Genetics in Medicine - 2023

223 articles | Last updated: 2025-12-03 14:12:56
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T A T A T A T A
Editorial Board
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Editorial Board
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Mouse and human studies support DSTYK loss of function as a low-penetrance and variable expressivity risk factor for congenital urinary tract anomalies
De novo PHF5A variants are associated with craniofacial abnormalities, developmental delay, and hypospadias
Calibrating variant curation by clinical context based on factors that influence patients’ tolerance of uncertainty
Lessons learned from the first national population-based genetic carrier-screening program for Duchenne muscular dystrophy
Functional evaluation allows ACMG/AMP-based re-classification of CNGA3 variants associated with achromatopsia
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ATP2B2 de novo variants as a cause of variable neurodevelopmental disorders that feature dystonia, ataxia, intellectual disability, behavioral symptoms, and seizures
Missense variants in ANKRD11 cause KBG syndrome by impairment of stability or transcriptional activity of the encoded protein
Heterozygous UCHL1 loss-of-function variants cause a neurodegenerative disorder with spasticity, ataxia, neuropathy, and optic atrophy
Breast cancer polygenic risk scores derived in White European populations are not calibrated for women of Ashkenazi Jewish descent White European populations; Ashkenazi Jewish descent
Long-term cost-effectiveness of a melanoma prevention program using genomic risk information compared with standard prevention advice in Australia
Long-term safety and efficacy of pegunigalsidase alfa: A multicenter 6-year study in adult patients with Fabry disease
Alpelisib for treatment of patients with PIK3CA-related overgrowth spectrum (PROS)
A test of automated use of electronic health records to aid in diagnosis of genetic disease
“I don’t need any more unknowns hanging over my head”: Views of patients with cancer on variants of uncertain significance and low/moderate risk results from genomic sequencing
Exome and genome sequencing for rare genetic disease diagnosis: A scoping review and critical appraisal of clinical guidance documents produced by genetics professional organizations
Elucidating the clinical and molecular spectrum of SMARCC2-associated NDD in a cohort of 65 affected individuals
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Low adenoma burden in unselected patients with a pathogenic APC variant
Multilevel barriers and facilitators to widespread use of preconception carrier screening in the United States
The landscape of reported VUS in multi-gene panel and genomic testing: Time for a change
Response to Benn et al
Correspondence on “Maternal carrier screening with single-gene NIPS provides accurate fetal risk assessments for recessive conditions” by Hoskovec et al
Factors that differentiate cancer risk management decisions among females with pathogenic/likely pathogenic variants in PALB2, CHEK2, and ATM
Autosomal dominant Zellweger spectrum disorder caused by de novo variants in PEX14 gene
Genomic testing for suspected monogenic kidney disease in children and adults: A health economic evaluation
Implementing preconception expanded carrier screening in a universal health care system: A model-based cost-effectiveness analysis
Management of individuals with germline pathogenic/likely pathogenic variants in CHEK2: A clinical practice resource of the American College of Medical Genetics and Genomics (ACMG)
Characterization of POT1 tumor predisposition syndrome: Tumor prevalence in a clinically diverse hereditary cancer cohort
Clinical, neuroradiological, and molecular characterization of mitochondrial threonyl-tRNA-synthetase (TARS2)-related disorder
What's in a name? Justifying terminology for genomic findings beyond the initial test indication: A scoping review
Phenylalanine hydroxylase deficiency treatment and management: A systematic evidence review of the American College of Medical Genetics and Genomics (ACMG)
HOXD13-associated synpolydactyly: Extending and validating the genotypic and phenotypic spectrum with 38 new and 49 published families
Most people share genetic test results with relatives even if the findings are normal: Family communication in a diverse population
Defining the clinical validity of genes reported to cause pulmonary arterial hypertension
The contribution of medical burden to 22q11.2 deletion syndrome quality of life and functioning
Provision and availability of genomic medicine services in Level IV neonatal intensive care units
De novo PHF5A variants are associated with craniofacial abnormalities, developmental delay, and hypospadias
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Missense variants in RPH3A cause defects in excitatory synaptic function and are associated with a clinically variable neurodevelopmental disorder
Correspondence on “Points to consider in the practice of postmortem genetic testing: A statement of the American College of Medical Genetics and Genomics (ACMG)” by Deignan, et al
Noninvasive prenatal screening (NIPS) for fetal chromosome abnormalities in a general-risk population: An evidence-based clinical guideline of the American College of Medical Genetics and Genomics (AC
ACMG SF v3.2 list for reporting of secondary findings in clinical exome and genome sequencing: A policy statement of the American College of Medical Genetics and Genomics (ACMG)
Phenotypic presentation of Mendelian disease across the diagnostic trajectory in electronic health records
Breast cancer risk stratification using genetic and non-genetic risk assessment tools for 246,142 women in the UK Biobank
Clinical genome sequencing: Three years’ experience at a tertiary children’s hospital
Contemplating syndromic autism
Rare variants found in clinical gene panels illuminate the genetic and allelic architecture of orofacial clefting
A scoping review of nutrition issues and management strategies in individuals with skeletal dysplasia
Association of deep phenotyping with diagnostic yield of prenatal exome sequencing for fetal brain abnormalities
Updated recommendations for CFTR carrier screening: A position statement of the American College of Medical Genetics and Genomics (ACMG)
Providing genetic testing and genetic counseling for Parkinson’s disease to the community
Correspondence on “Loss-of-function variants in SRRM2 cause a neurodevelopmental disorder” by Cuinat et al.
Response to Chunquan Cai et al
Correspondence on “Noninvasive prenatal screening (NIPS) for fetal chromosome abnormalities in a general-risk population: An evidence-based clinical guideline of the American College of Medical Geneti
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Phenotypic continuum between Waardenburg syndrome and idiopathic hypogonadotropic hypogonadism in humans with SOX10 variants
Response to Prensky and Persson
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Response to Spurdle et al
Correspondence on “Technical standards for the interpretation and reporting of constitutional copy-number variants: a joint consensus recommendation of the American College of Medical Genetics and Gen
Education and electronic medical records and genomics network, challenges, and lessons learned from a large-scale clinical trial using polygenic risk scores
Response to Beretich and Beretich
Correspondence on “The evolving role of medical geneticists in the era of gene therapy: An urgency to prepare” by Vockley et al
Biallelic variants in NSUN6 cause an autosomal recessive neurodevelopmental disorder
Optimization of polygenic risk scores in BRCA1/2 pathogenic variant heterozygotes in epithelial ovarian cancer
Information-seeking preferences in diverse patients receiving a genetic testing result in the Clinical Sequencing Evidence-Generating Research (CSER) study
Biallelic variants in ribonuclease inhibitor (RNH1), an inflammasome modulator, are associated with a distinctive subtype of acute, necrotizing encephalopathy
Committing to genomic answers for all kids: Evaluating inequity in genomic research enrollment
Meta-analysis of the diagnostic and clinical utility of exome and genome sequencing in pediatric and adult patients with rare diseases across diverse populations
Inactivation of DRG1, encoding a translation factor GTPase, causes a recessive neurodevelopmental disorder
A new neurodevelopmental disorder linked to heterozygous variants in UNC79
Poison exon annotations improve the yield of clinically relevant variants in genomic diagnostic testing
Molecular diagnostic yield of genome sequencing versus targeted gene panel testing in racially and ethnically diverse pediatric patients
Genotype-phenotype correlations in RHOBTB2-associated neurodevelopmental disorders
Identification of genetic variants and phenotypic characterization of a large cohort of patients with congenital hypopituitarism and related disorders
Performance of prenatal cfDNA screening for sex chromosomes
Validation of lung cancer polygenic risk scores in a high-risk case-control cohort
Pathogenic variants in the paired-related homeobox 1 gene (PRRX1) cause craniosynostosis with incomplete penetrance
A clinical screening tool to detect genetic cancer predisposition in pediatric oncology shows high sensitivity but can miss a substantial percentage of affected children
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Development and evaluation of a novel educational program for providers on the use of polygenic risk scores
DNA methylation episignature and comparative epigenomic profiling of HNRNPU-related neurodevelopmental disorder
Frequency of epistaxis and telangiectasia in patients with hereditary hemorrhagic telangiectasia (HHT) in comparison with the general population: Curaçao diagnostic criteria revisited
Reading skills in males with 47,XXY: Risk factors and the influence of hormonal replacement therapy (HRT)
Monoallelic loss-of-function BMP2 variants result in BMP2-related skeletal dysplasia spectrum
The value of intersectionality for genomic research on human behavior
eyeVarP: A computational framework for the identification of pathogenic variants specific to eye disease
Heterozygous pathogenic variants in POMC are not responsible for monogenic obesity: Implication for MC4R agonist use
Barriers to completion of expanded carrier screening in an inner city population
De novo variants in CNOT9 cause a neurodevelopmental disorder with or without epilepsy
Dominant-negative variants in CBX1 cause a neurodevelopmental disorder
Clinical and functional heterogeneity associated with the disruption of retinoic acid receptor beta
Clinical, technical, and environmental biases influencing equitable access to clinical genetics/genomics testing: A points to consider statement of the American College of Medical Genetics and Genomic
Genotypic and phenotypic spectrum of infantile liver failure due to pathogenic TRMU variants
The behavioral profile of 49,XXXXY and the potential impact of testosterone replacement therapy
Breast cancer polygenic risk scores derived in White European populations are not calibrated for women of Ashkenazi Jewish descent White European populations; Ashkenazi Jewish descent
Route of delivery does not impact postnatal surgical morbidity in pregnancies affected by fetal achondroplasia
Contributions from medical geneticists in clinical trials of genetic therapies: A points to consider statement of the American College of Medical Genetics and Genomics (ACMG)
Clinical implications of conflicting variant interpretations in the cancer genetics clinic
De novo missense variants in RRAGC lead to a fatal mTORopathy of early childhood
LHX2 haploinsufficiency causes a variable neurodevelopmental disorder
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De novo variants in MRTFB have gain-of-function activity in Drosophila and are associated with a novel neurodevelopmental phenotype with dysmorphic features
Biallelic variants in CRIPT cause a Rothmund-Thomson-like syndrome with increased cellular senescence
YWHAE loss of function causes a rare neurodevelopmental disease with brain abnormalities in human and mouse
The 2019 medical genetics workforce: A focus on laboratory geneticists
Laboratory perspectives in the development of polygenic risk scores for disease: A points to consider statement of the American College of Medical Genetics and Genomics (ACMG)
Individualized interventions for rare genetic conditions and the research-treatment spectrum: Stakeholder perspectives
Microcosting diagnostic genomic sequencing: A systematic review
Evaluation of an automated genome interpretation model for rare disease routinely used in a clinical genetic laboratory
Validation of the International Breast Cancer Intervention Study (IBIS) model in the High Risk Ontario Breast Screening Program: A retrospective cohort study
How do members of the public expect to use variants of uncertain significance in their health care? A population-based survey
The clinical application of polygenic risk scores: A points to consider statement of the American College of Medical Genetics and Genomics (ACMG)
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Biallelic variants in ADAMTS15 cause a novel form of distal arthrogryposis
Cascade testing after exome sequencing: Retrospective analysis of linked family data at 2 US laboratories
Points to consider in the practice of postmortem genetic testing: A statement of the American College of Medical Genetics and Genomics (ACMG)
The effect of genetic education on the referral of patients to genetic evaluation: Findings from a national survey of nephrologists
Economic evaluation of population-based, expanded reproductive carrier screening for genetic diseases in Australia
Implementing digital systems to facilitate genetic testing for hereditary cancer syndromes: An observational study of 4 clinical workflows
Practical considerations for reinterpretation of individual genetic variants
Primary care physician use of patient race and polygenic risk scores in medical decision-making
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Impact of integrated translational research on clinical exome sequencing
Discovering a new part of the phenotypic spectrum of Coffin-Siris syndrome in a fetal cohort
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Updated clinical practice recommendations for managing children with 22q11.2 deletion syndrome
Updated clinical practice recommendations for managing adults with 22q11.2 deletion syndrome
Benefits, harms, and costs of newborn genetic screening for hypertrophic cardiomyopathy: Estimates from the PreEMPT model
Pathogenic variants in CLXN encoding the outer dynein arm docking–associated calcium-binding protein calaxin cause primary ciliary dyskinesia
Insurance denials and diagnostic rates in a pediatric genomic research cohort
A Solve-RD ClinVar-based reanalysis of 1522 index cases from ERN-ITHACA reveals common pitfalls and misinterpretations in exome sequencing
Exploring Autistic adults’ perspectives on genetic testing for autism
Diagnosis, treatment and disclosure: A qualitative exploration of participant challenges in a Monogenic Diabetes Registry
The evolving role of medical geneticists in the era of gene therapy: An urgency to prepare
Predictors of genetic risk recall among the participants of a randomized controlled precision prevention trial against melanoma
Cardiomyopathy prevalence exceeds 30% in individuals with TTN variants and early atrial fibrillation
Returning integrated genomic risk and clinical recommendations: The eMERGE study
The splicing effect of variants at branchpoint elements in cancer genes
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Laboratory diagnosis of biotinidase deficiency, 2017 update: a technical standard and guideline of the American College of Medical Genetics and Genomics
The designated record set for clinical genetic and genomic testing: A points to consider statement of the American College of Medical Genetics and Genomics (ACMG)
Safety and efficacy of avalglucosidase alfa in individuals with infantile-onset Pompe disease enrolled in the phase 2, open-label Mini-COMET study: The 6-month primary analysis report
Parents’ decision-making regarding whether to receive adult-onset only genetic findings for their children: Findings from the BabySeq Project
Clustered variants in the 5′ coding region of TRA2B cause a distinctive neurodevelopmental syndrome
Informational needs of individuals from families harboring BRCA pathogenic variants: A systematic review and content analysis
Solid organ transplantation in methylmalonic acidemia and propionic acidemia: A points to consider statement of the American College of Medical Genetics and Genomics (ACMG)
Optical genome mapping and revisiting short-read genome sequencing data reveal previously overlooked structural variants disrupting retinal disease−associated genes
Noninvasive prenatal screening (NIPS) for fetal chromosome abnormalities in a general-risk population: An evidence-based clinical guideline of the American College of Medical Genetics and Genomics (AC
Biallelic variants in OGDH encoding oxoglutarate dehydrogenase lead to a neurodevelopmental disorder characterized by global developmental delay, movement disorder, and metabolic abnormalities
Words matter: The language of difference in human genetics
Hospital-level variation in genetic testing in children’s hospitals’ neonatal intensive care units from 2016 to 2021
FDA approval summary for lonafarnib (Zokinvy) for the treatment of Hutchinson-Gilford progeria syndrome and processing-deficient progeroid laminopathies
Points to consider in the detection of germline structural variants using next-generation sequencing: A statement of the American College of Medical Genetics and Genomics (ACMG)
The PrU: Development and validation of a measure to assess personal utility of genomic results
Optimization of the biochemical genetics laboratory rotation using a multidesign approach to curriculum
Precision medicine for developmental and epileptic encephalopathies in Africa—strategies for a resource-limited setting
A comparative analysis of RAS variants in patients with disorders of somatic mosaicism
Functional genomics for curation of variants in telomere biology disorder associated genes: A systematic review
Continuous Bayesian variant interpretation accounts for incomplete penetrance among Mendelian cardiac channelopathies
Rare disease therapeutics: The future of medical genetics in a changing landscape
OXGR1 is a candidate disease gene for human calcium oxalate nephrolithiasis
Access to clinically indicated genetic tests for pediatric patients with Medicaid: Evidence from outpatient genetics clinics in Texas
An intervention strategy to improve genetic testing for dilated cardiomyopathy in a heart failure clinic
Neurofilament light chain in cerebrospinal fluid as a novel biomarker in evaluating both clinical severity and therapeutic response in Niemann-Pick disease type C1
Natural history of TANGO2 deficiency disorder: Baseline assessment of 73 patients
Long-term effects of eliglustat on skeletal manifestations in clinical trials of patients with Gaucher disease type 1
A concurrent dual analysis of genomic data augments diagnoses: Experiences of 2 clinical sites in the Undiagnosed Diseases Network
Incidental molecular diagnoses and heterozygous risk alleles in a carrier screening cohort
Maternal carrier screening with single-gene NIPS provides accurate fetal risk assessments for recessive conditions
Evidence and recommendation for mucopolysaccharidosis type II newborn screening in the United States
Importance of adopting standardized MANE transcripts in clinical reporting
Intermediate repeat expansions of TBP and STUB1: Genetic modifier or pure digenic inheritance in spinocerebellar ataxias?
Biallelic variants in HECT E3 paralogs, HECTD4 and UBE3C, encoding ubiquitin ligases cause neurodevelopmental disorders that overlap with Angelman syndrome
Evaluating brain white matter hyperintensity, IQ scores, and plasma neurofilament light chain concentration in early-treated patients with infantile-onset Pompe disease
Moving toward more consistency in variant classification and clinical action
Biallelic PRMT7 pathogenic variants are associated with a recognizable syndromic neurodevelopmental disorder with short stature, obesity, and craniofacial and digital abnormalities
DNA methylation episignature for Witteveen-Kolk syndrome due to SIN3A haploinsufficiency
Correspondence on “Frequency of truncating FLCN variants and Birt-Hogg-Dubé-associated phenotypes in a health care system population” by Savatt et al
“Extremely slow and capricious”: A qualitative exploration of genetic researcher priorities in selecting shared data resources
Response to van Riel et al
Correspondence on “The views of people with a lived experience of deafness and the general public regarding genetic testing for deafness in the reproductive setting: A systematic review” by Freeman et
Response to Li and Sun
Phenotypic shift in copy number variants: Evidence in 16p11.2 duplication syndrome
Diagnosing, discarding, or de-VUSsing: A practical guide to (un)targeted metabolomics as variant-transcending functional tests
Expanding SPTAN1 monoallelic variant associated disorders: From epileptic encephalopathy to pure spastic paraplegia and ataxia
Models of communication for polygenic scores and associated psychosocial and behavioral effects on recipients: A systematic review
Biallelic variants in PIGN cause Fryns syndrome, multiple congenital anomalies-hypotonia-seizures syndrome, and neurologic phenotypes: A genotype–phenotype correlation study
Delineation of a KDM2B-related neurodevelopmental disorder and its associated DNA methylation signature
Brain monoamine vesicular transport disease caused by homozygous SLC18A2 variants: A study in 42 affected individuals
Genotypic and phenotypic spectrum of infantile liver failure due to pathogenic TRMU variants
Informing variant assessment using structured evidence from prior classifications (PS1, PM5, and PVS1 sequence variant interpretation criteria)
Genetics professionals are key to the integration of genetic testing within the practice of frontline clinicians
Damaging variants in FOXI3 cause microtia and craniofacial microsomia
COVID-19 in people with neurofibromatosis 1, neurofibromatosis 2, or schwannomatosis
Standardizing variation: Scaling up clinical genomics in Australia