European Journal of Human Genetics - 2022

252 articles | Last updated: 2025-12-03 14:12:56
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T A T A T A T A
Challenges for the implementation of next generation sequencing-based expanded carrier screening: Lessons learned from the ciliopathies
Heterozygous and homozygous variants in STX1A cause a neurodevelopmental disorder with or without epilepsy
Research priorities in psychiatric genetic counselling: how to talk to children and adolescents about genetics and psychiatric disorders
Barriers and facilitators for the implementation of patient-centered care in cardiogenetics: a Delphi study among ERN GUARD-heart members
Functional analysis of PTEN variants of unknown significance from PHTS patients unveils complex patterns of PTEN biological activity in disease
Expanding the speech and language phenotype in Koolen-de Vries syndrome: late onset and periodic stuttering a novel feature
Correction to: Reply to Letter by Tellier et al., ‘Scientific refutation of ESHG statement on embryo selection’
Two unrelated cases with biallelic CHEK2 variants:a novel condition with constitutional chromosomal instability?
Gene selection by incorporating genetic networks into case-control association studies
Somatic and germinal mosaicism in a Han Chinese family with laminopathies
Panel testing for the molecular genetic diagnosis of congenital hypogonadotropic hypogonadism – a clinical perspective
The implementation of large-scale genomic screening or diagnostic programmes: A rapid evidence review
Pathogenic REST variant causing Jones syndrome and a review of the literature
Genetic testing for mitochondrial disease: the United Kingdom best practice guidelines
Dutch pharmacogenetics working group (DPWG) guideline for the gene-drug interaction of CYP2D6 and COMT with atomoxetine and methylphenidate
Scientific refutation of ESHG statement on embryo selection
Non-invasive prenatal testing in Germany: a unique ethical and policy landscape
Experiences of pregnant women with genome-wide non-invasive prenatal testing in a national screening program
Managing expectations, rights, and duties in large-scale genomics initiatives: a European comparison
Genome sequencing with gene panel-based analysis for rare inherited conditions in a publicly funded healthcare system: implications for future testing
High molecular diagnostic yields and novel phenotypic expansions involving syndromic anorectal malformations
Correction to: Evaluation of somatic and/or germline mosaicism in congenital malformation of the eye
Reply to Letter by Tellier et al., ‘Scientific refutation of ESHG statement on embryo selection’
ARHGAP35 is a novel factor disrupted in human developmental eye phenotypes
A second look at exome sequencing data: detecting mobile elements insertion in a rare disease cohort
LARS2 variants can present as premature ovarian insufficiency in the absence of overt hearing loss
The utility of population level genomic research
Examination of a novel expression-based gene-SNP annotation strategy to identify tissue-specific contributions to heritability in multiple traits
An spanish study of secondary findings in families affected with mendelian disorders: choices, prevalence and family history
Comparing saliva and blood for the detection of mosaic genomic abnormalities that cause syndromic intellectual disability
Dutch pharmacogenetics working group (DPWG) guideline for the gene–drug interaction between UGT1A1 and irinotecan
Millennium-old pathogenic Mendelian mutation discovery for multiple osteochondromas from a Gaelic Medieval graveyard
Variable number tandem repeats (VNTRs) as modifiers of breast cancer risk in carriers of BRCA1 185delAG
Expanding the genetics and phenotypic spectrum of Lysine-specific demethylase 5C (KDM5C): a report of 13 novel variants
Key drivers of family-level utility of pediatric genomic sequencing: a qualitative analysis to support preference research
The role of TBX18 in congenital heart defects in humans not confirmed
The more you do it, the easier it gets: using behaviour change theory to support health care professionals offering reproductive genetic carrier screening
Evaluation of somatic and/or germline mosaicism in congenital malformation of the eye
Patients carrying Arg1809 substitution with no choroidal abnormalities: a further proof of a “Quasi-Incomplete” NF1 phenotype
FarGen: Elucidating the distribution of coding variants in the isolated population of the Faroe Islands
Investigation on the high recurrence of the ATTRv-causing transthyretin variant Val142Ile in central Italy
Genome-wide association study of a lipedema phenotype among women in the UK Biobank identifies multiple genetic risk factors
A 25 Mainland Chinese cohort of patients with PURA-related neurodevelopmental disorders: clinical delineation and genotype–phenotype correlations
Is there a “pandemic effect” on individuals’ willingness to take genetic tests?
Short stature in PRMT7 Mutations: first evidence of response to growth hormone treatment
Not putting the cart before the horse: the complex social and ethical terrain of prenatal exome sequencing
Correction: Genetic discrimination still casts a large shadow in 2022
Lymphedema as first clinical presentation of Cantu Syndrome: reversed phenotyping after identification of gain-of-function variant in ABCC9
Genetic Counsellors play a key role in supporting ethically responsible expanded universal carrier screening
Genetic prediction of male pattern baldness based on large independent datasets European subjects
Genome sequencing—do you know what you are getting into?
Clinical management approaches in Bardet–Biedl syndrome
The genetic counsellor role in the United Kingdom
ParseCNV2: efficient sequencing tool for copy number variation genome-wide association studies
Scope of professional roles for genetic counsellors and clinical geneticists in the United Kingdom
Genome-wide identification of disease-causing copy number variations in 450 individuals with anorectal malformations
On Clustering of Juvenile Canavan disease in an Indian community due to population bottleneck and isolation: Genomic signatures of a founder event
Somatic genetic variation in healthy tissue and non-cancer diseases
Genome sequencing as a single comprehensive test in molecular diagnosis
A rare genotype of biallelic mosaic variants in BCOR gene causing a bilateral ocular anterior segment dysgenesis and cataracts
How does the genomic naive public perceive whole genomic testing for health purposes? A scoping review
Proband-mediated interventions to increase disclosure of genetic risk in families with a BRCA or Lynch syndrome condition: a systematic review
Biallelic loss of LDB3 leads to a lethal pediatric dilated cardiomyopathy
Updated Stroke Gene Panels: Rapid evolution of knowledge on monogenic causes of stroke
The phenotypic spectrum associated with loss-of-function variants in monogenic epilepsy genes in the general population
Correction to: Factors influencing the attainment of major motor milestones in CDKL5 deficiency disorder
Direct-to-consumer genetic testing: an updated systematic review of healthcare professionals’ knowledge and views, and ethical and legal concerns
Early illustrations of the importance of systematic phenotyping
Comment on: Disease gene identification strategies for exome sequencing by Gilissen et al. 2012
Commentary: Von Hippel–Lindau disease: A clinical and scientific review
The European Journal of Human Genetics is turning 30: a selection of major cancer genetics papers published by the Journal
Happy 30th birthday to the European Journal of Human Genetics! European Journal of Human Genetics
Whole-genome sequencing of multiple related individuals with type 2 diabetes reveals an atypical likely pathogenic mutation in the PAX6 gene
Clustering of Juvenile Canavan disease in an Indian community due to population bottleneck and isolation: genomic signatures of a founder event
Analysis of Fibroblast Growth Factor 14 (FGF14) structural variants reveals the genetic basis of the early onset nystagmus locus NYS4 and variable ataxia
The role of the Genetic Counsellor in the multidisciplinary team: the perception of geneticists in Europe
Identification and characterization of two DMD pedigrees with large inversion mutations based on a long-read sequencing pipeline
Exome sequencing as first-tier genetic testing in infantile-onset pharmacoresistant epilepsy: diagnostic yield and treatment impact
Research participants: critical friends, agents for change
Applying the Clinician-reported Genetic testing Utility InDEx (C-GUIDE) to genome sequencing: further evidence of validity
A qualitative study exploring the consumer experience of receiving self-initiated polygenic risk scores from a third-party website
Lessons learned during the process of reporting individual genomic results to participants of a population-based biobank
Assessing the digenic model in rare disorders using population sequencing data
Genetic discrimination still casts a large shadow in 2022
Identification and in-silico characterization of splice-site variants from a large cardiogenetic national registry
The performance of genome sequencing as a first-tier test for neurodevelopmental disorders
Prevalence of hereditary tubulointerstitial kidney diseases in the German Chronic Kidney Disease study
Societal implications of expanded universal carrier screening: a scoping review ancestry-based, ethnic stigmatization, at-risk populations
Correction: Changes in opinions about human germline gene editing as a result of the Dutch DNA-dialogue project
Exome sequencing for structurally normal fetuses—yields and ethical issues
A novel nonsense variant in the CENPP gene segregates in a Swiss family with autosomal dominant low-frequency sensorineural hearing loss
Developmental implications of genetic testing for physical indications
Approaching discussions about genetics with palliative patients and their families: a qualitative exploration with genetic health professionals
Ten years of dynamic consent in the CHRIS study: informed consent as a dynamic process
Dutch pharmacogenetics working group guideline for the gene-drug interaction of ABCG2, HLA-B and Allopurinol, and MTHFR, folic acid and methotrexate
Guidelines, guidelines everywhere—and still I’m not sure what to do
Rare autosomal trisomies detected by non-invasive prenatal testing
“Hypothesis: Patient with possible disturbance in programmed cell death”: further insights in pathogenicity and clinical features of Fraser syndrome
Correction to: Identification of novel genes whose expression in adipose tissue affects body fat mass and distribution: an RNA-Seq and Mendelian Randomization study
Should variants of unknown significance (VUS) be disclosed to patients in cardiogenetics or not; only in case of high suspicion of pathogenicity?
Spectrum of movement disorders and motor abnormalities in adults with a 22q11.2 microdeletion: Comment on the literature and retrospective study of 92 adults
Parents’ understanding of genome and exome sequencing for pediatric health conditions: a systematic review
Correction: The use of polygenic risk scores in pre-implantation genetic testing: an unproven, unethical practice
Factors influencing the attainment of major motor milestones in CDKL5 deficiency disorder
Identification of novel genes whose expression in adipose tissue affects body fat mass and distribution: an RNA-Seq and Mendelian Randomization study
Genetics follow up after rapid genomic sequencing in intensive care: current practices and recommendations for service delivery
Hemoglobinopathy screening in primary care in the Netherlands: exploring the problems and needs of patients and general practitioners
Unexpected diagnosis of myotonic dystrophy type 2 repeat expansion by genome sequencing
Whole exome and genome sequencing in mendelian disorders: a diagnostic and health economic analysis
KBG syndrome: videoconferencing and use of artificial intelligence driven facial phenotyping in 25 new patients
Exome sequencing—one test to rule them all?
The experiences of UK-based genetic counsellors working in mainstream settings
Minors at risk of von Hippel-Lindau disease: 10 years’ experience of predictive genetic testing and follow-up adherence
Rare autosomal trisomies detected by non-invasive prenatal testing: an overview of current knowledge
Variable expressivity in a four-generation ACDMPV family with a non-coding hypermorphic SNV in trans to the frameshifting FOXF1 variant
Health professionals’ views and experiences of the Australian moratorium on genetic testing and life insurance: A qualitative study
Views of Canadian healthcare professionals on the future uses of non-invasive prenatal testing: a mixed method study
Intragenic inversions in NF1 gene as pathogenic mechanism in neurofibromatosis type 1
Extending the PAX1 spectrum: a dominantly inherited variant causes oculo-auriculo-vertebral syndrome
The value of testing for ATXN2 intermediate repeat expansions in routine clinical practice for amyotrophic lateral sclerosis
School performance of children with neurofibromatosis 1: a nationwide population-based study
Fine mapping and accurate prediction of complex traits using Bayesian Variable Selection models applied to biobank-size data
An estimate of the cumulative paediatric prevalence of rare diseases in Ireland and comment on the literature
COVID-19: a challenge and an opportunity
‘Diagnostic shock’: the impact of results from ultrarapid genomic sequencing of critically unwell children on aspects of family functioning
A unique Smith-Magenis patient with a de novo intragenic deletion on the maternally inherited overexpressed RAI1 allele
Clinical genomics testing: mainstreaming and globalising
Host genomics of SARS-CoV-2 infection
A neurodevelopmental disorder caused by a novel de novo SVA insertion in exon 13 of the SRCAP gene
A novel HRAS c.466C>T p.(Phe156Leu) variant in two patients with attenuated features of Costello syndrome
The genetic and evolutionary determinants of COVID-19 susceptibility
Incidence of Duchenne muscular dystrophy in the modern era; an Australian study
Estimation of the number of inherited prion disease mutation carriers in the UK
Rapid exome sequencing in critically ill infants: implementation in routine care from French regional hospital’s perspective
Microsatellite instability in gastrointestinal cancers
Ethical, legal and social/societal implications (ELSI) of recall-by-genotype (RbG) and genotype-driven-research (GDR) approaches: a scoping review
Quality of life, illness perceptions, and parental lived experiences in TANGO2-related metabolic encephalopathy and arrhythmias
KERATIN 17-related recessive atypical pachyonychia congenita with variable hair and tooth anomalies
Splicing analyses for variants in MMR genes: best practice recommendations from the European Mismatch Repair Working Group
Functional characterization of a novel p.Ser76Thr variant in IGFBP4 that associates with body mass index in American Indians American Indians
Correction to: Estimation of the number of people with Down syndrome in Europe
What’s new in genetics in June 2022?
Correction to: Genetics etiologies and genotype phenotype correlations in a cohort of individuals with central conducting lymphatic anomaly
Compound heterozygous variants in MAN2B2 identified in a Chinese child with congenital disorders of glycosylation Chinese
Assessing the contribution of genetic nurture to refractive error
Host genetic basis of COVID-19: from methodologies to genes
An integrated multiomic approach as an excellent tool for the diagnosis of metabolic diseases: our first 3720 patients
Reply to Pubpeer anonymous contributors: incomplete penetrance and phenotypic variability of 6q16 deletions including SIM1
Genetics etiologies and genotype phenotype correlations in a cohort of individuals with central conducting lymphatic anomaly
Mild MDPL in a patient with a novel de novo missense variant in the Cys-B region of POLD1
Silencing XIST on the future active X: Searching human and bovine preimplantation embryos for the repressor
Ethically robust reproductive genetic carrier screening needs to measure outcomes that matter to patients
“Atypical” Krabbe disease in two siblings harboring biallelic GALC mutations including a deep intronic variant
A unique service: how an embedded psychology team can help patients and genetics clinicians within a clinical genetics service
Perceptions of causal attribution and attitudes to genetic testing among people with schizophrenia and their first-degree relatives
Same performance of exome sequencing before and after fetal autopsy for congenital abnormalities: toward a paradigm shift in prenatal diagnosis?
A comprehensive SARS-CoV-2 and COVID-19 review, Part 1: Intracellular overdrive for SARS-CoV-2 infection
Recommendations for whole genome sequencing in diagnostics for rare diseases
Changes in opinions about human germline gene editing as a result of the Dutch DNA-dialogue project
No gene to predict the future?
The genetics of hereditary cancer risk syndromes in Brazil: a comprehensive analysis of 1682 patients
Characterisation of a novel OPA1 splice variant resulting in cryptic splice site activation and mitochondrial dysfunction
Genetic analysis of the PCSK9 locus in psychological, psychiatric, metabolic and cardiovascular traits in UK Biobank
Returning individual research results in international direct-to-participant genomic research: results from a 31-country study
Exome sequencing in individuals with cardiovascular laterality defects identifies potential candidate genes
Association of microsatellite instability (MSI) status with the 5-year outcome and genetic ancestry in a large Brazilian cohort of colorectal cancer
Germline variant in Ctcf links mental retardation to Wilms tumor predisposition
Social motivation a relative strength in DYRK1A syndrome on a background of significant speech and language impairments
Correction: Incorporating patient perspectives in the development of a core outcome set for reproductive genetic carrier screening: a sequential systematic review
Assessment of psychosocial difficulties by genetic clinicians and distress in women at high risk of breast cancer: a prospective study
Predictive genetic testing for Motor neuron disease: time for a guideline?
Abstracts from the 54th European Society of Human Genetics (ESHG) Conference: Oral Presentations
Abstracts from the 54th European Society of Human Genetics (ESHG) Conference
Abstracts from the 54th European Society of Human Genetics (ESHG) Conference: e-Posters
16p13.11p11.2 triplication syndrome: a new recognizable genomic disorder characterized by optical genome mapping and whole genome sequencing
Bi-allelic variants in human TCTE1/DRC5 cause asthenospermia and male infertility
No April fools in clinical genomics
The natural history of adults with Rubinstein-Taybi syndrome: a families-reported experience
DNA methylation signature associated with Bohring-Opitz syndrome: a new tool for functional classification of variants in ASXL genes
ERN GENTURIS clinical practice guidelines for the diagnosis, treatment, management and surveillance of people with schwannomatosis
Recommendations for reporting results of diagnostic genomic testing
Novel genes bearing mutations in rare cases of early-onset ataxia with cerebellar hypoplasia
Coronavirus Host Genetics South Africa (COHG-SA) database—a variant database for gene regions associated with SARS-CoV-2 outcomes
Grandmaternal smoking during pregnancy is associated with differential DNA methylation in peripheral blood of their grandchildren
Incorporating patient perspectives in the development of a core outcome set for reproductive genetic carrier screening: a sequential systematic review
Allelic imbalance of HLA-B expression in human lung cells infected with coronavirus and other respiratory viruses
Trio-based exome sequencing reveals a high rate of the de novo variants in intellectual disability
Gene-lifestyle interactions in the genomics of human complex traits
Correction: Polygenic risk modeling for prediction of epithelial ovarian cancer risk
Biallelic PAN2 variants in individuals with a syndromic neurodevelopmental disorder and multiple congenital anomalies
Knowledge, attitudes and preferences regarding reproductive genetic carrier screening among reproductive-aged men and women in Flanders (Belgium)
Reviewer recognition
Correction: The use of polygenic risk scores in pre-implantation genetic testing: an unproven, unethical practice
The need for recognition of core professional groups in genetics healthcare services in Europe
Preferences for return of germline genome sequencing results for cancer patients and their genetic relatives in a research setting
Detection of a pathogenic Alu element insertion in PALB2 gene from targeted NGS diagnostic data
A human paradigm of LHX4 and NR5A1 developmental gene interaction in the pituitary gland and ovary?
Participant experiences of genome sequencing for rare diseases in the 100,000 Genomes Project: a mixed methods study
Good genotype-phenotype relationships in rare disease are hard to find
The 2019 and 2021 International Workshops on Alport Syndrome
Receiving results of uncertain clinical relevance from population genetic screening: systematic review & meta-synthesis of qualitative research
Exploring the ethics of genetic prioritisation for COVID-19 vaccines
Correction to: The stepwise process of integrating a genetic counsellor into primary care
Human mitochondrial RNA modifications associate with tissue-specific changes in gene expression, and are affected by sunlight and UV exposure
A novel in-frame GFAP p.E138_L148del mutation in Type II Alexander disease with atypical phenotypes
Correction: 2021 at European Journal of Human Genetics: the year in review
Demolishing the silo: towards team-based genomics in primary care
Healthcare professionals’ perceptions of implementing a decision support intervention for cascade screening for beta-thalassemia in Pakistan
Comment on Informing relatives of their genetic risk: an examination of the Belgian context
Genome-wide linkage analysis combined with genome sequencing in large families with intracranial aneurysms
Hemoglobinopathy prevention in primary care: a reflection of underdetection and difficulties with accessibility of medical care, a quantitative study Northern European countries
Cross-continental admixture in the Kho population from northwest Pakistan
Missense mutation of MAL causes a rare leukodystrophy similar to Pelizaeus-Merzbacher disease
An analysis of genetically regulated gene expression and the role of co-expression networks across 16 psychiatric and substance use phenotypes
Prevalent versus incident breast cancers: benefits of clinical and radiological monitoring in women with pathogenic BRCA1/2 variants
Comment on Future trends in clinical genetic and genomic services by Borle et al.
A new system for variant classification?
Consistency of variant interpretations among bioinformaticians and clinical geneticists in hereditary cancer panels
NIPT and the concerns regarding ‘routinisation’
The case for screening in early life for ‘non-treatable’ disorders: ethics, evidence and proportionality. A report from the Health Council of the Netherlands
ZNF711 puts a spell on DNA
Good quality practices for artificial intelligence in genetics
Breakpoints characterisation of the genomic deletions identified by MLPA in alkaptonuria patients
Mosaicism in PTEN—new case and comment on the literature
The stepwise process of integrating a genetic counsellor into primary care
Genetic diagnosis for rare diseases in the Dutch Caribbean: a qualitative study on the experiences and associated needs of parents
A novel variant in SMG9 causes intellectual disability, confirming a role for nonsense-mediated decay components in neurocognitive development
Leave no one behind: inclusion of alpha-1 antitrypsin deficiency patients in COVID-19 vaccine trials
Neonatal-lethal dilated cardiomyopathy due to a homozygous LMOD2 donor splice-site variant
Variant pathogenic prediction by locus variability: the importance of the current picture of evolution
Understanding the assumptions underlying Mendelian randomization
Outcomes of support groups for carriers of BRCA 1/2 pathogenic variants and their relatives: a systematic review
Clinical genetics: past, present and future
Index case identification and outcomes of cascade testing in high-risk breast and colorectal cancer predisposition genes
Prevalence and natural history of schwannomas in neurofibromatosis type 2 (NF2): the influence of pathogenic variants
Osteopathia striata with cranial sclerosis: a new case supporting the link with bilateral Wilms tumor
Bi-allelic SMO variants in hypothalamic hamartoma: a recessive cause of Pallister-Hall syndrome
Where is genetic medicine headed? Exploring the perspectives of Canadian genetic professionals on future trends using the Delphi method
A systematic review of geographical inequities for accessing clinical genomic and genetic services for non-cancer related rare disease
Polygenic risk modeling for prediction of epithelial ovarian cancer risk women of European ancestries; BRCA1 and BRCA2 pathogenic variant carriers of European ancestries; women of East Asian ancestries; women of African ancestries
The Young Geneticists Network and the ESHG-Young committee, a forward-looking international community
Novel biallelic variants expand the SLC5A6-related phenotypic spectrum
Complementing the phenotypical spectrum of TUBA1A tubulinopathy and its role in early-onset epilepsies from four European countries
Informing relatives of their genetic risk: an examination of the Belgian legal context
2021 at European Journal of Human Genetics: the year in review
Clinical findings and a DNA methylation signature in kindreds with alterations in ZNF711
Genetic counselling and testing for neurodegenerative disorders using a proposed standard of practice for ALS/MND: diagnostic testing comes first
Multilocus Inherited Neoplasia Allele Syndrome (MINAS): an update
A tool for translating polygenic scores onto the absolute scale using summary statistics
The simplest explanation does not have to be preferred: co-occurrence of pathogenic variants in cancer-predisposing genes
Novel insights into the consequences of obesity: a phenotype-wide Mendelian randomization study
Singleton exome sequencing of 90 fetuses with ultrasound anomalies revealing novel disease-causing variants and genotype–phenotype correlations