European Journal of Human Genetics - 2019

249 articles | Last updated: 2025-12-03 14:12:56
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Identification of Lynch syndrome by microsatellite instability and mismatch repair deficiency testing on colorectal adenomas
SweHLA: the high confidence HLA typing bio-resource drawn from 1000 Swedish genomes European populations
Response to multiple glucose-lowering agents in a sib-pair with a novel HNF1α (MODY3) variant
Dwarna: a blockchain solution for dynamic consent in biobanking
Mixed-model admixture mapping identifies smoking-dependent loci of lung function in African Americans African Americans
Diagnostic yield of panel-based genetic testing in syndromic inherited retinal disease
The paternal and maternal genetic history of Vietnamese populations
Exome sequencing in infants with congenital hearing impairment: a population-based cohort study
Targeted deep-intronic sequencing in a cohort of unexplained cases of suspected Lynch syndrome
Novel clinical and genetic insight into CXorf56-associated intellectual disability
“Patient Journeys”: improving care by patient involvement
A de novo variant in the human HIST1H4J gene causes a syndrome analogous to the HIST1H4C-associated neurodevelopmental disorder
Methylated premutation of the FMR1 gene in three sisters: correlating CGG expansion and epigenetic inactivation
Primary care provider perspectives on using genomic sequencing in the care of healthy children
Members of the public in the USA, UK, Canada and Australia expressing genetic exceptionalism say they are more willing to donate genomic data
A bird’s-eye view of Italian genomic variation through whole-genome sequencing Italian; Tuscans; Italian peninsula; Italian population; cohorts
The functional variant of NTN1 contributes to the risk of nonsyndromic cleft lip with or without cleft palate
Biallelic variants in EFEMP1 in a man with a pronounced connective tissue phenotype
Update of a classic
New frontiers to cure Alport syndrome: COL4A3 and COL4A5 gene editing in podocyte-lineage cells
Lessons from Ciência Viva: how teaching human genetics to XXIst century students must go beyond the classroom
Dutch Pharmacogenetics Working Group (DPWG) guideline for the gene–drug interaction of DPYD and fluoropyrimidines
Ultralow amounts of DNA from long-term archived serum samples produce quality genotypes
A multimodal attempt to follow-up linkage regions using RNA expression, SNPs and CpG methylation in schizophrenia and bipolar disorder kindreds
A variant of neonatal progeroid syndrome, or Wiedemann–Rautenstrauch syndrome, is associated with a nonsense variant in POLR3GL
Microsatellite instability screening in colorectal adenomas to detect Lynch syndrome patients? A systematic review and meta-analysis
Further delineation of neuropsychiatric findings in Tatton-Brown-Rahman syndrome due to disease-causing variants in DNMT3A: seven new patients
Genetic correlations between pain phenotypes and depression and neuroticism
Including hope in the treatment scheme—paradigms in the management of patients with cerebral palsy
Overgrowth—a clinical review not only for geneticists
Knowledge and views about genetics: a public-based cross-sectional study
PRPF31 reduction causes mis-splicing of the phototransduction genes in human organotypic retinal culture
A characterization of cis- and trans-heritability of RNA-Seq-based gene expression
Longitudinal changes in the frequency of mosaic chromosome Y loss in peripheral blood cells of aging men varies profoundly between individuals
Public reactions to direct-to-consumer genetic health tests: A comparison across the US, UK, Japan and Australia
Correction to: The Dutch Y-chromosomal landscape
The complexity of screening PMS2 in DNA isolated from formalin-fixed paraffin-embedded material
Abstracts from the 52nd European Society of Human Genetics (ESHG) Conference: Posters
Abstracts from the 52nd European Society of Human Genetics (ESHG) Conference: Posters
Abstracts from the 52nd European Society of Human Genetics (ESHG) Conference: Oral Presentations
ESHG PPPC Comments on postmortem use of genetic data for research purposes
Immunochip meta-analysis in European and Argentinian populations identifies two novel genetic loci associated with celiac disease European populations
Fryns type mesomelic dysplasia of the upper limbs caused by inverted duplications of the HOXD gene cluster
Effect of non-normality and low count variants on cross-phenotype association tests in GWAS
Reassessing the pathogenicity of c.2858G>T(p.(G953V)) in COL4A5 Gene: report of 19 Chinese families
GP-provided couple-based expanded preconception carrier screening in the Dutch general population: who accepts the test-offer and why?
A de novo splicing variant supports the candidacy of TLL1 in ASD pathogenesis
Next generation sequencing as second-tier test in high-throughput newborn screening for nephropathic cystinosis
One byte at a time: evidencing the quality of clinical service next-generation sequencing for germline and somatic variants
Functional variants in ADH1B and ALDH2 are non-additively associated with all-cause mortality in Japanese population East Asian population; Japanese population; Japanese cohort
Severe neurodevelopmental disease caused by a homozygous TLK2 variant
Robin D Clark & Cynthia J Curry: Genetic Consultations in the Newborn
Comprehensive genetic testing of Chinese SNHL patients and variants interpretation using ACMG guidelines and ethnically matched normal controls ethnically matched normal hearing adult controls; ethnically matched MAF filtering
Ethical, legal, and social issues (ELSI) in rare diseases: a landscape analysis from funders
The yield of postmortem genetic testing in sudden death cases with structural findings at autopsy
Rethinking the ethical principles of genomic medicine services
MNS1 variant associated with situs inversus and male infertility
What do cancer patients’ relatives think about addressing cancer family history and performing genetic testing in palliative care?
Sequencing results from multiple individuals of different ethnicities strongly question the existence of the KCNE1B pseudogene different ethnicities
Congenital myasthenic syndrome with mild intellectual disability caused by a recurrent SLC25A1 variant
Distributed Ledger Technology in genomics: a call for Europe
Genome sequencing in healthcare: understanding the UK general public’s views and implications for clinical practice
Estimating cumulative point prevalence of rare diseases: analysis of the Orphanet database
Declining detection rates for APC and biallelic MUTYH variants in polyposis patients, implications for DNA testing policy
Stakeholders’ perspectives on the post-mortem use of genetic and health-related data for research: a systematic review
Correction: Phenotypic spectrum associated with a CRADD founder variant underlying frontotemporal predominant pachygyria in the Finnish population
KIF1A variants are a frequent cause of autosomal dominant hereditary spastic paraplegia
The Dutch Y-chromosomal landscape Dutch population; Netherlands; northern Belgium
CUGC for Stromme syndrome and CENPF-related disorders
GWAS of five gynecologic diseases and cross-trait analysis in Japanese
Sketching the prevalence of pharmacogenomic biomarkers among populations for clinical pharmacogenomics
Copy number variants in lipid metabolism genes are associated with gallstones disease in men
Correction: DPH1 syndrome: two novel variants and structural and functional analyses of seven missense variants identified in syndromic patients
School level of children carrying a HNF1B variant or a deletion
Phenotype and mutation expansion of the PTPN23 associated disorder characterized by neurodevelopmental delay and structural brain abnormalities
APC transcription studies and molecular diagnosis of familial adenomatous polyposis
Meeting report: The Human Genome Meeting (HGM) 2019 in Seoul, Korea
Current management of transition of young people affected by rare renal conditions in the ERKNet
Heritability of human visual contour integration—an integrated genomic study
Genetics-related service and information needs of childhood cancer survivors and parents: a mixed-methods study
High-throughput genetic newborn screening for spinal muscular atrophy by rapid nucleic acid extraction from dried blood spots and 384-well qPCR
Ethnic differences in prevalence of Dupuytren disease can partly be explained by known genetic risk variants
Early diagnosis of Pearson syndrome in neonatal intensive care following rapid mitochondrial genome sequencing in tandem with exome sequencing
Frequencies of clinically important CYP2C19 and CYP2D6 alleles are graded across Europe
De novo variants in an extracellular matrix protein coding gene, fibulin-5 (FBLN5) are associated with pseudoexfoliation
Correction to: Genetic discrimination by Australian insurance companies: a survey of consumer experiences
Anophthalmia including next-generation sequencing-based approaches
Assessment of genetic variant burden in epilepsy-associated brain lesions
Germline genome editing: public dialogue is urgent but not self-evident
Comprehensive clinical and molecular studies in split-hand/foot malformation: identification of two plausible candidate genes (LRP6 and UBA2)
Full-length transcript amplification and sequencing as universal method to test mRNA integrity and biallelic expression in mismatch repair genes
Mendelian disease caused by variants affecting recognition of Z-DNA and Z-RNA by the Zα domain of the double-stranded RNA editing enzyme ADAR
Differentiation of MISSLA and Fanconi anaemia by computer-aided image analysis and presentation of two novel MISSLA siblings
A head-to-head evaluation of the diagnostic efficacy and costs of trio versus singleton exome sequencing analysis
Biological characterization of expression quantitative trait loci (eQTLs) showing tissue-specific opposite directional effects
Exploring the effect of ascertainment bias on genetic studies that use clinical pedigrees
Regarding the rights and duties of Clinical Laboratory Geneticists in genetic healthcare systems: results of a survey in over 50 countries
Genetic discrimination by Australian insurance companies: a survey of consumer experiences
Clinical utility gene card: for incontinentia pigmenti
A functional assay to study the pathogenicity of CHD7 protein variants encountered in CHARGE syndrome patients
Expansion of B4GALT7 linkeropathy phenotype to include perinatal lethal skeletal dysplasia
De novo substitutions of TRPM3 cause intellectual disability and epilepsy
Genetic relationships of European, Mediterranean, and SW Asian populations using a panel of 55 AISNPs European populations; Mediterranean, SW Asian populations
Report on three additional patients and genotype–phenotype correlation in SLC25A22-related disorders group
Abstracts from the 2018 European Meeting on Psychosocial Aspects of Genetics
Abstracts from the 51st European Society of Human Genetics Conference: Electronic Posters
Abstracts from the 51st European Society of Human Genetics Conference: Posters
Abstracts from the 51st European Society of Human Genetics Conference: Oral Presentations
Abstracts from the 51st European Society of Human Genetics (ESHG) Conference, in conjunction with the European Meeting on Psychosocial Aspects of Genetics (EMPAG)
Reply to DM Hougaard et al.
Response to “Newborn dried blood spot samples in Denmark: the hidden figures of secondary use and research participation”
Increased diagnostic and new genes identification outcome using research reanalysis of singleton exome sequencing
The Incontinentia Pigmenti Genetic Biobank: study design and cohort profile to facilitate research into a rare disease worldwide
Clinical Utility Gene Card for: PGM3 defective congenital disorder of glycosylation
Sex specific associations in genome wide association analysis of renal cell carcinoma
Prospective head-to-head comparison of accuracy of two sequencing platforms for screening for fetal aneuploidy by cell-free DNA: the PEGASUS study
European recommendations integrating genetic testing into multidisciplinary management of sudden cardiac death
After the fact—the case of CRISPR babies
Update of the EMQN/ACGS best practice guidelines for molecular analysis of Prader-Willi and Angelman syndromes
Small posterior fossa in Chiari I malformation affected families is significantly linked to 1q43-44 and 12q23-24.11 using whole exome sequencing
Variability in gene-based knowledge impacts variant classification: an analysis of FBN1 missense variants in ClinVar
Global, pathway and gene coverage of three Illumina arrays with respect to inflammatory and immune-related pathways
A pilot study of the implementation of pharmacogenomic pharmacist initiated pre-emptive testing in primary care
The nurturing of nature
De novo variants in CNOT3 cause a variable neurodevelopmental disorder
CUGC for posterior polymorphous corneal dystrophy (PPCD)
Yemenite-Jewish families with Machado–Joseph disease (MJD/SCA3) share a recent common ancestor
Challenges and strategies proposed by genetic health professionals to assist with family communication
Searching for secondary findings: considering actionability and preserving the right not to know
A retrospective analysis of the prevalence of imprinting disorders in Estonia from 1998 to 2016
APOB gene polymorphisms may affect the risk of minor or minimal bleeding complications in patients on warfarin maintaining therapeutic INR
Current practices for the genetic diagnosis of autoinflammatory diseases: results of a European Molecular Genetics Quality Network Survey
DEGS1 variant causes neurological disorder
Do health professionals value genomic testing? A discrete choice experiment in inherited cardiovascular disease
A feline orthologue of the human MYH7 c.5647G>A (p.(Glu1883Lys)) variant causes hypertrophic cardiomyopathy in a Domestic Shorthair cat
Truncating SLC12A6 variants cause different clinical phenotypes in humans and dogs
Microdeletion of pseudogene chr14.232.a affects LRFN5 expression in cells of a patient with autism spectrum disorder
Attitudes of Australian health professionals towards rapid genomic testing in neonatal and paediatric intensive care
Attitudes of blood donors to their sample and data donation for biobanking
The destructive role of Trofim Lysenko in Russian Science
Implementation of public health genomics in Pakistan
Biallelic variants in POLR3GL cause endosteal hyperostosis and oligodontia
Bone Dysplasia: a must-have Atlas for the clinician
Population genetic screening: current issues in a European country
Informing relatives at risk of inherited cardiac conditions: experiences and attitudes of healthcare professionals and counselees
Truncation of TGF-β docking receptor GARP is linked to human disease
Specific phenotype semantics facilitate gene prioritization in clinical exome sequencing
Disparities in discovery of pathogenic variants for autosomal recessive non-syndromic hearing impairment by ancestry by ancestry
A novel de novo MTOR gain-of-function variant in a patient with Smith-Kingsmore syndrome and Antiphospholipid syndrome
gr/gr deletion predisposes to testicular germ cell tumour independently from altered spermatogenesis: results from the largest European study
Splice-site variant in ACSL5: a marker promoting opposing effect on cell viability and protein expression
Heterogeneity in the extent of linkage disequilibrium among exonic, intronic, non-coding RNA and intergenic chromosome regions
Perspectives in genetic counseling for spinal muscular atrophy in the new therapeutic era: early pre-symptomatic intervention and test in minors
Rare missense variants in the ALPK1 gene may predispose to periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome
Variable expressivity of syndromic BMP4-related eye, brain, and digital anomalies: A review of the literature and description of three new cases
Trends in BRCA testing and socioeconomic deprivation
Reappraisal of variants previously linked with sudden infant death syndrome: results from three population-based cohorts
Recessive DES cardio/myopathy without myofibrillar aggregates: intronic splice variant silences one allele leaving only missense L190P-desmin
Abstracts from the 50th European Society of Human Genetics Conference: 7th International Workshop on the History of Human Genetics
Secondary actionable findings identified by exome sequencing: expected impact on the organisation of care from the study of 700 consecutive tests
The GDPR and the research exemption: considerations on the necessary safeguards for research biobanks
LRSAM1 variants and founder effect in French families with ataxic form of Charcot-Marie-Tooth type 2
Variants in DOCK3 cause developmental delay and hypotonia
A genome-wide analysis in consanguineous families reveals new chromosomal loci in specific language impairment (SLI)
Biallelic variants in AGTPBP1, involved in tubulin deglutamylation, are associated with cerebellar degeneration and motor neuropathy
Immunity and mental illness: findings from a Danish population-based immunogenetic study of seven psychiatric and neurodevelopmental disorders Danish population-based
Homozygous stop-gain variant in LRRC32, encoding a TGFβ receptor, associated with cleft palate, proliferative retinopathy, and developmental delay
Y-chromosomal analysis of clan structure of Kalmyks, the only European Mongol people, and their relationship to Oirat-Mongols of Inner Asia "the only European Mongol people" (title); "the only Mongolic-speaking population in Europe"; "in th; Uses ethnic/population terms such as "Mongolic-speaking", "Oirat-Mongols", "Kalmyks", "Oirats", "Bur
Unique bioinformatic approach and comprehensive reanalysis improve diagnostic yield of clinical exomes
Managing uncertainty in inherited cardiac pathologies—an international multidisciplinary survey
Evaluation of telephone genetic counselling to facilitate germline BRCA1/2 testing in women with high-grade serous ovarian cancer
Linkage analysis revealed risk loci on 6p21 and 18p11.2-q11.2 in familial colon and rectal cancer, respectively
Schilbach–Rott syndrome associated with 9q22.32q22.33 duplication, involving the PTCH1 gene
The potential presence of the highly similar paralogue gene KCNE1B blurs the genetic basis of KCNE1-LQTS patients
Three de novo DDX3X variants associated with distinctive brain developmental abnormalities and brain tumor in intellectually disabled females
Stakeholder views and attitudes towards prenatal and postnatal transplantation of fetal mesenchymal stem cells to treat Osteogenesis Imperfecta
Regarding the rights and duties of Clinical Laboratory Geneticists in genetic healthcare systems; results of a survey in over 50 countries
Correction: Educational delay and attainment in persons with neurofibromatosis 1 in Denmark
Phenotypic spectrum associated with a CRADD founder variant underlying frontotemporal predominant pachygyria in the Finnish population Finnish population
Mitochondrial DNA variability of the Polish population
Perceptions of genetic variant reclassification in patients with inherited cardiac disease
Haploinsufficiency of ARHGAP42 is associated with hypertension
Genotype–phenotype correlation study in 364 osteogenesis imperfecta Italian patients
DPH1 syndrome: two novel variants and structural and functional analyses of seven missense variants identified in syndromic patients
Precision medicine for a man presented with diabetes at 2-month old
A case-note review of continued pregnancies found to be at a high risk of Huntington’s disease: considerations for clinical practice
De novo single-nucleotide and copy number variation in discordant monozygotic twins reveals disease-related genes
Reviewer recognition
Hearing impairment locus heterogeneity and identification of PLS1 as a new autosomal dominant gene in Hungarian Roma Hungarian Roma
Deletions and loss-of-function variants in TP63 associated with orofacial clefting
Continental drift? Do European clinical genetic testing laboratories have a patent problem?
Development of patient “profiles” to tailor counseling for incidental genomic sequencing results
A pathway-driven predictive model of tramadol pharmacogenetics
Educational delay and attainment in persons with neurofibromatosis 1 in Denmark
Copy number variation analysis in bicuspid aortic valve-related aortopathy identifies TBX20 as a contributing gene
Evolutionary conserved networks of human height identify multiple Mendelian causes of short stature
Characteristics and quality of genetics and genomics mobile apps: a systematic review
SMAD4 rare variants in individuals and families with thoracic aortic aneurysms and dissections
Confirmation of the role of pathogenic SMAD6 variants in bicuspid aortic valve-related aortopathy
A novel dominant-negative FGFR1 variant causes Hartsfield syndrome by deregulating RAS/ERK1/2 pathway
Disruption of KCNQ1 prevents methylation of the ICR2 and supports the hypothesis that its transcription is necessary for imprint establishment
A pilot eConsultation service in Eastern Ontario: bridging clinical genetics and primary care
De novo variants in HK1 associated with neurodevelopmental abnormalities and visual impairment
People from Ibiza: an unexpected isolate in the Western Mediterranean
Using dried blood spot samples from a trio for linked-read whole-exome sequencing
Biological insights into multiple birth: genetic findings from UK Biobank
Expanded reproductive carrier screening—how can we do the most good and cause the least harm?
A novel variant of the human mitochondrial DnaJ protein, Tid1, associates with a human disease exhibiting developmental delay and polyneuropathy
Feasibility of couple-based expanded carrier screening offered by general practitioners
Homogentisate 1,2-dioxygenase (HGD) gene variants, their analysis and genotype–phenotype correlations in the largest cohort of patients with AKU
Segmental and total uniparental isodisomy (UPiD) as a disease mechanism in autosomal recessive lysosomal disorders: evidence from SNP arrays
Imputation of behavioral candidate gene repeat variants in 486,551 publicly-available UK Biobank individuals
Homozygous frame shift variant in ATP7B exon 1 leads to bypass of nonsense-mediated mRNA decay and to a protein capable of copper export
SCAPER localizes to primary cilia and its mutation affects cilia length, causing Bardet-Biedl syndrome
Case–control analysis identifies shared properties of rare germline variation in cancer predisposing genes
Autonomous decision-making for antenatal screening in Pakistan: views held by women, men and health professionals in a low–middle income country
Breakpoint mapping at nucleotide resolution in X-autosome balanced translocations associated with clinical phenotypes
Motivations for data sharing—views of research participants from four European countries: A DIRECT study
Analysis of cilia dysfunction phenotypes in zebrafish embryos depleted of Origin recognition complex factors
Exome sequencing in clinical settings: preferences and experiences of parents of children with rare diseases (SEQUAPRE study)
Comparison of methods for multivariate gene-based association tests for complex diseases using common variants
Delivering effective genetic services for patients and families affected by cleft lip and/or palate
Estimating the prevalence of allelic variants in the transthyretin gene by analysing large-scale sequencing data
CUGC for Simpson-Golabi-Behmel syndrome (SGBS)
Patient views on research use of clinical data without consent: Legal, but also acceptable?
ART-DeCo: easy tool for detection and characterization of cross-contamination of DNA samples in diagnostic next-generation sequencing analysis
A validated PROM in genetic counselling: the psychometric properties of the Dutch version of the Genetic Counselling Outcome Scale
The Marquesans at the fringes of the Austronesian expansion
Multiple genomic copy number variants associated with periventricular nodular heterotopia indicate extreme genetic heterogeneity
De novo variants in FBXO11 cause a syndromic form of intellectual disability with behavioral problems and dysmorphisms
Genome-wide association meta-analysis of 30,000 samples identifies seven novel loci for quantitative ECG traits
Exome sequencing of fetal anomaly syndromes: novel phenotype–genotype discoveries
Genetics of the patella
Australians’ views and experience of personal genomic testing: survey findings from the Genioz study
Spatially clustering de novo variants in CYFIP2, encoding the cytoplasmic FMRP interacting protein 2, cause intellectual disability and seizures
Genetic basis for plasma amino acid concentrations based on absolute quantification: a genome-wide association study in the Japanese population
Enriched power of disease-concordant twin-case-only design in detecting interactions in genome-wide association studies
Reply to Bombard and Mighton
Biallelic intragenic duplication in ADGRB3 (BAI3) gene associated with intellectual disability, cerebellar atrophy, and behavioral disorder
A genetic perspective on Longobard-Era migrations
Rapid and accurate interpretation of clinical exomes using Phenoxome: a computational phenotype-driven approach
Inverse PCR to perform long-distance haplotyping: main applications to improve preimplantation genetic diagnosis in hemophilia
Value of genetic analysis for confirming inborn errors of metabolism detected through the Spanish neonatal screening program
Towards establishing consistency in triage in a tertiary specialty
Duplication of 10q24 locus: broadening the clinical and radiological spectrum
Biallelic sequence variants in INTS1 in patients with developmental delays, cataracts, and craniofacial anomalies
Return of individual genomic research results: are laws and policies keeping step?
Genetic architecture of laterality defects revealed by whole exome sequencing
An Amish founder variant consolidates disruption of CEP55 as a cause of hydranencephaly and renal dysplasia Amish