Search results for "Disposition"

showing 10 items of 832 documents

Individual analysis of patients with HoFH participating in a phase 3 trial with lomitapide: The Italian cohort

2015

Abstract Background and aims The efficacy and safety of lomitapide as adjunct treatment for adults with homozygous familial hypercholesterolaemia (HoFH) have been confirmed in a phase 3 trial. Given the small number of patients (N = 29), and variations in patient characteristics, examining individual cases provides additional details regarding patient management with lomitapide. Here, we examine the details of the Italian patient cohort in the phase 3 trial. Methods and results The methodology of the multinational, single-arm, open-label, 78-week, dose-escalation, phase 3 trial has been previously reported. The current report details the Italian cohort of six patients (three males, three fe…

MalePediatricsTime FactorsSettore MED/09 - Medicina InternaEndocrinology Diabetes and MetabolismHoFHMedicine (miscellaneous)030204 cardiovascular system & hematologychemistry.chemical_compound0302 clinical medicineEndocrinologyReceptorsNutrition and DieteticMedicine030212 general & internal medicineFamilial hypercholesterolaemia; FH; HoFH; Homozygous familial hypercholesterolaemia; Lomitapide; Therapy; Medicine (miscellaneous); Nutrition and Dietetics; Endocrinology Diabetes and Metabolism; Cardiology and Cardiovascular MedicineFH; Familial hypercholesterolaemia; HoFH; Homozygous familial hypercholesterolaemia; Lomitapide; Therapy; Adolescent; Adult; Anticholesteremic Agents; Benzimidazoles; Biomarkers; Cholesterol LDL; Female; Genetic Predisposition to Disease; Humans; Hyperlipoproteinemia Type II; Italy; Male; Middle Aged; Phenotype; Receptors LDL; Time Factors; Treatment Outcome; Young Adult; Heterozygote; MutationNutrition and DieteticsAnticholesteremic AgentsMiddle AgedPatient managementDiabetes and MetabolismCholesterolPhenotypeTreatment OutcomeTolerabilityItalyCohortPopulation studyFemaleFamilial hypercholesterolaemiaCardiology and Cardiovascular MedicineAdultmedicine.medical_specialtyHeterozygoteAdolescentSocio-culturaleFHLDLHyperlipoproteinemia Type II03 medical and health sciencesIndividual analysisYoung AdultHomozygous familial hypercholesterolaemiaHumansGenetic Predisposition to DiseaseAdverse effectbusiness.industryCholesterol LDLLomitapideLomitapideClinical trialchemistryReceptors LDLMutationBenzimidazolesTherapybusinessBiomarkers
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Cross-sectional comparison of the characteristics of respiratory allergy in immigrants and Italian children.

2014

Background: Immigrants represent a good epidemiological model to evaluate the relative influence of environmental and inherited factors on the development of allergy. Several studies on allergy in adults have been published, but few data in children are available. We aimed to investigate the differences, between Italian and immigrant children, in clinical characteristics of respiratory allergy. Methods: This was a multicentre cross-sectional study involving children born in Italy from Italian parents and children born either in Italy or abroad from immigrants. Children referred firstly for allergic respiratory disease (rhinitis/asthma), with an ascertained clinical diagnosis and IgE sensiti…

MalePediatricsmedicine.medical_specialtyAllergyAdolescentmedia_common.quotation_subjectImmunologyImmigrationAllergic asthmaEmigrants and ImmigrantsDiseaseEpidemiologyAllergic rhinitimedicineEthnicityRespiratory HypersensitivityImmunology and AllergyHumansGenetic Predisposition to DiseaseChildChildrenmedia_commonAsthmabusiness.industryRespiratory diseaseRespiratory allergyInfantmedicine.diseaseCross-Sectional StudiesItalyClinical diagnosisChild PreschoolPediatrics Perinatology and Child HealthFemalebusinessImmigrantPediatric allergy and immunology : official publication of the European Society of Pediatric Allergy and Immunology
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Gender-Dependent Effect of GSTM1 Genotype on Childhood Asthma Associated with Prenatal Tobacco Smoke Exposure

2014

It remains unclear whether the GSTM1 genotype interacts with tobacco smoke exposure (TSE) in asthma development. This study aimed to investigate the interactions among GSTM1 genotype, gender, and prenatal TSE with regard to childhood asthma development. In a longitudinal birth cohort in Taiwan, 756 newborns completed a 6-year follow-up, and 591 children with DNA samples available for GSTM1 genotyping were included in the study,and the interactive influences of gender-GSTM1 genotyping-prenatal TSE on childhood asthma development were analyzed. Among these 591 children, 138 (23.4%) hadphysician-diagnosed asthmaat 6 years of age, and 347 (58.7%) werenull-GSTM1. Prenatal TSE significantly incre…

MalePediatricsmedicine.medical_specialtyArticle Subjectanimal diseasesTaiwanlcsh:MedicineComorbidityLower riskPolymorphism Single NucleotideGeneral Biochemistry Genetics and Molecular BiologyPregnancyRisk FactorsGenotypePrevalencemedicineHumansGenetic Predisposition to DiseaseSex DistributionChildneoplasmsGenotypingGlutathione TransferaseAsthmaChildhood asthmaintegumentary systemGeneral Immunology and Microbiologybusiness.industrylcsh:RTobacco smoke exposureInfant NewbornInfantTotal igeGeneral Medicinemedicine.diseaseAsthmanervous system diseasesCausalityChild PreschoolPrenatal Exposure Delayed EffectsFemaleTobacco Smoke PollutionbusinessBirth cohortResearch ArticleBioMed Research International
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A Polymorphism in a Gene Encoding Perilipin 4 Is Associated with Height but not with Bone Measures in Individuals from the Framingham Osteoporosis St…

2011

There is increasing interest in identifying new pathways and candidate genes that confer susceptibility to osteoporosis. There is evidence that adipogenesis and osteogenesis may be related, including a common bone marrow progenitor cell for both adipocytes and osteoblasts. Perilipin 1 (PLIN1) and Perilipin 4 (PLIN4) are members of the PATS family of genes and are involved in lipolysis of intracellular lipid deposits. A previous study reported gender-specific associations between one polymorphism of PLIN1 and bone mineral density (BMD) in a Japanese population. We hypothesized that polymorphisms in PLIN1 and PLIN4 would be associated with bone measures in adult Caucasian participants of the …

MalePerilipin-1medicine.medical_specialtyCandidate geneGenotypeBone densityEndocrinology Diabetes and MetabolismOsteoporosisSingle-nucleotide polymorphismBiologyPerilipin-4Polymorphism Single NucleotideArticleAbsorptiometry PhotonEndocrinologyBone DensityInternal medicinemedicineBone mineral densityHumansSNPGenetic Predisposition to DiseaseOrthopedics and Sports MedicineHuman heightBone mineralBone geometryFramingham Osteoporosis StudyMiddle AgedPhosphoproteinsmedicine.diseaseBody HeightPerilipin 4EndocrinologyPerilipin 1PerilipinOsteoporosisFemaleCarrier Proteins
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Gender-Specific Association of a Perilipin Gene Haplotype with Obesity Risk in a White Population

2004

Objective: Perilipin is a class of protein-coating lipid droplets in adipocytes and steroidogenic cells. Our purpose was to examine the association between common single-nucleotide polymorphisms (SNPs) at the perilipin (PLIN) locus and obesity, as well as related phenotypes, in unrelated American adults. Research Methods and Procedures: Four PLIN SNPs (PLIN 6209T>C, 11482G>A, 13041A>G, and 14995A>T) were typed in 734 white subjects (373 men and 361 women) attending a residential lifestyle intervention program. The baseline anthropometric and biochemical measures were used. Obesity was defined as BMI ≥ 30 kg/m2. Results: Multivariate analysis demonstrated that, in women, two of the SNPs (130…

MalePerilipin-1medicine.medical_specialtyWaistGenotypeEndocrinology Diabetes and MetabolismMedicine (miscellaneous)Single-nucleotide polymorphismPolymorphism Single NucleotideWhite PeopleBody Mass IndexEndocrinologyWaist–hip ratioInternal medicinemedicineHumansGenetic Predisposition to DiseaseObesityAnalysis of VarianceSex CharacteristicsWaist-Hip Ratiobusiness.industryHaplotypePublic Health Environmental and Occupational HealthDNAOdds ratioMiddle AgedPhosphoproteinsmedicine.diseaseObesityEndocrinologyAdipose TissueHaplotypesBody CompositionLinear ModelsPerilipinFemaleCarrier ProteinsbusinessBody mass indexFood ScienceObesity Research
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Transient Receptor Potential Channel Polymorphisms Are Associated with the Somatosensory Function in Neuropathic Pain Patients

2011

Transient receptor potential channels are important mediators of thermal and mechanical stimuli and play an important role in neuropathic pain. The contribution of hereditary variants in the genes of transient receptor potential channels to neuropathic pain is unknown. We investigated the frequency of transient receptor potential ankyrin 1, transient receptor potential melastin 8 and transient receptor potential vanilloid 1 single nucleotide polymorphisms and their impact on somatosensory abnormalities in neuropathic pain patients. Within the German Research Network on Neuropathic Pain (Deutscher Forscbungsverbund Neuropathischer Schmerz) 371 neuropathic pain patients were phenotypically ch…

MalePharmacologyTransient receptor potential channelTransient Receptor Potential ChannelsAnesthesiologyMolecular Cell BiologyMembrane Receptor SignalingMultidisciplinaryQRMiddle AgedNeurologyHyperalgesiaNeuropathic painMedicineFemaleSensory Perceptionmedicine.symptomResearch ArticleSignal TransductionAdultAnkyrinsGenotypeScienceMedizinische Fakultät -ohne weitere Spezifikation-Receptor potential610TRPV Cation ChannelsSensory systemSingle-nucleotide polymorphism-Polymorphism Single NucleotidemedicineGeneticsHumansPain ManagementGenetic Predisposition to Diseaseddc:610BiologyGenetic Association StudiesAgedHypoalgesiaPolymorphism GeneticPopulation Biologybusiness.industryHuman Geneticsmedicine.diseaseNeuralgiaGenetic PolymorphismNeuralgiabusinessPopulation GeneticsNeuroscience
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Targeted next-generation sequencing of deafness genes in hearing-impaired individuals uncovers informative mutations

2014

Purpose: Targeted next-generation sequencing provides a remarkable opportunity to identify variants in known disease genes, particularly in extremely heterogeneous disorders such as nonsyndromic hearing loss. The present study attempts to shed light on the complexity of hearing impairment. Methods: Using one of two next-generation sequencing panels containing either 80 or 129 deafness genes, we screened 30 individuals with nonsyndromic hearing loss (from 23 unrelated families) and analyzed 9 normal-hearing controls. Results: Overall, we found an average of 3.7 variants (in 80 genes) with deleterious prediction outcome, including a number of novel variants, in individuals with nonsyndromic h…

MaleProbandUsher syndromeGene DosageDeafnessBioinformaticsmedicine.disease_causesensorineural hearing lossConnexinsCohort Studiestargeted next-generation sequencingOriginal Research Articlemutational loadChildGenetics (clinical)Oligonucleotide Array Sequence AnalysisGeneticsMutationmedicine.diagnostic_testHomozygoteHigh-Throughput Nucleotide SequencingPedigreeConnexin 26Treatment OutcomeChild PreschoolFemalemedicine.symptomAdultAdolescentSequence analysisHearing lossdeafness gene panelMolecular Sequence DataBiologynonsyndromic hearing lossDNA sequencingYoung AdultAudiometryGenetic variationotorhinolaryngologic diseasesmedicineHumansGenetic Predisposition to DiseaseFamily HealthBase SequenceGenetic VariationInfantDNASequence Analysis DNAmedicine.diseaseMutationAudiometryGene DeletionGenetics in Medicine
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Polymorphisms of an innate immune gene, toll-like receptor 4, and aggressive prostate cancer risk: a systematic review and meta-analysis.

2014

Background: Toll-like receptor 4 (TLR4) is one of the best known TLR members expressed on the surface of several leukocytes and tissue cells and has a key function in detecting pathogen and danger-associated molecular patterns. The role of TLR4 in the pathophysiology of several age-related diseases is also well recognized, such as prostate cancer (PCa). TLR4 polymorphisms have been related to PCa risk, but the relationship between TLR4 genotypes and aggressive PCa risk has not been evaluated by any systematic reviews. Methods: We performed a systematic review and meta-analysis of candidate-gene and genome-wide association studies analyzing this relationship and included only white populatio…

MaleProstate cancer polymorphisms of TLR4 aggressive prostate cancer risk meta-analysisSystematic Reviewslcsh:MedicineGenome-wide association studySingle-nucleotide polymorphismBiologyResearch and Analysis MethodsBioinformaticsPolymorphism Single NucleotideProstate cancerGenotypeGeneticsCancer GeneticsmedicineHumansSettore MED/05 - Patologia ClinicaGenetic Predisposition to DiseaseStatistical Methodslcsh:ScienceGenotypingGenetic associationEvolutionary BiologyMultidisciplinarylcsh:RProstatic NeoplasmsBiology and Life SciencesResearch Assessmentmedicine.diseaseImmunity InnateToll-Like Receptor 4Systematic reviewMeta-analysisPhysical SciencesGenetic Polymorphismlcsh:QPopulation GeneticsMathematicsStatistics (Mathematics)Genome-Wide Association StudyResearch ArticleMeta-AnalysisPLoS ONE
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Identification of a novel candidate locus for juvenile idiopathic arthritis at 14q13.2 in the Latvian population by association analysis with microsa…

2010

To identify novel juvenile idiopathic arthritis (JIA) susceptibility loci, a 270 kb genomic region encompassing FAM177A1, KIAA0391, and PSMA6 genes was genotyped in 97 oligoarthritis (JIoA) and 50 polyarthritis (JIpA) patients and 230 individuals without autoimmune disorders by five microsatellites (MS) previously described as HSMS markers of the 14q13.2 region. Direct sequencing revealed two variable components of the (CAA)(n)(A)(m) motif in HSMS602 marker (FAM177A1 gene). Repeat (AC)(5)AT(AC)(n) of the HSMS701 (KIAA0391 gene) was variable in the Latvian population only in its downstream part. Allele (AC)(5)AT(AC)(15) of HSMS701 was found to be strongly associated with JIA (p = 4.91 x 10(-…

MaleProteasome Endopeptidase ComplexGenetic LinkagePopulationPSMA6BiologyGenotypeGeneticsmedicineOdds RatioHumansGenetic Predisposition to DiseaseAlleleeducationMolecular BiologyAllelesGenetic associationGeneticsChromosomes Human Pair 14education.field_of_studyOligoarthritisPolymorphism GeneticCell BiologyGeneral Medicinemedicine.diseaseLatviaArthritis JuvenileGenetic markerGenetic LociCase-Control StudiesPolyarthritisFemaleMicrosatellite RepeatsDNA and cell biology
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Juvenile idiopathic arthritis subtype- and sex-specific associations with genetic variants in the PSMA6/PSMC6/PSMA3 gene cluster.

2014

BackgroundThe ubiquitin proteasome system plays an exceptional biological role in the antigen processing and immune response and it could potentially be involved in pathogenesis of many immunity-related diseases, including juvenile idiopathic arthritis (JIA).MethodsThe PSMB5 (rs11543947), PSMA6 (rs2277460, rs1048990), PSMC6 (rs2295826, rs2295827), and PSMA3 (rs2348071) proteasomal genes were genotyped on JIA subtype- and sex-specific association; plasma proteasome levels was measured in patients having risk and protective four-locus genotypes and eventual functional significance of allele substitutions was evaluated in silico.ResultsLoci rs11543947 and rs1048990 were identified as disease n…

MaleProteasome Endopeptidase ComplexGenotypeArthritisPSMA6PSMA3Polymorphism Single NucleotidePSMC6polymorphismPathogenesisGenotypeOdds RatioMedicineHumansgenotype–sex interactionplasma proteasomeGenetic Predisposition to DiseasePediatrics Perinatology and Child HealthAlleleChildbusiness.industrylcsh:RJ1-570PSMC6lcsh:PediatricsPSMA6medicine.diseaseArthritis JuvenilePSMB5PSMA3Multigene FamilyPediatrics Perinatology and Child HealthImmunologyjuvenile idiopathic arthritisFemalebusinessPediatrics and neonatology
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