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Hyper-IgM Immunodeficiency Syndrome (Hyper-IgM Syndrome)

A rare inherited immunodeficiency syndrome characterized by normal or elevated serum IMMUNOGLOBULIN M levels with absence of IMMUNOGLOBULIN G; IMMUNOGLOBULIN A; and IMMUNOGLOBULIN E. It results in a profound susceptibility to BACTERIAL INFECTIONS and an increased susceptibility to OPPORTUNISTIC INFECTIONS. Several subtypes of hyper-IgM immunodeficiency syndrome exist depending upon the location of genetic mutation.
Also Known As:
Hyper-IgM Syndrome; HIGM2 Syndrome; HIGM3 Syndrome; HIGM5 Syndrome; Hyper-IgM Immunodeficiency Syndrome Type 2; Hyper-IgM Immunodeficiency Syndrome Type 3; Hyper-IgM Immunodeficiency Syndrome Type 5; Hyper-IgM Syndrome 2; Hyper-IgM Syndrome 3; Hyper-IgM Syndrome 5; Immunodeficiency with Hyper-IgM Syndrome; Immunodeficiency with Hyper-IgM, Type 2; Immunodeficiency with Hyper-IgM, Type 3; Immunodeficiency with Hyper-IgM, Type 5; HIGM2 Syndromes; HIGM3 Syndromes; HIGM5 Syndromes; Hyper IgM Immunodeficiency Syndrome; Hyper IgM Immunodeficiency Syndrome Type 2; Hyper IgM Immunodeficiency Syndrome Type 3; Hyper IgM Immunodeficiency Syndrome Type 5; Hyper IgM Immunodeficiency Syndrome, Type 2; Hyper IgM Immunodeficiency Syndrome, Type 3; Hyper IgM Immunodeficiency Syndrome, Type 5; Hyper IgM Syndrome; Hyper IgM Syndrome 2; Hyper IgM Syndrome 3; Hyper IgM Syndrome 5; Hyper-IgM Immunodeficiency Syndromes; Hyper-IgM Syndrome 5s; Hyper-IgM Syndromes; Immunodeficiency Syndrome, Hyper-IgM; Immunodeficiency Syndromes, Hyper-IgM; Immunodeficiency with Hyper IgM Syndrome; Immunodeficiency with Hyper IgM, Type 2; Immunodeficiency with Hyper IgM, Type 3; Immunodeficiency with Hyper IgM, Type 5; Syndrome, Hyper-IgM Immunodeficiency; Syndromes, Hyper-IgM Immunodeficiency; Hyper-IgM Immunodeficiency Syndrome, Type 2; Hyper-IgM Immunodeficiency Syndrome, Type 3; Hyper-IgM Immunodeficiency Syndrome, Type 5
Networked: 187 relevant articles (1 outcomes, 5 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Toxoplasmosis
2. Common Variable Immunodeficiency
3. Infections
4. Type 1 Hyper-IgM Immunodeficiency Syndrome
5. Primary Immunodeficiency Diseases

Experts

1. Durandy, Anne: 11 articles (01/2020 - 10/2003)
2. Aghamohammadi, Asghar: 8 articles (04/2022 - 01/2004)
3. Rezaei, Nima: 8 articles (01/2014 - 01/2004)
4. Imai, Kohsuke: 7 articles (10/2018 - 10/2003)
5. Nonoyama, Shigeaki: 7 articles (10/2018 - 03/2003)
6. Fischer, Alain: 7 articles (01/2007 - 10/2003)
7. Notarangelo, Luigi D: 5 articles (10/2018 - 07/2003)
8. Morio, Tomohiro: 5 articles (01/2018 - 03/2003)
9. Abolhassani, Hassan: 4 articles (04/2022 - 11/2009)
10. Kavli, Bodil: 4 articles (06/2012 - 10/2003)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Hyper-IgM Immunodeficiency Syndrome:
1. Immunoglobulins (Immunoglobulin)IBA
2. Cytidine DeaminaseIBA
3. Immunoglobulin M (IgM)IBA
4. LigandsIBA
5. Proteins (Proteins, Gene)FDA Link
6. CD40 Ligand (CD40L)IBA
7. Immunoglobulin G (IgG)IBA
8. Immunoglobulin A (IgA)IBA
9. Immunoglobulin E (IgE)IBA
10. Immunoglobulin IsotypesIBA

Therapies and Procedures

1. Therapeutics
2. Immunomodulation
3. Stem Cell Transplantation
4. Hematopoietic Stem Cell Transplantation
5. Liver Transplantation