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showing 10 items of 15327 documents

Molecular diagnosis of PIK3CA-related overgrowth spectrum (PROS) in 162 patients and recommendations for genetic testing.

2017

Postzygotic activating mutations of PIK3CA cause a wide range of mosaic disorders collectively referred to as PIK3CA-related overgrowth spectrum (PROS). We describe the diagnostic yield and characteristics of PIK3CA sequencing in PROS. We performed ultradeep next-generation sequencing (NGS) of PIK3CA in various tissues from 162 patients referred to our clinical laboratory and assessed diagnostic yield by phenotype and tissue tested. We identified disease-causing mutations in 66.7% (108/162) of patients, with mutant allele levels as low as 1%. The diagnostic rate was higher (74%) in syndromic than in isolated cases (35.5%; P = 9.03 × 10−5). We identified 40 different mutations and found stro…

0301 basic medicineAdultMalePathologymedicine.medical_specialtyAdolescentGenotypeClass I Phosphatidylinositol 3-KinasesPrenatal diagnosisBioinformaticsmedicine.disease_causeDNA sequencing03 medical and health sciencesYoung Adult0302 clinical medicinePrenatal DiagnosisGenotypemedicineHumansGenetic Predisposition to DiseaseGenetic TestingAlleleChildGenetics (clinical)AllelesGenetic Association StudiesGrowth DisordersGenetic testingMutationmedicine.diagnostic_testbusiness.industryMosaicismInfant NewbornDisease ManagementHigh-Throughput Nucleotide SequencingInfantSequence Analysis DNAPhenotype030104 developmental biologyPhenotypeAmino Acid SubstitutionChild PreschoolMutationAllelic heterogeneityFemalebusiness030217 neurology & neurosurgeryGenetics in medicine : official journal of the American College of Medical Genetics
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Malignant granular cell tumor of soft tissues: a study of two new cases.

2001

We describe 2 cases of malignant granular cell tumor. A marked discrepancy exists concerning the criteria of malignancy of this rare entity, which was diagnosed in male patients aged 41 and 52 years, respectively. They presented with tumors measuring more then 3.5 cm; one arose in the dermis and subcutaneous tissue in the region of the scapula, whereas the other was situated in deeper soft tissue in the pelvis. One case, with previous diagnosis of benign granular cell tumor, presented local recurrence 2 years after the initial diagnosis. The other case presented a fascicular pattern of growth invading adjacent muscular tissue. Both neoplasms were composed of polygonal and spindle cells, sho…

0301 basic medicineAdultMalePathologymedicine.medical_specialtyBenign Granular Cell TumorVimentinSoft Tissue NeoplasmsBiologyMalignancyPathology and Forensic Medicine03 medical and health sciences0302 clinical medicineDermismedicineMalignant Granular Cell TumorHumansSoft tissueAnatomyMiddle Agedmedicine.diseaseImmunohistochemistry030104 developmental biologymedicine.anatomical_structureGranular Cell Tumor030220 oncology & carcinogenesisbiology.proteinImmunohistochemistrySurgeryAnatomySubcutaneous tissueInternational journal of surgical pathology
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Cancer-associated circulating large extracellular vesicles in cholangiocarcinoma and hepatocellular carcinoma.

2017

Background & Aims Large extracellular vesicles, specifically AnnexinV + EpCAM + CD147 + tumour-associated microparticles (taMPs), facilitate the detection of colorectal carcinoma (CRC), non-small cell lung carcinoma (NSCLC) as well as pancreas carcinoma (PaCa). Here we assess the diagnostic value of taMPs for detection and monitoring of hepatocellular carcinoma (HCC) and cholangiocarcinoma (CCA). Specifically, the aim of this study was to differentiate liver taMPs from other cancer taMPs, such as CRC and NSCLC. Methods Fluorescence-activated cell scanning (FACS) was applied to detect various taMP populations in patients' sera that were associated with the presence of a tumour (AnnexinV + Ep…

0301 basic medicineAdultMalePathologymedicine.medical_specialtyCirrhosisCarcinoma HepatocellularColorectal cancerAsialoglycoprotein ReceptorCholangiocarcinomaDiagnosis Differential03 medical and health scienceschemistry.chemical_compoundYoung Adult0302 clinical medicineCell-Derived MicroparticlesCell Line TumorCarcinomaBiomarkers TumorMedicineHumansLiquid biopsyAnnexin A5AgedHepatologybusiness.industryLiver NeoplasmsEpithelial cell adhesion moleculeHep G2 CellsMiddle Agedmedicine.diseaseEpithelial Cell Adhesion MoleculeTumor Burden030104 developmental biologychemistryBile Duct Neoplasms030220 oncology & carcinogenesisHepatocellular carcinomaCancer cellCancer researchBasiginFemalebusinessLiver cancerJournal of hepatology
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Improving tumour budding evaluation in colon cancer by extending the assessment area in colectomy specimens.

2019

AIMS It is recommended that tumour budding in colon cancer be counted on haematoxylin and eosin-stained sections in a hotspot area of 0.785 mm2 with a ×20 microscope objective. However, tumour buds may be difficult to visualise on haematoxylin and eosin-stained sections, and counting in such a limited area may result in overestimation in cases with focal budding. The aim of this study was to assess the contributions of various factors to improving tumour budding risk stratification: increasing the number of fields counted, using cytokeratin immunostaining, and recording proliferation, the apoptotic index and the emperipoletic index in tumour buds. METHODS AND RESULTS We created an explorato…

0301 basic medicineAdultMalePathologymedicine.medical_specialtyHistologyMitotic indexColorectal cancermedicine.medical_treatmentKaplan-Meier EstimateDisease-Free SurvivalPathology and Forensic Medicine03 medical and health sciencesCytokeratin0302 clinical medicineTumor buddingMedicineHumansGrading (tumors)ColectomyColectomyAgedAged 80 and overPathology Clinicalbusiness.industryHazard ratioGeneral MedicineMiddle Agedmedicine.disease030104 developmental biology030220 oncology & carcinogenesisColonic NeoplasmsFemaleNeoplasm GradingbusinessImmunostainingHistopathologyReferences
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The Amount of Melanin Influences p16 Loss in Spitzoid Melanocytic Lesions: Correlation With CDKN2A Status by FISH and MLPA.

2019

AIMS The risk assessment of spitzoid lesions is one of the most difficult challenges in dermatopathology practice. In this regard, the loss of p16 expression and the homozygous deletion of CDKN2A, have been pointed in the literature as reliable indicators of high risk. However, these findings are poorly reproducible, and the molecular bases underlying the loss of p16 expression remain unclear. We aimed to identify the underlying events causing loss of CDKN2A/p16 in spitzoid tumors. MATERIALS AND METHODS We evaluated the immunohistochemical expression of p16, and the presence of CDKN2A genetic alterations detected through fluorescence in situ hybridization (FISH) and multiplex ligation-depen…

0301 basic medicineAdultMalePathologymedicine.medical_specialtyHistologySkin NeoplasmsPathology and Forensic MedicineMelanin03 medical and health sciencesYoung Adult0302 clinical medicineCDKN2ANevus Epithelioid and Spindle CellmedicineBiomarkers TumorNevusHumansMultiplex ligation-dependent probe amplificationneoplasmsMelanomaCyclin-Dependent Kinase Inhibitor p16In Situ Hybridization FluorescenceMelaninsmedicine.diagnostic_testbusiness.industryMelanomamedicine.diseaseImmunohistochemistryGene Expression Regulation NeoplasticMedical Laboratory Technology030104 developmental biology030220 oncology & carcinogenesisMutationImmunohistochemistryMelanocytesFemaleDermatopathologybusinessMultiplex Polymerase Chain ReactionFluorescence in situ hybridizationApplied immunohistochemistrymolecular morphology : AIMM
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Expression of NF-κB and IL-6 in oral precancerous and cancerous lesions: An immunohistochemical study.

2014

Background The purpose of this study was to evaluate the immunohistochemical expression of NF-κB and IL-6 in oral premalignant and malignant lesions and to investigate their possible correlation with the presence of subepithelial inflammation. Material and Methods Thirty two oral premalignant lesions, clinically compatible with leukoplakia or erythroplakia, were investigated. Microscopically, 11 of them showed hyperkeratosis and acanthosis (epithelial hyperplasia) and 21 showed dysplasia of varying degrees. Nine cases of OSCC and four control cases of normal oral mucosa were also included in the study. Immunohistochemical staining with NF-κB (p65) and IL-6 was performed. IL-6 and nuclear NF…

0301 basic medicineAdultMalePathologymedicine.medical_specialtyHyperkeratosisOdontologíaAcanthosis03 medical and health sciences0302 clinical medicineMedicineHumansGeneral DentistryLeukoplakiaAgedMouth neoplasmAged 80 and overErythroplakiaOral Medicine and Pathologybusiness.industryInterleukin-6ResearchNF-kappa BMiddle Aged:CIENCIAS MÉDICAS [UNESCO]medicine.diseaseCiencias de la saludImmunohistochemistrystomatognathic diseases030104 developmental biologyOtorhinolaryngologyDysplasia030220 oncology & carcinogenesisUNESCO::CIENCIAS MÉDICASImmunohistochemistrySurgeryFemaleMouth NeoplasmsbusinessPrecancerous ConditionsImmunostainingLeukoplakiaMedicina oral, patologia oral y cirugia bucal
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Perivascular Cells in Diffuse Cutaneous Systemic Sclerosis Overexpress Activated ADAM12 and Are Involved in Myofibroblast Transdifferentiation and De…

2016

Objective.Microvascular damage is pivotal in the pathogenesis of systemic sclerosis (SSc), preceding fibrosis, and whose trigger is not still fully understood. Perivascular progenitor cells, with profibrotic activity and function, are identified by the expression of the isoform 12 of ADAM (ADAM12) and this molecule may be upregulated by transforming growth factor-β (TGF-β). The goal of this work was to evaluate whether pericytes in the skin of patients with diffuse cutaneous SSc (dcSSc) expressed ADAM12, suggesting their potential contribution to the fibrotic process, and whether TGF-β might modulate this molecule.Methods.After ethical approval, mesenchymal stem cells (MSC) and fibroblasts …

0301 basic medicineAdultMalePathologymedicine.medical_specialtyImmunologyADAM12 Protein03 medical and health sciencesYoung AdultRheumatologyFibrosisTransforming Growth Factor betamedicineImmunology and AllergyHumansProgenitor cellMyofibroblastsSkinintegumentary systembusiness.industryMedicine (all)FIBROSIS; PERICYTE; SYSTEMIC SCLEROSIS; Rheumatology; Immunology; Immunology and AllergyMesenchymal stem cellTransdifferentiationMesenchymal Stem CellsMiddle Agedmedicine.diseaseFibrosisActinsUp-RegulationSettore MED/16 - Reumatologia030104 developmental biologymedicine.anatomical_structurePERICYTEFIBROSIS; PERICYTE; SYSTEMIC SCLEROSIS; Immunology and Allergy; Rheumatology; Immunology; Medicine (all)SYSTEMIC SCLEROSISCell TransdifferentiationScleroderma DiffuseFemalePericyteBone marrowbusinessPericytesMyofibroblastTransforming growth factorThe Journal of rheumatology
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Endothelial cell damage is the central part of COVID-19 and a mouse model induced by injection of the S1 subunit of the spike protein☆

2021

Neurologic complications of symptomatic COVID-19 are common. Brain tissues from 13 autopsies of people who died of COVID-19 were examined. Cultured endothelial and neuronal cells were incubated with and wild type mice were injected IV with different spike subunits. In situ analyses were used to detect SARS-CoV-2 proteins and the host response. In 13/13 brains from fatal COVID-19, pseudovirions (spike, envelope, and membrane proteins without viral RNA) were present in the endothelia of microvessels ranging from 0 to 14 positive cells/200× field (mean 4.3). The pseudovirions strongly co-localized with caspase-3, ACE2, IL6, TNFα, and C5b-9. The surrounding neurons demonstrated increased NMDAR2…

0301 basic medicineAdultMalePathologymedicine.medical_specialtyProtein subunitH&E stainCaspase 3Spike proteinThirstPathology and Forensic Medicine03 medical and health sciencesMice0302 clinical medicineS1 subunitmedicineAnimalsHumansAgedAged 80 and overChemistrySARS-CoV-2COVID-19Endothelial CellsGeneral MedicineOriginal ContributionMiddle AgedMolecular biologyEndothelial stem cellDisease Models AnimalProtein Subunits030104 developmental biologyMembrane protein030220 oncology & carcinogenesisMicrovesselsSpike Glycoprotein CoronavirusImmunohistochemistryRNA ViralTumor necrosis factor alphaFemaleAutopsymedicine.symptomAnnals of Diagnostic Pathology
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Detection of RET rearrangements in papillary thyroid carcinoma using RT-PCR and FISH techniques - A molecular and clinical analysis.

2019

Abstract Introduction Oncogenic BRAF and RAS mutations as well as multiple known (and yet unknown) RET fusion oncogenes comprise the majority of causative molecular alterations in papillary thyroid carcinoma (PTC). Apparently “mutation-negative” PTCs encompass a heterogenous group impeding analysis of prognostic significance of underlying genetics. Material and methods BRAF wild type PTC tissue of 56 patients was analyzed using two established methods: hybrid-specific RT-PCR for the predominant rearrangement RET/PTC1 and fluorescent in situ hybridization (FISH). Clinical features of the cases with and without RET rearrangement were compared (patient age, gender, tumor size, focality, lymph …

0301 basic medicineAdultMaleProto-Oncogene Proteins B-rafmedicine.medical_specialtyendocrine system diseasesIn situ hybridizationThyroid carcinomaIodine Radioisotopes03 medical and health sciences0302 clinical medicinemedicineHumansAvidityOncogene FusionThyroid NeoplasmsLymph nodeIn Situ Hybridization FluorescenceAgedRET/PTC RearrangementGene RearrangementClinical pathologybusiness.industryReverse Transcriptase Polymerase Chain ReactionProto-Oncogene Proteins c-retGeneral MedicineMiddle AgedTumor BurdenReverse transcription polymerase chain reaction030104 developmental biologymedicine.anatomical_structureReal-time polymerase chain reactionOncologyThyroid Cancer Papillary030220 oncology & carcinogenesisCancer researchSurgeryFemaleLymph NodesbusinessEuropean journal of surgical oncology : the journal of the European Society of Surgical Oncology and the British Association of Surgical Oncology
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Systemic redox biomarkers and their relationship to prognostic risk markers in autosomal dominant polycystic kidney disease and IgA nephropathy.

2017

Abstract Background Oxidative stress is evident from an early stage in chronic kidney disease (CKD). Therefore, we investigated redox biomarkers in polycystic kidney disease (ADPKD) and IgA nephropathy (IGAN). Methods This is a case-control study with three groups: ADPKD (n = 54), IGAN (n = 58) and healthy controls (n = 86). The major plasma aminothiols with their redox species were examined: homocysteine (Hcy), cysteinglycine (CG), cysteine (Cys) and glutathione (GSH). The redox ratio was the ratio of reduced free and oxidized aminothiols in plasma. We investigated malonedialdehyde (MDA) and advanced oxidation protein products (AOPP), and ten single nucleotide polymorphisms of antioxidant …

0301 basic medicineAdultMaleRiskmedicine.medical_specialtyHomocysteineClinical Biochemistry030232 urology & nephrologyAutosomal dominant polycystic kidney diseaseurologic and male genital diseasesmedicine.disease_causePolymorphism Single NucleotideNephropathy03 medical and health scienceschemistry.chemical_compound0302 clinical medicineInternal medicinePolycystic kidney diseaseMedicineHumansHomocysteineGenetic Association StudiesProteinuriabusiness.industrySuperoxide DismutaseGlomerulonephritis IGAGeneral MedicineDipeptidesMiddle Agedmedicine.diseasePolycystic Kidney Autosomal DominantPrognosisOxidative Stress030104 developmental biologyEndocrinologychemistryAdvanced Oxidation Protein ProductsCase-Control StudiesDisease ProgressionFemaleGene polymorphismLipid Peroxidationmedicine.symptombusinessOxidoreductasesOxidation-ReductionOxidative stressBiomarkersKidney diseaseClinical biochemistry
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