1,721,741 research outputs found
Guidelines for diagnosis and management of beta-thalassemia intermedia
No full textBeta-thalassemia intermedia (β-TI) is a genetic variant of beta-thalassemias with a clinical disorder whose severity falls between thalassemia minor and thalassemia major. Different genetic defects are involved in this disorder and, based on severity of disease, clinical complications like skeletal deformities and growth retardation, splenomegaly, extramedullary hematopoiesis, heart failure, and endocrine disorders may be present in untreated patients. Precise diagnosis and management are essential in these patients for prevention of later clinical complications. Diagnosis of TI is based on clinical and laboratory data. There are some treatment strategies like modulation of gamma-globulin chain production with hydroxyurea or other drugs, transfusion, splenectomy, and stem cell transplantation. Iron chelation therapy is also needed in many of these patients even if they are not transfused. The aim of this manuscript is to review the clinical manifestations, complications, genetic defects, and unmet treatments needs in TI.Aessopos A, 2007, ATHEROSCLEROSIS, V191, P427, DOI 10.1016-j.atherosclerosis.2006.04.015; Aessopos A, 2007, TRANSFUSION, V47, P792, DOI 10.1111-j.1537-2995.2007.01192.x; Amoozgar H, 2011, EUR J HAEMATOL, V85, P549; Atichartakarn V, 2003, INT J HEMATOL, V78, P139, DOI 10.1007-BF02983382; Cadili A, 2008, AM J MED, V121, P371, DOI 10.1016-j.amjmed.2008.02.014; CAMASCHELLA C, 1995, HAEMATOLOGICA, V80, P58; Camaschella C, 1997, AM J HEMATOL, V55, P83, DOI 10.1002-(SICI)1096-8652(199706)55:283::AID-AJH63.3.CO;2-M; Cappellini MD, 2005, SEMIN HEMATOL, V42, pS19, DOI 10.1053-j.seminhematol.2005.01.001; Cappellini MD, 2000, BRIT J HAEMATOL, V111, P467, DOI 10.1046-j.1365-2141.2000.02376.x; Derakhshan A, 2008, SAUDI J KIDNEY DIS T, V19, P206; De Sanctis V, 1998, J PEDIATR ENDOCR MET, V11, P965; Dixit A, 2005, ANN HEMATOL, V84, P441, DOI 10.1007-s00277-005-1026-4; Elalfy MS, 2013, EUR J HAEMATOL, V91, P522, DOI 10.1111-ejh.12182; El Rassi F, 2008, PEDIATR ANN, V37, P322; Galanello R, 1998, ANN NY ACAD SCI, V850, P325, DOI 10.1111-j.1749-6632.1998.tb10489.x; Gamberini MR1, 2004, PEDIAT ENDOCRINOL S2, P319; Gladwin MT, 2003, NAT MED, V9, P496, DOI 10.1038-nm0503-496; Gladwin MT, 2008, NEW ENGL J MED, V359, P2254, DOI 10.1056-NEJMra0804411; Haddad A, 2014, TURK J HEMATOL, V31, P5, DOI 10.4274-Tjh.2014.0032; Haghpanah S, 2014, HEMATOLOGY, V19, P187, DOI 10.1179-1607845413Y.0000000121; Harmatz P, 2008, HAEMATOL-HEMATOL J, V93, P1247, DOI 10.3324-haematol.12352; Karimi M, 2012, INT J HEMATOL, V95, P51, DOI 10.1007-s12185-011-0985-6; Karimi M, 2012, ANN HEMATOL, V91, P1833, DOI 10.1007-s00277-012-1527-x; Karimi M, 2009, EUR J HAEMATOL, V82, P213, DOI 10.1111-j.1600-0609.2008.01192.x; Karimi M, 2012, HEMATOLOGY, V17, P122, DOI 10.1179-102453312X13221316477778; Karimi M, 2010, EUR J HAEMATOL, V84, P52, DOI 10.1111-j.1600-0609.2009.01356.x; Karimi M, 2014, HEMATOLOGY; Karimi M, 2007, INT J LAB HEMATOL, V29, P321, DOI 10.1111-j.1365-2257.2006.00856.x; Karimi M, 2010, THROMB HAEMOSTASIS, V103, P989, DOI 10.1160-TH09-09-0661; Karimi M, 2011, EUR J INTERN MED, V22, P607, DOI 10.1016-j.ejim.2011.05.013; Karimi M, 2008, LANCET, V372, P1436, DOI 10.1016-S0140-6736(08)61590-1; Karimi M, 2008, AM J HEMATOL, V83, P77, DOI 10.1002-ajh.20938; Karimi M, 2005, J PEDIAT HEMATOL ONC, V27, P380, DOI 10.1097-01.mph.0000174386.13109.28; Karimi M, 2010, PEDIATR HEMAT ONCOL, V27, P205, DOI 10.3109-08880011003639952; Mancuso A, 2006, HEMOGLOBIN, V30, P119, DOI 10.1080-03630260500455565; Manfre L, 1999, AM J ROENTGENOL, V173, P1477; Matta BN, 2013, J EUR ACAD DERMATOL; Moorchung N, 2006, HAEMA, V9, P505; Musallam KM, 2011, EUR J HAEMATOL, V87, P73, DOI 10.1111-j.1600-0609.2011.01623.x; Musallam KM, 2011, HAEMATOL-HEMATOL J, V96, P1605, DOI 10.3324-haematol.2011.047852; Musallam KM, 2012, THROMB RES, V130, P695, DOI 10.1016-j.thromres.2012.07.013; Olivieri NF, 1999, NEW ENGL J MED, V341, P99, DOI 10.1056-NEJM199907083410207; Pakbaz Z, 2005, ANN NY ACAD SCI, V1054, P457, DOI 10.1196-annals.1345.059; Pierre T.G., 2005, BLOOD, V105, P855; Rachid H, 2010, EUR SPINE J, V19, P871; Rachmilewitz A, 2011, BLOOD, V118, P3479; Rachmilewitz EA, 1998, ANN NY ACAD SCI, V850, P129, DOI 10.1111-j.1749-6632.1998.tb10470.x; Pantalone GR, 2010, BRIT J HAEMATOL, V150, P245, DOI 10.1111-j.1365-2141.2010.08180.x; Rund D, 2005, NEW ENGL J MED, V353, P1135, DOI 10.1056-NEJMra050436; SPANOS T, 1990, VOX SANG, V58, P50; Taher A, 2006, THROMB HAEMOSTASIS, V96, P488, DOI 10.1160-TH06-05-0267; Taher A, 2006, BLOOD CELL MOL DIS, V37, P12, DOI 10.1016-j.bcmd.2006.04.005; Taher AT, 2013, ANN HEMATOL, V92, P1485, DOI 10.1007-s00277-013-1808-z; Taher AT, 2011, BRIT J HAEMATOL, V152, P512, DOI 10.1111-j.1365-2141.2010.08486.x; Taher AT, 2010, J THROMB HAEMOST, V8, P2152, DOI 10.1111-j.1538-7836.2010.03940.x; Taher AT, 2010, J THROMB HAEMOST, V8, P54, DOI 10.1111-j.1538-7836.2009.03651.x; Taher AT, 2010, BLOOD, V115, P1886, DOI 10.1182-blood-2009-09-243154; Taher AT, 2012, BLOOD REV, V26, pS24, DOI 10.1016-S0268-960X(12)70008-5; Thein SL, 2004, BRIT J HAEMATOL, V124, P264, DOI 10.1046-j.1365-2141.2003.04769.x; Voskaridou E, 2010, BRIT J HAEMATOL, V148, P332, DOI 10.1111-j.1365-2141.2009.07930.x; WEATHERALL D, 1995, MOL MED TODAY, V1, P15, DOI 10.1016-1357-4310(95)80014-X; Weatherall DJ, 2001, J HEMATOL S1, V86, P186; Weatherall DJ, 2001, NAT REV GENET, V2, P245, DOI 10.1038-35066048; Wood JC, 2005, BLOOD, V106, P1460, DOI 10.1182-blood-2004-10-39821
Contemporary approaches to treatment of beta-thalassemia intermedia
No full textBeta-thalassemia intermedia (TI) is associated with a variety of serious clinical complications that require proactive and comprehensive management. These include skeletal deformities and osteopenia, compensatory extramedullary hematopoiesis and tumor formation, progressive splenomegaly, a hypercoagulable state resulting in thromboembolic events and pulmonary hypertension, and increased gastrointestinal iron absorption that often results in nontransfusional iron overload and liver damage. Although TI is generally considered a non-transfusion-dependent thalassemia, transfusion therapy may be an important part of the comprehensive management of this disease. This review describes the current state of the art for medical management of TI, with particular focus on the roles of splenectomy, transfusion, and iron chelation therapy. © 2012 Elsevier Ltd.Aessopos A, 2005, ANN NY ACAD SCI, V1054, P342, DOI 10.1196-annals.1345.041; Aessopos A, 2007, HAEMATOL-HEMATOL J, V92, P658, DOI 10.3324-haematol.10915; Aessopos A, 2001, BLOOD, V97, P3411, DOI 10.1182-blood.V97.11.3411; Amoozgar H, 2011, EUR J HAEMATOL, V87, P61, DOI 10.1111-j.1600-0609.2011.01620.x; [Anonymous], FERR SUMM PROD CHAR; [Anonymous], 2010, EXJADE PACK INS; [Anonymous], EXJADE SUMM PROD CHA; Ataga KI, 2007, BRIT J HAEMATOL, V139, P3, DOI 10.1111-j.1365-2141.2007.06740.x; Atichartakarn V, 2003, INT J HEMATOL, V78, P139, DOI 10.1007-BF02983382; Bisharat N, 2001, J INFECTION, V43, P182, DOI 10.1053-jinf.2001.0904; Borgna-Pignatti C, 2010, ANN NY ACAD SCI, V1202, P214, DOI 10.1111-j.1749-6632.2010.05550.x; Borgna-Pignatti C, 2007, BRIT J HAEMATOL, V138, P291, DOI 10.1111-j.1365-2141.2007.06654.x; Cappellini MD, 2005, SEMIN HEMATOL, V42, pS19, DOI 10.1053-j.seminhematol.2005.01.001; Cappellini MD, 2000, BRIT J HAEMATOL, V111, P467, DOI 10.1046-j.1365-2141.2000.02376.x; COSSU P, 1981, EUR J PEDIATR, V137, P267, DOI 10.1007-BF00443255; Davies JM, 2011, BRIT J HAEMATOL, V155, P308, DOI 10.1111-j.1365-2141.2011.08843.x; Eder AF, 2007, ARCH PATHOL LAB MED, V131, P708; Gardenghi S, 2010, J CLIN INVEST, V120, P4466, DOI 10.1172-JCI41717; Haidar R, 2010, EUR SPINE J, V19, P871, DOI 10.1007-s00586-010-1357-2; Karimi M, 2010, EUR J HAEMATOL, V84, P52, DOI 10.1111-j.1600-0609.2009.01356.x; Karimi M, 2011, EUR J INTERN MED, V22, P607, DOI 10.1016-j.ejim.2011.05.013; Karimi M, 2010, PEDIATR HEMAT ONCOL, V27, P205, DOI 10.3109-08880011003639952; Kontoghiorghes GJ, 2010, HEMOGLOBIN, V34, P251, DOI 10.3109-03630269.2010.486335; Ladis V, 2010, BRIT J HAEMATOL, V151, P504, DOI 10.1111-j.1365-2141.2010.08346.x; Li HH, 2010, NAT MED, V16, P177, DOI 10.1038-nm.2073; Musallam KM, 2011, EUR J HAEMATOL, V87, P73, DOI 10.1111-j.1600-0609.2011.01623.x; Musallam KM, 2011, HAEMATOL-HEMATOL J, V96, P1605, DOI 10.3324-haematol.2011.047852; Novartis Pharmaceutical Corporation, 2010, DESF PACK INS; Porter J, 2011, 16 C EUR HEM ASS JUN; Porter JB, 2001, SEMIN HEMATOL, V38, P63, DOI 10.1053-shem.2001.20145; Rigano P, 2010, BRIT J HAEMATOL, V151, P509, DOI 10.1111-j.1365-2141.2010.08397.x; Sankaran VG, 2010, NEW ENGL J MED, V363, P2258, DOI 10.1056-NEJMcibr1010767; STURGEON P, 1955, BRIT J HAEMATOL, V1, P264, DOI 10.1111-j.1365-2141.1955.tb05509.x; Taher A, 2008, HAEMATOL-HEMATOL J, V93, P1584, DOI 10.3324-haematol.13098; Taher A, 2006, THROMB HAEMOSTASIS, V96, P488, DOI 10.1160-TH06-05-0267; Taher A, 2006, BLOOD CELL MOL DIS, V37, P12, DOI 10.1016-j.bcmd.2006.04.005; Taher A, 2009, BRIT J HAEMATOL, V147, P634, DOI 10.1111-j.1365-2141.2009.07848.x; Taher A, 2002, HEMOGLOBIN, V26, P107, DOI 10.1081-HEM-120005447; Taher AT, 2008, BLOOD REV, V22, P283, DOI 10.1016-j.blre.2008.04.001; Taher AT, 2010, BRIT J HAEMATOL, V150, P486, DOI 10.1111-j.1365-2141.2010.08220.x; Taher AT, 2011, BRIT J HAEMATOL, V152, P512, DOI 10.1111-j.1365-2141.2010.08486.x; Taher AT, 2010, J THROMB HAEMOST, V8, P54, DOI 10.1111-j.1538-7836.2009.03651.x; Taher AT, 2010, BLOOD, V115, P1886, DOI 10.1182-blood-2009-09-243154; Taher ATPJ, 2011, 53 AM SOC HEM ANN M; Treadwell MJ, 2001, SEMIN HEMATOL S1, V38, P77; Voskaridou E, 2010, BRIT J HAEMATOL, V148, P332, DOI 10.1111-j.1365-2141.2009.07930.x9151
Going Beyond Counting First Authors in Author Co-citation Analysis
Full text linkThe present study examines one of the fundamental aspects of author co-citation analysis (ACA) - the way co-citation
counts are defined. Co-citation counting provides the data on which all subsequent statistical analyses and mappings
are based, and we compare ACA results based on two different types of co-citation counting - the traditional type that
only counts the first one among a cited work's authors on the one hand and a non-traditional type that takes into
account the first 5 authors of a cited work on the other hand. Results indicate that the picture produced through this non-traditional author co-citation counting contains more coherent author groups and is therefore considerably clearer. However, this picture represents fewer specialties in the research field being studied than that produced through the traditional first-author co-citation counting when the same number of top-ranked authors is selected and analyzed. Reasons for these effects are discussed
Variations on the Author
Full text link“Variations on the Author” discusses two of Eduardo Coutinho’s recent films (Um Dia na Vida, from 2010, and Últimas Conversas, posthumously released in 2015) and their contribution to the general question of documentary authorship. The director’s filmography is characterized by a consistent yet self-effacing form of authorial self-inscription: Coutinho often features as an interviewer that rather than express opinions propels discourses; an interviewer that is good at listening. This mode of self-inscription characterizes him as an author who is not expressive but who is nonetheless markedly present on the screen. In Um Dia na Vida, however, Coutinho is completely absent form the image, while Últimas Conversas, on the contrary, includes a confessional prologue that moves the director from the margins to the center of his films. This article examines the ways in which these works stand out in the filmography of a director who offers new insights into the notion of cinematic authorship
Aeroelastic analysis of rectangular plates coupled to sloshing fluid
No full textIn this study, an aeroelastic model that accounts for the fluid–structure interaction is developed to investigate vibration and stability of rectangular plates in contact with sloshing fluid on one side and under supersonic aeroelastic load on the other side. The fifth-order shear deformation theory, which is capable of considering rotary inertia and transverse shear stress, is employed to model the structure. Bulging and sloshing modes of the incompressible, inviscid and irrotational fluid are obtained with satisfying Laplace’s equation and fluid boundary conditions. The first-order piston theory is applied to consider the supersonic aeroelastic load. On the basis of Hamilton’s principle, the governing equations of the coupled fluid–structure system are derived and discretized using the Galerkin method. Numerical results for specific cases are compared with available results in the literature and an excellent agreement is observed. In the discussion section, influences of various parameters such as the dimensions of the fluid domain, plate dimensions and aerodynamic parameters on the natural frequencies and flutter behavior are studied
Appropriate Similarity Measures for Author Cocitation Analysis
Full text linkWe provide a number of new insights into the methodological discussion about author cocitation analysis. We first argue that the use of the Pearson correlation for measuring the similarity between authors’ cocitation profiles is not very satisfactory. We then discuss what kind of similarity measures may be used as an alternative to the Pearson correlation. We consider three similarity measures in particular. One is the well-known cosine. The other two similarity measures have not been used before in the bibliometric literature. Finally, we show by means of an example that our findings have a high practical relevance.information science;Pearson correlation;cosine;similarity measure;author cocitation analysis
FIGURE 1 in Paragigagnathus iraniensis n. sp. (Acari: Phytoseiidae) from Western Iran
No full textFIGURE 1: Paragigagnathus iraniensis n. sp. (Female): A – Dorsal view of idiosoma; B – Dorsal seta J5; C – Chelicera; D – Tectum; E – Ventral view of idiosoma; F – Spermatheca; G – Genu II; H – Basitarsus IV.Published as part of Khanjani, M., Karimi, M., Asali Fayaz, B. & Ueckermann, E.A., 2016, Paragigagnathus iraniensis n. sp. (Acari: Phytoseiidae) from Western Iran, pp. 195-201 in Acarologia 56 (2) on page 197, DOI: 10.1051/acarologia/20162246, http://zenodo.org/record/466738
Dispelling the Myths Behind First-author Citation Counts
Full text linkWe conducted a full-scale evaluative citation analysis study of scholars in the XML research field to explore just how different from each other author rankings resulting from different citation counting methods actually are, and to demonstrate the capability of emerging data and tools on the Web in supporting more realistic citation counting methods. Our results contest some common arguments for the continued
use of first-author citation counts in the evaluation of scholars, such as high correlations between author rankings by first-author citation counts and other citation
counting methods, and high costs of using more realistic citation counting methods that are not well-supported by the ISI databases. It is argued that increasingly available digital full text research papers make it possible for citation analysis studies to go beyond what the ISI databases have directly supported and to employ more
sophisticated methods
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