Ellis Neufeld

Summary

Affiliation: Harvard University
Country: USA

Publications

  1. ncbi Complications of beta-thalassemia major in North America
    Melody J Cunningham
    Children s Hospital, Dana Farber Cancer Institute and Harvard Medical School, Boston, MA 02115, USA
    Blood 104:34-9. 2004
  2. pmc Beliefs about chelation among thalassemia patients
    Felicia L Trachtenberg
    New England Research Institutes, 9 Galen Street, Watertown, MA, 02472, USA
    Health Qual Life Outcomes 10:148. 2012
  3. doi Dosing, efficacy, and safety of recombinant factor VIIa (rFVIIa) in pediatric versus adult patients: the experience of the Hemostasis and Thrombosis Research Society (HTRS) Registry (2004-2008)
    Ellis J Neufeld
    Boston Children s Hospital, Boston, Massachusetts 02115, USA
    Pediatr Blood Cancer 60:1178-83. 2013
  4. doi Effect of acute bleeding on daily quality of life assessments in patients with congenital hemophilia with inhibitors and their families: observations from the dosing observational study in hemophilia
    Ellis J Neufeld
    Children s Hospital Boston, Boston, MA 02115, USA
    Value Health 15:916-25. 2012
  5. doi Exposure and safety of higher doses of recombinant factor VIIa ≥250 μg kg(-1) in individuals with congenital haemophilia complicated by alloantibody inhibitors: the Haemophilia and Thrombosis Research Society Registry experience (2004-2008)
    E J Neufeld
    Children s Hospital Boston, Boston, MA, USA
    Haemophilia 17:650-6. 2011
  6. pmc Oral chelators deferasirox and deferiprone for transfusional iron overload in thalassemia major: new data, new questions
    Ellis J Neufeld
    Division of Hematology, Children s Hospital, Karp 08210, 300 Longwood Ave, Boston, MA 02115, USA
    Blood 107:3436-41. 2006
  7. doi Quality of life in thalassemia: a comparison of SF-36 results from the thalassemia longitudinal cohort to reported literature and the US norms
    A Sobota
    Children s Hospital Boston, Massachusetts, USA
    Am J Hematol 86:92-5. 2011
  8. doi Oral chelation: should it be used with young children?
    Lauren M Mednick
    Department of Psychiatry, Children s Hospital Boston, Harvard Medical School, Boston, Massachusetts 02115, USA
    Pediatr Blood Cancer 55:603-5. 2010
  9. pmc Deletion of SLC19A2, the high affinity thiamine transporter, causes selective inner hair cell loss and an auditory neuropathy phenotype
    M C Liberman
    Eaton Peabody Laboratory, Massachusetts Eye and Ear Infirmary, 243 Charles Street, Boston, MA 02114, USA
    J Assoc Res Otolaryngol 7:211-7. 2006
  10. ncbi Passive cigarette smoking and reduced HDL cholesterol levels in children with high-risk lipid profiles
    E J Neufeld
    Children s Hospital, and the Department of Pediatrics, Harvard Medical School, Boston, Mass 02115, USA
    Circulation 96:1403-7. 1997

Research Grants

Collaborators

Detail Information

Publications34

  1. ncbi Complications of beta-thalassemia major in North America
    Melody J Cunningham
    Children s Hospital, Dana Farber Cancer Institute and Harvard Medical School, Boston, MA 02115, USA
    Blood 104:34-9. 2004
    ..We conclude that hepatitis C, iron-related organ dysfunction, and complications of iron chelation therapy are strongly age-dependent in North American patients with beta-thalassemia...
  2. pmc Beliefs about chelation among thalassemia patients
    Felicia L Trachtenberg
    New England Research Institutes, 9 Galen Street, Watertown, MA, 02472, USA
    Health Qual Life Outcomes 10:148. 2012
    ..Understanding patients' views about medication is crucial to maximize adherence. Thalassemia is a congenital blood disorder requiring chronic blood transfusions and daily iron chelation therapy...
  3. doi Dosing, efficacy, and safety of recombinant factor VIIa (rFVIIa) in pediatric versus adult patients: the experience of the Hemostasis and Thrombosis Research Society (HTRS) Registry (2004-2008)
    Ellis J Neufeld
    Boston Children s Hospital, Boston, Massachusetts 02115, USA
    Pediatr Blood Cancer 60:1178-83. 2013
    ..The longitudinal registry of the Hemostasis and Thrombosis Research Society (HTRS) provided data on treatment of bleeding episodes for pediatric and adult patients with congenital hemophilia complicated by inhibitors to factor VIII or IX...
  4. doi Effect of acute bleeding on daily quality of life assessments in patients with congenital hemophilia with inhibitors and their families: observations from the dosing observational study in hemophilia
    Ellis J Neufeld
    Children s Hospital Boston, Boston, MA 02115, USA
    Value Health 15:916-25. 2012
    ..Periodic QOL assessments typically made on nonbleed days may not provide complete reflections of the burden on patients/families...
  5. doi Exposure and safety of higher doses of recombinant factor VIIa ≥250 μg kg(-1) in individuals with congenital haemophilia complicated by alloantibody inhibitors: the Haemophilia and Thrombosis Research Society Registry experience (2004-2008)
    E J Neufeld
    Children s Hospital Boston, Boston, MA, USA
    Haemophilia 17:650-6. 2011
    ..No serious adverse drug-related events or thrombotic complications were reported. This data analysis from the HTRS Registry provides evidence of the safe and effective use of higher doses of rFVIIa (≥250 μg kg(-1) ) in US practice...
  6. pmc Oral chelators deferasirox and deferiprone for transfusional iron overload in thalassemia major: new data, new questions
    Ellis J Neufeld
    Division of Hematology, Children s Hospital, Karp 08210, 300 Longwood Ave, Boston, MA 02115, USA
    Blood 107:3436-41. 2006
    ..These developments herald a new era for iron chelation, but many unanswered questions remain...
  7. doi Quality of life in thalassemia: a comparison of SF-36 results from the thalassemia longitudinal cohort to reported literature and the US norms
    A Sobota
    Children s Hospital Boston, Massachusetts, USA
    Am J Hematol 86:92-5. 2011
    ..The SF-36 should become a standard instrument for assessing HRQOL in thalassemia to determine predictors of low HRQOL which may be better addressed by a multidisciplinary team...
  8. doi Oral chelation: should it be used with young children?
    Lauren M Mednick
    Department of Psychiatry, Children s Hospital Boston, Harvard Medical School, Boston, Massachusetts 02115, USA
    Pediatr Blood Cancer 55:603-5. 2010
    ..We review developmental challenges associated with oral chelation in this age group and provide suggestions to improve adherence in this population...
  9. pmc Deletion of SLC19A2, the high affinity thiamine transporter, causes selective inner hair cell loss and an auditory neuropathy phenotype
    M C Liberman
    Eaton Peabody Laboratory, Massachusetts Eye and Ear Infirmary, 243 Charles Street, Boston, MA 02114, USA
    J Assoc Res Otolaryngol 7:211-7. 2006
    ..Such a pattern is consistent with the observed discrepancy between ABR and OAE threshold shifts. The possible role of thiamine transport in other reported cases of selective inner hair cell loss is considered...
  10. ncbi Passive cigarette smoking and reduced HDL cholesterol levels in children with high-risk lipid profiles
    E J Neufeld
    Children s Hospital, and the Department of Pediatrics, Harvard Medical School, Boston, Mass 02115, USA
    Circulation 96:1403-7. 1997
    ..Previous studies have demonstrated an association of decreased HDL cholesterol with passive smoking in children but have not adjusted for potential confounding factors...
  11. ncbi Thiamine-responsive megaloblastic anemia syndrome: a disorder of high-affinity thiamine transport
    E J Neufeld
    Division of Hematology, Children s Hospital, Boston, Massachusetts 02115, USA
    Blood Cells Mol Dis 27:135-8. 2001
    ..Here we review the current status of studies aimed at understanding the pathophysiology of this unique transport defect...
  12. ncbi Characterization of a murine high-affinity thiamine transporter, Slc19a2
    J C Fleming
    Division of Hematology, Children s Hospital, Boston, Massachusetts 02115, USA
    Mol Genet Metab 74:273-80. 2001
    ..Immunohistochemistry studies indicate that Slc19a2 is expressed on the cell surface and intracellularly, and is specifically localized to a subpopulation of cells in cochlea, small intestine, and pancreas...
  13. ncbi Risk factors for thromboembolism in teens: when should I test?
    Amy E Sass
    Division of Adolescent and Young Adult Medicine, Children s Hospital, Boston, Massachusetts 02115, USA
    Curr Opin Pediatr 14:370-8. 2002
    ..Instead, clinicians can use thoughtful screening questions to identify potentially high-risk patients for thrombophilia and consider testing for inherited risk factors on a case-specific basis...
  14. doi The incidence and implications of anti-heparin-platelet factor 4 antibody formation in a pediatric cardiac surgical population
    Mary P Mullen
    Children s Hospital Boston, Boston, MA 02115, USA
    Anesth Analg 107:371-8. 2008
    ....
  15. doi Safety and efficacy of pegylated interferon alpha-2a and ribavirin for the treatment of hepatitis C in patients with thalassemia
    Paul Harmatz
    Children s Hospital and Research Center Oakland, 747 52 Street, Oakland, CA 94609, USA
    Haematologica 93:1247-51. 2008
    ..The molar efficacy of deferoxamine improved with reduction in liver inflammation on biopsy (p=0.001). In conclusion, antiviral treatment is safe if transfusion requirement, iron toxicities and neutropenia are monitored...
  16. doi Inflammation and oxidant-stress in beta-thalassemia patients treated with iron chelators deferasirox (ICL670) or deferoxamine: an ancillary study of the Novartis CICL670A0107 trial
    Patrick B Walter
    Children s Hospital and Research Center Oakland, 747 52nd Street, Oakland, CA 94609, USA
    Haematologica 93:817-25. 2008
    ..We assessed whether oxidant-stress and inflammation in beta-thalassemia could be controlled by the novel oral iron chelator deferasirox as effectively as by deferoxamine...
  17. pmc Relative response of patients with myelodysplastic syndromes and other transfusion-dependent anaemias to deferasirox (ICL670): a 1-yr prospective study
    John Porter
    University College London, London, UK
    Eur J Haematol 80:168-76. 2008
    ..Dosage was determined by baseline liver iron concentration (LIC)...
  18. ncbi Evaluation of the coagulation system in children with two-ventricle congenital heart disease
    Kirsten C Odegard
    Department of Anesthesiology, Children s Hospital Boston, Harvard Medical School, Boston, Massachusetts 02115, USA
    Ann Thorac Surg 83:1797-803. 2007
    ..This study used age-matched controls to evaluate coagulation factors in children with two-ventricle congenital heart disease (CHD)...
  19. ncbi Successful use of bivalirudin for cardiac transplantation in a child with heparin-induced thrombocytopenia
    Christopher S D Almond
    Department of Cardiology, Children s Hospital, Boston, Massachusetts 02215, USA
    J Heart Lung Transplant 25:1376-9. 2006
    ..We present the case of a child with heparin-induced thrombocytopenia with thrombosis (HIT Type II) who underwent successful orthotopic cardiac transplantation using bivalirudin as the primary anti-coagulant for cardiopulmonary bypass...
  20. doi Practical implications of liver and heart iron load assessment by T2*-MRI in children and adults with transfusion-dependent anemias
    Sarah Deborah Chirnomas
    Division of Hematology Oncology, Children s Hospital Boston, Harvard Medical School, Boston, Massachusetts 02115, USA
    Am J Hematol 83:781-3. 2008
    ..These findings were present in both adult and pediatric patients. We recommend therapeutic monitoring of iron burden by evaluation of both liver and myocardial iron with T2*-MRI...
  21. ncbi Coagulation abnormalities in patients with single-ventricle physiology precede the Fontan procedure
    Kirsten C Odegard
    Department of Anesthesia, Division of Hematology, Children s Hospital, 300 Longwood Avenue, Boston, MA 02115, USA
    J Thorac Cardiovasc Surg 123:459-65. 2002
    ..A hypercoagulable state with deficiencies in proteins C and S has been implicated. Using age-matched control subjects, we evaluated whether an altered coagulation state is present earlier in the course of staged single-ventricle repair...
  22. ncbi Urinary hepcidin in congenital chronic anemias
    Susan L Kearney
    Division of Hematology Oncology, Children s Hospital, Boston, Massachusetts, USA
    Pediatr Blood Cancer 48:57-63. 2007
    ..Hepcidin, a regulator for iron homeostasis, is induced by inflammation and iron burden and suppressed by anemia and hypoxia. This study was conducted to determine the hepcidin levels in patients with congenital chronic anemias...
  23. ncbi Effectiveness and safety of ICL670 in iron-loaded patients with thalassaemia: a randomised, double-blind, placebo-controlled, dose-escalation trial
    Eric Nisbet-Brown
    Department of Paediatrics, Harvard Medical School, Boston, MA, USA
    Lancet 361:1597-602. 2003
    ..We aimed to investigate short-term efficacy, pharmacokinetic/pharma- codynamic (PK/PD) relations, and safety of ICL670, a novel, tridentate, orally active iron chelator...
  24. ncbi Prospective reevaluation of the association between thrombotic diathesis and legg-perthes disease
    M Timothy Hresko
    Department of Orthopaedic Surgery, Harvard Medical School, Boston, Massachusetts 02115, USA
    J Bone Joint Surg Am 84:1613-8. 2002
    ..We analyzed the value of screening for inherited thrombophilia in patients with Legg-Perthes disease by examining the association between Legg-Perthes disease and abnormalities in the thrombotic pathway...
  25. ncbi Lymphoid apoptosis and myeloid hyperplasia in CCAAT displacement protein mutant mice
    A M Sinclair
    Department of Pathology and Laboratory of Molecular Pathology, University of Texas Southwestern Medical Center, Dallas, 75390 9072, USA
    Blood 98:3658-67. 2001
    ..These data suggest that cux/CDP regulates normal hematopoiesis, in part, by modulating the levels of survival and/or apoptosis factors expressed by the microenvironment...
  26. ncbi The gene mutated in thiamine-responsive anaemia with diabetes and deafness (TRMA) encodes a functional thiamine transporter
    J C Fleming
    Division of Hematology, Children s Hospital, Dana Farber Cancer Institute, and Harvard Medical School, Boston, Massachusetts 02115, USA
    Nat Genet 22:305-8. 1999
    ..3 critical region. Here we present evidence that the gene SLC19A2 (for solute carrier family 19 (thiamine transporter), member 2) encodes the first known mammalian thiamine transporter, which we designate thiamine transporter-1 (THTR-1)...
  27. pmc Localization of the gene for thiamine-responsive megaloblastic anemia syndrome, on the long arm of chromosome 1, by homozygosity mapping
    E J Neufeld
    Division of Hematology, Children s Hospital, Dana Farber Cancer Institute, Boston, USA
    Am J Hum Genet 61:1335-41. 1997
    ..Characterization of the metabolic defect of TRMA may shed light on the role of thiamine deficiency in such common diseases...
  28. ncbi A novel mutation in the SLC19A2 gene in a Tunisian family with thiamine-responsive megaloblastic anaemia, diabetes and deafness syndrome
    S Gritli
    Division of Hematology and Oncology, Children's Hospital and Dana Farber Cancer Institute, Boston 02115, USA
    Br J Haematol 113:508-13. 2001
    ..We demonstrated that fibroblasts from this patient exhibited defective thiamine transport. These data confirm that the SLC19A2 gene is the high-affinity thiamine carrier and that this novel mutation is responsible for TRMA syndrome...
  29. ncbi X-linked gray platelet syndrome due to a GATA1 Arg216Gln mutation
    Venée N Tubman
    Division of Hematology Oncology, Children s Hospital Boston, 320 Longwood Avenue, Boston, MA, USA
    Blood 109:3297-9. 2007
    ..Our findings suggest that XLTT is within a spectrum of disorders constituting the gray platelet syndrome, and we propose that GATA1 is an upstream regulator of the genes required for platelet alpha-granule biogenesis...
  30. ncbi Myocardial immediate early gene activation after cardiopulmonary bypass with cardiac ischemia-reperfusion
    David P Nelson
    Department of Cardiology, Children s Hospital, Harvard Medical School, Boston, Massachusetts, USA
    Ann Thorac Surg 73:156-62. 2002
    ..The inflammatory process after cardiopulmonary bypass is accompanied by alterations in gene expression for various inflammatory mediators...
  31. ncbi Extracorporeal membrane oxygenation as a bridge to cardiac transplantation in a patient with cardiomyopathy and hemophilia A
    Ravi R Thiagarajan
    Department of Cardiology, Children s Hospital Boston, 300 Longwood Avenue, Boston, MA 02115, USA
    Intensive Care Med 29:985-8. 2003
    ..To report the use of extracorporeal membrane oxygenation (ECMO) as a bridge to cardiac transplantation in a patient with hemophilia A and dilated cardiomyopathy...
  32. ncbi Time course of early induction of intracellular adhesion molecule-1 messenger RNA during reperfusion, following cardiopulmonary bypass with hypothermic circulatory arrest in lambs
    Sarah Tabbutt
    Department of Cardiology, Children s Hospital, Dana Farber Cancer Institute, and Harvard Medical School, Boston, MA 02115, USA
    Pediatr Crit Care Med 4:83-8. 2003
    ..We investigated the time course of intracellular adhesion molecule-1 messenger RNA induction following cardiopulmonary bypass with hypothermic circulatory arrest...
  33. ncbi Chronic disseminated intravascular coagulation and childhood-onset skin necrosis resulting from homozygosity for a protein C Gla domain mutation, Arg15Trp
    Mara Steinkamp
    Division of Hematology, Children s Hospital, 300 Longwood Avenue, Boston, MA 02115, USA
    J Pediatr Hematol Oncol 24:685-8. 2002
    ..Chronic consumption coagulopathy without purpura fulminans or venous thrombosis is a rare presentation of defective protein C pathway. The result of this mutation is a mixed type I (low antigen) and type II (low function) phenotype...
  34. pmc Screening and counseling for thalassemia
    David H K Chui
    Blood 107:1735-7. 2006

Research Grants13

  1. Clinical Hematology Research Career Development Program (K12)
    Ellis Neufeld; Fiscal Year: 2007
    ..Formal annual evaluation of the program and of trainees' progress will be used for ongoing program improvements. ..
  2. Transfusion Medicine/Hemostasis Clinical Trials Network
    Ellis Neufeld; Fiscal Year: 2007
    ..children's hospital boston critiques ..
  3. THALASSEMIA CLINICAL RESEARCH NETWORK
    Ellis Neufeld; Fiscal Year: 2007
    ....
  4. Patient-Oriented Research in Pediatric Hematology
    Ellis Neufeld; Fiscal Year: 2007
    ..Using this approach, several unique disorders of blood clotting and anemia have been studied. ..
  5. Pathophysiology of thiamine-responsive anemia syndrome
    Ellis Neufeld; Fiscal Year: 2006
    ....