Search results for "BLAST"
showing 10 items of 2136 documents
Heterogeneity of osteogenesis imperfecta. Biochemical and morphological findings in a case of type III according to Sillence.
1986
A male infant with pale-blue sclerae, who died at the age of 6 weeks through the aspiration of food, presented multiple fractures and deformation of the long tubular bones. The clinical and radiological findings and the course indicated osteogenesis imperfecta, type III, according to Sillence's classification. The family history was unremarkable. Light and electron microscopic studies of iliac crest bone obtained postmortem, showed an abrupt interruption of endochondral ossification, with an active periosteal ossification. In the region of the fractures, a mixed desmochondral callus was seen. The endoplasmic reticulum of the osteoblasts was markedly dilated, the mitochondria were swollen. T…
Vascular effects of sildenafil in patients with pulmonary fibrosis and pulmonary hypertension: an ex vivo/in vitro study
2015
Sildenafil improves the 6-min walking distance in patients with idiopathic pulmonary fibrosis (IPF) and right-sided ventricular systolic dysfunction.We analysed the previously unexplored role of sildenafil on vasoconstriction and remodelling of pulmonary arteries from patients with IPF and pulmonary hypertension (PH) ex vivo. Pulmonary arteries from 18 donors without lung disease, nine IPF, eight PH+IPF and four PH patients were isolated to measure vasodilator and anti-contractile effects of sildenafil in isometric organ bath. Ventilation/perfusion was explored in an animal model of bleomycin lung fibrosis.Sildenafil relaxed serotonin (5-HT) pre-contracted pulmonary arteries in healthy dono…
MUC1 intracellular bioactivation mediates lung fibrosis
2019
BackgroundSerum KL6/mucin 1 (MUC1) has been identified as a potential biomarker in idiopathic pulmonary fibrosis (IPF), but the role of MUC1 intracellular bioactivation in IPF is unknown.ObjectiveTo characterise MUC1 intracellular bioactivation in IPF.Methods and resultsThe expression and phosphorylation of Thr41 and Tyr46 on the intracellular MUC1-cytoplasmic tail (CT) was increased in patients with IPF (n=22) compared with healthy subjects (n=21) and localised to fibroblasts and hyperplastic alveolar type II cells. Transforming growth factor (TGF)-β1 phosphorylated SMAD3 and thereby increased the phosphorylation of MUC1-CT Thr41 and Tyr46 in lung fibroblasts and alveolar type II cells, ac…
Expression of KIT (CD117) in Biphasic Pulmonary Blastoma. Novel Data on Histogenesis
2003
Biphasic pulmonary blastoma (BPB) is a rare primary neoplasm of the lung and its histogenesis is still uncertain. It has been proposed that BPB is derived from mesoderm or endoderm. Others suggested an origin from a single pluripotential cell. We present a case of a BPB with emphasis on expression of the stem cell factor receptor KIT (CD117). We describe a 61-year-old male patient with a BPB of the upper right lobe. Immunohistochemical analysis was performed using a panel of several antibodies including anti-CD117. Strong cytoplasmic expression of CD117 was found in the epithelium (cytokeratin-positive) as well as in the spindle cells (cytokeratin-negative). Expression of CD117 in both mese…
Upregulation of basic fibroblast growth factor in smokers with chronic bronchitis
2006
The aim of the study was to investigate the expression of basic fibroblast growth factor (bFGF) and its receptor, fibroblast growth factor receptor (FGFR)-1, in the central airways of smokers with chronic bronchitis. The lobar bronchi from 17 subjects undergoing thoracotomy for solitary nodules were examined. All had a history of cigarette smoking, nine had symptoms of chronic bronchitis and airflow limitation, and eight were asymptomatic with normal lung function. Using immunohistochemical methods, bFGF and FGFR-1 expression in the total airway wall and the different airway compartments, i.e. bronchial glands, submucosal vessels and smooth muscle, was quantified. Moreover, to investigate t…
Mechanical strain causes adaptive change in bronchial fibroblasts enhancing profibrotic and inflammatory responses
2016
Asthma is characterized by periodic episodes of bronchoconstriction and reversible airway obstruction; these symptoms are attributable to a number of factors including increased mass and reactivity of bronchial smooth muscle and extracellular matrix (ECM) in asthmatic airways. Literature has suggested changes in cell responses and signaling can be elicited via modulation of mechanical stress acting upon them, potentially affecting the microenvironment of the cell. In this study, we hypothesized that mechanical strain directly affects the (myo)fibroblast phenotype in asthma. Therefore, we characterized responses of bronchial fibroblasts, from 6 normal and 11 asthmatic non-smoking volunteers,…
Ameloblastic fibro-odontoma: A conservative surgical approach
2009
Ameloblastic fibro-odontoma (AFO) is a rare benign mixed odontogenic tumor that occurs predominantly in children and young adults with no gender predilection and anatomic site, usually appearing as a painless swelling. We present a case of an 11-year-old non-Caucasian boy complaining of large painless isolated swelling in the right mandibular body. Intraoral examination revealed a tumoral mass with cortical bone expansion, covered by normal mucosa measuring 4.0 x 2.0 cm, located on both the lingual and buccal surfaces of the right body of the mandible, with displacement of the neighboring teeth. Panoramic radiography revealed an expansile, radiolucent and well circumscribed lesion with scat…
Salvage Re-irradiation Options in Adult Medulloblastoma: A Case Report and Review of the Literature
2020
Background/aim Medulloblastoma is a rare tumor of adult age, while it occurs more frequently in children. Given the rarity, there is a lack of evidence for the treatment of recurrent disease. Few data are available about salvage re-irradiation collecting very heterogeneous series. Case report A 51-year-old male presented with headache, nausea, double vision, and gait disorders. A contrast-enhanced brain-MRI showed the presence of multifocal medulloblastoma. After surgery, adjuvant craniospinal radiotherapy was performed, chemotherapy was stopped due to toxicity. After 27 months, a new MRI and a Methionine-PET revealed a late pontocerebellar relapse; multidisciplinary board decided for a SBR…
Maternal Supplementation with Folic Acid and Other Vitamins and Risk of Leukemia in Offspring
2014
Maternal prenatal supplementation with folic acid and other vitamins has been inconsistently associated with a reduced risk of childhood acute lymphoblastic leukemia (ALL). Little is known regarding the association with acute myeloid leukemia (AML), a rarer subtype.We obtained original data on prenatal use of folic acid and vitamins from 12 case-control studies participating in the Childhood Leukemia International Consortium (enrollment period: 1980-2012), including 6,963 cases of ALL, 585 cases of AML, and 11,635 controls. Logistic regression was used to estimate pooled odds ratios (ORs) and 95% confidence intervals (CIs), adjusted for child's age, sex, ethnicity, parental education, and s…
Cellular composition and three-dimensional organization of the subventricular germinal zone in the adult mammalian brain.
1997
The adult mammalian subventricular zone (SVZ) contains stem cells that give rise to neurons and glia.In vivo, SVZ progeny migrate 3–8 mm to the olfactory bulb, where they form neurons. We show here that the SVZ of the lateral wall of the lateral ventricles in adult mice is composed of neuroblasts, glial cells, and a novel putative precursor cell. The topographical organization of these cells suggests how neurogenesis and migration are integrated in this region. Type A cells had the ultrastructure of migrating neuronal precursors. These cells were arranged as chains parallel to the walls of the ventricle and were polysialylated neural adhesion cell molecule- (PSA–NCAM), TuJ1- (β-tubulin), an…