C M Roifman

Summary

Affiliation: The Hospital for Sick Children
Country: Canada

Publications

  1. doi request reprint Defining combined immunodeficiency
    Chaim M Roifman
    Division of Immunology and Allergy, Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada
    J Allergy Clin Immunol 130:177-83. 2012
  2. ncbi request reprint Mutations in the RNA component of RNase mitochondrial RNA processing might cause Omenn syndrome
    Chaim M Roifman
    Division of Immunology and Allergy and Program of Infection, Immunity, Injury and Repair, The Hospital for Sick Children and the University of Toronto, Ontario, Canada
    J Allergy Clin Immunol 117:897-903. 2006
  3. doi request reprint Hematopoietic stem cell transplantation for profound T-cell deficiency (combined immunodeficiency)
    Chaim M Roifman
    Division of Immunology Allergy, The Canadian Centre for Primary Immunodeficiency, The Jeffrey Modell Research Laboratory for the Diagnosis of Primary Immunodeficiency, The Hospital for Sick Children, University of Toronto, Toronto, ON M5G 1X8, Canada
    Immunol Allergy Clin North Am 30:209-19. 2010
  4. doi request reprint Combined immunodeficiency, facial dysmorphism, optic nerve atrophy, skeletal anomalies and developmental delay: a new syndrome
    C M Roifman
    Division of Immunology and Allergy, The Hospital for Sick Children and the University of Toronto, Toronto, Ontario, Canada
    Clin Genet 76:449-57. 2009
  5. ncbi request reprint A novel syndrome of combined immunodeficiency, autoimmunity and spondylometaphyseal dysplasia
    C M Roifman
    Immunology and Allergy, Department of Paediatrics, Infection, Immunity, Injury and Repair Program, Research Institute, The Hospital for Sick Children and the University of Toronto, Toronto, Ontario, Canada
    Clin Genet 63:522-9. 2003
  6. ncbi request reprint Purine nucleoside phosphorylase deficiency associated with a dysplastic marrow morphology
    Yigal Dror
    Division of Immunology and Allergy, The Hospital for Sick Children, 555 University Ave, Rm 7279 Elm Wing, Toronto, Ontario, M5G 1X8, Canada
    Pediatr Res 55:472-7. 2004
  7. ncbi request reprint Signals find their target: drug design and diagnosis based on intracellular signalling
    Chaim M Roifman
    Division of Immunology and Allergy, Program of Infection, Immunity, Injury and Repair, The Hospital for Sick Children, The University of Toronto, Toronto, Ont
    Clin Invest Med 27:282-4. 2004
  8. ncbi request reprint CD3 delta immunodeficiency
    Chaim M Roifman
    Division of Immunology and Allergy, and Program of Infection, Immunity, Injury and Repair, The Hospital for Sick Children and the University of Toronto, Ontario M5G 1X8, Canada
    Curr Opin Allergy Clin Immunol 4:479-84. 2004
  9. ncbi request reprint Studies of patients' thymi aid in the discovery and characterization of immunodeficiency in humans
    Chaim M Roifman
    Division of Immunology and Allergy, and Program of Infection, Immunity, Injury and Repair, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
    Immunol Rev 203:143-55. 2005
  10. ncbi request reprint Matched unrelated bone marrow transplant for severe combined immunodeficiency
    Chaim M Roifman
    Division of Immunology and Allergy and Infection, Immunity, Injury and Repair Program, The Canadian Centre for Primary Immunodeficiency, The Hospital for Sick Children and the University of Toronto, Toronto, ON, M5G 1X8, Canada
    Immunol Res 38:191-200. 2007

Collaborators

Detail Information

Publications51

  1. doi request reprint Defining combined immunodeficiency
    Chaim M Roifman
    Division of Immunology and Allergy, Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada
    J Allergy Clin Immunol 130:177-83. 2012
    ..Therefore it is important to define the numbers of autologous cells usually detected in patients with CID versus those with SCID...
  2. ncbi request reprint Mutations in the RNA component of RNase mitochondrial RNA processing might cause Omenn syndrome
    Chaim M Roifman
    Division of Immunology and Allergy and Program of Infection, Immunity, Injury and Repair, The Hospital for Sick Children and the University of Toronto, Ontario, Canada
    J Allergy Clin Immunol 117:897-903. 2006
    ..We identified 2 patients who presented with clinical features consistent with Omenn syndrome but had no mutations in RAG or Artemis. Both patients also had cartilage-hair hypoplasia (CHH)...
  3. doi request reprint Hematopoietic stem cell transplantation for profound T-cell deficiency (combined immunodeficiency)
    Chaim M Roifman
    Division of Immunology Allergy, The Canadian Centre for Primary Immunodeficiency, The Jeffrey Modell Research Laboratory for the Diagnosis of Primary Immunodeficiency, The Hospital for Sick Children, University of Toronto, Toronto, ON M5G 1X8, Canada
    Immunol Allergy Clin North Am 30:209-19. 2010
    ..Until such studies become instructive, the protocols in current use seem to provide excellent, although not perfect, outcome in patients with PTD/CID...
  4. doi request reprint Combined immunodeficiency, facial dysmorphism, optic nerve atrophy, skeletal anomalies and developmental delay: a new syndrome
    C M Roifman
    Division of Immunology and Allergy, The Hospital for Sick Children and the University of Toronto, Toronto, Ontario, Canada
    Clin Genet 76:449-57. 2009
    ..To the best of our knowledge, this is a hitherto new syndrome with most probably autosomal recessive inheritance and unknown etiology...
  5. ncbi request reprint A novel syndrome of combined immunodeficiency, autoimmunity and spondylometaphyseal dysplasia
    C M Roifman
    Immunology and Allergy, Department of Paediatrics, Infection, Immunity, Injury and Repair Program, Research Institute, The Hospital for Sick Children and the University of Toronto, Toronto, Ontario, Canada
    Clin Genet 63:522-9. 2003
    ..Presumably because of dysregulation of the immune system, all patients also developed autoimmune manifestations...
  6. ncbi request reprint Purine nucleoside phosphorylase deficiency associated with a dysplastic marrow morphology
    Yigal Dror
    Division of Immunology and Allergy, The Hospital for Sick Children, 555 University Ave, Rm 7279 Elm Wing, Toronto, Ontario, M5G 1X8, Canada
    Pediatr Res 55:472-7. 2004
    ..We conclude that marrows from patients with PNP deficiency might have hypersensitivity to irradiation and can develop dysplastic morphology, caused either directly or indirectly by the inherited enzymatic defect...
  7. ncbi request reprint Signals find their target: drug design and diagnosis based on intracellular signalling
    Chaim M Roifman
    Division of Immunology and Allergy, Program of Infection, Immunity, Injury and Repair, The Hospital for Sick Children, The University of Toronto, Toronto, Ont
    Clin Invest Med 27:282-4. 2004
  8. ncbi request reprint CD3 delta immunodeficiency
    Chaim M Roifman
    Division of Immunology and Allergy, and Program of Infection, Immunity, Injury and Repair, The Hospital for Sick Children and the University of Toronto, Ontario M5G 1X8, Canada
    Curr Opin Allergy Clin Immunol 4:479-84. 2004
    ..The review also compares CD3 delta deficiency with other human CD3 subunit deficiencies and with corresponding animal models...
  9. ncbi request reprint Studies of patients' thymi aid in the discovery and characterization of immunodeficiency in humans
    Chaim M Roifman
    Division of Immunology and Allergy, and Program of Infection, Immunity, Injury and Repair, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
    Immunol Rev 203:143-55. 2005
    ..Finally, immunohistochemical analysis of the thymus was critical in pinpointing the roles of Zap-70, CD25, and CD3delta in the development of human T cells...
  10. ncbi request reprint Matched unrelated bone marrow transplant for severe combined immunodeficiency
    Chaim M Roifman
    Division of Immunology and Allergy and Infection, Immunity, Injury and Repair Program, The Canadian Centre for Primary Immunodeficiency, The Hospital for Sick Children and the University of Toronto, Toronto, ON, M5G 1X8, Canada
    Immunol Res 38:191-200. 2007
    ..In conclusion, MUD BMT appears vastly superior to MMRD BMT and should be offered as first choice of treatment for patients with SCID when RID is unavailable...
  11. doi request reprint Indications for hemopoietic stem cell transplantation
    Chaim M Roifman
    Division of Immunology and Allergy, The Canadian Centre for Primary Immunodeficiency, The Jeffrey Modell Research Laboratory for the Diagnosis of Primary Immunodeficiency, The Hospital for Sick Children, University of Toronto, 555 University Avenue, Toronto, ON M5G 1X8, Canada
    Immunol Allergy Clin North Am 30:261-2. 2010
    ..Included are: severe combined immunodeficiency, profound T cell defects, autoimmune and autoinflammatory syndromes, innate immune defects, hemophagocytic disorders, and other conditions. Some causes and limitations are included...
  12. doi request reprint Characterization of ΞΆ-associated protein, 70 kd (ZAP70)-deficient human lymphocytes
    Chaim M Roifman
    Division of Immunology and Allergy, Canadian Centre for Primary Immunodeficiency, Jeffrey Modell Research Laboratory for the diagnosis of Primary Immunodeficiency, Hospital for Sick Children, Toronto, Ontario, Canada
    J Allergy Clin Immunol 126:1226-33.e1. 2010
    ..Patients who live beyond infancy can also experience autoimmune manifestations...
  13. ncbi request reprint A partial deficiency of interleukin-7R alpha is sufficient to abrogate T-cell development and cause severe combined immunodeficiency
    C M Roifman
    Division of Immunology Allergy, Department of Paediatrics, The Hospital for Sick Children, Toronto, Canada
    Blood 96:2803-7. 2000
    ..This underscores the critical role of IL-7R alpha chain in the differentiation of T cells. (Blood. 2000;96:2803-2807)..
  14. ncbi request reprint Defective T cell receptor signaling and CD8+ thymic selection in humans lacking zap-70 kinase
    E Arpaia
    Division of Immunology and Allergy, Hospital for Sick Children, Toronto, Ontario, Canada
    Cell 76:947-58. 1994
    ..Thus, Zap-70 kinase appears to be indispensable for the development of CD8 single-positive T cells as well as for signal transduction and function of single-positive CD4 T cells...
  15. doi request reprint Matched unrelated bone marrow transplant for T+ combined immunodeficiency
    C M Roifman
    Division of Immunology and Allergy, The Hospital for Sick Children, Ontario, Canada ickkids ca
    Bone Marrow Transplant 41:947-52. 2008
    ..We have shown here that matched unrelated donor BMT is highly effective in curing patients with T+ CID. This mode of treatment should be preferred for patients with T+ CID when a related identical donor is not available...
  16. ncbi request reprint Immune function in patients with Shwachman-Diamond syndrome
    Y Dror
    Division of Haematology and Oncology, The Research Institute, The Hospital for Sick Children, Toronto, Ontario, Canada
    Br J Haematol 114:712-7. 2001
    ....
  17. pmc Mitochondrial basis for immune deficiency. Evidence from purine nucleoside phosphorylase-deficient mice
    E Arpaia
    Division of Immunology Allergy, Department of Paediatrics, Hospital for Sick Children, The University of Toronto, Ontario, Canada
    J Exp Med 191:2197-208. 2000
    ..The end result is increased sensitivity of T cells to spontaneous mitochondrial DNA damage, leading to T cell depletion by apoptosis...
  18. pmc An interleukin-2 receptor gamma chain mutation with normal thymus morphology
    N Sharfe
    Division of Immunology and Allergy, Department of Pediatrics, Hospital for Sick Children and the University of Toronto, Toronto, Canada M5G 1X8
    J Clin Invest 100:3036-43. 1997
    ..Early clinical recognition of this phenotype is critical as a delay in diagnosis may result in a fatal infection...
  19. ncbi request reprint Antibody deficiency, growth retardation, spondyloepiphyseal dysplasia and retinal dystrophy: a novel syndrome
    C M Roifman
    Department of Pediatrics, The Research Institute, The Hospital for Sick Children and the University of Toronto, ON, Canada
    Clin Genet 55:103-9. 1999
    ..Increased awareness of this syndrome will hopefully lead to the description of a larger number of affected individuals, which ultimately might be critical for its genetic characterization...
  20. ncbi request reprint Two novel mutations in a purine nucleoside phosphorylase (PNP)-deficient patient
    I Dalal
    Division of Immunology and Allergy, Department of Pediatrics, University of Toronto, Toronto, Ontario, Canada
    Clin Genet 59:430-7. 2001
    ..Both mutations predict severely truncated proteins resulting in a complete deficiency of PNP enzymatic activity, yet the development of profound immunodeficiency in this patient is greatly delayed...
  21. doi request reprint Management of primary antibody deficiency with replacement therapy: summary of guidelines
    Chaim M Roifman
    Division of Immunology and Allergy, Department of Paediatrics, The Research Institute of Hospital for Sick Children, 555 University Avenue, Toronto, Ontario, Canada M5G 1X8
    Immunol Allergy Clin North Am 28:875-6, x. 2008
    ..This article summarizes the guidelines for using replacement therapy in the management of patients who have primary antibody deficiency...
  22. ncbi request reprint Cloning and characterization of a lymphoid-specific, inducible human protein tyrosine phosphatase, Lyp
    S Cohen
    Division of Immunology and Allergy, Department of Paediatrics, Research Institute, The Hospital for Sick Children, Toronto, Ontario, Canada
    Blood 93:2013-24. 1999
    ..Overexpression of lyp1 reduces Cbl tyrosine phosphorylation, suggesting that it may be a substrate of the phosphatase. Thus, Lyp may play a role in regulating the function of Cbl and its associated protein kinases...
  23. ncbi request reprint Bone marrow transplantation for cartilage-hair-hypoplasia
    R Guggenheim
    Division of Immunology and Allergy, The Hospital for Sick Children, Toronto, Ontario, Canada
    Bone Marrow Transplant 38:751-6. 2006
    ..Repeated evaluation of the immune system showed normal cellular and humoral immunity. Our results should encourage the use of BMT in patients with CHH who have profound immunodeficiency...
  24. ncbi request reprint A mutation in zap-70 protein tyrosine kinase results in a selective immunodeficiency
    C M Roifman
    Division of Immunology Allergy, Hospital for Sick Children, Toronto, Ontario, Canada
    J Clin Immunol 15:52S-62S. 1995
    ..Thus zap-70 kinase appears to be indispensable for the development of CD8 single-positive T cells as well as for the signal transduction and function of single-positive CD4 T cells...
  25. ncbi request reprint Signal-transduction defects in T cells
    E Grunebaum
    IIIR Program, Research Institute, Hospital for Sick Children and the University of Toronto, Canada
    Clin Rev Allergy Immunol 20:27-42. 2001
    ..Further, mutations that result in partial deficiencies may result in a different phenotype from null mutations. This information may aid in improving structure/function analysis of these signaling components...
  26. doi request reprint Bone marrow transplantation using HLA-matched unrelated donors for patients suffering from severe combined immunodeficiency
    Eyal Grunebaum
    Division of Immunology and Allergy, Department of Pediatrics, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario M5G 1X8, Canada
    Immunol Allergy Clin North Am 30:63-73. 2010
    ..In conclusion, the excellent long-term survival, immune reconstitution, and normal quality of life after MUD BMT suggests that in the absence of RID or PMD, MUD BMT should be offered for patients suffering from SCID...
  27. ncbi request reprint Effect of CD3delta deficiency on maturation of alpha/beta and gamma/delta T-cell lineages in severe combined immunodeficiency
    Harjit K Dadi
    Divisions of Immunology and Allergy and the Infection, Immunity, Injury and Repair Program, The Research Institute and The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada
    N Engl J Med 349:1821-8. 2003
  28. ncbi request reprint Replacement therapy with high dose intravenous gamma-globulin improves chronic sinopulmonary disease in patients with hypogammaglobulinemia
    C M Roifman
    Division of Immunology and Rheumatology, Hospital for Sick Children, Toronto, Canada
    Pediatr Infect Dis J 7:S92-6. 1988
    ..High dose therapy with immune serum globulin suitable for intravenous administration appears to be the treatment of choice in patients with sinopulmonary disease...
  29. ncbi request reprint Fatal lung fibrosis associated with immunodeficiency and gonadal dysgenesis in 46XX sisters--a new syndrome
    Raz Somech
    Department of Pediatrics, The Canadian Centre for Primary Immunodeficiency, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
    Am J Med Genet A 146:8-14. 2008
    ..The distinct findings in these two sisters have not been reported before and thus suggest a hitherto unknown autosomal recessive condition that includes immune dysfunction, gonadal dysgenesis, and pulmonary fibrosis...
  30. doi request reprint Matched unrelated bone marrow transplant for Omenn syndrome
    Amit Nahum
    The Canadian Centre for Primary Immunodeficiency, Toronto, ON, Canada
    Immunol Res 44:25-34. 2009
    ..This mode of treatment should be preferred for patients with OS when a related identical donor is not available...
  31. ncbi request reprint Bone marrow transplantation for severe combined immune deficiency
    Eyal Grunebaum
    Division of Immunology Allergy, Department of Paediatrics, Research Institute, Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada
    JAMA 295:508-18. 2006
    ..However, compared with RIDs, use of MMRDs for BMT is associated with reduced survival and inferior long-term immune reconstitution. Use of HLA-matched unrelated donors (MUDs) represents another potential alternative for BMT...
  32. doi request reprint EdU incorporation is an alternative non-radioactive assay to [(3)H]thymidine uptake for in vitro measurement of mice T-cell proliferations
    Yongmao Yu
    The Division of Allergy and Clinical Immunology and Developmental and Stem Cell Biology Program, The Hospital for Sick Children, 555 University Ave, Toronto, Ontario M5G 1X8, Canada
    J Immunol Methods 350:29-35. 2009
    ..We developed the 5-ethynyl-2'-deoxyuridine (EdU) and Cu(I)-catalyzed cycloaddition "click" reaction assay to measure T-cell responses by flow cytometry...
  33. pmc Human immune disorder arising from mutation of the alpha chain of the interleukin-2 receptor
    N Sharfe
    Department of Pediatrics, University of Toronto, ON, Canada
    Proc Natl Acad Sci U S A 94:3168-71. 1997
    ..While displaying normal development of CD2, CD3, CD4, and CD8 expression, CD25-deficient cortical thymocytes do not express CD1, and furthermore they fail to normally down-regulate levels of the anti-apoptotic protein bcl-2...
  34. doi request reprint Lentivirus gene therapy for purine nucleoside phosphorylase deficiency
    Pu Liao
    Division of Allergy and Clinical Immunology, Research Institute, Hospital for Sick Children, Toronto, Canada
    J Gene Med 10:1282-93. 2008
    ....
  35. ncbi request reprint Novel mutations and hot-spots in patients with purine nucleoside phosphorylase deficiency
    Eyal Grunebaum
    Division of Clinical Immunology and Allergy, Infection, Immunity, Injury and Repair Program, Research Institute, The Hospital for Sick Children, Toronto, Ontario, Canada
    Nucleosides Nucleotides Nucleic Acids 23:1411-5. 2004
    ..Analysis of the gene encoding PNP in these patients reveals several recurring mutations. Identification of these hot-spots for mutation may allow faster confirmation of the diagnosis in suspected cases...
  36. ncbi request reprint Novel RAG1 mutation in a case of severe combined immunodeficiency
    Junyan Zhang
    Division of Immunology and Allergy, Hospital for Sick Children, University of Toronto, Toronto, Canada
    Pediatrics 116:e445-9. 2005
    ..Mutations in RAG1 or RAG2 can lead to a spectrum of disorders, ranging from typical (B-)(T-) severe combined immunodeficiency to Omenn's syndrome. We present a unique presentation of RAG1 deficiency...
  37. doi request reprint Cardiac chamber hypertrophy following hematopoietic stem cell transplantation for primary immunodeficiency
    Sean R Bulley
    Division of Immunology Allergy, Blood and Marrow Transplant Unit, Department of Paediatrics, The Hospital for Sick Children and the University of Toronto, Toronto, Canada
    Biol Blood Marrow Transplant 14:229-35. 2008
    ..We conclude that following HSCT, young children who suffer from aGVHD, treated with high CS doses, and have excessive hypertension are at risk of developing CCH...
  38. ncbi request reprint Role of MHC class I in immune surveillance of mitochondrial DNA integrity
    Yiping Gu
    Division of Immunology and Allergy, Department of Pediatrics and Immunology, Research Institute, University of Toronto and Hospital for Sick Children, Toronto, Ontario, Canada
    J Immunol 170:3603-7. 2003
    ..These observations in both mice and humans suggest a role for the immune system in preventing reversion of mitochondrial DNA back into a parasitic state following deleterious mutations affecting mitochondrial oxidative phosphorylation...
  39. ncbi request reprint Mutation analysis should be performed to rule out gammac deficiency in children with functional severe combined immune deficiency despite apparently normal immunologic tests
    Raz Somech
    Division of Immunology Allergy and the Infection, Immunity, Injury and Repair Program, The Research Institute and The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
    J Pediatr 147:555-7. 2005
    ..Both had infections suggestive of immunodeficiency, but their immune function and lymphoid tissues were normal. They were found to have an identical R222C mutation in the gammac gene...
  40. ncbi request reprint Human T cell immunodeficiency: when signal transduction goes wrong
    Eyal Grunebaum
    Division of Immunology Allergy and the Infection, Immunity, Injury and Repair Program, The Research Institute and The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
    Immunol Res 35:117-26. 2006
    ..This review summarizes several of these discoveries and some of their impact on our understanding of T cells development, function, and homeostasis in humans...
  41. ncbi request reprint The EphB6 receptor inhibits JNK activation in T lymphocytes and modulates T cell receptor-mediated responses
    Andrew Freywald
    Division of Immunology and Allergy, Department of Pediatrics, Infection, Immunity, Injury, and Repair Program, Research Institute, The Hospital for Sick Children and the University of Toronto, Ontario M5G 1X8, Canada
    J Biol Chem 278:10150-6. 2003
    ..Thus, EphB6 may play an important role in regulating thymocyte differentiation and modulating responses of mature T cells...
  42. ncbi request reprint Comparison of the efficacy of IGIV-C, 10% (caprylate/chromatography) and IGIV-SD, 10% as replacement therapy in primary immune deficiency. A randomized double-blind trial
    Chaim M Roifman
    Division of Immunology Allergy, Hospital for Sick Children, Toronto, ON, Canada M5G 1X8
    Int Immunopharmacol 3:1325-33. 2003
    ..No evidence of viral transmission was observed. IGIV-C appears to be superior to IGIV-SD in preventing validated sinopulmonary infections, especially acute sinusitis, in patients with PID...
  43. doi request reprint Variability of clinical and laboratory features among patients with ribonuclease mitochondrial RNA processing endoribonuclease gene mutations
    Fotini D Kavadas
    Division of Immunology and Allergy, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
    J Allergy Clin Immunol 122:1178-84. 2008
    ..Typical features of cartilage hair hypoplasia include short stature, a predisposition to malignancy, and a variable degree of impairment of cellular immunity...
  44. ncbi request reprint The kinase-null EphB6 receptor undergoes transphosphorylation in a complex with EphB1
    Andrew Freywald
    Immunology and Allergy, Department of Paediatrics, Infection, Immunity, Injury and Repair Program, Research Institute, The Hospital for Sick Children and the University of Toronto, Toronto M5G 1X8, Canada
    J Biol Chem 277:3823-8. 2002
    ..Our findings demonstrate that EphB6 is an actively signaling receptor that undergoes transphosphorylation upon ligand binding and that can initiate specific cytoplasmic signaling events...
  45. ncbi request reprint Gene abnormalities in patients with hemophagocytic lymphohistiocytosis
    Eyal Grunebaum
    Division of Immunology, Program of Infection, Immunity, Injury and Repair, Research Institute, Hospital for Sick Children, Toronto, Ontario, Canada
    Isr Med Assoc J 4:366-9. 2002
    ..Since abnormal function of these genes is associated with a wide clinical spectrum, HLH is probably another manifestation of immune deficiency and a thorough immune evaluation should be done in all such patients...
  46. ncbi request reprint EphA and ephrin-A proteins regulate integrin-mediated T lymphocyte interactions
    Nigel Sharfe
    Immunology and Allergy, Department of Paediatrics, Hospital for Sick Children and University of Toronto, Toronto, Ontario M5G 1X8, Canada
    Mol Immunol 45:1208-20. 2008
    ..Given the ubiquitous nature of EphA/ephrin-A expression in tissues, these proteins likely play a significant role in regulating T-cell interactions...
  47. ncbi request reprint Burkitt's lymphoma in a patient with adenosine deaminase deficiency-severe combined immunodeficiency treated with polyethylene glycol-adenosine deaminase
    Maitham Husain
    Division of Immunology Allergy, The Hospital for Sick Children and the University of Toronto, Toronto, Ontario, Canada
    J Pediatr 151:93-5. 2007
    ..Although treatment corrected the metabolic abnormalities caused by ADA deficiency, it failed to fully restore cellular immunity...
  48. ncbi request reprint Severe combined immunodeficiency caused by a splicing abnormality of the CD3delta gene
    Hidetoshi Takada
    Department of Paediatrics, Graduate School of Medical Sciences, Kyushu University, 3 1 1 Maidashi, 812 8582 Higashi ku, Fukuoka, Japan
    Eur J Pediatr 164:311-4. 2005
    ..Analysis of patients' mononuclear cells revealed the CD3delta splicing abnormality. Chest X-ray films and computed tomography revealed small sized thymuses in these patients...
  49. ncbi request reprint EphA receptors inhibit anti-CD3-induced apoptosis in thymocytes
    Andrew Freywald
    Department of Chemistry and Biochemistry, University of Regina, Saskatchewan, Canada
    J Immunol 176:4066-74. 2006
    ..Our findings suggest that EphA receptor activation is antithetical to TCR signaling in thymocytes, and that the level of engagement by ephrin-A proteins on thymic APCs regulates thymocyte selection...
  50. ncbi request reprint Health-related quality of life and treatment satisfaction in North American patients with primary immunedeficiency diseases receiving subcutaneous IgG self-infusions at home
    Uwe Nicolay
    Section of Clinical Immunology, Department of Laboratory Medicine, Karolinska Institutet at Karolinska University Hospital, Stockholm, Sweden
    J Clin Immunol 26:65-72. 2006
    ..Treatment satisfaction was significantly improved in Group A. The preference for the subcutaneous route and for home therapy was respectively 81% and 90% in Group A. In Group B, 69% preferred the subcutaneous route and 92% home therapy...
  51. doi request reprint Adenosine deaminase deficiency can present with features of Omenn syndrome
    Chaim M Roifman
    J Allergy Clin Immunol 121:1056-8. 2008