Concepedia

Hyper Igm Syndromes

Hyper IgM Syndromes is a group of rare primary immunodeficiency disorders characterized by defective class-switch recombination in B cells, leading to elevated or normal levels of immunoglobulin M with markedly reduced levels of immunoglobulin G, immunoglobulin A, and immunoglobulin E, resulting in heightened susceptibility to recurrent bacterial, opportunistic, and fungal infections.

284

Publications

20.4K

Citations

1.7K

Authors

558

Institutions

Publications per year

2017–2026

25

Variants

Hyper-igm Immunodeficiency SyndromesHyper-igm Syndromes

Parents

Publications

284

Activation-Induced Cytidine Deaminase (AID) Deficiency Causes the Autosomal Recessive Form of the Hyper-IgM Syndrome (HIGM2)

Patrick Revy, Taro Muto, Yves Lévy et al. · Cell · 2000 · 1.6K citations · Full text

, , +13

CD40 Ligand Gene Defects Responsible for X-Linked Hyper-IgM Syndrome

R.Cutler Allen, Richard J. Armitage, Mary Ellen Conley et al. · Science · 1993 · 827 citations

, , +17

The CD40 ligand, gp39, is defective in activated T cells from patients with X-linked hyper-IgM syndrome

Alejandro Aruffo, Mary Farrington, Diane Hollenbaugh et al. · Cell · 1993 · 824 citations

, , +11

Defective expression of T-cell CD40 ligand causes X-linked immunodeficiency with hyper-IgM

Ulf Korthäuer, Daniel Graf, Hans Werner Mages et al. · Nature · 1993 · 725 citations

, , +13

CD40 ligand mutations in X-linked immunodeficiency with hyper-IgM

J Y Bonnefoy, J. F. Gauchatt, Alain Fischer et al. · Nature · 1993 · 702 citations

, , +17

1–5 of 284

Authors

1.7K

  1. HD
    Hans D. Ochs

    University of Washington, United States

    25 publications · 3.1K citations

    22

    h-index

  2. AF
    Alain Fischer

    Inserm, France

    16 publications · 3.8K citations

    16

    h-index

  3. AD
    Anne Durandy

    Inserm, France

    17 publications · 3.4K citations

    15

    h-index

  4. SN
    Shigeaki Nonoyama

    National Defense Medical College, Japan

    17 publications · 1.7K citations

    15

    h-index

  5. AA
    Asghar Aghamohammadi

    Tehran University of Medical Sciences, Iran

    13 publications · 417 citations

    13

    h-index

1–5 of 1.7K

Institutions

558

  1. Inserm

    Paris, France

    20 h-index

    91 publications

    25.7K citations

  2. University of Brescia

    Brescia, Italy

    19 h-index

    77 publications

    15.7K citations

  3. University of Washington

    Seattle, United States

    17 h-index

    59 publications

    5.8K citations

  4. Hôpital Necker-Enfants Malades

    Paris, France

    15 h-index

    67 publications

    26.1K citations

  5. Tokyo Medical and Dental University

    Tokyo, Japan

    13 h-index

    39 publications

    3K citations

  6. Duke University

    Durham, United States

    12 h-index

    29 publications

    2.2K citations

  7. University College London

    London, United Kingdom

    12 h-index

    23 publications

    1.8K citations

  8. National Institutes of Health

    Bethesda, United States

    12 h-index

    35 publications

    1.6K citations

1–8 of 558

Venues

  1. PubMed

    Repository · 28 publications · 1K citations

    16

    h-index

  2. Journal of Clinical Investigation

    JCI · 13 publications · 1.1K citations

    13

    h-index

  3. The Journal of Immunology

    JI · 12 publications · 598 citations

    11

    h-index

  4. Journal of Clinical Immunology

    12 publications · 491 citations

    11

    h-index

  5. Clinical Immunology

    9 publications · 712 citations

    9

    h-index

1–5