Genetics in Medicine - 2017

228 articles | Last updated: 2025-12-03 14:12:56
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T A T A T A T A
Causes of death in Prader-Willi syndrome: Prader-Willi Syndrome Association (USA) 40-year mortality survey
News Briefs
News Briefs
In This Issue
In This Issue
News Briefs
Management of ophthalmologic manifestations of mitochondrial diseases
Response to Newman et al.
News Briefs
In This Issue
Is it time to retire fragile X testing as a first-tier test for developmental delay, intellectual disability, and autism spectrum disorder?
Response to Hartley et al. and Mullegama et al.
Fragile X testing as a second-tier test
In This Issue
Response to Koeller et al.
News Briefs
In This Issue
Correction: Corrigendum: The expanding clinical phenotype of Bosch-Boonstra-Schaaf optic atrophy syndrome: 20 new cases and possible genotype–phenotype correlations
Diagnosis and treatment of tyrosinemia type I: a US and Canadian consensus group review and recommendations
Health effects of the CPT1A P479L variant: responsible public health policy
Should we implement population screening for fragile X?
Patient care standards for primary mitochondrial disease: a consensus statement from the Mitochondrial Medicine Society
Diagnostic cytogenetic testing following positive noninvasive prenatal screening results: a clinical laboratory practice resource of the American College of Medical Genetics and Genomics (ACMG)
A new era in the interpretation of human genomic variation
In This Issue
News Briefs
Laboratory diagnosis of biotinidase deficiency, 2017 update: a technical standard and guideline of the American College of Medical Genetics and Genomics
Quantifying survival in patients with Proteus syndrome
Response to Herbert et al.
Informed decision making and psychosocial outcomes in pregnant and nonpregnant women offered population fragile X carrier screening
Temple syndrome: comprehensive molecular and clinical findings in 32 Japanese patients
The importance of communicating histories of gender assignment and reassignment to genetic laboratories
Pitfalls of trio-based exome sequencing: imprinted genes and parental mosaicism—MAGEL2 as an example
Report on the Banbury Summit Meeting on medical genetics training in the genomic era, 23–26 February 2014
Treatment of mucopolysaccharidosis type II (Hunter syndrome): results from a systematic evidence review
Genome-wide cfDNA screening: clinical laboratory experience with the first 10,000 cases
Medical genetics and genomics education: how do we define success? Where do we focus our resources?
Oral D-galactose supplementation in PGM1-CDG
Utilization of genomic sequencing for population screening of immunodeficiencies in the newborn
In This Issue
News Briefs
Sources of discordance among germ-line variant classifications in ClinVar
Revisiting the prevalence of nonclassic congenital adrenal hyperplasia in US Ashkenazi Jews and Caucasians Ashkenazi Jews
Sharing data under the 21st Century Cures Act
A quantitative cSMART assay for noninvasive prenatal screening of autosomal recessive nonsyndromic hearing loss caused by GJB2 and SLC26A4 mutations
Do the data really support ordering fragile X testing as a first-tier test without clinical features?
Prenatal exome sequencing in anomalous fetuses: new opportunities and challenges
Using high-resolution variant frequencies to empower clinical genome interpretation
Population-based impact of noninvasive prenatal screening on screening and diagnostic testing for fetal aneuploidy
Gene patents still alive and kicking: their impact on provision of genetic testing for long QT syndrome in the Canadian public health-care system
In This Issue
Quantifying family dissemination and identifying barriers to communication of risk information in Australian BRCA families
Improving the evaluation of milestones for students completing a clinical genetics elective
Response to Laissue et al.
Sherloc: a comprehensive refinement of the ACMG–AMP variant classification criteria
Response to de Vries et al.
Correction: Corrigendum: ACMG recommendations for reporting of incidental findings in clinical exome and genome sequencing
Identifying “ownership” through role descriptions to support implementing universal colorectal cancer tumor screening for Lynch syndrome
The clinical spectrum and natural history of early-onset diseases due to DNA polymerase gamma mutations
Electronic health record phenotype in subjects with genetic variants associated with arrhythmogenic right ventricular cardiomyopathy: a study of 30,716 subjects with exome sequencing
A microcosting and cost–consequence analysis of clinical genomic testing strategies in autism spectrum disorder
Genomic sequencing in cystic fibrosis newborn screening: what works best, two-tier predefined CFTR mutation panels or second-tier CFTR panel followed by third-tier sequencing?
A novel approach in pediatric telegenetic services: geneticist, pediatrician and genetic counselor team
Assessment of the ExAC data set for the presence of individuals with pathogenic genotypes implicated in severe Mendelian pediatric disorders
Making genomic medicine evidence-based and patient-centered: a structured review and landscape analysis of comparative effectiveness research
ACMG secondary findings 2.0
Consensus recommendation for a diagnostic guideline for acid sphingomyelinase deficiency
Response to Biesecker
Molecular genetic findings and clinical correlations in 100 patients with Joubert syndrome and related disorders prospectively evaluated at a single center
CDH1 germline mutations: different syndromes, same management?
Whole-exome sequencing on deceased fetuses with ultrasound anomalies: expanding our knowledge of genetic disease during fetal development
Addressing a patient-controlled approach for genomic data sharing
Possible barriers for genetic counselors returning actionable genetic research results across state lines
Mutation of WIF1: a potential novel cause of a Nail-Patella-like disorder
In This Issue
The collaborative African genomics network training program: a trainee perspective on training the next generation of African scientists
Quantitative clinical characteristics of 53 patients with MPS VII: a cross-sectional analysis
News Briefs
Correction: Corrigendum: Commentary on the decision of the American Board of Medical Genetics and Genomics to create a 24-month specialty of Laboratory Genetics and Genomics
Correction: Corrigendum: Recommendations for reporting of secondary findings in clinical exome and genome sequencing, 2016 update (ACMG SF v2.0): a policy statement of the American College of Medical
Autozygosity reveals recessive mutations and novel mechanisms in dominant genes: implications in variant interpretation
Mosaic trisomy 16: what are the obstetric and long-term childhood outcomes?
Exome sequencing–based molecular autopsy of formalin-fixed paraffin-embedded tissue after sudden death
21-Gene recurrence score testing among Medicare beneficiaries with breast cancer in 2010–2013
A clinical utility study of exome sequencing versus conventional genetic testing in pediatric neurology
Genetic susceptibility to bilateral tinnitus in a Swedish twin cohort
Prediction of breast cancer risk based on flow-variant analysis of circulating peripheral blood B cells
Blepharocheilodontic syndrome is a CDH1 pathway–related disorder due to mutations in CDH1 and CTNND1
The psychosocial effects of the Li-Fraumeni Education and Early Detection (LEAD) program on individuals with Li-Fraumeni syndrome
Exploring the feasibility of delivering standardized genomic care using ophthalmology as an example
Clinical laboratories collaborate to resolve differences in variant interpretations submitted to ClinVar
News Briefs
In This Issue
ADDENDUM: Reviewer Acknowledgment 2016
The Exome Clinic and the role of medical genetics expertise in the interpretation of exome sequencing results
A familial study of azoospermic men identifies three novel causative mutations in three new human azoospermia genes
Molecular diagnosis of PIK3CA-related overgrowth spectrum (PROS) in 162 patients and recommendations for genetic testing
Efficacy of early treatment in patients with cobalamin C disease identified by newborn screening: a 16-year experience
Clinical and molecular consequences of disease-associated de novo mutations in SATB2
In This Issue
News Briefs
Prospective comparison of the cost-effectiveness of clinical whole-exome sequencing with that of usual care overwhelmingly supports early use and reimbursement
Motivations of participants in the citizen science of microbiomics: data from the British Gut Project
Next-generation sequencing improves thalassemia carrier screening among premarital adults in a high prevalence population: the Dai nationality, China
Cognitive outcomes and age of detection of severe mucopolysaccharidosis type 1
Assigning clinical meaning to somatic and germ-line whole-exome sequencing data in a prospective cancer precision medicine study
Genome editing in clinical genetics: points to consider—a statement of the American College of Medical Genetics and Genomics
Carnitine palmitoyltransferase 1A P479L and infant death: policy implications of emerging data
Utilization of genetic tests: analysis of gene-specific billing in Medicare claims data
GUCA1A mutation causes maculopathy in a five-generation family with a wide spectrum of severity
Frequent inactivating germline mutations in DNA repair genes in patients with Ewing sarcoma
The next generation of population-based spinal muscular atrophy carrier screening: comprehensive pan-ethnic SMN1 copy-number and sequence variant analysis by massively parallel sequencing
Recontacting participants for expanded uses of existing samples and data: a case study
Conflicts of interest in genetic counseling: acknowledging and accepting
Promoting appropriate genetic testing: the impact of a combined test review and consultative service
Clinical utility of next-generation sequencing for inherited bone marrow failure syndromes
Mutation spectrum in the ABCC6 gene and genotype–phenotype correlations in a French cohort with pseudoxanthoma elasticum
Transthyretin V122I (pV142I)* cardiac amyloidosis: an age-dependent autosomal dominant cardiomyopathy too common to be overlooked as a cause of significant heart disease in elderly African Americans African Americans
A taxonomy of medical uncertainties in clinical genome sequencing
The clinical utility of DNA-based screening for fetal aneuploidy by primary obstetrical care providers in the general pregnancy population
A curated gene list for reporting results of newborn genomic sequencing
Genome sequencing and carrier testing: decisions on categorization and whether to disclose results of carrier testing
PhenX measures for phenotyping rare genetic conditions
The current state of implementation science in genomic medicine: opportunities for improvement
In This Issue
News Briefs
Reviewer Acknowledgment 2016
Correction: Corrigendum: Low-pass whole-genome sequencing in clinical cytogenetics: a validated approach
Participant-perceived understanding and perspectives on pharmacogenomics: the Mayo Clinic RIGHT protocol (Right Drug, Right Dose, Right Time)
Laboratory and clinical genomic data sharing is crucial to improving genetic health care: a position statement of the American College of Medical Genetics and Genomics
Laboratory diagnosis of creatine deficiency syndromes: a technical standard and guideline of the American College of Medical Genetics and Genomics
The value of genetic testing: beyond clinical utility
Public variant databases: liability?
Population screening for BRCA1/BRCA2 founder mutations in Ashkenazi Jews: proactive recruitment compared with self-referral Ashkenazi Jews
The Precision Medicine Initiative’s All of Us Research Program: an agenda for research on its ethical, legal, and social issues
Evaluating the quality of Marfan genotype–phenotype correlations in existing FBN1 databases
The cost of molecular-guided therapy in oncology: a prospective cost study alongside the MOSCATO trial
Microduplication of the ARID1A gene causes intellectual disability with recognizable syndromic features
Population screening for BRCA1/BRCA2 mutations: lessons from qualitative analysis of the screening experience
The ABMGG’s response to a commentary on the decision to create a 24-month specialty of Laboratory Genetics and Genomics
Recommendations for reporting of secondary findings in clinical exome and genome sequencing, 2016 update (ACMG SF v2.0): a policy statement of the American College of Medical Genetics and Genomics
Commentary on the decision of the American Board of Medical Genetics and Genomics to create a 24-month specialty of Laboratory Genetics and Genomics
Neuroimaging findings in Mowat–Wilson syndrome: a study of 54 patients
A survey of current practices for genomic sequencing test interpretation and reporting processes in US laboratories
Evaluation of a Web-based decision aid for people considering the APOE genetic test for Alzheimer risk
β-Thalassemia
Ciliopathy variant burden and developmental delay in children with hypoplastic left heart syndrome
Detection of clinically relevant copy-number variants by exome sequencing in a large cohort of genetic disorders
Precision engagement: the PMI’s success will depend on more than genomes and big data
A comprehensive strategy for exome-based preconception carrier screening
The need for a next-generation public health response to rare diseases
Phenotypic predictors and final diagnoses in patients referred for RASopathy testing by targeted next-generation sequencing
Managing sickle cell carrier results generated through newborn screening in Ontario: a precedent-setting policy story
Engagement and communication among participants in the ClinSeq Genomic Sequencing Study
Genetics/genomics education for nongenetic health professionals: a systematic literature review
Copy-number variation is an important contributor to the genetic causality of inherited retinal degenerations
Health screening behaviors among adults with hereditary hemorrhagic telangiectasia in North America
Experiences from a pilot program bringing BRCA1/2 genetic screening to the
Improving biobank consent comprehension: a national randomized survey to assess the effect of a simplified form and review/retest intervention
Early manifestations of type 1 Gaucher disease in presymptomatic children diagnosed after parental carrier screening
Numerous Brugada syndrome–associated genetic variants have no effect on J-point elevation, syncope susceptibility, malignant cardiac arrhythmia, and all-cause mortality
Genetic modifiers of CHEK2*1100delC-associated breast cancer risk
Universal tumor screening for Lynch syndrome: health-care providers’ perspectives
Increasing the sensitivity of clinical exome sequencing through improved filtration strategy
Streamlined genetic education is effective in preparing women newly diagnosed with breast cancer for decision making about treatment-focused genetic testing: a randomized controlled noninferiority tri
Intratumoral genetic heterogeneity and number of cytogenetic aberrations provide additional prognostic significance in chronic lymphocytic leukemia
Multidisciplinary model to implement pharmacogenomics at the point of care
Mastering genomic terminology
The collective impact of rare diseases in Western Australia: an estimate using a population-based cohort
The validation of pharmacogenetics for the identification of Fabry patients to be treated with migalastat
Successful outcomes of older adolescents and adults with profound biotinidase deficiency identified by newborn screening
Payer decision making for next-generation sequencing–based genetic tests: insights from cell-free DNA prenatal screening
Whole-exome sequencing in the molecular diagnosis of individuals with congenital anomalies of the kidney and urinary tract and identification of a new causative gene
All your data (effectively) belong to us: data practices among direct-to-consumer genetic testing firms
Ethical considerations for genetic testing in the context of mandated cardiac screening before athletic participation
Using large sequencing data sets to refine intragenic disease regions and prioritize clinical variant interpretation
Prescription medication changes following direct-to-consumer personal genomic testing: findings from the Impact of Personal Genomics (PGen) Study
A dominant variant in DMXL2 is linked to nonsyndromic hearing loss
My46: a Web-based tool for self-guided management of genomic test results in research and clinical settings
Clinical relevance of small copy-number variants in chromosomal microarray clinical testing
Engaging Māori in biobanking and genomic research: a model for biobanks to guide culturally informed governance, operational, and community engagement activities
Genome-wide multilocus imprinting disturbance analysis in Temple syndrome and Kagami-Ogata syndrome
Loss-of-function mutations in the X-linked biglycan gene cause a severe syndromic form of thoracic aortic aneurysms and dissections
Switch to agalsidase alfa after shortage of agalsidase beta in Fabry disease: a systematic review and meta-analysis of the literature
A secondary benefit: the reproductive impact of carrier results from newborn screening for cystic fibrosis
arrEYE: a customized platform for high-resolution copy number analysis of coding and noncoding regions of known and candidate retinal dystrophy genes and retinal noncoding RNAs
Estimation of the number of people with Down syndrome in the United States
Stakeholder views on secondary findings in whole-genome and whole-exome sequencing: a systematic review of quantitative and qualitative studies
Accuracy and clinical value of maternal incidental findings during noninvasive prenatal testing for fetal aneuploidies
Milestones for medical students completing a clinical genetics elective
Effect of decision aid for breast cancer prevention on decisional conflict in women with a BRCA1 or BRCA2 mutation: a multisite, randomized, controlled trial
Exploring the importance of case-level clinical information for variant interpretation
Eliciting preferences on secondary findings: the Preferences Instrument for Genomic Secondary Results
Reassessment of Mendelian gene pathogenicity using 7,855 cardiomyopathy cases and 60,706 reference samples
Assessing the capability of massively parallel sequencing for opportunistic pharmacogenetic screening
Genetic testing and genetic counseling for amyotrophic lateral sclerosis: an update for clinicians
Obesity in adults with 22q11.2 deletion syndrome
A placebo-controlled trial of simvastatin therapy in Smith-Lemli-Opitz syndrome
Candidate-gene criteria for clinical reporting: diagnostic exome sequencing identifies altered candidate genes among 8% of patients with undiagnosed diseases
Personalized risk prediction for type 2 diabetes: the potential of genetic risk scores
Development of carrier testing for common inborn errors of metabolism in the Wisconsin Plain population
NR5A1 is a novel disease gene for 46,XX testicular and ovotesticular disorders of sex development
Modeling the prevention of colorectal cancer from the combined impact of host and behavioral risk factors
Distributive justice, diversity, and inclusion in precision medicine: what will success look like?
Is “incidental finding” the best term?: a study of patients’ preferences
EFHC1 variants in juvenile myoclonic epilepsy: reanalysis according to NHGRI and ACMG guidelines for assigning disease causality
The significant impact of education, poverty, and race on Internet-based research participant engagement
Macrocephaly associated with the DICER1 syndrome
Systematic reanalysis of clinical exome data yields additional diagnoses: implications for providers
Standardizing terms for clinical pharmacogenetic test results: consensus terms from the Clinical Pharmacogenetics Implementation Consortium (CPIC)
How genetic testing can lead to targeted management of XIAP deficiency–related inflammatory bowel disease
Prospective phenotyping of NGLY1-CDDG, the first congenital disorder of deglycosylation
Prediction of CYP2D6 phenotype from genotype across world populations
Pompe disease in adulthood: effects of antibody formation on enzyme replacement therapy
Electronic health record interventions at the point of care improve documentation of care processes and decrease orders for genetic tests commonly ordered by nongeneticists
Cell-free DNA testing of an extended range of chromosomal anomalies: clinical experience with 6,388 consecutive cases
The genetic basis of classic nonketotic hyperglycinemia due to mutations in GLDC and AMT
Newborn screening for X-linked adrenoleukodystrophy: evidence summary and advisory committee recommendation
Comprehensive genetic analysis of pregnancy loss by chromosomal microarrays: outcomes, benefits, and challenges
Newborn genetic screening for hearing impairment: a population-based longitudinal study
Variants of uncertain significance in newborn screening disorders: implications for large-scale genomic sequencing
Tracheal cartilaginous sleeves in children with syndromic craniosynostosis
The phenotypic spectrum of Schaaf-Yang syndrome: 18 new affected individuals from 14 families
Molecular characterization of NRXN1 deletions from 19,263 clinical microarray cases identifies exons important for neurodevelopmental disease expression
Enrichment of mutations in chromatin regulators in people with Rett syndrome lacking mutations in MECP2
Breast cancer risk prediction using a polygenic risk score in the familial setting: a prospective study from the Breast Cancer Family Registry and kConFab
Cystic fibrosis newborn screening programs: implications of the CFTR variant spectrum in nonwhite patients nonwhite
Rare genetic variants in cellular transporters, metabolic enzymes, and nuclear receptors can be important determinants of interindividual differences in drug response