A Etzioni

Summary

Country: Israel

Publications

  1. ncbi request reprint Leukocyte adhesion deficiency III: a group of integrin activation defects in hematopoietic lineage cells
    Amos Etzioni
    Meyer Children s Hospital, B Rappaport Faculty of Medicine, Technion, Haifa 31096, Israel
    Curr Opin Allergy Clin Immunol 4:485-90. 2004
  2. doi request reprint Loss of Kindlin-3 in LAD-III eliminates LFA-1 but not VLA-4 adhesiveness developed under shear flow conditions
    Eugenia Manevich-Mendelson
    Department of Immunology, Weizmann Institute of Science, Rehovot, Israel
    Blood 114:2344-53. 2009
  3. doi request reprint Genetic etiologies of leukocyte adhesion defects
    Amos Etzioni
    Meyer s Children Hospital, Rappaport Faculty of Medicine, Technion, Haifa, Israel
    Curr Opin Immunol 21:481-6. 2009
  4. ncbi request reprint The hyper IgM syndrome--an evolving story
    Amos Etzioni
    Department of Pediatrics, Meyer Children s Hospital, B Rappaport Faculty of Medicine, Technion, Haifa, Israel 31096
    Pediatr Res 56:519-25. 2004
  5. ncbi request reprint Fatal varicella associated with selective natural killer cell deficiency
    Amos Etzioni
    Meyer Children s Hospital, Department of Immunology, B Rappaport Medical School, Technion, Haifa, Israel
    J Pediatr 146:423-5. 2005
  6. ncbi request reprint Immunodeficiency: nobody is immune
    Amos Etzioni
    Meyer Children s Hospital, Rambam Medical Center, Haifa, Israel
    Isr Med Assoc J 7:756-7. 2005
  7. ncbi request reprint Leukocyte adhesion deficiency (LAD) type II/carbohydrate deficient glycoprotein (CDG) IIc founder effect and genotype/phenotype correlation
    Amos Etzioni
    Department of Pediatrics and Immunology, Rambam Med Ctr, B Rapapport School of Medicine, Technion, Haifa, Israel
    Am J Med Genet 110:131-5. 2002
  8. ncbi request reprint Novel aspects of hypogammaglobulinemic states
    Amos Etzioni
    Department of Pediatrics, Rambam Medical Center, Department of Immunology, Rappaport Faculty of Medicine, Technion Israel Institute of Technology, Haifa, Israel
    Isr Med Assoc J 4:294-7. 2002
  9. ncbi request reprint Novel aspects of phagocytic cell disorders
    A Etzioni
    Department of Pediatrics, Rambam Medical Centre and B Rappaport School of Medicine, Technion, Haifa, Israel
    Curr Opin Allergy Clin Immunol 1:535-40. 2001
  10. ncbi request reprint Ataxia-telangiectasia in twins presenting as autosomal recessive hyper-immunoglobulin M syndrome
    Amos Etzioni
    Department of Pediatrics, Meyer Children s Hospital, Haifa, Israel
    Isr Med Assoc J 9:406-7. 2007

Collaborators

Detail Information

Publications38

  1. ncbi request reprint Leukocyte adhesion deficiency III: a group of integrin activation defects in hematopoietic lineage cells
    Amos Etzioni
    Meyer Children s Hospital, B Rappaport Faculty of Medicine, Technion, Haifa 31096, Israel
    Curr Opin Allergy Clin Immunol 4:485-90. 2004
    ..The clinical manifestations and molecular basis of this syndrome and related cases will be reviewed...
  2. doi request reprint Loss of Kindlin-3 in LAD-III eliminates LFA-1 but not VLA-4 adhesiveness developed under shear flow conditions
    Eugenia Manevich-Mendelson
    Department of Immunology, Weizmann Institute of Science, Rehovot, Israel
    Blood 114:2344-53. 2009
    ..Deletion of the putative beta(1) Kindlin-3 binding site also retained VLA-4 adhesiveness. Thus, our study provides the first evidence that Kindlin-3 is more critical to LFA-1 than to VLA-4-adhesive functions in human lymphocytes...
  3. doi request reprint Genetic etiologies of leukocyte adhesion defects
    Amos Etzioni
    Meyer s Children Hospital, Rappaport Faculty of Medicine, Technion, Haifa, Israel
    Curr Opin Immunol 21:481-6. 2009
    ..Just recently mutations in Kindlin 3, a newly recognized component, which binds the cytoplasmic tail of integrin, and is important in integrin activation, the second phase of the adhesion cascade, were found...
  4. ncbi request reprint The hyper IgM syndrome--an evolving story
    Amos Etzioni
    Department of Pediatrics, Meyer Children s Hospital, B Rappaport Faculty of Medicine, Technion, Haifa, Israel 31096
    Pediatr Res 56:519-25. 2004
    ..Thus, the molecular definition of these rare primary immune deficiency disorders has shed light on the complex events leading to the production of high-affinity, antigen-specific antibodies of different isotypes...
  5. ncbi request reprint Fatal varicella associated with selective natural killer cell deficiency
    Amos Etzioni
    Meyer Children s Hospital, Department of Immunology, B Rappaport Medical School, Technion, Haifa, Israel
    J Pediatr 146:423-5. 2005
    ..No natural killer (NK) cells were detected, and NK activity was markedly decreased. The interleukin (IL)15/IL15R signaling pathway was intact. This case emphasizes the role of NK cells in controlling herpes viral infection...
  6. ncbi request reprint Immunodeficiency: nobody is immune
    Amos Etzioni
    Meyer Children s Hospital, Rambam Medical Center, Haifa, Israel
    Isr Med Assoc J 7:756-7. 2005
  7. ncbi request reprint Leukocyte adhesion deficiency (LAD) type II/carbohydrate deficient glycoprotein (CDG) IIc founder effect and genotype/phenotype correlation
    Amos Etzioni
    Department of Pediatrics and Immunology, Rambam Med Ctr, B Rapapport School of Medicine, Technion, Haifa, Israel
    Am J Med Genet 110:131-5. 2002
    ..Genotype/phenotype correlation studies show that the two different mutations are distinguished by differences in response to fucose supplementation and in the clinical phenotypes...
  8. ncbi request reprint Novel aspects of hypogammaglobulinemic states
    Amos Etzioni
    Department of Pediatrics, Rambam Medical Center, Department of Immunology, Rappaport Faculty of Medicine, Technion Israel Institute of Technology, Haifa, Israel
    Isr Med Assoc J 4:294-7. 2002
  9. ncbi request reprint Novel aspects of phagocytic cell disorders
    A Etzioni
    Department of Pediatrics, Rambam Medical Centre and B Rappaport School of Medicine, Technion, Haifa, Israel
    Curr Opin Allergy Clin Immunol 1:535-40. 2001
    ..Novel therapeutic modalities to treat phagocyte abnormalities, which are currently under clinical trials, are also discussed...
  10. ncbi request reprint Ataxia-telangiectasia in twins presenting as autosomal recessive hyper-immunoglobulin M syndrome
    Amos Etzioni
    Department of Pediatrics, Meyer Children s Hospital, Haifa, Israel
    Isr Med Assoc J 9:406-7. 2007
  11. ncbi request reprint Leukocyte adhesion deficiencies: molecular basis, clinical findings, and therapeutic options
    Amos Etzioni
    Meyer Children Hospital, the Rappaport School of Medicine, Technion, Haifa, Israel
    Adv Exp Med Biol 601:51-60. 2007
    ..Although the primary defect is still unknown, it is clear that all hematopoietic integrin activation processes are defective, which lead to severe infection as observed in LAD I and to marked increase tendency for bleeding problems...
  12. ncbi request reprint Leukocyte adhesion deficiency II-from A to almost Z
    A Etzioni
    Department of Pediatrics, Rambam Medical Center, B Rappaport School of Medicine, Technion, Haifa, Israel
    Immunol Rev 178:138-47. 2000
    ..It seems that the primary defect is somewhat different and, therefore, fucose administration was effective in the Turkish child, but did not show any beneficial results in the patients of Arabic origin...
  13. doi request reprint Defects in the leukocyte adhesion cascade
    Amos Etzioni
    Meyer Children Hospital, Rappaport School of Medicine, Technion, Haifa, Israel
    Clin Rev Allergy Immunol 38:54-60. 2010
    ..The various genetic defects leading to all adhesion molecules syndrome will be discussed...
  14. ncbi request reprint Immune deficiency and autoimmunity
    Amos Etzioni
    Meyer Children s Hospital, Haifa and the B Rappaport Faculty of Medicine, Technion, Haifa, 36019, Israel
    Autoimmun Rev 2:364-9. 2003
    ..Treatment with intravenous gammagluobuilin may ameliorate the autoimmune disorder and bone marrow transplantation can cure both conditions...
  15. ncbi request reprint A novel genetic leukocyte adhesion deficiency in subsecond triggering of integrin avidity by endothelial chemokines results in impaired leukocyte arrest on vascular endothelium under shear flow
    Ronen Alon
    Department of Immunology, The Weizmann Institute of Science Rehovot, Israel
    Blood 101:4437-45. 2003
    ..This syndrome is the first example of a human integrin-activation deficiency associated with defective GPCR stimulation of integrin avidity at subsecond contacts, a key step in leukocyte arrest on vascular endothelium under shear flow...
  16. ncbi request reprint The influence of oral insulin on the development of autoimmune diabetes in NOD mice fed a hypoallergenic diet
    N Shehadeh
    Pediatric Diabetes Unit, Rambam Medical Center, Haifa, Israel
    Diabetes Nutr Metab 17:1-5. 2004
    ..However, it has been reported that autoimmune diabetes can be induced by oral administration of an auto-antigen (insulin), and great caution is therefore warranted when applying the oral tolerance approach to prevent Type 1 diabetes...
  17. ncbi request reprint Importance of insulin content in infant diet: suggestion for a new infant formula
    N Shehadeh
    Department of Pediatrics, Rambam Medical Center, Haifa, Israel
    Acta Paediatr 90:93-5. 2001
    ..32 +/- 5.98 microU/ml mean +/- SD) and that insulin is hardly detectable in infant formulas. We propose the addition of human insulin to infant formula to match its composition more closely to human milk...
  18. ncbi request reprint Influence of oral insulin supplementation on carbohydrate, lipid and protein metabolism in weaned Balb/c mice
    Naim Shehadeh
    Juvenile Diabetes Unit, Rambam Medical Center and the B Rapapport Faculty of Medicine, Technion lsrael Institute of Technology, Haifa, Israel
    J Pediatr Endocrinol Metab 16:431-7. 2003
    ..Objective: To examine local and systemic effects of oral insulin supplementation in the post-weaning period...
  19. ncbi request reprint Safe and efficacious allogeneic bone marrow transplantation for nonmalignant disorders using partial T cell depletion and no posttransplantation graft-versus-host-disease prophylaxis
    Ronit Elhasid
    Department of Pediatric Hemato Oncology, Meyer Children Hospital, Haifa, Israel
    Biol Blood Marrow Transplant 13:329-38. 2007
    ....
  20. ncbi request reprint Familial severe congenital neutropenia associated with infantile osteoporosis: a new entity
    R Elhasid
    Division of Pediatric Hemato Oncology, Meyer Children s Hospital, Haifa, Israel
    Am J Hematol 72:34-7. 2003
    ..A possible role for a new recognized cytokine system involved in bone remodeling, the osteoprotegerin/receptor activator of nuclear factor-kappa B ligand, is suggested...
  21. ncbi request reprint Adhesion molecule deficiencies and their clinical significance
    A Etzioni
    Department of Pediatrics, Rambam Medical Center, Bruce Rappaport Faculty of Medicine, Technion Israel Institute of Technology, Haifa
    Cell Adhes Commun 2:257-60. 1994
    ..Studies of these two rare conditions emphasized the important in vivo roles of adhesion molecules in host defense mechanism...
  22. ncbi request reprint Repeated BCG vaccination is more effective than a single dose in preventing diabetes in non-obese diabetic (NOD) mice
    N Shehadeh
    Juvenile Diabetes Unit, Rambam Medical Center, Haifa, Israel
    Isr J Med Sci 33:711-5. 1997
    ..Our report demonstrates that repeated BCG vaccination is safe and more effective than a single dose in preventing type I diabetes in NOD mice. This data should be considered in planning new human trials with BCG...
  23. pmc Compound heterozygosity for abetalipoproteinaemia and familial hypobetalipoproteinaemia
    S Keidar
    Lipid Research Centre, Rambam Medical Centre, Haifa, Israel
    J Med Genet 27:133-4. 1990
    ..This is the first report of abetalipoproteinaemia resulting from compound heterozygosity for abetalipoproteinaemia and familial hypobetalipoproteinaemia...
  24. doi request reprint Chronic granulomatous disease in Israel: clinical, functional and molecular studies of 38 patients
    Baruch Wolach
    Department of Pediatrics, Meir Medical Center, Kfar Saba, Israel
    Clin Immunol 129:103-14. 2008
    ..Given that bone-marrow transplantation was successful in five of seven patients, it is recommended to perform it as early as possible before tissue damage is irreversible...
  25. pmc A LAD-III syndrome is associated with defective expression of the Rap-1 activator CalDAG-GEFI in lymphocytes, neutrophils, and platelets
    Ronit Pasvolsky
    Department of Immunology, The Weizmann Institute of Science, Rehovot 76100, Israel
    J Exp Med 204:1571-82. 2007
    ..Chemokine-triggered VLA-4 adhesiveness in T lymphocytes was partially defective as well. These studies identify CalDAG-GEFI as a critical regulator of inside-out integrin activation in human T lymphocytes, neutrophils, and platelets...
  26. ncbi request reprint LAD-III, a leukocyte adhesion deficiency syndrome associated with defective Rap1 activation and impaired stabilization of integrin bonds
    Tatsuo Kinashi
    Department of Immunology, The Weizmann Institute of Science, Rehovot, 76100, Israel
    Blood 103:1033-6. 2004
    ..This is a first report of an inherited Rap1 activation defect associated with a pathologic disorder in leukocyte integrin function, we herein term it "LAD-III."..
  27. ncbi request reprint LAD-III, a novel group of leukocyte integrin activation deficiencies
    Ronen Alon
    Department of Immunology, The Weizmann Institute of Science, 76100 Rehovot, Israel
    Trends Immunol 24:561-6. 2003
    ..Here, the mechanisms by which GPCRs might regulate leukocyte and platelet integrins are outlined with respect to this new family of LAD cases. We propose to term this the LAD-III family...
  28. ncbi request reprint Intravenous immunoglobulin in immunodeficiency states: state of the art
    Elias Toubi
    Department of Immunology, Rappaport Medical School, Technion, Haifa, Israel
    Clin Rev Allergy Immunol 29:167-72. 2005
    ..The route of immunoglobulin G replacement (intravenously vs subcutaneously) was reported to be of similar benefit. However, guidelines for usage and choice of route should be established and might be of help...
  29. doi request reprint The role of Toll-like receptor signaling in human immunodeficiencies
    Hanna Suhir
    Meyer s Children Hospital, the Rappaport School of Medicine, Technion, Haifa, Israel
    Clin Rev Allergy Immunol 38:11-9. 2010
    ..Furthermore, we highlight the published data associating TLR polymorphism with an altered susceptibility to infectious diseases...
  30. ncbi request reprint Autoimmunity in severe combined immunodeficiency (SCID)
    Ronit Elhasid
    Blood 100:2677-8; author reply 2678-9. 2002
  31. ncbi request reprint The incidence of primary immunodeficiency syndromes in Israel
    Hana Golan
    Sheba Medical Center, Tel Hashomer, Israel
    Isr Med Assoc J 4:868-71. 2002
  32. ncbi request reprint LAD III versus LAD I variant
    Amos Etzioni
    Blood 110:4129; author reply 4129-30. 2007
  33. doi request reprint Kindlin-3: a new gene involved in the pathogenesis of LAD-III
    Adi Mory
    Blood 112:2591. 2008
  34. ncbi request reprint Differential terminal fucosylation of N-linked glycans versus protein O-fucosylation in leukocyte adhesion deficiency type II (CDG IIc)
    Laura Sturla
    Giannina Gaslini Institute, 16147 Genova, Italy
    J Biol Chem 278:26727-33. 2003
    ....
  35. doi request reprint Autoimmunity in severe combined immunodeficiency (SCID): lessons from patients and experimental models
    Joshua D Milner
    Laboratory of Immunology, National Institute of Allergy and Infectious Diseases, NIAID, NIH, Bethesda, MD, USA
    J Clin Immunol 28:S29-33. 2008
    ..It is thus of no surprise when both central and peripheral tolerance are impaired that autoimmunity can be observed in SCID...
  36. ncbi request reprint Interleukin-7 receptor alpha (IL-7Ralpha) deficiency: cellular and molecular bases. Analysis of clinical, immunological, and molecular features in 16 novel patients
    Silvia Giliani
    Angelo Nocivelli Institute of Molecular Medicine, Department of Pediatrics, University of Brescia, Italy
    Immunol Rev 203:110-26. 2005
    ..We also report on the clinical and immunological features of 16 novel patients with IL-7Ralpha deficiency and discuss the results of hematopoietic stem cell transplantation...
  37. ncbi request reprint Immune deficiencies: introduction
    Amos Etzioni
    Isr Med Assoc J 4:50. 2002
  38. ncbi request reprint Mutation analysis and clinical implications of von Willebrand factor-cleaving protease deficiency
    Karin Assink
    Department of Pediatric Nephrology, University Medical Centre Nijmegen, Nijmegen, The Netherlands
    Kidney Int 63:1995-9. 2003
    ..Deficiency of a recently identified novel metalloprotease, the von Willebrand factor (vWF)-cleaving protease, originating from mutations in the ADAMTS13 gene plays a major role in the development of TTP...