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  1. Article ; Online: Precision Medicine in the Treatment of Primary Immune Deficiency Patients With Disorders of Immune Dysregulation.

    Ballow, Mark / Leiding, Jennifer W

    Clinical reviews in allergy & immunology

    2021  Volume 63, Issue 1, Page(s) 1–8

    Abstract: There are now more than 450 described monogenic germline mutations for inborn errors of immunity that result in the loss of expression, loss of function (LOF), or gain in function (GOF) of the encoded protein. Molecular characterization of these inborn ... ...

    Abstract There are now more than 450 described monogenic germline mutations for inborn errors of immunity that result in the loss of expression, loss of function (LOF), or gain in function (GOF) of the encoded protein. Molecular characterization of these inborn errors of immunity has not only allowed us to characterize on a genetic basis these immune deficiency disorders but has provided a better understanding of the immunobiology of these inborn errors of immunity. More recently, these advances have allowed us to apply targeted therapy or precision medicine in their treatment. Of particular interest related to this review are those inborn errors of immunity that result in gain-of-function (GOF) genetic abnormalities. Many of these inborn errors of immunity fall into a new category referred to as diseases of immune dysregulation in which many of the patients not only exhibit an increased susceptibility to infection but also have a clinical phenotype associated with autoimmune processes and lymphoproliferative disease.
    MeSH term(s) Humans ; Immunologic Deficiency Syndromes/drug therapy ; Immunologic Deficiency Syndromes/therapy ; Phenotype ; Precision Medicine ; Primary Immunodeficiency Diseases/genetics ; Primary Immunodeficiency Diseases/therapy
    Language English
    Publishing date 2021-06-24
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 1239045-8
    ISSN 1559-0267 ; 1080-0549
    ISSN (online) 1559-0267
    ISSN 1080-0549
    DOI 10.1007/s12016-021-08871-4
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    In stock of ZB MED Cologne/Königswinter

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  2. Article ; Online: Redefining Precision Medicine in Disorders of Immune Dysregulation.

    Leiding, Jennifer W / Ballow, Mark

    The journal of allergy and clinical immunology. In practice

    2020  Volume 7, Issue 8, Page(s) 2801–2803

    MeSH term(s) Abatacept ; Precision Medicine
    Chemical Substances Abatacept (7D0YB67S97)
    Language English
    Publishing date 2020-02-10
    Publishing country United States
    Document type Editorial ; Comment
    ZDB-ID 2843237-X
    ISSN 2213-2201 ; 2213-2198
    ISSN (online) 2213-2201
    ISSN 2213-2198
    DOI 10.1016/j.jaip.2019.07.026
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    In stock of ZB MED Cologne/Königswinter

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  3. Article: Neutrophil Evolution and Their Diseases in Humans.

    Leiding, Jennifer W

    Frontiers in immunology

    2017  Volume 8, Page(s) 1009

    Abstract: Granulocytes have been preserved and have evolved across species, developing into cells that provide one of the first lines of host defense against pathogens. In humans, neutrophils are involved in early recognition and killing of infectious pathogens. ... ...

    Abstract Granulocytes have been preserved and have evolved across species, developing into cells that provide one of the first lines of host defense against pathogens. In humans, neutrophils are involved in early recognition and killing of infectious pathogens. Disruption in neutrophil production, emigration, chemotaxis, and function cause a spectrum of primary immune defects characterized by host susceptibility to invasive infections.
    Language English
    Publishing date 2017
    Publishing country Switzerland
    Document type Journal Article ; Review
    ZDB-ID 2606827-8
    ISSN 1664-3224
    ISSN 1664-3224
    DOI 10.3389/fimmu.2017.01009
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  4. Article: STAT3 gain-of-function syndrome.

    Vogel, Tiphanie P / Leiding, Jennifer W / Cooper, Megan A / Forbes Satter, Lisa R

    Frontiers in pediatrics

    2023  Volume 10, Page(s) 770077

    Abstract: STAT3 gain-of-function (GOF) syndrome is a multi-organ primary immune regulatory disorder characterized by early onset autoimmunity. Patients present early in life, most commonly with lymphoproliferation, autoimmune cytopenias, and growth delay. However, ...

    Abstract STAT3 gain-of-function (GOF) syndrome is a multi-organ primary immune regulatory disorder characterized by early onset autoimmunity. Patients present early in life, most commonly with lymphoproliferation, autoimmune cytopenias, and growth delay. However, disease is often progressive and can encompass a wide range of clinical manifestations such as: enteropathy, skin disease, pulmonary disease, endocrinopathy, arthritis, autoimmune hepatitis, and rarely neurologic disease, vasculopathy, and malignancy. Treatment of the autoimmune and immune dysregulatory features of STAT3-GOF patients relies heavily on immunosuppression and is often challenging and fraught with complications including severe infections. Defects in the T cell compartment leading to effector T cell accumulation and decreased T regulatory cells may contribute to autoimmunity. While T cell exhaustion and apoptosis defects likely contribute to the lymphoproliferative phenotype, no conclusive correlations are yet established. Here we review the known mechanistic and clinical characteristics of this heterogenous PIRD.
    Language English
    Publishing date 2023-02-09
    Publishing country Switzerland
    Document type Journal Article ; Review
    ZDB-ID 2711999-3
    ISSN 2296-2360
    ISSN 2296-2360
    DOI 10.3389/fped.2022.770077
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  5. Article ; Online: The Use of Biologic Modifiers as a Bridge to Hematopoietic Cell Transplantation in Primary Immune Regulatory Disorders.

    Arnold, Danielle E / Chellapandian, Deepak / Leiding, Jennifer W

    Frontiers in immunology

    2021  Volume 12, Page(s) 692219

    Abstract: Recently, primary immune regulatory disorders have been described as a subset of inborn errors of immunity that are dominated by immune mediated pathology. As the pathophysiology of disease is elucidated, use of biologic modifiers have been increasingly ... ...

    Abstract Recently, primary immune regulatory disorders have been described as a subset of inborn errors of immunity that are dominated by immune mediated pathology. As the pathophysiology of disease is elucidated, use of biologic modifiers have been increasingly used successfully to treat disease mediated clinical manifestations. Hematopoietic cell transplant (HCT) has also provided definitive therapy in several PIRDs. Although biologic modifiers have been largely successful at treating disease related manifestations, data are lacking regarding long term efficacy, safety, and their use as a bridge to HCT. This review highlights biologic modifiers in the treatment of several PIRDs and there use as a therapeutic bridge to HCT.
    MeSH term(s) Biological Products/therapeutic use ; Hematopoietic Stem Cell Transplantation ; Humans ; Immune System Diseases/drug therapy ; Immune System Diseases/genetics ; STAT1 Transcription Factor/genetics ; STAT3 Transcription Factor/genetics
    Chemical Substances Biological Products ; STAT1 Transcription Factor ; STAT1 protein, human ; STAT3 Transcription Factor ; STAT3 protein, human
    Language English
    Publishing date 2021-06-24
    Publishing country Switzerland
    Document type Journal Article ; Research Support, Non-U.S. Gov't ; Review
    ZDB-ID 2606827-8
    ISSN 1664-3224 ; 1664-3224
    ISSN (online) 1664-3224
    ISSN 1664-3224
    DOI 10.3389/fimmu.2021.692219
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  6. Article ; Online: Mechanism-Based Precision Therapy for the Treatment of Primary Immunodeficiency and Primary Immunodysregulatory Diseases.

    Leiding, Jennifer W / Forbes, Lisa R

    The journal of allergy and clinical immunology. In practice

    2019  Volume 7, Issue 3, Page(s) 761–773

    Abstract: Advances in understanding the mechanism, immunobiology, and pathophysiology of primary immunodeficiency diseases have created opportunities for the use of precision medicines for the treatment of disease-related manifestations. Modulation of the immune ... ...

    Abstract Advances in understanding the mechanism, immunobiology, and pathophysiology of primary immunodeficiency diseases have created opportunities for the use of precision medicines for the treatment of disease-related manifestations. Modulation of the immune system to treat autoimmunity began with the use of intravenous immunoglobulin, improved with the development of monoclonal antibodies, and has now become standard in certain diseases with mechanistic-based targets that alter the molecular mechanism of disease. In this article, we review targeted therapies for disorders of hyperinflammation, primary immunodysregulatory diseases, and primary immunodeficiencies. We also look to the future where gene editing will tailor therapy in an even more precise way for each individual disease and patient.
    MeSH term(s) Animals ; Anti-Inflammatory Agents/therapeutic use ; Antibodies, Monoclonal/therapeutic use ; Genetic Therapy ; Humans ; Immunoglobulins/therapeutic use ; Immunologic Deficiency Syndromes/therapy ; Molecular Targeted Therapy ; Precision Medicine
    Chemical Substances Anti-Inflammatory Agents ; Antibodies, Monoclonal ; Immunoglobulins
    Language English
    Publishing date 2019-03-05
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 2843237-X
    ISSN 2213-2201 ; 2213-2198
    ISSN (online) 2213-2201
    ISSN 2213-2198
    DOI 10.1016/j.jaip.2018.12.017
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    In stock of ZB MED Cologne/Königswinter

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  7. Article ; Online: High-flow 20% subcutaneous immunoglobulin G in patients with primary immunodeficiency disease.

    McCrary, Kaley / Leiding, Jennifer W / Duff, Carla

    Annals of allergy, asthma & immunology : official publication of the American College of Allergy, Asthma, & Immunology

    2020  Volume 125, Issue 3, Page(s) 344–346

    MeSH term(s) Adolescent ; Adult ; Aged ; Aged, 80 and over ; Child ; Child, Preschool ; Female ; Humans ; Immunoglobulin G/administration & dosage ; Immunologic Deficiency Syndromes/drug therapy ; Injections, Subcutaneous/methods ; Male ; Middle Aged ; Primary Immunodeficiency Diseases/drug therapy ; Retrospective Studies ; Young Adult
    Chemical Substances Immunoglobulin G
    Language English
    Publishing date 2020-05-28
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ZDB-ID 1228189-x
    ISSN 1534-4436 ; 0003-4738 ; 1081-1206
    ISSN (online) 1534-4436
    ISSN 0003-4738 ; 1081-1206
    DOI 10.1016/j.anai.2020.05.022
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  8. Article ; Online: Precision Therapy for the Treatment of Primary Immunodysregulatory Diseases.

    Chellapandian, Deepak / Chitty-Lopez, Maria / Leiding, Jennifer W

    Immunology and allergy clinics of North America

    2020  Volume 40, Issue 3, Page(s) 511–526

    Abstract: Precision therapy is a concept in which medical treatment is tailored to the patient's individual needs based on individual characteristics and mechanism of disease. Primary immunodysregulatory disorders are an expanding group of primary immunodeficiency ...

    Abstract Precision therapy is a concept in which medical treatment is tailored to the patient's individual needs based on individual characteristics and mechanism of disease. Primary immunodysregulatory disorders are an expanding group of primary immunodeficiency diseases that are characterized by early onset autoimmunity and autoinflammation. Precision therapy allows for the alteration of the aberrant immune response leading to clinical improvement of disease related manifestations. This article reviews targeted precision-based therapy for treatment of cytotoxic T-lymphocyte antigen haploinsufficiency, lipopolysaccharide-responsive beige-like anchor deficiency, activated PI3K deficiency syndrome, signal transducer and activator of transcription- 1 and -3 - gain-of-function disorders, and disorders of inflammasome activation.
    MeSH term(s) Biomarkers ; Diagnosis, Differential ; Disease Management ; Disease Susceptibility ; Gain of Function Mutation ; Genetic Predisposition to Disease ; Haploinsufficiency ; Humans ; Immunologic Deficiency Syndromes/diagnosis ; Immunologic Deficiency Syndromes/etiology ; Immunologic Deficiency Syndromes/therapy ; Molecular Targeted Therapy ; Precision Medicine/methods ; Signal Transduction
    Chemical Substances Biomarkers
    Language English
    Publishing date 2020-06-09
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 92606-1
    ISSN 1557-8607 ; 0889-8561
    ISSN (online) 1557-8607
    ISSN 0889-8561
    DOI 10.1016/j.iac.2020.04.001
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    Se 710: Show issues Location:
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  9. Article ; Online: Precision medicine in the treatment of primary immunodeficiency diseases.

    Leiding, Jennifer W / Ballow, Mark

    Current opinion in allergy and clinical immunology

    2018  Volume 18, Issue 2, Page(s) 159–166

    Abstract: Purpose of the review: Since the 1990s with the advances in molecular biology, a number of genetic defects have been described. The International Union of Immunological Sciences has recently updated the classification of genetic defects associated with ... ...

    Abstract Purpose of the review: Since the 1990s with the advances in molecular biology, a number of genetic defects have been described. The International Union of Immunological Sciences has recently updated the classification of genetic defects associated with primary immune deficiencies that now number 354. With the ever-expanding list of new monogenic disorders and a better understanding of the immunobiology and function of these defective genes, new therapies have emerged particularly aimed at the autoimmune and inflammatory conditions that plague these patients.
    Recent findings: Immune deficiencies associated with gain-of-function (GOF) mutations are a potential category for targeted therapies to control the GOF activities of the mutated gene. In addition to the increased susceptibility to infections these patients have autoimmune and inflammatory diseases that are difficult to control with conventional therapies. The dysregulated immune functions of the activated phospholipase-3-kinase δ syndrome, cytotoxic T lymphocyte-associated antigen-4 haploinsufficiency, lipopolysaccharide-responsive beige-like anchor deficiency, the GOF mutations of signal transducer and activator of transcription 1 and 3 immune deficiencies will be reviewed. The targeted therapies for each of these immune deficiencies using small molecule kinase inhibitors and fusion protein biologic modifiers will be described.
    Summary: In this review, we explore the recent advances in precision medicine treatment of several primary immunodeficiency syndromes in which immune dysregulation is a key feature. Understanding the immunobiology associated with these GOF mutations has led to the use of biologic therapies to better control the associated autoimmune and inflammatory manifestations.
    MeSH term(s) Autoimmune Diseases/drug therapy ; Autoimmune Diseases/genetics ; Autoimmune Diseases/immunology ; Autoimmunity/genetics ; CTLA-4 Antigen/antagonists & inhibitors ; CTLA-4 Antigen/genetics ; CTLA-4 Antigen/immunology ; CTLA-4 Antigen/metabolism ; Class I Phosphatidylinositol 3-Kinases/antagonists & inhibitors ; Class I Phosphatidylinositol 3-Kinases/genetics ; Class I Phosphatidylinositol 3-Kinases/immunology ; Class I Phosphatidylinositol 3-Kinases/metabolism ; Gain of Function Mutation ; Humans ; Immunologic Deficiency Syndromes/drug therapy ; Immunologic Deficiency Syndromes/genetics ; Immunologic Deficiency Syndromes/immunology ; Molecular Targeted Therapy/methods ; Precision Medicine/methods ; Protein Kinase Inhibitors/pharmacology ; Protein Kinase Inhibitors/therapeutic use ; STAT Transcription Factors/antagonists & inhibitors ; STAT Transcription Factors/genetics ; STAT Transcription Factors/immunology ; STAT Transcription Factors/metabolism ; Signal Transduction/drug effects ; Signal Transduction/genetics ; Signal Transduction/immunology
    Chemical Substances CTLA-4 Antigen ; CTLA4 protein, human ; Protein Kinase Inhibitors ; STAT Transcription Factors ; Class I Phosphatidylinositol 3-Kinases (EC 2.7.1.137) ; PIK3CD protein, human (EC 2.7.1.137)
    Language English
    Publishing date 2018-03-15
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 2088710-3
    ISSN 1473-6322 ; 1528-4050
    ISSN (online) 1473-6322
    ISSN 1528-4050
    DOI 10.1097/ACI.0000000000000431
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    Zs.A 5666: Show issues Location:
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  10. Article ; Online: Antibiotic Prophylaxis for Dental Treatment in Patients with Immunodeficiency.

    Squire, Jacqueline D / Gardner, Pamela J / Moutsopoulos, Niki M / Leiding, Jennifer W

    The journal of allergy and clinical immunology. In practice

    2019  Volume 7, Issue 3, Page(s) 819–823

    Abstract: Routine antibacterial prophylaxis is recommended before dental procedures in select patient populations. Currently, no guidelines are in place for routine prophylaxis before dental procedures in patients with primary immunodeficiency diseases. We review ... ...

    Abstract Routine antibacterial prophylaxis is recommended before dental procedures in select patient populations. Currently, no guidelines are in place for routine prophylaxis before dental procedures in patients with primary immunodeficiency diseases. We review risk factors and provide recommendations on routine dental care and antibacterial prophylaxis in patients with primary immunodeficiency diseases.
    MeSH term(s) Antibiotic Prophylaxis ; Antibodies ; Complement System Proteins/deficiency ; Dental Care ; Humans ; Immunologic Deficiency Syndromes/drug therapy ; Microbiota ; Mouth/microbiology ; Neutrophils
    Chemical Substances Antibodies ; Complement System Proteins (9007-36-7)
    Language English
    Publishing date 2019-01-21
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 2843237-X
    ISSN 2213-2201 ; 2213-2198
    ISSN (online) 2213-2201
    ISSN 2213-2198
    DOI 10.1016/j.jaip.2019.01.016
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