Clinical Genetics - 2015

142 articles | Last updated: 2025-12-03 14:12:56
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<i>Clinical Genetics</i> in the age of Genomics and Genome editing
Liver anomalies as a phenotype parameter of Bardet–Biedl syndrome
<scp>JP–HHT</scp> phenotype in Danish patients with <i><scp>SMAD4</scp></i> mutations
A novel<i><scp>CHST3</scp></i>allele associated with spondyloepiphyseal dysplasia and hearing loss in Pakistani kindred
Is there really an increased risk of early colorectal cancer in women with BRCA1 pathogenic mutations?
Genetic causes of MCPH in consanguineous Pakistani families
Risk assessment of medically assisted reproduction and advanced maternal ages in the development of Prader–Willi syndrome due to UPD(15)mat Japanese general population
Mutational and phenotypical spectrum of phenylalanine hydroxylase deficiency in Denmark
Clinical reappraisal of <scp>SHORT</scp> syndrome with <i><scp>PIK3R1</scp></i> mutations: toward recommendation for molecular testing and management
Response to Evans et al.
Genomic copy number alterations in non‐syndromic hearing loss
Mutations in <i><scp>SEC24D</scp></i> cause autosomal recessive osteogenesis imperfecta
Prevalence of type 5 familial hemophagocytic lymphohistiocytosis in Korea and novel mutations in <i><scp>STXBP2</scp></i>
Consideration surrounding incidental findings throughout multigene panel testing in cancer genetics
<i>DLL4</i> loss‐of‐function heterozygous mutations cause Adams–Oliver syndrome
<i><scp>PDGFRB</scp></i> mutation causes autosomal‐dominant Penttinen syndrome
Fetal phenotypes in otopalatodigital spectrum disorders
<i>IRF6</i> mutation screening in non‐syndromic orofacial clefting: analysis of 1521 families
A Mayan founder mutation is a common cause of deafness in Guatemala Native American, European, and African populations
Comprehensive genetic testing with ethnic‐specific filtering by allele frequency in a Japanese hearing‐loss population ethnic-specific, Japanese, non-Japanese ethnicities, in-house database of 200 normal-hearing Japanes
Mutation spectrum of the <i>MTM1</i> gene in XLMTM patients: 10 years of experience in prenatal and postnatal diagnosis
Gastrointestinal involvement in Fabry disease. So important, yet often neglected
Ataxia with oculomotor apraxia type 2 in the Canadian aboriginal population Canadian aboriginal population
<i>COX6A1</i> mutation causes axonal hereditary motor and sensory neuropathy – the confirmation of the primary report
Antibody deficiency in Rubinstein‐Taybi syndrome
Functional characterization of two new STAT3 mutations associated with hyper‐IgE syndrome in a Mexican cohort Mexican
Non‐truncating <scp>LIFR</scp> mutation: causal for prominent congenital pain insensitivity phenotype with progressive vertebral destruction?
A large multiexonic genomic deletion within the <i><scp>ALMS1</scp></i> gene causes Alström syndrome in a consanguineous Pakistani family
Utility of whole‐exome sequencing for those near the end of the diagnostic odyssey: time to address gaps in care
Sclerosteosis caused by a novel nonsense mutation of <scp>SOST</scp> in a consanguineous family
Genetic evaluation of patients with Alström syndrome in the Polish population Polish origin; Polish individuals
Screening adherence and cancer risk perceptions in colorectal cancer survivors with Lynch‐like syndrome
Genomic study of congenital insensitivity to pain provides new avenues for the development of analgesics
Whole‐genome sequencing provides insight into the genetics of major depressive disorder
<i><scp>NAPB</scp></i> – a novel <scp>SNARE</scp>‐associated protein for early‐onset epileptic encephalopathy Irish Traveller descent
Genetic testing for <i><scp>BRCA1</scp></i> and <i><scp>BRCA2</scp></i> in the Province of Ontario
Neurological and endocrine phenotypes of fragile X carrier women
Alpha‐mannosidosis: characterization of CNS pathology and correlation between CNS pathology and cognitive function
Confirmation of <i>TTC8</i> as a disease gene for nonsyndromic autosomal recessive retinitis pigmentosa (RP51) ethnically matched controls from same population
Sixteenth‐century German woodcut of a male infant with possible disorganization
Genomic futures of prenatal screening: ethical reflection
Genetic aspects of Huntington's disease in Latin America. A systematic review Europeans; European descent
Fragile‐X syndrome – a 20‐year follow‐up study of male patients
High‐resolution microarray analysis unravels complex Xq28 aberrations in patients and carriers affected by X‐linked blue cone monochromacy
Biallelic <i><scp>HERC1</scp></i> mutations in a syndromic form of overgrowth and intellectual disability
(Epi)genotype–phenotype correlations in Beckwith–Wiedemann syndrome: a paradigm for genomic medicine
Whole‐exome sequencing improves the diagnosis yield in sporadic infantile spasm syndrome
<i><scp>SLC1A4</scp></i> mutations cause a novel disorder of intellectual disability, progressive microcephaly, spasticity and thin corpus callosum Ashkenazi; Ashkenazi-Iraqi; Jewish Ashkenazi
A novel microdeletion affecting the <i>CETP</i> gene raises HDL‐associated cholesterol levels
Growth in neurofibromatosis 1 microdeletion patients
Hearing loss in Waardenburg syndrome: a systematic review
Comprehensive review of <i><scp>BAP1</scp></i> tumor predisposition syndrome with report of two new cases
Glanzmann thrombasthenia in Pakistan: molecular analysis and identification of novel mutations
Are physicians prepared for whole genome sequencing? a qualitative analysis
Uptake of health monitoring and disease self‐management in Australian adults with neurofibromatosis type 1: strategies to improve care
Psychiatric and cognitive symptoms in Huntington's disease are modified by polymorphisms in catecholamine regulating enzyme genes Danish
Congenital generalized lipodystrophy: identification of novel variants and expansion of clinical spectrum
Neurofibromatosis type 1 and malignancy in childhood
Something old, something new: <i>TBK1</i>, a novel gene in known amyotrophic lateral sclerosis pathways
Obtaining a genetic diagnosis in a child with disability: impact on parental quality of life
Clinical applications of next‐generation sequencing‐based gene panel in patients with muscular dystrophy: Korean experience
Measuring genetic knowledge: a brief survey instrument for adolescents and adults
Comorbidity in the Tunisian population
<i><scp>ALMS1</scp></i> null mutations: a common cause of Leber congenital amaurosis and early‐onset severe cone–rod dystrophy Chinese
Cranio‐facial clefts in pre‐hispanic America
Frequent incidence of <i><scp>BARD1</scp></i>‐truncating mutations in germline <scp>DNA</scp> from triple‐negative breast cancer patients
Maintaining the balance: both gain‐ and loss‐of‐function <i><scp>KCNA2</scp></i> mutants cause epileptic encephalopathy
Exploration of the cognitive, adaptive and behavioral functioning of patients affected with Bardet–Biedl syndrome
X‐chromosome inactivation in female patients with Fabry disease
Regulator of dendritic cell migration, <scp>ASAP1</scp> is associated with increased susceptibility to tuberculosis
Warfarin pharmacogenetics in the era of new oral anticoagulants
Mutations in <i><scp>SLC6A17</scp></i> cause autosomal‐recessive intellectual disability
Mutation analysis in Norwegian families with hereditary hemorrhagic telangiectasia: founder mutations in <i><scp>ACVRL1</scp></i> Norwegian
Novel homozygous <i>ALX4</i> mutation causing frontonasal dysplasia‐2 in a patient with meningoencephalocele
Toward an in‐depth profiling of <scp>DTC</scp> users
Mutations in <i><scp>RIT1</scp></i> cause Noonan syndrome – additional functional evidence and expanding the clinical phenotype
Molecular basis of inherited thrombocytopenias
<scp>CRISPR</scp> screen: a high‐throughput approach for cancer genetic research
Evaluating a counselling strategy for diagnostic WES in paediatric neurology: an exploration of parents' information and communication needs
A homozygous mutation in <i><scp>SLC1A4</scp></i> in siblings with severe intellectual disability and microcephaly Ashkenazi Jewish ancestry
Diagnosis and treatment of inherited thrombocytopenias
The risk of contralateral breast cancer in daughters of women with and without breast cancer
Prevalence of founder mutations in the <scp>BRCA1</scp> and <scp>BRCA2</scp> genes among unaffected women from the Bahamas
Clinical presentations of 23 half‐siblings from a mosaic neurofibromatosis type 1 sperm donor
Preferences for return of incidental findings from genome sequencing among women diagnosed with breast cancer at a young age
Clinical implementation of <scp>NIPT</scp> – technical and biological challenges
A review of craniofacial disorders caused by spliceosomal defects
Spinal neurofibromatosis and phenotypic heterogeneity in NF1
Fabry disease in a geriatric population
Diagnostic algorithms in Charcot–Marie–Tooth neuropathies: experiences from a German genetic laboratory on the basis of 1206 index patients
Evaluating stakeholder's perspective on referred out genetic testing in Canada: a discrete choice experiment
Diagnostic pitfall in antenatal manifestations of<scp>CPT II</scp>deficiency
Additional evidence that <i><scp>PGAP1</scp></i> loss of function causes autosomal recessive global developmental delay and encephalopathy
Low penetrance of paraganglioma and pheochromocytoma in an extended kindred with a germline <i><scp>SDHB</scp></i> exon 3 deletion Dutch; Netherlands
Fabry disease and enzyme replacement therapy in classic patients with same mutation: different formulations – different outcome?
Delineation of clinical features in Wiedemann–Steiner syndrome caused by <i><scp>KMT2A</scp></i> mutations
Response to Dylan Mordaunt and Alisha McLauchlan
New insights into Brunner syndrome and potential for targeted therapy
Extreme variability in clinical penetrance for a splice‐site <i>Plakophilin‐2</i> mutation in a Bangladeshi family
HDAC8‐deficiency causes an X‐linked dominant disorder with a wide range of severity
Origin of <i><scp>MLH1</scp></i>, <i><scp>MSH2</scp></i>, <i><scp>MSH6</scp></i> and <i>PMS2</i> mutations can help inform long‐term care strategies for patients with colorectal and endometrial cancer
Establishing cancer risks associated with <i><scp>PMS2</scp></i> germline mutations in Lynch syndrome
The implications of <i>de novo</i> coding mutations in simplex autism families
Lynch syndrome: five unanswered questions
The <scp>SickKids</scp> Genome Clinic: developing and evaluating a pediatric model for individualized genomic medicine
Gene therapy for primary immunodeficiencies
Disclosing genetic risk for coronary heart disease: effects on perceived personal control and genetic counseling satisfaction
Utility of next‐generation sequencing technologies for the efficient genetic resolution of haematological disorders
Characterization of patients referred for non‐specific intellectual disability testing: the importance of autosomal genes for diagnosis
Dysmorphology services: a snapshot of current practices and a vision for the future
Clinical and genetic data on Lafora disease patients of Serbian/Montenegrin origin
Epidemiology of Huntington disease: first post‐<scp><i>HTT</i></scp> gene analysis of prevalence in Italy
Phenotype analysis impacts testing strategy in patients with Currarino syndrome
Xq25 duplication: the crucial role of the <i><scp>STAG2</scp></i> gene in this novel human cohesinopathy
Practical considerations in the clinical application of whole‐exome sequencing
Ethical considerations of population screening for late‐onset genetic disease
Mutations in <scp>FLVCR2</scp> associated with Fowler syndrome and survival beyond infancy
Malignancy in Noonan syndrome and related disorders
Improving the uptake of predictive testing and colorectal screening in Lynch syndrome: a regional primary care survey
Broadening of cohesinopathies: exome sequencing identifies mutations in <i><scp>ANKRD11</scp></i> in two patients with Cornelia de Lange‐overlapping phenotype
Genetic counselors' practices and confidence regarding variant of uncertain significance results and reclassification from <i><scp>BRCA</scp></i> testing patient ancestry
X‐chromosomal inactivation directly influences the phenotypic manifestation of X‐linked protoporphyria
Adult‐onset vanishing white matter disease with novel missense mutations in a subunit of translational regulator, <i><scp>EIF2B4</scp></i>
Clinical Exome/Genome Reports‐Announcement
Exceptions to the rule: Case studies in the prediction of pathogenicity for genetic variants in hereditary cancer genes
LIMS2 mutations are associated with a novel muscular dystrophy, severe cardiomyopathy and triangular tongues
Predictive genetic testing in minors for Myocilin juvenile onset open angle glaucoma
Adult onset distal and proximal myopathy with complete ophthalmoplegia associated with a novel <i>de novo</i> p.(<scp>Leu1877Pro</scp>) mutation in <i><scp>MYH2</scp></i>
Depression among adults with neurofibromatosis type 1: prevalence and impact on quality of life
Compound heterozygous mutations in <scp>TTC7A</scp> cause familial multiple intestinal atresias and severe combined immunodeficiency
Genetic counselors' (<scp>GC</scp>) knowledge, awareness, understanding of clinical next‐generation sequencing (<scp>NGS</scp>) genomic testing
<scp>mtDNA</scp> mutations variously impact <scp>mtDNA</scp> maintenance throughout the human embryofetal development
A founder <i>EIF2AK4</i> mutation causes an aggressive form of pulmonary arterial hypertension in Iberian Gypsies Iberian Gypsies
Whole exome sequencing is an efficient, sensitive and specific method for determining the genetic cause of short‐rib thoracic dystrophies
Ataxia and myoclonic epilepsy due to a heterozygous new mutation in <i><scp>KCNA2</scp></i>: proposal for a new channelopathy
Genetic testing for <i>RAD51C</i> mutations: in the clinic and community
Exome sequencing in 32 patients with anophthalmia/microphthalmia and developmental eye defects
The clinical utility of whole‐exome sequencing in the context of rare diseases – the changing tides of medical practice
Resolving clinical diagnoses for syndromic cleft lip and/or palate phenotypes using whole‐exome sequencing
Pancreatic cancer genomics: where can the science take us?
Intrafamilial variability of <i><scp>ZRS</scp></i>‐associated syndrome: characterization of a mosaic <i><scp>ZRS</scp></i> mutation by pyrosequencing