European Journal of Human Genetics - 2023

240 articles | Last updated: 2025-12-03 14:12:56
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Perception of genomic newborn screening among peripartum mothers
De novo variants underlying monogenic syndromes with intellectual disability in a neurodevelopmental cohort from India
Genetic basis of osteogenesis imperfecta from a single tertiary centre in South Africa mixed ancestry
Exome sequencing of ATP1A3-negative cases of alternating hemiplegia of childhood reveals SCN2A as a novel causative gene
Are disease-specific patient-reported outcomes measures (PROMs) used in cardiogenetics? A systematic review
Associating CYP2A6 structural variants with ovarian and lung cancer risk in the UK Biobank: replication and extension
Complex rearrangement in TBC1D4 in an individual with diabetes due to severe insulin resistance syndrome
Novel phenotype of SIN3A-related disorder diagnosed in adulthood with multi-system involvement
Glowing gels and pipettes aplenty: how do commercial stock image banks portray genetic tests?
Correction: CDK13-related disorder: a deep characterization of speech and language abilities and addition of 33 novel cases
Psychological impact of additional findings detected by genome-wide Non-Invasive Prenatal Testing (NIPT): TRIDENT-2 study
Surveillance of multiple congenital anomalies; searching for new associations
Association of variants in GJA8 with familial acorea-microphthalmia-cataract syndrome
Ethical considerations for genetic research in low-income countries: perceptions of informed consent, data sharing, and expectations in Nicaragua
Ambivalence and regret in genome sequencing
Correction: Clinical findings and a DNA methylation signature in kindreds with alterations in ZNF711
Comment on Gustavson syndrome is caused by an in-frame deletion in RBMX associated with potentially disturbed SH3 domain interactions
Correction: A framework for evaluating long-term impact of newborn screening
A 39 kb structural variant causing Lynch Syndrome detected by optical genome mapping and nanopore sequencing
The molecular landscape of oculocutaneous albinism in India and its therapeutic implications
Estimating the proportion of nonsense variants undergoing the newly described phenomenon of manufactured splice rescue
Biallelic variants in the synaptic vesicle glycoprotein 2 A are associated with epileptic encephalopathy
Correction: Mobile element insertions in rare diseases: a comparative benchmark and reanalysis of 60,000 exome samples
Commentary on Eichinger J, Zimmermann B, Elger B, McLennan S, Filges I, Koné I. 2023. ‘It’s a nightmare’: informed consent in paediatric genome-wide sequencing. A qualitative expert interview study fr
A comprehensive SARS-CoV-2 and COVID-19 review, Part 2: host extracellular to systemic effects of SARS-CoV-2 infection
Phenotypic similarity-based approach for variant prioritization for unsolved rare disease: a preliminary methodological report European population; European Reference Networks
Biallelic ATP2B1 variants as a likely cause of a novel neurodevelopmental malformation syndrome with primary hypoparathyroidism
Molecular investigation in individuals with orofacial clefts and microphthalmia-anophthalmia-coloboma spectrum
Deep phenotyping and population-level data can help resolve genomic variants
GWAS of lipids in Greenlanders finds association signals shared with Europeans and reveals an independent PCSK9 association signal Europeans (and "European data")
Advancing intercontinental collaboration in human genetics: success story of the African and European Young Investigator Forum
Bi-allelic truncating variants in CASP2 underlie a neurodevelopmental disorder with lissencephaly
Episignatures in practice: independent evaluation of published episignatures for the molecular diagnostics of ten neurodevelopmental disorders
Rapid genomic testing in critically ill patients with genetic conditions: position statement by the Human Genetics Society of Australasia
Parents’ attitudes towards research involving genome sequencing of their healthy children: a qualitative study
Mobile element insertions in rare diseases: a comparative benchmark and reanalysis of 60,000 exome samples
Specific measures for data-intensive health research without consent: a systematic review of soft law instruments and academic literature
“I just wanted more”: Hereditary cancer syndromes patients’ perspectives on the utility of circulating tumour DNA testing for cancer screening
A loss of function variant in AGPAT3 underlies intellectual disability and retinitis pigmentosa (IDRP) syndrome
Genomic medicine in neonatal care: progress and challenges
Ambivalence in genomic healthcare provision, cure or symptom?
Mitochondrial Cytochrome C deficiency can show the first disease signs in the prenatal stage
Two new patients with acromesomelic dysplasia, PRKG2 type—identification and characterization of the first missense variant
A framework for evaluating long-term impact of newborn screening
Knowledge, attitudes and decision regret: a longitudinal survey study of participants offered genome sequencing in the 100,000 Genomes Project
Expanding what we know about rare genetic diseases
Emerging cancer risks in BRCA2 pathogenic germline variant carriers
‘It’s a nightmare’: informed consent in paediatric genome-wide sequencing. A qualitative expert interview study from Germany and Switzerland
Risk perception and intended behavior change after uninformative genetic results for adult-onset hereditary conditions in unselected patients
A linear weighted combination of polygenic scores for a broad range of traits improves prediction of coronary heart disease European ancestry
Methylation signatures in clinically variable syndromic disorders: a familial DNMT3A variant in two adults with Tatton-Brown–Rahman syndrome
Goldilocks principle and recessive disease
Evaluation of CTRL: a web application for dynamic consent and engagement with individuals involved in a cardiovascular genetic disorders cohort
Börjeson–Forssman–Lehmann syndrome: delineating the clinical and allelic spectrum in 14 new families
The reuse of genetic information in research and informed consent
The molecular genetics of nELAVL in brain development and disease
A population-based survey of FBN1 variants in Iceland reveals underdiagnosis of Marfan syndrome
Variants in EFCAB7 underlie nonsyndromic postaxial polydactyly
Clinical exome sequencing efficacy and phenotypic expansions involving anomalous pulmonary venous return
Oro-dental phenotyping and report of three families with RELT-associated amelogenesis imperfecta
Valuable insights into hereditary spinocerebellar degeneration from clusters of homozygosity in Africa
CUX1-related neurodevelopmental disorder: deep insights into phenotype-genotype spectrum and underlying pathology
Clinical practice guidelines for the diagnosis and surveillance of BAP1 tumour predisposition syndrome
Palliative care and genetics: personalized medicine for the patient and family
Dealing with ambivalence in the practice of advanced genetic healthcare: towards an ethical choreography
Beyond the hype: a comprehensive critique of ChatGPT’s applicability in genetics
Importance of critical thinking to understand ChatGPT
Response to correspondence regarding “Analysis of large-language model versus human performance for genetics questions”
Metaplastic breast cancer and BRCA1: first strong evidence of a link
Record-matching of STR profiles with fragmentary genomic SNP data
Why don’t we all use genomic testing?
Novel homozygous variants in PRORP expand the genotypic spectrum of combined oxidative phosphorylation deficiency 54
A new impact factor for EJHG in 2022
Consanguinity and willingness to perform premarital genetic screening in Sudan
Genomic testing for rare disease diagnosis—where are we now, and where should we be heading? The reflections of a behavioural scientist
Clinical trio genome sequencing facilitates the interpretation of variants in cancer predisposition genes in paediatric tumour patients
Heterozygous rare variants in NR2F2 cause a recognizable multiple congenital anomaly syndrome with developmental delays
Nemaline myopathy: reclassification of previously reported variants according to ACMG guidelines, and report of novel genetic variants
Re-evaluation and re-analysis of 152 research exomes five years after the initial report reveals clinically relevant changes in 18%
Germline pathogenic variants in metaplastic breast cancer patients and the emerging role of the BRCA1 gene
The contribution of morbidity and unemployment for the reduced labor market participation of individuals with neurofibromatosis 1 in Finland Finnish
A biallelic variant in COX18 cause isolated Complex IV deficiency associated with neonatal encephalo-cardio-myopathy and axonal sensory neuropathy
No need for options for choice for unsolicited findings in informed consent for clinical genetic testing
EMQN: Recommendations for genetic testing in inherited cardiomyopathies and arrhythmias
At a glance: the largest Niemann-Pick type C1 cohort with 602 patients diagnosed over 15 years
Public attitudes challenge clinical practice on genetic risk disclosure in favour of healthcare-provided direct dissemination to relatives
Persistent thinness and anorexia nervosa differ on a genomic level
Unveiling the hidden: revisiting the potential of old genetic data for rare disease research
From Mendel to multi-omics: shifting paradigms
Can ChatGPT understand genetics?
Germline pathogenic variants in HNRNPU are associated with alterations in blood methylome
The Korean Genetic Diagnosis Program for Rare Disease Phase II: outcomes of a 6-year national project
Unusual genomic variants require unusual analyses
MBD4-associated neoplasia syndrome: screening of cases with suggestive phenotypes
Comment on Australian public perspectives on genomic data governance by Lynch et al. in the EJHG
Uptake of funded genomic testing for syndromic and non-syndromic intellectual disability in Australia
Hypomorphic pathogenic variant in SFTPB leads to adult pulmonary fibrosis
Episignature analysis of moderate effects and mosaics
Epidemiology of the disorders of the Pik3ca-related overgrowth spectrum (Pros)
Interventions to support patients with sharing genetic test results with at-risk relatives: a synthesis without meta-analysis (SWiM)
A survey of genetic and palliative care health professionals’ views of integrating genetics into palliative care
BRAT1–related disorders: phenotypic spectrum and phenotype-genotype correlations from 97 patients
What is health and what do we mean when we say an intervention improves health?
Preimplantation genetic testing for Neurofibromatosis type 1: more than 20 years of clinical experience
Genetic characterization of non-5q proximal spinal muscular atrophy in a French cohort: the place of whole exome sequencing
Correction: The future is mainstream: genetic counselling should be embedded in mainstream medicine
Assessing the general public’s view of direct-to-consumer (DTC) genetic testing and their interpretation of DTC website disclaimer messages
Reconciling the biomedical data commons and the GDPR: three lessons from the EUCAN ELSI collaboratory
The human genome harbours widespread exclusive yin yang haplotypes
Defining critical educational components of informed consent for genetic testing: views of US-based genetic counselors and medical geneticists
What matters to parents? A scoping review of parents’ service experiences and needs regarding genetic testing for rare diseases
The complex genomics of single gene disorders
The next step toward personalized recommendations for genetic cardiomyopathies
Direct notification by health professionals of relatives at-risk of genetic conditions (with patient consent): views of the Australian public
Three generation families: Analysis of de novo variants in autism
Consanguineous couples’ experiences and views regarding expanded carrier screening: Barriers and facilitators in the decision-making process
Gustavson syndrome is caused by an in-frame deletion in RBMX associated with potentially disturbed SH3 domain interactions Swedish
Comment on: “The expanding genetic and clinical landscape associated with Meier-Gorlin syndrome” by Nielsen-Dandoroff et al.
The future is mainstream: genetic counselling should be embedded in mainstream medicine
Exploring genotype–phenotype correlations in CREBBP: comment on the literature and description of an additional patient with an atypical outcome
Analysis of large-language model versus human performance for genetics questions
An adaptive and robust method for multi-trait analysis of genome-wide association studies using summary statistics
Genetic testing in monogenic early-onset atrial fibrillation
Correction: Community concerns about genetic discrimination in life insurance persist in Australia: A survey of consumers offered genetic testing
New insights into the molecular basis of spinal neurofibromatosis type 1
A novel genomic mutation in ADNP leading to intellectual disability
Diagnostic and prognostic relevance of using large gene panels in the genetic testing of patients with dilated cardiomyopathy
Diagnostic yield and clinical relevance of expanded germline genetic testing for nearly 7000 suspected HBOC patients
Investigation and management of pediatric moyamoya arteriopathy in the era of genotype-phenotype correlation studies
2p25.3 microduplications involving MYT1L: further phenotypic characterization through an assessment of 16 new cases and a literature review
Broadening the phenotypic and molecular spectrum of FINCA syndrome: Biallelic NHLRC2 variants in 15 novel individuals
Searching for a sense of closure: parental experiences of recontacting after a terminated pregnancy for congenital malformations
Identification and in vivo functional investigation of a HOMER2 nonstop variant causing hearing loss
Molecular explanations for variability of clinical phenotypes
Dopa-responsive dystonia in Bulgarian patients: report of three cases
Abstracts from the 55th European Society of Human Genetics (ESHG) Conference
Abstracts from the 55th European Society of Human Genetics (ESHG) Conference: Oral Presentations
Abstracts from the 55th European Society of Human Genetics (ESHG) Conference: e-Posters
Abdominal obesity is a more important causal risk factor for pancreatic cancer than overall obesity
Abstracts from the 55th European Society of Human Genetics (ESHG) Conference: Hybrid Posters
Australian public perspectives on genomic data governance: responsibility, regulation, and logistical considerations
Contributing to the on-going debate around the clinical application of preimplantation embryo selection based on polygenic risk scores (PGT-P)
Community concerns about genetic discrimination in life insurance persist in Australia: A survey of consumers offered genetic testing
Global trends and themes in genetic counseling research
Expanding the phenotypic spectrum of NAA10-related neurodevelopmental syndrome and NAA15-related neurodevelopmental syndrome
Underrepresentation of racial and ethnic minorities in cascade testing for hereditary cancer syndromes
Correction: Clinical and genetic analysis further delineates the phenotypic spectrum of ALDH1A3-related anophthalmia and microphthalmia
April, again
Lynch syndrome: influence of additional susceptibility variants on cancer risk
Highly variable hearing loss due to POU4F3 (c.37del) is revealed by longitudinal, frequency specific analyses Israeli Jewish; Dutch; North American
Human molecular genetics sheds light on the physiological significance of ribonuclease inhibitor (RNH1)
Correction: Genome-wide meta-analysis identifies novel loci conferring risk of acne vulgaris
ADNP in reverse gear
Clinical profiling of MRD48 and functional characterization of two novel pathogenic RAC1 variants
The expanding genetic and clinical landscape associated with Meier-Gorlin syndrome
STRavinsky STR database and PGTailor PGT tool demonstrate superiority of CHM13-T2T over hg38 and hg19 for STR-based applications
The absence of CFHR3 and CFHR1 genes from the T2T-CHM13 assembly can limit the molecular diagnosis of complement-related diseases
Tools to differentiate between Filamin C and Titin truncating variant carriers: value of MRI
Biological data studies, scale-up the potential with machine learning
Population screening for 15q11-q13 duplications: corroboration of the difference in impact between maternally and paternally inherited alleles
Studying ultra-rare variants in STX1A uncovers a novel neurodevelopmental disorder
The genetic landscape and clinical implication of pediatric Moyamoya angiopathy in an international cohort
Old and new challenges regarding comparable and viable data sharing in population-scale genomic research
Deciphering the genetic structure of the Quebec founder population using genealogies European colonization; ethnocultural groups; Acadians; Sagueneans; Gaspé and North Shore groups; Quebec founder population
Clinical and genetic analysis further delineates the phenotypic spectrum of ALDH1A3-related anophthalmia and microphthalmia
Uncovering the genetic architecture of paediatric moyamoya angiopathy: implications for disease pathogenesis
Clinical phenotyping and genetic diagnosis of a large cohort of Sudanese families with hereditary spinocerebellar degenerations
Should testing for mosaic genome-wide paternal uniparental disomy in Beckwith-Wiedemann spectrum (BWSp) be implemented in diagnostic testing?
Diagnosis and management of vascular Ehlers-Danlos syndrome: Experience of the UK national diagnostic service, Sheffield
A fresh cup of DCAF: DCAF13 implicated in a neuromuscular disorder
Immortal data: a qualitative exploration of patients’ understandings of genomic data
Dutch Pharmacogenetics Working Group (DPWG) guideline for the gene-drug interaction between CYP2D6, CYP3A4 and CYP1A2 and antipsychotics
A practical checklist for return of results from genomic research in the European context
Genes=disease (?)
Two sporadic cases of childhood-onset Hailey-Hailey disease with superimposed mosaicism
Down syndrome screening and diagnosis practices in Europe, United States, Australia, and New Zealand from 1990–2021
Exome sequencing in individuals with congenital anomalies of the kidney and urinary tract (CAKUT): a single-center experience
Expanding the phenotype associated with biallelic SLC20A2 variants
Molecular diagnosis of 405 individuals with autism spectrum disorder
Predicting personal cardiovascular disease risk based on family health history: Development of expert-based family criteria for the general population
Uncertainties experienced by parents of children diagnosed with severe combined immunodeficiency through newborn screening
Equity implications of patient-initiated recontact and follow-up in clinical genetics
A genome-wide association analysis of loss of ambulation in dystrophinopathy patients suggests multiple candidate modifiers of disease severity
Limitations, concerns and potential: attitudes of healthcare professionals toward preimplantation genetic testing using polygenic risk scores
Expanding the Australian Newborn Blood Spot Screening Program using genomic sequencing: do we want it and are we ready?
Ribonuclease inhibitor 1 (RNH1) deficiency cause congenital cataracts and global developmental delay with infection-induced psychomotor regression and anemia
Genome-wide meta-analysis identifies novel loci conferring risk of acne vulgaris European-ancestry cohorts
Clinical case study meets population cohort: identification of a BRCA1 pathogenic founder variant in Orcadians Orcadian ancestry; Orcadians; Orcadian grandparents; Westray ancestry; Isle of Westray
Skewed X-chromosome inactivation in unsolved neurodevelopmental disease cases can guide re-evaluation For X-linked genes
Evaluation and pilot testing of a multidisciplinary model of care to mainstream genomic testing for paediatric inborn errors of immunity
Genome sequencing-based discovery of a novel deep intronic APC pathogenic variant causing exonization
Loss of function of ADNP by an intragenic inversion
Experiences of nonpregnant couples after receiving reproductive genetic carrier screening results in Belgium
Rare-variant association analysis reveals known and new age-related hearing loss genes
KidneyNetwork: using kidney-derived gene expression data to predict and prioritize novel genes involved in kidney disease
Perceived benefits and barriers to implementing precision preventive care: Results of a national physician survey
CHAMP1-related disorders: pathomechanisms triggered by different genomic alterations define distinct nosological categories
Identification of a DNA methylation signature for Renpenning syndrome (RENS1), a spliceopathy
Defining the heterogeneity of unbalanced structural variation underlying breast cancer susceptibility by nanopore genome sequencing
Correction: Dutch Pharmacogenetics Working Group (DPWG) guideline for the gene–drug interaction between UGT1A1 and irinotecan
SMAD6 variants in nonsyndromic craniosynostosis
A biallelic variant of DCAF13 implicated in a neuromuscular disorder in humans
Copy number variants from 4800 exomes contribute to ~7% of genetic diagnoses in movement disorders, muscle disorders and neuropathies
Views of healthcare professionals on the inclusion of genes associated with non-syndromic hearing loss in reproductive genetic carrier screening
Johann Gregor Mendel: the victory of statistics over human imagination
Correction: The role of the genetic counsellor in the multidisciplinary team: the perception of geneticists in Europe
The European Society of Human Genetics—Young committee- activities and achievements between 2019–2022
The value of exomes across the ages
Towards UGT1A1 guided irinotecan dosing
2022: the year that was in the European Journal of Human Genetics
Comparing 2D and 3D representations for face-based genetic syndrome diagnosis
ENROLL-HD for MND?
The neurodevelopmental and facial phenotype in individuals with a TRIP12 variant
Elusive variants in autosomal recessive disease: how can we improve timely diagnosis?
KiT-GENIE, the French genetic biobank of kidney transplantation
GBA/GBN-position on the feedback of incidental findings in biobank-based research: consensus-based workflow for hospital-based biobanks
Clinical interest of molecular study in cases of isolated midline craniosynostosis
Correction to: High rate of HDR in gene editing of p.(Thr158Met) MECP2 mutational hotspot
A family with ulcerative colitis maps to 7p21.1 and comprises a region with regulatory activity for the aryl hydrocarbon receptor gene
Comment on Intragenic inversions in NF1 gene as pathogenic mechanism in neurofibromatosis type 1
Correction: Consensus statement on standards and guidelines for the molecular diagnostics of Alport syndrome: refining the ACMG criteria
The cause of Jones syndrome put to REST: a mutation in the REST gene causes gingival fibromatosis and hearing loss
FANCM missense variants and breast cancer risk: a case-control association study of 75,156 European women controls of European ancestry
An encounter with the mild side of LARS2-associated Perrault syndrome and its implications on the diagnostic odyssey
Correction: The 2019 and 2021 International workshops on Alport syndrome
Reanalysis of clinical exome identifies the second variant in two individuals with recessive disorders
Correction: New frontiers to cure Alport syndrome: COL4A3 and COL4A5 gene editing in podocyte-lineage cells
“I am not a number!” Opinions and preferences of people with intellectual disability about genetic healthcare
Attitudes of professional stakeholders towards implementation of reproductive genetic carrier screening: a systematic review
Balancing the safeguarding of privacy and data sharing: perceptions of genomic professionals on patient genomic data ownership in Australia
Community Genetics screening in a pandemic: solutions for pre-test education, informed consent, and specimen collection Jewish community
ParseCNV2: a versatile and integrated tool for copy number variation association studies
New year, new genes
Improving diagnosis of mitochondrial fatty-acid oxidation disorders
Clinical variability in DYNC2H1-related skeletal ciliopathies includes Ellis-van Creveld syndrome
Participant perspective on the recall-by-genotype research approach: a mixed-method embedded study with participants of the CHRIS study
Truncating variants in the penultimate exon of TGFBR1 escaping nonsense-mediated mRNA decay cause Loeys-Dietz syndrome
X-linked C1GALT1C1 mutation causes atypical hemolytic uremic syndrome
CDK13-related disorder: a deep characterization of speech and language abilities and addition of 33 novel cases
High molecular diagnostic yields and novel phenotypic expansions involving syndromic anorectal malformations