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Species | Rebecca H BuckleySummaryAffiliation: Duke University Medical Center Country: USA Publications
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Publications
Epstein-Barr-associated leiomyomatosis and T-cell chimerism after haploidentical bone marrow transplantation for severe combined immunodeficiency diseaseSrilatha Atluri
Department of Medicine, Indiana University School of Medicine, Indianapolis, IN 46202, USA
J Pediatr Hematol Oncol 29:166-72. 2007..The clinical course of Epstein-Barr virus (EBV)-associated smooth muscle tumors is variable and there are no reports in patients with mixed T-cell chimerism after bone marrow transplantation (BMT)...
Post-Transplantation B Cell Function in Different Molecular Types of SCIDRebecca H Buckley
Department of Pediatrics, Duke University Medical Center, Box 2898, 363 Jones Building, Durham, NC, 27710, USA
J Clin Immunol 33:96-110. 2013..However, the development of B cell function has been more problematic. We report here results of analyses of B cell function in 125 SCID recipients prior to and long-term after non-ablative BMT, according to their molecular type...- The long quest for neonatal screening for severe combined immunodeficiencyRebecca H Buckley
Departments of Pediatrics and Immunology, Duke University Medical Center, Durham, NC 27710, USA
J Allergy Clin Immunol 129:597-604; quiz 605-6. 2012..Even more important will be their roles in establishing accurate diagnoses for infants with positive screen results and in ensuring that they are given the best possible treatment... - Molecular defects in human severe combined immunodeficiency and approaches to immune reconstitutionRebecca H Buckley
Department of Pediatrics, Duke University Medical Center, Durham, North Carolina 27710, USA
Annu Rev Immunol 22:625-55. 2004..Gene therapy was highly successful in nine infants with X-linked SCID, but the trials have been placed on hold due to the development of a leukemic process in two of the children because of insertional oncogenesis... - B-cell function in severe combined immunodeficiency after stem cell or gene therapy: a reviewRebecca H Buckley
Departments of Pediatrics and Immunology, Duke University Medical Center, Durham, NC, USA
J Allergy Clin Immunol 125:790-7. 2010.... - Treatment options for genetically determined immunodeficiencyRebecca H Buckley
Duke University Medical Center, Durham, NC 27710, USA
Lancet 361:541-2. 2003 - The multiple causes of human SCIDRebecca H Buckley
Department of Pediatrics, Duke University Medical Center, Durham, North Carolina, USA
J Clin Invest 114:1409-11. 2004..In this issue of the JCI, a report describes how complete deficiency of the CD3epsilon chain of the T cell antigen receptor/CD3 complex causes human SCID... 
Transplantation of hematopoietic stem cells in human severe combined immunodeficiency: longterm outcomesRebecca H Buckley
Departments of Pediatrics and Immunology, Duke University Medical Center, Box 2898 or 363 Jones Building, Durham, NC 27710, USA
Immunol Res 49:25-43. 2011..3 years and reviews published reports of longterm outcomes of transplants in SCID performed at other centers...- Gene therapy for SCID--a complication after remarkable progressRebecca H Buckley
Duke University Medical Center, Durham, NC, USA
Lancet 360:1185-6. 2002 - Primary immunodeficiency diseases: dissectors of the immune systemRebecca H Buckley
Duke University School of Medicine, Durham, NC 27710, USA
Immunol Rev 185:206-19. 2002..Finally, the past 3 years have witnessed the first truly successful gene therapy. The impressive results in X-linked severe combined immunodeficiency offer hope that this approach can be extended to many more diseases in the future... - Immunoglobulin G subclass deficiency: fact or fancy?Rebecca H Buckley
Pediatrics Allergy Immunology, Duke University School of Medicine, Box 2898, Durham, NC 27710, USA
Curr Allergy Asthma Rep 2:356-60. 2002..Such assays provide no information about the patient's capacity to produce specific antibodies to protein, polysaccharide, or viral antigens... - Variable phenotypic expression of mutations in genes of the immune systemRebecca H Buckley
Department of Pediatrics, Duke University Medical Center, Durham, North Carolina 27710, USA
J Clin Invest 115:2974-6. 2005..describe a third phenotype for mutations in recombination activating gene 1 (RAG1), in addition to the already known phenotypes of SCID and Omenn syndrome (see the related article beginning on page 3291)... - Primary immunodeficiency or not? Making the correct diagnosisRebecca H Buckley
Duke University School of Medicine, Durham, NC 27710, USA
J Allergy Clin Immunol 117:756-8. 2006..Had the physicians chosen tests of immune function rather than relying on immunoglobulin levels or cell counts, they would have arrived at the true diagnoses... - Advances in the understanding and treatment of human severe combined immunodeficiencyR H Buckley
Department of Pediatrics, Duke University Medical Center, Durham, NC 27710, USA
Immunol Res 22:237-51. 2000..5 mo of life, making early diagnosis crucial. Recently, gene therapy has also been successful in infants with X-linked SCID... - Pulmonary complications of primary immunodeficienciesRebecca H Buckley
Departments of Pediatrics and Immunology, Duke University Medical Center, Durham, NC 27710, USA
Paediatr Respir Rev 5:S225-33. 2004..Finally, advances in treatment of the underlying diseases as well as their infectious complications will be covered... - Thymic output, T-cell diversity, and T-cell function in long-term human SCID chimerasMARCELLA SARZOTTI-KELSOE
Departments of Immunology, Duke University Medical Center, Durham, NC 27710, USA
Blood 114:1445-53. 2009.... - T-B+NK+ severe combined immunodeficiency caused by complete deficiency of the CD3zeta subunit of the T-cell antigen receptor complexJoseph L Roberts
Department of Pediatrics and Immunology, Duke University Medical Center, Durham, NC 27710, USA
Blood 109:3198-206. 2007..Taken together, these findings provide the first demonstration that complete CD3zeta deficiency in humans can cause SCID by preventing normal TCR assembly and surface expression... - Unusual clinical and immunologic manifestations of transplacentally acquired maternal T cells in severe combined immunodeficiencyKricia Palmer
Division of Pediatric Allergy and Immunology, Duke University Medical Center, Durham, NC 27710, USA
J Allergy Clin Immunol 120:423-8. 2007..We present evidence that these cells can cause allograft rejection and immune cytopenias... - T cell repertoire development in humans with SCID after nonablative allogeneic marrow transplantationMarcella Sarzotti
Department of Immunology, Medicine, and Pediatrics, Duke University Medical Center, Durham, NC 27710, USA
J Immunol 170:2711-8. 2003..The TCR diversity positively correlates in these patients with TREC levels... - Why newborn screening for severe combined immunodeficiency is essential: a case reportMehdi M Adeli
Division of Pediatric Allergy and Immunology, Department of Pediatrics, Duke University Medical Center, Durham, North Carolina, USA
Pediatrics 126:e465-9. 2010..Recognition of the characteristic lymphopenia of SCID can facilitate early diagnosis... - Complete DiGeorge syndrome: development of rash, lymphadenopathy, and oligoclonal T cells in 5 casesM Louise Markert
Department of Pediatrics, Duke University Medical Center, Durham, NC 27710, USA
J Allergy Clin Immunol 113:734-41. 2004..Prompt diagnosis is necessary for appropriate management... - Hematopoietic stem cell transplantation for severe combined immunodeficiency in the neonatal period leads to superior thymic output and improved survivalLaurie A Myers
Duke University Medical Center, Durham, NC 27710, USA
Blood 99:872-8. 2002..An improved outcome for this otherwise fatal syndrome could be achieved with newborn screening for lymphopenia so that transplantation could be performed under favorable thymopoietic conditions... - Primary cellular immunodeficienciesRebecca H Buckley
Division of Allergy and Immunology, Department of Pediatrics, Duke University Medical Center, Durham, NC 27710, USA
J Allergy Clin Immunol 109:747-57. 2002..Fully defining the molecular defects of such patients is also essential for genetic counseling of family members and prenatal diagnosis... - Janus kinase 3 (JAK3) deficiency: clinical, immunologic, and molecular analyses of 10 patients and outcomes of stem cell transplantationJoseph L Roberts
Department of Pediatrics, Duke University Medical Center, Durham, NC 27710, USA
Blood 103:2009-18. 2004..Hence, bone marrow transplantation is an effective means for reconstitution of T-cell immunity in this defect but is less successful for restoration of B-cell and NK cell functions... - Long-term clinical outcome of patients with severe combined immunodeficiency who received related donor bone marrow transplants without pretransplant chemotherapy or post-transplant GVHD prophylaxisMary Dell Railey
Department of Pediatrics, Duke University Medical Center, Durham, NC 27710, USA
J Pediatr 155:834-840.e1. 2009..Only 16 (10%) had HLA-identical donors... - Transplantation immunology: solid organ and bone marrowJavier Chinen
Department of Pediatrics, Allergy Immunology, Baylor College of Medicine, Houston, USA
J Allergy Clin Immunol 125:S324-35. 2010.... - The effect of natural killer cell killer Ig-like receptor alloreactivity on the outcome of bone marrow stem cell transplantation for severe combined immunodeficiency (SCID)M D Keller
Division of Pediatric Allergy and Immunology, Duke University Medical Center, Durham, North Carolina, USA
J Clin Immunol 27:109-16. 2007..This study suggests that inhibitory KIR/HLA interactions do not play a significant role in bone marrow transplantation for SCID... - Abnormal development of thymic dendritic and epithelial cells in human X-linked severe combined immunodeficiencyLaura P Hale
Department of Pathology, Duke University Medical Center, Durham, NC 27710, USA
Clin Immunol 110:63-70. 2004..These histopathologic findings indicate that in addition to T cells, thymic DC development and differentiation of TE cells are also abnormal in X-SCID... - The long and the short of telomeres in bone marrow recipient SCID patientsMARCELLA SARZOTTI-KELSOE
Department of Immunology, Duke University Medical Center, Durham, NC 27710, USA
Immunol Res 49:44-8. 2011..Our study of seven SCID patients, following successful bone marrow transplantation, demonstrates that the patients' peripheral T cells did not exhibit greater than normal telomere shortening... - American Pediatric Society Presidential Address 2000: reflections on the 20th and 21st centuriesRebecca H Buckley
Duke University Medical Center, Durham, North Carolina 27710, USA
Pediatr Res 51:119-23. 2002 - Transplantation of thymus tissue in complete DiGeorge syndromeM L Markert
Department of Pediatrics, Duke Comprehensive Cancer Center, Duke University Medical Center, Durham, NC 27710, USA
N Engl J Med 341:1180-9. 1999..The DiGeorge syndrome is a congenital disorder that affects the heart, parathyroid glands, and thymus. In complete DiGeorge syndrome, patients have severely reduced T-cell function... - Successful formation of a chimeric human thymus allograft following transplantation of cultured postnatal human thymusM L Markert
Department of Pediatrics, Duke University Medical Center, Durham, NC 27710, USA
J Immunol 158:998-1005. 1997.... - Thymic function after hematopoietic stem-cell transplantation for the treatment of severe combined immunodeficiencyD D Patel
Department of Medicine, Human Vaccine Institute, Duke University Medical Center, Durham, NC 27710, USA
N Engl J Med 342:1325-32. 2000..The role of the thymus in this process is unknown... - CD45-deficient severe combined immunodeficiency caused by uniparental disomyJoseph L Roberts
Department of Pediatrics, Duke University Medical Center, Durham, NC 27710, USA
Proc Natl Acad Sci U S A 109:10456-61. 2012..Evaluation for alterations in other genes affected by UPD should also be considered in such cases... - A historical review of bone marrow transplantation for immunodeficienciesRebecca H Buckley
Departments of Pediatrics and Immunology, Duke University School of Medicine, Durham, NC 27710, USA
J Allergy Clin Immunol 113:793-800. 2004 - 27. Transplantation immunology: organ and bone marrowRebecca H Buckley
Department of Pediatrics, Allergy/Immunology, Duke University Medical Center, 362 Jones Building (Campus Box 2898, Durham, NC 27710-0001, USA
J Allergy Clin Immunol 111:S733-44. 2003..The major obstacle to the performance of solid organ transplantation currently is the shortage of donor organs... - 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 trialChaim 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... - Jak3 and the pathogenesis of severe combined immunodeficiencyMatthew Husa
Molecular Immunology and Inflammation Branch, National Institute of Arthritis and Musculoskeletal and Skin Diseases, MSC 1820, 10 Center Drive, Bethesda, MD 20892, USA
Mol Immunol 41:727-37. 2004..Further study of Jak3 will hopefully provide insights into the clinical treatment of patients suffering from immune-mediated diseases... - A novel mutation in IFN-gamma receptor 2 with dominant negative activity: biological consequences of homozygous and heterozygous statesSergio D Rosenzweig
Laboratory of Host Defenses, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892, USA
J Immunol 173:4000-8. 2004..The mutant construct 791delG exerts dominant negative effects on IFN-gamma signaling without cell surface display, suggesting that it is acting on pathways other than those involved in cell surface recognition of ligand...