M H Steinberg

Summary

Affiliation: Boston University
Country: USA

Publications

  1. ncbi Therapies to increase fetal hemoglobin in sickle cell disease
    Martin H Steinberg
    Boston University School of Medicine, Room E211, 88 East Newton Street, Boston, MA 02118, USA
    Curr Hematol Rep 2:95-101. 2003
  2. ncbi Hydroxyurea treatment for sickle cell disease
    Martin H Steinberg
    Boston University School of Medicine, 88 E Newton St, Boston, MA 02118, USA
    ScientificWorldJournal 2:1706-28. 2002
  3. ncbi Effect of hydroxyurea on mortality and morbidity in adult sickle cell anemia: risks and benefits up to 9 years of treatment
    Martin H Steinberg
    Boston University School of Medicine, Center of Excellence in Sickle Cell Disease, Boston Medical Center, Boston, Mass 02118, USA
    JAMA 289:1645-51. 2003
  4. ncbi Concordant fetal hemoglobin response to hydroxyurea in siblings with sickle cell disease
    Martin H Steinberg
    Boston University School of Medicine, Room 211, 88 E Newton Street, Boston, MA 02118, USA
    Am J Hematol 72:121-6. 2003
  5. ncbi Pathophysiological-based approaches to treatment of sickle cell disease
    Martin H Steinberg
    Department of Medicine and Pediatrics, Boston University School of Medicine, 88 E Newton Street, Boston, Massachusetts 02118, USA
    Annu Rev Med 54:89-112. 2003
  6. ncbi Developing treatment for sickle cell disease
    Martin H Steinberg
    Boston University School of Medicine, Boston, Massachusetts 02118, USA
    Expert Opin Investig Drugs 11:645-59. 2002
  7. ncbi Modulation of fetal hemoglobin in sickle cell anemia
    M H Steinberg
    G V Sonny Montgomery Department of Veterans Affairs Medical Center, Jackson, MS 39216, USA
    Hemoglobin 25:195-211. 2001
  8. ncbi Fetal hemoglobin in sickle cell anemia: genetic determinants of response to hydroxyurea
    Q Ma
    Department of Medicine, Boston University School of Medicine, Boston, MA, USA
    Pharmacogenomics J 7:386-94. 2007
  9. ncbi Predicting clinical severity in sickle cell anaemia
    M H Steinberg
    Department of Medicine, Boston University School of Medicine and the Center of Excellence in Sickle Cell Disease, Boston Medical Center, 88 E Newton Street, Boston, MA 02118, USA
    Br J Haematol 129:465-81. 2005
  10. ncbi Genetic dissection and prognostic modeling of overt stroke in sickle cell anemia
    Paola Sebastiani
    Department of Biostatistics, Boston University School of Public Health, Boston, Massachusetts 02118, USA
    Nat Genet 37:435-40. 2005

Detail Information

Publications65

  1. ncbi Therapies to increase fetal hemoglobin in sickle cell disease
    Martin H Steinberg
    Boston University School of Medicine, Room E211, 88 East Newton Street, Boston, MA 02118, USA
    Curr Hematol Rep 2:95-101. 2003
    ..Its use in young children and in combination with other classes of HbF-inducing agents is being studied...
  2. ncbi Hydroxyurea treatment for sickle cell disease
    Martin H Steinberg
    Boston University School of Medicine, 88 E Newton St, Boston, MA 02118, USA
    ScientificWorldJournal 2:1706-28. 2002
    ..Still, its effects are inconsistent, trials in infants and children are ongoing, and its ultimate value--and peril--when started early in life are still unknown...
  3. ncbi Effect of hydroxyurea on mortality and morbidity in adult sickle cell anemia: risks and benefits up to 9 years of treatment
    Martin H Steinberg
    Boston University School of Medicine, Center of Excellence in Sickle Cell Disease, Boston Medical Center, Boston, Mass 02118, USA
    JAMA 289:1645-51. 2003
    ..Hydroxyurea increases levels of fetal hemoglobin (HbF) and decreases morbidity from vaso-occlusive complications in patients with sickle cell anemia (SCA). High HbF levels reduce morbidity and mortality...
  4. ncbi Concordant fetal hemoglobin response to hydroxyurea in siblings with sickle cell disease
    Martin H Steinberg
    Boston University School of Medicine, Room 211, 88 E Newton Street, Boston, MA 02118, USA
    Am J Hematol 72:121-6. 2003
    ..Our results provide additional evidence that some elements that regulate HbF expression are linked to the beta-globin gene cluster...
  5. ncbi Pathophysiological-based approaches to treatment of sickle cell disease
    Martin H Steinberg
    Department of Medicine and Pediatrics, Boston University School of Medicine, 88 E Newton Street, Boston, Massachusetts 02118, USA
    Annu Rev Med 54:89-112. 2003
    ..Future treatment prospects include gene therapy, interruption of the interaction of sickle cells with the endothelium, inhibition of oxidative damage, and protection of an injured endothelium...
  6. ncbi Developing treatment for sickle cell disease
    Martin H Steinberg
    Boston University School of Medicine, Boston, Massachusetts 02118, USA
    Expert Opin Investig Drugs 11:645-59. 2002
    ..Pharmacological treatment of the disease is focused on the inhibition of sickle haemoglobin polymerisation, prevention or repair of red cell dehydration and interruption of the interaction of sickle cells with the endothelium...
  7. ncbi Modulation of fetal hemoglobin in sickle cell anemia
    M H Steinberg
    G V Sonny Montgomery Department of Veterans Affairs Medical Center, Jackson, MS 39216, USA
    Hemoglobin 25:195-211. 2001
    ..Although this can be accomplished clinically with drugs like hydroxyurea, a complete understanding of the molecular and cellular basis of fetal hemoglobin regulation may suggest new and better ways of attaining this goal...
  8. ncbi Fetal hemoglobin in sickle cell anemia: genetic determinants of response to hydroxyurea
    Q Ma
    Department of Medicine, Boston University School of Medicine, Boston, MA, USA
    Pharmacogenomics J 7:386-94. 2007
    ..Polymorphisms in genes regulating HbF expression, HU metabolism and erythroid progenitor proliferation might modulate the patient response to HU...
  9. ncbi Predicting clinical severity in sickle cell anaemia
    M H Steinberg
    Department of Medicine, Boston University School of Medicine and the Center of Excellence in Sickle Cell Disease, Boston Medical Center, 88 E Newton Street, Boston, MA 02118, USA
    Br J Haematol 129:465-81. 2005
    ....
  10. ncbi Genetic dissection and prognostic modeling of overt stroke in sickle cell anemia
    Paola Sebastiani
    Department of Biostatistics, Boston University School of Public Health, Boston, Massachusetts 02118, USA
    Nat Genet 37:435-40. 2005
    ..We validated this model in a different population by predicting the occurrence of stroke in 114 individuals with 98.2% accuracy...
  11. ncbi Association of polymorphisms of IGF1R and genes in the transforming growth factor- beta /bone morphogenetic protein pathway with bacteremia in sickle cell anemia
    Adeboye H Adewoye
    Department of Medicine, Boston University School of Medicine, Boston, Massachusetts, USA
    Clin Infect Dis 43:593-8. 2006
    ..We suggest that both IGF1R and the TGF-beta /BMP pathway could play important roles in immune function in sickle cell anemia and their polymorphisms may help identify a "bacteremia-prone" phenotype...
  12. ncbi Sickle cell leg ulcers: associations with haemolysis and SNPs in Klotho, TEK and genes of the TGF-beta/BMP pathway
    Vikki G Nolan
    Department of Medicine, Boston University School of Medicine, Boston, MA, USA
    Br J Haematol 133:570-8. 2006
    ..Haemolysis-driven phenotypes, such as leg ulcers, could be improved by agents that reduce sickle erythrocyte density or increase NO bioavailability...
  13. ncbi Estimated glomerular filtration rate in sickle cell anemia is associated with polymorphisms of bone morphogenetic protein receptor 1B
    Vikki G Nolan
    Department of Medicine, Boston University School of Medicine, Boston, Massachusetts 02118, USA
    Am J Hematol 82:179-84. 2007
    ..Our results suggest that, as with other subphenotypes of sickle cell disease, renal function may be genetically modulated...
  14. ncbi Association of single nucleotide polymorphisms in klotho with priapism in sickle cell anaemia
    Vikki G Nolan
    Department of Medicine, Boston University School of Medicine, Boston, MA, USA
    Br J Haematol 128:266-72. 2005
    ..These findings may have broader implications in sickle cell disease, as KL encodes a membrane protein that regulates many vascular functions, including vascular endothelial growth factor expression and endothelial nitric oxide release...
  15. ncbi Abnormal pulmonary function in adults with sickle cell anemia
    Elizabeth S Klings
    Pulmonary Center, Department of Medicine, Boston Comprehensive Sickle Cell Center, Boston University School of Medicine and School of Public Health, Boston, MA 02118, USA
    Am J Respir Crit Care Med 173:1264-9. 2006
    ..Pulmonary complications of sickle cell anemia (Hb-SS) commonly cause morbidity, yet few large studies of pulmonary function tests (PFTs) in this population have been reported...
  16. ncbi Association of klotho, bone morphogenic protein 6, and annexin A2 polymorphisms with sickle cell osteonecrosis
    Clinton Baldwin
    Center for Human Genetics, Department of Pediatrics, Boston University School of Medicine, 715 Albany St, W408, Boston, MA 02118, USA
    Blood 106:372-5. 2005
    ..Our results may provide insight into the pathogenesis of osteonecrosis in sickle cell disease, help identify individuals who are at high risk for osteonecrosis, and thus allow earlier and more effective therapeutic intervention...
  17. ncbi A hierarchical and modular approach to the discovery of robust associations in genome-wide association studies from pooled DNA samples
    Paola Sebastiani
    Department of Biostatistics, Boston University School of Public Health, Boston 02118 MA, USA
    BMC Genet 9:6. 2008
    ..One of the challenges of the analysis of pooling-based genome wide association studies is to identify authentic associations among potentially thousands of false positive associations...
  18. ncbi Differential gene expression in pulmonary artery endothelial cells exposed to sickle cell plasma
    Elizabeth S Klings
    The Pulmonary Center, Boston University School of Public Health, Boston, Massachusetts 02118, USA
    Physiol Genomics 21:293-8. 2005
    ..An altered EC phenotype elicited by SCD plasma may contribute to the pathogenesis of sickle vasoocclusion...
  19. ncbi Genetic etiologies for phenotypic diversity in sickle cell anemia
    Martin H Steinberg
    Boston University School of Medicine, Boston, MA 02118, USA
    ScientificWorldJournal 9:46-67. 2009
    ..Genetic association studies can have immediate prognostic value; they might also help to identify new pathophysiological pathways that could be susceptible to modulation...
  20. ncbi Fetal hemoglobin in sickle cell anemia: Bayesian modeling of genetic associations
    Paola Sebastiani
    Department of Biostatistics, Boston University School of Public Heath, Boston, Massachusetts 02118, USA
    Am J Hematol 83:189-95. 2008
    ..By stratifying patients by age, our results also suggest that different genes might modulate the rate of decline of HbF and the final level of HbF levels in sickle cell anemia...
  21. ncbi RNA editing genes associated with extreme old age in humans and with lifespan in C. elegans
    Paola Sebastiani
    Department of Biostatistics, Boston University School of Public Health, Boston, Massachusetts, United States of America
    PLoS ONE 4:e8210. 2009
    ..The majority of genes found thus far to be associated with longevity primarily function in lipoprotein metabolism and insulin/IGF-1 signaling. There are likely many more genetic modifiers of human longevity that remain to be discovered...
  22. ncbi Genetic modifiers of the severity of sickle cell anemia identified through a genome-wide association study
    Paola Sebastiani
    Department of Biostatistics, Boston University School of Public Health, Boston, MA, USA
    Am J Hematol 85:29-35. 2010
    ..Additional validation, resequencing, and functional studies to understand the biology and reveal mechanisms by which candidate genes might have their effects are the future goals of this work...
  23. ncbi Lacrimal gland enlargement in sickle cell disease
    Adeboye H Adewoye
    Department of Medicine and The Center of Excellence in Sickle Cell Disease, Boston University School of Medicine, Boston, MA 02118, USA
    Am J Hematol 81:888-9. 2006
    ....
  24. ncbi Pulmonary arterial hypertension and left-sided heart disease in sickle cell disease: clinical characteristics and association with soluble adhesion molecule expression
    Elizabeth S Klings
    The Pulmonary Center, Boston University School of Medicine, Boston, Massachusetts 02118, USA
    Am J Hematol 83:547-53. 2008
    ....
  25. ncbi Modifier genes and sickle cell anemia
    Martin H Steinberg
    Department of Medicine, Boston University School of Medicine and the Center of Excellence in Sickle Cell Disease, Boston Medical Center, Boston, Massachussetts 02118, USA
    Curr Opin Hematol 13:131-6. 2006
    ..This review highlights genetic polymorphisms that have provided insight into the pathophysiology underlying the many phenotypes of sickle cell disease...
  26. ncbi Pathophysiologically based drug treatment of sickle cell disease
    Martin H Steinberg
    Center of Excellence in Sickle Cell Disease, E248, Boston Medical Center, 88 E Newton Street, Boston, MA 02118, USA
    Trends Pharmacol Sci 27:204-10. 2006
    ..A therapeutic approach that targets several sites of pathobiology might be most promising...
  27. ncbi Clustering by genetic ancestry using genome-wide SNP data
    Nadia Solovieff
    Department of Biostatistics, Boston University School of Public Health, Boston, MA 02118, USA
    BMC Genet 11:108. 2010
    ..An alternative solution is genetic matching of cases and controls that requires, however, well defined population strata for appropriate selection of cases and controls...
  28. ncbi Genome-wide association studies and the genetic dissection of complex traits
    Paola Sebastiani
    Department of Biostatistics, Boston University School of Public Health, Boston, Massachusetts 02118, USA
    Am J Hematol 84:504-15. 2009
    ..In this article, we will review the common approach to analysis of GWAS data and then discuss options to learn more from these data. We will use examples from our ongoing studies of sickle cell anemia and also GWAS in multigenic traits...
  29. ncbi Effect of sodium butyrate on lung vascular TNFSF15 (TL1A) expression: differential expression patterns in pulmonary artery and microvascular endothelial cells
    Surinder Safaya
    Center of Excellence in Sickle Cell Disease and Division of Hematology Oncology, 88 East Newton St, Boston, MA 02118, USA
    Cytokine 46:72-8. 2009
    ..The dual effects of butyrate-dependant TNFSF15 regulation in lung endothelium may help in identify inflammatory pathways and understand the role of HMVEC in pathogenesis of vasoocclusion in SCD...
  30. ncbi Sickle cell anemia, the first molecular disease: overview of molecular etiology, pathophysiology, and therapeutic approaches
    Martin H Steinberg
    Boston University School of Medicine, Boston, MA 02118, USA
    ScientificWorldJournal 8:1295-324. 2008
    ..Its complex pathophysiology, of which we have a reasonable understanding, provides multiple loci for potential therapeutic intervention...
  31. ncbi Association between wind speed and the occurrence of sickle cell acute painful episodes: results of a case-crossover study
    Vikki G Nolan
    Department of Epidemiology, Boston University School of Public Health, Boston, MA 02118, USA
    Br J Haematol 143:433-8. 2008
    ....
  32. ncbi The risks and benefits of long-term use of hydroxyurea in sickle cell anemia: A 17.5 year follow-up
    Martin H Steinberg
    Department of Medicine, Boston University School of Medicine, Boston Medical Center, Massachusetts, USA
    Am J Hematol 85:403-8. 2010
    ..Our results, while no longer the product of a randomized study because of the ethical concerns of withholding an efficacious treatment, suggest that long-term use of hydroxyurea is safe and might decrease mortality...
  33. ncbi Pathophysiology of sickle cell disease: role of cellular and genetic modifiers
    M H Steinberg
    Department of Medicine, Boston University School of Medicine, Boston, MA, USA
    Semin Hematol 38:299-306. 2001
    ..We review the "classic" aspects of the pathophysiology of sickle cell disease and focus on known and potential modulators of the phenotype of this disorder...
  34. ncbi BCL11A is a major HbF quantitative trait locus in three different populations with beta-hemoglobinopathies
    Amanda E Sedgewick
    Department of Biostatistics, Boston University School of Public Health, Boston, Massachusetts 02118, USA
    Blood Cells Mol Dis 41:255-8. 2008
    ..Taken together, the data suggest that the functional motifs responsible for modulating F-cells and HbF levels reside within a 3 kb region in the second intron of BCL11A...
  35. ncbi Sickle cell disease due to compound heterozygosity for Hb S and a novel 7.7-kb beta-globin gene deletion
    B Anders R Andersson
    Hemoglobin Diagnostic Reference Laboratory, Boston Medical Center, Boston, MA 02118, USA
    Eur J Haematol 78:82-5. 2007
    ..This is the second known deletion that removes the 3'-end but preserves the integrity of the 5'-end of the beta-globin gene. Furthermore, the identification of the deletion allows proper genetic counseling for affected families...
  36. ncbi A novel sickle hemoglobin: hemoglobin S-south end
    Hong-yuan Luo
    Hemoglobin Diagnostic Reference Laboratory, Boston Medical Center, Boston, Massachusetts, USA
    J Pediatr Hematol Oncol 26:773-6. 2004
    ..Furthermore, the variant hemoglobin mimics Hb A on high-pressure liquid chromatography, and its identity is not easily diagnosed. A succinct review of variant sickle hemoglobins is also presented...
  37. ncbi Variation and heritability of Hb F and F-cells among beta-thalassemia heterozygotes in Hong Kong
    Geoffrey T Gibney
    Division of Hematology Oncology, Department of Medicine, Boston University, Boston, Massachusetts02118, USA
    Am J Hematol 83:458-64. 2008
    ....
  38. ncbi Identification of oxidative post-translational modification of serum albumin in patients with idiopathic pulmonary arterial hypertension and pulmonary hypertension of sickle cell anemia
    Adam Odhiambo
    The Pulmonary Center, Boston University School of Medicine, Boston, MA 02118, USA
    Rapid Commun Mass Spectrom 21:2195-203. 2007
    ..This finding supports the notion that oxidative stress modulates the pathogenesis of PH of SCA and suggests that this and other post-translational modifications may be important biomarkers of disease...
  39. ncbi A T-to-G transversion at nucleotide -567 upstream of HBG2 in a GATA-1 binding motif is associated with elevated hemoglobin F
    Zhiyi Chen
    Center of Excellence in Sickle Cell Disease, Division of Hematology Oncology, Department of Medicine, Boston University School of Medicine, Boston, Massachusetts 02118, USA
    Mol Cell Biol 28:4386-93. 2008
    ..The T-to-G mutation in this motif disrupts GATA-1 binding and the associated repressor complex, abolishing its silencing effect and resulting in the up-regulation of gamma-globin gene expression in adults...
  40. ncbi Gene expression profiling during erythroid differentiation of K562 cells
    T Mitchell
    G.V. (Sonny) Montgomery Department of Veterans Affairs Medical Center, University of Mississippi School of Medicine, Jackson, Mississippi 39216, USA
    Blood Cells Mol Dis 27:309-19. 2001
    ..Some differentially expressed clones were transcription factors and 25 expressed fragments with open reading frames were found whose function remains unknown...
  41. ncbi Dominantly inherited beta thalassaemia intermedia caused by a new single nucleotide deletion in exon 2 of the beta globin gene: Hb morgantown (beta91 CTG>CG)
    H-Y Luo
    Hemoglobin Diagnostic Reference Laboratory, Boston Medical Center, Boston University School of Medicine, Boston, MA 02118, USA
    J Clin Pathol 58:1110-2. 2005
    ..This study highlights the importance of considering dominantly inherited beta thalassemia in the investigation of anaemia, even in patients with ethnic backgrounds not usually associated with beta thalassaemia...
  42. ncbi Polymorphisms near a chromosome 6q QTL area are associated with modulation of fetal hemoglobin levels in sickle cell anemia
    D F Wyszynski
    Department of Medicine, Genetics Program, Center of Excellence in Sickle Cell Disease, E211, Boston Medical Center, 88 E. Newton Street, Boston, MA 02118, USA
    Cell Mol Biol (Noisy-le-grand) 50:23-33. 2004
    ..Genetic elements abutting the 6q22.3-q23.2 QTL, may harbor trans-acting elements that help modulate baseline HbF level in sickle cell anemia...
  43. ncbi Sickle cell anemia is associated with reduced nitric oxide bioactivity in peripheral conduit and resistance vessels
    Robert T Eberhardt
    Evans Department of Medicine, Boston University School of Medicine, Boston, Massachusetts 02118 2393, USA
    Am J Hematol 74:104-11. 2003
    ..Resistance vessels have preserved response to exogenous NO donors but have diminished contribution of NO to endothelium-dependent vasodilation. Conduit vessels demonstrate impaired vasodilation to endogenous and exogenous NO...
  44. ncbi Hemolysis-associated priapism in sickle cell disease
    Vikki G Nolan
    Department of Medicine, Boston University School of Medicine, MA, USA
    Blood 106:3264-7. 2005
    ..These findings suggest an association of priapism with increased hemolysis. Hemolysis decreases the availability of circulating nitric oxide, which plays an important role in erectile function...
  45. ncbi Sickle cell bone disease: response to vitamin D and calcium
    Adeboye H Adewoye
    The Center of Excellence in Sickle Cell Disease and the Vitamin D, Skin and Bone Research Laboratories, Boston University School of Medicine, Boston, MA 02118, USA
    Am J Hematol 83:271-4. 2008
    ..Treatment of adult SCD with vitamin D and calcium can restore 25(OH)D levels to normal and improve BMD, but, markers of bone resorption remained unchanged. Screening for vitamin D deficiency and BMD in SCD patients seems warranted...
  46. ncbi BCL11A represses HBG transcription in K562 cells
    Zhiyi Chen
    Center of Excellence in Sickle Cell Disease, Division of Hematology Oncology, Department of Medicine, Boston University School of Medicine, Boston, MA 02118, USA
    Blood Cells Mol Dis 42:144-9. 2009
    ....
  47. ncbi Hemoglobinopathies mimicking Hb S/beta-thalassemia: Hb S/S with alpha-thalassemia and Hb S/Volga
    Hong-yuan Luo
    Hemoglobin Diagnostic Reference Laboratory, Boston Medical Center, Boston, Massachusetts 02118, USA
    Am J Hematol 81:361-5. 2006
    ..These studies underscore the importance to correlate clinical course with laboratory diagnosis and to make DNA-based diagnostics more widely available for patients with unusual or complicated hemoglobin disorders...
  48. ncbi Hemoglobin SE disease: a concise review
    David Masiello
    The Center of Excellence in Sickle Cell Disease, Department of Medicine, School of Medicine, Boston University, Boston, MA, USA
    Am J Hematol 82:643-9. 2007
    ..Patients with Hb SE disease should be followed and managed in a similar fashion as those with Hb S/beta(+)-thalassemia, and treated appropriately when they develop sickling-related symptoms and complications...
  49. ncbi Hemoglobin Kenya composed of alpha- and ((A)gammabeta)-fusion-globin chains, associated with hereditary persistence of fetal hemoglobin
    Ibifiri Wilcox
    Hemoglobin Diagnostic Reference Laboratory, Boston Medical Center, Boston, MA 02118, USA
    Am J Hematol 84:55-8. 2009
    ..Definitive diagnosis that is necessary for proper patient management is best done by DNA-based gap-PCR tests...
  50. ncbi Sickle cell vaso-occlusive crisis induces the release of circulating serum heat shock protein-70
    Adeboye H Adewoye
    Center for Excellence in Sickle Cell Disease, Boston University School of Medicine, Boston, Massachusetts 02118, USA
    Am J Hematol 78:240-2. 2005
    ..05) and a significant increase in Hsp70 levels in SCD at baseline compared with normal controls (P <0.05). Taken together, these results indicate that circulating serum Hsp70 might be a marker for VOC in SCD...
  51. ncbi Bone marrow transplantation in sickle cell disease: indications and successes
    Martin H Steinberg
    Boston University Medical Campus, Boston, MA 02118, USA
    Clin Adv Hematol Oncol 1:406-7. 2003
  52. ncbi Hb Hope [beta136(H14)Gly-->Asp (GGT-->GAT)]: interactions with Hb S [beta6(A3)Glu-->Val (GAG-->GTG)], other variant hemoglobins and thalassemia
    John Ingle
    Department of Medicine and Pathology, Division of Hematology/Oncology, Boston University School of Medicine, Boston, Massachusetts, USA
    Hemoglobin 28:277-85. 2004
    ..DNA-based diagnostics can help solve this potential problem...
  53. ncbi Two new alpha-thalassemia frameshift mutations
    Hong Yuang Luo
    Hemoglobin Diagnostic Reference Laboratory, Boston Medical Center, Boston, Massachusetts 02118, USA
    Hemoglobin 31:135-9. 2007
    ....
  54. ncbi Hemoglobin Titusville, a low oxygen affinity variant hemoglobin, in a family of Northern European background
    Hong-yuan Luo
    Hemoglobin Diagnostic Reference Laboratory, Boston Medical Center, Boston, Massachusetts 02118, USA
    Am J Hematol 77:384-6. 2004
    ..Correct diagnosis is clinically important to spare affected individuals extensive investigations into other causes of low oxygen saturation in peripheral blood...
  55. ncbi A network model to predict the risk of death in sickle cell disease
    Paola Sebastiani
    Boston University School of Public Health, MA 02118, USA
    Blood 110:2727-35. 2007
    ..The severity score could serve as an estimate of overall disease severity in genotype-phenotype association studies, and the model provides an additional method to study the complex pathophysiology of sickle cell disease...
  56. ncbi Differential modulation of endotoxin responsiveness by human caspase-12 polymorphisms
    Maya Saleh
    Department of Biochemistry, Molecular Biology and Pharmacology, Merck Frosst Centre for Therapeutic Research, Montreal, Quebec H9H 3L1, Canada
    Nature 429:75-9. 2004
    ..Thus, Csp12-L attenuates the inflammatory and innate immune response to endotoxins and in doing so may constitute a risk factor for developing sepsis...
  57. ncbi The paradox of hemoglobin SC disease
    Ronald L Nagel
    Division of Hematology, Department of Medicine, Albert Einstein College of Medicine and Montefiore Medical Center, The Bronx, NY, USA
    Blood Rev 17:167-78. 2003
    ..This situation offers a unique opportunity: if we could inhibit the effect of HbC on K(+) transport we can cure the disease...
  58. ncbi Chronic hyper-hemolysis in sickle cell anemia: association of vascular complications and mortality with less frequent vasoocclusive pain
    James G Taylor
    Pulmonary and Vascular Medicine Branch, National Heart Lung and Blood Institute, National Institutes of Health, Bethesda, Maryland, United States of America
    PLoS ONE 3:e2095. 2008
    ..We have previously reported that intense hemolysis is associated with increased risk of vascular complications in a small cohort of adults with sickle cell disease. These observations have not been validated in other populations...
  59. ncbi N-terminal pro-brain natriuretic peptide levels and risk of death in sickle cell disease
    Roberto F Machado
    Vascular Medicine Branch, Clinical Center, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20892 1454, USA
    JAMA 296:310-8. 2006
    ..Levels of N-terminal pro-brain natriuretic peptide (NT-proBNP) provide such information in patients with idiopathic pulmonary arterial hypertension...
  60. ncbi Deconstructing sickle cell disease: reappraisal of the role of hemolysis in the development of clinical subphenotypes
    Gregory J Kato
    Vascular Medicine Branch, National Heart, Lung and Blood Institute, Critical Care Medicine Department, Clinical Center, National Institutes of Health, 10 Center Drive, Building 10CRC 5 5140, Bethesda, MD 20892 1476, USA
    Blood Rev 21:37-47. 2007
    ..Some of these drugs are now being studied in clinical trials...
  61. ncbi Erythrocyte glutamine depletion, altered redox environment, and pulmonary hypertension in sickle cell disease
    Claudia R Morris
    Department of Emergency Medicine, Children s Hospital and Research Center Oakland, 747 52nd St, Oakland, CA 94609, USA
    Blood 111:402-10. 2008
    ..Decreased erythrocyte glutathione and glutamine levels contribute to alterations in the erythrocyte redox environment, which may compromise erythrocyte integrity, contribute to hemolysis, and play a role in the pathogenesis of PH of SCD...
  62. ncbi Clinical trials in sickle cell disease: adopting the combination chemotherapy paradigm
    Martin H Steinberg
    Am J Hematol 83:1-3. 2008
  63. ncbi Patients with thalassemia in the United States
    Hong-yuan Luo
    Blood 105:4896-7. 2005
  64. ncbi Effectiveness of a dedicated day hospital for management of acute sickle cell pain
    Adeboye H Adewoye
    Haematologica 92:854-5. 2007
    ..1 We report on the benefit of treating acute pain in SCD in a day hospital (DH)...
  65. ncbi Pneumococcus and sickle cell disease: the beginning of the end?
    Martin H Steinberg
    Clin Infect Dis 44:1434-5. 2007