Publications by authors named "Edward M Behrens"

77Publications

Activated CD8 T-cell Hepatitis in Children with Indeterminate Acute Liver Failure: Results from a Multicenter Cohort.

J Pediatr Gastroenterol Nutr 2020 Aug 10. Epub 2020 Aug 10.

Department of Pediatrics, Northwestern University, Feinberg School of Medicine, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago IL.

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http://dx.doi.org/10.1097/MPG.0000000000002893DOI Listing
August 2020

IFNs Drive Development of Novel IL-15-Responsive Macrophages.

J Immunol 2020 Aug 20;205(4):1113-1124. Epub 2020 Jul 20.

Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104;

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http://dx.doi.org/10.4049/jimmunol.2000184DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7415599PMC
August 2020

Vitamin C deficiency mimicking inflammatory bone disease of the hand.

Pediatr Rheumatol Online J 2020 Jun 9;18(1):45. Epub 2020 Jun 9.

Division of Rheumatology, The Children's Hospital of Philadelphia, 3401 Civic Center Blvd, Philadelphia, PA, 19104, USA.

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http://dx.doi.org/10.1186/s12969-020-00439-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7285567PMC
June 2020

Multisystem Inflammatory Syndrome in Children During the Coronavirus 2019 Pandemic: A Case Series.

J Pediatric Infect Dis Soc 2020 Jul;9(3):393-398

Division of Infectious Diseases, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.

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http://dx.doi.org/10.1093/jpids/piaa069DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7313950PMC
July 2020

A Spontaneous RAG1 Nonsense Mutation Unveils Naturally Occurring N-Terminal Truncated RAG1 Isoforms.

Immunohorizons 2020 03 6;4(3):119-128. Epub 2020 Mar 6.

Institute for Immunology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104;

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http://dx.doi.org/10.4049/immunohorizons.2000001DOI Listing
March 2020

Genetic Deficiency of Interferon-γ Reveals Interferon-γ-Independent Manifestations of Murine Hemophagocytic Lymphohistiocytosis.

Arthritis Rheumatol 2020 02 15;72(2):335-347. Epub 2019 Dec 15.

Perelman School of Medicine at the University of Pennsylvania and Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.

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http://dx.doi.org/10.1002/art.41076DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6994338PMC
February 2020

Altered X-chromosome inactivation in T cells may promote sex-biased autoimmune diseases.

JCI Insight 2019 04 4;4(7). Epub 2019 Apr 4.

Department of Biomedical Sciences, School of Veterinary Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.

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https://insight.jci.org/articles/view/126751
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http://dx.doi.org/10.1172/jci.insight.126751DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6483655PMC
April 2019

Myeloid Folliculin balances mTOR activation to maintain innate immunity homeostasis.

JCI Insight 2019 03 7;5. Epub 2019 Mar 7.

Department of Medicine, Cardiovascular Institute, Perelman School of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.

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http://dx.doi.org/10.1172/jci.insight.126939DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6483010PMC
March 2019

Microbiota-dependent signals are required to sustain TLR-mediated immune responses.

JCI Insight 2019 Jan 10;4(1). Epub 2019 Jan 10.

Division of Pediatric Rheumatology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.

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http://dx.doi.org/10.1172/jci.insight.124370DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6485364PMC
January 2019

Disruption of IL-33 Signaling Limits Early CD8+ T Cell Effector Function Leading to Exhaustion in Murine Hemophagocytic Lymphohistiocytosis.

Front Immunol 2018 20;9:2642. Epub 2018 Nov 20.

Division of Rheumatology, Children's Hospital of Philadelphia, Philadelphia, PA, United States.

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http://dx.doi.org/10.3389/fimmu.2018.02642DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6256032PMC
October 2019

Brief Report: Interferon-γ-Mediated Immunopathology Potentiated by Toll-Like Receptor 9 Activation in a Murine Model of Macrophage Activation Syndrome.

Arthritis Rheumatol 2019 01 24;71(1):161-168. Epub 2018 Nov 24.

The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.

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http://dx.doi.org/10.1002/art.40683DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6310087PMC
January 2019

CD8 T Cell Memory Increases Immunopathology in the Perforin-Deficient Model of Hemophagocytic Lymphohistiocytosis Secondary to TNF-α.

Immunohorizons 2018 Feb;2(2):67-73

Division of Rheumatology, Childen's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.

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http://dx.doi.org/10.4049/immunohorizons.1800003DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5961491PMC
February 2018

Muscularis macrophage development in the absence of an enteric nervous system.

Proc Natl Acad Sci U S A 2018 05 17;115(18):4696-4701. Epub 2018 Apr 17.

Abramson Research Center, The Children's Hospital of Philadelphia Research Institute, Philadelphia, PA 19104-4318;

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http://dx.doi.org/10.1073/pnas.1802490115DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5939112PMC
May 2018

Indeterminate pediatric acute liver failure is uniquely characterized by a CD103 CD8 T-cell infiltrate.

Hepatology 2018 09 23;68(3):1087-1100. Epub 2018 Jul 23.

Department of Pediatrics, Northwestern University, Feinberg School of Medicine, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL.

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http://dx.doi.org/10.1002/hep.29901DOI Listing
September 2018

ECSIT links TLR and BMP signaling in FOP connective tissue progenitor cells.

Bone 2018 04 27;109:201-209. Epub 2017 Dec 27.

Department of Orthopaedic Surgery, The Perelman School of Medicine of The University of Pennsylvania, Philadelphia, PA, United States; Department of Medicine, The Perelman School of Medicine of The University of Pennsylvania, Philadelphia, PA, United States; The Center for Research in FOP & Related Disorders, The Perelman School of Medicine of The University of Pennsylvania, Philadelphia, PA, United States. Electronic address:

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http://dx.doi.org/10.1016/j.bone.2017.12.024DOI Listing
April 2018

Weathering the storm: Improving therapeutic interventions for cytokine storm syndromes by targeting disease pathogenesis.

Curr Treatm Opt Rheumatol 2017 Mar 7;3(1):33-48. Epub 2017 Feb 7.

Division of Pediatric Rheumatology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.

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http://dx.doi.org/10.1007/s40674-017-0059-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5606329PMC
March 2017

Reply.

J Allergy Clin Immunol 2017 07 29;140(1):316-317. Epub 2017 Apr 29.

Children's Hospital of Philadelphia, Philadelphia, Pa.

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http://dx.doi.org/10.1016/j.jaci.2017.03.016DOI Listing
July 2017

Review: Cytokine Storm Syndrome: Looking Toward the Precision Medicine Era.

Arthritis Rheumatol 2017 06;69(6):1135-1143

Weill Cornell Medical College, New York, New York.

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http://dx.doi.org/10.1002/art.40071DOI Listing
June 2017

IL-10 distinguishes a unique population of activated, effector-like CD8 T cells in murine acute liver inflammation.

J Leukoc Biol 2017 04 29;101(4):1037-1044. Epub 2016 Dec 29.

Division of Rheumatology, Children's Hospital of Philadelphia, Philadelphia, PA, USA;

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http://doi.wiley.com/10.1189/jlb.3A0916-221RR
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http://dx.doi.org/10.1189/jlb.3A0916-221RRDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5346180PMC
April 2017

Pediatric acute liver failure of undetermined cause: A research workshop.

Hepatology 2017 03 6;65(3):1026-1037. Epub 2017 Jan 6.

Liver Diseases Research Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD.

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http://dx.doi.org/10.1002/hep.28944DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5372202PMC
March 2017

Editorial: Caught in the Act: Dissecting Natural Killer Cell Function in Systemic Juvenile Idiopathic Arthritis.

Authors:
Edward M Behrens

Arthritis Rheumatol 2017 01;69(1):6-8

Children's Hospital of Philadelphia, University of Pennsylvania Perelman School of Medicine, Philadelphia.

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http://dx.doi.org/10.1002/art.39934DOI Listing
January 2017

TLR9-mediated inflammation drives a Ccr2-independent peripheral monocytosis through enhanced extramedullary monocytopoiesis.

Proc Natl Acad Sci U S A 2016 09 12;113(39):10944-9. Epub 2016 Sep 12.

Division of Pediatric Rheumatology, Children's Hospital of Philadelphia, Philadelphia, PA 19104

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http://www.pnas.org/lookup/doi/10.1073/pnas.1524487113
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5047205PMC
http://dx.doi.org/10.1073/pnas.1524487113DOI Listing
September 2016

ST2 contributes to T-cell hyperactivation and fatal hemophagocytic lymphohistiocytosis in mice.

Blood 2016 Jan 30;127(4):426-35. Epub 2015 Oct 30.

Division of Rheumatology, The Children's Hospital of Philadelphia, Philadelphia, PA; Institute for Immunology, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA;

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http://www.bloodjournal.org/content/early/2015/10/30/blood-2
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http://www.bloodjournal.org/cgi/doi/10.1182/blood-2015-07-65
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http://dx.doi.org/10.1182/blood-2015-07-659813DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4731846PMC
January 2016

BIRC4 Mutation: An Important Rare Cause of Uveitis.

J Clin Rheumatol 2015 Dec;21(8):444-7

From the Division of Rheumatology, Children's Hospital of Philadelphia, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA.

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http://dx.doi.org/10.1097/RHU.0000000000000327DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4654974PMC
December 2015

Patient-reported Outcomes across Categories of Juvenile Idiopathic Arthritis.

J Rheumatol 2015 Oct 1;42(10):1914-21. Epub 2015 Sep 1.

From the Department of Pediatric Rheumatology, and Center for Pediatric Clinical Effectiveness, The Children's Hospital of Philadelphia; Center for Epidemiology and Biostatistics, The University of Pennsylvania, Philadelphia, Pennsylvania, USA.A.J. Taxter, MD, Fellow, Department of Pediatric Rheumatology, The Children's Hospital of Philadelphia; E.P. Wileyto, PhD, Associate Professor, Center for Epidemiology and Biostatistics, The University of Pennsylvania; E.M. Behrens, MD, Associate Professor, Department of Pediatric Rheumatology, The Children's Hospital of Philadelphia; P.F. Weiss, MD, MSCE, Associate Professor, Department of Pediatric Rheumatology, and Center for Pediatric Clinical Effectiveness, The Children's Hospital of Philadelphia.

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http://dx.doi.org/10.3899/jrheum.150092DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4592479PMC
October 2015

Does Viral Hemorrhagic Fever Represent Reactive Hemophagocytic Syndrome?

J Rheumatol 2015 Jul;42(7):1078-80

Department of Medicine, University of Alabama at Birmingham, Birmingham, Alabama, USA.

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http://dx.doi.org/10.3899/jrheum.150108DOI Listing
July 2015

Kill or be killed.

J Immunol 2015 Jun;194(11):5041-3

Division of Rheumatology, Department of Pediatrics, University of Alabama at Birmingham, Birmingham, AL 35233

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http://dx.doi.org/10.4049/jimmunol.1500774DOI Listing
June 2015

Toll-like receptor 9 and interferon-γ receptor signaling suppress the B-cell fate of uncommitted progenitors in mice.

Eur J Immunol 2015 May 26;45(5):1313-25. Epub 2015 Feb 26.

Division of Rheumatology, The Children's Hospital of Philadelphia, Philadelphia, PA, USA.

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http://doi.wiley.com/10.1002/eji.201445319
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http://dx.doi.org/10.1002/eji.201445319DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4913483PMC
May 2015

Genetic defects in cytolysis in macrophage activation syndrome.

Curr Rheumatol Rep 2014 ;16(9):439

University of Alabama at Birmingham, 1825 University Blvd., Shelby 306, Birmingham, AL, 35294, USA,

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http://dx.doi.org/10.1007/s11926-014-0439-2DOI Listing
February 2015

Hyperinflammation, rather than hemophagocytosis, is the common link between macrophage activation syndrome and hemophagocytic lymphohistiocytosis.

Curr Opin Rheumatol 2014 Sep;26(5):562-9

Division of Pediatric Rheumatology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.

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https://insights.ovid.com/crossref?an=00002281-201409000-000
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http://dx.doi.org/10.1097/BOR.0000000000000093DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4227502PMC
September 2014

Measuring cytotoxicity by bioluminescence imaging outperforms the standard chromium-51 release assay.

PLoS One 2014 19;9(2):e89357. Epub 2014 Feb 19.

Department of Veterinary & Animal Sciences, University of Massachusetts, Amherst, Massachusetts, United States of America.

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0089357PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3929704PMC
October 2014

The ζ isoform of diacylglycerol kinase plays a predominant role in regulatory T cell development and TCR-mediated ras signaling.

Sci Signal 2013 Nov 26;6(303):ra102. Epub 2013 Nov 26.

1Abramson Family Cancer Research Institute, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA.

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http://dx.doi.org/10.1126/scisignal.2004373DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4096120PMC
November 2013

Chronic non-bacterial osteomyelitis in a patient with common variable immunodeficiency.

Joint Bone Spine 2014 Mar 20;81(2):193-4. Epub 2013 Sep 20.

Division of Rheumatology, The Children's Hospital of Philadelphia, Philadelphia, USA; Division of Allergy and Immunology, The Children's Hospital of Philadelphia, 3615 Civic Center Boulevard, Abramson Research Center 1214, Philadelphia, PA 19104, USA.

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http://dx.doi.org/10.1016/j.jbspin.2013.08.005DOI Listing
March 2014

Spleen tyrosine kinase (Syk)-dependent calcium signals mediate efficient CpG-induced exocytosis of tumor necrosis factor α (TNFα) in innate immune cells.

J Biol Chem 2013 May 20;288(18):12448-58. Epub 2013 Mar 20.

Division of Pediatric Rheumatology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA.

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http://dx.doi.org/10.1074/jbc.M113.454405DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3642293PMC
May 2013

How to use... antinuclear antibodies in paediatric rheumatic diseases.

Arch Dis Child Educ Pract Ed 2013 Apr;98(2):64-70

Department of Pediatric Rheumatology, The Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA.

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http://dx.doi.org/10.1136/archdischild-2012-303326DOI Listing
April 2013

Myeloid dendritic cells from B6.NZM Sle1/Sle2/Sle3 lupus-prone mice express an IFN signature that precedes disease onset.

J Immunol 2012 Jul 1;189(1):80-91. Epub 2012 Jun 1.

Laboratory of Dendritic Cell Biology, Department of Microbiology and Immunology, Temple University School of Medicine, Philadelphia, PA 19140, USA.

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http://www.jimmunol.org/cgi/doi/10.4049/jimmunol.1101686
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http://dx.doi.org/10.4049/jimmunol.1101686DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3381850PMC
July 2012

Making sense of the cytokine storm: a conceptual framework for understanding, diagnosing, and treating hemophagocytic syndromes.

Pediatr Clin North Am 2012 Apr;59(2):329-44

Division of Rheumatology, The Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA.

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http://linkinghub.elsevier.com/retrieve/pii/S003139551200003
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http://dx.doi.org/10.1016/j.pcl.2012.03.002DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3368378PMC
April 2012

Not all hemophagocytes are created equally: appreciating the heterogeneity of the hemophagocytic syndromes.

Curr Opin Rheumatol 2012 Jan;24(1):113-8

The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA.

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http://dx.doi.org/10.1097/BOR.0b013e32834dd37eDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3285509PMC
January 2012

Enthesitis in an inception cohort of enthesitis-related arthritis.

Arthritis Care Res (Hoboken) 2011 Sep;63(9):1307-12

The Children's Hospital of Philadelphia, Pennsylvania, USA.

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http://doi.wiley.com/10.1002/acr.20508
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http://dx.doi.org/10.1002/acr.20508DOI Listing
September 2011

Repeated TLR9 stimulation results in macrophage activation syndrome-like disease in mice.

J Clin Invest 2011 Jun 16;121(6):2264-77. Epub 2011 May 16.

Division of Rheumatology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.

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http://dx.doi.org/10.1172/JCI43157DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3104738PMC
June 2011

Apoptotic cell-mediated immunoregulation of dendritic cells does not require iC3b opsonization.

J Immunol 2008 Sep;181(5):3018-26

Laboratory of Dendritic Cell Biology, Division of Rheumatology, Joseph Stokes, Jr. Research Institute, Children's Hospital of Philadelphia, Philadelphia, PA 19104-4318, USA.

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http://dx.doi.org/10.4049/jimmunol.181.5.3018DOI Listing
September 2008

Macrophage activation syndrome in rheumatic disease: what is the role of the antigen presenting cell?

Authors:
Edward M Behrens

Autoimmun Rev 2008 Feb 4;7(4):305-8. Epub 2008 Jan 4.

Department of Pediatrics, Division of Rheumatology, Children's Hospital of Philadelphia and University of Pennsylvania School of Medicine, Philadelphia, PA 19104-4318, USA.

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http://linkinghub.elsevier.com/retrieve/pii/S156899720700193
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http://dx.doi.org/10.1016/j.autrev.2007.11.025DOI Listing
February 2008

Evaluation of the presentation of systemic onset juvenile rheumatoid arthritis: data from the Pennsylvania Systemic Onset Juvenile Arthritis Registry (PASOJAR).

J Rheumatol 2008 Feb 15;35(2):343-8. Epub 2007 Dec 15.

Department of Pediatrics, Division of Rheumatology, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104-4318, USA.

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February 2008

IL-4 suppresses dendritic cell response to type I interferons.

J Immunol 2007 Nov;179(10):6446-55

Laboratory of Dendritic Cell Biology, Joseph Stokes Jr. Research Institute, Division of Rheumatology, Department of Pediatrics, University of Pennsylvania School of Medicine, The Children's Hospital of Philadelphia, Philadelphia, PA 19104, USA.

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http://dx.doi.org/10.4049/jimmunol.179.10.6446DOI Listing
November 2007

Complement receptor 3 ligation of dendritic cells suppresses their stimulatory capacity.

J Immunol 2007 May;178(10):6268-79

Laboratory of Dendritic Cell Biology, Joseph Stokes, Jr. Research Institute, Children's Hospital of Philadelphia, and Department of Pediatrics, University of Pennsylvania School of Medicine, Philadelphia, PA 19104-4318, USA.

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http://www.jimmunol.org/content/178/10/6268.full.pdf
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http://dx.doi.org/10.4049/jimmunol.178.10.6268DOI Listing
May 2007

Occult macrophage activation syndrome in patients with systemic juvenile idiopathic arthritis.

J Rheumatol 2007 May 1;34(5):1133-8. Epub 2007 Mar 1.

Departments of Pathology and Pediatrics, Division of Rheumatology, Children's Hospital of Philadelphia, and University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104-4318, USA.

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http://www.jrheum.org/content/34/5/1133.full.pdf
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May 2007

Juvenile idiopathic arthritis classification criteria: loopholes and diagnosis software.

J Rheumatol 2007 Jan;34(1):234; author reply 234-5

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January 2007

Interleukin 1 receptor antagonist to treat cytophagic histiocytic panniculitis with secondary hemophagocytic lymphohistiocytosis.

J Rheumatol 2006 Oct;33(10):2081-4

Department of Pathology and Pediatrics, Division of Rheumatology, Children's Hospital of Philadelphia and University of Pennsylvania School of Medicine, Philadelphia, PA 19104-4318, USA.

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October 2006

The mer receptor tyrosine kinase: expression and function suggest a role in innate immunity.

Eur J Immunol 2003 Aug;33(8):2160-7

University of Pennsylvania, Philadelphia, USA.

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http://dx.doi.org/10.1002/eji.200324076DOI Listing
August 2003