Clinical Genetics - 2019

196 articles | Last updated: 2025-12-03 14:12:56
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T A T A T A T A
Is <i>BRCA2</i> involved in early onset colorectal cancer risk?
Front Cover
Issue Information – Editorial Board
<i>VPS26C</i> homozygous nonsense variant in two cousins with neurodevelopmental deficits, growth failure, skeletal abnormalities, and distinctive facial features
Psychological factors that determine people's willingness‐to‐share genetic data for research
Uptake of polygenic risk information among women at increased risk of breast cancer
Widening the spectrum of genetic testing in familial hypercholesterolaemia: Will it translate into better patient and population outcomes?
Occurrence and characterization of medulloblastoma in a patient with Curry‐Jones syndrome
Missense mutations in <i>SLC25A1</i> are associated with congenital myasthenic syndrome type 23
<i>COL1</i>‐related overlap disorder: A novel connective tissue disorder incorporating the osteogenesis imperfecta/Ehlers‐Danlos syndrome overlap
Integrated analysis of <i>COL2A1</i> variant data and classification of type II collagenopathies
Phenotypic delineation of the retinal arterial macroaneurysms with supravalvular pulmonic stenosis syndrome
Null variants in <i>AGRN</i> cause lethal fetal akinesia deformation sequence
The great mimicker: Phenotypic overlap between constitutional mismatch repair deficiency and Tuberous Sclerosis complex
Front Cover
Clinical and genetic findings in a cohort of Chinese patients with autosomal recessive spinocerebellar ataxia
Issue Information ‐ Editorial Board
Functional characterization of novel <i>MFSD8</i> pathogenic variants anticipates neurological involvement in juvenile isolated maculopathy
Chromatinopathies: A focus on Cornelia de Lange syndrome
Sorting nexin 27 (<i>SNX27</i>) variants associated with seizures, developmental delay, behavioral disturbance, and subcortical brain abnormalities
Hereditary spastic paraplegia is a novel phenotype for germline de novo <i>ATP1A1</i> mutation
X‐linked intellectual disability: Phenotypic expression in carrier females
Novel <i>CDH3</i> variants in Brazilian families with hypotrichosis and juvenile macular dystrophy revealed by exome sequencing
Clinical variability and probable founder effect in oculocutaneous albinism type 7
Cerebellar ataxia with normal intellect associated with a homozygous truncating variant in <i>CA8</i>
Who should access germline genome sequencing? A mixed methods study of patient views
Phenotype‐to‐genotype approach reveals head‐circumference‐associated genes in an autism spectrum disorder cohort
Loss‐of‐function mutations in centrosomal protein 112 is associated with human acephalic spermatozoa phenotype
Decisional conflict among adolescents and parents making decisions about genomic sequencing results
Novel Arg128Ala variant in Catechol‐O‐methyltransferase gene influence persistent pain
Clinical and genetic spectrum in 33 Egyptian families with suspected primary ciliary dyskinesia
Novel variants in <i>CDH2</i> are associated with a new syndrome including Peters anomaly
Clinical diagnostic exome sequencing in dystonia: Genetic testing challenges for complex conditions
Issue Information – Editorial Board
Skeletal abnormalities are common features in Aymé‐Gripp syndrome
Patterns and predictors of genetic referral among ovarian cancer patients at a National Cancer Institute‐Comprehensive Cancer Center
Identification of a novel heterozygous guanosine monophosphate reductase (<i>GMPR</i>) variant in a patient with a late‐onset disorder of mitochondrial DNA maintenance
Application of the 2017 criteria for vascular Ehlers‐Danlos syndrome in 50 patients ascertained according to the Villefranche nosology
A genetic risk score predicts coronary artery disease in familial hypercholesterolaemia: enhancing the precision of risk assessment
Mutational spectrum by phenotype: panel‐based NGS testing of patients with clinical suspicion of RASopathy and children with multiple café‐au‐lait macules
A novel homozygous <i>CFAP65</i> mutation in humans causes male infertility with multiple morphological abnormalities of the sperm flagella
Evaluating empowerment in genetic counseling using patient‐reported outcomes
Targeted panel sequencing in pediatric primary cardiomyopathy supports a critical role of <i>TNNI3</i>
Classical Ehlers‐Danlos syndrome with a propensity to arterial events: A new report on a French family with a <i>COL1A1</i> p.(Arg312Cys) variant French family
The homozygous variant c.245G &gt; A/p.G82D in PNPLA2 is associated with arrhythmogenic cardiomyopathy phenotypic manifestations
Variant filtering, digenic variants, and other challenges in clinical sequencing: a lesson from fibrillinopathies
Growth factor and receptor malfunctions associated with human genetic deafness
A deep intronic <i>SMARCB1</i> variant associated with schwannomatosis
Crisponi/cold‐induced sweating syndrome: Differential diagnosis, pathogenesis and treatment concepts
Issue Information ‐ Editorial Board
Whole‐exome sequencing identifies rare pathogenic and candidate variants in sporadic Chinese Han deaf patients Chinese Han; ethnically matched controls
Novel mutations in <i>DPM3</i> cause dystroglycanopathy with central nervous system involvement
Novel homozygous variations in <i>PLCZ1</i> lead to poor or failed fertilization characterized by abnormal localization patterns of PLCζ in sperm
The activating p.Ser466Arg change in STAT1 causes a peculiar phenotype with features of interferonopathies
Causative and common <i>PHOX2B</i> variants define a broad phenotypic spectrum
The genome empowerment scale: An assessment of parental empowerment in families with undiagnosed disease
A homozygous missense variant in <i>CHRM3</i> associated with familial urinary bladder disease
Multiple roles and regulatory mechanisms of the transcription factor GATA6 in human cancers
Urogenital and pelvic complications in the Ehlers‐Danlos syndromes and associated hypermobility spectrum disorders: A scoping review
Prognostic and predictive role of DNA mismatch repair status in stage II‐III colorectal cancer: A systematic review and meta‐analysis
Familial pancreatic adenocarcinoma: A retrospective analysis of germline genetic testing in a French multicentre cohort
Molecular defects in thyroid dysgenesis
Novel variant p.E269K confirms causative role of <i>PLS1</i> mutations in autosomal dominant hearing loss
Whole exome and targeted gene sequencing to detect pathogenic recessive variants in early onset cerebellar ataxia
Meckel syndrome: Clinical and mutation profile in six fetuses
Sex‐limited penetrance of lymphedema to females with <i>CELSR1</i> haploinsufficiency: A second family
Reevaluation of genetic variants previously associated with arrhythmogenic right ventricular cardiomyopathy integrating population‐based cohorts and proteomics data
Issue Information ‐ Editorial Board
Speech, language, and feeding phenotypes of <i>SATB2</i>‐associated syndrome
p21 protein‐activated kinase 1 is associated with severe regressive autism, and epilepsy
<i>MAGEL2</i>‐related disorders: A study and case series
Identification of a dominant <i>MYH11</i> causal variant in chronic intestinal pseudo‐obstruction: Results of whole‐exome sequencing
Genetic spectrum and clinical profiles in a southeast Chinese cohort of Charcot‐Marie‐Tooth disease
Comprehensive characterization of a Canadian cohort of von Hippel‐Lindau disease patients
What is new about the genetic background of Hirschsprung disease?
FAM160B1 deficit associated with microcephaly, severe intellectual disability, ataxia, behavioral abnormalities and speech problems
<i>FARSA</i> mutations mimic phenylalanyl‐tRNA synthetase deficiency caused by <i>FARSB</i> defects
Functional analysis of novel <i>RUNX2</i> mutations identified in patients with cleidocranial dysplasia Polish
Syndromic chorioretinal coloboma associated with heterozygous de novo <i>RARA</i> mutation affecting an amino acid critical for retinoic acid interaction
Serum deprivation response functions as a tumor suppressor gene in papillary thyroid cancer
Genetic spectrum of renal disease for 1001 Chinese children based on a multicenter registration system
Heterozygous <i>CTNNB1</i> and <i>TBX4</i> variants in a patient with abnormal lung growth, pulmonary hypertension, microcephaly, and spasticity
Bioinformatic detection of copy number variation in <i>HNF4A</i> causing maturity onset diabetes of the young
Development of a measure of genome sequencing knowledge for young people: The kids‐KOGS
Neurodevelopmental disorder with progressive microcephaly, spasticity, and brain anomalies in China caused by novel mutations of <i>PLAA</i>
The third family with Eiken syndrome
Whole exome sequencing of men with multiple morphological abnormalities of the sperm flagella reveals novel homozygous <i>QRICH2</i> mutations
<i>FGF12</i>p.Gly112Ser variant as a cause of phenytoin/phenobarbital responsive epilepsy
Issue Information ‐ Editorial Board
Confirmation that variants in <i>TTI2</i> are responsible for autosomal recessive intellectual disability
Germline mutations and new copy number variants among 40 pediatric cancer patients suspected for genetic predisposition
Biallelic mutations in Sperm flagellum 2 cause human multiple morphological abnormalities of the sperm flagella (MMAF) phenotype
Intrafamilial variable spastic paraplegia/ataxia/ALS phenotype linked to a novel KIF5A mutation
Novel heterozygous variants in <i>KMT2D</i> associated with holoprosencephaly
A review of neurocognitive functioning of children with sex chromosome trisomies: Identifying targets for early intervention
Clinicogenetic lessons from 370 patients with autosomal recessive limb‐girdle muscular dystrophy
French‐style genetics v. 2.0: The “e‐CohortE” project
A novel bi‐allelic loss‐of‐function variant in <i>MYOD1</i>: Further evidence for gene‐disease association and phenotypic variability in <i>MYOD1</i>‐related myopathy
<i>FLNC</i> pathogenic variants in patients with cardiomyopathies: Prevalence and genotype‐phenotype correlations
Advances in the gene therapy of monogenic blood cell diseases
Application of <i>CRISPR/Cas9</i> gene editing technique in the study of cancer treatment
Novel TRRAP mutation causes autosomal dominant non‐syndromic hearing loss
Two additional males with X‐linked, syndromic mental retardation carry de novo mutations in <i>HNRNPH2</i>
Pycnodysostosis: Natural history and management guidelines from 27 French cases and a literature review
Evaluation of <i>SHOX</i> defects in the era of next‐generation sequencing
Issue Information ‐ Editorial Board
Front Cover
Molecular and phenotypic spectrum of Noonan syndrome in Chinese patients Chinese; China
Genotype‐phenotype relationships in mucopolysaccharidosis type I (MPS I): Insights from the International MPS I Registry
Exome sequencing findings in 27 patients with myoclonic‐atonic epilepsy: Is there a major genetic factor?
Inter‐lab concordance of variant classifications establishes clinical validity of expanded carrier screening
Issue Information ‐ Editorial Board
Allele frequency of pathogenic variants related to adult‐onset Mendelian diseases
Epithelial ovarian cancer risk: A review of the current genetic landscape
The choice not to undergo genetic testing for Huntington disease: Results from the PHAROS study
Delineation of <i>MidXq28‐duplication syndrome</i> distal to <i>MECP2</i> and proximal to <i>RAB39B</i> genes
Functional characterization of novel germline <i>TP53</i> variants in Swedish families
A novel <i>WARS</i> mutation (p.Asp314Gly) identified in a Chinese distal hereditary motor neuropathy family Chinese
Heterozygous missense variants of <i>SPTBN2</i> are a frequent cause of congenital cerebellar ataxia
Low‐depth sequencing for copy number abnormalities in multiple myeloma supersedes fluorescent in situ hybridization in scope and resolution
Congenital muscular dystrophies in China
A novel homozygous splice‐site variant of <i>NCAPD2</i> gene identified in two siblings with primary microcephaly: The second case report
16p13.11 microdeletion uncovers loss‐of‐function of a <i>MYH11</i> missense variant in a patient with megacystis‐microcolon‐intestinal‐hypoperistalsis syndrome
Molecular genetic study of acute intermittent porphyria in Russia: <i>HMBS</i> gene mutation spectrum and problem of penetrance
Whole genome sequencing and variant discovery in the ASPIRE autism spectrum disorder cohort
Old treatments for new genetic conditions: Sirolimus therapy in a child affected by mosaic overgrowth with fibroadipose hyperplasia
The clinical presentation caused by truncating <i>CHD8</i> variants
An overview of the genetic basis of epidermolysis bullosa in Brazil: discovery of novel and recurrent disease‐causing variants
Issue Information ‐ Editorial Board
Trends in phenotype in the English paediatric neurofibromatosis type 2 cohort stratified by genetic severity
Clinical implications of the oncometabolite succinate in <i>SDHx</i>‐mutation carriers
Brugada syndrome with SCN5A mutations exhibits more pronounced electrophysiological defects and more severe prognosis: A meta‐analysis
Identification of disease‐causing variants in the <i>EXOSC</i> gene family underlying autosomal recessive intellectual disability in Iranian families Iranian families
Whole‐exome sequencing identified <i>ARL2</i> as a novel candidate gene for MRCS (microcornea, rod‐cone dystrophy, cataract, and posterior staphyloma) syndrome
Whole‐exome sequencing revealed a nonsense mutation in <i>STKLD1</i> causing non‐syndromic pre‐axial polydactyly type A affecting only upper limb Pakistani origin
The combination of whole‐exome sequencing and copy number variation sequencing enables the diagnosis of rare neurological disorders
Consensus recommendations for diagnosis, management and treatment of Fabry disease in paediatric patients
Bone Dysplasias. An Atlas of Genetic Disorders of Skeletal Development (Fourth Edition)
Current knowledge of medical complications in adults with achondroplasia: A scoping review
Early activating somatic <i>PIK3CA</i> mutations promote ectopic muscle development and upper limb overgrowth
Heterogeneity and overlaps in nucleotide excision repair disorders
<i>ADA2</i> deficiency due to a novel structural variation in 22q11.1
Novel <i>COL12A1</i> variant as a cause of mild familial extracellular matrix‐related myopathy
Autosomal recessive limb‐girdle and Miyoshi muscular dystrophies in the Netherlands: The clinical and molecular spectrum of 244 patients
Identification of <i>SLC20A2</i> deletions in patients with primary familial brain calcification
<i>ATP1A3</i> mosaicism in families with alternating hemiplegia of childhood
Issue Information ‐ Editorial Board
Bain type of X‐linked syndromic mental retardation in boys
Genome‐wide association study identifies new susceptibility loci for diabetic nephropathy in Korean patients with type 2 diabetes mellitus Korean
Phenotypic spectrum of <i>NRXN1</i> mono‐ and bi‐allelic deficiency: A systematic review
Genetic counselors' preferences for coverage of preimplantation genetic diagnosis: A discrete choice experiment
Exome sequencing in Crisponi/cold‐induced sweating syndrome–like individuals reveals unpredicted alternative diagnoses
Delineation of dominant and recessive forms of <i>LZTR1</i>‐associated Noonan syndrome
Assessing optimism and pessimism about genomic medicine: Development of a genomic orientation scale
Lessons from exome sequencing in prenatally diagnosed heart defects: A basis for prenatal testing
Uncertainty, hope, and coping efficacy among mothers of children with Duchenne/Becker muscular dystrophy
A novel gene (<i>FAM20B</i> encoding glycosaminoglycan xylosylkinase) for neonatal short limb dysplasia resembling Desbuquois dysplasia
Exome sequencing reveals novel variants and unique allelic spectrum for hearing impairment in Filipino cochlear implantees
Telomeropathies: Etiology, diagnosis, treatment and follow‐up. Ethical and legal considerations
Generality of genomic findings on blood pressure traits and its usefulness in precision medicine in diverse populations: A systematic review Europeans
<i>DNAH2</i> is a novel candidate gene associated with multiple morphological abnormalities of the sperm flagella
A homozygous frameshift variant in an alternatively spliced exon of <i>DLG5</i> causes hydrocephalus and renal dysplasia
Characterizing the phenotypic effect of Xq28 duplication size in <i>MECP2</i> duplication syndrome
Clinical delineation of <i>GTPBP2</i>‐associated neuro‐ectodermal syndrome: Report of two new families and review of the literature black African
Systematic review of quality of life in persons with hereditary thoracic aortic aneurysm and dissection diagnoses
PKD1L1‐related situs inversus associated with sideroblastic anemia
Clinical and genetic characterization of a cohort of Chinese patients with hereditary spastic paraplegia
Issue Information ‐ Editorial Board
Front Cover
<i>ADAMTSL1</i> and mandibular prognathism Thai
Further quantitative insights into the decrease of heteroplasmy of m.3243A&gt;G with age in leukocytes
Expanding the clinical spectrum associated with <i>PACS2</i> mutations
Deep phenotyping of 14 new patients with <i>IQSEC2</i> variants, including monozygotic twins of discordant phenotype
Liquid biopsy in breast cancer: A comprehensive review
Diagnosis and management in Pitt‐Hopkins syndrome: First international consensus statement
Assessment of pre‐implantation genetic testing for embryo aneuploidies: A SWOT analysis
Difficulties and challenges in the development of precision medicine
Clinical and molecular diagnosis of non‐phosphomannomutase 2 N‐linked congenital disorders of glycosylation in Spain
Newly identified set of obesity‐related genotypes and abdominal fat influence the risk of insulin resistance in a Korean population
Hypermobile Ehlers‐Danlos‐like syndrome in Fabry disease
Constitutional mosaicism in <i>RASA1</i>‐related capillary malformation‐arteriovenous malformation
Correction to: Mutational characteristics of <i>ANK1</i> and <i>SPTB</i> genes in hereditary spherocytosis
Neurodevelopmental disorder with dysmorphic facies and distal limb anomalies syndrome due to disruption of <i>BPTF</i> in a 35‐year‐old man initially diagnosed with Silver‐Russell syndrome
Fetal phenotype of Rubinstein‐Taybi syndrome caused by <i>CREBBP</i> mutations
Clinical, biomarker and genetic spectrum of Niemann‐Pick type C in Egypt: The detection of nine novel <i>NPC1</i> mutations
Issue Information ‐ Editorial Board
Novel mutations in <i>WEE2</i>: Expanding the spectrum of mutations responsible for human fertilization failure
MRX93 syndrome (<i>BRWD3</i> gene): five new patients with novel mutations
Variants of the <i>ectodysplasin A1 receptor</i> gene underlying homozygous cases of autosomal recessive hypohidrotic ectodermal dysplasia Icelandic population; families of Turkish, Austrian, and German-American origin; Icelandic population
A novel homozygous sequence variant in <i>GLI1</i> underlies first case of autosomal recessive pre‐axial polydactyly
Bardet‐Biedl syndrome: Antenatal presentation of forty‐five fetuses with biallelic pathogenic variants in known Bardet‐Biedl syndrome genes
Long non‐coding RNAs differential expression in breast cancer subtypes: What do we know?
The genetic pathogenesis, diagnosis and therapeutic insight of rheumatoid arthritis
A study in a Polish ataxia cohort indicates genetic heterogeneity and points to <i>MTCL1</i> as a novel candidate gene
MicroRNA single‐nucleotide polymorphisms and diabetes mellitus: A comprehensive review
A genome‐wide association study identifies new genes associated with developmental dysplasia of the hip Chinese Han