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  1. Book: Primary immunodeficiency disorders

    Etzioni, Amos / Ochs, Hans D.

    a historic and scientific perspective

    2014  

    Author's details ed. by Amos Etzioni ; Hans D. Ochs
    Keywords Immunologic diseases/Research/History ; Inflammation/Research/History
    Subject code 616.97009
    Language English
    Size XXI, 353, [30] S. : Ill., graph. Darst., 24 cm
    Edition 1. ed.
    Publisher Elsevier Acad. Press
    Publishing place Amsterdam u.a.
    Publishing country Netherlands
    Document type Book
    HBZ-ID HT018446275
    ISBN 978-0-12-407179-7 ; 0-12-407179-1
    Database Catalogue ZB MED Medicine, Health

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    In stock of ZB MED Cologne/Königswinter

    Shelf markLoan statusLocation
    2014 A 4196 available for loan (reading room) Lesesaal EG

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  2. Book: Primary immunodeficiency diseases

    Ochs, Hans D. / Smith, Edvard / Puck, Jennifer M.

    a molecular and genetic approach

    2014  

    Author's details ed. by Hans D. Ochs ; C. I. Edvard Smith ; Jennifer M. Puck
    Keywords Immunologic Deficiency Syndromes / genetics
    Language English
    Size XVIII, 911 S. : Ill., graph. Darst.
    Edition 3. ed.
    Publisher Oxford Univ. Press
    Publishing place Oxford u.a.
    Publishing country Great Britain
    Document type Book
    HBZ-ID HT018133184
    ISBN 978-0-19-538983-8 ; 9780199971039 ; 0-19-538983-2 ; 019997103X
    Database Catalogue ZB MED Medicine, Health

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    In stock of ZB MED Cologne/Königswinter

    Shelf markLoan statusLocation
    2014 A 243 available for loan (reading room) Lesesaal EG

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  3. Book: Primary immunodeficiency diseases

    Ochs, Hans D.

    a molecular and genetic approach

    2007  

    Author's details ed. by Hans D. Ochs
    Keywords Immunologic Deficiency Syndromes / genetics
    Language English
    Size XVII, 624 S. : Ill., graph. Darst.
    Edition 2. ed.
    Publisher Oxford Univ. Press
    Publishing place Oxford u.a.
    Publishing country Great Britain
    Document type Book
    HBZ-ID HT014678867
    ISBN 978-0-19-514774-2 ; 0-19-514774-X
    Database Catalogue ZB MED Medicine, Health

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    In stock of ZB MED Cologne/Königswinter

    Shelf markLoan statusLocation
    2007 A 535 order for loan Magazin

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  4. Article ; Online: STAT6 joins the gain-of-function club.

    Chen, Karin / Ochs, Hans D / Allenspach, Eric J

    The Journal of allergy and clinical immunology

    2023  Volume 152, Issue 1, Page(s) 53–55

    MeSH term(s) Humans ; Gain of Function Mutation ; Asthma ; Dermatitis, Atopic ; STAT6 Transcription Factor/genetics
    Chemical Substances STAT6 Transcription Factor ; STAT6 protein, human
    Language English
    Publishing date 2023-05-14
    Publishing country United States
    Document type Editorial ; Comment
    ZDB-ID 121011-7
    ISSN 1097-6825 ; 1085-8725 ; 0091-6749
    ISSN (online) 1097-6825 ; 1085-8725
    ISSN 0091-6749
    DOI 10.1016/j.jaci.2023.05.003
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Uh III Zs.92: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 2021: Bestellungen von Artikeln über das Online-Bestellformular
    ab Jg. 2022: Lesesaal (EG)

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  5. Article ; Online: Diagnosis and clinical management of Wiskott-Aldrich syndrome: current and emerging techniques.

    Cavannaugh, Corey / Ochs, Hans D / Buchbinder, David

    Expert review of clinical immunology

    2022  Volume 18, Issue 6, Page(s) 609–623

    Abstract: Introduction: Wiskott-Aldrich syndrome (WAS) serves as the prototype of how variants in a gene, which encodes a protein central to actin cytoskeletal homeostasis can manifest clinically in a variety of ways including infection, atopy, autoimmunity, ... ...

    Abstract Introduction: Wiskott-Aldrich syndrome (WAS) serves as the prototype of how variants in a gene, which encodes a protein central to actin cytoskeletal homeostasis can manifest clinically in a variety of ways including infection, atopy, autoimmunity, inflammation, bleeding, neutropenia, non-malignant lymphoproliferation, and malignancy. Despite the discovery of the
    Areas covered: This review will provide an overview of the approach to the diagnosis of WAS as well as the management of its associated complications. Advances in the use of allogeneic hematopoietic stem cell transplantation (HSCT) and gene therapy as well as the associated challenges unique to WAS will be discussed.
    Expert opinion: Basic research, combined with clinical research focusing on longitudinal analysis of WAS patients, will help clarify determinants that influence WAS pathogenesis as well as clinical complications and outcomes. Advances in curative approaches including the use of alternative donor HSCT for WAS continue to evolve. Gene therapy employing safer and more effective protocols ensuring full correction of WAS will provide life-saving benefit to WAS patients who are unable to undergo HSCT.
    MeSH term(s) Genetic Therapy/methods ; Hematopoietic Stem Cell Transplantation/methods ; Humans ; Neutropenia ; Wiskott-Aldrich Syndrome/diagnosis ; Wiskott-Aldrich Syndrome/genetics ; Wiskott-Aldrich Syndrome/therapy
    Language English
    Publishing date 2022-05-19
    Publishing country England
    Document type Journal Article ; Review
    ZDB-ID 2274260-8
    ISSN 1744-8409 ; 1744-666X
    ISSN (online) 1744-8409
    ISSN 1744-666X
    DOI 10.1080/1744666X.2022.2074400
    Database MEDical Literature Analysis and Retrieval System OnLINE

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 6661: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 2021: Bestellungen von Artikeln über das Online-Bestellformular
    ab Jg. 2022: Lesesaal (EG)

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  6. Book: Primary immunodeficiency diseases

    Ochs, Hans D.

    a molecular and genetic approach

    1999  

    Author's details ed. by Hans D. Ochs
    Keywords Immunologic Deficiency Syndromes / genetics ; Immunkrankheit ; Immundefekt ; Primärer Immundefekt ; Genetik
    Subject Allgemeine Genetik ; Erbbiologie ; Erbforschung ; Erblehre ; Vererbungslehre ; Vererbungswissenschaft ; Erblichkeitslehre ; Immunabwehrschwäche ; Immundefizienz ; Immunschwäche ; Immunmangel ; Abwehrschwäche ; Immunopathie ; Immunologische Krankheit ; Immunologische Erkrankung
    Language English
    Size XIII, 501, [16] S. : Ill., graph. Darst.
    Publisher Oxford Univ. Press
    Publishing place New York u.a.
    Publishing country United States
    Document type Book
    HBZ-ID HT010612598
    ISBN 0-19-510486-2 ; 978-0-19-510486-8
    Database Catalogue ZB MED Medicine, Health

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    In stock of ZB MED Cologne/Königswinter

    Shelf markLoan statusLocation
    1999 A 3639 order for loan Magazin

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    This service is chargeable due to the Delivery terms set by subito. Orders including an article and supplementary material will be classified as separate orders. In these cases, fees will be demanded for each order.

  7. Article: Hematopoietic Stem Cell Therapy for Wiskott-Aldrich Syndrome: Improved Outcome and Quality of Life.

    Mallhi, Kanwaldeep K / Petrovic, Aleksandra / Ochs, Hans D

    Journal of blood medicine

    2021  Volume 12, Page(s) 435–447

    Abstract: The Wiskott-Aldrich syndrome (WAS) is an X-linked disorder caused by mutations in ... ...

    Abstract The Wiskott-Aldrich syndrome (WAS) is an X-linked disorder caused by mutations in the
    Language English
    Publishing date 2021-06-11
    Publishing country New Zealand
    Document type Journal Article ; Review
    ZDB-ID 2587464-0
    ISSN 1179-2736
    ISSN 1179-2736
    DOI 10.2147/JBM.S232650
    Database MEDical Literature Analysis and Retrieval System OnLINE

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  8. Article ; Online: Lazy Leukocyte Syndrome-an Enigma Finally Solved?

    Etzioni, Amos / Ochs, Hans D

    Journal of clinical immunology

    2019  Volume 40, Issue 1, Page(s) 9–12

    MeSH term(s) Humans ; Leukocyte Disorders/congenital ; Leukocyte Disorders/genetics ; Leukocyte Disorders/pathology ; Neutropenia/genetics ; Neutropenia/pathology
    Language English
    Publishing date 2019-11-25
    Publishing country Netherlands
    Document type Editorial
    ZDB-ID 779361-3
    ISSN 1573-2592 ; 0271-9142
    ISSN (online) 1573-2592
    ISSN 0271-9142
    DOI 10.1007/s10875-019-00718-0
    Database MEDical Literature Analysis and Retrieval System OnLINE

    Full text online

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    In stock of ZB MED Cologne/Königswinter

    Zs.A 1640: Show issues Location:
    Je nach Verfügbarkeit (siehe Angabe bei Bestand)
    bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular
    Jg. 1995 - 2021: Lesesall (1.OG)
    ab Jg. 2022: Lesesaal (EG)

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  9. Book: Immunologic disorders in infants & children

    Stiehm, E. Richard / Ochs, Hans D. / Winkelstein, Jerry A.

    2004  

    Author's details E. Richard Stiehm ; Hans D. Ochs ; Jerry A. Winkelstein
    Keywords Immunologic Diseases ; Infant ; Child ; Immunologic diseases in children ; Immunologic diseases in infants ; Immunkrankheit ; Kind
    Subject Kindheit ; Kindesalter ; Kindschaft ; Kinder ; Immunopathie ; Immunologische Krankheit ; Immunologische Erkrankung
    Subject code 618.9297
    Language English
    Size XXI, 1512 S. : Ill., graph. Darst.
    Edition 5. ed.
    Publisher Elsevier Saunders
    Publishing place Philadelphia, Pa
    Publishing country United States
    Document type Book
    HBZ-ID HT013911957
    ISBN 0-7216-8964-7 ; 978-0-7216-8964-7
    Database Catalogue ZB MED Medicine, Health

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    In stock of ZB MED Cologne/Königswinter

    Shelf markLoan statusLocation
    2004 A 2338 order for loan Magazin

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  10. Article ; Online: From clinical observations and molecular dissection to novel therapeutic strategies for primary immunodeficiency disorders.

    Ochs, Hans D / Petroni, Daniel

    American journal of medical genetics. Part A

    2017  Volume 176, Issue 4, Page(s) 784–803

    Abstract: The field of primary immunodeficiency diseases (PID) is rapidly expanding with more than 300 genetically defined disorders that have been clinically described and molecularly analyzed. The molecular dissection of these entities has led to the discovery ... ...

    Abstract The field of primary immunodeficiency diseases (PID) is rapidly expanding with more than 300 genetically defined disorders that have been clinically described and molecularly analyzed. The molecular dissection of these entities has led to the discovery of new immunologic pathways and to novel and effective disease-specific therapies. This review provides a summary of these primary immune defects categorized by clinical phenotype and molecular similarity as defined by the International Union of Immunologic Societies (IUIS) Expert Committee for PID. In this synopsis, we discuss the molecular basis of various categories of PIDs including, but not limited to, severe combined immunodeficiencies, primary antibody deficiencies, immune dysregulation syndromes, as well as defects of the innate immune system such as phagocytic abnormalities, autoinflammatory fever syndromes, and complement deficiencies. We have attempted to focus on current strategies to prevent complications, ameliorate symptoms, or cure these disorders by promptly using antimicrobial therapies, immunoglobulin replacement, and hematopoietic stem cell transplantation. In addition, we will explore novel therapies such as molecularly targeted immunosuppression with monoclonal antibodies and specific immunomodulatory agents. Finally, we address experimental therapies targeting specific molecular defects, including gene therapy and gene editing.
    MeSH term(s) Animals ; Autoimmunity ; Biomarkers ; Disease Management ; Disease Susceptibility ; Genetic Predisposition to Disease ; Humans ; Immunity, Innate ; Immunologic Deficiency Syndromes/etiology ; Immunologic Deficiency Syndromes/metabolism ; Immunologic Deficiency Syndromes/therapy ; Immunomodulation ; Symptom Assessment
    Chemical Substances Biomarkers
    Language English
    Publishing date 2017-09-21
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 1493479-6
    ISSN 1552-4833 ; 1552-4825
    ISSN (online) 1552-4833
    ISSN 1552-4825
    DOI 10.1002/ajmg.a.38480
    Database MEDical Literature Analysis and Retrieval System OnLINE

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