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Diane Jean Nugent

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, Patel SK, Casillas JN, Nugent DJ, Neudorf S, Sender LS, Torno L, Huszti H, Zeltzer LK, Parsons SK. Parent proxy assessment of sibling quality of life following pediatric hematopoietic cell transplantation. Health Qual Life Outcomes. 2019 Oct 29; 17(1):162. PMID: 31665035.
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    2. Puthenveetil G, Nugent D. Hemophilia - Impact of Recent Advances on Management. Indian J Pediatr. 2019 Sep 16. PMID: 31529382.
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    3. Pipe SW, Sabatino DE, Nugent DJ, Hooper WC, Soucie JM, Keith Hoots W, DiMichele DM. Executive summary of the NHLBI State of the Science (SOS) Workshop: Overview and next steps in generating a national blueprint for future research on factor VIII inhibitors. Haemophilia. 2019 Jul; 25(4):610-615. PMID: 31329363.
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    4. Sabatino DE, Pipe SW, Nugent DJ, Soucie JM, Hooper WC, Hoots WK, DiMichele DM. Origins and organization of the NHLBI State of the Science Workshop: Generating a national blueprint for future research on factor VIII inhibitors. Haemophilia. 2019 Jul; 25(4):575-580. PMID: 31329369.
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    5. Nossair F, Mahajerin A, Hoang J, Diaz D, Nugent D. Promising biomarkers for the prediction of catheter-related venous thromboembolism in hospitalized children: An exploratory study. Pediatr Blood Cancer. 2019 Oct; 66(10):e27870. PMID: 31222954.
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    6. 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; 39(1):81-89. PMID: 30607663.
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    7. 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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    8. 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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    9. 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 07; 40(5):e319-e322. PMID: 29200144.
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    10. O'Mahony B, Dolan G, Nugent D, Goodman C. Patient-centred value framework for haemophilia. Haemophilia. 2018 Nov; 24(6):873-879. PMID: 29626368.
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    11. 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 03; 118(3):451-460. PMID: 29448295.
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    12. Kenet G, Oladapo A, Epstein JD, Thompson C, Novack A, Nugent DJ. Estimating the potential cost of a high dose immune tolerance induction (ITI) therapy relative to the cost of a combined therapy of a low dose ITI therapy with bypassing agent prophylaxis. Haemophilia. 2017 Sep; 23(5):e394-e402. PMID: 28641362.
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    13. 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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    14. 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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    15. 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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    16. Kulkarni R, Presley RJ, Lusher JM, Shapiro AD, Gill JC, Manco-Johnson M, Koerper MA, Abshire TC, DiMichele D, Hoots WK, Mathew P, Nugent DJ, Geraghty S, Evatt BL, Soucie JM. Complications of haemophilia in babies (first two years of life): a report from the Centers for Disease Control and Prevention Universal Data Collection System. Haemophilia. 2017 Mar; 23(2):207-214. PMID: 27813214.
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    17. 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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    18. 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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    19. de Moerloose P, Schved JF, Nugent D. Rare coagulation disorders: fibrinogen, factor VII and factor XIII. Haemophilia. 2016 Jul; 22 Suppl 5:61-5. PMID: 27405678.
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    20. 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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    21. 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.
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    22. 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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    23. 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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    24. 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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    25. 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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    26. Brand-Staufer B, Carcao M, Kerlin BA, Will A, Williams M, Tornøe CW, Sandberg Lundblad M, Nugent D. Pharmacokinetic characterization of recombinant factor XIII (FXIII)-A2 across age groups in patients with FXIII A-subunit congenital deficiency. Haemophilia. 2015 May; 21(3):380-5. PMID: 25643920.
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    27. 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.
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    28. Nugent DJ, Ashley C, García-Talavera J, Lo LC, Mehdi AS, Mangione A. Pharmacokinetics and safety of plasma-derived factor XIII concentrate (human) in patients with congenital factor XIII deficiency. Haemophilia. 2015 Jan; 21(1):95-101. PMID: 25458735.
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    29. Ashley C, Chang E, Davis J, Mangione A, Frame V, Nugent DJ. Efficacy and safety of prophylactic treatment with plasma-derived factor XIII concentrate (human) in patients with congenital factor XIII deficiency. Haemophilia. 2015 Jan; 21(1):102-8. PMID: 25377187.
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    30. Nugent D, Kalnins W, Querol F, Gregory M, Pilgaard T, Cooper DL, Iorio A. Haemophilia Experiences, Results and Opportunities (HERO) study: treatment-related characteristics of the population. Haemophilia. 2015 Jan; 21(1):e26-38. PMID: 25377442.
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    31. Kerlin B, Brand B, Inbal A, Halimeh S, Nugent D, Lundblad M, Tehranchi R. Pharmacokinetics of recombinant factor XIII at steady state in patients with congenital factor XIII A-subunit deficiency. J Thromb Haemost. 2014 Dec; 12(12):2038-43. PMID: 25263390.
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    32. 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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    33. 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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    34. Gresele P, Harrison P, Bury L, Falcinelli E, Gachet C, Hayward CP, Kenny D, Mezzano D, Mumford AD, Nugent D, Nurden AT, Orsini S, Cattaneo M. Diagnosis of suspected inherited platelet function disorders: results of a worldwide survey. J Thromb Haemost. 2014 Sep; 12(9):1562-9. PMID: 24976115.
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    35. 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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    36. Cassis FR, Buzzi A, Forsyth A, Gregory M, Nugent D, Garrido C, Pilgaard T, Cooper DL, Iorio A. Haemophilia Experiences, Results and Opportunities (HERO) Study: influence of haemophilia on interpersonal relationships as reported by adults with haemophilia and parents of children with haemophilia. Haemophilia. 2014 Jul; 20(4):e287-95. PMID: 24800872.
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    37. Buchbinder D, Nugent DJ, Fillipovich AH. Wiskott-Aldrich syndrome: diagnosis, current management, and emerging treatments. Appl Clin Genet. 2014; 7:55-66. PMID: 24817816.
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    38. 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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    39. 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.
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    40. Forsyth AL, Gregory M, Nugent D, Garrido C, Pilgaard T, Cooper DL, Iorio A. Haemophilia Experiences, Results and Opportunities (HERO) Study: survey methodology and population demographics. Haemophilia. 2014 Jan; 20(1):44-51. PMID: 23902228.
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    41. Buchbinder D, Puthenveetil G, Soni A, Hsieh L, Nugent D, Church JA. Newborn screening for severe combined immunodeficiency: an opportunity for intervention. J Perinatol. 2013 Aug; 33(8):657-8. PMID: 23897312.
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    42. Cattaneo M, Cerletti C, Harrison P, Hayward CP, Kenny D, Nugent D, Nurden P, Rao AK, Schmaier AH, Watson SP, Lussana F, Pugliano MT, Michelson AD. Recommendations for the Standardization of Light Transmission Aggregometry: A Consensus of the Working Party from the Platelet Physiology Subcommittee of SSC/ISTH. J Thromb Haemost. 2013 Apr 10. PMID: 23574625.
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    43. 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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    44. 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.
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    45. 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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    46. 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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    47. Hsieh L, Nugent D. Rare factor deficiencies. Curr Opin Hematol. 2012 Sep; 19(5):380-4. PMID: 22872158.
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    48. 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.
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    49. 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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    50. 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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    51. 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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    52. 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.
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    53. 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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    54. 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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    55. 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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    56. Byams VR, Kouides PA, Kulkarni R, Baker JR, Brown DL, Gill JC, Grant AM, James AH, Konkle BA, Maahs J, Dumas MM, McAlister S, Nance D, Nugent D, Philipp CS, Soucie JM, Stang E. Surveillance of female patients with inherited bleeding disorders in United States Haemophilia Treatment Centres. Haemophilia. 2011 Jul; 17 Suppl 1:6-13. PMID: 21692922.
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    57. 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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    58. 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.
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    59. Bujnicki HC, Sidonio RF, Kempton C, Kouides PA, Kulkarni R, Nugent DJ, Yee DL, Moore CG, Ragni MV. Screening for von Willebrand disease in children: a case-control study. J Thromb Haemost. 2011 May; 9(5):1086-9. PMID: 21410642.
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    60. 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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    61. Chitlur M, Sorensen B, Rivard GE, Young G, Ingerslev J, Othman M, Nugent D, Kenet G, Escobar M, Lusher J. Standardization of thromboelastography: a report from the TEG-ROTEM working group. Haemophilia. 2011 May; 17(3):532-7. PMID: 21323795.
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    62. 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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    63. Kaveri S, Mannucci PM, Kurth MH, Ewing N, Kessler CM, Nugent DJ, Gomperts ED. von Willebrand factor: what is its role in the immune response in haemophilia? Haemophilia. 2011 Jan; 17(1):e235-8. PMID: 20546026.
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    64. 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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    65. 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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    66. Kunicki TJ, Nugent DJ. The genetics of normal platelet reactivity. Blood. 2010 Oct 14; 116(15):2627-34. PMID: 20610812.
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    67. 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.
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    68. 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.
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    69. Young G, Zhang R, Miller R, Yassin D, Nugent DJ. Comparison of kaolin and tissue factor activated thromboelastography in haemophilia. Haemophilia. 2010 May; 16(3):518-24. PMID: 20028419.
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    70. Lusher J, Pipe SW, Alexander S, Nugent D. Prophylactic therapy with Fibrogammin P is associated with a decreased incidence of bleeding episodes: a retrospective study. Haemophilia. 2010 Mar; 16(2):316-21. PMID: 20017752.
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    71. Kunicki TJ, Williams SA, Salomon DR, Harrison P, Crisler P, Nakagawa P, Mondala TS, Head SR, Nugent DJ. Genetics of platelet reactivity in normal, healthy individuals. J Thromb Haemost. 2009 Dec; 7(12):2116-22. PMID: 19740098.
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    72. 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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    73. Kulkarni R, Soucie JM, Lusher J, Presley R, Shapiro A, Gill J, Manco-Johnson M, Koerper M, Mathew P, Abshire T, Dimichele D, Hoots K, Janco R, Nugent D, Geraghty S, Evatt B. Sites of initial bleeding episodes, mode of delivery and age of diagnosis in babies with haemophilia diagnosed before the age of 2 years: a report from The Centers for Disease Control and Prevention's (CDC) Universal Data Collection (UDC) project. Haemophilia. 2009 Nov; 15(6):1281-90. PMID: 19637999.
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    74. 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.
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    75. 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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    76. 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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    77. Hsieh L, Nugent D. Factor XIII deficiency. Haemophilia. 2008 Nov; 14(6):1190-200. PMID: 19141159.
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    78. Simon D, Kunicki T, Nugent D. Platelet function defects. Haemophilia. 2008 Nov; 14(6):1240-9. PMID: 19141164.
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    79. Powell JS, Nugent DJ, Harrison JA, Soni A, Luk A, Stass H, Gorina E. Safety and pharmacokinetics of a recombinant factor VIII with pegylated liposomes in severe hemophilia A. J Thromb Haemost. 2008 Feb; 6(2):277-83. PMID: 18039351.
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    80. 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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    81. 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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    82. Young G, Tarantino MD, Wohrley J, Weber LC, Belvedere M, Nugent DJ. Pilot dose-finding and safety study of bivalirudin in infants <6 months of age with thrombosis. J Thromb Haemost. 2007 Aug; 5(8):1654-9. PMID: 17663736.
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    83. Aledort LM, Salama A, Kovaleva L, Robak T, Newland AC, Nugent DJ, Brenner B, Zenker O. Efficacy and safety of intravenous anti-D immunoglobulin (Rhophylac) in chronic immune thrombocytopenic purpura. Hematology. 2007 Aug; 12(4):289-95. PMID: 17654054.
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    84. 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.
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    85. Schneiderman J, Rubin E, Nugent DJ, Young G. Sequential therapy with activated prothrombin complex concentrates and recombinant FVIIa in patients with severe haemophilia and inhibitors: update of our previous experience. Haemophilia. 2007 May; 13(3):244-8. PMID: 17498072.
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    86. 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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    87. Young G, Blain R, Nakagawa P, Nugent DJ. Individualization of bypassing agent treatment for haemophilic patients with inhibitors utilizing thromboelastography. Haemophilia. 2006 Nov; 12(6):598-604. PMID: 17083509.
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    88. 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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    89. Rattray B, Nugent DJ, Young G. Celecoxib in the treatment of haemophilic synovitis, target joints, and pain in adults and children with haemophilia. Haemophilia. 2006 Sep; 12(5):514-7. PMID: 16919082.
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    90. Nugent DJ. Prophylaxis in rare coagulation disorders -- factor XIII deficiency. Thromb Res. 2006; 118 Suppl 1:S23-8. PMID: 16616323.
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    91. 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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    92. 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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    93. Nugent DJ. Immune thrombocytopenic purpura of childhood. Hematology Am Soc Hematol Educ Program. 2006; 97-103. PMID: 17124046.
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    94. 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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    95. 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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    96. Rattray B, Nugent DJ, Young G. Rofecoxib as adjunctive therapy for haemophilic arthropathy. Haemophilia. 2005 May; 11(3):240-4. PMID: 15876269.
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    97. Young G, McDaniel M, Nugent DJ. Prophylactic recombinant factor VIIa in haemophilia patients with inhibitors. Haemophilia. 2005 May; 11(3):203-7. PMID: 15876264.
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    98. 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.
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    99. 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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    100. Schneiderman J, Nugent DJ, Young G. Sequential therapy with activated prothrombin complex concentrate and recombinant factor VIIa in patients with severe haemophilia and inhibitors. Haemophilia. 2004 Jul; 10(4):347-51. PMID: 15230948.
      View in: PubMed
    101. 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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    102. Armenian S, Raffel JL, Nugent DJ, Young G. Painless urethral bleeding: an unusual presentation of Von Willebrand disease. Haemophilia. 2003 May; 9(3):332-5. PMID: 12694526.
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    103. Young G, Manco-Johnson M, Gill JC, Dimichele DM, Tarantino MD, Abshire T, Nugent DJ. Clinical manifestations of the prothrombin G20210A mutation in children: a pediatric coagulation consortium study. J Thromb Haemost. 2003 May; 1(5):958-62. PMID: 12871361.
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    104. 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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    105. 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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    106. 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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    107. 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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    108. 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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    109. Nugent DJ. Controversies in the treatment of pediatric immune thrombocytopenias. Blood Rev. 2002 Mar; 16(1):15-7. PMID: 11913987.
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    110. Nugent DJ. Childhood immune thrombocytopenic purpura. Blood Rev. 2002 Mar; 16(1):27-9. PMID: 11913990.
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    111. Di Paola J, Nugent D, Young G. Current therapy for rare factor deficiencies. Haemophilia. 2001 Jan; 7 Suppl 1:16-22. PMID: 11240614.
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    112. Young G, Nugent DJ. Prevention of Bleeding Complications in Neonates With Liver Failure Undergoing Surgery Using Recombinant Factor VIIa. Hematology. 2001; 6(5):341-6. PMID: 27405529.
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    113. Nugent D, Xu Y. Sphingosine-1-phosphate: characterization of its inhibition of platelet aggregation. Platelets. 2000 Jun; 11(4):226-32. PMID: 10938902.
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    114. Buzby JS, Brewer G, Nugent DJ. Developmental regulation of RNA transcript destabilization by A + U-rich elements is AUF1-dependent. J Biol Chem. 1999 Nov 26; 274(48):33973-8. PMID: 10567360.
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    115. Di Paola J, Federici AB, Mannucci PM, Canciani MT, Kritzik M, Kunicki TJ, Nugent D. Low platelet alpha2beta1 levels in type I von Willebrand disease correlate with impaired platelet function in a high shear stress system. Blood. 1999 Jun 01; 93(11):3578-82. PMID: 10339461.
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    116. Meirow D, Fasouliotis SJ, Nugent D, Schenker JG, Gosden RG, Rutherford AJ. A laparoscopic technique for obtaining ovarian cortical biopsy specimens for fertility conservation in patients with cancer. Fertil Steril. 1999 May; 71(5):948-51. PMID: 10231063.
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    117. Nugent DJ. Immune thrombocytopenic purpura: why treat? J Pediatr. 1999 Jan; 134(1):3-4. PMID: 9880438.
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    118. Kritzik M, Savage B, Nugent DJ, Santoso S, Ruggeri ZM, Kunicki TJ. Nucleotide polymorphisms in the alpha2 gene define multiple alleles that are associated with differences in platelet alpha2 beta1 density. Blood. 1998 Oct 01; 92(7):2382-8. PMID: 9746778.
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    119. Mammen EF, Comp PC, Gosselin R, Greenberg C, Hoots WK, Kessler CM, Larkin EC, Liles D, Nugent DJ. PFA-100 system: a new method for assessment of platelet dysfunction. Semin Thromb Hemost. 1998; 24(2):195-202. PMID: 9579642.
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    120. Kunicki TJ, Kritzik M, Annis DS, Nugent DJ. Hereditary variation in platelet integrin alpha 2 beta 1 density is associated with two silent polymorphisms in the alpha 2 gene coding sequence. Blood. 1997 Mar 15; 89(6):1939-43. PMID: 9058714.
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    121. Ishida F, Gruel Y, Brojer E, Nugent DJ, Kunicki TJ. Repertoire cloning of a human IgG inhibitor of alpha IIB beta 3 function. The OG idiotype. Mol Immunol. 1995 Jun; 32(9):613-22. PMID: 7543974.
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    122. Gruel YJ, Nugent DJ, Kunicki TJ. Molecular specificity of anti-IIb/IIIa human antibodies. Semin Thromb Hemost. 1995; 21(1):60-7. PMID: 7604292.
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    123. Gruel Y, Brojer E, Nugent DJ, Kunicki TJ. Further characterization of the thrombasthenia-related idiotype OG. Antiidiotype defines a novel epitope(s) shared by fibrinogen B beta chain, vitronectin, and von Willebrand factor and required for binding to beta 3. J Exp Med. 1994 Dec 01; 180(6):2259-67. PMID: 7525851.
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    124. Tarantino MD, Nugent DJ. Severe electrical burns in a hemophiliac wearing a medical alert neck chain. Clin Pediatr (Phila). 1994 Jun; 33(6):382-3. PMID: 8200177.
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    125. Tarantino MD, Kunicki TJ, Nugent DJ. The estrogen receptor is present in human megakaryocytes. Ann N Y Acad Sci. 1994 Apr 18; 714:293-6. PMID: 8017783.
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    126. Devine DV, Andestad G, Nugent D, Carter CJ. Platelet-associated factor XIII as a marker of platelet activation in patients with peripheral vascular disease. Arterioscler Thromb. 1993 Jun; 13(6):857-62. PMID: 8499406.
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    127. Nugent DJ. Alloimmunization to platelet antigens. Semin Hematol. 1992 Jul; 29(3 Suppl 2):83-8. PMID: 1509298.
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    128. Nugent DJ. IVIG in the treatment of children with acute and chronic idiopathic thrombocytopenic purpura and the autoimmune cytopenias. Clin Rev Allergy. 1992 Spring-Summer; 10(1-2):59-71. PMID: 1606524.
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    129. Kunicki TJ, Annis DS, Gorski J, Nugent DJ. Nucleotide sequence of the human autoantibody 2E7 specific for the platelet integrin IIb heavy chain. J Autoimmun. 1991 Jun; 4(3):433-46. PMID: 1716898.
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    130. Kunicki TJ, Plow EF, Kekomaki R, Nugent DJ. Human monoclonal autoantibody 2E7 is specific for a peptide sequence of platelet glycoprotein IIb. Localization of the epitope to IIb231-238 with an immunodominant Trp235. J Autoimmun. 1991 Jun; 4(3):415-31. PMID: 1716897.
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    131. Kunicki TJ, Furihata K, Kekomaki R, Scott JP, Nugent DJ. A human monoclonal autoantibody specific for human platelet glycoprotein IIb (integrin alpha IIb) heavy chain. Hum Antibodies Hybridomas. 1990; 1(2):83-95. PMID: 1715776.
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    132. Hiraiwa A, Nugent DJ, Milner EC. Sequence analysis of monoclonal antibodies derived from a patient with idiopathic thrombocytopenic purpura. Autoimmunity. 1990; 8(2):107-13. PMID: 2104187.
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    133. Nugent DJ. Human monoclonal autoantibodies to characterize platelet antigens in immune-mediated thrombocytopenia. Blut. 1989 Jul; 59(1):52-8. PMID: 2752176.
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    134. Roth GJ, Ozols J, Nugent DJ, Williams SA. Isolation and characterization of human platelet glycoprotein IX. Biochem Biophys Res Commun. 1988 Oct 31; 156(2):931-9. PMID: 3056407.
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    135. Kunicki TJ, Nugent DJ, Staats SJ, Orchekowski RP, Wayner EA, Carter WG. The human fibroblast class II extracellular matrix receptor mediates platelet adhesion to collagen and is identical to the platelet glycoprotein Ia-IIa complex. J Biol Chem. 1988 Apr 05; 263(10):4516-9. PMID: 2832397.
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    136. Piotrowicz RS, Orchekowski RP, Nugent DJ, Yamada KY, Kunicki TJ. Glycoprotein Ic-IIa functions as an activation-independent fibronectin receptor on human platelets. J Cell Biol. 1988 Apr; 106(4):1359-64. PMID: 2966181.
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    137. Kunicki TJ, Furihata K, Nugent D. Glycoprotein IIb-IIIa as an immunologic target. Curr Stud Hematol Blood Transfus. 1988; (54):44-63. PMID: 3280255.
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    138. Furihata K, Nugent DJ, Bissonette A, Aster RH, Kunicki TJ. On the association of the platelet-specific alloantigen, Pena, with glycoprotein IIIa. Evidence for heterogeneity of glycoprotein IIIa. J Clin Invest. 1987 Dec; 80(6):1624-30. PMID: 2445781.
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    139. Canfield VA, Ozols J, Nugent D, Roth GJ. Isolation and characterization of the alpha and beta chains of human platelet glycoprotein Ib. Biochem Biophys Res Commun. 1987 Sep 15; 147(2):526-34. PMID: 3632685.
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    140. Nugent DJ, Kunicki TJ, Berglund C, Bernstein ID. A human monoclonal autoantibody recognizes a neoantigen on glycoprotein IIIa expressed on stored and activated platelets. Blood. 1987 Jul; 70(1):16-22. PMID: 2439150.
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    141. Shulman HM, Gown AM, Nugent DJ. Hepatic veno-occlusive disease after bone marrow transplantation. Immunohistochemical identification of the material within occluded central venules. Am J Pathol. 1987 Jun; 127(3):549-58. PMID: 2438942.
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    142. Kunicki TJ, Furihata K, Bull B, Nugent DJ. The immunogenicity of platelet membrane glycoproteins. Transfus Med Rev. 1987 Apr; 1(1):21-33. PMID: 2980263.
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    143. Odell JM, Haas JE, Tapper D, Nugent D. Infantile hemorrhagic angiodysplasia. Pediatr Pathol. 1987; 7(5-6):629-36. PMID: 3502423.
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    144. Nugent DJ, Bray GL, Counts RB, Clements MJ, Thompson AR. Danazol fails to increase factor VIII or IX levels in a double-blind crossover study of patients with haemophilia A and B. Br J Haematol. 1986 Nov; 64(3):493-502. PMID: 3098273.
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    145. Kunicki TJ, Nugent DJ, Piotrowicz RS, Lai CS. Covalent attachment of sulfhydryl-specific, electron spin resonance spin-labels to Fab' fragments of murine monoclonal antibodies that recognize human platelet membrane glycoproteins. Development of membrane protein specific spin probes. Biochemistry. 1986 Sep 09; 25(18):4979-83. PMID: 3021207.
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