α-chain Hemoglobin Variants With Electrophoretic Mobility Similar To That Of Hemoglobin S In Newborn Screening Programs.

3585-

PENGARUH PENGGUNAAN MODEL TREFFINGER TERHADAP HASIL BELAJAR SISWA PADA MATA PELAJARAN AQIDAH AKHLAK DI MTsN MODEL KUOK KECAMATAN KUOK

Penelitian ini bertujuan untuk mengetahui ada tidaknya pengaruh penggunaan model treffinger terhadap hasil belajar siswa pada mata pelajaran Aqidah Akhlak di MTsN Model Kuok Kecamatan Kuok. Penelitian ini merupakan penelitian eksperimen. Subjek dalam penelitian ini adalah siswa MTsN Model Kuok sedangkan objek dalam penelitian ini adalah pengaruh penggunaan model treffingerdalam proses pembelajaran terhadap hasil belajar siswa di MTsN Model Kuok, dan Populasi dalam penelitian ini adalah siswa kelas VIII MTsN Model Kuok Kecamatan Kuok yang berjumlah 120 orang, terdiri dari 4 kelas yaitu kelas VIII.1, VIII.2, VIlI.3 dan VIII.4 yang telah diuji homogenitasnya menggunakan uji barlet. Kajian penelitian ini adalah hasil belajar siswa. Untuk melihat hasil penelitian tersebut, digunakan uji normalitas data, kemudian digunakan rumus tes-t untuk mengetahui ada atau tidaknya pengaruh hasil belajar siswa selama proses pembelajaran berlangsung dengan menggunakan Model Treffinger terhadap hasil belajar siswa pada mata pelajaran Aqidah Akhlak. Berdasarkan hasil analisis data tersebut diperoleh nilai thitung = 3,77 yang berarti lebih besar dari tt (t0> tt) baik pada taraf signifikan 5% maupun 1% yaitu (2,00 2,66) sehingga hipotesis nihil ditolak dan hipotesis alternatif diterima. Sehingga dapat di ambil kesimpulan bahwa terdapat pengaruh terhadap hasil belajar siswa pada mata pelajaran Aqidah Akhlak melalui Model Treffinger

Quality of life and body composition : characteristics of successful aging

Orientador: Roberto VilartaTese (doutorado) - Universidade Estadual de Campinas, Faculdade de Educação FísicaResumo: A funcionalidade corporal e a autonomia são fatores essenciais para o desempenho das atividades diárias do idoso. Os cuidados para com a saúde, focados no envelhecimento saudável, envolvem o estudo das relações entre a composição corporal, a prática da atividade física e a qualidade de vida. Esse trabalho teve por objetivo o conhecimento da relação entre essas variáveis em adultos e idosos visando estabelecer estratégias promotoras da saúde. Os sujeitos da pesquisa foram adultos praticantes de bike indoor e hidroginástica de academias de Campinas e dos projetos de extensão da Faculdade de Educação Física da Universidade Estadual de Campinas e idosos praticantes de atividade física na Universidade da Terceira Idade da Universidade Estadual de São Paulo em Piracicaba. Foi avaliado o estado nutricional a partir das medidas de peso corporal, estatura e calculado o índice de massa corporal. A composição corporal foi verificada por bioimpedância tetrapolar. A percepção da qualidade de vida avaliada através do Whoqol-bref. O nível de atividade física pelo instrumento IPAQ versão curta. O nível socioeconômico coletado através de questionário. Os dados foram tratados utilizando-se software SAS; SPSS e Bioestat. Para a análise dos dados os 132 voluntários foram classificados em três grupos etários, respeitando as faixas de idade de 40 à 52 anos - Grupo 1 (n=45)- , de 53 à 66 anos - Grupo 2 (n=43) - de 67 à 80 anos - Grupo 3 (n=44) -. Houve diferença significativa entre os grupos para as variáveis gordura corporal (entre Grupos 1 e 2 e entre Grupos 1 e 3); massa livre de gordura (entre Grupos 1 e 3 e entre Grupos 2 e 3); domínio das relações sociais (entre Grupos 1 e 3); faceta 4 (entre Grupos 1 e 3); faceta 8 (entre Grupos 2 e 3); faceta 10 (entre Grupos 1 e 2) e faceta 11 (entre Grupos 1 e 2). O estudo concluiu que o envelhecimento provoca uma importante diminuição no valor de massa corporal e que este está relacionado com a massa livre de gordura. O controle dessas medidas, durante o processo de envelhecimento, se faz necessário, pois a perda da MLG, principalmente a massa muscular, está diretamente relacionada com a dependência do idoso e ela é significativamente maior depois dos setenta anos de idade. A qualidade de vida mostrou alterações durante o processo de envelhecimento sugerindo que mulheres mais velhas que se exercitam têm melhor qualidade de vida quanto ao domínio de relações sociais que as mais novasAbstract: The body functionality and autonomy are essentials factors because to the performance of daily activities of the elderly. The health care involving the body composition, physical activity and quality of life value healthy aging. This study aimed to evaluate the relationship of these variables between adults and elderly and develop strategies that promote health. The research subjects were adults practicing indoor bike and aqua gym in Campinas and extension projects of the Faculty of Physical Education, State University of Campinas and elderly physically active at the University of the Third Age, State University of Sao Paulo in Piracicaba. Nutritional status was assessed from measurements of body weight, height and calculated body mass index. The body composition was assessed by tetrapolar bioimpedance. The perception of quality of life assessed using the WHOQOL-bref. The level of physical activity by IPAQ short version instrument. The socioeconomic status collected through a questionnaire. The data were processed using the software SAS, SPSS and Bioestat. For the analysis the 132 volunteers were classified into three age groups, respecting the age groups 40-52 years Group 1 (n = 45) - 53 to 66 years Group 2 (n = 43) - 67-80 years, Group 3 (n = 44) -. There were significant differences between groups for body fat (between Groups 1 and 2 and between Groups 1 and 3) fat-free mass (between Groups 1 and 3 and between Groups 2 and 3) the field of social relations (between Groups 1 and 3) the fourth facet (between Groups 1 and 3) 8 facet (among Groups 2 and 3); facet 10 (between Groups 1 and 2) and facet 11 (between Groups 1 and 2). The study concluded that the aging causes a significant reduction in the amount of body mass and that this is related to the fat free mass. The control of these measures during the aging process, is need because the loss of FFM, particularly muscle mass, is directly related to the dependence of the elderly and it is significantly higher after the seventy years of age. The quality of life showed changes during the aging process, suggesting that older women who exercise have better quality of life as to the field of social relationships that the youngerDoutoradoAtividade Fisica, Adaptação e SaudeDoutor em Educação Físic

Molecular identification of Sicilian (deltaß)º-thalassemia associated with ß-thalassemia and hemoglobin S in Brazil

We describe the clinical and molecular characteristics of two unrelated Brazilian families with an association of the Sicilian form of (deltaß)º-thalassemia with hemoglobin S and ß-thalassemia. Direct sequencing of the ß-globin gene showed only the hemoglobin S mutation in patient 1 and the ß-thalassemia IVS1-110 in patient 2. The other allele was deleted in both patients and PCR of DNA samples of the breakpoint region of both patients showed a band of approximately 1,150 bp, expected to be observed in the DNA of carriers of Sicilian (deltaß)º-thalassemia. The nucleotide sequence of this fragment confirmed the Sicilian deletion. There are few reports concerning the Hb S/(deltaß)º-thalassemia association and patient 2 is the first reported case of Sicilian type of (deltaß)º-thalassemia in association with ß-thalassemia documented at the molecular level.87387

High Prevalence Of α-thalassemia Among Individuals With Microcytosis And Hypochromia Without Anemia

In order to determine the contribution of α-thalassemia to microcytosis and hypochromia, 339 adult outpatients seen at Unicamp University Hospital (with the exception of the Clinical Hematology outpatient clinics), who showed normal hemoglobin (Hb) levels and reduced mean corpuscular volume and mean corpuscular hemoglobin, were analyzed. Ninety-eight were Blacks (28.9%) and 241 were Caucasians (71.1%). In all cases, Hb A2 and F levels were either normal or low. The most common deletional and nondeletional forms of α-thalassemia [-α3.7, -α4.2, -MED, -(α)20.5, αHphIα, αNcolα, ααNcoI and αTSAUDI] were investigated by PCR and restriction enzyme analyses. A total of 169 individuals (49.9%) presented α-thalassemia: 145 (42.8%) were heterozygous for the -α3.7 deletion (-α3.7/αα) and 18 (5.3%) homozygous (-α3.7/-α3.7), 5 (1.5%) were heterozygous for the nondeletional form αHPhlα/αα, and 1 (0.3%) was a -MED carrier (-MED/αα). Among the Blacks, 56 (57.1%) showed the -α3.7/ αα genotype, whereas 12 (12.2%) were -α3.7/-α3.7 and I (1.0%) was an αHPhlα carrier; among the Caucasians, 89 (36.9%) were -α3.7/αα, 6 (2.5%) had the -α3.7/-α3.7 genotype, 4 (1.7%) presented the nondeletional form (αHPhlα/αα), and 1 (0.4%) was a -MED carrier. These results demonstrate that α-thalassemia, mainly through the -α3.7 deletion, is an important cause of microcytosis and hypochromia in individuals without anemia. These data are of clinical relevance since these hematological alterations are often interpreted as indicators of iron deficiency.346759762Weatherall, D.J., Clegg, J.G., (1981) The Thalassaemia Syndromes. 3rd Edn., , Blackwell Scientific Publications, OxfordBunn, H.F., Forget, B.G., (1986) Hemoglobin: Molecular, Genetics and Clinical Aspects, , W.B. Saunders, PhiladelphiaHiggs, D.R., Vickers, M.A., Wilkie, A.O.M., Pretorius, I.M., Jarman, A.P., Weatherall, D.J., A review of the molecular genetics of the human α-globin gene cluster (1989) Blood, 73, pp. 1081-1104Kazazian H., Jr., The thalassemia syndromes: Molecular basis and prenatal diagnosis in 1990 (1990) Seminars in Hematology, 27, pp. 209-228Harteveld, K.L., Losekoot, M., Ajgam, H., Van Der Wielen, M., Giordano, P.C., Bernini, L.F., α-Thalassaemia in the Netherlands: A heterogeneous spectrum of both deletions and point mutations (1997) Human Genetics, 100, pp. 465-471Higgs, D.R., α-Thalassaemia (1993) Baillieres Clinical Haematology, 6, pp. 117-150Kattamis, A.C., Camaschella, C., Sivera, P., Surrey, S., Fortina, P., Human α-thalassemia syndromes: Detection of molecular defects (1996) American Journal of Hematology, 53, pp. 81-91Bianco, I., Cappabianca, M.P., Foglietta, E., Lerone, M., Deidda, G., Morlupi, L., Grisanti, P., Graziani, B., Silent thalassemias: Genotypes and phenotypes (1997) Haematologica, 82, pp. 269-280Galanello, R., Sollaino, C., Paglietti, E., Barella, S., Perra, C., Doneddu, I., Pirroni, M.G., Cao, A., α-Thalassemia carrier identification by DNA analysis in the screening for thalassemia (1998) American Journal of Hematology, 59, pp. 273-278Sonati, M.F., Costa, F.F., Hemoglobin Bart's in a Brazilian black population (1990) Brazilian Journal of Medical and Biological Research, 23, pp. 395-396Sonati, M.F., Farah, S.B., Ramalho, A.S., Costa, F.F., High prevalence of α-thalassemia in a Black population of Brazil (1991) Hemoglobin, 15, pp. 309-311Zago, M.A., Costa, F.F., Bottura, C., Hemoglobin H disease in three Brazilian families (1984) Revista Brasileira de Genética, 7, pp. 137-147Wenning, M.R.S.C., Kimura, E.M., Costa, F.F., Saad, S.T.O., Gervásio, S.A., De Jorge, S.B., Borges, E., Sonati, M.F., α-Globin genes: Thalassemic and structural alterations in a Brazilian population (2000) Brazilian Journal of Medical and Biological Research, 33, pp. 1041-1045Dodé, C., Krishnamoorthy, R., Lamb, J., Rochette, J., Rapid analysis of -α3.7 thalassaemia and αααanti 3.7 triplication by enzymatic amplification analysis (1993) British Journal of Haematology, 82, pp. 105-111Bowden, D.K., Vickers, M.A., Higgs, D.R., A PCR-based strategy to detect the common severe determinants of a thalassaemia (1992) British Journal of Haematology, 81, pp. 104-108Oron-Karni, V., Filon, D., Oppenheim, A., Rund, D., Rapid detection of the common Mediterranean α-globin deletions/rearrangements using PCR (1998) American Journal of Hematology, 58, pp. 306-310Hall, G.W., Thein, S.L., Newland, C.A., Chisholm, J.T.S., Kanavakis, E., Kattamis, C., Higgs, D.R., A base substitution (T→C) in codon 29 of the α2-globin gene causes α thalassemia (1993) British Journal of Haematology, 85, pp. 546-552Pearson, H.A., Ehrenkranz, R.A., Rinder, H.M., Hemosiderosis in a normal child secondary to oral iron medication (2000) Pediatrics, 105, pp. 429-43

The Genetics Of Blood Disorders: Hereditary Hemoglobinopathies

Objective: To summarize recently published data on the pathophysiology, diagnosis and treatment of sickle cell diseases and β-Thalassemias, the most relevant hereditary hemoglobinopathies in the global population. Sources: Searches were run on the MEDLINE and SCIELO databases, limited to the period from 2003 to May 2008, using the terms hereditary hemoglobinopathies, sickle cell diseases and β-thalassemia. Two books and two chapters were also included. Summary of the findings: More than 2,000 articles were identified; those providing the most important information and broadest views were selected. Conclusions: Morbidity and mortality rates from sickle cell diseases and β-thalassemia are still very high and represent an important challenge. Increased understanding of pathophysiological aspects has lead to significant improvements in treatment and prevention of these diseases. Copyright © 2008 by Sociedade Brasileira de Pediatria.844 SUPPL.S40S51Steiner, L.A., Gallagher, P.G., Erythrocyte disorders in the perinatal period (2007) Semin Perinatol, 31, pp. 254-261Tolentino, K., Friedman, J.F., An update on anemia in less developed countries (2007) Am J Trop Med Hyg, 77, pp. 44-51(2004) Wintrobe's clinical hematology, , Wintrobe MM, Foerster J, editors, 11th ed. Philadelphia: Lippincott Williams & Wilkins;Dhaliwal, G., Cornett, P.A., Tierney Jr., L.M., Hemolytic anemia (2004) Am Fam Physician, 69, pp. 2599-2606Madigan, C., Malik, P., Pathophysiology and therapy for haemoglobinopathies. Part I: Sickle cell disease (2006) Expert Rev Mol Med, 8, pp. 1-23Urbinati, F., Madigan, C., Malik, P., Pathophysiology and therapy for haemoglobinopathies. 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B-CAM/LU expression and the role of B-CAM/LU activation in binding of low- and high-density red cells to laminin in sickle cell disease. Am J Hematol. 2004;75:63-7Assis, A., Conran, N., Canalli, A.A., Lorand-Metze, I., Saad, S.T., Costa, F.F., Effect of cytokines and chemokines on sickle neutrophil adhesion to fibronectin (2005) Acta Haematol, 113, pp. 130-136Canalli, A.A., Costa, F.F., Saad, S.T., Conran, N., Granulocytic adhesive interactions and their role in sickle cell vaso-occlusion (2005) Hematology, 10, pp. 419-425Conran, N., Saad, S.T., Costa, F.F., Ikuta, T., Leukocyte numbers correlate with plasma levels of granulocyte-macrophage colony-stimulating factor in sickle cell disease (2007) Ann Hematol, 86, pp. 255-261Canalli, A.A., Franco-Penteado, C.F., Traina, F., Saad, S.T., Costa, F.F., Conran, N., Role for cAMP-protein kinase A signalling in augmented neutrophil adhesion and chemotaxis in sickle cell disease (2007) Eur J Haematol, 79, pp. 330-337Conran, N., Almeida, C.B., Lanaro, C., Ferreira, R.P., Traina, F., Saad, S.T., Inhibition of caspase-dependent spontaneous apoptosis via a cAMP-protein kinase A dependent pathway in neutrophils from sickle cell disease patients (2007) Br J Haematol, 139, pp. 148-158Canalli, A.A., Conran, N., Fattori, A., Saad, S.T., Costa, F.F., Increased adhesive properties of eosinophils in sickle cell disease (2004) Exp Hematol, 32, pp. 728-734Belcher, J.D., Bryant, C.J., Nguyen, J., Bowlin, P.R., Kielbik, M.C., Bischof, J.C., Transgenic sickle mice have vascular inflammation (2003) Blood, 101, pp. 3953-3959Perelman, N., Selvaraj, S.K., Batra, S., Luck, L.R., Erdreich-Epstein, A., Coates, T.D., Placenta growth factor activates monocytes and correlates with sickle cell disease severity (2003) Blood, 102, pp. 1506-1514Solovey, A., Kollander, R., Shet, A., Milbauer, L.C., Choong, S., Panoskaltsis-Mortari, A., Endothelial cell expression of tissue factor in sickle mice is augmented by hypoxia/reoxygenation and inhibited by lovastatin (2004) Blood, 104, pp. 840-846Aslan, M., Freeman, B.A., Redox-dependent impairment of vascular function in sickle cell disease (2007) Free Radic Biol Med, 43, pp. 1469-1483Conran, N., Gambero, A., Ferreira, H.H., Antunes, E., de Nucci, G., Nitric oxide has a role in regulating VLA-4-integrin expression on the human neutrophil cell surface (2003) Biochem Pharmacol, 66, pp. 43-50Kaul, D.K., Liu, X.D., Chang, H.Y., Nagel, R.L., Fabry, M.E., Effect of fetal hemoglobin on microvascular regulation in sickle transgenic-knockout mice (2004) J Clin Invest, 114, pp. 1136-1145Steinberg, M.H., Predicting clinical severity in sickle cell anaemia (2005) Br J Haematol, 129, pp. 465-481Fathallah, H., Atweh, G.F., (2006) Induction of fetal hemoglobin in the treatment of sickle cell disease, pp. 58-62. , Hematology Am Soc Hematol Educ ProgramAdorno, E.V., Zanette, A., Lyra, I., Souza, C.C., Santos, L.F., Menezes, J.F., The beta-globin gene cluster haplotypes in sickle cell anemia patients from Northeast Brazil: A clinical and molecular view (2004) Hemoglobin, 28, pp. 267-271Hoppe, C., Klitz, W., Noble, J., Vigil, L., Vichinsky, E., Styles, L., Distinct HLA associations by stroke subtype in children with sickle cell anemia (2003) Blood, 101, pp. 2865-2869Sebastiani, P., Ramoni, M.F., Nolan, V., Baldwin, C.T., Steinberg, M.H., Genetic dissection and prognostic modeling of overt stroke in sickle cell anemia (2005) Nat Genet, 37, pp. 435-440Castro, V., Alberto, F.L., Costa, R.N., Lepikson-Neto, J., Gualandro, S.F., Figueiredo, M.S., Polymorphism of the human platelet antigen-5 system is a risk factor for occlusive vascular complications in patients with sickle cell anemia (2004) Vox Sang, 87, pp. 118-123Steinberg, M.H., Adewoye, A.H., Modifier genes and sickle cell anemia (2006) Curr Opin Hematol, 13, pp. 131-136Kutlar, F., Diagnostic approach to hemoglobinopathies (2007) Hemoglobin, 31, pp. 243-250Colah, R.B., Surve, R., Sawant, P., D'Souza, E., Italia, K., Phanasgaonkar, S., HPLC studies in hemoglobinopathies (2007) Indian J Pediatr, 74, pp. 657-662Cremonesi, L., Ferrari, M., Giordano, P.C., Harteveld, C.L., Kleanthous, M., Papasavva, T., An overview of current microarray-based human globin gene mutation detection methods (2007) Hemoglobin, 31, pp. 289-311Kutlar, A., Sickle cell disease: A multigenic perspective of a single gene disorder (2007) Hemoglobin, 31, pp. 209-224Steinberg, M.H., Barton, F., Castro, O., Pegelow, C.H., Ballas, S.K., Kutlar, A., Effect of hydroxyurea on mortality and morbidity in adult sickle cell anemia: Risks and benefits up to 9 years of treatment (2003) JAMA, 289, pp. 1645-1651Davies, S.C., Gilmore, A., The role of hydroxyurea in the management of sickle cell disease (2003) Blood Rev, 17, pp. 99-109Conran, N., Fattori, A., Saad, S.T., Costa, F.F., Increased levels of soluble ICAM-1 in the plasma of sickle cell patients are reversed by hydroxyurea (2004) Am J Hematol, 76, pp. 343-347De Franceschi, L., Corrocher, R., Established and experimental treatments for sickle cell disease (2004) Haematologica, 89, pp. 348-356Gambero, S., Canalli, A.A., Traina, F., Albuquerque, D.M., Saad, S.T., Costa, F.F., Conran, N., Therapy with hydroxyurea is associated with reduced adhesion molecule gene and protein expression in sickle red cells with a concomitant reduction in adhesive properties (2007) Eur J Haematol, 78, pp. 144-151Haynes Jr, J., Obiako, B., Hester, R.B., Baliga, B.S., Stevens, T., Hydroxyurea attenuates activated neutrophil-mediated sickle erythrocyte membrane phosphatidylserine exposure and adhesion to pulmonary vascular endothelium (2008) Am J Physiol Heart Circ Physiol, 294, pp. H379-H385Johnson, C., Telen, M.J., Adhesion molecules and hydroxyurea in the pathophysiology of sickle cell disease (2008) Haematologica, 93, pp. 481-485Moreira, L.S., de Andrade, T.G., Albuquerque, D.M., Cunha, A.F., Fattori, A., Saad, S.T., Costa, F.F., Identification of differentially expressed genes induced by hydroxyurea in reticulocytes from sickle cell anaemia patients (2008) Clin Exp Pharmacol Physiol, 35, pp. 651-655Fattori, A., de Souza, R.A., Saad, S.T., Costa, F.F., Acute myocardial infarction in sickle cell disease: A possible complication of hydroxyurea treatment (2005) Hematol J, 5, pp. 589-590Steinberg, M.H., Brugnara, C., Pathophysiological-based approaches to treatment of sickle cell disease (2003) Annu Rev Med, 54, pp. 89-112Locatelli, F., Rocha, V., Reed, W., Bernaudin, F., Ertem, M., Grafakos, S., Related umbilical cord blood transplantation in patients with thalassemia and sickle cell disease (2003) Blood, 101, pp. 2137-2143Cheung, A.T., Chan, M.S., Ramanujam, S., Rangaswami, A., Curl, K., Franklin, P., Effects of poloxamer 188 treatment on sickle cell vaso-occlusive crisis: Computer-assisted intravital microscopy study (2004) J Investig Med, 52, pp. 402-406Makis, A.C., Hatzimichael, E.C., Stebbing, J., The genomics of new drugs in sickle cell disease (2006) Pharmacogenomics, 7, pp. 909-917Stocker, J.W., De Franceschi, L., McNaughton-Smith, G.A., Corrocher, R., Beuzard, Y., Brugnara, C., ICA-17043, a novel Gardos channel blocker, prevents sickled red blood cell dehydration in vitro and in vivo in SAD mice (2003) Blood, 101, pp. 2412-2418Mack, A.K., Kato, G.J., Sickle cell disease and nitric oxide: A paradigm shift? (2006) Int J Biochem Cell Biol, 38, pp. 1237-1243Machado, R.F., Martyr, S., Kato, G.J., Barst, R.J., Anthi, A., Robinson, M.R., Sildenafil therapy in patients with sickle cell disease and pulmonary hypertension (2005) Br J Haematol, 130, pp. 445-453Canalli, A.A., Franco-Penteado, C.F., Saad, S.T., Conran, N., Costa, F.F., Increased adhesive properties of neutrophils in sickle cell disease may be reversed by pharmacological nitric oxide donation (2008) Haematologica, 93, pp. 605-609Bank, A., On the road to gene therapy for beta-thalassemia and sickle cell anemia (2008) Pediatr Hematol Oncol, 25, pp. 1-4Shen, T.J., Rogers, H., Yu, X., Lin, F., Noguchi, C.T., Ho, C., Modification of globin gene expression by RNA targeting strategies (2007) Exp Hematol, 35, pp. 1209-1218Chang, J.C., Ye, L., Kan, Y.W., Correction of the sickle cell mutation in embryonic stem cells (2006) Proc Natl Acad Sci U S A, 103, pp. 1036-1040Wu, L.C., Sun, C.W., Ryan, T.M., Pawlik, K.M., Ren, J., Townes, T.M., Correction of sickle cell disease by homologous recombination in embryonic stem cells (2006) Blood, 108, pp. 1183-1188Birgens, H., Ljung, R., The thalassaemia syndromes (2007) Scand J Clin Lab Invest, 67, pp. 11-25Araújo, A.S., Silva, W.A., Leão, S.A., Bandeira, F.C., Petrou, M., Modell, B., A different molecular pattern of beta-thalassemia mutations in northeast (2003) Brazil. Hemoglobin, 27, pp. 211-217Taher, A., Isma'eel, H., Cappellini, M.D., Thalassemia intermedia: Revisited (2006) Blood Cells Mol Dis, 37, pp. 12-20Rund, D., Rachmilewitz, E., Beta-thalassemia (2005) N Engl J Med, 353, pp. 1135-1146Kuypers, F.A., de Jong, K., The role of phosphatidylserine in recognition and removal of erythrocytes (2004) Cell Mol Biol (Noisy-le-grand), 50, pp. 147-158Datta, P., Basu, S., Chakravarty, S.B., Chakravarty, A., Banerjee, D., Chandra, S., Enhanced oxidative cross-linking of hemoglobin E with spectrin and loss of erythrocyte membrane asymmetry in hemoglobin E beta-thalassemia (2006) Blood Cells Mol Dis, 37, pp. 77-81De Franceschi, L., Ronzoni, L., Cappellini, M.D., Cimmino, F., Siciliano, A., Alper, S.L., K-CL co-transport plays an important role in normal and beta thalassemic erythropoiesis (2007) Haematologica, 92, pp. 1319-1326Ayi, K., Turrini, F., Piga, A., Arese, P., Enhanced phagocytosis of ring-parasitized mutant erythrocytes: A common mechanism that may explain protection against falciparum malaria in sickle trait and beta-thalassemia trait (2004) Blood, 104, pp. 3364-3371Weiss, M.J., Zhou, S., Feng, L., Gell, D.A., Mackay, J.P., Shi, Y., Role of alpha-hemoglobin-stabilizing protein in normal erythropoiesis and beta-thalassemia (2005) Ann N Y Acad Sci, 1054, pp. 103-117Toumba, M., Sergis, A., Kanaris, C., Skordis, N., Endocrine complications in patients with Thalassaemia Major (2007) Pediatr Endocrinol Rev, 5, pp. 642-648Aessopos, A., Berdoukas, V., Tsironi, M., The heart in transfusion dependent homozygous thalassaemia today - prediction, prevention and management (2008) Eur J Haematol, 80, pp. 93-106Weizer-Stern, O., Adamsky, K., Amariglio, N., Levin, C., Koren, A., Breuer, W., Downregulation of hepcidin and haemojuvelin expression in the hepatocyte cell-line HepG2 induced by thalassaemic sera (2006) Br J Haematol, 135, pp. 129-138Tanno, T., Bhanu, N.V., Oneal, P.A., Goh, S.H., Staker, P., Lee, Y.T., High levels of GDF15 in thalassemia suppress expression of the iron regulatory protein hepcidin (2007) Nat Med, 13, pp. 1096-1101Cohen, A.R., Galanello, R., Pennell, D.J., Cunningham, M.J., Vichinsky, E., (2004) Thalassemia, pp. 14-34. , Hematology Am Soc Hematol Educ ProgramAtaga, K.I., Cappellini, M.D., Rachmilewitz, E.A., Beta-thalassaemia and sickle cell anaemia as paradigms of hypercoagulability (2007) Br J Haematol, 139, pp. 3-13Panigrahi, I., Agarwal, S., Thromboembolic complications in beta-thalassemia: Beyond the horizon (2007) Thromb Res, 120, pp. 783-789Amer, J., Fibach, E., N-acetylcysteine amide, a novel cell-permeating thiol, restores cellular glutathione and protects human red blood cells from oxidative stress (2005) Free Radic Biol Med, 38, pp. 136-145Pattanapanyasat, K., Gonwong, S., Chaichompoo, P., Noulsri, E., Lerdwana, S., Sukapirom, K., Activated platelet-derived microparticles in thalassaemia (2007) Br J Haematol, 136, pp. 462-471Weinstein, B.I., Erramouspe, B., Albuquerque, D.M., Oliveira, D.M., Kimura, E.M., Costa, F.F., Hb Florida: A novel elongated C-terminal beta-globin variant causing dominant beta-thalassemia phenotype (2006) Am J Hematol, 81, pp. 358-360Singh, S.P., Gupta, S., Molecular pathogenesis and clinical variability of homozygous beta 0-thalassemia in populations of Jammu region of J&K state (India) (2006) Hematology, 11, pp. 271-275Kimura, E.M., Grignoli, C.R., Pinheiro, V.R., Costa, F.F., Sonati, M.F., Thalassemia intermedia as a result of heterozygosis for beta 0-thalassemia and alpha alpha alpha anti-3,7 genotype in a Brazilian patient (2003) Braz J Med Biol Res, 36, pp. 699-701Bailey, L., Kuroyanagi, Y., Franco-Penteado, C.F., Conran, N., Costa, F.F., Ausenda, S., Expression of the gamma-globin gene is sustained by the cAMP-dependent pathway in beta-thalassaemia (2007) Br J Haematol, 138, pp. 382-395dos Santos, C.O., Costa, F.F., AHSP and beta-thalassemia: A possible genetic modifier (2005) Hematology, 10, pp. 157-161dos Santos, C.O., Zhou, S., Secolin, R., Wang, X., Cunha, A.F., Higgs, D.R., Population analysis of the alpha hemoglobin stabilizing protein (AHSP) gene identifies sequence variants that alter expression and function (2008) Am J Hematol, 83, pp. 103-108Franchini, M., Veneri, D., Iron-chelation therapy: An update (2004) Hematol J, 5, pp. 287-292Pootrakul, P., Sirankapracha, P., Sankote, J., Kachintorn, U., Maungsub, W., Sriphen, K., Clinical trial of deferiprone iron chelation therapy in beta-thalassaemia/haemoglobin E patients in Thailand (2003) Br J Haematol, 122, pp. 305-310Tsironi, 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, pp. 84-85Neufeld, E.J., Oral chelators deferasirox and deferiprone for transfusional iron overload in thalassemia major: New data, new questions (2006) Blood, 107, pp. 3436-3441Choudhry, V.P., Naithani, R., Current status of iron overload and chelation with deferasirox (2007) Indian J Pediatr, 74, pp. 759-764Yang, L.P., Keam, S.J., Keating, G.M., Deferasirox: A review of its use in the management of transfusional chronic iron overload (2007) Drugs, 67, pp. 2211-2230Rivera, S., Nemeth, E., Gabayan, V., Lopez, M.A., Farshidi, D., Ganz, T., Synthetic hepcidin causes rapid dose-dependent hypoferremia and is concentrated in ferroportin-containing organs (2005) Blood, 106, pp. 2196-2199Higgs, D.R., (2004) Gene regulation in hematopoiesis: New lessons from thalassemia, pp. 1-13. , Hematology Am Soc Hematol Educ ProgramWeatherall, D.J., Thalassaemia: The long road from bedside to genome (2004) Nat Rev Genet, 5, pp. 625-631Quek, L., Thein, S.L., Molecular therapies in beta-thalassaemia (2007) Br J Haematol, 136, pp. 353-365Dissayabutra, T., Tosukhowong, P., Seksan, P., The benefits of vitamin C and vitamin E in children with beta-thalassemia with high oxi

A Combination Of The -α3.7 And -medii Alleles Causing Hemoglobin H Disease In A Brazilian Patient

Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)39180832014/00984-3, FAPESP, Fundação de Amparo à Pesquisa do Estado de São PauloFundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP

Designing And Validating The Methodology For The Internet Assessment Of Fish Consumption At A University Setting

Assessing fish consumption is complex and involves several factors; however, the use of questionnaires in surveys and the use of the Internet as tool to collect data have been considered promising approaches. Therefore, the objective of this research was to design a data collection technique using a questionnaire to assess fish consumption by making it available on a specific home page on the Internet. A bibliographical survey or review was carried out to identify the features of the instrument, and therefore pre-tests were conducted with previous instruments, followed by the Focus Group technique. Specialists then performed an analysis and conducted an online pre-test. Multivariate data analysis was applied using the SmartPLS software. The results indicate that 1.966 participants belonging to the University of São Paulo (USP) community participated in the test, and after the exclusion of some variables, a statistically significant results were obtained. The final constructs comprised consumption, quality, and general characteristics. The instrument consisted of behavioral statements in a 5-point Likert scale and multiple-choice questions. The Cronbach's alpha reliability coefficient was 0.66 for general characteristics, 0.98 for quality, and 0.91 for consumption, which indicate good reliability of the instrument. In conclusion, the results proved that the Internet assessment is efficient. The instrument of analysis allowed us to better understand the process of buying and consuming fish in the country, and it can be used as base for further research.342315323Bargas-Avila, J.A., Lotscher, J., Orsini, S., Opwis, K., Intranet satisfaction questionnaire: Development and validation of a questionnaire to measure user satisfaction with the Intranet (2009) Computers In Human Behavior, 25 (6), pp. 1241-1250. , http://dx.doi.org/10.1016/j.chb.2009.05.014Batagelj, Z., Vehovar, V., Technical and methodological issues in surveys (1998) Proceedings of the 53° Annual Conference of the American Association For Public Opinion Research (AAPOR), , http://www.ris.org/ris98/stlouis, St. Louis. 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A. Marcoulides (Ed.), Mahwah: Lawrence Erlbaum AssociatesCronk, B.C., West, J.L., Personality research on the Internet: A comparison of web based and traditional instruments in take-home and in-class settings (2002) Behavior Research Methods Instruments and Computers, 34 (2), pp. 177-180. , http://dx.doi.org/10.3758/BF03195440Falk, R.F., Miller, N.B., (1992) A Primer For Soft Modeling, , New York: Akron Presshttp://www.brasilfoodtrends.com.br/Brasil_Food_Trends/index.html, Federação das Industrias do Estado de São Paulo-FIESP. (2010), Brasil food trends, São Paulo: FIESP. 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Porto Alegre: BookmanHanning, R.M.D., Royall, J.E., Toews, L., Blashill, L., Wegener, J., Driezen, P., Web-based food behaviour questionnaire: Validation with grades six to eight students (2009) Canadian Journal of Dietetic Practice and Research, 70, pp. 172-178. , http://dx.doi.org/10.3148/70.4.2009.172Hanscom, B., Lurie, J.D., Homa, K., Weinstein, J., Computerized questionnaires and the quality of survey data (2002) Spine, 27 (6), pp. 1797-1801. , http://dx.doi.org/10.1097/00007632-200208150-00020Hershberger, S.L., Marcoulides, G.A., Parramore, M.M., Structural equation modeling: An introduction (2003) Structural Equation Modeling: Applications In Ecological and Evolutionary Biology, , http://dx.doi.org/10.1017/CBO9780511542138.002, In B. H. Pugesek, A. Tomer & A. V. Eye (Eds.), Cambridge: Cambridge University PressKlem, L., Structural equation modeling (2006) Reading and Understanding More Multivariate Statistics, pp. 227-260. , In L. G. Grimm & P. R. Yarnold (Eds.), Washington: American Psychological AssociationMalhotra, N., (2008) Pesquisa De Marketing: Uma Orientação Aplicada, , 5. ed.). Porto Alegre: BookmanMaruayama, G.M., (1998) Basic of Structural Equation Modeling, , Thousand Oaks: Sage PublicationsMatthys, C., Pynaert, I., de Keyzer, W., Henauw, S., Validity and reproducibility of an adolescent Web-based food frequency questionnaire (2007) Journal of the American Dietetic Association, 107 (4), pp. 605-610. , http://dx.doi.org/10.1016/j.jada.2007.01.005Mina, K., Fritschi, L., Knuiman, M., A valid semiquantitative food frequency questionnaire to measure fish consumption (2007) European Journal of Clinical Nutrition, 61, pp. 1023-1031. , http://dx.doi.org/10.1038/sj.ejcn.1602617Morgan, D., (1997) Focus Group As Qualitative Research, pp. 80-88. , London: Sage Publications. Qualitative Research Methods Series 16Mueller, R.O., Structural equation modeling: Back to basics (1997) Structural Equation Modeling, 4 (4), pp. 353-369. , http://dx.doi.org/10.1080/10705519709540081Oetterer, M., (2002) Industrialização Do Pescado Cultivado, , Guaíba: AgropecuáriaPasquali, L., (2003) Psicometria: Teoria Dos Testes Na Psicologia E Na Educação, , Petrópolis: VozesPerez, G., Zwicker, R., Fatores determinantes da adoção de sistemas de informação na área de saúde: Um estudo sobre o prontuário médico eletrônico (2010) Revista De Administração Mackenzie, 11 (1), pp. 174-200. , http://dx.doi.org/10.1590/S1678-69712010000100008Ribeiro, R.C.L., Panato, E., Silva, M.M.S., Rosado, L.E.F., Rosado, G.P., Validade relativa de um questionário de freqüência alimentar para utilização em adultos (2009) Revista De Nutrição, 22 (1), pp. 81-95. , http://dx.doi.org/10.1590/S1415-52732009000100008Rodrigues, D.S., Ramos, R.A.R., Mendes, J.F.G., A system to evaluate and monitor quality of life in a University Campus (2005) Proceedings of the 9th International Conference On Computers In Urban Planning and Urban Management (CUPUM), , http://repositorium.sdum.uminho.pt/bitstream/1822/5064/1/Rodrigues_CI_1_2005.pdf, London. 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Universidade de São Paulo, PiracicabaSoriano, J.M., Molto, J.C., Manes, J., Dietary intake and food pattern among university students (2000) Nutrition Research, 20 (9), pp. 1249-1258. , http://dx.doi.org/10.1016/S0271-5317(00)00217-7Stanton, J.M., Rogelberg, S.G., Using Internet/intranet web pages to collect organizational research data (2001) Organizational Research Methods, 4 (3), pp. 200-217. , http://dx.doi.org/10.1177/109442810143002Touvier, M., Kesse-Guyot, E., Méjean, C., Pollet, C., Malon, A., Castetbon, K., Hercberg, S., Comparison between an interactive web-based self-administered 24 h dietary record and an interview by a dietitian for large-scale epidemiological studies (2001) British Journal of Nutrition, 105 (7), pp. 1055-1064. , http://dx.doi.org/10.1017/S0007114510004617Trondsen, T., Scholderer, J., Lund, E., Lund, E., Eggen, A., Perceived barriers to consumption of fish among Norwegian women (2003) Appetite, 41 (3), pp. 301-314. , http://dx.doi.org/10.1016/S0195-6663(03)00108-9(2010) Anuário Estatístico, , http://sistemas3.usp.br/anuario, Universidade de São Paulo-USP, Brasil: USP. Retrieved fromVanderslice, J., Laflamme, D.M., Wynkoop-Simmons, K., Comparing fish consumption information collected via a telephone survey and a food frequency questionnaire (2008) Epidemiology, 19 (6), pp. S315Veiga, L., Gondim, S.M.G., A utilização de métodos qualitativos na ciência política e no marketing político (2001) Opinião Pública, 2 (1), pp. 1-15Vinholis, M.M.B., Azevedo, P.F., Segurança do alimento e rastreabilidade: O caso BSE (2002) RAE Eletrônica, 1 (2), pp. 1-19. , http://dx.doi.org/10.1590/S1676-56482002000200008Zwicker, R., Souza, C.A., Bido, D.S., (2008) Uma Revisão Do Modelo Do Grau De Informatização De Empresas: Novas Propostas De Estimação E Modelagem Usando PLS (Partial Least Squares), , In Anais do 32° Encontro da ANPAD, Rio de Janeiro. CD-RO

The genetics of blood disorders: hereditary hemoglobinopathies

Abstract Objective: To summarize recently published data on the pathophysiology, diagnosis and treatment of sickle cell diseases and β-Thalassemias, the most relevant hereditary hemoglobinopathies in the global population. Sources: Searches were run on the MEDLINE and SCIELO databases, limited to the period from 2003 to May 2008, using the terms hereditary hemoglobinopathies, sickle cell diseases and β-thalassemia. Two books and two chapters were also included. Summary of the findings: More than 2,000 articles were identified; those providing the most important information and broadest views were selected. Conclusions: Morbidity and mortality rates from sickle cell diseases and β-thalassemia are still very high and represent an important challenge. Increased understanding of pathophysiological aspects has lead to significant improvements in treatment and prevention of these diseases. J Pediatr (Rio J). 2008;84(4 Suppl):S40-51: Hereditary hemoglobinopathies, sickle cell diseases, β-thalassemia

THE RELATIONSHIP BETWEEN RISK FACTORS FOR METABOLIC SYNDROME IN THE PHYSICALLY ACTIVE ELDERLY

O envelhecimento tem um impacto significativo na diminuição de massa magra e do nível de atividade física, estando relacionada à redução na taxa metabólica basal e ao aumento de sobrepeso e obesidade em idosos. O objetivo desse estudo foi identificar correlações entre os fatores de risco da Síndrome Metabólica, a medição do Perímetro Abdominal (PA) e Índice de Massa Corporal (IMC) com os parâmetros sanguíneos de Glicemia em Jejum (GJ), Colesterol Total (CT) e Triglicerídeos (TG), em idosos ativos. Para cálculo do IMC foram coletadas medidas de peso e altura e adotados os critérios de classificação da Organização Mundial da Saúde (OMS), a medida da perímetro abdominal (PA) foi efetuada em centímetros. Os parâmetros sanguíneos de glicemia de jejum (GJ), colesterol total (CT) e triglicérides (TGL) foram avaliados pelo método de coleta de sangue na ponta de dedo com leitura em equipamento Accutrend Plus (Roche). Para análise dos resultados foi realizado o cálculo do coeficiente de correlação de Pearson entre as medidas antropométricas (variáveis independentes) e parâmetros sanguíneos (variáveis dependentes). Regressão linear simples foi aplicada sobre as variáveis significativas (0.05%). A avaliação do IMC mostrou que 71% dos idosos estavam acima do peso e 34% eram obesos. No ponto de corte recomendada, 57% da amostra ter sido indicado por PA. O teste de correlação mostraram evidências sobre a existência de uma associação significativa entre GJ e PA e também entre PA e TG comparado ao uso do IMC. A medição de PA parece ser uma indicação eficaz da relação entre os factores de risco para o SM e deve ser incorporada rotinas para avaliar idosos como um indicador de obesidade abdominal.