Clinical Genetics - 2020

212 articles | Last updated: 2025-12-03 14:12:56
Caucasian
1
White
1
European
2
Other
22
Article Title Cauc. White Euro. Other Phrases Used
T A T A T A T A
Issue Information
Congenital hypothyroidism and hearing loss without inner ear malformation: Think <scp><i>TPO</i></scp>
<i>Clinical Genetics</i> paving the way to the future
Molecular intrinsic versus clinical subtyping in breast cancer: A comprehensive review
Progress on the role of extrachromosomal <scp>DNA</scp> in tumor pathogenesis and evolution
Biallelic mutations of <scp><i>CFAP58</i></scp> are associated with multiple morphological abnormalities of the sperm flagella
Dominant variants in <scp><i>PRR12</i></scp> result in unilateral or bilateral complex microphthalmia
Infantile Liver Failure Syndrome 1 associated with a novel variant of the <scp><i>LARS1</i></scp> gene: Clinical, genetic, and functional characterization
Novel <scp><i>ACTG2</i></scp> variants disclose allelic heterogeneity and bi‐allelic inheritance in pediatric chronic intestinal <scp>pseudo‐obstruction</scp>
Neuropsychological study in 19 French patients with <scp>White‐Sutton</scp> syndrome and <scp><i>POGZ</i></scp> mutations
A novel stop codon variant affecting <scp>ΔNp63</scp> isoforms associated with non‐syndromic <scp>limb‐mammary</scp> phenotype and uterine cervix dysplasia
Indigenous Arabs have an intermediate frequency of a Neanderthal‐derived <scp>COVID</scp>‐19 risk haplotype compared with other world populations
Molecular and histologic insights on early onset cardiomyopathy in Danon disease females
Functional evidence of <scp>mTORβ</scp> splice variant involvement in the pathogenesis of congenital heart defects
Selective forces acting on spinocerebellar ataxia type 3/<scp>Machado–Joseph</scp> disease recurrency: A systematic review and meta‐analysis
<scp>Genotype‐phenotype</scp> study and expansion of <scp><i>ARL6IP1</i>‐related</scp> complicated hereditary spastic paraplegia
Issue Information – Editorial Board
Deficiency of <scp>acyl‐CoA</scp> synthetase 5 is associated with a severe and treatable failure to thrive of neonatal onset
Genotype <scp><i>FBN1</i></scp>/phenotype relationship in a cohort of patients with Marfan syndrome
Description of neurodevelopmental phenotypes associated with 10 genetic neurodevelopmental disorders: A scoping review
Genetic spectrum of <scp>Charcot–Marie–Tooth</scp> disease associated with myelin protein zero gene variants in Japan
A <scp><i>BBS1</i> SVA</scp> F retrotransposon insertion is a frequent cause of <scp>Bardet‐Biedl</scp> syndrome
PIGH deficiency can be associated with severe neurodevelopmental and skeletal manifestations
A review on age‐related cancer risks in <scp>PTEN</scp> hamartoma tumor syndrome
Identification of the third <scp><i>FGF9</i></scp> variant in a girl with multiple synostosis–comparison of the genotype:phenotype of <scp><i>FGF9</i></scp> variants in humans and mice
Expanding the molecular spectrum and the neurological phenotype related to <scp><i>CAMTA1</i></scp> variants
An X‐linked syndrome with severe neurodevelopmental delay, hydrocephalus, and early lethality caused by a missense variation in the <scp><i>OTUD5</i></scp> gene
<scp>Parkes‐Weber</scp> syndrome related to <scp><i>RASA1</i></scp> mosaic mutation
<scp><i>WDR34</i></scp>, a candidate gene for non‐syndromic rod‐cone dystrophy
A novel monogenic preimplantation genetic testing strategy for sporadic polycystic kidney caused by <i>de novo</i><scp><i>PKD1</i></scp> mutation
Psychosis in <scp>NUS1</scp> de novo mutation: New phenotypical presentation
Clinical application of a phenotype‐based <scp>NGS</scp> panel for differential diagnosis of inherited kidney disease and beyond
Vestibular <scp>phenotype‐genotype</scp> correlation in a cohort of 90 patients with Usher syndrome
Novel loss‐of‐function variants in <scp><i>DNAH17</i></scp> cause multiple morphological abnormalities of the sperm flagella in humans and mice Pakistani and Chinese origins
Bifid nose as the sole manifestation of <scp>BNAR</scp> syndrome, a <scp><i>FREM1</i></scp>‐related condition
Issue Information
An additional piece in the <scp><i>TBX6</i></scp> gene dosage model: A novel nonsense variant in a fetus with severe spondylocostal dysostosis
Cutis laxa: A comprehensive overview of clinical characteristics and pathophysiology
Precise variant interpretation, phenotype ascertainment, and genotype–phenotype correlation of children in the <scp>EARLY PRO‐TECT</scp> Alport trial
Exploring genotype‐phenotype relationships in the <scp>CDKL5</scp> deficiency disorder using an international dataset
Feedback of extended panel sequencing in 1530 patients referred for suspicion of hereditary predisposition to adult cancers
Genetic disorders with central nervous system white matter abnormalities: An update
Missing heritability in Bloom syndrome: First report of a deep intronic variant leading to pseudo‐exon activation in the <scp><i>BLM</i></scp> gene
Age of onset and behavioral manifestations in Huntington's disease: An <scp>Enroll‐HD</scp> cohort analysis
<scp><i>QRICH1</i></scp> variants in <scp>Ververi‐Brady</scp> syndrome—delineation of the genotypic and phenotypic spectrum
Expanding the genetic and phenotypic spectrum of the subcortical maternal complex genes in recurrent preimplantation embryonic arrest
Mutation analysis of tubulin beta 8 class <scp>VIII</scp> in infertile females with oocyte or embryonic defects
Heterozygous de novo variants in <scp><i>CSNK1G1</i></scp> are associated with syndromic developmental delay and autism spectrum disorder
Copy number alterations involving 59 <scp>ACMG</scp>‐recommended secondary findings genes
Novel <scp><i>GZF1</i></scp> pathogenic variants identified in two Chinese patients with Larsen syndrome Chinese
Hydrothorax in fetal cases of Opitz G/<scp>BBB</scp> diagnosis: Extending the phenotype?
Variability of non‐lethal Fowler syndrome phenotype associated with <scp>FLVCR2</scp> variants
Issue Information – Editorial Board
Front Cover
Identification of a novel de novo mutation of <scp><i>ENG</i></scp> gene in a fetus with pulmonary arteriovenous malformation
Biallelic variants in <scp><i>GLE1</i></scp> with survival beyond neonatal period
Comprehensive <scp>genotype‐phenotype</scp> correlation in <scp>AP</scp>‐4 deficiency syndrome; Adding data from a large cohort of Iranian patients Iranian
Identifying haplotypes in recessive inherited retinal dystrophies using <scp>whole‐genome linked‐read</scp> sequencing
The evolution of the nosology of osteogenesis imperfecta
<scp><i>MYORG</i></scp> gene disease‐causing variants in a family with primary familial brain calcification presenting with stroke‐like episodes
Diagnostic yield and clinical utility of whole exome sequencing using an automated variant prioritization system, <scp>EVIDENCE</scp>
Phenotypic and molecular spectrum of pyridoxamine‐5′‐phosphate oxidase deficiency: A scoping review of 87 cases of pyridoxamine‐5′‐phosphate oxidase deficiency
Homozygous <scp><i>GLUL</i></scp> deletion is embryonically viable and leads to glutamine synthetase deficiency
Issue Information – Editorial Board
<scp><i>EMC10</i></scp> homozygous variant identified in a family with global developmental delay, mild intellectual disability, and speech delay
Premature aging disorders: A clinical and genetic compendium
Spectrum and frequencies of non <scp><i>GJB2</i></scp> gene mutations in Czech patients with early non‐syndromic hearing loss detected by gene panel NGS and whole‐exome sequencing
<scp><i>ASAH1</i></scp>‐related disorders: Description of 15 novel pediatric patients and expansion of the clinical phenotype Egyptian
A novel missense variant in <scp><i>RBM10</i></scp> can cause a mild form of <scp>TARP</scp> syndrome with developmental delay and dysmorphic features
Novel pathogenic <scp><i>EIF2S3</i></scp> missense variants causing clinically variable <scp>MEHMO</scp> syndrome with impaired <scp>eIF2γ</scp> translational function, and literature review
Development of a new expanded next‐generation sequencing panel for genetic diseases involved in dyslipidemia
Arteriovenous malformation phenotype resembling congenital hemangioma contains <scp><i>KRAS</i></scp> mutations
Exome sequencing in patients with microphthalmia, anophthalmia, and coloboma (<scp>MAC</scp>) from a consanguineous population
Autosomal dominant neuronal ceroid lipofuscinosis: Clinical features and molecular basis
Homozygous <scp><i>TAF1C</i></scp> variants are associated with a novel childhood‐onset neurological phenotype
Ethical questions concerning newborn genetic screening
Heterogeneous phenotypes in families with duplications of the paternal allele within the imprinting center 1 (<i><scp>H19</scp>/<scp>IGF2</scp></i>:<scp>TSS‐DMR</scp>) in 11p15.5
Mutation spectrum and polygenic score in German patients with familial hypercholesterolemia German
Craniosynostosis‐microphthalmia syndrome belongs to the spectrum of <scp><i>BCOR</i></scp>‐related disorders
Micro and Martsolf syndromes in 34 new patients: Refining the phenotypic spectrum and further molecular insights
Compound heterozygous splicing <scp><i>CDON</i></scp> variants result in isolated ocular coloboma
Clinical features and underlying genetic causes in neonatal encephalopathy: A large cohort study Chinese patients
Evidence of the milder phenotypic spectrum of c.<scp>1582G</scp>&gt;A <scp><i>PIGT</i></scp> variant: Delineation based on seven novel Polish patients Polish patients
Whole‐exome sequencing in adult patients with developmental and epileptic encephalopathy: It is never too late Italian cohort
Corpus callosum thinning in autosomal dominant hereditary spastic paraplegia associated with a novel <scp><i>TUBβ4A</i></scp> mutation
Inherited cases of <scp><i>CNOT3</i></scp>‐associated intellectual developmental disorder with speech delay, autism, and dysmorphic facies
Siblings with a novel <scp><i>MED12</i></scp> variant and Odho syndrome with immune defects
<scp>COG6‐CDG</scp>: Expanding the phenotype with emphasis on glycosylation defects involved in the causation of male disorders of sex development
Spectrum of genes for inherited hearing loss in the Israeli Jewish population, including the novel human deafness gene <scp><i>ATOH1</i></scp>
Loss‐of‐function mutation in <scp><i>DNAH8</i></scp> induces asthenoteratospermia associated with multiple morphological abnormalities of the sperm flagella
Carrier screening of spinal muscular atrophy in North Indian population and its public health implications North Indian population
Combined in vitro and in silico analyses of <scp>FGFR1</scp> variants: genotype‐phenotype study in idiopathic hypogonadotropic hypogonadism Chinese population of central China
Issue Information – Editorial Board
Biallelic variants in <scp><i>PPP1R13L</i></scp> cause paediatric dilated cardiomyopathy
A homozygous <scp><i>HOXA11</i></scp> variation as a potential novel cause of autosomal recessive congenital anomalies of the kidney and urinary tract
Description of a family with X‐linked oculo‐auriculo‐vertebral spectrum associated with polyalanine tract expansion in <scp><i>ZIC3</i></scp>
Chromosome microarray analysis should be offered to all invasive prenatal diagnostic testing following a normal rapid aneuploidy test result
Fetal megacystis‐microcolon: Genetic mutational spectrum and identification of <scp><i>PDCL3</i></scp> as a novel candidate gene
A homozygous pathogenic variant in <scp><i>SHROOM3</i></scp> associated with anencephaly and cleft lip and palate
<i>De novo</i> missense variants in the <scp><i>RAP1B</i></scp> gene identified in two patients with syndromic thrombocytopenia
Pituitary stalk interruption syndrome broadens the clinical spectrum of the <scp>TTC26</scp> ciliopathy
An exome‐wide exploration of cases of primary ovarian insufficiency uncovers novel sequence variants and candidate genes
Phenotypic bases of <scp><i>NOTCH2NLC</i> GGC</scp> expansion positive neuronal intranuclear inclusion disease in a Southeast Asian cohort
The role of long non‐coding <scp>RNAs</scp> in drug resistance of cancer
A homozygous stop gain mutation in <scp><i>BOD1</i></scp> gene in a Lebanese patient with syndromic intellectual disability Lebanese; Iranian
Novel <scp><i>PCNT</i></scp> variants in <scp>MOPDII</scp> with attenuated growth restriction and pachygyria
Early‐onset nucleotide excision repair disorders with neurological impairment: Clues for early diagnosis and prognostic counseling
Issue Information – Editorial Board
Expanding the phenotypic spectrum consequent upon de novo <scp><i>WDR37</i></scp> missense variants
Genotype‐phenotype correlations on epidermolysis bullosa with congenital absence of skin: A comprehensive review
Diagnostic yield of targeted sequential and massive panel approaches for inherited neuropathies
Gene editing technology for improving life quality: A dream coming true?
Next‐generation sequencing through multigene panel testing for the diagnosis of hereditary epidermolysis bullosa in Chinese population
<scp><i>MECP2</i></scp> mutation spectrum and its clinical characteristics in a Chinese cohort
Next generation sequencing analysis of consecutive Russian patients with clinical suspicion of inborn errors of immunity
A novel ribosomal protein <scp>S20</scp> variant in a family with unexplained colorectal cancer and polyposis
Issue Information
Refinement of the clinical and mutational spectrum of <scp>UBE2A</scp> deficiency syndrome
Prenatal exome sequencing in fetuses with congenital heart defects
Clinical and radiological characterization of novel <scp><i>FIG4</i></scp>‐related combined system disease with neuropathy
Genetic predisposition in type 2 diabetes: A promising approach toward a personalized management of diabetes
A homozygous variant in <scp><i>NDUFA8</i></scp> is associated with developmental delay, microcephaly, and epilepsy due to mitochondrial complex I deficiency
Epidermolysis bullosa with congenital absence of skin: Clinical and genetic characterization of a <scp>23‐case</scp> series
A strategy using <scp>SNP</scp> linkage analysis for monogenic diseases <scp>PGD</scp> combined with <scp>HLA</scp> typing
<scp><i>GREB1L</i></scp> variants in familial and sporadic hereditary urogenital adysplasia and <scp>Mayer‐Rokitansky‐Kuster‐Hauser</scp> syndrome
High prevalence of <scp>Bardet‐Biedl</scp> syndrome in <i>La Réunion</i><scp>Island</scp> is due to a founder variant in <i>ARL6/BBS3</i>
Complete resolution of epileptic spasms with vigabatrin in a patient with 3‐methylglutaconic aciduria caused by <scp><i>TIMM50</i></scp> gene mutation
A novel <scp><i>TRAF3IP2</i></scp> variant causing familial scarring alopecia with mixed features of discoid lupus erythematosus and folliculitis decalvans consanguineous Lebanese family
Deletions of specific exons of <scp><i>FHOD3</i></scp> detected by next‐generation sequencing are associated with hypertrophic cardiomyopathy
<scp>Next‐generation</scp> sequencing approaches and challenges in the diagnosis of developmental anomalies and intellectual disability
<scp><i>HNRNPH1</i></scp>‐related syndromic intellectual disability: Seven additional cases suggestive of a distinct syndromic neurodevelopmental syndrome
Expanding the clinical spectrum of Fowler syndrome: Three siblings with survival into adulthood and systematic review of the literature
A small 7q11.23 microduplication involving <scp><i>GTF2I</i></scp> in a family with intellectual disability
Genetic Consultations in the Newborn. Robin D.Clark and Cynthia JCurry. Oxford University Press, 2019. <scp>ISBN</scp> 978‐0‐19‐999 099‐3
<scp><i>ADAMTS19</i></scp>‐associated heart valve defects: Novel genetic variants consolidating a recognizable cardiac phenotype
Front Cover
Issue Information
Kosaki overgrowth syndrome: A novel pathogenic variant in <scp><i>PDGFRB</i></scp> and expansion of the phenotype including cerebrovascular complications
Novel mutations in <scp><i>PMFBP1</i></scp>, <scp><i>TSGA10</i></scp> and <scp><i>SUN5</i></scp>: Expanding the spectrum of mutations that may cause acephalic spermatozoa
<scp><i>PDCD6IP</i></scp>, encoding a regulator of the <scp>ESCRT</scp> complex, is mutated in microcephaly
Comprehensive molecular analysis of 61 Egyptian families with hereditary nonsyndromic hearing loss European populations
Further delineation of the female phenotype with <scp><i>KDM5C</i></scp> disease causing variants: 19 new individuals and review of the literature
<scp><i>VEGFA</i></scp> variants as prognostic markers for the retinopathy in pseudoxanthoma elasticum
New insight into the role of substance P/neurokinin‐1 receptor system in breast cancer progression and its crosstalk with <scp>microRNAs</scp>
Primrose syndrome: Characterization of the phenotype in 42 patients
Epigenetic variations due to nutritional status in early‐life and its later impact on aging and disease
Mutational and clinical spectrum in a cohort of Chinese patients with hereditary nemaline myopathy Chinese
A novel homozygous variant in <scp><i>NLRP5</i></scp> is associate with human early embryonic arrest in a consanguineous Chinese family Chinese
Overlapping phenotypes between <scp>SHORT</scp> and Noonan syndromes in patients with <scp><i>PTPN11</i></scp> pathogenic variants
Genetics of feline hypertrophic cardiomyopathy
A nonsense variant in <scp><i>NME5</i></scp> causes human primary ciliary dyskinesia with radial spoke defects
A genome‐wide association study identifies <scp><i>FSHR</i></scp> rs2300441 associated with follicle‐stimulating hormone levels Chinese females
Encephalopathy due to defective mitochondrial and peroxisomal fission 2 caused by a novel <scp><i>MFF</i></scp> gene mutation in a young child Indian descent
A new 1p36.13‐1p36.12 microdeletion syndrome characterized by learning disability, behavioral abnormalities, and ptosis
When to think outside the autozygome: Best practices for exome sequencing in “consanguineous” families
Clinical implications of experimental analyses of AID function on predictive computational tools: Challenge of missense variants
Coexistence of autosomal dominant polycystic kidney disease type 1 and hereditary renal hypouricemia type 2: A model of early‐onset and fast cyst progression
Front Cover
Issue Information – Editorial Board
A paternally inherited non‐sense variant c.424G&gt;T (p.G142*) in the first exon of <i>XLαs</i> in an adult patient with hypophosphatemia and osteopetrosis
B4GALT1‐congenital disorders of glycosylation: Expansion of the phenotypic and molecular spectrum and review of the literature
Exploring the genetic pathogenicity of aortic dissection from 72 Han Chinese individuals using next‐generation sequencing Han Chinese
First report of childhood progressive cerebellar atrophy due to compound heterozygous <i>MTFMT</i> variants
PGT‐A preimplantation genetic testing for aneuploidies and embryo selection in routine ART cycles: Time to step back?
DNA methylation changes in cystic fibrosis: Cause or consequence?
A <scp><i>GLI3</i></scp> variant leading to polydactyly in heterozygotes and Pallister‐Hall‐like syndrome in a homozygote
A recurrent pathogenic variant in <scp><i>TPM2</i></scp> reveals further phenotypic and genetic heterogeneity in multiple pterygium syndrome‐related disorders
upd(20)mat is a rare cause of the Silver‐Russell‐syndrome‐like phenotype: Two unrelated cases and screening of large cohorts
Consequences of epigenetic derepression in facioscapulohumeral muscular dystrophy
Novel phenotype of syndromic premature ovarian insufficiency associated with <scp><i>TP63</i></scp> molecular defect
Functional analysis of <scp><i>SEMA3A</i></scp> variants identified in Chinese patients with isolated hypogonadotropic hypogonadism Chinese
Issue Information – Editorial Board
Front Cover
“RE: Lin JL, et al. ‘Immunologic assessment and KMT2D mutation detection in Kabuki syndrome.’ <i>Clin Genet</i>. 2015;88(3):255‐260”
New missense variants in <i>RELT</i> causing hypomineralised amelogenesis imperfecta UK Pakistani population
The genetic and clinical landscape of nanophthalmos and posterior microphthalmos in an Australian cohort predominantly European ancestry
Genetic basis of neurodevelopmental disorders in 103 Jordanian families
Molecular characterization of Spanish patients with <i>MECP2</i> duplication syndrome Spanish
Genetics of Wilson disease and Wilson‐like phenotype in a clinical series from eastern Spain
Applications of CRISPR‐Cas9 in gynecological cancer research
Longitudinal outcomes with cancer multigene panel testing in previously tested<i>BRCA1/2</i>negative patients
<i>LEF1</i> haploinsufficiency causes ectodermal dysplasia
Clinical and pathological characterization of <scp><i>FLNC</i></scp>‐related myofibrillar myopathy caused by founder variant c.8129G&gt;A in Hong Kong Chinese
Genetic heterogeneity in Leigh syndrome: Highlighting treatable and novel genetic causes
An <i>M1AP</i> homozygous splice‐site mutation associated with severe oligozoospermia in a consanguineous family
The third family with TAF6‐related phenotype: Alazami‐Yuan syndrome
Report of the first patient with a homozygous <i>OTUD7A</i> variant responsible for epileptic encephalopathy and related proteasome dysfunction
Identification of FOXH1 mutations in patients with sporadic conotruncal heart defect
The p.Arg377Trp variant in <i>ACTL6A</i> underlines a recognizable BAF‐opathy phenotype
Further definition of the proximal 19p13.3 microdeletion/microduplication syndrome and implication of <scp><i>PIAS4</i></scp> as the major contributor
Next‐generation sequencing through multi‐gene panel testing for diagnosis of hereditary ichthyosis in Chinese Chinese
Issue Information ‐ Editorial Board
Front Cover
Genetic and epigenetic regulation of abdominal aortic aneurysms
Variants in <i>NGLY1</i> lead to intellectual disability, myoclonus epilepsy, sensorimotor axonal polyneuropathy and mitochondrial dysfunction
Novel EWSR1‐VGLL1 fusion in a pediatric neuroepithelial neoplasm
Mitochondrial DNA pathogenic mutations in multiple symmetric lipomatosis
Bivariate genome‐wide association analysis identified three pleiotropic loci underlying osteoporosis and obesity
Diffuse capillary malformation with overgrowth contains somatic <i>PIK3CA</i> variants
Novel ZP1 pathogenic variants identified in an infertile patient and a successful live birth following ICSI treatment
A splice site mutation c.1251G&gt;A of <i>ISPD</i> gene is a common cause of congenital muscular dystrophy in Chinese patients Chinese patients
The familial hypercholesterolaemia phenotype: Monogenic familial hypercholesterolaemia, polygenic hypercholesterolaemia and other causes
Three novel patients with epileptic encephalopathy due to biallelic mutations in the <scp><i>PLCB1</i></scp> gene
Non‐syndromic X linked intellectual disability: Current knowledge in light of the recent advances in molecular and functional studies
Phenotypic spectrum of <i>TGFB3</i> disease‐causing variants in a Dutch‐French cohort and first report of a homozygous patient
Confirming the recessive inheritance of <i>PERP</i>‐related erythrokeratoderma
Diagnostic strategy for females suspected of Fabry disease
New evidence that biallelic loss of function in <i>EEF1B2</i> gene leads to intellectual disability
Expanding the clinical and molecular spectrum of lethal congenital contracture syndrome 8 associated with biallelic variants <i>of ADCY6</i>
Adult onset tubulo‐interstitial nephropathy in MT‐ND5‐related phenotypes
Multicenter study of the clinical features and mutation gene spectrum of Chinese children with Dent disease
Toni Mochty: Bardet Biedl syndrome “avant la lettre”