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Nugent, Diane Jean

Title(s)Health Sciences Professor, Pediatrics
Phone(714) 509-8459
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    Collapse Research 
    Collapse Research Activities and Funding
    Preventing Bleeding Disorder Complications thru Hemophilia Tx Ctrs in Region IX
    CDC/NCBDD U01DD000204Sep 30, 2006 - Sep 29, 2011
    Role: Principal Investigator
    PATHOGENESIS OF AUTOIMMUNE THROMBOCYTOPENIA
    NIH/NHLBI R01HL061846Sep 30, 1998 - Feb 28, 2004
    Role: Principal Investigator
    PLATELET AUTOANTIGENS
    NIH/NHLBI K08HL001649Feb 1, 1986 - Jan 31, 1991
    Role: Principal Investigator
    HUMAN MONOCLONAL ANTIBODIES
    NIH/NIDDK R01DK033345Jan 1, 1984 - Jun 30, 1995
    Role: Principal Investigator

    Collapse Bibliographic 
    Collapse Publications
    Publications listed below are automatically derived from MEDLINE/PubMed and other sources, which might result in incorrect or missing publications. Researchers can login to make corrections and additions, or contact us for help.
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    1. Buchbinder D, Hauck F, Albert MH, Rack A, Bakhtiar S, Shcherbina A, Deripapa E, Sullivan KE, Perelygina L, Eloit M, Neven B, Pérot P, Moshous D, Suarez F, Bodemer C, Bonilla FA, Vaz LE, Krol AL, Klein C, Seppanen M, Nugent DJ, Singh J, Ochs HD. Rubella Virus-Associated Cutaneous Granulomatous Disease: a Unique Complication in Immune-Deficient Patients, Not Limited to DNA Repair Disorders. J Clin Immunol. 2019 Jan 03. PMID: 30607663.
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    2. Nugent DJ, Romano AA, Sabharwal S, Cooper DL. Evaluation of bleeding disorders in patients with Noonan syndrome: a systematic review. J Blood Med. 2018; 9:185-192. PMID: 30464668.
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    3. Nugent D, O'Mahony B, Dolan G. Value of prophylaxis vs on-demand treatment: Application of a value framework in hemophilia. Haemophilia. 2018 Sep; 24(5):755-765. PMID: 30088692.
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    4. Buchbinder D, Seppanen M, Rao VK, Uzel G, Nugent D. Clinical Challenges: Identification of Patients With Novel Primary Immunodeficiency Syndromes. J Pediatr Hematol Oncol. 2018 Jul; 40(5):e319-e322. PMID: 29200144.
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    5. Carcao M, Altisent C, Castaman G, Fukutake K, Kerlin BA, Kessler C, Lassila R, Nugent D, Oldenburg J, Garly ML, Rosholm A, Inbal A. Recombinant FXIII (rFXIII-A2) Prophylaxis Prevents Bleeding and Allows for Surgery in Patients with Congenital FXIII A-Subunit Deficiency. Thromb Haemost. 2018 Mar; 118(3):451-460. PMID: 29448295.
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    6. Buchbinder D, Nugent D, Hsieh L. Spotlight on romiplostim in the treatment of children with chronic immune thrombocytopenia: design, development, and potential place in therapy. Drug Des Devel Ther. 2017; 11:1055-1063. PMID: 28408804.
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    7. Buzby JS, Williams SA, Schaffer L, Head SR, Nugent DJ. Allele-specific wild-type TP53 expression in the unaffected carrier parent of children with Li-Fraumeni syndrome. Cancer Genet. 2017 02; 211:9-17. PMID: 28279309.
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    8. Nugent D, Kunicki T. Platelet genomics: the role of platelet size and number in health and disease. Platelets. 2017 Jan; 28(1):27-33. PMID: 28032522.
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    9. Carcao M, Fukutake K, Inbal A, Kerlin B, Lassila R, Oldenburg J, Garly ML, Nugent D. Developing the First Recombinant Factor XIII for Congenital Factor XIII Deficiency: Clinical Challenges and Successes. Semin Thromb Hemost. 2017 Feb; 43(1):59-68. PMID: 27556350.
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    10. John T, Walter JE, Schuetz C, Chen K, Abraham RS, Bonfim C, Boyce TG, Joshi AY, Kang E, Carvalho BT, Mahajerin A, Nugent D, Puthenveetil G, Soni A, Su H, Cowan MJ, Notarangelo L, Buchbinder D. Unrelated Hematopoietic Cell Transplantation in a Patient with Combined Immunodeficiency with Granulomatous Disease and Autoimmunity Secondary to RAG Deficiency. J Clin Immunol. 2016 10; 36(7):725-32. PMID: 27539235.
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    11. Elalfy MS, Nugent D. Viruses, anti-viral therapy, and viral vaccines in children with immune thrombocytopenia. Semin Hematol. 2016 04; 53 Suppl 1:S70-2. PMID: 27312173.
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    12. Forsyth AL, Witkop M, Lambing A, Garrido C, Dunn S, Cooper DL, Nugent DJ. Associations of quality of life, pain, and self-reported arthritis with age, employment, bleed rate, and utilization of hemophilia treatment center and health care provider services: results in adults with hemophilia in the HERO study. Patient Prefer Adherence. 2015; 9:1549-60. PMID: 26604708; PMCID: PMC4631419.
      View in: PubMed, PubMed Central
    13. Buchbinder D, Hsieh L, Mahajerin A, Puthenveetil G, Soni A, Nugent D. Successful treatment of secondary graft failure following unrelated cord blood transplant with hematopoietic growth factors in a pediatric patient with Fanconi anemia. Pediatr Transplant. 2015 Nov; 19(7):E181-4. PMID: 26228438.
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    14. Holm E, Abshire TC, Bowen J, Álvarez MT, Bolton-Maggs P, Carcao M, Federici AB, Gill JC, Halimeh S, Kempton C, Key NS, Kouides P, Lail A, Landorph A, Leebeek F, Makris M, Mannucci P, Mauser-Bunschoten EP, Nugent D, Valentino LA, Winikoff R, Berntorp E. Changes in bleeding patterns in von Willebrand disease after institution of long-term replacement therapy: results from the von Willebrand Disease Prophylaxis Network. Blood Coagul Fibrinolysis. 2015 Jun; 26(4):383-8. PMID: 25688461.
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    15. Nurden AT, Pillois X, Fiore M, Alessi MC, Bonduel M, Dreyfus M, Goudemand J, Gruel Y, Benabdallah-Guerida S, Latger-Cannard V, Négrier C, Nugent D, Oiron RD, Rand ML, Sié P, Trossaert M, Alberio L, Martins N, Sirvain-Trukniewicz P, Couloux A, Canault M, Fronthroth JP, Fretigny M, Nurden P, Heilig R, Vinciguerra C. Expanding the Mutation Spectrum Affecting aIIbß3 Integrin in Glanzmann Thrombasthenia: Screening of the ITGA2B and ITGB3 Genes in a Large International Cohort. Hum Mutat. 2015 May; 36(5):548-61. PMID: 25728920.
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    16. Buchbinder D, Stinson JR, Nugent DJ, Heurtier L, Suarez F, Sukumar G, Dalgard CL, Masson C, Parisot M, Zhang Y, Matthews HF, Su HC, Durandy A, Fischer A, Kracker S, Snow AL. Mild B-cell lymphocytosis in patients with a CARD11 C49Y mutation. J Allergy Clin Immunol. 2015 Sep; 136(3):819-821.e1. PMID: 25930198.
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    17. Buchbinder D, Baker R, Lee YN, Ravell J, Zhang Y, McElwee J, Nugent D, Coonrod EM, Durtschi JD, Augustine NH, Voelkerding KV, Csomos K, Rosen L, Browne S, Walter JE, Notarangelo LD, Hill HR, Kumánovics A. Identification of patients with RAG mutations previously diagnosed with common variable immunodeficiency disorders. J Clin Immunol. 2015 Feb; 35(2):119-24. PMID: 25516070; PMCID: PMC4479182.
      View in: PubMed, PubMed Central
    18. Buchbinder D, Hsieh L, Krance R, Nugent DJ. Successful treatment of post-transplant thrombocytopenia with romiplostim in a pediatric patient with X-linked chronic granulomatous disease. Pediatr Transplant. 2014 Nov; 18(7):E252-4. PMID: 25118016.
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    19. Janbain M, Nugent DJ, Powell JS, St-Louis J, Frame VB, Leissinger CA. Use of Factor XIII (FXIII) concentrate in patients with congenital FXIII deficiency undergoing surgical procedures. Transfusion. 2015 Jan; 55(1):45-50. PMID: 25070582.
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    20. Ramaswamy K, Hsieh L, Leven E, Thompson MV, Nugent D, Bussel JB. Thrombopoietic agents for the treatment of persistent and chronic immune thrombocytopenia in children. J Pediatr. 2014 Sep; 165(3):600-5.e4. PMID: 24857517.
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    21. Buchbinder D, Nugent DJ, Fillipovich AH. Wiskott-Aldrich syndrome: diagnosis, current management, and emerging treatments. Appl Clin Genet. 2014; 7:55-66. PMID: 24817816; PMCID: PMC4012343.
      View in: PubMed, PubMed Central
    22. Wang RY, Monuki ES, Powers J, Schwartz PH, Watkins PA, Shi Y, Moser A, Shrier DA, Waterham HR, Nugent DJ, Abdenur JE. Effects of hematopoietic stem cell transplantation on acyl-CoA oxidase deficiency: a sibling comparison study. J Inherit Metab Dis. 2014 Sep; 37(5):791-9. PMID: 24619150.
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    23. Buzby JS, Williams SA, Imfeld KL, Kunicki TJ, Nugent DJ. Tissue factor inflammatory response regulated by promoter genotype and p38 MAPK in neonatal vs. adult microvascular endothelial cells. Inflamm Res. 2014 Apr; 63(4):299-308. PMID: 24385191; PMCID: PMC3954954.
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    24. Buchbinder D, Hsieh L, Puthenveetil G, Soni A, Stites J, Huynh V, Kirov I, Neudorf S, Rubin E, Sender L, Torno L, Margolis D, Childs R, Moore T, Nugent D. Successful autologous cord blood transplantation in a child with acquired severe aplastic anemia. Pediatr Transplant. 2013 May; 17(3):E104-7. PMID: 23464883.
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    25. Habart D, Cheli Y, Nugent DJ, Ruggeri ZM, Kunicki TJ. Conditional knockout of integrin a2ß1 in murine megakaryocytes leads to reduced mean platelet volume. PLoS One. 2013; 8(1):e55094. PMID: 23359821; PMCID: PMC3554675.
      View in: PubMed, PubMed Central
    26. Nugent D. Corifact™/Fibrogammin® P in the prophylactic treatment of hereditary factor XIII deficiency: results of a prospective, multicenter, open-label study. Thromb Res. 2012 Dec; 130 Suppl 2:S12-4. PMID: 23439001.
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    27. Kunicki TJ, Williams SA, Nugent DJ. Genetic variants that affect platelet function. Curr Opin Hematol. 2012 Sep; 19(5):371-9. PMID: 22872156.
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    28. Hsieh L, Nugent D. Rare factor deficiencies. Curr Opin Hematol. 2012 Sep; 19(5):380-4. PMID: 22872158.
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    29. Buchbinder D, Steward CG, Puthenveetil G, Nugent D, Hsieh L, Kirov I, Neudorf S, Soni A. Successful cord blood transplantation in a patient with malignant infantile osteopetrosis and hemophilia. Pediatr Transplant. 2013 Feb; 17(1):E20-4. PMID: 22913475.
      View in: PubMed
    30. Buchbinder D, Nugent DJ, Brazauskas R, Wang Z, Aljurf MD, Cairo MS, Chow R, Duncan C, Eldjerou LK, Gupta V, Hale GA, Halter J, Hayes-Lattin BM, Hsu JW, Jacobsohn DA, Kamble RT, Kasow KA, Lazarus HM, Mehta P, Myers KC, Parsons SK, Passweg JR, Pidala J, Reddy V, Sales-Bonfim CM, Savani BN, Seber A, Sorror ML, Steinberg A, Wood WA, Wall DA, Winiarski JH, Yu LC, Majhail NS. Late effects in hematopoietic cell transplant recipients with acquired severe aplastic anemia: a report from the late effects working committee of the center for international blood and marrow transplant research. Biol Blood Marrow Transplant. 2012 Dec; 18(12):1776-84. PMID: 22863842.
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    31. Buchbinder D, Park S, Nugent D. X-linked hyper IgM syndrome: a novel sequence variant associated with an atypical mild phenotype. J Pediatr Hematol Oncol. 2012 Jul; 34(5):e212-4. PMID: 22322937.
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    32. Inbal A, Oldenburg J, Carcao M, Rosholm A, Tehranchi R, Nugent D. Recombinant factor XIII: a safe and novel treatment for congenital factor XIII deficiency. Blood. 2012 May 31; 119(22):5111-7. PMID: 22451421.
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    33. Kunicki TJ, Williams SA, Diaz D, Farndale RW, Nugent DJ. Platelet adhesion to decorin but not collagen I correlates with the integrin a2 dimorphism E534K, the basis of the human platelet alloantigen (HPA)-5 system. Haematologica. 2012 May; 97(5):692-5. PMID: 22133774; PMCID: PMC3342970.
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    34. Buchbinder DK, Zadeh T, Nugent D. A patient with familial bone marrow failure and an inversion of chromosome 8. J Pediatr Hematol Oncol. 2011 Dec; 33(8):626-7. PMID: 22042281.
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    35. Kunicki TJ, Williams SA, Nugent DJ, Yeager M. Mean platelet volume and integrin alleles correlate with levels of integrins a(IIb)ß(3) and a(2)ß(1) in acute coronary syndrome patients and normal subjects. Arterioscler Thromb Vasc Biol. 2012 Jan; 32(1):147-52. PMID: 22015659.
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    36. Buchbinder D, Younes B, Sassoon A, Soni A, Hsieh L, Puthenveetil G, Stites J, Ness-Jorden T, Neudorf S, Nugent D. Inflammatory polyps following successful HLA-matched cord blood transplantation in a patient with X-linked lymphoproliferative syndrome. Pediatr Transplant. 2012 Aug; 16(5):E188-91. PMID: 21736679.
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    37. Buchbinder D, Nadeau K, Nugent D. Monozygotic twin pair showing discordant phenotype for X-linked thrombocytopenia and Wiskott-Aldrich syndrome: a role for epigenetics? J Clin Immunol. 2011 Oct; 31(5):773-7. PMID: 21710275.
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    38. Grace RF, Bennett CM, Ritchey AK, Jeng M, Thornburg CD, Lambert MP, Neier M, Recht M, Kumar M, Blanchette V, Klaassen RJ, Buchanan GR, Kurth MH, Nugent DJ, Thompson AA, Stine K, Kalish LA, Neufeld EJ. Response to steroids predicts response to rituximab in pediatric chronic immune thrombocytopenia. Pediatr Blood Cancer. 2012 Feb; 58(2):221-5. PMID: 21674758; PMCID: PMC3863944.
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    39. Bussel JB, Buchanan GR, Nugent DJ, Gnarra DJ, Bomgaars LR, Blanchette VS, Wang YM, Nie K, Jun S. A randomized, double-blind study of romiplostim to determine its safety and efficacy in children with immune thrombocytopenia. Blood. 2011 Jul 07; 118(1):28-36. PMID: 21502541.
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    40. Young G, Yee DL, O'Brien SH, Khanna R, Barbour A, Nugent DJ. FondaKIDS: a prospective pharmacokinetic and safety study of fondaparinux in children between 1 and 18 years of age. Pediatr Blood Cancer. 2011 Dec 01; 57(6):1049-54. PMID: 21319285.
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    41. Buchbinder D, Nugent D, Vu D, Soni A, Stites J, Hsieh L, Puthenveetil G. Unrelated hematopoietic stem cell transplantation in a patient with congenital dyserythropoietic anemia and iron overload. Pediatr Transplant. 2012 May; 16(3):E69-73. PMID: 21108711.
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    42. Chira P, Nugent L, Miller K, Park T, Donahue S, Soni A, Nugent D, Sandborg C. Living Profiles: design of a health media platform for teens with special healthcare needs. J Biomed Inform. 2010 Oct; 43(5 Suppl):S9-12. PMID: 20937487.
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    43. Kunicki TJ, Nugent DJ. The genetics of normal platelet reactivity. Blood. 2010 Oct 14; 116(15):2627-34. PMID: 20610812; PMCID: PMC2974578.
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    44. Tamary H, Roganovic J, Chitlur M, Nugent DJ. Consensus Paper-ICIS Expert Meeting Basel 2009 treatment milestones in immune thrombocytopenia. Ann Hematol. 2010 Jul; 89 Suppl 1:5-10. PMID: 20358200; PMCID: PMC2900598.
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    45. Cheli Y, Williams SA, Ballotti R, Nugent DJ, Kunicki TJ. Enhanced binding of poly(ADP-ribose)polymerase-1 and Ku80/70 to the ITGA2 promoter via an extended cytosine-adenosine repeat. PLoS One. 2010 Jan 15; 5(1):e8743. PMID: 20090957; PMCID: PMC2806922.
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    46. Young G, Wicklund B, Neff P, Johnson C, Nugent DJ. Off-label use of rFVIIa in children with excessive bleeding: a consecutive study of 153 off-label uses in 139 children. Pediatr Blood Cancer. 2009 Aug; 53(2):179-83. PMID: 19415741.
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    47. Trifiro E, Williams SA, Cheli Y, Furihata K, Pulcinelli FM, Nugent DJ, Kunicki TJ. The low-frequency isoform of platelet glycoprotein VIb attenuates ligand-mediated signal transduction but not receptor expression or ligand binding. Blood. 2009 Aug 27; 114(9):1893-9. PMID: 19465689; PMCID: PMC2738573.
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    48. Nugent D, McMillan R, Nichol JL, Slichter SJ. Pathogenesis of chronic immune thrombocytopenia: increased platelet destruction and/or decreased platelet production. Br J Haematol. 2009 Sep; 146(6):585-96. PMID: 19466980.
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    49. Kunicki TJ, Williams SA, Nugent DJ, Harrison P, Segal HC, Syed A, Rothwell PM. Lack of association between aspirin responsiveness and seven candidate gene haplotypes in patients with symptomatic vascular disease. Thromb Haemost. 2009 Jan; 101(1):123-33. PMID: 19132198.
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    50. Young G, Yonekawa KE, Nakagawa PA, Blain RC, Lovejoy AE, Nugent DJ. Recombinant activated factor VII effectively reverses the anticoagulant effects of heparin, enoxaparin, fondaparinux, argatroban, and bivalirudin ex vivo as measured using thromboelastography. Blood Coagul Fibrinolysis. 2007 Sep; 18(6):547-53. PMID: 17762530.
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    51. Manco-Johnson MJ, Abshire TC, Shapiro AD, Riske B, Hacker MR, Kilcoyne R, Ingram JD, Manco-Johnson ML, Funk S, Jacobson L, Valentino LA, Hoots WK, Buchanan GR, DiMichele D, Recht M, Brown D, Leissinger C, Bleak S, Cohen A, Mathew P, Matsunaga A, Medeiros D, Nugent D, Thomas GA, Thompson AA, McRedmond K, Soucie JM, Austin H, Evatt BL. Prophylaxis versus episodic treatment to prevent joint disease in boys with severe hemophilia. N Engl J Med. 2007 Aug 09; 357(6):535-44. PMID: 17687129.
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    52. Cheli Y, Kanaji S, Jacquelin B, Chang M, Nugent DJ, Kunicki TJ. Transcriptional and epigenetic regulation of the integrin collagen receptor locus ITGA1-PELO-ITGA2. Biochim Biophys Acta. 2007 Sep-Oct; 1769(9-10):546-58. PMID: 17669516; PMCID: PMC2682338.
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    53. Young G, Yonekawa KE, Nakagawa PA, Blain RC, Lovejoy AE, Nugent DJ. Differential effects of direct thrombin inhibitors and antithrombin-dependent anticoagulants on the dynamics of clot formation. Blood Coagul Fibrinolysis. 2007 Mar; 18(2):97-103. PMID: 17287624.
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    54. Hoots WK, Nugent DJ. Evidence for the benefits of prophylaxis in the management of hemophilia A. Thromb Haemost. 2006 Oct; 96(4):433-40. PMID: 17003919.
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    55. Nugent DJ. Prophylaxis in rare coagulation disorders -- factor XIII deficiency. Thromb Res. 2006; 118 Suppl 1:S23-8. PMID: 16616323.
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    56. Tarantino MD, Young G, Bertolone SJ, Kalinyak KA, Shafer FE, Kulkarni R, Weber LC, Davis ML, Lynn H, Nugent DJ. Single dose of anti-D immune globulin at 75 microg/kg is as effective as intravenous immune globulin at rapidly raising the platelet count in newly diagnosed immune thrombocytopenic purpura in children. J Pediatr. 2006 Apr; 148(4):489-94. PMID: 16647411.
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    57. Lovejoy AE, Reynolds TC, Visich JE, Butine MD, Young G, Belvedere MA, Blain RC, Pederson SM, Ishak LM, Nugent DJ. Safety and pharmacokinetics of recombinant factor XIII-A2 administration in patients with congenital factor XIII deficiency. Blood. 2006 Jul 01; 108(1):57-62. PMID: 16556896.
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    58. Nugent DJ. Immune thrombocytopenic purpura of childhood. Hematology Am Soc Hematol Educ Program. 2006; 97-103. PMID: 17124046.
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    59. Flood VH, Johnson FL, Boshkov LK, Thomas GA, Nugent DJ, Bakke AC, Nicholson HS, Tilford D, Brown MP, Godder KT. Sustained engraftment post bone marrow transplant despite anti-platelet antibodies in Glanzmann thrombasthenia. Pediatr Blood Cancer. 2005 Dec; 45(7):971-5. PMID: 15768382.
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    60. Rook JL, Nugent DJ, Young G. Pediatric stroke and methylenetetrahydrofolate reductase polymorphisms: an examination of C677T and A1298C mutations. J Pediatr Hematol Oncol. 2005 Nov; 27(11):590-3. PMID: 16282888.
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    61. Kanaji S, Kanaji T, Jacquelin B, Chang M, Nugent DJ, Komatsu N, Moroi M, Izuhara K, Kunicki TJ. Thrombopoietin initiates demethylation-based transcription of GP6 during megakaryocyte differentiation. Blood. 2005 May 15; 105(10):3888-92. PMID: 15701720; PMCID: PMC1895081.
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    62. McMillan R, Nugent D. The effect of antiplatelet autoantibodies on megakaryocytopoiesis. Int J Hematol. 2005 Feb; 81(2):94-9. PMID: 15765775.
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    63. Young G, Yonekawa KE, Nakagawa P, Nugent DJ. Argatroban as an alternative to heparin in extracorporeal membrane oxygenation circuits. Perfusion. 2004; 19(5):283-8. PMID: 15506032.
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    64. Chang M, Nakagawa PA, Williams SA, Schwartz MR, Imfeld KL, Buzby JS, Nugent DJ. Immune thrombocytopenic purpura (ITP) plasma and purified ITP monoclonal autoantibodies inhibit megakaryocytopoiesis in vitro. Blood. 2003 Aug 01; 102(3):887-95. PMID: 12676790.
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    65. Locatelli F, Rocha V, Reed W, Bernaudin F, Ertem M, Grafakos S, Brichard B, Li X, Nagler A, Giorgiani G, Haut PR, Brochstein JA, Nugent DJ, Blatt J, Woodard P, Kurtzberg J, Rubin CM, Miniero R, Lutz P, Raja T, Roberts I, Will AM, Yaniv I, Vermylen C, Tannoia N, Garnier F, Ionescu I, Walters MC, Lubin BH, Gluckman E. Related umbilical cord blood transplantation in patients with thalassemia and sickle cell disease. Blood. 2003 Mar 15; 101(6):2137-43. PMID: 12424197.
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    66. Zaldivar F, Nugent DJ, Imfeld K, Berman MA. Identification of a novel regulatory element in the human interleukin 1 alpha (IL-1alpha) gene promoter. Cytokine. 2002 Nov 07; 20(3):130-5. PMID: 12453471.
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    67. Kunicki TJ, Nugent DJ. The influence of platelet glycoprotein polymorphisms on receptor function and risk for thrombosis. Vox Sang. 2002 Aug; 83 Suppl 1:85-90. PMID: 12617110.
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    68. Furihata K, Nugent DJ, Kunicki TJ. Influence of platelet collagen receptor polymorphisms on risk for arterial thrombosis. Arch Pathol Lab Med. 2002 Mar; 126(3):305-9. PMID: 11860304.
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