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Job Syndrome (Hyper-IgE Syndrome)

Primary immunodeficiency syndrome characterized by recurrent infections and hyperimmunoglobulinemia E. Most cases are sporadic. Of the rare familial forms, the dominantly inherited subtype has additional connective tissue, dental and skeletal involvement that the recessive type does not share.
Also Known As:
Hyper-IgE Syndrome; Buckley Syndrome; HIE Syndrome; HIES, Autosomal Dominant; Hyper-IgE Recurrent Infection Syndrome, Autosomal Recessive; Hyper-IgE Syndrome, Autosomal Dominant; Hyper-Immunoglobulin E Syndrome, Autosomal Dominant; Hyperimmunoglobulin E, Recurrent Infection Syndrome; Hyperimmunoglobulinemia E Syndrome; Job-Buckley Syndrome; Autosomal Dominant HIES; Autosomal Dominant HIESs; Autosomal Recessive HIES; Autosomal Recessive HIESs; Buckley Syndromes; HIE Syndromes; HIESs, Autosomal Dominant; HIESs, Autosomal Recessive; Hyper IgE Recurrent Infection Syndrome, Autosomal Recessive; Hyper IgE Syndrome; Hyper IgE Syndrome, Autosomal Dominant; Hyper IgE Syndrome, Autosomal Recessive; Hyper Immunoglobulin E Syndrome, Autosomal Dominant; Hyper Immunoglobulin E Syndrome, Autosomal Recessive; Hyper-IgE Syndromes; Hyperimmunoglobulin E Recurrent Infection Syndrome; Hyperimmunoglobulinemia E Syndromes; Job Buckley Syndrome; Job Syndromes; Job-Buckley Syndromes; Jobs Syndrome; Syndrome, Job; Syndromes, Job; HIES, Autosomal Recessive; Hyper-IgE Syndrome, Autosomal Recessive; Hyper-Immunoglobulin E Syndrome, Autosomal Recessive; Hyperimmunoglobulin E-Recurrent Infection Syndrome; Job's Syndrome
Networked: 466 relevant articles (21 outcomes, 25 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Atopic Dermatitis (Atopic Eczema)
2. Eczema
3. Pneumonia (Pneumonitis)
4. Infections
5. Hypersensitivity (Allergy)

Experts

1. Freeman, Alexandra F: 37 articles (01/2021 - 11/2005)
2. Holland, Steven M: 27 articles (10/2020 - 12/2004)
3. Renner, Ellen D: 11 articles (01/2021 - 07/2008)
4. Grimbacher, Bodo: 10 articles (01/2021 - 10/2007)
5. Minegishi, Yoshiyuki: 8 articles (10/2021 - 08/2007)
6. Puel, Anne: 8 articles (01/2021 - 09/2012)
7. Hagl, Beate: 7 articles (01/2021 - 07/2013)
8. Casanova, Jean-Laurent: 7 articles (01/2020 - 09/2012)
9. Puck, Jennifer M: 7 articles (06/2013 - 05/2007)
10. Keles, Sevgi: 6 articles (12/2020 - 08/2009)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Job Syndrome:
1. dupilumabIBA
2. Immunoglobulin E (IgE)IBA
3. Anti-Bacterial Agents (Antibiotics)IBA
4. Cyclosporine (Ciclosporin)FDA LinkGeneric
5. Immunoglobulins (Immunoglobulin)IBA
6. Interleukin-4 (Interleukin 4)IBA
7. Intravenous Immunoglobulins (IVIG)FDA Link
8. Proteins (Proteins, Gene)FDA Link
9. Omalizumab (Xolair)FDA Link
10. anti-IgE antibodiesIBA

Therapies and Procedures

1. Therapeutics
2. Plasmapheresis
3. Drug Therapy (Chemotherapy)
4. Hematopoietic Stem Cell Transplantation
5. Bone Marrow Transplantation (Transplantation, Bone Marrow)