1,375 research outputs found
Does absolute excess of alpha chains compromise the benefit of splenectomy in patients with thalassemia intermedia?
No full text[No abstract available]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, 2010, ANN NY ACAD SCI, V1202, P231, DOI 10.1111-j.1749-6632.2010.05548.x; Cappellini MD, 2000, BRIT J HAEMATOL, V111, P467, DOI 10.1046-j.1365-2141.2000.02376.x; Harteveld CL, 2008, BLOOD CELL MOL DIS, V40, P312, DOI 10.1016-j.bcmd.2007.11.006; Harteveld CL, 2005, J MED GENET, V42, P922, DOI 10.1136-jmg.2005.033597; MANNU F, 1995, BLOOD, V86, P2014; SAMPIETRO M, 1983, BRIT J HAEMATOL, V55, P709, DOI 10.1111-j.1365-2141.1983.tb02854.x; SHINAR E, 1990, SEMIN HEMATOL, V27, P70; Sollaino MC, 2009, HAEMATOL-HEMATOL J, V94, P1445, DOI 10.3324-haematol.2009.005728; 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.x11
Long-term experience with deferasirox (ICL670), a once-daily oral iron chelator, in the treatment of transfusional iron overload
No full textBackground: Chronic iron overload from frequent blood transfusions to treat patients with severe anaemias leads to significant morbidity and mortality. While deferoxamine, the current standard of care, is an effective iron chelator, it requires subcutaneous infusion for 8-12 h- day, 5-7 days-week. This regimen is problematic and impacts significantly on patients' daily life. Objective: To evaluate the efficacy and tolerability of deferasirox, a once-daily oral iron chelator. Method: To review the available data reported in peer-reviewed journals (using PubMed) and at medical conferences. Results-conclusions: Deferasirox is effective in reducing or maintaining iron burden in patients with transfusion-dependent anaemias. As deferasirox is orally administered, the inconvenience of parenteral administration with deferasirox is avoided. Deferasirox improves patient satisfaction and is expected to improve compliance with iron chelation therapy. © 2008 Informa UK Ltd.Abdelrazik N, 2007, HEMATOLOGY, V12, P577, DOI 10.1080-10245330701521614; Aydinok Y, 1999, ACTA HAEMATOL-BASEL, V102, P17, DOI 10.1159-000040962; Borgna-Pignatti C, 1998, ANN NY ACAD SCI, V850, P227, DOI 10.1111-j.1749-6632.1998.tb10479.x; BRITTENHAM GM, 1994, NEW ENGL J MED, V331, P567, DOI 10.1056-NEJM199409013310902; Cappellini MD, 2007, CLIN THER, V29, P909, DOI 10.1016-j.clinthera.2007.05.007; CAPPELLINI MD, 2007, BLOOD, V110; Cappellini MD, 2006, BLOOD, V107, P3455, DOI 10.1182-blood-2005-08-3430; Ceci A, 2002, BRIT J HAEMATOL, V118, P330, DOI 10.1046-j.1365-2141.2002.03554.x; COHEN AR, 2006, HEMATOLOGY AM SOC HE, V42; Cohen AR, 2003, BLOOD, V102, P1583, DOI 10.1182-blood-2002-10-3280; Cunningham MJ, 2004, BLOOD, V104, P34, DOI 10.1182-blood-2003-09-3167; DAAR S, 2006, BLOOD, V108; Delea TE, 2007, TRANSFUSION, V47, P1919, DOI 10.1111-j.1537-2995.2007.01416.x; ELEFTHERIOU P, 2006, HAEMATOLOGICA S1, V91; Gabutti V, 1996, ACTA HAEMATOL-BASEL, V95, P26; Galanello R, 2006, HAEMATOL-HEMATOL J, V91, P1241; Galanello R, 2003, J CLIN PHARMACOL, V43, P565, DOI 10.1177-0091270003253350; Galanello R, 2006, HAEMATOL-HEMATOL J, V91, P1343; Gattermann N, 2005, HEMATOL ONCOL CLI S1, V19, P18; Gattermann N, 2007, LEUKEMIA RES, V31, pS109, DOI 10.1016-S0145-2126(07)70199-6; Glickstein H, 2006, BLOOD, V108, P3195, DOI 10.1182-blood-2006-05-020867; Karnon J, 2008, CURR MED RES OPIN, V24, P1609, DOI 10.1185-03007990802077442; Leitch HA, 2007, LEUKEMIA RES, V31, pS7, DOI 10.1016-S0145-2126(07)70460-5; MAHINDRA A, 2007, BLOOD, V110; Nick H, 2003, CURR MED CHEM, V10, P1065, DOI 10.2174-0929867033457610; Nick H, 2007, SEMIN HEMATOL, V44, pS12, DOI 10.1053-j.seminhematol.2007.03.005; Nick H, 2002, ADV EXP MED BIOL, V509, P185; Nisbet-Brown E, 2003, LANCET, V361, P1597, DOI 10.1016-S0140-6736(03)13309-0; *NOV PHARM AG, 2006, EXJ DEF SUMM PROD CH; *NOV PHARM AG, 2006, EXJ BAS PRESCR INF; Olivieri NF, 1999, NEW ENGL J MED, V341, P99, DOI 10.1056-NEJM199907083410207; OLIVIERI NF, 1994, NEW ENGL J MED, V331, P574, DOI 10.1056-NEJM199409013310903; Olivieri NF, 1997, BLOOD, V89, P739; Otto-Duessel M, 2007, EXP HEMATOL, V35, P1069, DOI 10.1016-j.exphem.2007.04.001; PIETRANGELO A, 2007, BLOOD, V110; Piga A, 2006, HAEMATOL-HEMATOL J, V91, P873; PLATZBECKER U, 2007, IMPACT TRANSFUSION D, V110; Porter JB, 2005, BLOOD, V106; PORTER JB, 2007, BLOOD, V110; Porter J, 2008, EUR J HAEMATOL, V80, P168, DOI 10.1111-j.1600-0609.2007.00985.x; Rose C, 2007, BLOOD, V110; ROSE C, 2006, HAEMATOLOGICA S1, V91; TAHER A, 2007, HAEMATOLOGICA S1, V92; Vichinsky E, 2007, BRIT J HAEMATOL, V136, P501, DOI 10.1111-j.1365-2141.2006.06455.x; Vichinsky E, 2008, ACTA HAEMATOL-BASEL, V119, P133, DOI 10.1159-000125550; Vichinsky E, 2008, AM J HEMATOL, V83, P398, DOI 10.1002-ajh.21119; Wood J, 2007, BLOOD, V110; Wood JC, 2006, TRANSL RES, V148, P272, DOI 10.1016-j.trsl.2006.05.005; 1999, FERRIPROX SUMMARY PR18171
Deferasirox (Exjade®) for the treatment of iron overload
No full textDeferasirox is a once-daily oral iron chelator with established dose-dependent efficacy in both adult and pediatric patients with transfusional iron overload. The clinical development program has demonstrated the efficacy of deferasirox for up to 4.5 years of treatment in patients with various underlying anemias, including β-thalassemia, myelodysplastic syndromes, sickle cell disease, aplastic anemia, and other rare anemias. In addition to reducing key indicators of total body iron levels (serum ferritin, liver iron concentration, and toxic labile plasma iron), deferasirox has also demonstrated the ability to remove cardiac iron and prevent future cardiac iron accumulation. Emerging long-term data confirm the tolerability profile of deferasirox, and data on patient compliance render deferasirox a suitable therapeutic option for patients with chronic conditions requiring ongoing iron chelation therapy. Data continue to accumulate in a wide range of patient groups, including those with non-transfusion-dependent anemias such as hereditary hemochromatosis. Copyright © 2009 S. Karger AG, Basel.Adams RJ, 1998, NEW ENGL J MED, V339, P5, DOI 10.1056-NEJM199807023390102; Adams RJ, 2005, NEW ENGL J MED, V353, P2769; Bennett JM, 2008, AM J HEMATOL, V83, P858, DOI 10.1002-ajh.21269; Beutler E, 2007, BLOOD CELL MOL DIS, V39, P140, DOI 10.1016-j.bcmd.2007.03.009; Cappellini M. D., 2008, BLOOD, V112, P3875; Cappellini MD, 2007, CLIN THER, V29, P909, DOI 10.1016-j.clinthera.2007.05.007; Cappellini MD, 2008, HAEMATOL-HEMATOL J, V93, P336; CAPPELLINI MD, 2008, BLOOD, V112, P3878; Cappellini MD, 2007, BLOOD, V110, p816A; Cappellini MD, 2008, BLOOD, V112, P5411; Cappellini MD, 2006, BLOOD, V107, P3455, DOI 10.1182-blood-2005-08-3430; Claster S, 2003, BRIT MED J, V327, P1151, DOI 10.1136-bmj.327.7424.1151; Cohen AR, 2008, BLOOD, V111, P583, DOI 10.1182-blood-2007-08-109306; COSSU P, 1981, EUR J PEDIATR, V137, P267, DOI 10.1007-BF00443255; Daar S, 2009, EUR J HAEMATOL, V82, P454, DOI 10.1111-j.1600-0609.2008.01204.x; Damanhouri G, 2008, BLOOD, V112, P5409; *DIAM BLACKF AN SU, DIAM BLACKF AN SYNDR; Esposito BP, 2003, BLOOD, V102, P2670, DOI 10.1182-blood-2003-03-0807; Galanello R, 2003, J CLIN PHARMACOL, V43, P565, DOI 10.1177-0091270003253350; Galanello R, 2006, HAEMATOL-HEMATOL J, V91, P1343; Garbowski M, 2008, BLOOD, V112, P116; Gattermann N, 2005, HEMATOL ONCOL CLI S1, V19, P1; Gattermann N, 2008, BLOOD, V112, P633; Goubran HA, 2007, BLOOD, V110, p676A; GREENBERG PL, 2008, BLOOD, V112, P5083; Hellstrom-Lindberg E, 2005, SEMIN HEMATOL, V42, pS10, DOI 10.1053-j.seminhematol.2005.01.002; Ibrahim AS, 2007, J CLIN INVEST, V117, P2649, DOI 10.1172-JCI32338; International Agranulocytosis and Aplastic Anemia Study, 1986, JAMA, V256, P1749; Jastaniah W, 2008, PEDIATR BLOOD CANCER, V50, P319, DOI 10.1002-pbe.21260; Kostler E, 2005, EXPERT OPIN PHARMACO, V6, P377, DOI 10.1517-14656566.6.3.377; LEE JW, 2008, BLOOD, V112, P439; LIST AF, 2008, BLOOD, V112, P634; Marsh JCW, 2003, BRIT J HAEMATOL, V123, P782, DOI 10.1046-j.1365-2141.2003.04721.x; MIN YH, 2008, BLOOD, V112, P3649; National Comprehensive Cancer Network, NCCN CLIN PRACT GUID; Nisbet-Brown E, 2003, LANCET, V361, P1597, DOI 10.1016-S0140-6736(03)13309-0; *NOV PHARM CORP, EXJ DEF PRESCR INF N; OLIVIERI NF, 1994, NEW ENGL J MED, V331, P574, DOI 10.1056-NEJM199409013310903; Olivieri NF, 1997, BLOOD, V89, P739; OLIVIERI NF, 1992, BLOOD, V79, P2741; Origa R, 2008, HAEMATOL-HEMATOL J, V93, P1095, DOI 10.3324-haematol.12484; PENNELL D, 2008, BLOOD, V112, P3874; Pennell DJ, 2008, BLOOD, V112, P3873; Pietrangelo A, 2007, BLOOD, V110, p788A; PIETRANGELO A, 2009, J HEPATOL UNPUB; Piga A, 2006, HAEMATOL-HEMATOL J, V91, P873; Piga A, 2008, BLOOD, V112, P3883; PIGA A, 2008, BLOOD, V112, P5413; PORTER JB, 2008, BLOOD, V112, P1048; PORTER JB, 2008, BLOOD, V112, P1419; Porter JB, 2008, BLOOD, V112, P3881; Porterfield D, 2005, WEED TECHNOL, V19, P1, DOI 10.1614-WT-02-006; Porter J, 2008, EUR J HAEMATOL, V80, P168, DOI 10.1111-j.1600-0609.2007.00985.x; Taher A, 2009, EUR J HAEMATOL, V82, P458, DOI 10.1111-j.1600-0609.2009.01228.x; Taher A, 2006, BLOOD CELL MOL DIS, V37, P12, DOI 10.1016-j.bcmd.2006.04.005; Vichinsky E, 2007, BRIT J HAEMATOL, V136, P501, DOI 10.1111-j.1365-2141.2006.06455.x; Vichinsky E, 2005, AM J HEMATOL, V80, P70, DOI 10.1002-ajh.20402; VICHINSKY E, 2008, BLOOD, V112, P1420; Vichinsky E, 2008, ACTA HAEMATOL-BASEL, V119, P133, DOI 10.1159-000125550; Vichinsky E, 2008, AM J HEMATOL, V83, P398, DOI 10.1002-ajh.21119; Wood JC, 2008, BLOOD, V112, P3882; Wood JC, 2006, TRANSL RES, V148, P272, DOI 10.1016-j.trsl.2006.05.005; ZURLO MG, 1989, LANCET, V2, P2726222
Thalassemia intermedia
No full textThalassemia intermedia is a clinical definition applied to patients whose clinical phenotype is milder than that of thalassemia major. Criteria used to define thalassemia intermedia including age at presentation, hemoglobin or fetal hemoglobin levels and transfusion independence, are unsatisfactory. The possibility of typing the molecular defect allows a retrospective analysis of patients and offers a new tool for the diagnosis of thalassemia intermedia. Nevertheless, because of several factors that interact in the disease expression, the β-genotype alone is not predictive of the phenotype in all cases. Although benign, the clinical course of thalassemia intermedia is characterized by several complications that can be prevented by an accurate follow-up. The conventional treatment of thalassemia intermedia remains controversial; it is hoped that recent advances in the pharmacological manipulation of hemoglobin switching will offer a therapeutic option in the future, at least to selected patients
Ring rolling with flat dies: An analytical method to optimize geometry, time or energy
No full textRing rolling process is a plastic deformation process used in the production of seamless rings having diameters in a range of meters. During production, rings simultaneously undergo to a width and height reduction and a diameter expansion, however located in different ring cross sections as a function of idle, axial and driving rolls action. Despite roll motion law could be set independently, their combination influences ring accuracy, production time and energy required. Accordingly, based on the results of simulation plan, the authors present an analytical model able to optimize rolls motion laws as a function of required geometrical accuracy and minimizing production time and energy. The analysis of these latter, allowed the definition of their regression models as a function of Idle roll feed rate and ring rotational speed. These models were then expressed as a function of selected geometrical precision parameter and a weighting factor, for balancing time and energy of the process. Afterwards, geometry and energy models were arranged to define an objective function that, once minimized, allowed to assess the optimized values of the process parameters able to achieve the selected ring precision while decreasing process time and energy. The proposed methodology was applied on different combinations of geometrical and weighting factors, and the resulting optimized conditions were tested by finite element simulations. The good comparison between modeled and simulated ring accuracy and energy, demonstrates the model efficacy in the selection of proper motion laws of ring rolling equipment
An analytical micro-milling force model based on the specific cutting pressure-feed dependence, in presence of ploughing and tool run-out effects
No full textThe growing request of extremely accurate small parts in the last decades led to the disruptive employment of micro-milling processes. Despite conventional milling knowledge is widespread in the industrial field, its application at micro-level remains a big effort because of phenomena usually neglectable at macro-scale, such as the so-called size-effect and tool run-out. Therefore, to correctly evaluate micro-milling forces, a model able to consider these effects is mandatory. In this paper a mechanistic model for predicting cutting forces in micro-milling of Ti-6Al-4V specimens, considering ploughing and tool run-out effects, is presented. The proposed model concerns the introduction of a dedicated coefficient for considering ploughing and shearing effects on tangential and radial components of the cutting forces. Differently from the previous scholars, the coefficients related to ploughing regime have been considered as a function of the specific cutting pressure. In turn, this latter has been characterized by a regression power law dependent on the feed rate since, in this regime, it has a significant influence. The good agreement between analytical and experimental results enforces the proposed model capabilities
An Innovative Shape Memory Actuator
No full textThe work describes a NiTi linear actuator. This material is able to realize a contraction with heating produced through Joule effect. Then a cooling of the active device is realized with forced air. Finally the lengthening is realized with another active element. The particular structure of the geometry allows for an increment of reliability, because the electrical connections are mechanically stabilized and the active elements are compelled to avoid undesired electrical contacts through an insulated cylindrical core
Iron overload in thalassaemia intermedia: Reassessment of iron chelation strategies
No full textThalassaemia intermedia (TI) is a syndrome marked by its diverse underlying genetic basis although its pathophysiology remains unclear, particularly regarding the nature of iron loading and toxicity. It is, however, evident that there are key differences from the extensively studied thalassaemia major (TM) population and caution is required when assessing iron load based on serum ferritin values, as this approach is known to underestimate the true extent of iron loading in patients with TI. Although effective iron chelation therapy has been available for many years, studies in TI-specific populations are rare and evidence suggests that management of iron levels may be less rigorous than in patients with TM and other chronic anaemias. Better understanding of the need to assess and treat iron overload in both transfused and non-transfused TI patients is clearly required. © 2009 Blackwell Publishing Ltd.Aessopos A, 2005, CHEST, V127, P1523, DOI 10.1378-chest.127.5.1523; 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; Aessopos A, 2007, TRANSFUSION, V47, P792, DOI 10.1111-j.1537-2995.2007.01192.x; Andrews NC, 2008, BLOOD, V112, P219, DOI 10.1182-blood-2007-12-077388; BRITTENHAM GM, 1993, AM J HEMATOL, V42, P81, DOI 10.1002-ajh.2830420116; BUONANNO G, 1984, SCAND J HAEMATOL, V32, P83; Cabantchik ZI, 2005, BEST PRACT RES CL HA, V18, P277, DOI 10.1016-j.beha.2004.10.003; Cappellini MD, 2008, GUIDELINES CLIN MANA; Cappellini MD, 2005, SEMIN HEMATOL, V42, pS19, DOI 10.1053-j.seminhematol.2005.01.001; Cappellini MD, 2006, BLOOD, V107, P3455, DOI 10.1182-blood-2005-08-3430; COSSU P, 1981, EUR J PEDIATR, V137, P267, DOI 10.1007-BF00443255; FIORELLI G, 1990, HAEMATOLOGICA, V75, P89; Fischer R, 2005, ANN NY ACAD SCI, V1054, P350, DOI 10.1196-annals.1345.043; Isma'eel H, 2008, AM J CARDIOL, V102, P363, DOI 10.1016-j.amjcard.2008.03.066; Jenkins ZA, 2007, PEDIATR HEMAT ONCOL, V24, P237, DOI 10.1080-08880010701360700; MAGGIO A, 2007, BLOOD, V110; Mohamed N, 1998, BLOOD REV, V12, P163, DOI 10.1016-S0268-960X(98)90014-5; Nemeth E, 2004, SCIENCE, V306, P2090, DOI 10.1126-science.1104742; OLIVIERI NF, 1995, NEW ENGL J MED, V332, P918, DOI 10.1056-NEJM199504063321404; OLIVIERI NF, 1992, BLOOD, V79, P2741; Origa R, 2008, HAEMATOL-HEMATOL J, V93, P1095, DOI 10.3324-haematol.12484; Origa R, 2007, HAEMATOL-HEMATOL J, V92, P583, DOI 10.3324-haematol.10842; Pakbaz Z, 2007, PEDIATR BLOOD CANCER, V49, P329, DOI 10.1002-pbc.21275; Peyssonnaux C, 2007, J CLIN INVEST, V117, P1926, DOI 10.1172-JCI31370; Piga A, 2009, AM J HEMATOL, V84, P29, DOI 10.1002-ajh.21317; Pippard M J, 1988, Birth Defects Orig Artic Ser, V23, P29; Pootrakul P, 2003, BRIT J HAEMATOL, V122, P305, DOI 10.1046-j.1365-2141.2003.04412.x; Porter J, 2007, SEMIN HEMATOL, V44, pS16, DOI 10.1053-j.seminhematol.2007.03.004; Porter J, 2008, EUR J HAEMATOL, V80, P168, DOI 10.1111-j.1600-0609.2007.00985.x; Silvestri L, 2008, BLOOD, V111, P924, DOI 10.1182-blood-2007-07-100677; St Pierre TG, 2005, BLOOD, V105, P855, DOI 10.1182-blood-2004-01-0177; Taher A, 2008, HAEMATOL-HEMATOL J, V93, P1584, DOI 10.3324-haematol.13098; Taher A, 2006, BLOOD CELL MOL DIS, V37, P12, DOI 10.1016-j.bcmd.2006.04.005; Tanno T, 2007, NAT MED, V13, P1096, DOI 10.1038-nm1629; Treadwell MJ, 2001, SEMIN HEMATOL, V38, P77, DOI 10.1053-shem.2001.20148; Valore EV, 2008, BLOOD CELL MOL DIS, V40, P132, DOI 10.1016-j.bcmd.2007.07.009; Vichinsky E, 2007, BRIT J HAEMATOL, V136, P501, DOI 10.1111-j.1365-2141.2006.06455.x; Wood JC, 2007, CURR OPIN HEMATOL, V14, P183, DOI 10.1097-MOH.0b013e3280d2b76b35393
- …
