50th ASH Annual Meeting and Exposition (December 6 - 9, 2008): Hemoglobinopathies, Excluding Thalassemia Poster II

Sunday, December 7, 2008: 6:00 PM-8:00 PM

Hall A (Moscone Center)

Assessing Endothelial Dysfunction in Pediatric Sickle Cell Disease

Krupa Sivamurthy, MD^*, Carlton Dampier, MD^*, MaryLou MacDermott, RN, CRNP^*, Maureen Meier, RN, CCRC^*, Michele Cahill, RN^* and Lewis L. Hsu, MD, PhD

Drexel University College of Medicine, Marian Anderson Comprehensive Sickle Cell Center, Nelson 2130, Philadelphia, PA

2471

Fetal Hemoglobin in Sickle Cell Anemia: A Genome-Wide Association Study of the Response to Hydroxyurea

Nadia Timofeev, MS¹^*, Paola Sebastiani, PhD¹^*, Steven H. Hartley, MS¹^*, Clinton T. Baldwin, PhD²^* and Martin H. Steinberg, MD³

¹Biostatistics, Boston University School of Public Health, Boston, MA
²Center for Human Genetics, Boston, MA
³Medicine, Boston University School of Medicine, Boston, MA

2472

Platelets from Sickle Cell Disease Individuals Demonstrate Increased Adhesive Properties That Are Reversed by Hydroxyurea Therapy in Association with Alterations in Intraplatelet cAMP and α_IIbβ₃ Integrin Activation

Renata P. Ferreira, B.Sc.^*, Carla F. Franco-Penteado, Ph.D.^*, Sara T.O. Saad, M.D., Ph.D., Fernando F. Costa, M.D., Ph.D. and Nicola Conran, Ph.D.

Hematology and Hemotherapy Center, University of Campinas, Campinas, SP, Brazil

2473

Mechanical Hypersensitivity Occurs in a Mouse Model of Sickle Cell Disease

Nancy J Wandersee, Teresa Uy^*, Dawn Retherford^*, Aziz Noon-Abdullah^*, Anne C Frei^*, Amanda M Brandow^*, Cheryl M Stucky^* and Cheryl A Hillery^*

Medical College of Wisconsin, Children's Research Institute, and Blood Research Institute, Milwaukee, WI

2474

Structural and Functional Interactions of KCl Cotransport Proteins KCC1 and KCC3 in Sickle and Normal Erythrocyte Membranes

Mary Risinger, PhD¹^*, Jesse Rinehart, PhD²^*, Scott Crable, MS¹^*, Anna Ottlinger¹^*, Richard Winkelmann, BA¹^*, Dao Pan, PhD³^*, Christian Huebner, MD⁴^*, Patrick G. Gallagher, MD⁵ and Clinton H. Joiner, MD, PhD¹

¹Hematology/Oncology, Cincinnati Comprehensive Sickle Cell Center, Cincinnati Children's Hospital Medical Center, Cincinnati, OH
²Genetics, Yale University, New Haven, CT
³Experimental Hematology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH
⁴Clinical Chemistry, Friedrich-Schiller-Universität Jena, Jena, Germany
⁵School of Medicine, Yale University, New Haven, CT

2475

Induction of Caspase-Independent Apoptosis by Sickle Cell Disease (SCD) Serum in Non-SCD Leukocytes

Camila B. Almeida, M.Sc.^*, Fernanda Gonçalves Pereira, M.Sc.^*, Irene Lorand Metze, MD, Ph.D.^*, Sara T.O. Saad, MD, PhD, Fernando F. Costa, PhD, MD and Nicola Conran, Ph.D.

Hematology and Hemotherapy Center, University of Campinas, Campinas, SP, Brazil

2476

Prolonged QTc in Sickle Cell Disease: A Potential Risk Factor for Early Death?

Sean Lindstedt¹^*, Lynne Neumayr, M.D.²^*, Gregory Kurio, M.D.³^*, Claudia Morris, M.D.⁴^*, Shanda Robertson²^*, Ellen Fung, PhD⁵^*, Paul Harmatz, M.D.⁶, John C Wood, MD, PhD⁷^* and Elliott P. Vichinsky, M.D.²

¹School of Medicine, University of Nevada, Reno, NV
²Hematology/Oncology Dept., Children's Hospital & Research Center Oakland, Oakland, CA
³Cardiology Dept., Children's Hospital & Research Center Oakland, Oakland, CA
⁴Emergency Dept., Children's Hospital & Research Center Oakland, Oakland, CA
⁵Center for Nutrition and Metabolism, Children's Hospital & Research Center Oakland, Oakland, CA
⁶Gastroenterology Dept., Children's Hospital & Research Center Oakland, Oakland, CA
⁷Cardiology Division, Children's Hospital Los Angeles, Los Angeles, CA

2477

Hydroxyurea Use Is Associated with Avascular Necrosis of the Femoral Head among Children with Sickle Cell Disease

Kris Michael Mahadeo, M.D.¹^*, Suzette Oyeku, M.D.²^*, Karen Moody, M.D.¹^*, Swapmil N. Rajpathak, M.D., Dr., Ph³^*, Abraham Groner, M.D.²^*, Ruth Santizo, B.S.¹^* and M. Catherine Driscoll, MD¹

¹Division of Pediatric Hematology-Oncology, Albert Einstein College of Medicine, Bronx, NY
²Division of General Pediatrics, Albert Einstein College of Medicine, Bronx, NY
³Department of Epidemiology and Population Health, Albert Einstein College of Medicine, Bronx, NY

2478

Erythrocyte Adhesion and Phosphatidylserine Exposure in HbSC Disease: Baseline Data from the CHAMPS Study

Marilyn J. Telen, MD¹, Martha Delahunty, PhD¹^*, Cathie Snyder²^* and Winfred C. Wang, MD³^*

¹Medicine, Duke University Medical Center, Durham, NC
²Rho Statistics and Data Management Center, Rho, Inc., Chapel Hill, NC
³St. Jude Children's Research Hospital, Memphis, TN

2479

Significance of, and Difficulty in Diagnosing, Erythropoietin Deficiency in Sickle Cell Anemia

Santosh L Saraf, MD^*, Seema Sidhwani, DO^*, Mohammed Farooqui, DO^*, Giovanni Infusino, MD^*, Lani Krauz, RN^*, Michael Gowhari, MD^*, Stephen Vara, MD^*, Louise Dorn, RN, MS^*, Joseph Desimone, PhD^*, Robert Molokie, MD^* and Yogen Saunthararajah, MD^*

Hematology - Sickle Cell Department, University of Illinois, Chicago, IL

2480

Molecular Phenotype of Sickle Chronic Lung Disease Revealed by Global Gene Expression Profiling

Fang Tan^*, Tianwei Yu^*, Yuhua Li^*, Samit Ghosh^*, Mario Mosunjac^* and Solomon F Ofori-Acquah

Emory University, Atlanta, GA

2481

Asthma in Pediatric Sickle Cell Acute Chest Syndrome: In An Inner City London Hospital

Jack L Bartram, MBChB, MRCPCH¹^*, Miriam R Fine-Goulden, MA, MBBS, MRCPCH¹^*, Dido Green¹^*, Rahail Ahmad, MBBS¹^* and Baba PD Inusa, MBBS, FMCPaed, FRCPCH, MRCP, DCP²

¹Department of Paediatrics, Evelina Children's Hospital, St Thomas' Hospital NHS Trust, London, United Kingdom
²Paediatrics and Haemoglobinopathies, Evelina Children's Hospital, St Thomas' Hospital NHS Trust, London, United Kingdom

2482

Association of Hemolysis with Clinical Manifestations of Sickle Cell Disease

Mehdi Nouraie¹^*, Caterina Minniti²^*, Craig Sable³^*, Andrew D. Campbell, MD⁴, Sohail R Rana, MD⁵^*, Gregory Ensing⁶^*, Niti Dham, MD⁷^*, Onyinye C. Onyekwere, MD, MS⁸, Deepika S. Darbari⁹^*, Gregory J. Kato, MD¹⁰, Mark Gladwin, MD², Oswaldo Castro, MD¹^* and Victor R. Gordeuk, MD¹¹

¹Center for Sickle cell Disease, Howard University, Washington, DC
²Vascular Medicine Branch, NHLBI, Bethesda, MD
³Children's National Medical Center, Washington, DC
⁴Pediatric Hematology/Oncology, Univ. of Michigan Med. Ctr., Ann Arbor, MI
⁵Department of Pediatric and Child Health, Howard University Hospital, Washington DC, DC
⁶Pediatric Cardiology, University of Michigan, Ann Arbor, MI
⁷Cardiology, Children's National Medical Center, Washington, DC
⁸Howard University Hospital, Washington, DC
⁹Pediatric Hematology/Oncology, Johns Hopkins University School of Medicine, Baltimore, MD
¹⁰Pulmonary and Vascular Medicine Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD
¹¹Howard University, Washington, DC

2483

Acute Lung Inflammation in Sickle Mice Is Mediated by Increase of CXC Chemokines and Matrix Metalloproteinases

Carla Fernanda Franco-Penteado, B.Sc., M.Sc., Ph.D¹^*, Carolina Lanaro, B.Sc., Ph.D¹^*, Dulcinéia M Albuquerque, B.Sc., M.Sc., Ph.D¹^*, Ana Paula Gimenes, B.Sc²^*, Luiz Augusto C Passos, B.Sc., M.Sc., Ph.D²^*, Marcus A.F. Corat, B.Sc., M.Sc., Ph.D²^* and Fernando F. Costa, PhD, MD¹

¹Hematology and Hemotherapy Center, State University of Campinas, Campinas, Brazil
²Multidisciplinary Center for the Investigation of Biological Science in Laboratory Animals, State University of Campinas, Campinas, Brazil

2484

Correlations Between Cytokines and Elevated Tricuspid Regurgitant Jet Velocity in Children and Adolescents with Sickle Cell Disease

Xiaomei Niu, MD¹^*, Mehdi Nouraie, MD, PhD²^*, Caterina Minniti, MD³^*, Craig Sable, MD⁴^*, Andrew Campbell, MD⁵^*, Sohail R Rana, MD⁶^*, Gregory J. Kato, MD⁷, Mark Gladwin, MD³, Oswaldo Castro, MD²^*, Tatiana Ammosova, PhD⁸^*, Sergei Nekhai, PhD⁹^* and Victor R. Gordeuk, MD¹⁰

¹Internal Medicine, Howard Univerity, Washington DC, DC
²Center for Sickle cell Disease, Howard University, Washington, DC
³Vascular Medicine Branch, NHLBI, Bethesda, MD
⁴Children's National Medical Center, Washington, DC
⁵Department of Pediatrics and Communicable Diseases, University of Michigan Medical Center, Ann Arbor, MI
⁶Department of Pediatric and Child Health, Howard University Hospital, Washington DC, DC
⁷Pulmonary and Vascular Medicine Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD
⁸Center for Sickle Cell Disease, HU, Howard University, Washington, DC
⁹Center for Sickle Cell Disease, Howard University, Washington, DC
¹⁰Howard University, Washington, DC

2486

Nitric Oxide-Dependent Blood Flow in Patients with Sickle Cell Disease Is Reflected by the Reactive Hyperemia-Peripheral Arterial Tonometry Index

Megan L. Krajewski, BS¹^*, James A. Kim, BM¹^*, Laurel G. Mendelsohn, BS¹^*, Candice B. Williams, MD¹^*, Mark T. Gladwin, MD²^* and Gregory J. Kato, MD³

¹Pulmonary and Vascular Medicine Branch, National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD
²Division of Pulmonary, Allergy, and Critical Care Medicine, University of Pittsburgh Medical Center, Pittsburgh, PA
³Critical Care Medicine Department, Clinical Center, National Institutes of Health, Bethesda, MD

2487

Risk Factors for Silent Cerebral Infarcts in a Pediatric Sickle Cell Anemia (SCA) Cohort

Francoise Bernaudin¹, Suzanne Verlhac²^*, Annie Kamdem¹^*, Cécile Arnaud¹^*, Lena Coïc¹^*, Emmanuelle Lesprit¹^*, Isabelle Hau¹^*, Philippe Reinert¹^* and Christophe Delacourt¹^*

¹Pediatrics, Reference Center for Sickle Cell Disease, Intercommunal Hospital, Creteil, France
²Medical Imagery, Reference Center for Sickle Cell Disease, Intercommunal Hospital, Creteil, France

2488

Exercise-Induced Pulmonary Hypertension in Sickle Cell Anemia: a Study with Exercise Stress Echocardiography

Newton Nunes, MD¹^*, Orlando Campos, MD, PhD¹^*, Maria Stella Figueiredo, MD, PhD²^*, Rodolfo Delfini Cancado, MD, PhD³^*, Perla Vicari, MD, PhD²^*, Antonio C C Carvalho, MD, PhD¹^* and Jose Orlando Bordin, MD⁴

¹Cardiology Division, Federal University of Sao Paulo, Sao Paulo, SP, Brazil
²Hematology and Transfusion Division, Federal University of Sao Paulo, Sao Paulo SP, Brazil
³Hematology/Oncology, Santa Casa School of Medicine, Sao Paulo, Brazil
⁴Disciplina de Hematologia e Hemoterapia, Universidade Federal de São Paulo, Sao Paulo, Brazil

2489

Simvastatin Reduces the in Vitro Adhesion of Sickle Cell Disease Neutrophils to Endothelial Layers

Andreia A Canalli, B.Sc., Ph.D.^*, Renata P. Ferreira, B.Sc.^*, Sara T.O. Saad, MD, PhD, Nicola Conran, B.Sc, PhD and Fernando F. Costa, M.D., Ph.D.^*

Hematology and Hemotherapy Center, University of Campinas, Campinas, Brazil

2490

Production and Expression of Inflammatory Mediators in Leukocytes of Sickle Cell Anaemia Patients and Effects of Hydroxyurea Therapy on This Production

Carolina Lanaro, B.Sc., Ph.D^*, Carla Fernanda Franco-Penteado, B.Sc., M.Sc., Ph.D^*, Dulcinéia M Albuquerque, B.Sc., M.Sc., Ph.D^*, Sara T.O. Saad, MD, PhD, Nicola Conran, B.Sc., Ph.D and Fernando F. Costa, PhD, MD

Hematology and Hemotherapy Center, State University of Campinas, Campinas, Brazil

2491

Fetal Hemoglobin in Sickle Cell Anemia: A Novel Method for High-Resolution Discovery of Associated Genomic Copy Number Variations

Daniel Dworkis, BS¹^*, Paola Sebastiani, PhD²^*, Efthymia Melista, BS³^*, Jason Parente, BS³^*, Griffin Lester⁴^*, Jeffrey F Quinn³^*, David H.K. Chui, MD⁵, Martin H. Steinberg, MD⁶ and Clinton T. Baldwin, PhD³^*

¹Boston University School of Medicine, Boston, MA
²Biostatistics, Boston University School of Public Health, Boston, MA
³Center for Human Genetics, Boston, MA
⁴Center for Human Genetic, Boston, MA
⁵Boston Univ. School of Med., Boston, MA
⁶Department of Medicine/Division of Hematology and Oncology, Boston University School of Medicine, Boston, MA

2492

Elevated Tricuspid Regurgitant Jet Velocity in Children and Adolescents with Sickle Cell Disease: Association with Hemolysis and Hemoglobin Oxygen Desaturation

Caterina P. Minniti, MD

Pulmonary and Vascular Medicine Branch, National Institutes of Health, Bethesda, MD

2493

Role of Hypoxia Induced Factor (HIF-1 alpha) in Pulmonary Hypertension in Sickle Cell Disease

Mukta Kumar, MD, MPH, Xuhui Zhu^* and Shilpa Buch, PhD^*

University of Kansas, Kansas City, KS

2494

Red Blood Cell Scramblase Activation Is Induced by Phorbol Ester and Prevented by Inhibition of Protein Kinase C

Latorya Arnold Barber, B.S.¹^*, Mary B Palascak, B.S¹^*, Clinton H. Joiner, MD, PhD² and Robert S. Franco, PhD³

¹Internal Medicine, University of Cincinnati, Cincinnati, OH
²Children's Hosp. Med. Ctr., Cincinnati, OH
³College of Medicine, University of Cincinnati, Cincinnati, OH

2495

Up-Regulation of NO/cGMP Signaling Pathway in Corpus Cavernosum of Sickle Cell Disease Transgenic Mice

Mário Angelo Claudino, B.Sc., Ph.D¹^*, Carla Fernanda Franco-Penteado, B.Sc., M.Sc., Ph.D²^*, Marcus A.F. Corat, B.Sc., M.Sc., Ph.D³^*, Luiz Augusto C Passos, B.Sc., M.Sc., Ph.D³^*, Ana Paula Gimenes, B.Sc³^*, Edson Antunes, B.Sc., M.Sc., Ph.D¹^* and Fernando F. Costa, PhD, MD²

¹Department of Pharmacology, State University of Campinas, Campinas, Brazil
²Hematology and Hemotherapy Center, State University of Campinas, Campinas, Brazil
³Multidisciplinary Center for the Investigation of Biological Science in Laboratory Animals, State University of Campinas, Campinas, Brazil

2496

Peripheral Arterial Tonometry Assessment of Endothelial Dysfunction in Sickle Cell Patients (For the 6R-BH4 in Sickle Cell Disease Study Group)

Lewis L. Hsu, MD, PhD¹, Kenneth I. Ataga, MD², Olise M. Nwose, MBBS, MRCPath³^* and Emil Kakkis, MD, PhD³^*

¹Drexel University College of Medicine, Marian Anderson Comprehensive Sickle Cell Center, Nelson 2130, Philadelphia, PA
²Univ. of North Carolina at Chapel Hill, Chapel Hill, NC
³BioMarin Pharmaceutical, Novato, CA

2497

Cerebral Vasculopathy Outcome in a Sickle Cell Anemia (SCA) Newborn Cohort Screened Early with Transcranial Doppler

Francoise Bernaudin¹, Suzanne Verlhac²^*, Cécile Arnaud¹^*, Annie Kamdem¹^*, Lena Coïc¹^*, Emmanuelle Lesprit¹^*, Isabelle Hau¹^*, Philippe Reinert¹^* and Christophe Delacourt¹^*

¹Pediatrics, Reference Center for Sickle Cell Disease, Intercommunal Hospital, Creteil, France
²Medical Imagery, Reference Center for Sickle Cell Disease, Intercommunal Hospital, Creteil, France

2498

Obstetric and Gynecological History in Sickle Cell Disease Females

Soheir S. Adam, M.D.¹, Jude C Jonassaint, RN²^*, Mary R. Abrams²^*, Charles R. Jonassaint, MA²^*, Marilyn J. Telen, MD³ and Laura M. De Castro, MD⁴

¹Medicine/ Hematology, UNC, Chapel Hill, NC
²Comprehensive Sickle Cell Center, Duke University, Durham, NC
³Medicine, Duke University Medical Center, Durham, NC
⁴Comprehensive Sickle Cell Center, Clin. Director Duke Adult, Durham, NC

2499

Sickle Cell Pulmonary Hypertension and Dysregulated NO Axis in a Mouse Model Are Modulated by Apolipoprotein a-1 Availability

Lewis L. Hsu, MD, PhD¹^*, Hazim El-Haddad, MD²^*, Marcelo Amar, MD³^*, Gregory J. Kato, MD⁴, Alan T Remaley, MD, PhD³^* and Hunter C. Champion, MD, PhD²^*

¹Drexel University College of Medicine, Marian Anderson Comprehensive Sickle Cell Center, Nelson 2130, Philadelphia, PA
²Division of Cardiology, Department of Medicine, Johns Hopkins Medical Institutions, Baltimore, MD
³Lab Medicine, National Institutes of Health, Bethesda, MD
⁴Pulmonary and Vascular Medicine Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD

2500

Association of Placenta Growth Factor with Hemolysis and Inflammation in Sickle Cell Disease (SCD)

Kenneth I. Ataga, MD¹, Susan K Jones, RN²^*, Ben Hulkower, BS³^*, Eugene P. Orringer, MD⁴ and Julia E. Brittain, PhD⁵^*

¹Univ. of North Carolina at Chapel Hill, Chapel Hill, NC
²Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC
³Department of Medicine, University of North Carolina, Chapel Hill, NC
⁴UNC at Chapel Hill, Chapel Hill, NC
⁵Departments of Biochemistry & Biophysics, Obstetrics & Gynecology, University of North Carolina at Chapel Hill, Chapel Hill, NC

2501

Hemolytic Rate and Nitric Oxide Bioavailability Modulate the Expression of Hypoxia-Inducible Factor-1α (HIF-1α) in Transgenic Sickle Mice

Dhananjay K. Kaul, PhD, Trisha Dasgupta, PhD^*, Xiaoqin Zhang, MS^* and Mary Fabry, PhD

Albert Einstein College of Medicine, Bronx, NY

2502

Clinical and Laboratory Correlates of Albuminuria in Sickle Cell Disease

Kenneth I. Ataga, MD¹, Julia E. Brittain, PhD²^*, Susan K Jones, RN, BSN³^*, Ben Hulkower, BS³^*, Soheir S. Adam, M.D.⁴, Riten Mitra, MS⁵^*, Rupa Redding-Lallinger, MD⁶, Patrick Nachman, MD³^* and Eugene P. Orringer, MD⁷

¹Univ. of North Carolina at Chapel Hill, Chapel Hill, NC
²Departments of Biochemistry & Biophysics, Obstetrics & Gynecology, University of North Carolina at Chapel Hill, Chapel Hill, NC
³Medicine, University of North Carolina, Chapel Hill, NC
⁴Medicine/ Hematology, UNC, Chapel Hill, NC
⁵Lineberger Comprehensive Cancer Center, University of North Carolina, Chapel Hill, NC
⁶Univ. of NC at Chapel Hill, Chapel Hill, NC
⁷UNC at Chapel Hill, Chapel Hill, NC

2503

Changes of BNP Level and Pulmonary Arterial Pressure during An Acute Sickle Cell Crisis, Comparison with Steady State

Aref Agheli, MD, Chenthil Rathnasabapathy, MD^*, Ashish Sangal, MD^*, Zili He, MD^*, William Steier, MD^*, Shahrokh Rafii, MD^* and Madhumati Kalavar, MD^*

Divisions of Hematology & Medical oncology, and Cardiology, Brookdale University Hospital and Medical Center, Brooklyn, NY

2504

Expression Levels of CD11b, CD18, CD32, CD62L (L-Selectin) and CD62P (P-Selectin) and Its Role in Sickle Cell Anemia Inflammatory State

Bruno Antônio Veloso Cerqueira Jr.¹^*, Wendell Vilas Boas Jr.²^*, Magda Oliveira Seixas Jr.²^*, Elder Trindade Damasceno Jr.²^*, Cyntia Cajado Souza Jr.²^*, Mitermayer Galvão Reis Sr.²^* and Marilda Souza Goncalves, Dr³^*

¹Universidade de Santa Cruz, Ilhéus, Brazil
²Centro de Pesquisas Gonçalo Moniz - FIOCRUZ, Salvador, Brazil
³Molecular Biology, Centro de Pesquisa Gonçalo Muniz-Fiocruz, Salvador, Brazil

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