16 research outputs found
Space law: da settore strategico a beneficio comune. percorsi di diritto privato tra comparazione ed analisi economica
No full textSull'assunto dell'inquadramento dello "spazio" come settore strategico, l'articolo si sofferma sull'analisi dell'incontro virtuoso tra imprese operanti nel settore spaziale e potere pubblico. In particolare, il lavoro tratta le possibili ricadute applicative dei golden powers in materia spaziale, offrendo alcune suggestioni sistematiche sulla scorta dell'esperienza recente
Report of a ‘consensus’ on the lines of therapy for primary immune thrombocytopenia in adults, promoted by the Italian Gruppo di Studio delle Piastrine
No full textDespite the publication in 2009 of a paper on ‘terms and definitions of immune thrombocytopenia’ (ITP), some unresolved issues remain and are reflected by the disagreement in the treatment suggested for primary ITP in adults. Considering that these disagreements could be ascribed to non-shared goals, we generated a ‘consensus’ on some terms, definitions, and assertions useful for classifying the different lines of treatment for primary ITP in adults according to their indications and goals. Agreement on the appropriateness of the single assertions was obtained by consensus for the following indicators: 1. classification of four ‘lines of therapy’; 2. acceptance of the expression ‘sequences of disease’ for the indications of the respective four lines of treatment; 3I. practicability of splenectomy; 3Ib. acceptance, with only some exceptions, of a ‘timing for elective splenectomy of 12 months’; and 4a-d. ‘goals of the four lines of therapy.’ On the basis of the consensus, a classification of four lines of treatment for primary ITP in adults was produced. In our opinion, this classification, whose validity is not influenced by the recently published new guidelines of the American Society of Hematology (ASH) and reviews, could reduce the disagreement that still exists regarding the treatment of the disease
Influenza vaccination in immunosuppressed and healthy individuals : DC-induced regulatory NK cell-function
No full textInfection with the human immunodeficiency virus (HIV) leads to immunosuppression, mainly
through depletion of CD4+ T cells. Anti-retroviral therapy (ART) inhibits virus replication and
thereby promotes recovery of T cell numbers and reconstitution of cellular immunity. Importantly,
ART improves control of opportunistic infections, increases quality of life and restores life
expectancy of HIV-infected individuals.
We assessed how HIV-infected individuals compare to HIV-negative individuals in terms of
mounting an influenza-vaccine specific humoral and cellular immune response. We were able to
show that the influenza-specific IgM- but not the IgG response was absent in HIV-infected
individuals with low CD4+ T cell counts. Moreover, expansion of IFN-γ secreting CD4+ T cells
was impaired. These findings point out the importance of building B cell memory while
immunological competence is maintained.
Fingolimod-treated individuals have –similar to HIV-infected untreated individuals– low CD4+ T
cell counts in their peripheral circulation. In the second section of the thesis we characterized
cellular and humoral immune responses to influenza-vaccine in fingolimod-treated patients
(diagnosed with multiple sclerosis), and in untreated healthy controls. Intriguingly, vaccinetriggered
T cells accumulated normally in blood of fingolimod-treated individuals, despite reduced
peripheral T cell counts. Concentrations of anti-influenza A/B IgM and IgG also increased
similarly in both groups. These results indicate that fingolimod-treated individuals can mount
vaccine-specific adaptive immune responses comparable to healthy controls.
Natural killer cells (NK cells) have the ability to link the innate to the adaptive immune system.
We determined the Killer-cell immunoglobulin-like receptor (KIR) genotype in two cohorts of
healthy and one cohort of HIV-infected individuals, and related genotype and influenza-vaccine
induced adaptive immune response(s). These experiments uncovered enhanced vaccinespecific
CD4+ T cell-immunity to be induced in HIV-negative but not HIV-positive individuals
bearing the KIR 2DS1/3DS1 genes. Following up on our observation that NK cell-receptor gene polymorphisms impact adaptive
immune responses we lastly tested the hypothesis that NK cells may directly impact the
interaction between dendritic cells (DCs) and T cells. Assessing this interaction in vitro we were
able to demonstrate that LPS-matured DCs, but not immature DCs, have the capacity to induce
immunosuppressive reactivity in autologous NK cells
Prospective evaluation of patient-reported outcomes during treatment with deferasirox or deferoxamine for iron overload in patients with beta-thalassemia.
No full textIron chelation therapy (ICT) with deferoxamine (DFO), the current standard for the treatment of iron overload in patients with transfusion-dependent disorders such as beta-thalassemia, requires regular subcutaneous or intravenous infusions. This can lead to reduced quality of life and poor adherence, resulting in increased morbidity and mortality in iron-overloaded patients with beta-thalassemia. Deferasirox is an orally administered iron chelator that has been approved for use in the United States, Switzerland, and other countries.Clinical Trial, Phase IIIJournal ArticleRandomized Controlled TrialResearch Support, Non-U.S. Gov'tSCOPUS: ar.jinfo:eu-repo/semantics/publishe
Brazilian Thalassemia Association Protocol For Iron Chelation Therapy In Patients Under Regular Transfusion
No full textIn the absence of an iron chelating agent, patients with beta-thalassemia on regular transfusions present complications of transfusion-related iron overload. Without iron chelation therapy, heart disease is the major cause of death; however, hepatic and endocrine complications also occur. Currently there are three iron chelating agents available for continuous use in patients with thalassemia on regular transfusions (desferrioxamine, deferiprone, and deferasirox) providing good results in reducing cardiac, hepatic and endocrine toxicity. These practice guidelines, prepared by the Scientific Committee of Associação Brasileira de Thalassemia (ABRASTA), presents a review of the literature regarding iron overload assessment (by imaging and laboratory exams) and the role of T2* magnetic resonance imaging (MRI) to control iron overload and iron chelation therapy, with evidence-based recommendations for each clinical situation. Based on this review, the authors propose an iron chelation protocol for patients with thalassemia under regular transfusions.356428434Borgna-Pignatti, C., Rugolotto, S., de Stefano, P., Zhao, H., Cappellini, M.D., Del Vecchio, G.C., Survival and complications in patients with thalassemia major treated with transfusion and deferoxamine (2004) Haematologica, 89 (10), pp. 1187-1193. , Comment in: Haematologica. 2004;89(10):1157-9Cunningham, M.J., Macklin, E.A., Neufeld, E.J., Cohen, A.R., Thalassemia Clinical Research Network. Complications of beta-thalassemia major in North America (2004) Blood, 104 (1), pp. 34-39Kirk, P., Roughton, M., Porter, J.B., Walker, J.M., Tanner, M.A., Patel, J., Cardiac T2* magnetic resonance for prediction of cardiac complications in thalassemia major (2009) Circulation, 120 (20), pp. 1961-1968. , Comment in: Circulation. 2009;120(20):1937-9Brittenham, G.M., Griffith, P.M., Nienhuis, A.W., McLaren, C.E., Young, N.S., Tucker, E.E., Efficacy of deferoxamine in preventing complications of iron overload in patients with thalassemia major (1994) N Engl J Med, 331 (9), pp. 567-573. , Comment in: N Engl J Med. 1995;332(4):270-1. N Engl J Med. 1995;332(4):271-2. N Engl J Med. 1994;331(9):609-10Olivieri, N.F., Nathan, D.G., Macmillan, J.H., Wayne, A.S., Liu, P.P., McGee, A., Survival in medically treated patients with homozygous beta-thalassemia (1994) N Engl J Med, 331 (9), pp. 574-578. , Comment in: N Engl J Med. 1995;332(4):271author reply 272-3N Engl J Med. 1995;332(4):271-2author reply 272-3N Engl J Med. 1994331(9):609-10Gabutti, V., Piga, A., Results of long-term iron-chelating therapy (1996) Acta Haematol, 95 (1), pp. 26-36Modell, B., Khan, M., Darlison, M., Survival in beta-thalassaemia major in the UK: Data from the UK Thalassaemia Register (2000) Lancet, 355 (9220), pp. 2051-2052Anderson, L.J., Westwood, M.A., Prescott, E., Walker, J.M., Pennell, D.J., Wonke, B., Development of thalassaemic iron overload cardiomyopathy despite low liver iron levels and meticulous compliance to desferrioxamine (2006) Acta Haematol, 115 (1-2), pp. 106-108Hoffbrand, A.V., Cohen, A., Hershko, C., Role of deferiprone in chelation therapy for transfusional iron overload (2003) Blood, 102 (1), pp. 17-24Victor Hoffbrand, A., Deferiprone therapy for transfusional iron overload (2005) Best Pract Res Clin Haematol, 18 (2), pp. 299-317Wonke, B., Wright, C., Hoffbrand, A.V., Combined therapy with deferiprone and desferrioxamine (1998) Br J Haematol, 103 (2), pp. 361-364. , Comment in: Br J Haematol. 1999;106(1):252-3Mourad, F.H., Hoffbrand, A.V., Sheikh-Taha, M., Koussa, S., Khoriaty, A.I., Taher, A., Comparison between desferrioxamine and combined therapy with desferrioxamine and deferiprone in iron overloaded thalassaemia patients (2003) Br J Haematol, 121 (1), pp. 187-189Kattamis, A., Kassou, C., Berdousi, H., Ladis, V., Papassotiriou, I., Kattamis, C., Combined therapy with desferrioxamine and deferiprone in thalassemic patients: Effect on urinary iron excretion (2003) Haematologica, 88 (12), pp. 1423-1425Wu, K.H., Chang, J.S., Tsai, C.H., Peng, C.T., Combined therapy with deferiprone and desferrioxamine successfully regresses severe heart failure in patients with beta-thalassemia major (2004) Ann Hematol, 83 (7), pp. 471-473Alymara, V., Bourantas, D., Chaidos, A., Bouranta, P., Gouva, M., Vassou, A., Effectiveness and safety of combined iron-chelation therapy with deferoxamine and deferiprone (2004) Hematol J, 5 (6), pp. 475-479Origa, R., Bina, P., Agus, A., Crobu, G., Defraia, E., Dessi, C., Combined therapy with deferiprone and desferrioxamine in thalassemia major (2005) Haematologica, 90 (10), pp. 1309-1314. , Comment in: Haematologica. 2005;90(10):1297ADaar, S., Pathare, A.V., Combined therapy with desferrioxamine and deferiprone in beta thalassemia major patients with transfusional iron overload (2006) Ann Hematol, 85 (5), pp. 315-319Kattamis, A., Ladis, V., Berdousi, H., Kelekis, N.L., Alexopoulou, E., Papasotiriou, I., Iron chelation treatment with combined therapy with deferiprone and deferioxamine: A 12-month trial (2006) Blood Cells Mol Dis, 36 (1), pp. 21-25Piga, A., Gaglioti, C., Fogliacco, E., Tricta, F., Comparative effects of deferiprone and deferoxamine on survival and cardiac disease in patients with thalassemia major: A retrospective analysis (2003) Haematologica, 88 (5), pp. 489-496. , Comment in: Haematologica. 2003;88(5):481-2Borgna-Pignatti, C., Cappellini, M.D., de Stefano, P., Del Vecchio, G.C., Forni, G.L., Gamberini, M.R., Cardiac morbidity and mortality in deferoxamine- or deferiprone-treated patients with thalassemia major (2006) Blood, 107 (9), pp. 3733-3737Pennell, D.J., Berdoukas, V., Karagiorga, M., Ladis, V., Piga, A., Aessopos, A., Randomized controlled trial of deferiprone or desferoxamine in beta-thalassemia major patients with asymptomatic myocardial siderosis (2006) Blood, 107 (9), pp. 3738-3744Tanner, M.A., Galanello, R., Dessi, C., Smith, G.C., Westwood, M.A., Agus, A., A randomized, placebo-controlled, double-blind trial of the effect of combined therapy with deferoxamine and deferiprone on myocardial iron in thalassemia major using cardiovascular magnetic resonance (2007) Circulation, 115 (14), pp. 1876-1884Tsironi, M., Deftereos, S., Andriopoulos, P., Farmakis, D., Meletis, J., Aessopos, A., Reversal of heart failure in thalassemia major by combined chelation therapy: A case report (2005) Eur J Haematol, 74 (1), pp. 84-85Davis, B.A., Porter, J.B., Long-term outcome of continuous 24-hour deferoxamine infusion via indwelling intravenous catheters in high-risk beta-thalassemia (2000) Blood, 95 (4), pp. 1229-1236Neufeld, E.J., Oral chelators deferasirox and deferiprone for transfusional iron overload in thalassemia major: New data, new questions (2006) Blood, 107 (9), pp. 3436-3441Cappellini, M.D., Cohen, A., Piga, A., Bejaoui, M., Perrotta, S., Agaoglu, L., A phase 3 study of deferasirox (ICL670), a once-daily oral iron chelator, in patients with beta-thalassemia (2006) Blood, 107 (9), pp. 3455-3462. , Comment in: Blood. 2006;108(5):1775-6Blood. 2006;108(2):774-5Blood. 2006;108(2):778Deugnier, Y., Turlin, B., Ropert, M., Cappellini, M.D., Porter, J.B., Giannone, V., Improvement in liver pathology of patients with β-thalassemia treated with deferasirox for at least 3 years (2011) Gastroenterology, 141 (4). , Comment in: Gastroenterology. 2011;141(4):1142-3 1202-1211+1211.e1-3Cappellini, M.D., Bejaoui, M., Agaoglu, L., Canatan, D., Capra, M., Cohen, A., Iron chelation with deferasirox in adult and pediatric patients with thalassemia major: Efficacy and safety during 5 years' follow-up (2011) Blood, 118 (4), pp. 884-893Eleftheriou, P., Tanner, M., Pennel, D., Porter, J.B., Response of myocardial T2* to oral deferasirox monotherapy for 1 year in 29 patients with transfusion-dependent anaemiasa subgroup analysis (2006) Haematologica, 91, p. 366Pennell, D.J., Porter, J.B., Cappellini, M.D., El-Beshlawy, A., Chan, L.L., Aydinok, Y., Efficacy of deferasirox in reducing and preventing cardiac iron overload in beta-thalassemia (2010) Blood, 115 (12), pp. 2364-2371. , Comment in: Blood. 2010;115(12):2333-4Pennell, D.J., Porter, J.B., Cappellini, M.D., Chan, L.L., El-Beshlawy, A., Aydinok, Y., Continued improvement in myocardial T2* over two years of deferasirox therapy in β-thalassemia major patients with cardiac iron overload (2011) Haematologica, 96 (1), pp. 48-54. , Comment in: Haematologica. 2011;96(1):5-8Pennell, D.J., Porter, J.B., Cappellini, M.D., Chan, L.L., El-Beshlawy, A., Aydinok, Y., Deferasirox for up to 3 years leads to continued improvement of myocardial T2* in patients with β-thalassemia major (2012) Haematologica, 97 (6), pp. 842-848De Assis, R.A., Ribeiro, A.A., Kay, F.U., Rosemberg, L.A., Nomura, C.H., Loggetto, S.R., Pancreatic iron stores assessed by magnetic resonance imaging (MRI) in beta thalassemic patients (2012) Eur J Radiol, 81 (7), pp. 1465-1470Wood, J.C., Tyszka, J.M., Carson, S., Nelson, M.D., Coates, T.D., Myocardial iron loading in transfusion-dependent thalassemia and sickle cell disease (2004) Blood, 103 (5), pp. 1934-1936Wood, J.C., Origa, R., Agus, A., Matta, G., Coates, T.D., Galanello, R., Onset of cardiac iron loading in pediatric patients with thalassemia major (2008) Haematologica, 93 (6), pp. 917-920. , Comment in: Haematologica. 2009;94(12):1776-7Fernandes, J.L., Fabron Jr., A., Verissimo, M., Early cardiac iron overload in children with transfusion-dependent anemias (2009) Haematologica, 94 (12), pp. 1776-1777. , Comment on: Haematologica. 2008;93(6):917-20Harrison, P.M., Arosio, P., The ferritins: Molecular properties, iron storage function and cellular regulation (1996) Biochim Biophys Acta, 1275 (3), pp. 161-203Olivieri, N.F., Brittenham, G.M., Matsui, D., Berkovitch, M., Blendis, L.M., Cameron, R.G., Iron-chelation therapy with oral deferiprone in patients with thalassemia major (1995) N Engl J Med, 332 (14), pp. 918-922. , Comment in: N Engl J Med. 1995;333(9):597-8N Engl J Med. 1995;333(9):598N Engl J Med. 1995;332(14):953-4Hershko, C., Link, G., Cabantchik, I., Pathophysiology of iron overload (1998) Ann N Y Acad Sci, 850, pp. 191-201Wood, J.C., Enriquez, C., Ghugre, N., Tyzka, J.M., Carson, S., Nelson, M.D., Coates, T.D., MRI R2 and R2* mapping accurately estimates hepatic iron concentration in transfusion-dependent thalassemia and sickle cell disease patients (2005) Blood, 106 (4), pp. 1460-1465Cappellini, M.D., Cohen, A., Eleftheriou, A., Piga, A., Porter, J., Taher, A., (2008) Guidelines For the Clinical Management of Thalassaemia [Internet], , http://www.thalassaemia.org.cy/wordpress/wp-content/uploads/2012/12/Guidelines-2nd-edition-revised-ENGLISH-lo.pdf, editors, 2nd rev ed. Cyprus: Thalassaemia International Federation, [cited 2011 Sep 21]. Available fromAnderson, L.J., Holden, S., Davis, B., Prescott, E., Charrier, C.C., Bunce, N.H., Cardiovascular T2-star (T2*) magnetic resonance for the early diagnosis of myocardial iron overload (2001) Eur Heart J, 22 (23), pp. 2171-2179. , Comment in: Eur Heart J. 2001;22(23):2140-1Ghugre, N.R., Enriquez, C.M., Coates, T.D., Nelson Jr., M.D., Wood, J.C., Improved R2* measurements in myocardial iron overload (2006) J Magn Reson Imaging, 23 (1), pp. 9-16Fernandes, J.L., Sampaio, E.F., Verissimo, M., Pereira, F.B., da Silva, J.A., Figueiredo, G.S., Heart and liver T2* assessment for iron overload using different software programs (2011) Eur Radiol, 21 (12), pp. 2503-2510Hankins, J.S., McCarville, M.B., Loeffler, R.B., Smeltzer, M.P., Onciu, M., Hoffer, F.A., R2* magnetic resonance imaging of the liver in patients with iron overload (2009) Blood, 113 (20), pp. 4853-4855Carpenter, J.P., He, T., Kirk, P., Roughton, M., Anderson, L.J., Noronha, S.V., On T2* magnetic resonance and cardiac iron (2011) Circulation, 123 (14), pp. 1519-1528Farmaki, K., Tzoumari, I., Pappa, C., Chouliaras, G., Berdoukas, V., Normalisation of total body iron load with very intensive combined chelation reverses cardiac and endocrine complications of thalassaemia major (2010) Br J Haematol, 148 (3), pp. 466-475. , Comment in: Br J Haematol. 2010;150(4):489-90Porter, J.B., Piga, A., Cohen, A., Ford, J.M., Bodner, J., Rojkjaer, L., Safety of deferasirox (Exjade®) in patients with transfusion-dependent anemias and iron overload who achieve serum ferritin levels < 1000 ng/mL during long-term treatment (2008) Blood, 112 (11). , http://abstracts.hematologylibrary.org/cgi/content/abstract/112/11/5423?maxtoshow=&hits=10&RESULTFORMAT=&fulltext=piga+a&searchid=1&FIRSTINDEX=0&volume=112&issue=11&resourcetype=HWCIT, (ASH Annual Meeting Abstracts) [Internet], abstract 5423. [cited 2012 Mar 21], Available fromCohen, A.R., Galanello, R., Piga, A., de Sanctis, V., Tricta, F., Safety and effectiveness of long-term therapy with the oral iron chelator deferiprone (2003) Blood, 102 (5), pp. 1583-1587Al-Refaie, F.N., Wonke, B., Hoffbrand, A.V., Deferiprone-associated myelotoxicity (1994) Eur J Haematol, 53 (5), pp. 298-301(2011) Projeto Diretrizes: Associação Médica Brasileira E Conselho Federal De Medicina, , http://www.projetodiretrizes.org.br/projeto_diretrizes/texto_introdutorio.pdf, São Paulo, [cited, Sep 14], Available fromFargion, S., Fracanzani, A.L., Rossini, A., Borzio, M., Riggio, O., Belloni, G., Iron reduction and sustained response to interferon-alpha therapy in patients with chronic hepatitis C: Results of an Italian multicenter randomized study (2002) Am J Gastroenterol, 97 (5), pp. 1204-1210. , Comment in: Am J Gastroenterol. 2002;97(5):1093-6Angelucci, E., Muretto, P., Nicolucci, A., Baronciani, D., Erer, B., Gaziev, J., Effects of iron overload and hepatitis C virus positivity in determining progression of liver fibrosis in thalassemia following bone marrow transplantation (2002) Blood, 100 (1), pp. 17-21Hershko, C., Pathogenesis and management of iron toxicity in thalassemia (2010) Ann N Y Acad Sci, 1202, pp. 1-9Piga, A., Longo, F., Duca, L., Roggero, S., Vinciguerra, T., Calabrese, R., High non transferrin bound iron levels and heart disease in thalassemia major (2009) Am J Hematol, 84 (1), pp. 29-33Farmaki, K., Tzoumari, I., Pappa, C., Oral chelators in transfusion-dependent thalassemia major patients may prevent or reverse iron overload complications (2011) Blood Cells Mol Dis, 47 (1), pp. 33-4
Immunogenicity and tolerability of a virosome influenza vaccine compared to split influenza vaccine in patients with sickle cell anemia
No full textThe immunogenicity and tolerability of virosome and of split influenza vaccines in patients with sickle cell anemia (SS) were evaluated Ninety SS patients from 8 to 34 years old were randomly assigned to receive either virosome (n = 43) or split vaccine (n = 47) Two blood samples were collected, one before and one 4-6 weeks after vaccination Antibodies against viral strains (2006) A/New Caledonia (H1N1), A/California (H3N2), B/Malaysia were determined using the hemagglutinin inhibition test Post-vaccine reactions were recorded over 7 days Seroconversion rates for HI NI, H3N2 and B were 65 1%. 60 4% and 83 7% for virosome vaccine, and 68 0%, 61 7% and 68 0% for split vaccine Seroprotection rates for HI NI, H3N2 e B were 100%. 97 6% and 69.7% for virosome. and 97 8%, 97 8% and 76 6% for split vaccine No severe adverse reactions were recorded Virosome and split vaccines in patients with sickle cell anemia were equally Immunogenic. with high seroconversion and seroprotection rates Both vaccines were well tolerated (C) 2009 Elsevier Ltd All rights reservedBerna Biotech, Ltd. Laboratory, SwitzerlandCenters for Disease Control and Prevention, Atlanta, US
Beta Thalassemia Major And Pregnancy During Adolescence: Report Of Two Cases [talassemia Beta Maior E Gestação Na Adolescência: Relato De Dois Casos]
No full textBeta thalassemia major is a rare hereditary blood disease in which impaired synthesis of beta globin chains causes severe anemia. Medical treatment consists of chronic blood transfusions and iron chelation. We describe two cases of adolescents with beta thalassemia major with unplanned pregnancies and late onset of prenatal care. One had worsening of anemia with increased transfusional requirement, fetal growth restriction, and placental senescence. The other was also diagnosed with hypothyroidism and low maternal weight, and was admitted twice during pregnancy due to dengue shock syndrome and influenza H1N1-associated respiratory infection. She also developed fetal growth restriction and underwent vaginal delivery at term complicated by uterine hypotonia. Both patients required blood transfusions after birth and chose medroxyprogesterone as a contraceptive method afterwards. This report highlights the importance of medical advice on contraceptive methods for these women and the role of a specialized prenatal follow-up in association with a hematologist. © 2015, Federacao Brasileira das Sociedades de Ginecologia e Obstetricia. All rights reserved.376291296Williams, T.N., Weatherall, D.J., World distribution, population genetics, and health burden of the hemoglobinopathies (2012) Cold Spring Harb Perspect Med, 2 (9)Higgs, D.R., Engel, J.D., Stamatoyannopoulos, G., (2012) Thalassaemia. Lancet, 379 (9813), pp. 373-383Sankaran, V.G., Weiss, M.J., Anemia: Progress in molecular mechanisms and therapies (2015) Nat Med, 21 (3), pp. 221-230Rachmilewitz, E.A., Giardina, P.J., How I treat thalassemia (2011) Blood, 118 (13), pp. 3479-3488Naik, R.P., Lanzkron, S., Baby on board: What you need to know about pregnancy in the hemoglobinopathies (2012) Hematology am Soc Hematol Educ Program, pp. 208-214. , 2012Waseem, F., Khemomal, K.A., Sajid, R., Antioxidant status in beta thalassemia major: A single-center study (2011) Indian J Pathol Microbiol, 54 (4), pp. 761-763Marsella, M., Borgna-Pignatti, C., Transfusional iron overload and iron chelation therapy in thalassemia major and sickle cell disease (2014) Hematol Oncol Clin North Am, 28 (4), pp. 703-27, viToumba, M., Sergis, A., Kanaris, C., Skordis, N., Endocrine complications in patients with Thalassaemia Major (2007) Pediatr Endocrinol Rev, 5 (2), pp. 642-648Borgna-Pignatti, C., Gamberini, M.R., Complications of thalassemia major and their treatment (2011) Expert Rev Hematol, 4 (3), pp. 353-366Toumba, M., Kanaris, C., Simamonian, K., Skordis, N., Outcome and management of pregnancy in women with thalassaemia in Cyprus (2008) East Mediterr Health J, 14 (3), pp. 628-635Singer, S.T., Vichinsky, E.P., Gildengorin, G., van Disseldorp, J., Rosen, M., Cedars, M.I., Reproductive capacity in iron overloaded women with thalassemia major (2011) Blood, 118 (10), pp. 2878-2881Origa, R., Piga, A., Quarta, G., Forni, G.L., Longo, F., Melpignano, A., Pregnancy and beta-thalassemia: An Italian multicenter experience (2010) Haematologica, 95 (3), pp. 376-381Weatherall, D.J., The challenge of haemoglobinopathies in resourcepoor countries (2011) Br J Haematol, 154 (6), pp. 736-744McCarthy, F.P., O’Brien, U., Kenny, L.C., The management of teenage pregnancy (2014) BMJ, 349Ganchimeg, T., Ota, E., Morisaki, N., Laopaiboon, M., Lumbiganon, P., Zhang, J., Pregnancy and childbirth outcomes among adolescent mothers: A World Health Organization multicountry study (2014) BJOG, 121, pp. 40-48Oliveira, F.C., Jr., Costa, M.L., Cecatti, J.G., Silva, J.L.P.E., Surita, F.G., Maternal morbidity and near miss associated with maternal age: The innovative approach of the 2006 Brazilian demographic health survey (2013) Clinics (São Paulo), 68 (7), pp. 922-927Santos, G.H., Martins, M.G., Sousa, M.S., Teenage pregnancy and factors associated with low birth weight (2008) Rev Bras Ginecol Obstet, 30 (5), pp. 224-231. , PortugueseAessopos, A., Karabatsos, F., Farmakis, D., Katsantoni, A., Hatziliami, A., Youssef, J., Pregnancy in patients with well-treated betathalassemia: Outcome for mothers and newborn infants (1999) Am J Obstet Gynecol, 180 (2), pp. 360-365Gulino, F.A., Vitale, S.G., Fauzia, M., Cianci, S., Pafumi, C., Palumbo, M.A., Beta-thalassemia major and pregnancy (2013) Bratisl Lek Listy, 114 (9), pp. 523-525Thompson, A.A., Kim, H.Y., Singer, S.T., Vichinsky, E., Eile, J., Yamashita, R., Pregnancy outcomes in women with thalassemia in North America and the United Kingdom (2013) Am J Hematol, 88 (9), pp. 771-773Verissimo, M., Loggetto, S.R., Fabron, J.A., Baldanzi, G.R., Hamerschlak, N., Fernandes, J.L., Brazilian Thalassemia Association protocol for iron chelation therapy in patients under regular transfusion (2013) Rev Bras Hematol Hemoter, 35 (6), pp. 428-434Singer, S.T., Vichinsky, E.P., Deferoxamine treatment during pregnancy: Is it harmful? (1999) Am J Hematol, 60 (1), pp. 24-26Maulik, D., Mundy, D., Heitmann, E., Maulik, D., Evidence-based approach to umbilical artery Doppler fetal surveillance in high-risk pregnancies: An update (2010) Clin Obstet Gynecol, 53 (4), pp. 869-878Afrakhteh, M., Moeini, A., Taheri, M.S., Haghighatkhah, H.R., Fakhri, M., Masoom, N., Uterine Doppler velocimetry of the uterine arteries in the second and third trimesters for the prediction of gestational outcome (2014) Rev Bras Ginecol Obstet, 36 (1), pp. 35-39Tavares, N.M., Ferreira, S.G., Bennini, J.R., Marussi, E.F., Barini, R., Peralta, C.F., Longitudinal reference intervals of maternal-fetal Doppler parameters (2013) Rev Bras Ginecol Obstet, 35 (1), pp. 33-38. , PortugueseOwen, P., Ogah, J., Bachmann, L.M., Khan, K.S., Prediction of intrauterine growth restriction with customised estimated fetal weight centiles (2003) BJOG, 110 (4), pp. 411-415Danjou, F., Francavilla, M., Anni, F., Satta, S., Demartis, F.R., Perseu, L., A genetic score for the prediction of beta-thalassemia severity (2015) Haematologica, 100 (4), pp. 452-457Silva, C.A., Baldim, L.B., Nhoncanse, G.C., Estevao, I.D., Melo, D.G., Neonatal screening for hemoglobinopathies in São Carlos, São Paulo, Brazil: Analysis of a series of cases (2015) Rev Paul Pediatr, 33 (1), pp. 19-27. , PortugueseContraception for adolescents (2014) Pediatrics, 134 (4), pp. e1244-e125
Novel Framework of Financial Hardship in Childhood Cancer: Incorporating Stakeholder Perspectives
No full textPURPOSEAlthough financial hardship in childhood cancer contributes to poor outcomes, no standardized tool to assess its impact exists. Existing methods are heterogeneous and designed using high-income country (HIC), adult perspectives. This project aimed to construct a stakeholder-informed conceptual framework of financial hardship in childhood cancer with global relevancy.METHODSGroup concept mapping, a participatory mixed-methods approach, was used. Participants were parents or caregivers of a child with cancer, childhood cancer survivors, and clinical or nonclinical support personnel, fluent in English, Spanish, or Portuguese. A representative panel established a comprehensive list of relevant items. Participants individually sorted these items into concepts and then rated each item for impact using a four-point Likert scale. Multidimensional scaling and hierarchical cluster analysis identified concepts. Descriptive statistics were calculated for impact ratings.RESULTSOne fourth (21/80) of participants were parents/caregivers or childhood cancer survivors. Participants worked in clinical (44/80), charity/volunteer (27/80), and other nonclinical (13/80) roles. Of the 41 represented countries, 78.0% (32) were low- and middle-income countries (LMICs). Conceptual themes spanned six distinct clusters: medical, nonmedical, assistance and support, treatment impact, family impact, and caregiver impact. These were distinct in composition compared with an existing framework for adult oncology. Caregiver impact (mean, 3.39) and treatment impact (mean, 3.29) were the highest rated clusters, and impact ratings were higher in LMICs compared with HICs.CONCLUSIONWe developed a framework for financial hardship in childhood cancer that reflects the voices of stakeholders, including parents and caregivers, from diverse global contexts. The findings lay a foundation for the development and validation of tools to systematically assess financial hardship in families of children with cancer and inform effective policies and interventions
Pancreatic iron stores assessed by magnetic resonance imaging (MRI) in beta thalassemic patients
No full textPurpose: To assess the correlation between MRI findings of the pancreas with those of the heart and liver in patients with beta thalassemia; to compare the pancreas T2* MRI results with glucose and ferritin levels and labile plasma iron (LPI). Materials and methods: We retrospectively evaluated chronically transfused patients, testing glucose with enzymatic tests, serum ferritin with chemiluminescence, LPI with cellular fluorescence, and T2* MRI to assess iron content in the heart, liver, and pancreas. MRI results were compared with one another and with serum glucose, ferritin, and LPI. Liver iron concentration (LIC) was determined in 11 patients' liver biopsies by atomic absorption spectrometry. Results: 289 MRI studies were available from 115 patients during the period studied. 9.4% of patients had overt diabetes and an additional 16% of patients had impaired fasting glucose. Both pancreatic and cardiac R2* had predictive power (p < 0.0001) for identifying diabetes. Cardiac and pancreatic R2* were modestly correlated with one another (r(2) = 0.20, p < 0.0001). Both were weakly correlated with LIC (r(2) = 0.09, p < 0.0001 for both) and serum ferritin (r(2) = 0.14, p < 0.0001 and r(2) = 0.03, p < 0.02, respectively). None of the three served as a screening tool for single observations. There is a strong log-log, or power-law, relationship between ratio of signal intensity (SIR) values and pancreas R2* with an r(2) of 0.91. Conclusions: Pancreatic iron overload can be assessed by MRI, but siderosis in other organs did not correlate significantly with pancreatic hemosiderosis. (C) 2011 Elsevier Ireland Ltd. All rights reserved.Brazilian Ministry of HealthBrazilian Ministry of Healt
