(Q50113904)

25 February 2018

title

Convergent pathways of the hyperferritinemic syndromes. (English)

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25 February 2018

author name string

Grant S Schulert

series ordinal

1

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25 February 2018

Scott W Canna

series ordinal

2

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25 February 2018

publication date

6 February 2018

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25 February 2018

published in

International Immunology

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25 February 2018

Evidence for intravascular coagulation in systemic onset, but not polyarticular, juvenile rheumatoid arthritis

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Acute hemorrhagic, hepatic, and neurologic manifestations in juvenile rheumatoid arthritis: possible relationship to drugs or infection

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12 December 2020

Consumption coagulopathy associated with systemic juvenile rheumatoid arthritis

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Macrophage activation syndrome and rheumatic disease in childhood: a report of four new cases

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Macrophage activation syndrome in systemic juvenile rheumatoid arthritis

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Induction and exhaustion of lymphocytic choriomeningitis virus-specific cytotoxic T lymphocytes visualized using soluble tetrameric major histocompatibility complex class I-peptide complexes.

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Aplastic anemia rescued by exhaustion of cytokine-secreting CD8+ T cells in persistent infection with lymphocytic choriomeningitis virus

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Defective natural killer cell function in patients with hemophagocytic lymphohistiocytosis and in first degree relatives

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12 December 2020

Perforin gene defects in familial hemophagocytic lymphohistiocytosis.

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12 December 2020

Regulation of antigen-specific CD8+ T cell homeostasis by perforin and interferon-gamma.

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12 December 2020

NK cell functions restrain T cell responses during viral infections

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12 December 2020

Macrophage activation syndrome is hemophagocytic lymphohistiocytosis--need for the right terminology

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12 December 2020

Natural killer cell dysfunction in patients with systemic-onset juvenile rheumatoid arthritis and macrophage activation syndrome

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12 December 2020

Increased serum levels of interferon-gamma-inducible protein 10 and monokine induced by gamma interferon in patients with haemophagocytic lymphohistiocytosis

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12 December 2020

Familial haemophagocytic reticulosis

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12 December 2020

An animal model of hemophagocytic lymphohistiocytosis (HLH): CD8+ T cells and interferon gamma are essential for the disorder

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12 December 2020

International pediatric sepsis consensus conference: definitions for sepsis and organ dysfunction in pediatrics

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12 December 2020

Natural killer cell dysfunction is a distinguishing feature of systemic onset juvenile rheumatoid arthritis and macrophage activation syndrome

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12 December 2020

A single amino acid change, A91V, leads to conformational changes that can impair processing to the active form of perforin

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12 December 2020

HLH-2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis.

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12 December 2020

The diagnostic significance of soluble CD163 and soluble interleukin-2 receptor alpha-chain in macrophage activation syndrome and untreated new-onset systemic juvenile idiopathic arthritis

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12 December 2020

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

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12 December 2020

Jinx, an MCMV susceptibility phenotype caused by disruption of Unc13d: a mouse model of type 3 familial hemophagocytic lymphohistiocytosis

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12 December 2020

Ferritin levels in children with severe sepsis and septic shock

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12 December 2020

Macrophage activation syndrome in patients with systemic juvenile idiopathic arthritis is associated with MUNC13-4 polymorphisms

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12 December 2020

S100A12 is a novel molecular marker differentiating systemic-onset juvenile idiopathic arthritis from other causes of fever of unknown origin

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12 December 2020

Secondary hemophagocytic lymphohistiocytosis and severe sepsis/ systemic inflammatory response syndrome/multiorgan dysfunction syndrome/macrophage activation syndrome share common intermediate phenotypes on a spectrum of inflammation

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12 December 2020

Temperature sensitivity of human perforin mutants unmasks subtotal loss of cytotoxicity, delayed FHL, and a predisposition to cancer

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12 December 2020

Defective phosphorylation of interleukin-18 receptor beta causes impaired natural killer cell function in systemic-onset juvenile idiopathic arthritis.

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12 December 2020

Mutations in the perforin gene can be linked to macrophage activation syndrome in patients with systemic onset juvenile idiopathic arthritis.

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12 December 2020

Neutralization of IFNgamma defeats haemophagocytosis in LCMV-infected perforin- and Rab27a-deficient mice

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12 December 2020

Distinct cytokine profiles of systemic-onset juvenile idiopathic arthritis-associated macrophage activation syndrome with particular emphasis on the role of interleukin-18 in its pathogenesis

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12 December 2020

XIAP deficiency: a unique primary immunodeficiency best classified as X-linked familial hemophagocytic lymphohistiocytosis and not as X-linked lymphoproliferative disease

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12 December 2020

Inherited defects in lymphocyte cytotoxic activity

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12 December 2020

A multicentre, randomised, double-blind, placebo-controlled trial with the interleukin-1 receptor antagonist anakinra in patients with systemic-onset juvenile idiopathic arthritis (ANAJIS trial)

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12 December 2020

Anakinra as first-line disease-modifying therapy in systemic juvenile idiopathic arthritis: report of forty-six patients from an international multicenter series

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12 December 2020

Disruption of MyD88 signaling suppresses hemophagocytic lymphohistiocytosis in mice

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12 December 2020

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

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12 December 2020

Perforin is a critical physiologic regulator of T-cell activation

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12 December 2020

Hemophagocytosis causes a consumptive anemia of inflammation

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12 December 2020

Hypomorphic mutations in PRF1, MUNC13-4, and STXBP2 are associated with adult-onset familial HLH.

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12 December 2020

Amplification of the response to Toll-like receptor ligands by prolonged exposure to interleukin-6 in mice: implication for the pathogenesis of macrophage activation syndrome.

1 reference

12 December 2020

The Toll-like receptor 4 agonist MRP8/14 protein complex is a sensitive indicator for disease activity and predicts relapses in systemic-onset juvenile idiopathic arthritis.

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12 December 2020

A prospective evaluation of degranulation assays in the rapid diagnosis of familial hemophagocytic syndromes

1 reference

12 December 2020

Macrophage activation syndrome as part of systemic juvenile idiopathic arthritis: diagnosis, genetics, pathophysiology and treatment.

1 reference

12 December 2020

Hemophagocytic lymphohistiocytosis in syntaxin-11-deficient mice: T-cell exhaustion limits fatal disease

1 reference

12 December 2020

Two randomized trials of canakinumab in systemic juvenile idiopathic arthritis

1 reference

12 December 2020

Distinct subsets of patients with systemic juvenile idiopathic arthritis based on their cytokine profiles

1 reference

12 December 2020

The risk of hemophagocytic lymphohistiocytosis in Hermansky-Pudlak syndrome type 2

1 reference

12 December 2020

Chimeric antigen receptor-modified T cells for acute lymphoid leukemia

1 reference

12 December 2020

Interferon-γ mediates anemia but is dispensable for fulminant toll-like receptor 9-induced macrophage activation syndrome and hemophagocytosis in mice

1 reference

12 December 2020

Reaching the threshold: a multilayer pathogenesis of macrophage activation syndrome

1 reference

12 December 2020

Hemophagocytic lymphohistiocytosis: advances in pathophysiology, diagnosis, and treatment

1 reference

12 December 2020

X-linked inhibitor of apoptosis (XIAP) deficiency: the spectrum of presenting manifestations beyond hemophagocytic lymphohistiocytosis.

1 reference

12 December 2020

Sustained elevation of serum interleukin-18 and its association with hemophagocytic lymphohistiocytosis in XIAP deficiency

1 reference

12 December 2020

Interleukin-18, more than a Th1 cytokine

1 reference

12 December 2020

Human natural killer cells prevent infectious mononucleosis features by targeting lytic Epstein-Barr virus infection

1 reference

12 December 2020

Graded defects in cytotoxicity determine severity of hemophagocytic lymphohistiocytosis in humans and mice

1 reference

12 December 2020

Hemophagocytic lymphohistiocytosis: review of etiologies and management

1 reference

12 December 2020

Whole-exome sequencing reveals overlap between macrophage activation syndrome in systemic juvenile idiopathic arthritis and familial hemophagocytic lymphohistiocytosis

1 reference

12 December 2020

Genetic defects in cytolysis in macrophage activation syndrome.

1 reference

12 December 2020

An activating NLRC4 inflammasome mutation causes autoinflammation with recurrent macrophage activation syndrome

1 reference

12 December 2020

Mutation of NLRC4 causes a syndrome of enterocolitis and autoinflammation

1 reference

12 December 2020

An inherited mutation in NLRC4 causes autoinflammation in human and mice

1 reference

12 December 2020

Hemophagocytic lymphohistiocytosis (HLH): A heterogeneous spectrum of cytokine-driven immune disorders.

1 reference

12 December 2020

11-Month-Old Infant With Periodic Fevers, Recurrent Liver Dysfunction, and Perforin Gene Polymorphism

1 reference

12 December 2020

A novel immunoregulatory role for NK-cell cytotoxicity in protection from HLH-like immunopathology in mice.

1 reference

12 December 2020

Hemophagocytic lymphohistiocytosis caused by dominant-negative mutations in STXBP2 that inhibit SNARE-mediated membrane fusion

1 reference

12 December 2020

Hemophagocytic lymphohistiocytosis in 2 patients with underlying IFN-γ receptor deficiency.

1 reference

12 December 2020

Molecular mechanisms in genetically defined autoinflammatory diseases: disorders of amplified danger signaling

1 reference

12 December 2020

Failed CTL/NK cell killing and cytokine hypersecretion are directly linked through prolonged synapse time.

1 reference

12 December 2020

Heterozygosity for the common perforin mutation, p.A91V, impairs the cytotoxicity of primary natural killer cells from healthy individuals

1 reference

12 December 2020

Macrophage activation syndrome in the course of monogenic autoinflammatory disorders.

1 reference

12 December 2020

Interleukin-18 for predicting the development of macrophage activation syndrome in systemic juvenile idiopathic arthritis

1 reference

12 December 2020

Inhibition of Natural Killer Cell Cytotoxicity by Interleukin-6: Implications for the Pathogenesis of Macrophage Activation Syndrome

1 reference

12 December 2020

2016 Classification Criteria for Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis: A European League Against Rheumatism/American College of Rheumatology/Paediatric Rheumatology International Trials Organisation Coll

1 reference

12 December 2020

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

1 reference

12 December 2020

Inflammasomes Coordinate Pyroptosis and Natural Killer Cell Cytotoxicity to Clear Infection by a Ubiquitous Environmental Bacterium

1 reference

12 December 2020

Screening assays for primary haemophagocytic lymphohistiocytosis in children presenting with suspected macrophage activation syndrome

1 reference

12 December 2020

Interleukin-1 Receptor Blockade Is Associated With Reduced Mortality in Sepsis Patients With Features of Macrophage Activation Syndrome: Reanalysis of a Prior Phase III Trial

1 reference

12 December 2020

Whole-Exome Sequencing Reveals Mutations in Genes Linked to Hemophagocytic Lymphohistiocytosis and Macrophage Activation Syndrome in Fatal Cases of H1N1 Influenza

1 reference

12 December 2020

Recurrent macrophage activation syndrome in spondyloarthritis and monoallelic missense mutations in PRF1: a description of one paediatric case.

1 reference

12 December 2020

Polygenic mutations in the cytotoxicity pathway increase susceptibility to develop HLH immunopathology in mice

1 reference

12 December 2020

A Heterozygous RAB27A Mutation Associated with Delayed Cytolytic Granule Polarization and Hemophagocytic Lymphohistiocytosis.

1 reference

12 December 2020

Mouse Cytomegalovirus Infection in BALB/c Mice Resembles Virus-Associated Secondary Hemophagocytic Lymphohistiocytosis and Shows a Pathogenesis Distinct from Primary Hemophagocytic Lymphohistiocytosis

1 reference

12 December 2020

Erythematous nodes, urticarial rash and arthralgias in a large pedigree withNLRC4-related autoinflammatory disease, expansion of the phenotype

1 reference

12 December 2020

Inflammasomes: mechanism of assembly, regulation and signalling

1 reference

12 December 2020

Elevated circulating levels of interferon-γ and interferon-γ-induced chemokines characterise patients with macrophage activation syndrome complicating systemic juvenile idiopathic arthritis.

1 reference

12 December 2020

Development of spondyloarthropathy following episodes of macrophage activation syndrome in children with heterozygous mutations in haemophagocytic lymphohistiocytosis-associated genes

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12 December 2020

Analysis of protein-coding genetic variation in 60,706 humans

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12 December 2020

Elevated serum levels of free interleukin-18 in adult-onset Still's disease.

1 reference

12 December 2020

Inflammatory Gene Expression Profile and Defective Interferon-γ and Granzyme K in Natural Killer Cells From Systemic Juvenile Idiopathic Arthritis Patients

1 reference

12 December 2020

Identification of a High-Frequency Somatic NLRC4 Mutation as a Cause of Autoinflammation by Pluripotent Cell-Based Phenotype Dissection.

1 reference

12 December 2020

Life-threatening NLRC4-associated hyperinflammation successfully treated with IL-18 inhibition.

1 reference

12 December 2020

Novel NLRC4 Mutation Causes a Syndrome of Perinatal Autoinflammation With Hemophagocytic Lymphohistiocytosis, Hepatosplenomegaly, Fetal Thrombotic Vasculopathy, and Congenital Anemia and Ascites.

1 reference

12 December 2020

Three Hypothetical Inflammation Pathobiology Phenotypes and Pediatric Sepsis-Induced Multiple Organ Failure Outcome

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12 December 2020

Biologic therapy modifies clinical and laboratory features of macrophage activation syndrome associated with systemic juvenile idiopathic arthritis.

1 reference

12 December 2020

Hyperferritinemia and inflammation

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12 December 2020

Neutralization of IFN-γ reverts clinical and laboratory features in a mouse model of macrophage activation syndrome.

1 reference

12 December 2020

Macrophage activation-like syndrome: an immunological entity associated with rapid progression to death in sepsis

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12 December 2020

Unopposed IL-18 signaling leads to severe TLR9-induced macrophage activation syndrome in mice

1 reference

12 December 2020

Interleukin-18 diagnostically distinguishes and pathogenically promotes human and murine macrophage activation syndrome.

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12 December 2020

Identifiers

DOI

10.1093/INTIMM/DXY012

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25 February 2018

PubMed ID

29420734

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25 February 2018