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  • 1
    Language: English
    In: Leukemia, 2014-05, Vol.28 (5), p.1132-1134
    Subject(s): Core Binding Factors - genetics ; Leukemia, Myeloid, Acute - diagnosis ; Prognosis ; Leukemia, Myeloid, Acute - pathology ; Humans ; Proto-Oncogene Proteins c-kit - genetics ; Mutation ; Leukemia, Myeloid, Acute - genetics ; Development and progression ; Transcription factors ; Genetic aspects ; Gene mutations ; Health aspects ; Leukemia in children ; Index Medicus
    ISSN: 0887-6924
    E-ISSN: 1476-5551
    Source: Nature Open Access
    Source: Academic Search Ultimate
    Source: Alma/SFX Local Collection
    Source: © ProQuest LLC All rights reserved〈img src="https://exlibris-pub.s3.amazonaws.com/PQ_Logo.jpg" style="vertical-align:middle;margin-left:7px"〉
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  • 2
    Language: English
    In: Leukemia, 2017-01, Vol.31 (1), p.18-25
    Description: Recurrent molecular markers have been routinely used in acute myeloid leukemia (AML) for risk assessment at diagnosis, whereas their post-induction monitoring still represents a debated issue. We evaluated the prognostic value and biological impact of minimal residual disease (MRD) and of the allelic ratio (AR) of FLT3-internal-tandem duplication (ITD) in childhood AML. We retrospectively screened 494 children with de novo AML for FLT3-ITD mutation, identifying 54 harboring the mutation; 51% of them presented high ITD-AR at diagnosis and had worse event-free survival (EFS, 19.2 versus 63.5% for low ITD-AR, 〈0.05). Forty-one percent of children with high levels of MRD after the 1st induction course, measured by a patient-specific real-time-PCR, had worse EFS (22.2 versus 59.4% in low-MRD patients, P〈0.05). Next, we correlated these parameters with gene expression, showing that patients with high ITD-AR or persistent MRD had characteristic expression profiles with deregulated genes involved in methylation and acetylation. Moreover, patients with high CyclinA1 expression presented an unfavorable EFS (20.3 versus 51.2% in low CyclinA1 group, P〈0.01). Our results suggest that ITD-AR levels and molecular MRD should be considered in planning clinical management of FLT3-ITD patients. Different transcriptional activation of epigenetic and oncogenic profiles may explain variability in outcome among these patients, for whom novel therapeutic approaches are desirable.
    Subject(s): fms-Like Tyrosine Kinase 3 - genetics ; Disease-Free Survival ; Leukemia, Myeloid, Acute - diagnosis ; Prognosis ; Epigenesis, Genetic - genetics ; Humans ; Child, Preschool ; Retrospective Studies ; Gene Expression Regulation, Leukemic ; Child ; Neoplasm, Residual - genetics ; Leukemia, Myeloid, Acute - genetics ; Molecular targeted therapy ; Gene mutations ; Gene expression ; Health aspects ; Innovations ; Index Medicus
    ISSN: 0887-6924
    E-ISSN: 1476-5551
    Source: Nature Open Access
    Source: Alma/SFX Local Collection
    Source: © ProQuest LLC All rights reserved〈img src="https://exlibris-pub.s3.amazonaws.com/PQ_Logo.jpg" style="vertical-align:middle;margin-left:7px"〉
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  • 3
    Language: English
    In: British journal of dermatology (1951), 2004-01, Vol.150 (1), p.127-131
    Description: Summary Background  In children with atopic dermatitis (AD), eczema is easily aggravated by contact with irritant factors (e.g. aggressive detergents, synthetic and woollen clothes, climatic factors). Objectives  To evaluate the effectiveness of a special silk fabric (MICROAIR DermaSilk®) in the treatment of young children affected by AD with acute lesions at the time of examination. Methods  Forty-six children (mean age 2 years) affected by AD in an acute phase were recruited: 31 received special silk clothes (group A) which they were instructed to wear for a week; the other 15 served as a control group (group B) and wore cotton clothing. Topical moisturizing creams or emulsions were the only topical treatment prescribed in both groups. The overall severity of the disease was evaluated using the SCORAD index. In addition, the local score of an area covered by the silk clothes was compared with the local score of an uncovered area in the same child. All patients were evaluated at baseline and 7 days after the initial examination. Results  At the end of the study a significant decrease in AD severity was observed in the children of group A (mean SCORAD decrease from 43 to 30; P = 0·003). At the same time, the improvement in the mean local score of the covered area (from 32 to 18·6; P = 0·001) was significantly greater than that of the uncovered area (from 31 to 26; P = 0·112). Conclusions  The use of special silk clothes may be useful in the management of AD in children.
    Subject(s): silk clothes ; skin care ; atopic dermatitis ; Immunopathology ; Biological and medical sciences ; Allergic diseases ; Medical sciences ; Dermatology ; Skin allergic diseases. Stinging insect allergies ; Severity of Illness Index ; Acute Disease ; Follow-Up Studies ; Clothing ; Humans ; Child, Preschool ; Infant ; Treatment Outcome ; Dermatitis, Atopic - rehabilitation ; Textiles ; Cotton Fiber ; Silk ; Child ; Insect Proteins ; Index Medicus
    ISSN: 0007-0963
    E-ISSN: 1365-2133
    Source: Wiley Online Library All Backfiles
    Source: Alma/SFX Local Collection
    Source: © ProQuest LLC All rights reserved〈img src="https://exlibris-pub.s3.amazonaws.com/PQ_Logo.jpg" style="vertical-align:middle;margin-left:7px"〉
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  • 4
    Language: English
    In: Reumatologia, 2016, Vol.54 (5), p.264-266
    Description: Alveolar hemorrhage (AH) is a rare syndrome that can often occur in autoimmune diseases, blood clotting disorders, infection or by acute inhalation injury, presenting rapid evolution and high mortality, especially with late diagnosis and treatment. Among the autoimmune diseases, there are reported cases in patients with primary antiphospholipid syndrome (PAPS), vasculitis and systemic lupus erythematosus (SLE). An early diagnosis is an essential tool in the successful management of this complication, requiring aggressive treatment based on vigorous immunosuppression and broad-spectrum antibiotic. We describe here a case of alveolar hemorrhage associated with glomerulonephritis as the open presentation in a patient with SLE.
    Subject(s): systemic lupus erythematosus ; lung disease ; respiratory insufficiency ; alveolar hemorrhage ; Case Report
    ISSN: 0034-6233
    E-ISSN: 2084-9834
    Source: PubMed Central
    Source: © ProQuest LLC All rights reserved〈img src="https://exlibris-pub.s3.amazonaws.com/PQ_Logo.jpg" style="vertical-align:middle;margin-left:7px"〉
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  • 5
    Language: English
    In: Leukemia, 2009, Vol.23 (4), p.708-711
    Description: Shwachman-Diamond syndrome (SDS) is an autosomal recessive disorder, characterized by exocrine pancreatic insufficiency, skeletal abnormalities and bone marrow (BM) dysfunction with an increased risk to develop myelodysplastic syndrome and/or acute myeloid leukaemia (MDS/AML). SDS is caused, in nearly 90% of cases, by two common mutations (that is, c.183_184TA〉CT and c.258+2T〉C) in exon 2 of the SBDS gene, localized on chromosome 7. Clonal chromosome anomalies are often found in the BM of SDS patients; the most frequent is an isochromosome for long arms of chromosome 7, i(7)(q10). We studied eight patients with SDS carrying the i(7)(q10) who were compound heterozygotes for SBDS mutations. By assessing the parental origin of the i(7)(q10) using microsatellite analysis, we inferred from the results which mutation was present in double dose in the isochromosome. We demonstrate that in all cases the i(7)(q10) carries a double dose of the c.258+2T〉C, and we suggest that, as the c.258+2T〉C mutation still allows the production of some amount of normal protein, this may contribute to the low incidence of MDS/AML in this subset of SDS patients.
    Subject(s): Hematologic and hematopoietic diseases ; Other diseases. Hematologic involvement in other diseases ; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis ; Biological and medical sciences ; Medical sciences ; Humans ; Child, Preschool ; Infant ; Leukemia, Myeloid, Acute - etiology ; Isochromosomes ; Chromosomes, Human, Pair 7 ; Myelodysplastic Syndromes - etiology ; Syndrome ; Proteins - genetics ; Young Adult ; Adolescent ; Heterozygote ; Mutation ; Child ; Complications and side effects ; Genetic disorders ; Research ; Gene mutations ; Health aspects ; Index Medicus
    ISSN: 0887-6924
    E-ISSN: 1476-5551
    Source: Nature Open Access
    Source: Academic Search Ultimate
    Source: Alma/SFX Local Collection
    Source: © ProQuest LLC All rights reserved〈img src="https://exlibris-pub.s3.amazonaws.com/PQ_Logo.jpg" style="vertical-align:middle;margin-left:7px"〉
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  • 6
    Language: English
    In: Allergy (Copenhagen), 2003-01, Vol.58 (1), p.38-45
    Description: Background: The diagnosis of allergic disease is performed by skin prick tests (SPT) or through the demonstration of specific IgE in a blood sample via an in vitro test. The measurement of IgE concentration against allergens provides critical information in clinical allergy. Standardized and reproducible methods contribute to the quality of diagnosis and treatment of allergic disease. Methods: In this study we evaluated the performance of a new specific IgE method, developed by ALK-Abellò for Bayer Diagnostics to run on their ADVIA Centaur immunoassay system. One hundred and fifty-one children with allergic diseases (both food and inhalant allergies) were tested for specific IgE (sIgE) via SPT and in vitro tests (UniCAP system, Pharmacia, and ADVIA Centaur immunoassay system, Bayer Diagnostics) and the test results were correlated with the clinical data. Results: Statistical analysis revealed no significant difference between the two in vitro tests compared with clinical history. The sensitivities and specificities are similar, but the UniCAP system method has higher sensitivity. In the children with cow's milk allergy, the UniCAP system has sensitivity of 91% and specificity of 70%; the ADVIA Centaur immunoassay has sensitivity of 82% and specificity of 74%. In hen's egg allergy, UniCAP system has 94% sensitivity and 64% specificity, and the ADVIA Centaur system has 88% sensitivity and 52% specificity. In inhalant allergies, the two methods show statistically similar performances for both grass pollen allergies (UniCAP sensitivity 100%, specificity 73%; ADVIA Centaur sensitivity 95%, specificity 79%) and in the dust mites allergies (UniCAP sensitivity 91%, specificity 62%; ADVIA Centaur sensitivity 86%, specificity 64%). In cat allergies, the systems showed equivalent results (UniCAP sensitivity 100%, specificity 71%; ADVIA Centaur sensitivity 100%, specificity 70%). Using the UniCAP system, the geometric mean of sIgE values in children with clinical allergy is significantly higher than in sensitized ones. The ADVIA Centaur system shows a similar trend with the exclusion of cow's milk and Dermatophagoides farinae allergens. With this last method the mean value of sIgE is higher in sensitized than in symptomatic children. Conclusion: The new ADVIA Centaur method compares favorably with the results obtained on the UniCAP system. If other studies continue to confirm this data, then the advantages are numerous: the use of only a small quantity of serum (25 µl per allergen), rapid turnaround time, minimal hands-on time, and no interference from IgG.
    Subject(s): food allergens ; IgE assay ; UNICAP® System ; inhalant allergens ; ADVIA Centaur ; Immunopathology ; Biological and medical sciences ; Allergic diseases ; Respiratory and ent allergic diseases ; Medical sciences ; Allergens - adverse effects ; Predictive Value of Tests ; Humans ; Milk Hypersensitivity - diagnosis ; Child, Preschool ; Mites ; Egg Hypersensitivity - diagnosis ; Infant ; Male ; Pollen - adverse effects ; Milk Hypersensitivity - immunology ; Immunoglobulin E - blood ; Egg Hypersensitivity - immunology ; Child Welfare ; Immunoglobulin E - immunology ; Antibody Specificity - immunology ; Hypersensitivity - etiology ; Sensitivity and Specificity ; Hypersensitivity - diagnosis ; Female ; Child ; Egg Hypersensitivity - blood ; Cats ; Immunization ; Skin Tests ; Milk Hypersensitivity - blood ; Respiratory Hypersensitivity - immunology ; Respiratory Hypersensitivity - blood ; Animals ; Respiratory Hypersensitivity - diagnosis ; Adolescent ; Hypersensitivity - blood ; Index Medicus
    ISSN: 0105-4538
    E-ISSN: 1398-9995
    Source: Wiley Online Library All Backfiles
    Source: Alma/SFX Local Collection
    Source: © ProQuest LLC All rights reserved〈img src="https://exlibris-pub.s3.amazonaws.com/PQ_Logo.jpg" style="vertical-align:middle;margin-left:7px"〉
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  • 7
    Language: English
    In: Journal of Neurophysiology, 2000-03-01, Vol.83 (3), p.1338-1345
    Description: Interstitial ionic shifts that accompany ouabain-induced spreading depression (SD) were studied in rat hippocampal and cortical slices in the presence and absence of extracellular Ca2+. A double-barreled ion-selective microelectrode specific for H+, K+, Na+, or Ca2+ was placed in the CA1 stratum radiatum or midcortical layer. Superfusion of 100 μM ouabain caused a rapid, negative, interstitial voltage shift (2–10 mV) after 3–5 min. The negativity was accompanied by a rapid alkaline transient followed by prolonged acidosis. In media containing 3 mM Ca2+, the alkalosis induced by ouabain averaged 0.07 ± 0.01 unit pH. In media with no added Ca2+ and 2 mM EGTA, the alkaline shift was not significantly different (0.09 ± 0.02 unit pH). The alkaline transient was unaffected by inhibiting Na+-H+ exchange with ethylisopropylamiloride (EIPA) or by blocking endoplasmic reticulum Ca2+ uptake with thapsigargin or cyclopiazonic acid. Alkaline transients were also observed in Ca2+-free media when SD was induced by microinjecting high K+. The late acidification accompanying ouabain-induced SD was significantly reduced in Ca2+-free media and in solutions containing EIPA. The ouabain-induced SD was associated with a rapid but relatively modest increase in [K+]o. In the presence of 3 mM external Ca2+, the mean peak elevation of [K+]o was 12 ± 0.62 mM. In Ca2+-free media, the elevation of [K+]o had a more gradual onset and reached a significantly larger peak value, which averaged 22 ± 1.1 mM. The decrease in [Na+]o that accompanied ouabain-induced SD was somewhat greater. The [Na+]o decreased by averages of 40 ± 7 and 33 ± 3 mM in Ca2+ and Ca2+-free media, respectively. In media containing 1.2 mM Ca2+, ouabain-induced SD was associated with a substantial decrease in [Ca2+]o that averaged 0.73 ± 0.07 mM. These data demonstrate that in comparison with conventional SD, ouabain-induced SD exhibits ion shifts that are qualitatively similar but quantitatively diminished. The presence of external Ca2+ can modulate the phenomenon but is irrelevant to the generation of the SD and its accompanying alkaline pH transient. Significance of these results is discussed in reference to the propagation of SD and the generation of interstitial pH changes.
    Subject(s): Potassium - metabolism ; Cations - metabolism ; Bicarbonates - pharmacology ; Enzyme Inhibitors - pharmacology ; Extracellular Space - drug effects ; Rats ; Cardiotonic Agents - pharmacology ; Hippocampus - drug effects ; Potassium - pharmacology ; Rats, Sprague-Dawley ; Cortical Spreading Depression - drug effects ; Extracellular Space - metabolism ; Animals ; Calcium - physiology ; Egtazic Acid - pharmacology ; Cerebral Cortex - drug effects ; In Vitro Techniques ; Ouabain - pharmacology ; Sodium-Hydrogen Exchangers - antagonists & inhibitors ; Hydrogen-Ion Concentration ; Index Medicus
    ISSN: 0022-3077
    E-ISSN: 1522-1598
    Source: HighWire Press (Free Journals)
    Source: © ProQuest LLC All rights reserved〈img src="https://exlibris-pub.s3.amazonaws.com/PQ_Logo.jpg" style="vertical-align:middle;margin-left:7px"〉
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  • 8
    Language: English
    In: Leukemia, 2004-08, Vol.18 (8), p.1427-1429
    Subject(s): Hematologic and hematopoietic diseases ; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis ; Biological and medical sciences ; Medical sciences ; Leukemia, Myeloid - epidemiology ; Acute Disease ; Humans ; Leukemia, Myeloid - genetics ; Child, Preschool ; Infant ; Male ; Molecular Epidemiology ; Gene Rearrangement ; Adolescent ; Survival Analysis ; Adult ; Female ; Italy - epidemiology ; Cytogenetic Analysis ; Leukemia, Myeloid - mortality ; Child ; Index Medicus
    ISSN: 0887-6924
    E-ISSN: 1476-5551
    Source: Nature Open Access
    Source: Academic Search Ultimate
    Source: Alma/SFX Local Collection
    Source: © ProQuest LLC All rights reserved〈img src="https://exlibris-pub.s3.amazonaws.com/PQ_Logo.jpg" style="vertical-align:middle;margin-left:7px"〉
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  • 9
    Language: English
    In: Haematologica (Roma), 2014-08, Vol.99 (8), p.e127-e129
    Subject(s): Hematopoietic Stem Cell Transplantation - trends ; Survival Rate - trends ; Follow-Up Studies ; Induction Chemotherapy - trends ; Humans ; Induction Chemotherapy - methods ; Infant ; Male ; Treatment Outcome ; Hematopoietic Stem Cell Transplantation - mortality ; Leukemia, Myeloid, Acute - mortality ; Female ; Italy - epidemiology ; Hematopoietic Stem Cell Transplantation - methods ; Societies, Medical - standards ; Child ; Induction Chemotherapy - mortality ; Infant, Newborn ; Leukemia, Myeloid, Acute - therapy ; Index Medicus ; hematopoietic stem cell transplantation ; Online Only ; infants ; intensive chemotherapy ; acute myeloid leukemia
    ISSN: 0390-6078
    E-ISSN: 1592-8721
    Source: HighWire Press (Free Journals)
    Source: PubMed Central
    Source: © ProQuest LLC All rights reserved〈img src="https://exlibris-pub.s3.amazonaws.com/PQ_Logo.jpg" style="vertical-align:middle;margin-left:7px"〉
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  • 10
    Language: English
    In: Bone marrow transplantation (Basingstoke), 2005-03, Vol.35 (S1), p.S89-S92
    Description: Aplastic anemia (AA) is a rare disease with a major autoimmune pathogenetic component. CTLA4 is a T-lymphocyte surface molecule involved in the maintenance of immune tolerance. Some polymorphisms associated with a reduced expression of CTLA4, and thus presumably with increased tendency to autoimmunity, have been associated with various autoimmune diseases. In this study, we evaluated the distribution of the low expression polymorphisms -318C 〉 T and 49A 〉 G of CTLA4 in a population of 67 patients with acquired AA and in 100 normal controls. There was no difference in the distribution of the tested polymorphism between patients and controls and, within the patient group, between those who responded to immunosuppression vs those who did not respond. This study indicates that the polymorphisms -318C 〉 T and 49A 〉 G of CTLA4 do not affect the risk of developing AA and do not influence the response to immunosuppression.
    Subject(s): Promoter Regions, Genetic ; Genetic Predisposition to Disease ; Humans ; Middle Aged ; Risk Factors ; Gene Expression Regulation ; Child, Preschool ; European Continental Ancestry Group ; Exons - genetics ; Infant ; Male ; Case-Control Studies ; CTLA-4 Antigen ; Adolescent ; Adult ; Female ; Antigens, Differentiation - genetics ; Polymorphism, Single Nucleotide ; Child ; Anemia, Aplastic - genetics ; Antigens, CD ; Care and treatment ; Usage ; Aplastic anemia ; Physiological aspects ; Genetic aspects ; Research ; T cells ; Health aspects ; Risk factors ; Genetic polymorphisms ; Index Medicus
    ISSN: 0268-3369
    E-ISSN: 1476-5365
    Source: Nature Open Access
    Source: Academic Search Ultimate
    Source: Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals
    Source: Alma/SFX Local Collection
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