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Elliott Vichinsky, MD

TitleProfessor
InstitutionUniversity of California San Francisco
DepartmentPediatrics
Address401 Parnassus Ave.
San Francisco CA 94143
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    Collapse Biography 
    Collapse Education and Training
    University of Wisconsin, MadisonB.S. 1970Biology
    State University of New York DownstateM.D.1974School of Medicine
    Children's Orthopedic Hospital and Medical Center, Seattle, WAInternship1975Pediatrics
    Children's Orthopedic Hospital and Medical Center, Seattle, WAFellowship1978Pediatrics
    University of Washington, Seattle, WASenior Research Fellow1979Dept of Pediatrics and Medicine
    Collapse Awards and Honors
    Sickle Cell Disease Association of America (SCDAA)2013Pioneer Award
    Cooley's Anemia Foundation2009Distinguished Leadership as Chair of the Medical Advisory Board
    Sickle Cell Disease Foundation of California2007Lifetime Achievement Award
    Children's Hospital & Research Center Oakland2005Bronze Bambino Lifetime Achievement Award
    Children's Miracle Network1995Miracle Maker Award
    Alpha Omega Alpha Honor Society1974Alpha Omega Award
    State University of New York Downstate1974Cum Laude
    University of Wisconsin1970Cum Laude

    Collapse Overview 
    Collapse Overview
    I am the Director of Hematology/Oncology at UCSF Benioff Children's Hospital Oakland, Adjunct Professor at University of California San Francisco, as well as the Director of the Northern California Sickle Cell and Thalassemia Centers. I am a board-certified pediatric hematologist/oncologist with a major interest in understanding and improving the care of patients with hemoglobinopathies. My career has focused on translational research in hemoglobinopathies to answer therapeutic questions in hematologic and iron overload disorders, including iron metabolism and its consequences in multiple diseases, alloimmunization, transfusion efficacy and risks in hematology.

    Over 30 years ago, I established the hemoglobinopathy center at Children's Hospital Oakland to provide primary through tertiary care to pediatric and adult patients. I have been a federally-funded research investigator for over 30 years and national principal investigator for many successful trials -- including transfusion therapy and chelation therapy in hemoglobinopathies. I have been principal investigator or co-investigator on many of the key translational studies, including understanding the mechanism and treatment of neurologic injury in sickle cell disease; iron trafficking and iron overload in hemoglobinopathies, and new therapies to change the pathophysiology of sickle cell biology.

    I have served as Editor-in-Chief of Pediatric Hematology and Oncology; Chairman of the Thalassemia Clinical Research Network, and Director of the Cooley’s Medical Board.


    Collapse Research 
    Collapse Research Activities and Funding
    SC-Links - Linking the Sickle Cell Community, Providers and Systems to Improve Sickle Cell Disease Care
    NIH/NHLBI U01HL134007Aug 5, 2016 - Jun 30, 2022
    Role: Principal Investigator
    Phase 2 Study of Deferiprone in the Treatment of NBIA
    FDA R01FD004103Sep 15, 2012 - Aug 31, 2016
    Role: Principal Investigator
    Ninth Cooley's Anemia Symposium
    NIH/NHLBI R13HL096359Sep 1, 2009 - Aug 31, 2010
    Role: Principal Investigator
    Prevention of Complications in Thalassemia
    CDC/NCBDD U01DD000310Sep 30, 2007 - Mar 29, 2013
    Role: Principal Investigator
    Eighth Cooley's Anemia Symposium
    NIH/NHLBI R13HL079823Jan 1, 2005 - Dec 31, 2005
    Role: Principal Investigator
    Northern California Comprehensive Sickle Cell Centers
    NIH U54HL070583Jul 1, 2003 - Mar 31, 2010
    Role: Co-Investigator
    Modulation of Iron Deposition in SCD and Other Hemoglobinopathies
    NIH/NIDDK R01DK057778Aug 1, 2000 - Jun 30, 2013
    Role: Co-Principal Investigator
    Thalassemia Clinical Research Network
    NIH/NHLBI U01HL065239Jul 1, 2000 - Jun 30, 2011
    Role: Principal Investigator
    E/BETA THALASSEMIA--NATURAL HISTORY &RESPONSE TO CHEMO
    NIH/NHLBI R01HL061186Sep 30, 1998 - Jul 31, 2004
    Role: Principal Investigator
    PEDIATRIC COHORT OF THE CSSCD
    NIH/NHLBI N01HB047107Apr 1, 1994 - Mar 31, 1999
    Role: Principal Investigator
    SELECTED PATIENT GROUPS FROM THE CSSCD
    NIH/NHLBI N01HB097054Oct 1, 1988 - Mar 31, 1994
    Role: Principal Investigator
    PEDIATRIC CLINICAL RESEARCH CENTER
    NIH/NCRR M01RR001271Dec 1, 1981 - Mar 31, 2007
    Role: Co-Investigator
    NORTHERN CALIFORNIA COMPREHENSIVE SICKLE CELL CENTER
    NIH P60HL020985Apr 1, 1978 - Mar 31, 2004
    Role: Co-Investigator

    Collapse ORNG Applications 
    Collapse Clinical Trials
    Collapse Featured Publications
    Collapse Websites
    Collapse In The News
    Collapse Featured Videos

    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. Lal A, Wong TE, Andrews J, Balasa VV, Chung JH, Forester CM, Ikeda AK, Keel SB, Pagano MB, Puthenveetil G, Shah SJ, Yu JC, Vichinsky EP. Transfusion practices and complications in thalassemia. Transfusion. 2018 Sep 27. PMID: 30260477.
      View in: PubMed
    2. Niihara Y, Miller ST, Kanter J, Lanzkron S, Smith WR, Hsu LL, Gordeuk VR, Viswanathan K, Sarnaik S, Osunkwo I, Guillaume E, Sadanandan S, Sieger L, Lasky JL, Panosyan EH, Blake OA, New TN, Bellevue R, Tran LT, Razon RL, Stark CW, Neumayr LD, Vichinsky EP. A Phase 3 Trial of l-Glutamine in Sickle Cell Disease. N Engl J Med. 2018 07 19; 379(3):226-235. PMID: 30021096.
      View in: PubMed
    3. Thompson AA, Walters MC, Kwiatkowski J, Rasko JEJ, Ribeil JA, Hongeng S, Magrin E, Schiller GJ, Payen E, Semeraro M, Moshous D, Lefrere F, Puy H, Bourget P, Magnani A, Caccavelli L, Diana JS, Suarez F, Monpoux F, Brousse V, Poirot C, Brouzes C, Meritet JF, Pondarré C, Beuzard Y, Chrétien S, Lefebvre T, Teachey DT, Anurathapan U, Ho PJ, von Kalle C, Kletzel M, Vichinsky E, Soni S, Veres G, Negre O, Ross RW, Davidson D, Petrusich A, Sandler L, Asmal M, Hermine O, De Montalembert M, Hacein-Bey-Abina S, Blanche S, Leboulch P, Cavazzana M. Gene Therapy in Patients with Transfusion-Dependent ß-Thalassemia. N Engl J Med. 2018 04 19; 378(16):1479-1493. PMID: 29669226.
      View in: PubMed
    4. Vichinsky E, Cohen A, Thompson AA, Giardina PJ, Lal A, Paley C, Cheng WY, McCormick N, Sasane M, Qiu Y, Kwiatkowski JL. Epidemiologic and clinical characteristics of nontransfusion-dependent thalassemia in the United States. Pediatr Blood Cancer. 2018 Apr 10; e27067. PMID: 29637688.
      View in: PubMed
    5. Carlberg KT, Singer ST, Vichinsky EP. Fertility and Pregnancy in Women with Transfusion-Dependent Thalassemia. Hematol Oncol Clin North Am. 2018 Apr; 32(2):297-315. PMID: 29458733.
      View in: PubMed
    6. Kato GJ, Piel FB, Reid CD, Gaston MH, Ohene-Frempong K, Krishnamurti L, Smith WR, Panepinto JA, Weatherall DJ, Costa FF, Vichinsky EP. Sickle cell disease. Nat Rev Dis Primers. 2018 Mar 15; 4:18010. PMID: 29542687.
      View in: PubMed
    7. Khurana M, Fung EB, Vichinsky EP, Theil EC. Dietary nonheme iron is equally bioavailable from ferritin or ferrous sulfate in thalassemia intermedia. Pediatr Hematol Oncol. 2017 Nov; 34(8):455-467. PMID: 29232169.
      View in: PubMed
    8. Vichinsky E. Chronic organ failure in adult sickle cell disease. Hematology Am Soc Hematol Educ Program. 2017 Dec 08; 2017(1):435-439. PMID: 29222290.
      View in: PubMed
    9. Serjeant GR, Vichinsky E. Variability of homozygous sickle cell disease: The role of alpha and beta globin chain variation and other factors. Blood Cells Mol Dis. 2018 May; 70:66-77. PMID: 28689691.
      View in: PubMed
    10. Winstead M, Sun PP, Martin K, Earl J, Neumayr L, Hoppe C, Vichinsky E. Encephaloduroarteriosynangiosis (EDAS) in young patients with cerebrovascular complications of sickle cell disease: Single-institution experience. Pediatr Hematol Oncol. 2017 Mar; 34(2):100-106. PMID: 28532265.
      View in: PubMed
    11. Minniti CP, Vichinsky E. Lifespan care in SCD: Whom to transition, the patients or the health care system? Am J Hematol. 2017 Jun; 92(6):487-489. PMID: 28211097.
      View in: PubMed
    12. Hoppe C, Jacob E, Styles L, Kuypers F, Larkin S, Vichinsky E. Simvastatin reduces vaso-occlusive pain in sickle cell anaemia: a pilot efficacy trial. Br J Haematol. 2017 05; 177(4):620-629. PMID: 28369718.
      View in: PubMed
    13. Vichinsky E, El-Beshlawy A, Al Zoebie A, Kamdem A, Koussa S, Chotsampancharoen T, Bruederle A, Gilotti G, Han J, Elalfy M. Long-term safety and efficacy of deferasirox in young pediatric patients with transfusional hemosiderosis: Results from a 5-year observational study (ENTRUST). Pediatr Blood Cancer. 2017 Sep; 64(9). PMID: 28296163.
      View in: PubMed
    14. Field JJ, Majerus E, Ataga KI, Vichinsky EP, Schaub R, Mashal R, Nathan DG. NNKTT120, an anti-iNKT cell monoclonal antibody, produces rapid and sustained iNKT cell depletion in adults with sickle cell disease. PLoS One. 2017; 12(2):e0171067. PMID: 28152086.
      View in: PubMed
    15. Paulukonis ST, Feuchtbaum LB, Coates TD, Neumayr LD, Treadwell MJ, Vichinsky EP, Hulihan MM. Emergency department utilization by Californians with sickle cell disease, 2005-2014. Pediatr Blood Cancer. 2017 Jun; 64(6). PMID: 28000344.
      View in: PubMed
    16. Kreger EM, Singer ST, Witt RG, Sweeters N, Lianoglou B, Lal A, Mackenzie TC, Vichinsky E. Favorable outcomes after in utero transfusion in fetuses with alpha thalassemia major: a case series and review of the literature. Prenat Diagn. 2016 Dec; 36(13):1242-1249. PMID: 27862048.
      View in: PubMed
    17. Neumayr L, Vichinsky E. Stroke recurrence in adult sickle cell patients: it is time for action! Transfusion. 2016 05; 56(5):1001-4. PMID: 27167352.
      View in: PubMed
    18. Vichinsky E. Non-transfusion-dependent thalassemia and thalassemia intermedia: epidemiology, complications, and management. Curr Med Res Opin. 2016; 32(1):191-204. PMID: 26479125.
      View in: PubMed
    19. Singer ST, Killilea D, Suh JH, Wang ZJ, Yuan Q, Ivani K, Evans P, Vichinsky E, Fischer R, Smith JF. Fertility in transfusion-dependent thalassemia men: effects of iron burden on the reproductive axis. Am J Hematol. 2015 Sep; 90(9):E190-2. PMID: 26044409; PMCID: PMC4676944.
    20. Morris CR, Kim HY, Klings ES, Wood J, Porter JB, Trachtenberg F, Sweeters N, Olivieri NF, Kwiatkowski JL, Virzi L, Hassell K, Taher A, Neufeld EJ, Thompson AA, Larkin S, Suh JH, Vichinsky EP, Kuypers FA. Dysregulated arginine metabolism and cardiopulmonary dysfunction in patients with thalassaemia. Br J Haematol. 2015 Jun; 169(6):887-98. PMID: 25907665; PMCID: PMC4452408.
    21. Ballas SK, Vichinsky EP. Is the medical home for adult patients with sickle cell disease a reality or an illusion? Hemoglobin. 2015; 39(2):130-3. PMID: 25806421.
      View in: PubMed
    22. Derderian SC, Jeanty C, Walters MC, Vichinsky E, MacKenzie TC. In utero hematopoietic cell transplantation for hemoglobinopathies. Front Pharmacol. 2014; 5:278. PMID: 25628564; PMCID: PMC4290536.
    23. Porter JB, Walter PB, Neumayr LD, Evans P, Bansal S, Garbowski M, Weyhmiller MG, Harmatz PR, Wood JC, Miller JL, Byrnes C, Weiss G, Seifert M, Grosse R, Grabowski D, Schmidt A, Fischer R, Nielsen P, Niemeyer C, Vichinsky E. Mechanisms of plasma non-transferrin bound iron generation: insights from comparing transfused diamond blackfan anaemia with sickle cell and thalassaemia patients. Br J Haematol. 2014 Dec; 167(5):692-6. PMID: 25209728; PMCID: PMC4577015.
    24. Paulukonis ST, Harris WT, Coates TD, Neumayr L, Treadwell M, Vichinsky E, Feuchtbaum LB. Population based surveillance in sickle cell disease: methods, findings and implications from the California registry and surveillance system in hemoglobinopathies project (RuSH). Pediatr Blood Cancer. 2014 Dec; 61(12):2271-6. PMID: 25176145.
      View in: PubMed
    25. Vichinsky, Elliott P., ed.Emerging Therapies Targeting the Pathophysiology of Sickle Cell Disease. Hematology/Oncology Clinics of North America. 2014; 155-414.
    26. Trachtenberg FL, Gerstenberger E, Xu Y, Mednick L, Sobota A, Ware H, Thompson AA, Neufeld EJ, Yamashita R. Relationship among chelator adherence, change in chelators, and quality of life in thalassemia. Qual Life Res. 2014 Oct; 23(8):2277-88. PMID: 24682717; PMCID: PMC4315322.
    27. Singer ST, Kuypers F, Fineman J, Gildengorin G, Larkin S, Sweeters N, Rosenfeld H, Kurio G, Higa A, Jeng M, Huang J, Vichinsky EP. Elevated tricuspid regurgitant jet velocity in subgroups of thalassemia patients: insight into pathophysiology and the effect of splenectomy. Ann Hematol. 2014 Jul; 93(7):1139-48. PMID: 24577514; PMCID: PMC4467727.
    28. Mackin RS, Insel P, Truran D, Vichinsky EP, Neumayr LD, Armstrong FD, Gold JI, Kesler K, Brewer J, Weiner MW. Neuroimaging abnormalities in adults with sickle cell anemia: associations with cognition. Neurology. 2014 Mar 11; 82(10):835-41. PMID: 24523480; PMCID: PMC3959758.
    29. Klings ES, Vichinsky EP, Morris CR. Response to "efficacy and safety of sildenafil for the treatment of severe pulmonary hypertension in patients with hemoglobinopathies: results from a long-term follow up " Haematologica 2014;99(2):e17-18. Haematologica. 2014 Feb; 99(2):e19. PMID: 24497564; PMCID: PMC3912978.
    30. Vichinsky EP. Emerging therapy in hemoglobinopathies: lessons from the past and optimism for the future. Hematol Oncol Clin North Am. 2014 Apr; 28(2):xiii-xviii. PMID: 24589274.
      View in: PubMed
    31. Klaassen RJ, Barrowman N, Merelles-Pulcini M, Vichinsky EP, Sweeters N, Kirby-Allen M, Neufeld EJ, Kwiatkowski JL, Wu J, Vickars L, Blanchette VS, Forgie M, Yamashita R, Wong-Rieger D, Young NL. Validation and reliability of a disease-specific quality of life measure (the TranQol) in adults and children with thalassaemia major. Br J Haematol. 2014 Feb; 164(3):431-7. PMID: 24180641.
      View in: PubMed
    32. Oliveros O, Trachtenberg F, Haines D, Gerstenberger E, Martin M, Carson S, Green S, Calamaras D, Hess P, Yamashita R, Vichinsky E. Pain over time and its effects on life in thalassemia. Am J Hematol. 2013 Nov; 88(11):939-43. PMID: 23918696; PMCID: PMC3872488.
    33. Marsh A, Golden C, Hoppe C, Quirolo K, Vichinsky E. Renal medullary carcinoma in an adolescent with sickle cell anemia. Pediatr Blood Cancer. 2014 Mar; 61(3):567. PMID: 24108577.
      View in: PubMed
    34. Vichinsky E, Torres M, Minniti CP, Barrette S, Habr D, Zhang Y, Files B. Efficacy and safety of deferasirox compared with deferoxamine in sickle cell disease: two-year results including pharmacokinetics and concomitant hydroxyurea. Am J Hematol. 2013 Dec; 88(12):1068-73. PMID: 23946212.
      View in: PubMed
    35. Fung EB, Kwiatkowski JL, Huang JN, Gildengorin G, King JC, Vichinsky EP. Zinc supplementation improves bone density in patients with thalassemia: a double-blind, randomized, placebo-controlled trial. Am J Clin Nutr. 2013 Oct; 98(4):960-71. PMID: 23945720; PMCID: PMC3778866.
    36. Pratini NR, Sweeters N, Vichinsky E, Neufeld JA. Treatment of classic pantothenate kinase-associated neurodegeneration with deferiprone and intrathecal baclofen. Am J Phys Med Rehabil. 2013 Aug; 92(8):728-33. PMID: 23370589; PMCID: PMC3707939.
    37. Vichinsky E, Neumayr L, Trimble S, Giardina PJ, Cohen AR, Coates T, Boudreaux J, Neufeld EJ, Kenney K, Grant A, Thompson AA. Transfusion complications in thalassemia patients: a report from the Centers for Disease Control and Prevention (CME). Transfusion. 2014 Apr; 54(4):972-81; quiz 971. PMID: 23889533; PMCID: PMC4410835.
    38. Thompson AA, Kim HY, Singer ST, Vichinsky E, Eile J, Yamashita R, Giardina PJ, Olivieri N, Parmar N, Trachtenberg F, Neufeld EJ, Kwiatkowski JL. Pregnancy outcomes in women with thalassemia in North America and the United Kingdom. Am J Hematol. 2013 Sep; 88(9):771-3. PMID: 23757266; PMCID: PMC4106126.
    39. Musallam KM, Rivella S, Vichinsky E, Rachmilewitz EA. Non-transfusion-dependent thalassemias. Haematologica. 2013 Jun; 98(6):833-44. PMID: 23729725; PMCID: PMC3669437.
    40. Porter JB, Wood J, Olivieri N, Vichinsky EP, Taher A, Neufeld E, Giardina P, Thompson A, Moore B, Evans P, Kim HY, Macklin EA, Trachtenberg F. Treatment of heart failure in adults with thalassemia major: response in patients randomised to deferoxamine with or without deferiprone. J Cardiovasc Magn Reson. 2013 May 20; 15:38. PMID: 23688265; PMCID: PMC3669105.
    41. Morris CR, Kuypers FA, Lavrisha L, Ansari M, Sweeters N, Stewart M, Gildengorin G, Neumayr L, Vichinsky EP. A randomized, placebo-controlled trial of arginine therapy for the treatment of children with sickle cell disease hospitalized with vaso-occlusive pain episodes. Haematologica. 2013 Sep; 98(9):1375-82. PMID: 23645695; PMCID: PMC3762093.
    42. Vichinsky EP. Clinical manifestations of a-thalassemia. Cold Spring Harb Perspect Med. 2013 May 01; 3(5):a011742. PMID: 23543077; PMCID: PMC3633183.
    43. Goldberg SL, Giardina PJ, Chirnomas D, Esposito J, Paley C, Vichinsky E. The palatability and tolerability of deferasirox taken with different beverages or foods. Pediatr Blood Cancer. 2013 Sep; 60(9):1507-12. PMID: 23637051.
      View in: PubMed
    44. Morris CR, Kim HY, Wood J, Porter JB, Klings ES, Trachtenberg FL, Sweeters N, Olivieri NF, Kwiatkowski JL, Virzi L, Singer ST, Taher A, Neufeld EJ, Thompson AA, Sachdev V, Larkin S, Suh JH, Kuypers FA, Vichinsky EP. Sildenafil therapy in thalassemia patients with Doppler-defined risk of pulmonary hypertension. Haematologica. 2013 Sep; 98(9):1359-67. PMID: 23585527; PMCID: PMC3762091.
    45. Cui J, Azimi M, Baysdorfer C, Vichinsky EP, Hoppe CC. Application of multiplex ligation-dependent probe amplification to screen for ß-globin cluster deletions: detection of two novel deletions in a multi ethnic population. Hemoglobin. 2013; 37(3):241-56. PMID: 23577918.
      View in: PubMed
    46. Switzer WM, Shankar A, Trimble SR, Thompson AA, Giardina PJ, Cohen AR, Coates TD, Vichinsky E, Neufeld EJ, Boudreaux JM, Heneine W. Human T cell lymphotropic virus type 1 infection among U.S. thalassemia patients. AIDS Res Hum Retroviruses. 2013 Jul; 29(7):1006-9. PMID: 23409829.
      View in: PubMed
    47. Haines D, Martin M, Carson S, Oliveros O, Green S, Coates T, Eile J, Schilling L, Dinu B, Mendoza T, Gerstenberger E, Trachtenberg F, Vichinsky E. Pain in thalassaemia: the effects of age on pain frequency and severity. Br J Haematol. 2013 Mar; 160(5):680-7. PMID: 23278768.
      View in: PubMed
    48. Walter PB, Porter J, Evans P, Kwiatkowski JL, Neufeld EJ, Coates T, Giardina PJ, Grady RW, Vichinsky E, Olivieri N, Trachtenberg F, Alberti D, Fung E, Ames B, Higa A, Harmatz P. Increased leucocyte apoptosis in transfused ß-thalassaemia patients. Br J Haematol. 2013 Feb; 160(3):399-403. PMID: 23216540; PMCID: PMC4597914.
    49. Lal A, Porter J, Sweeters N, Ng V, Evans P, Neumayr L, Kurio G, Harmatz P, Vichinsky E. Combined chelation therapy with deferasirox and deferoxamine in thalassemia. Blood Cells Mol Dis. 2013 Feb; 50(2):99-104. PMID: 23151373; PMCID: PMC3592978.
    50. Kutlar A, Ataga K, Reid M, Vichinsky EP, Neumayr L, Blair-Britt L, Labotka R, Glass J, Keefer JR, Wargin WA, Berenson R, Perrine SP. A phase 1/2 trial of HQK-1001, an oral fetal globin inducer, in sickle cell disease. Am J Hematol. 2012 Nov; 87(11):1017-21. PMID: 22887019; PMCID: PMC3904792.
    51. Fung EB, Gariepy CA, Sawyer AJ, Higa A, Vichinsky EP. The effect of whole body vibration therapy on bone density in patients with thalassemia: a pilot study. Am J Hematol. 2012 Oct; 87(10):E76-9. PMID: 22886910; PMCID: PMC3845963.
    52. Fung EB, Xu Y, Trachtenberg F, Odame I, Kwiatkowski JL, Neufeld EJ, Thompson AA, Boudreaux J, Quinn CT, Vichinsky EP. Inadequate dietary intake in patients with thalassemia. J Acad Nutr Diet. 2012 Jul; 112(7):980-90. PMID: 22551675; PMCID: PMC3419338.
    53. Wahl SK, Garcia A, Hagar W, Gildengorin G, Quirolo K, Vichinsky E. Lower alloimmunization rates in pediatric sickle cell patients on chronic erythrocytapheresis compared to chronic simple transfusions. Transfusion. 2012 Dec; 52(12):2671-6. PMID: 22519830.
      View in: PubMed
    54. Kutlar A, Ataga KI, McMahon L, Howard J, Galacteros F, Hagar W, Vichinsky E, Cheung AT, Matsui N, Embury SH. A potent oral P-selectin blocking agent improves microcirculatory blood flow and a marker of endothelial cell injury in patients with sickle cell disease. Am J Hematol. 2012 May; 87(5):536-9. PMID: 22488107.
      View in: PubMed
    55. Vichinsky E. Advances in the treatment of alpha-thalassemia. Blood Rev. 2012 Apr; 26 Suppl 1:S31-4. PMID: 22631041.
      View in: PubMed
    56. Cui J, Baysdorfer C, Azimi M, Vichinsky EP, Hoppe CC. Identification of three novel Hb F variants: Hb F-Hayward [G?1(NA1)Gly?Asp, GGT>GAT], Hb F-Chori-I [A?T16(A13)Gly?Asp, GGC>GAC] and Hb F-Chori-II [A?I29(B11)Gly?Glu, GGA>GAA]. Hemoglobin. 2012; 36(3):305-9. PMID: 22384921.
      View in: PubMed
    57. DeBaun MR, Armstrong FD, McKinstry RC, Ware RE, Vichinsky E, Kirkham FJ. Silent cerebral infarcts: a review on a prevalent and progressive cause of neurologic injury in sickle cell anemia. Blood. 2012 May 17; 119(20):4587-96. PMID: 22354000; PMCID: PMC3367871.
    58. Vichinsky E.. British Journal of Haematology. Safety of deferasirox in sickle cell disease patients with co-existing liver impairment – response to Sinakos et al. 2012; 157(4):506–507.
    59. Kwiatkowski JL, Kim HY, Thompson AA, Quinn CT, Mueller BU, Odame I, Giardina PJ, Vichinsky EP, Boudreaux JM, Cohen AR, Porter JB, Coates T, Olivieri NF, Neufeld EJ. Chelation use and iron burden in North American and British thalassemia patients: a report from the Thalassemia Longitudinal Cohort. Blood. 2012 Mar 22; 119(12):2746-53. PMID: 22279056; PMCID: PMC3327453.
    60. Vichinsky E. Emerging 'A' therapies in hemoglobinopathies: agonists, antagonists, antioxidants, and arginine. Hematology Am Soc Hematol Educ Program. 2012; 2012:271-5. PMID: 23233591.
      View in: PubMed
    61. Olivieri NF, Pakbaz Z, Vichinsky E. Hb E/beta-thalassaemia: a common & clinically diverse disorder. Indian J Med Res. 2011 Oct; 134:522-31. PMID: 22089616; PMCID: PMC3237252.
    62. Morris CR, Kim HY, Trachtenberg F, Wood J, Quinn CT, Sweeters N, Kwiatkowski JL, Thompson AA, Giardina PJ, Boudreaux J, Olivieri NF, Porter JB, Neufeld EJ, Vichinsky EP. Risk factors and mortality associated with an elevated tricuspid regurgitant jet velocity measured by Doppler-echocardiography in thalassemia: a Thalassemia Clinical Research Network report. Blood. 2011 Oct 06; 118(14):3794-802. PMID: 21772051; PMCID: PMC3193260.
    63. Singer ST, Vichinsky EP, Gildengorin G, van Disseldorp J, Rosen M, Cedars MI. Reproductive capacity in iron overloaded women with thalassemia major. Blood. 2011 Sep 08; 118(10):2878-81. PMID: 21757620; PMCID: PMC3172803.
    64. Olivieri NF, Saunthararajah Y, Thayalasuthan V, Kwiatkowski J, Ware RE, Kuypers FA, Kim HY, Trachtenberg FL, Vichinsky EP. A pilot study of subcutaneous decitabine in ß-thalassemia intermedia. Blood. 2011 Sep 08; 118(10):2708-11. PMID: 21700776; PMCID: PMC3172790.
    65. Vichinsky E, Bernaudin F, Forni GL, Gardner R, Hassell K, Heeney MM, Inusa B, Kutlar A, Lane P, Mathias L, Porter J, Tebbi C, Wilson F, Griffel L, Deng W, Giannone V, Coates T. Long-term safety and efficacy of deferasirox (Exjade) for up to 5 years in transfusional iron-overloaded patients with sickle cell disease. Br J Haematol. 2011 Aug; 154(3):387-97. PMID: 21592110; PMCID: PMC3170481.
    66. Trachtenberg F, Vichinsky E, Haines D, Pakbaz Z, Mednick L, Sobota A, Kwiatkowski J, Thompson AA, Porter J, Coates T, Giardina PJ, Olivieri N, Yamashita R, Neufeld EJ. Iron chelation adherence to deferoxamine and deferasirox in thalassemia. Am J Hematol. 2011 May; 86(5):433-6. PMID: 21523808; PMCID: PMC4599708.
    67. Singer ST, Vichinsky EP, Sweeters N, Rachmilewitz E. Darbepoetin alfa for the treatment of anaemia in alpha- or beta- thalassaemia intermedia syndromes. Br J Haematol. 2011 Jul; 154(2):281-4. PMID: 21496003.
      View in: PubMed
    68. Hoppe C, Kuypers F, Larkin S, Hagar W, Vichinsky E, Styles L. A pilot study of the short-term use of simvastatin in sickle cell disease: effects on markers of vascular dysfunction. Br J Haematol. 2011 Jun; 153(5):655-63. PMID: 21477202; PMCID: PMC3601917.
    69. Fung EB, Vichinsky EP, Kwiatkowski JL, Huang J, Bachrach LK, Sawyer AJ, Zemel BS. Characterization of low bone mass in young patients with thalassemia by DXA, pQCT and markers of bone turnover. Bone. 2011 Jun 01; 48(6):1305-12. PMID: 21443975; PMCID: PMC3095710.
    70. Gold JI, Treadwell M, Weissman L, Vichinsky E. The mediating effects of family functioning on psychosocial outcomes in healthy siblings of children with sickle cell disease. Pediatr Blood Cancer. 2011 Dec 01; 57(6):1055-61. PMID: 21360652.
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    71. Lal A, Goldrich ML, Haines DA, Azimi M, Singer ST, Vichinsky EP. Heterogeneity of hemoglobin H disease in childhood. N Engl J Med. 2011 Feb 24; 364(8):710-8. PMID: 21345100.
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    72. Thompson AA, Cunningham MJ, Singer ST, Neufeld EJ, Vichinsky E, Yamashita R, Giardina P, Kim HY, Trachtenberg F, Kwiatkowski JL. Red cell alloimmunization in a diverse population of transfused patients with thalassaemia. Br J Haematol. 2011 Apr; 153(1):121-8. PMID: 21323889.
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    73. Wang, W., Brugnara, C., Snyder, C., Wynn, L., Rogers, Z., Kalinyak, K., Brown, C., Qureshi, A., Bigelow, C., Neumayr, L., Smith-Whitley, K., Chui, D. H. K., Delahunty, M., Woolson, R., Steinberg, M., Telen, M. and Kesler, K.The effects of hydroxycarbamide and magnesium on haemoglobin SC disease: results of the multi-centre CHAMPS trial. British Journal of Haematology. 2011; 152(6):771–776.
    74. Jacobs JE, Quirolo K, Vichinsky E. Novel influenza A (H1N1) viral infection in pediatric patients with sickle-cell disease. Pediatr Blood Cancer. 2011 Jan; 56(1):95-8. PMID: 20973097.
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    75. Rienhoff HY, Viprakasit V, Tay L, Harmatz P, Vichinsky E, Chirnomas D, Kwiatkowski JL, Tapper A, Kramer W, Porter JB, Neufeld EJ. A phase 1 dose-escalation study: safety, tolerability, and pharmacokinetics of FBS0701, a novel oral iron chelator for the treatment of transfusional iron overload. Haematologica. 2011 Apr; 96(4):521-5. PMID: 21173101; PMCID: PMC3069228.
    76. Olivieri NF, Pakbaz Z, Vichinsky E. HbE/ß-thalassemia: basis of marked clinical diversity. Hematol Oncol Clin North Am. 2010 Dec; 24(6):1055-70. PMID: 21075280.
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    77. Neumayr L, Pringle S, Giles S, Quirolo KC, Paulukonis S, Vichinsky EP, Treadwell MJ. Chart Card: feasibility of a tool for improving emergency department care in sickle cell disease. J Natl Med Assoc. 2010 Nov; 102(11):1017-23. PMID: 21141289; PMCID: PMC4950679.
    78. Pakbaz Z, Treadwell M, Kim HY, Trachtenberg F, Parmar N, Kwiatkowski JL, Cunningham MJ, Martin M, Sweeters N, Neufeld EJ, Giardina PJ, Olivieri N, Yamashita RC, Vichinsky E. Education and employment status of children and adults with thalassemia in North America. Pediatr Blood Cancer. 2010 Oct; 55(4):678-83. PMID: 20535817; PMCID: PMC2932798.
    79. Gladwin MT, Barst RJ, Castro OL, Gordeuk VR, Hillery CA, Kato GJ, Kim-Shapiro DB, Machado R, Morris CR, Steinberg MH, Vichinsky EP. Pulmonary hypertension and NO in sickle cell. Blood. 2010 Aug 05; 116(5):852-4. PMID: 20688967.
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    80. Morris CR, Vichinsky EP. Pulmonary hypertension in thalassemia. Ann N Y Acad Sci. 2010 Aug; 1202:205-13. PMID: 20712794.
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    81. Vichinsky EP, Neufeld EJ. Preface to Cooley's Anemia: Ninth Symposium. Ann N Y Acad Sci. 2010 Aug; 1202:ix-x. PMID: 20712763.
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    82. Singer ST, Sweeters N, Vega O, Higa A, Vichinsky E, Cedars M. Fertility potential in thalassemia major women: current findings and future diagnostic tools. Ann N Y Acad Sci. 2010 Aug; 1202:226-30. PMID: 20712797.
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    83. Vichinsky E. Complexity of alpha thalassemia: growing health problem with new approaches to screening, diagnosis, and therapy. Ann N Y Acad Sci. 2010 Aug; 1202:180-7. PMID: 20712791.
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    84. Vichinsky EP, Ohene-Frempong K, Thein SL, Lobo CL, Inati A, Thompson AA, Smith-Whitley K, Kwiatkowski JL, Swerdlow PS, Porter JB, Marks PW. Transfusion and chelation practices in sickle cell disease: a regional perspective. Pediatr Hematol Oncol. 2011 Mar; 28(2):124-33. PMID: 20673030.
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    85. Vichinsky EP, Ohene-Frempong K. Approaches to transfusion therapy and iron overload in patients with sickle cell disease: results of an international survey. Pediatr Hematol Oncol. 2011 Feb; 28(1):37-42. PMID: 20673029.
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    86. Fung EB, Xu Y, Kwiatkowski JL, Vogiatzi MG, Neufeld E, Olivieri N, Vichinsky EP, Giardina PJ. Relationship between chronic transfusion therapy and body composition in subjects with thalassemia. J Pediatr. 2010 Oct; 157(4):641-7, 647.e1-2. PMID: 20547400; PMCID: PMC2936667.
    87. Vichinsky EP, Neumayr LD, Gold JI, Weiner MW, Rule RR, Truran D, Kasten J, Eggleston B, Kesler K, McMahon L, Orringer EP, Harrington T, Kalinyak K, De Castro LM, Kutlar A, Rutherford CJ, Johnson C, Bessman JD, Jordan LB, Armstrong FD. Neuropsychological dysfunction and neuroimaging abnormalities in neurologically intact adults with sickle cell anemia. JAMA. 2010 May 12; 303(18):1823-31. PMID: 20460621; PMCID: PMC2892214.
    88. James EB, Vreman HJ, Wong RJ, Stevenson DK, Vichinsky E, Schumacher L, Hall JY, Simon J, Golden DW, Harmatz P. Elevated exhaled carbon monoxide concentration in hemoglobinopathies and its relation to red blood cell transfusion therapy. Pediatr Hematol Oncol. 2010 Mar; 27(2):112-21. PMID: 20201692.
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    89. Wahl S, Vichinsky E. Pulmonary hypertension in hemolytic anemias. F1000 Med Rep. 2010 Feb 11; 2. PMID: 20948876; PMCID: PMC2948405.
    90. Wang ZJ, Fischer R, Chu Z, Mahoney DH, Mueller BU, Muthupillai R, James EB, Krishnamurthy R, Chung T, Padua E, Vichinsky E, Harmatz P. Assessment of cardiac iron by MRI susceptometry and R2* in patients with thalassemia. Magn Reson Imaging. 2010 Apr; 28(3):363-71. PMID: 20061110; PMCID: PMC2839041.
    91. Gabrovsky A, Aderinto A, Aderinto A, Spevak M, Vichinsky E, Resar LM. Low dose, oral epsilon aminocaproic acid for renal papillary necrosis and massive hemorrhage in hemoglobin SC disease. Pediatr Blood Cancer. 2010 Jan; 54(1):148-50. PMID: 19785026.
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    92. Walter PB, Harmatz P, Vichinsky E. Iron metabolism and iron chelation in sickle cell disease. Acta Haematol. 2009; 122(2-3):174-83. PMID: 19907155.
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    93. Singer ST, Kim HY, Olivieri NF, Kwiatkowski JL, Coates TD, Carson S, Neufeld E, Cunningham MJ, Giardina PJ, Mueller BU, Quinn CT, Fung E, Vichinsky E. Hemoglobin H-constant spring in North America: an alpha thalassemia with frequent complications. Am J Hematol. 2009 Nov; 84(11):759-61. PMID: 19787795; PMCID: PMC4254706.
    94. Buchanan G, Vichinsky E, Krishnamurti L, Shenoy S. Severe sickle cell disease--pathophysiology and therapy. Biol Blood Marrow Transplant. 2010 Jan; 16(1 Suppl):S64-7. PMID: 19819341; PMCID: PMC2832723.
    95. Taher A, Cappellini MD, Vichinsky E, Galanello R, Piga A, Lawniczek T, Clark J, Habr D, Porter JB. Efficacy and safety of deferasirox doses of >30 mg/kg per d in patients with transfusion-dependent anaemia and iron overload. Br J Haematol. 2009 Dec; 147(5):752-9. PMID: 19764988; PMCID: PMC2779992.
    96. Adamkiewicz TV, Abboud MR, Paley C, Olivieri N, Kirby-Allen M, Vichinsky E, Casella JF, Alvarez OA, Barredo JC, Lee MT, Iyer RV, Kutlar A, McKie KM, McKie V, Odo N, Gee B, Kwiatkowski JL, Woods GM, Coates T, Wang W, Adams RJ. Serum ferritin level changes in children with sickle cell disease on chronic blood transfusion are nonlinear and are associated with iron load and liver injury. Blood. 2009 Nov 19; 114(21):4632-8. PMID: 19721013; PMCID: PMC2780299.
    97. Vogiatzi MG, Macklin EA, Trachtenberg FL, Fung EB, Cheung AM, Vichinsky E, Olivieri N, Kirby M, Kwiatkowski JL, Cunningham M, Holm IA, Fleisher M, Grady RW, Peterson CM, Giardina PJ. Differences in the prevalence of growth, endocrine and vitamin D abnormalities among the various thalassaemia syndromes in North America. Br J Haematol. 2009 Sep; 146(5):546-56. PMID: 19604241; PMCID: PMC2798591.
    98. Hoppe C, Klitz W, Vichinsky E, Styles L. HLA type and risk of alloimmunization in sickle cell disease. Am J Hematol. 2009 Jul; 84(7):462-4. PMID: 19484735.
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    99. Michlitsch J, Azimi M, Hoppe C, Walters MC, Lubin B, Lorey F, Vichinsky E. Newborn screening for hemoglobinopathies in California. Pediatr Blood Cancer. 2009 Apr; 52(4):486-90. PMID: 19061217; PMCID: PMC4755934.
    100. Vogiatzi MG, Macklin EA, Fung EB, Cheung AM, Vichinsky E, Olivieri N, Kirby M, Kwiatkowski JL, Cunningham M, Holm IA, Lane J, Schneider R, Fleisher M, Grady RW, Peterson CC, Giardina PJ. Bone disease in thalassemia: a frequent and still unresolved problem. J Bone Miner Res. 2009 Mar; 24(3):543-57. PMID: 18505376; PMCID: PMC3276604.
    101. Vichinsky EP. Alpha thalassemia major--new mutations, intrauterine management, and outcomes. Hematology Am Soc Hematol Educ Program. 2009; 35-41. PMID: 20008180.
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    102. Gladwin MT, Vichinsky E. Pulmonary complications of sickle cell disease. N Engl J Med. 2008 Nov 20; 359(21):2254-65. PMID: 19020327.
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    103. Vichinsky E, Rodgers GP, Rachmilewitz E. Hemoglobinopathies. Curr Mol Med. 2008 Nov; 8(7):591. PMID: 18991644.
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    104. Singer ST, Vichinsky EP, Larkin S, Olivieri N, Sweeters N, Kuypers FA. Hydroxycarbamide-induced changes in E/beta thalassemia red blood cells. Am J Hematol. 2008 Nov; 83(11):842-5. PMID: 18821710; PMCID: PMC2881160.
    105. Gold JI, Mahrer NE, Treadwell M, Weissman L, Vichinsky E. Psychosocial and behavioral outcomes in children with sickle cell disease and their healthy siblings. J Behav Med. 2008 Dec; 31(6):506-16. PMID: 18784995.
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    106. Harmatz P, Jonas MM, Kwiatkowski JL, Wright EC, Fischer R, Vichinsky E, Giardina PJ, Neufeld EJ, Porter J, Olivieri N. Safety and efficacy of pegylated interferon alpha-2a and ribavirin for the treatment of hepatitis C in patients with thalassemia. Haematologica. 2008 Aug; 93(8):1247-51. PMID: 18556414.
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    107. Vichinsky E. Oral iron chelators and the treatment of iron overload in pediatric patients with chronic anemia. Pediatrics. 2008 Jun; 121(6):1253-6. PMID: 18519495.
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    108. Gold JI, Johnson CB, Treadwell MJ, Hans N, Vichinsky E. Detection and assessment of stroke in patients with sickle cell disease: neuropsychological functioning and magnetic resonance imaging. Pediatr Hematol Oncol. 2008 Jun; 25(5):409-21. PMID: 18569843.
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    109. Fung EB, Harmatz PR, Milet M, Balasa V, Ballas SK, Casella JF, Hilliard L, Kutlar A, McClain KL, Olivieri NF, Porter JB, Vichinsky EP. Disparity in the management of iron overload between patients with sickle cell disease and thalassemia who received transfusions. Transfusion. 2008 Sep; 48(9):1971-80. PMID: 18513257; PMCID: PMC4613769.
    110. Walter PB, Macklin EA, Porter J, Evans P, Kwiatkowski JL, Neufeld EJ, Coates T, Giardina PJ, Vichinsky E, Olivieri N, Alberti D, Holland J, Harmatz P. Inflammation and oxidant-stress in beta-thalassemia patients treated with iron chelators deferasirox (ICL670) or deferoxamine: an ancillary study of the Novartis CICL670A0107 trial. Haematologica. 2008 Jun; 93(6):817-25. PMID: 18469351.
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    111. Vichinsky E. Clinical application of deferasirox: practical patient management. Am J Hematol. 2008 May; 83(5):398-402. PMID: 18058997.
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    112. Vichinsky E, Pakbaz Z, Onyekwere O, Porter J, Swerdlow P, Coates T, Lane P, Files B, Mueller BU, Coïc L, Forni GL, Fischer R, Marks P, Rofail D, Abetz L, Baladi JF. Patient-reported outcomes of deferasirox (Exjade, ICL670) versus deferoxamine in sickle cell disease patients with transfusional hemosiderosis. Substudy of a randomized open-label phase II trial. Acta Haematol. 2008; 119(3):133-41. PMID: 18408362.
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    113. Fung EB, Harmatz PR, Milet M, Coates TD, Thompson AA, Ranalli M, Mignaca R, Scher C, Giardina P, Robertson S, Neumayr L, Vichinsky EP. Fracture prevalence and relationship to endocrinopathy in iron overloaded patients with sickle cell disease and thalassemia. Bone. 2008 Jul; 43(1):162-168. PMID: 18430624; PMCID: PMC2500183.
    114. Hagar W, Vichinsky E. Advances in clinical research in sickle cell disease. Br J Haematol. 2008 May; 141(3):346-56. PMID: 18341629.
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    115. Jastaniah W, Harmatz P, Pakbaz Z, Fischer R, Vichinsky E, Walters MC. Transfusional iron burden and liver toxicity after bone marrow transplantation for acute myelogenous leukemia and hemoglobinopathies. Pediatr Blood Cancer. 2008 Feb; 50(2):319-24. PMID: 17557314.
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    116. Ataga KI, Smith WR, De Castro LM, Swerdlow P, Saunthararajah Y, Castro O, Vichinsky E, Kutlar A, Orringer EP, Rigdon GC, Stocker JW. Efficacy and safety of the Gardos channel blocker, senicapoc (ICA-17043), in patients with sickle cell anemia. Blood. 2008 Apr 15; 111(8):3991-7. PMID: 18192510.
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    117. Porter JB, Taher AT, Cappellini MD, Vichinsky EP. Ethical issues and risk/benefit assessment of iron chelation therapy: advances with deferiprone/deferoxamine combinations and concerns about the safety, efficacy and costs of deferasirox [Kontoghiorghes GJ, Hemoglobin 2008; 32(1-2):1-15.]. Hemoglobin. 2008; 32(6):601-7. PMID: 19065340.
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    118. Porter J, Galanello R, Saglio G, Neufeld EJ, Vichinsky E, Cappellini MD, Olivieri N, Piga A, Cunningham MJ, Soulières D, Gattermann N, Tchernia G, Maertens J, Giardina P, Kwiatkowski J, Quarta G, Jeng M, Forni GL, Stadler M, Cario H, Debusscher L, Della Porta M, Cazzola M, Greenberg P, Alimena G, Rabault B, Gathmann I, Ford JM, Alberti D, Rose C. Relative response of patients with myelodysplastic syndromes and other transfusion-dependent anaemias to deferasirox (ICL670): a 1-yr prospective study. Eur J Haematol. 2008 Feb; 80(2):168-76. PMID: 18028431; PMCID: PMC2268958.
    119. Hagar RW, Michlitsch JG, Gardner J, Vichinsky EP, Morris CR. Clinical differences between children and adults with pulmonary hypertension and sickle cell disease. Br J Haematol. 2008 Jan; 140(1):104-12. PMID: 17916102.
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    120. Morris CR, Suh JH, Hagar W, Larkin S, Bland DA, Steinberg MH, Vichinsky EP, Shigenaga M, Ames B, Kuypers FA, Klings ES. Erythrocyte glutamine depletion, altered redox environment, and pulmonary hypertension in sickle cell disease. Blood. 2008 Jan 01; 111(1):402-10. PMID: 17848621; PMCID: PMC2200820.
    121. Pakbaz Z, Fischer R, Fung E, Nielsen P, Harmatz P, Vichinsky E. Serum ferritin underestimates liver iron concentration in transfusion independent thalassemia patients as compared to regularly transfused thalassemia and sickle cell patients. Pediatr Blood Cancer. 2007 Sep; 49(3):329-32. PMID: 17554789.
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    122. Harmatz P, Grady RW, Dragsten P, Vichinsky E, Giardina P, Madden J, Jeng M, Miller B, Hanson G, Hedlund B. Phase Ib clinical trial of starch-conjugated deferoxamine (40SD02): a novel long-acting iron chelator. Br J Haematol. 2007 Aug; 138(3):374-81. PMID: 17614825.
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    123. Jenkins ZA, Hagar W, Bowlus CL, Johansson HE, Harmatz P, Vichinsky EP, Theil EC. Iron homeostasis during transfusional iron overload in beta-thalassemia and sickle cell disease: changes in iron regulatory protein, hepcidin, and ferritin expression. Pediatr Hematol Oncol. 2007 Jun; 24(4):237-43. PMID: 17613866.
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    124. Cappellini MD, Bejaoui M, Agaoglu L, Porter J, Coates T, Jeng M, Lai ME, Mangiagli A, Strauss G, Girot R, Watman N, Ferster A, Loggetto S, Abish S, Cario H, Zoumbos N, Vichinsky E, Opitz H, Ressayre-Djaffer C, Abetz L, Rofail D, Baladi JF. Prospective evaluation of patient-reported outcomes during treatment with deferasirox or deferoxamine for iron overload in patients with beta-thalassemia. Clin Ther. 2007 May; 29(5):909-917. PMID: 17697909.
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    125. Fung EB, Harmatz P, Milet M, Ballas SK, De Castro L, Hagar W, Owen W, Olivieri N, Smith-Whitley K, Darbari D, Wang W, Vichinsky E. Morbidity and mortality in chronically transfused subjects with thalassemia and sickle cell disease: A report from the multi-center study of iron overload. Am J Hematol. 2007 Apr; 82(4):255-65. PMID: 17094096.
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    126. Vichinsky E, Onyekwere O, Porter J, Swerdlow P, Eckman J, Lane P, Files B, Hassell K, Kelly P, Wilson F, Bernaudin F, Forni GL, Okpala I, Ressayre-Djaffer C, Alberti D, Holland J, Marks P, Fung E, Fischer R, Mueller BU, Coates T. A randomised comparison of deferasirox versus deferoxamine for the treatment of transfusional iron overload in sickle cell disease. Br J Haematol. 2007 Feb; 136(3):501-8. PMID: 17233848; PMCID: PMC1974786.
    127. Moskowitz JT, Butensky E, Harmatz P, Vichinsky E, Heyman MB, Acree M, Wrubel J, Wilson L, Folkman S. Caregiving time in sickle cell disease: psychological effects in maternal caregivers. Pediatr Blood Cancer. 2007 Jan; 48(1):64-71. PMID: 16622841.
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    128. Vichinsky E. Hemoglobin e syndromes. Hematology Am Soc Hematol Educ Program. 2007; 79-83. PMID: 18024613.
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    129. Neumayr LD, Aguilar C, Earles AN, Jergesen HE, Haberkern CM, Kammen BF, Nancarrow PA, Padua E, Milet M, Stulberg BN, Williams RA, Orringer EP, Graber N, Robertson SM, Vichinsky EP. Physical therapy alone compared with core decompression and physical therapy for femoral head osteonecrosis in sickle cell disease. Results of a multicenter study at a mean of three years after treatment. J Bone Joint Surg Am. 2006 Dec; 88(12):2573-82. PMID: 17142406.
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    130. Lal A, Fung EB, Pakbaz Z, Hackney-Stephens E, Vichinsky EP. Bone mineral density in children with sickle cell anemia. Pediatr Blood Cancer. 2006 Dec; 47(7):901-6. PMID: 16317761.
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    131. Ataga KI, Orringer EP, Styles L, Vichinsky EP, Swerdlow P, Davis GA, Desimone PA, Stocker JW. Dose-escalation study of ICA-17043 in patients with sickle cell disease. Pharmacotherapy. 2006 Nov; 26(11):1557-64. PMID: 17064199.
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    132. Fung EB, Harmatz PR, Lee PD, Milet M, Bellevue R, Jeng MR, Kalinyak KA, Hudes M, Bhatia S, Vichinsky EP. Increased prevalence of iron-overload associated endocrinopathy in thalassaemia versus sickle-cell disease. Br J Haematol. 2006 Nov; 135(4):574-82. PMID: 17054676.
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    133. Walter PB, Fung EB, Killilea DW, Jiang Q, Hudes M, Madden J, Porter J, Evans P, Vichinsky E, Harmatz P. Oxidative stress and inflammation in iron-overloaded patients with beta-thalassaemia or sickle cell disease. Br J Haematol. 2006 Oct; 135(2):254-63. PMID: 17010049; PMCID: PMC2185791.
    134. Hoppe C, Watson RM, Long CM, Lorey F, Robles L, Klitz W, Styles L, Vichinsky E. Prevalence of HFE mutations in California newborns. Pediatr Hematol Oncol. 2006 Sep; 23(6):507-16. PMID: 16849282.
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    135. Singer ST, Kuypers FA, Styles L, Vichinsky EP, Foote D, Rosenfeld H. Pulmonary hypertension in thalassemia: association with platelet activation and hypercoagulable state. Am J Hematol. 2006 Sep; 81(9):670-5. PMID: 16795058.
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    136. Treadwell MJ, McClough L, Vichinsky E. Using qualitative and quantitative strategies to evaluate knowledge and perceptions about sickle cell disease and sickle cell trait. J Natl Med Assoc. 2006 May; 98(5):704-10. PMID: 16749645; PMCID: PMC2569269.
    137. Vichinsky EP, MacKlin EA, Waye JS, Lorey F, Olivieri NF. Changes in the epidemiology of thalassemia in North America: a new minority disease. Pediatrics. 2005 Dec; 116(6):e818-25. PMID: 16291734.
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    138. Singer ST, Kuypers FA, Olivieri NF, Weatherall DJ, Mignacca R, Coates TD, Davies S, Sweeters N, Vichinsky EP. Fetal haemoglobin augmentation in E/beta(0) thalassaemia: clinical and haematological outcome. Br J Haematol. 2005 Nov; 131(3):378-88. PMID: 16225658.
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    139. Aguilar C, Vichinsky E, Neumayr L. Bone and joint disease in sickle cell disease. Hematol Oncol Clin North Am. 2005 Oct; 19(5):929-41, viii. PMID: 16214653.
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    140. Vichinsky E, Butensky E, Fung E, Hudes M, Theil E, Ferrell L, Williams R, Louie L, Lee PD, Harmatz P. Comparison of organ dysfunction in transfused patients with SCD or beta thalassemia. Am J Hematol. 2005 Sep; 80(1):70-4. PMID: 16138345.
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    141. Morris CR, Kato GJ, Poljakovic M, Wang X, Blackwelder WC, Sachdev V, Hazen SL, Vichinsky EP, Morris SM, Gladwin MT. Dysregulated arginine metabolism, hemolysis-associated pulmonary hypertension, and mortality in sickle cell disease. JAMA. 2005 Jul 06; 294(1):81-90. PMID: 15998894.
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    142. Aguilar CM, Neumayr LD, Eggleston BE, Earles AN, Robertson SM, Jergesen HE, Stulberg BN, Vichinsky EP. Clinical evaluation of avascular necrosis in patients with sickle cell disease: Children's Hospital Oakland Hip Evaluation Scale--a modification of the Harris Hip Score. Arch Phys Med Rehabil. 2005 Jul; 86(7):1369-75. PMID: 16003666.
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    143. Treadwell MJ, Law AW, Sung J, Hackney-Stephens E, Quirolo K, Murray E, Glendenning GA, Vichinsky E. Barriers to adherence of deferoxamine usage in sickle cell disease. Pediatr Blood Cancer. 2005 May; 44(5):500-7. PMID: 15602707.
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    144. Butensky E, Pakbaz Z, Foote D, Walters MC, Vichinsky EP, Harmatz P. Treatment of hepatitis C virus infection in thalassemia. Ann N Y Acad Sci. 2005; 1054:290-9. PMID: 16339677.
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    145. Pakbaz Z, Treadwell M, Yamashita R, Quirolo K, Foote D, Quill L, Singer T, Vichinsky EP. Quality of life in patients with thalassemia intermedia compared to thalassemia major. Ann N Y Acad Sci. 2005; 1054:457-61. PMID: 16339697.
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    146. Qureshi N, Foote D, Walters MC, Singer ST, Quirolo K, Vichinsky EP. Outcomes of preimplantation genetic diagnosis therapy in treatment of beta-thalassemia: A retrospective analysis. Ann N Y Acad Sci. 2005; 1054:500-3. PMID: 16339706.
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    147. Qureshi N, Avasarala K, Foote D, Vichinsky EP. Utility of Holter electrocardiogram in iron-overloaded hemoglobinopathies. Ann N Y Acad Sci. 2005; 1054:476-80. PMID: 16339701.
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    148. Morris CR, Kuypers FA, Kato GJ, Lavrisha L, Larkin S, Singer T, Vichinsky EP. Hemolysis-associated pulmonary hypertension in thalassemia. Ann N Y Acad Sci. 2005; 1054:481-5. PMID: 16339702.
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    149. Offer T, Bhagat A, Lal A, Atamna W, Singer ST, Vichinsky EP, Kuypers FA, Ames BN. Measuring chromosome breaks in patients with thalassemia. Ann N Y Acad Sci. 2005; 1054:439-44. PMID: 16339694.
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    150. Butensky E, Fischer R, Hudes M, Schumacher L, Williams R, Moyer TP, Vichinsky E, Harmatz P. Variability in hepatic iron concentration in percutaneous needle biopsy specimens from patients with transfusional hemosiderosis. Am J Clin Pathol. 2005 Jan; 123(1):146-52. PMID: 15762291.
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    151. Vichinsky EP. Changing patterns of thalassemia worldwide. Ann N Y Acad Sci. 2005; 1054:18-24. PMID: 16339647.
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    152. Premawardhena A, De Silver S, Arambepola M, Olivieri NF, Vichinsky EP, Merson L, Muraco G, Allen A, Fisher C, Peto T, Weatherall DJ. Hemoglobin E-beta-thalassemia: Progress report from the International Study Group. Ann N Y Acad Sci. 2005; 1054:33-9. PMID: 16339649.
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    153. Singer ST, Kuypers FA, Olivieri NF, Weatherall DJ, Mignacca R, Coates TD, Davies S, Sweeters N, Vichinsky EP. Single and combination drug therapy for fetal hemoglobin augmentation in hemoglobin E-beta 0-thalassemia: Considerations for treatment. Ann N Y Acad Sci. 2005; 1054:250-6. PMID: 16339672.
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    154. Pakbaz Z, Fischer R, Treadwell M, Yamashita R, Fung EB, Calvelli L, Quirolo K, Foote D, Harmatz P, Vichinsky EP. A simple model to assess and improve adherence to iron chelation therapy with deferoxamine in patients with thalassemia. Ann N Y Acad Sci. 2005; 1054:486-91. PMID: 16339703.
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    155. Jeng MR, Vichinsky E. Hematologic problems in immigrants from Southeast Asia. Hematol Oncol Clin North Am. 2004 Dec; 18(6):1405-22, x. PMID: 15511622.
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    156. Jones A, Granger S, Brambilla D, Gallagher D, Vichinsky E, Woods G, Berman B, Roach S, Nichols F, Adams RJ. Can peak systolic velocities be used for prediction of stroke in sickle cell anemia? Pediatr Radiol. 2005 Jan; 35(1):66-72. PMID: 15517239.
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    157. Lal A, Vichinsky E. The role of fetal hemoglobin-enhancing agents in thalassemia. Semin Hematol. 2004 Oct; 41(4 Suppl 6):17-22. PMID: 15534853.
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    158. Premawardhena A, De Silva S, Arambepola M, Olivieri N, Merson L, Muraco J, Allen A, Fisher C, Peto T, Vichinsky E, Weatherall D. Thalassemia in Sri Lanka: a progress report. Hum Mol Genet. 2004 Oct 01; 13 Spec No 2:R203-6. PMID: 15358726.
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    159. Vichinsky EP. Pulmonary hypertension in sickle cell disease. N Engl J Med. 2004 Feb 26; 350(9):857-9. PMID: 14985481.
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    160. Adams RJ, Brambilla DJ, Granger S, Gallagher D, Vichinsky E, Abboud MR, Pegelow CH, Woods G, Rohde EM, Nichols FT, Jones A, Luden JP, Bowman L, Hagner S, Morales KH, Roach ES. Stroke and conversion to high risk in children screened with transcranial Doppler ultrasound during the STOP study. Blood. 2004 May 15; 103(10):3689-94. PMID: 14751925.
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    161. Cohen AR, Galanello R, Pennell DJ, Cunningham MJ, Vichinsky E. Thalassemia. Hematology Am Soc Hematol Educ Program. 2004; 14-34. PMID: 15561674.
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    162. Claster S, Vichinsky EP. Managing sickle cell disease. BMJ. 2003 Nov 15; 327(7424):1151-5. PMID: 14615343; PMCID: PMC261819.
    163. Hoppe C, Klitz W, Cheng S, Apple R, Steiner L, Robles L, Girard T, Vichinsky E, Styles L. Gene interactions and stroke risk in children with sickle cell anemia. Blood. 2004 Mar 15; 103(6):2391-6. PMID: 14615367.
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    164. Adamkiewicz TV, Sarnaik S, Buchanan GR, Iyer RV, Miller ST, Pegelow CH, Rogers ZR, Vichinsky E, Elliott J, Facklam RR, O'Brien KL, Schwartz B, Van Beneden CA, Cannon MJ, Eckman JR, Keyserling H, Sullivan K, Wong WY, Wang WC. Invasive pneumococcal infections in children with sickle cell disease in the era of penicillin prophylaxis, antibiotic resistance, and 23-valent pneumococcal polysaccharide vaccination. J Pediatr. 2003 Oct; 143(4):438-44. PMID: 14571216.
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    165. Morris CR, Vichinsky EP, van Warmerdam J, Machado L, Kepka-Lenhart D, Morris SM, Kuypers FA. Hydroxyurea and arginine therapy: impact on nitric oxide production in sickle cell disease. J Pediatr Hematol Oncol. 2003 Aug; 25(8):629-34. PMID: 12902916.
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    166. Neumayr L, Lennette E, Kelly D, Earles A, Embury S, Groncy P, Grossi M, Grover R, McMahon L, Swerdlow P, Waldron P, Vichinsky E. Mycoplasma disease and acute chest syndrome in sickle cell disease. Pediatrics. 2003 Jul; 112(1 Pt 1):87-95. PMID: 12837872.
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    167. Brittenham GM, Nathan DG, Olivieri NF, Porter JB, Pippard M, Vichinsky EP, Weatherall DJ. Deferiprone and hepatic fibrosis. Blood. 2003 Jun 15; 101(12):5089-90; author reply 5090-1. PMID: 12788794.
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    168. Steinberg MH, Barton F, Castro O, Pegelow CH, Ballas SK, Kutlar A, Orringer E, Bellevue R, Olivieri N, Eckman J, Varma M, Ramirez G, Adler B, Smith W, Carlos T, Ataga K, DeCastro L, Bigelow C, Saunthararajah Y, Telfer M, Vichinsky E, Claster S, Shurin S, Bridges K, Waclawiw M, Bonds D, Terrin M. Effect of hydroxyurea on mortality and morbidity in adult sickle cell anemia: risks and benefits up to 9 years of treatment. JAMA. 2003 Apr 02; 289(13):1645-51. PMID: 12672732.
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    169. Hagar W, Vichinsky EP, Theil EC. Liver ferritin subunit ratios in neonatal hemochromatosis. Pediatr Hematol Oncol. 2003 Apr-May; 20(3):229-35. PMID: 12637219.
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    170. Morris CR, Morris SM, Hagar W, Van Warmerdam J, Claster S, Kepka-Lenhart D, Machado L, Kuypers FA, Vichinsky EP. Arginine therapy: a new treatment for pulmonary hypertension in sickle cell disease? Am J Respir Crit Care Med. 2003 Jul 01; 168(1):63-9. PMID: 12626350.
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    171. Dean D, Neumayr L, Kelly DM, Ballas SK, Kleman K, Robertson S, Iyer RV, Ware RE, Koshy M, Rackoff WR, Pegelow CH, Waldron P, Benjamin L, Vichinsky E. Chlamydia pneumoniae and acute chest syndrome in patients with sickle cell disease. J Pediatr Hematol Oncol. 2003 Jan; 25(1):46-55. PMID: 12544773.
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    172. Hoppe C, Klitz W, Noble J, Vigil L, Vichinsky E, Styles L. Distinct HLA associations by stroke subtype in children with sickle cell anemia. Blood. 2003 Apr 01; 101(7):2865-9. PMID: 12517810.
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    173. Lo L, Singer ST, Vichinsky E. Pancytopenia induced by hypothermia. J Pediatr Hematol Oncol. 2002 Nov; 24(8):681-4. PMID: 12439045.
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    174. Hagar W, Theil EC, Vichinsky EP. Diseases of iron metabolism. Pediatr Clin North Am. 2002 Oct; 49(5):893-909. PMID: 12430618.
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    175. Vichinsky E. New therapies in sickle cell disease. Lancet. 2002 Aug 24; 360(9333):629-31. PMID: 12241949.
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    176. VanderJagt DJ, Harmatz P, Scott-Emuakpor AB, Vichinsky E, Glew RH. Bioelectrical impedance analysis of the body composition of children and adolescents with sickle cell disease. J Pediatr. 2002 Jun; 140(6):681-7. PMID: 12072870.
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    177. Files B, Brambilla D, Kutlar A, Miller S, Vichinsky E, Wang W, Granger S, Adams RJ. Longitudinal changes in ferritin during chronic transfusion: a report from the Stroke Prevention Trial in Sickle Cell Anemia (STOP). J Pediatr Hematol Oncol. 2002 May; 24(4):284-90. PMID: 11972097.
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    178. Pegelow CH, Macklin EA, Moser FG, Wang WC, Bello JA, Miller ST, Vichinsky EP, DeBaun MR, Guarini L, Zimmerman RA, Younkin DP, Gallagher DM, Kinney TR. Longitudinal changes in brain magnetic resonance imaging findings in children with sickle cell disease. Blood. 2002 Apr 15; 99(8):3014-8. PMID: 11929794.
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    179. Jeng MR, Feusner J, Skibola C, Vichinsky E. Central venous catheter complications in sickle cell disease. Am J Hematol. 2002 Feb; 69(2):103-8. PMID: 11835345.
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    180. Franck LS, Treadwell M, Jacob E, Vichinsky E. Assessment of sickle cell pain in children and young adults using the adolescent pediatric pain tool. J Pain Symptom Manage. 2002 Feb; 23(2):114-20. PMID: 11844631.
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    181. Wang WC, Helms RW, Lynn HS, Redding-Lallinger R, Gee BE, Ohene-Frempong K, Smith-Whitley K, Waclawiw MA, Vichinsky EP, Styles LA, Ware RE, Kinney TR. Effect of hydroxyurea on growth in children with sickle cell anemia: results of the HUG-KIDS Study. J Pediatr. 2002 Feb; 140(2):225-9. PMID: 11865275.
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    182. Treadwell MJ, Franck LS, Vichinsky E. Using quality improvement strategies to enhance pediatric pain assessment. Int J Qual Health Care. 2002 Feb; 14(1):39-47. PMID: 11871628.
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    183. Walters MC, Nienhuis AW, Vichinsky E. Novel therapeutic approaches in sickle cell disease. Hematology Am Soc Hematol Educ Program. 2002; 10-34. PMID: 12446417.
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    184. Gaylor JL, Delwiche CV, Brady DR, Green AJ. Preparation and properties of a cell-free system from rat skin that catalyzes sterol biosynthesis. J Lipid Res. 1966 Jul; 7(4):501-10. PMID: 4290536.
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    185. Taher A, Vichinsky E, Musallam K, Cappellini MD, Viprakasit V.Guidelines for the Management of Non Tranfusion Dependent Thalassemia (NTDT). Thalassemia International Federation, 2013.
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