frequencies of structural hemoglobin variants in newborn screening surveys in Africa and the Middle East: deviations from Hardy-Weinberg equilibrium
Generating and evaluating evidence of the clinical utility of molecular diagnostic tests in oncology
The global prevalence of HFE and non-HFE hemochromatosis estimated from analysis of next-generation sequencing data
Molecular diagnostic experience of whole-exome sequencing in adult patients
What works in genomics education: outcomes of an evidenced-based instructional model for community-based physicians
Institutional review board perspectives on obligations to disclose genetic incidental findings to research participants
Using a gene expression signature when controversy exists regarding the indication for adjuvant systemic treatment reduces the proportion of patients receiving adjuvant chemotherapy: a nationwide stud
A systematic literature review of individuals’ perspectives on broad consent and data sharing in the United States
Computational evaluation of exome sequence data using human and model organism phenotypes improves diagnostic efficiency
Improving hearing loss gene testing: a systematic review of gene evidence toward more efficient next-generation sequencing–based diagnostic testing and interpretation
Participant use and communication of findings from exome sequencing: a mixed-methods study
Clinical follow-up and breast and ovarian cancer screening of true BRCA1/2 noncarriers: a qualitative investigation
The promise and peril of genomic screening in the general population
Bias in CFTR screening panels
Perceived economic burden associated with an inherited cardiac condition: a qualitative inquiry with families affected by arrhythmogenic right ventricular cardiomyopathy
Response to Sosnay et al.
Novel mutations in genes causing hereditary spastic paraplegia and Charcot-Marie-Tooth neuropathy identified by an optimized protocol for homozygosity mapping based on whole-exome sequencing
Retraction Note: Retraction notice for: “Validation of a multiplex genotyping platform using a novel genomic database approach”
Your DNA is not your diagnosis: getting diagnoses right following secondary genomic findings
Multigene panels in prostate cancer risk assessment: a systematic review
Cesarean delivery is not associated with decreased at-birth fracture rates in osteogenesis imperfecta
Capture-based high-coverage NGS: a powerful tool to uncover a wide spectrum of mutation types
Comprehensive genetic exploration of skeletal dysplasia using targeted exome sequencing
The role of combined SNV and CNV burden in patients with distal symmetric polyneuropathy
Expanding the clinical, allelic, and locus heterogeneity of retinal dystrophies
Expanded genetic screening panel for the Ashkenazi Jewish population
Development and preliminary evaluation of an online educational video about whole-genome sequencing for research participants, patients, and the general public
LINE-1 in cancer: multifaceted functions and potential clinical implications
Utility of whole-genome sequencing for detection of newborn screening disorders in a population cohort of 1,696 neonates
Rapid progression and mortality of lysosomal acid lipase deficiency presenting in infants
A germline chromothripsis event stably segregating in 11 individuals through three generations
The genetic landscape of Alzheimer disease: clinical implications and perspectives
Genetic causal attributions for weight status and weight loss during a behavioral weight gain prevention intervention
What is the role of clinical genetics in the patient-centered medical home?: A commentary from the Medical Home Workgroup of the Heartland Regional Genetics and Newborn Screening Collaborative
A critical reappraisal of dietary practices in methylmalonic acidemia raises concerns about the safety of medical foods. Part 2: cobalamin C deficiency
A semiquantitative metric for evaluating clinical actionability of incidental or secondary findings from genome-scale sequencing
A critical reappraisal of dietary practices in methylmalonic acidemia raises concerns about the safety of medical foods. Part 1: isolated methylmalonic acidemias
Maternal uniparental disomy of chromosome 20: a novel imprinting disorder of growth failure
The current state of cancer genetic counseling access and availability
Comprehensive analysis via exome sequencing uncovers genetic etiology in autosomal recessive nonsyndromic deafness in a large multiethnic cohort
The Cockayne Syndrome Natural History (CoSyNH) study: clinical findings in 102 individuals and recommendations for care
The time-consuming demands of the practice of medical genetics in the era of advanced genomic testing
Mutations in JMJD1C are involved in Rett syndrome and intellectual disability
Shift happens: family background influences clinical variability in genetic neurodevelopmental disorders
Is prenatal cytogenetic diagnosis with genomic array indicated in pregnancies at risk for a molecular or metabolic disorder?
Aortic dilation, genetic testing, and associated diagnoses
POLE and POLD1 mutations in 529 kindred with familial colorectal cancer and/or polyposis: review of reported cases and recommendations for genetic testing and surveillance
The effect of genotypes and parent of origin on cancer risk and age of cancer development in PMS2 mutation carriers
Health-care professionals’ responsibility to patients’ relatives in genetic medicine: a systematic review and synthesis of empirical research
Prevalence of meconium ileus marks the severity of mutations of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene
Cystic fibrosis carrier screening effects on birth prevalence and newborn screening
Individual DNA samples and health information sold by 23andMe
Fetal growth and gestational factors as predictors of schizophrenia in 22q11.2 deletion syndrome
Parents’ experiences 12 years after newborn screening for genetic susceptibility to type 1 diabetes and their attitudes to whole-genome sequencing in newborns
Clinical phenotype of the recurrent 1q21.1 copy-number variant
State-offered ethnically targeted reproductive genetic testing
Mutational analysis of a cohort with clinical diagnosis of familial hypercholesterolemia: considerations for genetic diagnosis improvement
Age-related macular degeneration: genome-wide association studies to translation
Exome sequencing identifies rare variants in multiple genes in atrioventricular septal defect
Economic evidence on identifying clinically actionable findings with whole-genome sequencing: a scoping review
Efficacy of a medical genetics rotation during pediatric training
With great (participant) rights comes great (researcher) responsibility
Brugada syndrome: clinical and genetic findings
Does rapid genetic counseling and testing in newly diagnosed breast cancer patients cause additional psychosocial distress? results from a randomized clinical trial
A classification system for clinical relevance of somatic variants identified in molecular profiling of cancer
Targeted mutation screening panels expose systematic population bias in detection of cystic fibrosis risk
Clinical performance of the CytoScan Dx Assay in diagnosing developmental delay/intellectual disability
The Israeli national population program of genetic carrier screening for reproductive purposes
Stakeholder perspectives on implementing a universal Lynch syndrome screening program: a qualitative study of early barriers and facilitators
Differential diagnosis of Bartter syndrome, Gitelman syndrome, and pseudo–Bartter/Gitelman syndrome based on clinical characteristics
Family decision maker perspectives on the return of genetic results in biobanking research
Translating rare-disease therapies into improved care for patients and families: what are the right outcomes, designs, and engagement approaches in health-systems research?
PMS2 monoallelic mutation carriers: the known unknown
Outcomes of four patients with homocysteine remethylation disorders detected by newborn screening
Patient feedback and early outcome data with a novel tiered-binned model for multiplex breast cancer susceptibility testing
A phase I/II study of intrathecal idursulfase-IT in children with severe mucopolysaccharidosis II
Novel first-dose adverse drug reactions during a phase I trial of olipudase alfa (recombinant human acid sphingomyelinase) in adults with Niemann–Pick disease type B (acid sphingomyelinase deficiency)
The challenge of comprehensive and consistent sequence variant interpretation between clinical laboratories
Risk of new tumors in von Hippel–Lindau patients depends on age and genotype
Interactive multimedia consent for biobanking: a randomized trial
Difficulties in diagnosing Marfan syndrome using current FBN1 databases
Consumers report lower confidence in their genetics knowledge following direct-to-consumer personal genomic testing
Treacher Collins syndrome: a clinical and molecular study based on a large series of patients
High incidence of unrecognized visceral/neurological late-onset Niemann-Pick disease, type C1, predicted by analysis of massively parallel sequencing data sets
Improving newborn screening for cystic fibrosis using next-generation sequencing technology: a technical feasibility study