Clinical Genetics - 2016

215 articles | Last updated: 2025-12-03 14:12:56
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Issue Information ‐ Editorial Board
Microcephaly, intractable seizures and developmental delay caused by biallelic variants in <i><scp>TBCD</scp></i>: further delineation of a new chaperone‐mediated tubulinopathy
Maternal genetic polymorphisms and unexplained recurrent miscarriage: a systematic review and meta‐analysis
A new mutation in <i>TUBB1</i> associated with thrombocytopenia confirms that C‐terminal part of β1‐tubulin plays a role in microtubule assembly
Clinical application of <scp>SNP</scp> array analysis in first‐trimester pregnancy loss: a prospective study
A novel <i>RAD21</i> variant associated with intrafamilial phenotypic variation in Cornelia de Lange syndrome – review of the literature
Emerging roles of <i>RAC1</i> in treating lung cancer patients
Molecular characterization of <i><scp>PI</scp>*Q0<sub>la palma</sub></i>, a new alpha‐1‐antitrypsin null allele that combines two defective genetic variants
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Genetics of primary ovarian insufficiency
Extension of the clinical and molecular phenotype of <i><scp>DIAPH1</scp></i>‐associated autosomal dominant hearing loss (<i><scp>DFNA1</scp></i>)
Single gene defects leading to sperm quantitative anomalies
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Mutation in <scp>JPH2</scp> cause dilated cardiomyopathy
Genetic abnormalities leading to qualitative defects of sperm morphology or function
Recent insights on the genetics and epigenetics of endometriosis
Biallelic truncating <i><scp>SCN9A</scp></i> mutation identified in four families with congenital insensitivity to pain from Pakistan
<i><scp>BRF1</scp></i> mutations in a family with growth failure, markedly delayed bone age, and central nervous system anomalies
Genetics of mitochondrial dysfunction and infertility
<i><scp>BRCA1</scp></i> and <i><scp>BRCA2</scp></i> mutation testing in Cyprus; a population based study Cypriot population; Mediterranean countries; other ethnic populations
Factors related to genetic testing in adults at risk for Huntington disease: the prospective Huntington at‐risk observational study (<scp>PHAROS</scp>)
A novel gain‐of‐function mutation in <i>ORAI1</i> causes late‐onset tubular aggregate myopathy and congenital miosis Italian family
A genetic risk score is significantly associated with statin therapy response in the elderly population
A lethal neonatal phenotype of mitochondrial short‐chain enoyl‐<scp>CoA</scp> hydratase‐1 deficiency
The biology of germ cell tumors in disorders of sex development
The human knockout phenotype of <i><scp>PADI6</scp></i> is female sterility caused by cleavage failure of their fertilized eggs
Heart‐hand syndrome <scp>IV</scp>: a second family with <i><scp>LMNA</scp></i>‐related cardiomyopathy and brachydactyly
Cardiovascular pharmacogenetics: a promise for genomically‐guided therapy and personalized medicine
Loss of the arginine methyltranserase PRMT7 causes syndromic intellectual disability with microcephaly and brachydactyly
Effects of <scp>S906T</scp> polymorphism on the severity of a novel borderline mutation <scp>I692M</scp> in Na<i><sub>v</sub></i>1.4 cause periodic paralysis central Europe; Germany
Genetics of Mayer–Rokitansky–Küster–Hauser (<scp>MRKH</scp>) syndrome
Severe infantile male encephalopathy is a result of early post‐zygotic <i><scp>WDR45</scp></i> somatic mutation
Hematopoietically expressed homeobox (<i>HHEX</i>) gene polymorphism (rs5015480) is associated with increased risk of gestational diabetes mellitus
A large cohort study confirming that specific haplotype <scp>4A161PAS</scp> is exclusively associated with the Chinese <scp>FSHD1</scp> Chinese
<scp>RE</scp>: frameshift variant <i><scp>FANCL</scp></i>*c.1096_1099dupATTA is not associated with high breast cancer risk
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Association of genetic polymorphisms of <i>de novo</i> nucleotide biosynthesis with increased <scp>CHD</scp> susceptibility in the northern Chinese population
Chromosomal microarray in a highly consanguineous population: diagnostic yield, utility of regions of homozygosity, and novel mutations
Choroidal abnormalities in café‐au‐lait syndromes: a new differential diagnostic tool?
An homozygous mutation in <i>KCNK3</i> is associated with an aggressive form of hereditary pulmonary arterial hypertension Spanish patients; Romani family
Epispadias and the associated embryopathies: genetic and developmental basis
Sociodemographic, psychosocial and clinical factors associated with uptake of genetic counselling for hereditary cancer: a systematic review
A <i>de novo</i> mutation in the <i>NALCN</i> gene in an adult patient with cerebellar ataxia associated with intellectual disability and arthrogryposis
Mutations of <i><scp>SGO2</scp></i> and <i><scp>CLDN14</scp></i> collectively cause coincidental Perrault syndrome
The impact of an expanded genetic testing program and selective oophorectomy on the incidence of ovarian cancer in West Pomerania
Genetics of syndromic and non‐syndromic hereditary nail disorders
Baraitser–Winter cerebrofrontofacial syndrome
Further evidence that <i>de novo</i> missense and truncating variants in <i><scp>ZBTB18</scp></i> cause intellectual disability with variable features
The genetic make‐up of ovarian development and function: the focus on the transcription factor <scp>FOXL2</scp>
Loss of the proprioception and touch sensation channel <scp>PIEZO2</scp> in siblings with a progressive form of contractures
Recognizing the tenascin‐X deficient type of Ehlers–Danlos syndrome: a cross‐sectional study in 17 patients
Genetic causes of moderate to severe hearing loss point to modifiers
Homozygous <i><scp>DNAH1</scp></i> frameshift mutation causes multiple morphological anomalies of the sperm flagella in Chinese Chinese; East Asian group
Mitochondrial epileptic encephalopathy, 3‐methylglutaconic aciduria and variable complex V deficiency associated with <i><scp>TIMM50</scp></i> mutations ethnic specific exome cohort
<i>De novo</i> loss of function mutations in <i><scp>KIAA2022</scp></i> are associated with epilepsy and neurodevelopmental delay in females
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Comprehensive review of the duplication 3q syndrome and report of a patient with Currarino syndrome and <i>de novo</i> duplication 3q26.32‐q27.2
The clinical spectrum of female epilepsy patients with <i><scp>PCDH19</scp></i> mutations in a Chinese population Chinese population
Integration of 60,000 exomes and <scp>ACMG</scp> guidelines question the role of Catecholaminergic Polymorphic Ventricular Tachycardia‐associated variants
Identification of a second <i><scp>HOXA2</scp></i> nonsense mutation in a family with autosomal dominant non‐syndromic microtia and distinctive ear morphology
Nasopharyngeal teratoma, congenital diaphragmatic hernia and Dandy–Walker malformation – a yet uncharacterized syndrome
Frameshift variant <i><scp>FANCL</scp></i>*c.1096_1099dupATTA is not associated with high breast cancer risk
Clinical application of whole‐genome low‐coverage next‐generation sequencing to detect and characterize balanced chromosomal translocations
Simple and rapid characterization of novel large germline deletions in <i><scp>SDHB</scp></i>, <i><scp>SDHC</scp></i> and <i><scp>SDHD</scp></i>‐related paraganglioma
Complex phenotypes blur conventional borders between Say–Barber–Biesecker–Young–Simpson syndrome and genitopatellar syndrome
<scp>DJ</scp>‐1 modulates mitochondrial response to oxidative stress: clues from a novel diagnosis of <scp>PARK7</scp>
Muscular dystrophies and myopathies: the spectrum of mutated genes in the Czech Republic Czech; Czech Republic
Phenotypic variability and diffuse arterial lesions in a family with Loeys–Dietz syndrome type 4
Molecular genetic analysis of 30 families with Joubert syndrome
Congenital heart diseases and their association with the variant distribution features on susceptibility genes
<scp>CLOVES</scp> syndrome: review of a <scp>PIK3CA</scp>‐related overgrowth spectrum (<scp>PROS</scp>)
Issue Information ‐ Editorial Board
Genetic risk factors for venous thrombosis in women using combined oral contraceptives: update of the <scp>PILGRIM</scp> study
<i><scp>KIF5A</scp> de novo</i> mutation associated with myoclonic seizures and neonatal onset progressive leukoencephalopathy
Comprehensive population screening in the Ashkenazi Jewish population for recurrent disease‐causing variants Ashkenazi Jewish (AJ) population
Choices of incidental findings of individuals undergoing genome wide sequencing, a single center's experience
A practical approach to ichthyoses with systemic manifestations
Haplotype analysis reveals that the recurrent <i><scp>BRCA1</scp></i> deletion of exons 23 and 24 is a Greek founder mutation Greek origin; Greek population; Greek descent; Asia Minor
Discovery of pathogenic variants in a large Korean cohort of inherited muscular disorders Korean; "Korean patients"; "Korean cohort"
Recent Advances in Imprinting Disorders
Impact of rare variants in <i><scp>ARHGAP29</scp></i> to the etiology of oral clefts: role of loss‐of‐function <i>vs</i> missense variants
Exome sequencing revealed a novel splice site variant in the <i><scp>ALX1</scp></i> gene underlying frontonasal dysplasia Pakistani; different ethnic groups
<scp>EMR</scp>documentation of physician–patient communication following genomic counseling for actionable complex disease and pharmacogenomic results
Identification of <i>C12orf4</i> as a gene for autosomal recessive intellectual disability Finnish; Dutch; North Eastern sub-isolate of Finland; Arab
<i><scp>DNM1L</scp></i>‐related encephalopathy in infancy with Leigh syndrome‐like phenotype and suppression‐burst
Utility of clinical high‐depth next generation sequencing for somatic variant detection in the <i><scp>PIK3CA</scp></i>‐related overgrowth spectrum
Evidence for the founder effect of a novel <i>ACVRL1</i> splice‐site mutation in Hungarian hereditary hemorrhagic telangiectasia families
Association of a type 2 diabetes genetic risk score with insulin secretion modulated by insulin sensitivity among Chinese Hans Chinese Han
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Expanding non‐invasive prenatal testing beyond chromosomes 21, 18, 13, X and Y
Cleidocranial dysplasia and RUNX2‐clinical phenotype–genotype correlation
Whole exome sequencing identifies multiple diagnoses in congenital glaucoma with systemic anomalies
The homozygous R504C mutation in <i>MTO1</i> gene is responsible for ONCE syndrome
Targeted genomic enrichment and massively parallel sequencing identifies novel nonsyndromic hearing impairment pathogenic variants in Cameroonian families
Expanding the clinical picture of the <i>MECP2</i> Duplication syndrome
Characterization of <i>SPATA5</i>‐related encephalopathy in early childhood
Epileptic encephalopathy with continuous spike-and-wave during sleep maps to a homozygous truncating mutation in AMPA receptor component FRRS1L
Improved diagnostic yield of neuromuscular disorders applying clinical exome sequencing in patients arising from a consanguineous population
Hypomyelinating leukodystrophies — a molecular insight into the white matter pathology
Next‐generation sequencing of Chinese stage IV lung cancer patients reveals an association between <i>EGFR</i> mutation status and survival outcome
Spinocerebellar ataxia: relationship between phenotype and genotype – a review
<scp>ALTE</scp> and smoking exposure: which role of detoxification genes polymorphisms?
Genetic, epidemiologic and clinicopathologic studies of Japanese Asian patients with Birt–Hogg–Dubé syndrome Japanese Asian; Japanese; Taiwanese
Prenatal diagnosis of congenital myopathies and muscular dystrophies
The use of whole exome sequencing for the diagnosis of autosomal recessive malignant infantile osteopetrosis
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<i><scp>NSD1</scp></i> duplication in Silver–Russell syndrome (<scp>SRS</scp>): molecular karyotyping in patients with <scp>SRS</scp> features
<scp>mRNA</scp>‐based detection of rare <scp>CFTR</scp> mutations improves genetic diagnosis of cystic fibrosis in populations with high genetic heterogeneity
The promoter polymorphisms of receptor for advanced glycation end products were associated with the susceptibility and progression of sepsis Chinese Han population
Advanced diagnostic genetic testing in inherited retinal disease: experience from a single tertiary referral centre in the <scp>UK</scp> National Health Service
Identification and characterization of 20 novel pathogenic variants in 60 unrelated Indian patients with mucopolysaccharidoses type I and type <scp>II</scp> Indian population
Whole gene duplication of <i>SCN2A</i> and <i>SCN3A</i> is associated with neonatal seizures and a normal intellectual development
Corrigendum
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Autosomal recessive truncating <i><scp>MAB21L1</scp></i> mutation associated with a syndromic scrotal agenesis
Social anxiety and autism spectrum traits among adult <i><scp>FMR1</scp></i> premutation carriers
Spondyloepimetaphyseal dysplasia with neurodegeneration associated with <i><scp>AIFM1</scp></i> mutation – a novel phenotype of the mitochondrial disease
Autosomal recessive mutations in <i><scp>THOC6</scp></i> cause intellectual disability: syndrome delineation requiring forward and reverse phenotyping
A novel mutation in <i><scp>NDUFB11</scp></i> unveils a new clinical phenotype associated with lactic acidosis and sideroblastic anemia
Resistance to hypertension and high Cl<sup>−</sup> excretion in humans with <i><scp>SLC26A4</scp></i> mutations
Down syndrome in adults: a 27‐year follow‐up of adaptive skills
National G6PD neonatal screening program in Gaza Strip of Palestine: rationale, challenges and recommendations
Rett‐like phenotypes: expanding the genetic heterogeneity to the <i><scp>KCNA2</scp></i> gene and first familial case of <i><scp>CDKL5</scp></i>‐related disease
Mutations in the <scp>IRBIT</scp> domain of <i><scp>ITPR1</scp></i> are a frequent cause of autosomal dominant nonprogressive congenital ataxia
Autosomal recessive <scp>IFT57</scp> hypomorphic mutation cause ciliary transport defect in unclassified oral–facial–digital syndrome with short stature and brachymesophalangia
A role for<i>TENM1</i>mutations in congenital general anosmia
Refining the phenotypical and mutational spectrum of Taybi‐Linder syndrome
Homozygous <i><scp>PKP2</scp></i> deletion associated with neonatal left ventricle noncompaction
Phenotype‐driven molecular autopsy for sudden cardiac death
<i><scp>RELN</scp></i> and <i><scp>VLDLR</scp></i> mutations underlie two distinguishable clinico‐radiological phenotypes
Is there a link between Holt-Oram Syndrome and ‘Mardini-Nyhan’ association? - need for further research
Specific mosaic <i><scp>KRAS</scp></i> mutations affecting codon 146 cause oculoectodermal syndrome and encephalocraniocutaneous lipomatosis
Expanding the genotypic spectrum of Perrault syndrome
Novel pathogenic <i><scp>SLC25A46</scp></i> splice‐site mutation causes an optic atrophy spectrum disorder Moroccan
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A<i>de novo</i>frameshift in<i>HNRNPK</i>causing a Kabuki-like syndrome with nodular heterotopia
Prenatal diagnosis of trisomy 21, 18 and 13 by quantitative pyrosequencing of segmental duplications
Expanding the phenotype of <i><scp>RTTN</scp></i> variations: a new family with primary microcephaly, severe growth failure, brain malformations and dermatitis
The <i><scp>MECP2</scp></i> variant c.<scp>925C</scp>&gt;T (p.<scp>Arg309Trp</scp>) causes intellectual disability in both males and females without classic features of Rett syndrome
Analysis copy number variation of Chinese children in early‐onset epileptic encephalopathies with unknown cause Chinese
Expanding the phenotype of <scp>CRB2</scp> mutations – A new ciliopathy syndrome? Ashkenazi Jewish
The role of <scp>COMT</scp> and plasma proline in the variable penetrance of autistic spectrum symptoms in 22q11.2 deletion syndrome
Different X-linked<i>KDM5C</i>mutations in affected male siblings: is maternal reversion error involved?
Diverse prognostic value of the <scp>GTn</scp> promoter polymorphism in squamous cell and adeno carcinoma of the oesophagus
<scp>L1</scp> syndrome diagnosis complemented with functional analysis of <scp>L1CAM</scp> variants located to the two N‐terminal Ig‐like domains
A common variant association study reveals novel susceptibility loci for low <scp>HDL</scp>‐cholesterol levels in ethnic Arabs ethnic Arabs; Asian population
A homozygous nonsense variant in <i><scp>IFT52</scp></i> is associated with a human skeletal ciliopathy
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Decision‐making for non‐invasive prenatal testing for Down syndrome: Hong Kong Chinese women's preferences for individual <i>vs</i> relational autonomy
Identification of a founder <i><scp>BRCA1</scp></i> mutation in the Moroccan population Moroccan population
Salt depletion syndrome as the initial presentation of cystic fibrosis in a toddler with the rare p.Ala309Gly (A309G) CFTR variant
Fetal growth patterns in Beckwith–Wiedemann syndrome
Unclassifiable arrhythmic cardiomyopathy associated with Emery–Dreifuss caused by a mutation in <scp>FHL1</scp>
Structure–function studies of <i><scp>HNF1A</scp></i> (<scp>MODY3</scp>) gene mutations in South Indian patients with monogenic diabetes
Variant haploinsufficiency and phenotypic non‐penetrance in <i><scp>PRPF31</scp></i>‐associated retinitis pigmentosa
When silence is noise: infantile‐onset Barth syndrome caused by a synonymous substitution affecting <i><scp>TAZ</scp></i> gene transcription
<i><scp>NOBOX</scp></i> is a strong autosomal candidate gene in Tunisian patients with primary ovarian insufficiency Tunisian; ethnically matched women
Simpson–Golabi–Behmel syndrome: a prenatal diagnosis in a foetus with <i><scp>GPC3</scp></i> and <i><scp>GPC4</scp></i> gene microduplications
Molecular genetic analysis in 14 Czech Kabuki syndrome patients is confirming the utility of phenotypic scoring
Developmental dysplasia of the hip: usefulness of next generation genomic tools for characterizing the underlying genes – a mini review
<scp>FGFR2</scp> splice site mutations in Crouzon and Pfeiffer syndromes: two novel variants
A novel <i><scp>HYLS1</scp></i> homozygous mutation in living siblings with Joubert syndrome
Clinical, biochemical and molecular characterization of prosaposin deficiency
Molecular and phenotypic characteristics of seven novel mutations causing branched-chain organic acidurias
Mutational characteristics of <i><scp>ANK1</scp></i> and <i><scp>SPTB</scp></i> genes in hereditary spherocytosis
Next‐generation sequencing confirms the implication of <i><scp>SLC24A1</scp></i> in autosomal‐recessive congenital stationary night blindness
<scp>HSMNR</scp> belongs to the most frequent types of hereditary neuropathy in the Czech Republic and is twice more frequent than <scp>HMSNL</scp> Roma origin
Hereditary truncating mutations of <scp>DNA</scp> repair and other genes in <i><scp>BRCA1</scp></i>/<i><scp>BRCA2</scp></i>/<i><scp>PALB2</scp></i>‐negatively tested breast cancer patients
Cerebellar ataxia and severe muscle <scp>CoQ<sub>10</sub></scp> deficiency in a patient with a novel mutation in <i><scp>ADCK3</scp></i>
High frequency of<i>OTOF</i>mutations in Chinese infants with congenital auditory neuropathy spectrum disorder
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<scp>WES</scp> in a family trio suggests involvement of <scp>TECPR2</scp> in a complex form of progressive motor neuron disease
Hermansky–Pudlak syndrome genes are frequently mutated in patients with albinism from the Arabian Peninsula
Disrupted nitric oxide signaling due to <i><scp>GUCY1A3</scp></i> mutations increases risk for moyamoya disease, achalasia and hypertension
Recurrent copy number variations as risk factors for autism spectrum disorders: analysis of the clinical implications Greek origin
Relevance of truncating titin mutations in dilated cardiomyopathy
A non‐sense <i><scp>MCM9</scp></i> mutation in a familial case of primary ovarian insufficiency
Genetics of human Bardet–Biedl syndrome, an updates
Wide allelic heterogeneity with predominance of large <i><scp>IDS</scp></i> gene complex rearrangements in a sample of Mexican patients with Hunter syndrome Mexican
Mutational spectrum of Korean patients with corneal dystrophy Korean patients
Complex mode of inheritance in holoprosencephaly revealed by whole exome sequencing
Association of <i><scp>COL4A1</scp></i> gene polymorphisms with cerebral palsy in a Chinese Han population
The c.65‐2A&gt;G splice site mutation is associated with a mild phenotype in Danon disease due to the transcription of normal LAMP2 mRNA
First‐trimester spontaneous pregnancy loss – molecular analysis using multiplex ligation‐dependent probe amplification
<scp>DMRTA2</scp> (<scp><i>DMRT5</i></scp>) is mutated in a novel cortical brain malformation
Identification of germline alterations in breast cancer predisposition genes among Malaysian breast cancer patients using panel testing
Diagnostic odyssey in severe neurodevelopmental disorders: toward clinical whole‐exome sequencing as a first‐line diagnostic test
Lentiginous phenotypes caused by diverse pathogenic genes (<i><scp>SASH1</scp></i> and <i><scp>PTPN11</scp></i>): clinical and molecular discrimination
<i>De novo</i> loss‐of‐function mutations in X‐linked <i><scp>SMC1A</scp></i> cause severe <scp>ID</scp> and therapy‐resistant epilepsy in females: expanding the phenotypic spectrum
Aneuploidy: the impact of chromosome imbalance on nuclear organization and overall genome expression
Acromelic frontonasal dysostosis and<i>ZSWIM6</i>mutation: phenotypic spectrum and mosaicism
A series of 38 novel germline and somatic mutations of <i><scp>NIPBL</scp></i> in Cornelia de Lange syndrome
Spinocerebellar ataxia type 3/Machado–Joseph disease: segregation patterns and factors influencing instability of expanded <scp>CAG</scp> transmissions
Refining the continuum of<scp>CFTR</scp>‐associated disorders in the era of newborn screening
Expanding the clinical spectrum of the ‘<i><scp>HDAC8</scp></i>‐phenotype’ – implications for molecular diagnostics, counseling and risk prediction
Association analysis between <scp><i>HFM1</i></scp> variation and primary ovarian insufficiency in Chinese women Chinese women; Chinese population; ethnic background
Recognition of the Cornelia de Lange syndrome phenotype with facial dysmorphology novel analysis
Application of whole‐exome sequencing for detecting copy number variants in CMT1A/HNPP
Homozygous FIBP nonsense variant responsible of syndromic overgrowth, with overgrowth, macrocephaly, retinal coloboma and learning disabilities
Expanding the <i>MYBPC1</i> phenotypic spectrum: a novel homozygous mutation causes arthrogryposis multiplex congenita Israeli‐Druze
Mutation analysis of genes within the dynactin complex in a cohort of hereditary peripheral neuropathies
Origin of mutation in sporadic cases of severe haemophilia A in Sweden
Health risks for ataxia‐telangiectasia mutated heterozygotes: a systematic review, meta‐analysis and evidence‐based guideline
The genetics and pathogenesis of thoracic aortic aneurysm disorder and dissections
Genetic and clinical analysis of spinocerebellar ataxia type 36 in Mainland China
Assessment of <scp>PAX6</scp> alleles in 66 families with aniridia
Early growth and development impairments in patients with ganglioside <scp>GM3</scp> synthase deficiency
Pathogenic <i>FBN1</i> variants in familial thoracic aortic aneurysms and dissections
Systematic review of chronic pain in persons with Marfan syndrome
Exome sequencing revealed a novel biallelic deletion in the<i>DCAF17</i>gene underlying Woodhouse Sakati syndrome
Mutations in <i><scp>WNT9B</scp></i> are associated with Mayer–Rokitansky–Küster–Hauser syndrome
<i>SATB2</i>‐associated syndrome presenting with Rett‐like phenotypes
Value of <scp>MRI</scp> olfactory bulb evaluation in the assessment of olfactory dysfunction in Bardet–Biedl syndrome
Genetic counselling difficulties and ethical implications of incidental findings from array‐CGH: a 7‐year national survey
Muscle hypertrophy as the presenting sign in a patient with a complete <i><scp>FHL1</scp></i> deletion