Francisco A Bonilla

Summary

Affiliation: Harvard University
Country: USA

Publications

  1. ncbi request reprint TACI mutation in common variable immunodeficiency and IgA deficiency
    Rima Rachid
    Division of Immunology, Children s Hospital, Boston, 300 Longwood Avenue, Boston, MA 02115, USA
    Curr Allergy Asthma Rep 6:357-62. 2006
  2. doi request reprint Interpretation of lymphocyte proliferation tests
    Francisco A Bonilla
    Division of Immunology, Children s Hospital Boston, Boston, Massachusetts, USA
    Ann Allergy Asthma Immunol 101:101-4. 2008
  3. doi request reprint Adaptive immunity
    Francisco A Bonilla
    Division of Immunology, Children s Hospital Boston, Boston, MA 02115, USA
    J Allergy Clin Immunol 125:S33-40. 2010
  4. doi request reprint Common variable immunodeficiency
    Francisco A Bonilla
    Department of Pediatrics, Harvard Medical School, Boston, Massachusetts 02115, USA
    Pediatr Res 65:13R-19R. 2009
  5. doi request reprint Pharmacokinetics of immunoglobulin administered via intravenous or subcutaneous routes
    Francisco A Bonilla
    Division of Immunology, Children s Hospital Boston, Fegan Building, 6th Floor, 300 Longwood Avenue, Boston, MA 02115, USA
    Immunol Allergy Clin North Am 28:803-19, ix. 2008
  6. ncbi request reprint 2. Update on primary immunodeficiency diseases
    Francisco A Bonilla
    Division of Immunology, Children s Hospital, Fegan Bldg 6th Floor, 300 Longwood Avenue, Boston, MA 02115, USA
    J Allergy Clin Immunol 117:S435-41. 2006
  7. ncbi request reprint The presentation and natural history of immunodeficiency caused by nuclear factor kappaB essential modulator mutation
    Jordan S Orange
    Department of Immunology, Children s Hospital, 300 Longwood Avenue, Boston, MA 02115, USA
    J Allergy Clin Immunol 113:725-33. 2004
  8. ncbi request reprint Practice parameter for the diagnosis and management of primary immunodeficiency
    Francisco A Bonilla
    Department of Medicine, Children s Hospital Boston, Harvard Medical School, Boston, Massachusetts, USA
    Ann Allergy Asthma Immunol 94:S1-63. 2005
  9. ncbi request reprint Human nuclear factor kappa B essential modulator mutation can result in immunodeficiency without ectodermal dysplasia
    Jordan S Orange
    Division of Immunology, Children s Hospital of Philadelphia, PA, USA
    J Allergy Clin Immunol 114:650-6. 2004
  10. ncbi request reprint Use of intravenous immunoglobulin in human disease: a review of evidence by members of the Primary Immunodeficiency Committee of the American Academy of Allergy, Asthma and Immunology
    Jordan S Orange
    Department of Pediatrics, University of Pennsylvania School of Medicine, The Children s Hospital of Philadelphia, Philadelphia 19104, USA
    J Allergy Clin Immunol 117:S525-53. 2006

Detail Information

Publications32

  1. ncbi request reprint TACI mutation in common variable immunodeficiency and IgA deficiency
    Rima Rachid
    Division of Immunology, Children s Hospital, Boston, 300 Longwood Avenue, Boston, MA 02115, USA
    Curr Allergy Asthma Rep 6:357-62. 2006
    ....
  2. doi request reprint Interpretation of lymphocyte proliferation tests
    Francisco A Bonilla
    Division of Immunology, Children s Hospital Boston, Boston, Massachusetts, USA
    Ann Allergy Asthma Immunol 101:101-4. 2008
  3. doi request reprint Adaptive immunity
    Francisco A Bonilla
    Division of Immunology, Children s Hospital Boston, Boston, MA 02115, USA
    J Allergy Clin Immunol 125:S33-40. 2010
    ..In subsequent encounters with the same pathogen, the memory cells are quickly activated to yield a more rapid and robust protective response...
  4. doi request reprint Common variable immunodeficiency
    Francisco A Bonilla
    Department of Pediatrics, Harvard Medical School, Boston, Massachusetts 02115, USA
    Pediatr Res 65:13R-19R. 2009
    ....
  5. doi request reprint Pharmacokinetics of immunoglobulin administered via intravenous or subcutaneous routes
    Francisco A Bonilla
    Division of Immunology, Children s Hospital Boston, Fegan Building, 6th Floor, 300 Longwood Avenue, Boston, MA 02115, USA
    Immunol Allergy Clin North Am 28:803-19, ix. 2008
    ..With either route of administration, there is large variation in individual IgG elimination rates. Periodic measurement of serum IgG concentration is critical to monitor the adequacy of replacement during therapy...
  6. ncbi request reprint 2. Update on primary immunodeficiency diseases
    Francisco A Bonilla
    Division of Immunology, Children s Hospital, Fegan Bldg 6th Floor, 300 Longwood Avenue, Boston, MA 02115, USA
    J Allergy Clin Immunol 117:S435-41. 2006
    ..Clinically defined immunodeficiencies, such as hyper-IgE syndrome and idiopathic CD4 lymphocytopenia, are also discussed. Finally, significant adverse effects in some patients have tempered initial enthusiasm for gene therapy...
  7. ncbi request reprint The presentation and natural history of immunodeficiency caused by nuclear factor kappaB essential modulator mutation
    Jordan S Orange
    Department of Immunology, Children s Hospital, 300 Longwood Avenue, Boston, MA 02115, USA
    J Allergy Clin Immunol 113:725-33. 2004
    ..Hypomorphic mutations in the NFkappaB essential modulator (NEMO) impair NFkappaB function and are linked to both immunodeficiency and ectodermal dysplasia (ED), as well as susceptibility to atypical mycobacterial infections...
  8. ncbi request reprint Practice parameter for the diagnosis and management of primary immunodeficiency
    Francisco A Bonilla
    Department of Medicine, Children s Hospital Boston, Harvard Medical School, Boston, Massachusetts, USA
    Ann Allergy Asthma Immunol 94:S1-63. 2005
  9. ncbi request reprint Human nuclear factor kappa B essential modulator mutation can result in immunodeficiency without ectodermal dysplasia
    Jordan S Orange
    Division of Immunology, Children s Hospital of Philadelphia, PA, USA
    J Allergy Clin Immunol 114:650-6. 2004
    ..Male subjects with these mutant NEMO molecules have an X-linked syndrome known as ectodermal dysplasia with immunodeficiency, which is characterized by severe infections, with herpesviruses, bacteria, and mycobacterial susceptibility...
  10. ncbi request reprint Use of intravenous immunoglobulin in human disease: a review of evidence by members of the Primary Immunodeficiency Committee of the American Academy of Allergy, Asthma and Immunology
    Jordan S Orange
    Department of Pediatrics, University of Pennsylvania School of Medicine, The Children s Hospital of Philadelphia, Philadelphia 19104, USA
    J Allergy Clin Immunol 117:S525-53. 2006
    ..Given the potential risks and inherent scarcity of IGIV, careful consideration of the indications for and administration of IGIV is warranted...
  11. doi request reprint Analysis of in vitro lymphocyte proliferation as a screening tool for cellular immunodeficiency
    Kelly D Stone
    Division of Immunology, Children s Hospital Boston, Boston, MA 02115, USA
    Clin Immunol 131:41-9. 2009
    ..Proliferation testing is a useful component of screening for cellular immunodeficiency, but is not a sensitive predictor of cellular immune compromise or risk of opportunistic infection...
  12. ncbi request reprint Acute chylothorax in children: selective retention of memory T cells and natural killer cells
    Jordan S Orange
    Division of Immunology, Children s Hospital, Enders Building, 300 Longwood Avenue, Boston, MA 02115, USA
    J Pediatr 143:243-9. 2003
    ..To assess for immunodeficiency in patients with hypogammaglobulinemia in the setting of draining acute chylothorax...
  13. doi request reprint NOD2-associated diseases: Bridging innate immunity and autoinflammation
    Arturo Borzutzky
    Children s Hospital Boston, MA 02115, USA
    Clin Immunol 134:251-61. 2010
    ..The patient had a susceptibility polymorphism of NOD2 previously described in CD, but not in BS or EOS. We discuss the complex role of NOD2 in innate immunity to microbes and the clinical consequences of disturbances in this system...
  14. pmc Wiskott-Aldrich syndrome protein is required for NK cell cytotoxicity and colocalizes with actin to NK cell-activating immunologic synapses
    Jordan S Orange
    Department of Molecular and Cellular Biology, 7 Divinity Avenue, Harvard University, Cambridge, MA 02138, USA
    Proc Natl Acad Sci U S A 99:11351-6. 2002
    ..Thus, WASp has an important function in NK cells. In patients with WASp mutations, the resulting NK cell defects are likely to contribute to their disease...
  15. doi request reprint The role of anti-IgA antibodies in causing adverse reactions to gamma globulin infusion in immunodeficient patients: a comprehensive review of the literature
    Rima Rachid
    Division of Immunology, Children s Hospital Boston, and the Department of Pediatrics, Harvard Medical School, Boston, MA 02115, USA
    J Allergy Clin Immunol 129:628-34. 2012
    ..The role of anti-IgA antibodies in causing anaphylaxis in IgA-deficient patients receiving gamma globulin therapy is still controversial. Larger (multicenter) studies are needed to further evaluate this association...
  16. ncbi request reprint Interleukin receptor-associated kinase-4 deficiency impairs Toll-like receptor-dependent innate antiviral immune responses
    Douglas R McDonald
    Division of Immunology, Children s Hospital Boston, Boston, MA 02115, USA
    J Allergy Clin Immunol 118:1357-62. 2006
    ..A recent report has described concomitant impairment of type I IFN production after activation of TLRs 7, 8, and 9, but not TLR3...
  17. pmc Pathogenesis, diagnosis, and management of primary antibody deficiencies and infections
    Ari J Fried
    Division of Immunology, Children s Hospital Boston, Fegan Building, 6th Floor, 300 Longwood Avenue, Boston, MA 02115, USA
    Clin Microbiol Rev 22:396-414. 2009
    ....
  18. ncbi request reprint IgG Fc receptor polymorphisms in human disease: implications for intravenous immunoglobulin therapy
    Bryce A Binstadt
    Division of Immunology, Children s Hospital Boston, MA 02115, USA
    J Allergy Clin Immunol 111:697-703. 2003
    ..In this article we review FcR polymorphisms in relation to autoimmune diseases for which intravenous immunoglobulin is used therapeutically...
  19. ncbi request reprint 12. Primary immunodeficiency diseases
    Francisco A Bonilla
    Division of Immunology, Children s Hospital, Enders 809, 300 Longwood Avenue, Boston, MA 02115, USA
    J Allergy Clin Immunol 111:S571-81. 2003
    ..Early diagnosis offers the best opportunity for reduced morbidity and survival and is critical for accurate genetic counseling...
  20. ncbi request reprint Disseminated varicella infection due to the vaccine strain of varicella-zoster virus, in a patient with a novel deficiency in natural killer T cells
    Ofer Levy
    Division Infectious Diseases, Children s Hospital, Harvard Medical School, Boston, Massachusetts 02115, USA
    J Infect Dis 188:948-53. 2003
    ....
  21. doi request reprint Hypomorphic Janus kinase 3 mutations result in a spectrum of immune defects, including partial maternal T-cell engraftment
    Federica Cattaneo
    Division of Hematology Oncology, The Manton Center for Orphan Disease Research, Department of Laboratory Medicine, Boston Children s Hospital, and Department of Pathology, Harvard Medical School, Boston, MA 02115, USA
    J Allergy Clin Immunol 131:1136-45. 2013
    ..Maternal T-cell engraftment is a common finding in infants with severe combined immunodeficiency but is not typically observed in patients with residual T-cell development...
  22. pmc A Markov model to analyze cost-effectiveness of screening for severe combined immunodeficiency (SCID)
    Kee Chan
    Department of Health Sciences, College of Health and Rehabilitation Sciences Sargent College, Boston University, Boston, MA 02215, USA
    Mol Genet Metab 104:383-9. 2011
    ..To evaluate the cost-effectiveness of universal neonatal screening for T cell lymphocytopenia in enhancing quality of life and life expectancy for children with severe combined immunodeficiency (SCID)...
  23. doi request reprint Reduced thymic output, cell cycle abnormalities, and increased apoptosis of T lymphocytes in patients with cartilage-hair hypoplasia
    Miguel A de la Fuente
    Division of Immunology and The Manton Center for Orphan Disease Research, Children s Hospital Boston, Boston, MA 02115, USA
    J Allergy Clin Immunol 128:139-46. 2011
    ..CHH is caused by mutations in the RNA component of the mitochondrial RNA processing (RMRP) endoribonuclease gene, which is involved in ribosomal assembly, telomere function, and cell cycle control...
  24. doi request reprint Humoral immune deficiency and hemifacial microsomia seen in one family
    Zamaneh Mikhak
    Harvard Medical School, Center for Immunology and Inflammatory Diseases, Massachusetts General Hospital, Boston, 02129, USA
    Cleft Palate Craniofac J 46:477-80. 2009
    ..The patient's 7-year-old sister also suffered from recurrent infections, had a left facial skin tag, and a left arachnoid cyst. We conclude that immune deficiency can occur in association with hemifacial microsomia...
  25. doi request reprint Correlation of pneumococcal antibody concentration and avidity with patient clinical and immunologic characteristics
    Ari J Fried
    Division of Immunology, Boston Children s Hospital, Boston, MA 02115, USA
    J Clin Immunol 33:847-56. 2013
    ..Here we studied correlations of avidities and concentrations of antibodies to pneumococcal polysaccharides with immunologic and clinical characteristics of patients with recurrent infections...
  26. pmc Mechanisms of tolerance to parental parathyroid tissue when combined with human allogeneic thymus transplantation
    Ivan K Chinn
    Department of Pediatrics, Division of Allergy and Immunology, Duke University Medical Center, Durham, NC 27710, USA
    J Allergy Clin Immunol 126:814-820.e8. 2010
    ..The induction of tolerance toward third-party solid organ grafts with allogeneic thymus tissue transplantation has not been previously demonstrated in human subjects...
  27. pmc IL-7 receptor deficient SCID with a unique intronic mutation and post-transplant autoimmunity due to chronic GVHD
    Manish J Butte
    Department of Pathology, Harvard Medical School, Boston, MA 02115, USA
    Clin Immunol 125:159-64. 2007
    ..Following hematopoietic stem cell transplantation of our patient, immune reconstitution was accompanied by two unusual complications, immune-mediated myositis and myasthenia gravis...
  28. pmc Deficient natural killer cell cytotoxicity in patients with IKK-gamma/NEMO mutations
    Jordan S Orange
    Division of Immunology, Children s Hospital and Department of Pediatrics, Harvard Medical School, Boston, Massachusetts 02115, USA
    J Clin Invest 109:1501-9. 2002
    ..These data suggest that NEMO participates in signaling pathways leading to NK cell cytotoxicity and that IL-2 can activate NF-kappaB and partially overcome the NK cell defect in patients with NEMO mutations...
  29. ncbi request reprint Management of an anaphylactoid reaction to methotrexate with a stepwise graded challenge
    Andrew J MacGinnitie
    Division of Allergy Immunology, Children s Hospital, Boston, MA 02446, USA
    Pediatr Allergy Immunol 14:409-11. 2003
    ..Because weekly methotrexate therapy is an important component of continuation chemotherapy for pediatric ALL, a stepwise graded challenge was employed to achieve drug tolerance...
  30. ncbi request reprint Are you immunodeficient?
    Francisco A Bonilla
    J Allergy Clin Immunol 116:423-5. 2005
  31. ncbi request reprint Characteristics of mycobacterial infection in patients with immunodeficiency and nuclear factor-kappaB essential modulator mutation, with or without ectodermal dysplasia
    Yang Shia Dai
    Department of Dermatology, National Taiwan University Hospital, Taipei, Taiwan
    J Am Acad Dermatol 51:718-22. 2004
    ..We describe clinical and histopathologic characteristics of 5 patients with nuclear factor kappaB essential modulator gene mutations and mycobacterial infections, two of whom had mycobacterial cutaneous infections...
  32. ncbi request reprint Carimune NF Liquid is a safe and effective immunoglobulin replacement therapy in patients with primary immunodeficiency diseases
    Melvin Berger
    Rainbow Babies and Children s Hospital, Division of Allergy Immunology, 111100 Euclid Avenue, Cleveland, Ohio 44106, USA
    J Clin Immunol 27:503-9. 2007
    ..Serum IgG trough levels were unchanged from baseline. Carimune NF Liquid, a ready-to-use, high-concentration, liquid immunoglobulin preparation is safe and effective...