Search results for "fibrosis"
showing 10 items of 901 documents
Role of the small heat shock protein alphaB-crystallin in pulmonary fibrosis and its implication in the signaling pathway of the Transforming Growth …
2013
Idiopathic pulmonary fibrosis (IPF) has no effective current treatment. It is characterized by a sub-pleural onset and the presence of myofibroblasts, responsible for the excessive extracellular matrix synthesis. Transforming Growth Factor (TGF)-β1 is considered as the major profibrotic cytokine. Its signaling pathway occurs through the Smads proteins, including Smad4. TGF-β1 allows the differentiation of lung fibroblasts and epithelial and mesothelial cells into myofibroblasts. AB-crystallin is a small heat shock protein overexpressed in liver, renal and vascular fibrosis and can be induced by TGF-β1. In this study, we assessed the role of αB-crystallin in pleural and pulmonary fibrosis. W…
The selective advantage of cystic fibrosis heterozygotes tested by aDNA analysis: A preliminary investigation
2000
Recently a heterozygote advantage was suggested to explain the high incidence (1:25 carrier individuals in Europeans) of the cystic fibrosis gene. This selective advantage was speculated to be due to a high resistance to chloride-secreting diarrhea, including cholera. Up to now the major efforts to test directly this hypothesis have been limited to animal models.
Asymptomatic Amyloidosis at the Time of Diagnostic Bone Marrow Biopsy in Newly Diagnosed Patients with Multiple Myeloma and Smoldering Multiple Myelo…
2009
Abstract Abstract 2803 Poster Board II-779 Background. The rate of asymptomatic amyloidosis (asym-amyloidosis) detected in patients with newly diagnosed multiple myeloma (MM) or smoldering multiple myeloma (SMM) is unknown. This topic is significant because unrecognized AL may be associated with increased mortality may change the patient's management. The objective of the present investigation was to evaluate the number and clinical significance of asym-amyloidosis in MM and SM patients at the time of the diagnostic bone marrow (BM) biopsy for MM. Materials and Methods. The study population was selected from the Mayo Clinic Dysproteinemia database and consisted of consecutive patients with …
Procollagen C-proteinase Enhancer Stimulates Procollagen Processing by Binding to the C-propeptide Region Only*
2011
Background: Procollagen C-proteinase enhancer-1 (PCPE-1) is an extracellular glycoprotein that increases activity of certain zinc metalloproteinases involved in tissue development and repair. Results: PCPE-1 binds uniquely to the C-propeptide region of the procollagen molecule. Conclusion: PCPE-1 enhances proteolysis by binding solely to the procollagen C-propeptides. Significance: These data may lead to future applications in the development of antifibrotic therapies.
Accuracy of Transient Elastography in Assessing Fibrosis at Diagnosis in Naïve Patients With Primary Biliary Cholangitis: A Dual Cut-Off Approach
2021
Background & aims Liver fibrosis holds a relevant prognostic meaning in primary biliary cholangitis (PBC). Non-invasive fibrosis evaluation using vibration-controlled transient elastography (VCTE) is routinely performed. However, there is limited evidence on its accuracy at diagnosis in PBC. We aimed to estimate the diagnostic accuracy of VCTE in assessing advanced fibrosis at disease presentation in PBC. Approach & results We collected data from 167 consecutive treatment-naive PBC patients who underwent liver biopsy(LB) at diagnosis at six Italian centers. VCTE examinations were completed within 12 weeks of LB. Biopsies were scored by two blinded expert pathologists, according to Ludwig sy…
Infección por mohos en pacientes con fibrosis quística. Impacto en su evolución clínica
2021
Antecedentes: Existen avances importantes en el manejo de la infección bacteriana en pacientes con fibrosis quística (FQ), pero hay muchas lagunas en el campo de las infecciones fúngicas. Objetivos: El objetivo del estudio fue analizar si la colonización respiratoria crónica por hongos filamentosos tenía impacto clínico y si el tratamiento antifúngico modificaba la enfermedad. Métodos: Estudio prospectivo, bicéntrico y descriptivo con un seguimiento de 3 años y controles médicos cuatrimestrales. Se incluyeron pacientes adultos de dos Unidades de Fibrosis Quística de hospitales terciarios. Se recogieron datos de variables clínicas, microbiológicas, analíticas y espirométricas; en un subgrupo…
Inversion of Left Atrial Appendage Will Cause Compressive Stresses in the Tissue: Simulation Study of Potential Therapy
2022
In atrial fibrillation (AF), thromboembolic events can result from the particular conformation of the left atrial appendage (LAA) bearing increased clot formation and accumulation. Current therapies to reduce the risk of adverse events rely on surgical exclusion or percutaneous occlusion, each of which has drawbacks limiting application and efficacy. We sought to quantify the hemodynamic and structural loads of a novel potential procedure to partially invert the “dead” LAA space to eliminate the auricle apex where clots develop. A realistic left atrial geometry was first achieved from the heart anatomy of the Living Heart Human Model (LHHM) and then the left atrial appendage inversion (LAAI…
La relación entre la proporción de masa grasa corporal y la función pulmonar en pacientes con Fibrosis Quística
2022
La fibrosis quística (FQ) es una enfermedad recesiva causada por la mutación del gen CFTR que altera el transporte del cloro en las células epiteliales y ocasiona principalmente alteraciones respiratorias y digestivas. Se han identificado más de 2000 mutaciones, siendo la más frecuente es F508del. Últimamente, la prevalencia de la desnutrición ha disminuido gracias al diagnóstico y tratamiento tempranos, ya que se ha demostrado que es un factor de riesgo predictor de morbimortalidad y deterioro en la calidad de vida, y por tanto la intervención en esta, enlentece el empeoramiento progresivo respiratorio. Las guías clínicas actuales recomiendan como objetivo para los pacientes adultos alcanz…
Cellular effects of HMG-CoA reductase inhibitors on blood cells (monocytes, macrophages, platelets)
2002
Atherosclerosis is the primary cause of ischaemic heart disease (IHD) and stroke and the leading cause of death and long-term disabilities in westernized societies. Several important environmental and genetic risk factors are associated with atherosclerosis. Over the past decade, it has become evident that atherosclerosis is not simply an inevitable degenerative consequence of ageing, but rather a chronic inflammatory condition connecting altered cholesterol metabolism and other risk factors to the development and progression of the atherosclerotic lesion and its sequelae like plaque rupture, fibrosis, calcification and thrombosis [1].