European Journal of Human Genetics - 2021

230 articles | Last updated: 2025-12-03 14:12:56
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T A T A T A T A
Genetic analysis of ALS cases in the isolated island population of Malta south of Europe
Parental mosaicism in Marfan and Ehlers–Danlos syndromes and related disorders
‘CTRL’: an online, Dynamic Consent and participant engagement platform working towards solving the complexities of consent in genomic research
A synonymous variant in MYO15A enriched in the Ashkenazi Jewish population causes autosomal recessive hearing loss due to abnormal splicing Ashkenazi Jewish population
The impact of needs-based education on the change of knowledge and attitudes towards medical genetics in medical students
Correction to: Origins, admixture and founder lineages in European Roma European Roma
A summary-statistics-based approach to examine the role of serotonin transporter promoter tandem repeat polymorphism in psychiatric phenotypes
The use of polygenic risk scores in pre-implantation genetic testing: an unproven, unethical practice
Correction to: Attitudes among parents of persons with autism spectrum disorder towards information about genetic risk and future health
Natural history of NF1 c.2970_2972del p.(Met992del): confirmation of a low risk of complications in a longitudinal study
30 year experience of index case identification and outcomes of cascade testing in high-risk breast and colorectal cancer predisposition genes
Reclassification of a TMC1 synonymous substitution as a variant disrupting splicing regulatory elements associated with recessive hearing loss
A polygenic risk score for multiple myeloma risk prediction
New year, new issue
Genomics elucidates both common and rare disease aetiology
Genetic testing decisions in non-western cultures: an opportunity for intergenerational decision making
Commentary on: Establishing the phenotypic spectrum of ZTTK syndrome by analysis of 52 individuals with variants in SON
Deafness—family matters
Genomic analysis of childhood hearing loss in the Yoruba population of Nigeria Yoruba ethno-lingual ancestry; Sub-Saharan regions of Africa; Yoruba samples; cohort; population
A tailored approach to informing relatives at risk of inherited cardiac conditions: results of a randomised controlled trial
Motives for withdrawal of participation in biobanking and participants’ willingness to allow linkages of their data
Invited Commentary on “My Research Results: a program to facilitate return of clinically actionable genomic research findings” by Willis et al.
Using deep-neural-network-driven facial recognition to identify distinct Kabuki syndrome 1 and 2 gestalt
Two distinct mechanisms underlie estrogen-receptor-negative breast cancer susceptibility at the 2p23.2 locus
Dutch Pharmacogenetics Working Group (DPWG) guideline for the gene-drug interaction between CYP2C19 and CYP2D6 and SSRIs
Accelerated genome sequencing with controlled costs for infants in intensive care units: a feasibility study in a French hospital network
Attitudes among parents of persons with autism spectrum disorder towards information about genetic risk and future health
DMPK hypermethylation in sperm cells of myotonic dystrophy type 1 patients
Taking seriousness seriously in genomic health
Hypoketotic hypoglycemia without neuromuscular complications in patients with SLC25A32 deficiency
Rapid genomic testing for critically ill children: time to become standard of care?
Biallelic in-frame deletion of SOX4 is associated with developmental delay, hypotonia and intellectual disability
Combining callers improves the detection of copy number variants from whole-genome sequencing
Identification of a novel variant of the ciliopathic gene FUZZY associated with craniosynostosis
Development and use of the Australian reproductive genetic carrier screening decision aid
An all-encompassing variant classification system proposed
Meiotic genes in premature ovarian insufficiency: variants in HROB and REC8 as likely genetic causes
ITSN1: a novel candidate gene involved in autosomal dominant neurodevelopmental disorder spectrum
Disorders of the eye, ear, skin, and nervous system in women with Turner syndrome –a nationwide cohort study
Lessons learned from unsolicited findings in clinical exome sequencing of 16,482 individuals
Exploiting family history in aggregation unit-based genetic association tests
Prenatal phenotype of PNKP-related primary microcephaly associated with variants affecting both the FHA and phosphatase domain
Exome first approach to reduce diagnostic costs and time – retrospective analysis of 111 individuals with rare neurodevelopmental disorders German cohort
Genomic health data generation in the UK: a 360 view
Further delineation of the clinical spectrum of White–Sutton syndrome: 12 new individuals and a review of the literature
Familial cleft tongue caused by a unique translation initiation codon variant in TP63
Genetics of diaphragmatic hernia
SOD1 D91A variant in the southernmost tip of Europe: a heterozygous ALS patient resident on the island of Gozo southern Europe; Europe; Mediterranean countries; Maltese population; North-South gradient
Genotyping arrays, population genetic studies and clinical implications
The ethical landscape(s) of non-invasive prenatal testing in England, France and Germany: findings from a comparative literature review
My Research Results: a program to facilitate return of clinically actionable genomic research findings
Cascade screening for beta-thalassaemia in Pakistan: relatives’ experiences of a decision support intervention in routine practice
Correction: Secondary research use of personal medical data: attitudes from patient and population surveys in The Netherlands and Germany
Correction: Dutch Pharmacogenetics Working Group (DPWG) guideline for the gene–drug interaction between CYP2D6 and opioids (codeine, tramadol and oxycodone)
What is a ‘serious’ genetic condition? The perceptions of people living with genetic conditions
Crosstalk between BH4, pain, and dystonia
Decision-making and experiences of preimplantation genetic diagnosis in inherited heart diseases: a qualitative study
A de novo mutation in mitochondrial ATPsynthase subunit α causes a life threatening disease in neonates which heals in infancy
The LOVD3 platform: efficient genome-wide sharing of genetic variants
Establishing the phenotypic spectrum of ZTTK syndrome by analysis of 52 individuals with variants in SON
Alkaptonuria in Russia
Fond farewell to clinical utility gene cards
Comment on: Bi-allelic variants in genes previously associated with dominant inheritance: CACNA1A, RET and SLC20A2
A recurrent de novo ATP5F1A substitution associated with neonatal complex V deficiency
TAB2 deletions and variants cause a highly recognisable syndrome with mitral valve disease, cardiomyopathy, short stature and hypermobility
Sequence variants in malignant hyperthermia genes in Iceland: classification and actionable findings in a population database Icelandic; Icelanders
Australian human research ethics committee members’ confidence in reviewing genomic research applications
Cascade health service use in family members following genetic testing in children: a scoping literature review
A new impact factor for European Journal of Human Genetics
Correction: Solving unsolved rare neurological diseases—a Solve-RD viewpoint
Pathogenic SLIRP variants as a novel cause of autosomal recessive mitochondrial encephalomyopathy with complex I and IV deficiency
Parents’ experiences of decision making for rapid genomic sequencing in intensive care
Speech, language and communication phenotyping in rare genetic syndromes: Commentary on Speech and language deficits are central to SETBP1 haploinsufficiency disorder
Biallelic loss-of-function variants in WDR11 are associated with microcephaly and intellectual disability
Pathogenic variations in Germ Cell Nuclear Acidic Peptidase (GCNA) are associated with human male infertility
Correction to: Towards a fine-scale picture of European genetic diversity
Non-invasive prenatal testing (NIPT) and pregnant women’s views on good motherhood: a qualitative study
Correction to: Solving patients with rare diseases through programmatic reanalysis of genome-phenome data
Physicians’ perceptions of the factors influencing disclosure of secondary findings in tumour genomic profiling in Japan: a qualitative study
Correction to: Solve-RD: systematic pan-European data sharing and collaborative analysis to solve rare diseases
Routinization of prenatal screening with the non-invasive prenatal test: pregnant women’s perspectives
The relative fitness of the de novo variants in general Lithuanian population vs. in individuals with intellectual disability
IMPDH2: a new gene associated with dominant juvenile-onset dystonia-tremor disorder Finnish family
Solving the enigma of POLD1 p.V295M as a potential cause of increased cancer risk
Biallelic P4HTM variants associated with HIDEA syndrome and mitochondrial respiratory chain complex I deficiency
Establishing a second-generation artificial intelligence-based system for improving diagnosis, treatment, and monitoring of patients with rare diseases
Screening of patients born small for gestational age with the Silver-Russell syndrome phenotype for DLK1 variants
Prioritization of disease genes from GWAS using ensemble-based positive-unlabeled learning
Multilocus disease-causing genomic variations for Mendelian disorders: role of systematic phenotyping and implications on genetic counselling
Why do people seek out polygenic risk scores for complex disorders, and how do they understand and react to results?
Clinical genetics—it’s polygenic
A systematic review assessing the existence of pneumothorax-only variants of FLCN. Implications for lifelong surveillance of renal tumours
Correction: A MT-TL1 variant identified by whole exome sequencing in an individual with intellectual disability, epilepsy, and spastic tetraparesis
Allele frequency differentiation at height-associated SNPs among continental human populations
Dutch Pharmacogenetics Working Group (DPWG) guideline for the gene–drug interaction between CYP2D6 and opioids (codeine, tramadol and oxycodone)
Biallelic variants in genes previously associated with dominant inheritance: CACNA1A, RET and SLC20A2
Pathogenic variants in PIDD1 lead to an autosomal recessive neurodevelopmental disorder with pachygyria and psychiatric features
Parent clinical trial priorities for fragile X syndrome: a best–worst scaling
Solving the unsolved rare diseases in Europe
Correction to: Toward the diagnosis of rare childhood genetic diseases: what do parents value most?
A comparison of genotyping arrays European and African populations
Mapping pleiotropic loci using a fast-sequential testing algorithm white European participants
ADAMTS1, MPDZ, MVD, and SEZ6: candidate genes for autosomal recessive nonsyndromic hearing impairment
Couples’ experiences with expanded carrier screening: evaluation of a university hospital screening offer
Cascade screening for beta-thalassemia in Pakistan: development, feasibility and acceptability of a decision support intervention for relatives
Genomic sequencing for the diagnosis of childhood mitochondrial disorders: a health economic evaluation
PRICKLE2 revisited—further evidence implicating PRICKLE2 in neurodevelopmental disorders
Contribution of common risk variants to multiple sclerosis in Orkney and Shetland
A MT-TL1 variant identified by whole exome sequencing in an individual with intellectual disability, epilepsy, and spastic tetraparesis
Exome reanalysis and proteomic profiling identified TRIP4 as a novel cause of cerebellar hypoplasia and spinal muscular atrophy (PCH1)
Solving patients with rare diseases through programmatic reanalysis of genome-phenome data
Solve-RD: systematic pan-European data sharing and collaborative analysis to solve rare diseases pan-European; European Reference Networks; Europe; European
A mosaic PIK3CA variant in a young adult with diffuse gastric cancer: case report
Correction to: First-line exome sequencing in Palestinian and Israeli Arabs with neurological disorders is efficient and facilitates disease gene discovery
Cell-based assay for ciliopathy patients to improve accurate diagnosis using ALPACA
Clinical utility gene card for: Long-QT syndrome
Phenotypic homogeneity in childhood epilepsies evolves in gene-specific patterns across 3251 patient-years of clinical data
Stakeholder engagement to ensure the sustainability of biobanks: a survey of potential users of biobank services
Consolidating biallelic SDHD variants as a cause of mitochondrial complex II deficiency
Clinical genomics—but faster
The genetic structure of Norway Uralic Sami ancestry; Norwegian population; Scandinavian; mainland Europe
Balancing scientific interests and the rights of participants in designing a recall by genotype study
Stepwise ABC system for classification of any type of genetic variant
Deletion of the last two exons of FGF10 in a family with LADD syndrome and pulmonary acinar hypoplasia
Out now in May’s EJHG
Solving unsolved rare neurological diseases—a Solve-RD viewpoint
Phylogenetic history of patrilineages rare in northern and eastern Europe from large-scale re-sequencing of human Y-chromosomes northern and eastern Europe
Frequency and origin of the c.2090T>G p.(Leu697Trp) MYO3A variant associated with autosomal dominant hearing loss
Additive effect of frequent polymorphism and rare synonymous variant alters splicing in twin patients with Niemann-Pick disease type C
Evidence of mosaicism in SPAST variant carriers in four French families
International transfers of personal data for health research following Schrems II: a problem in need of a solution
Correction to: Resolving pathogenicity classification for the CDH1 c.[715G>A] (p.Gly239Arg) variant
Polygenic risk score analysis for amyotrophic lateral sclerosis leveraging cognitive performance, educational attainment and schizophrenia
Speech and language deficits are central to SETBP1 haploinsufficiency disorder
How do non-geneticist physicians deal with genetic tests? A qualitative analysis
Identifying challenges in neurofibromatosis: a modified Delphi procedure
Toward the diagnosis of rare childhood genetic diseases: what do parents value most?
A 127 kb truncating deletion of PGRMC1 is a novel cause of X-linked isolated paediatric cataract
Germline variants in UNC13D and AP3B1 are enriched in COVID-19 patients experiencing severe cytokine storms
Patient-reported anticipated barriers and benefits to sharing cancer genetic risk information with family members
Clinical delineation of SETBP1 haploinsufficiency disorder
Moving somatic gene editing to the clinic: routes to market access and reimbursement in Europe
Genetic drift from the out-of-Africa bottleneck leads to biased estimation of genetic architecture and selection
Consensus statement on standards and guidelines for the molecular diagnostics of Alport syndrome: refining the ACMG criteria
Familial wild-type gastrointestinal stromal tumour in association with germline truncating variants in both SDHA and PALB2
Comprehensive germline-genomic and clinical profiling in 160 unselected children and adolescents with cancer
What people really change after genetic testing (GT) performed in private labs: results from an Italian study
Biallelic loss-of-function variants in KCNJ16 presenting with hypokalemic metabolic acidosis
Confirmation of COL4A6 variants in X-linked nonsyndromic hearing loss and its clinical implications
What’s new in EJHG in April
Fine-scale population structure in five rural populations from the Spanish Eastern Pyrenees using high-coverage whole-genome sequence data European rural areas
Deletion in COL4A2 is associated with a three-generation variable phenotype: from fetal to adult manifestations
Regional heritability mapping identifies several novel loci (STAT4, ULK4, and KCNH5) for primary biliary cholangitis in the Japanese population
Molecular genetic testing of congenital adrenal hyperplasia due to 21-hydroxylase deficiency should include CAH-X chimeras
Reply to Lao Q and Merke DP
Evaluating the monogenic contribution and genotype–phenotype correlation in patients with isolated thoracic aortic aneurysm
Allelic and phenotypic heterogeneity in Junctophillin-3 related neurodevelopmental and movement disorders
Biallelic hypomorphic mutations in HEATR5B, encoding HEAT repeat-containing protein 5B, in a neurological syndrome with pontocerebellar hypoplasia
Preferences and values for rapid genomic testing in critically ill infants and children: a discrete choice experiment
Stakeholders’ views of integrating universal tumour screening and genetic testing for colorectal and endometrial cancer into routine oncology
Maternal transmission of a mild Coffin–Siris syndrome phenotype caused by a SOX11 missense variant
Whole exome sequencing reveals putatively novel associations in retinopathies and drusen formation
Widening the lens of actionability: A qualitative study of primary care providers’ views and experiences of managing secondary genomic findings
Peter Harper
Reviewer recognition
Autosomal recessive cataract (CTRCT18) in the Yakut population isolate of Eastern Siberia: a novel founder variant in the FYCO1 gene
Identification of novel locus associated with coronary artery aneurysms and validation of loci for susceptibility to Kawasaki disease European descent; European GWAS; Europeans; East Asian; Japanese
Correction: Long-read trio sequencing of individuals with unsolved intellectual disability
Remembering GertJan van Ommen
Novel bi-allelic variants expand the SPTBN4-related genetic and phenotypic spectrum
Albert de la Chapelle (1933–2020)
The Ami and Yami aborigines of Taiwan and their genetic relationship to East Asian and Pacific populations
Unraveling a fine-scale high genetic heterogeneity and recent continental connections of an Arabian Peninsula population sub-Saharan/Western Africa; West Eurasia; Arabian Peninsula; Kuwait population; continental
Analysis of HLA gene polymorphisms in East Africans reveals evidence of gene flow in two Semitic populations from Sudan
Is it time to report carrier state for recessive disorders in every microarray analysis?—A pilot model based on hearing loss genes deletions
Resequencing of candidate genes for Keratoconus reveals a role for Ehlers–Danlos Syndrome genes
Natural human knockouts and Mendelian disorders: deep phenotyping in Italian isolates
Mainstream genetic testing for breast cancer patients: early experiences from the Parkville Familial Cancer Centre
Correction: Speech and language phenotype in Phelan-McDermid (22q13.3) syndrome
Homozygous variant in MADD, encoding a Rab guanine nucleotide exchange factor, results in pleiotropic effects and a multisystemic disorder
Correction: Self-reported hearing loss questions provide a good measure for genetic studies: a polygenic risk score analysis from UK Biobank
Allele frequency of variants reported to cause adenine phosphoribosyltransferase deficiency European population; Irish population; Japan; Iceland; Japanese
Correction: Bi-allelic loss of function variants in SLC30A5 as cause of perinatal lethal cardiomyopathy
Genome-wide haplotype association study in imaging genetics using whole-brain sulcal openings of 16,304 UK Biobank subjects
The limits of normal approximation for adult height
Colocalization analysis of polycystic ovary syndrome to identify potential disease-mediating genes and proteins European ancestry; European descent; Han Chinese
Germline FFPE inherited cancer panel testing in deceased family members: implications for clinical management of unaffected relatives
MCM complex members MCM3 and MCM7 are associated with a phenotypic spectrum from Meier-Gorlin syndrome to lipodystrophy and adrenal insufficiency
Novel variants in TUBA1A cause congenital fibrosis of the extraocular muscles with or without malformations of cortical brain development
Direct to consumer genetic testing in Denmark—public knowledge, use, and attitudes
Lung expression of genes putatively involved in SARS-CoV-2 infection is modulated in cis by germline variants
Heterozygous COL9A3 variants cause severe peripheral vitreoretinal degeneration and retinal detachment
Clinical utility gene card for FRMD7-related infantile nystagmus
Complete mitogenomes document substantial genetic contribution from the Eurasian Steppe into northern Pakistani Indo-Iranian speakers Eurasian Steppe
Genetic correlations between traits associated with hyperuricemia, gout, and comorbidities
The impact of unsolicited findings in clinical exome sequencing, a qualitative interview study
Resolving pathogenicity classification for the CDH1 c.[715G>A] (p.Gly239Arg) Variant
Stakeholder views on opportunistic genomic screening in the Netherlands: a qualitative study
Further delineation of BCAP31-linked intellectual disability: description of 17 new families with LoF and missense variants
Normalising life at risk of Huntington’s disease. A qualitative study of backgrounds and coping strategies of fears of genetic discrimination
The question of WGS’s clinical utility remains unanswered
Quantitative facial phenotyping for Koolen-de Vries and 22q11.2 deletion syndrome
Syndromic disorders caused by gain-of-function variants in KCNH1, KCNK4, and KCNN3—a subgroup of K+ channelopathies
Correction: ESHG warns against misuses of genetic tests and biobanks for discrimination purposes
ERBB4 exonic deletions on chromosome 2q34 in patients with intellectual disability or epilepsy
Reply letter to Battke et al.
The rise of point-of-care genetics: how the SARS-CoV-2 pandemic will accelerate adoption of genetic testing in the acute setting
Polygenic risk modeling with latent trait-related genetic components
Evaluating the potential of novel genetic approaches for the treatment of Duchenne muscular dystrophy
Bi-allelic loss of function variants in SLC30A5 as cause of perinatal lethal cardiomyopathy
Origins of human genetics. A personal perspective
Novel variants in the stem cell niche factor WNT2B define the disease phenotype as a congenital enteropathy with ocular dysgenesis
Clinical utility gene card for oculocutaneous (OCA) and ocular albinism (OA)—an update
Lifestyle Risk Score: handling missingness of individual lifestyle components in meta-analysis of gene-by-lifestyle interactions
Semi-parametric empirical Bayes factor for genome-wide association studies
Homozygous variants in PANX1 cause human oocyte death and female infertility
Identifying the nature and extent of public and donor concern about the commercialisation of biobanks for genomic research
Employing a systematic approach to biobanking and analyzing clinical and genetic data for advancing COVID-19 research
Parents, their children, whole exome sequencing and unsolicited findings: growing towards the child’s future autonomy
The ethics of genomic medicine: redefining values and norms in the UK and France
The genetic landscape of polycystic kidney disease in Ireland
In Memoriam: Gertjan van Ommen (1947–2020)
A reply to: Longitudinal changes in the frequency of mosaic chromosome Y loss in peripheral blood cells of aging men varies profoundly between individuals
ESHG warns against misuses of genetic tests and biobanks for discrimination purposes
Reply to Veitia
A randomized experimental study to test the effects of discussing uncertainty during cancer genetic counseling: different strategies, different outcomes?
Comprehensive study of 28 individuals with SIN3A-related disorder underscoring the associated mild cognitive and distinctive facial phenotype
Genomic diagnostics in polycystic kidney disease: an assessment of real-world use of whole-genome sequencing
Extension of SKAT to multi-category phenotypes through a geometrical interpretation
Correction: Determination of the phylogenetic origins of the Árpád Dynasty based on Y chromosome sequencing of Béla the Third