Hyper-IgM syndrome type 1, 2, 3, 4 and 5[2][3][4][5][6]
Diagnostic method
MRI, Chest radiography[1]
Treatment
Allogeneic hematopoietic cell transplantation[7]
Hyper IgM syndrome is a rare primary immune deficiency disorders characterized by low or absent levels of serum IgG, IgA, IgE and normal or increased levels of serum IgM.[8]
They are resulting from mutations in the pathway from B-cell activation to isotype class switching. Patients with HIGM are usually diagnosed within the first two years of life and experience severe immunosuppression. This syndrome is also known as immunoglobulin class switch recombination (Ig-CSR) deficiencies.[9] The most common causes are mutations in the CD40 Ligand (CD40LG) gene located at Xq26.3-27 leading to X-linked HIGM (XHIGM) in males.[10]
^ abCite error: The named reference emed was invoked but never defined (see the help page).
^Cite error: The named reference om1 was invoked but never defined (see the help page).
^Cite error: The named reference om2 was invoked but never defined (see the help page).
^"OMIM Entry – # 606843 – IMMUNODEFICIENCY WITH HYPER-IgM, TYPE 3; HIGM3". omim.org. Retrieved 16 November 2016.
^Cite error: The named reference om5 was invoked but never defined (see the help page).
^"OMIM Entry – 608184 – IMMUNODEFICIENCY WITH HYPER-IgM, TYPE 4; HIGM4". omim.org. Retrieved 2 January 2018.
^Cite error: The named reference gen was invoked but never defined (see the help page).
^Leven, Emily A.; Maffucci, Patrick; Ochs, Hans D.; Scholl, Paul R.; Buckley, Rebecca H.; Fuleihan, Ramsay L.; Geha, Raif S.; Cunningham, Coleen K.; Bonilla, Francisco A.; Conley, Mary Ellen; Ferdman, Ronald M.; Hernandez-Trujillo, Vivian; Puck, Jennifer M.; Sullivan, Kathleen; Secord, Elizabeth A. (July 2016). "Hyper IgM Syndrome: a Report from the USIDNET Registry". Journal of Clinical Immunology. 36 (5): 490–501. doi:10.1007/s10875-016-0291-4. ISSN 0271-9142. PMC 5039943. PMID 27189378.
and 19 Related for: Hyper IgM syndrome information
HyperIgMsyndrome is a rare primary immune deficiency disorders characterized by low or absent levels of serum IgG, IgA, IgE and normal or increased levels...
to undergo immunoglobulin class switching and is associated with hyperIgMsyndrome. Absence of CD154 also stops the formation of germinal centers and...
immunoglobulins, which are IgA, IgE and IgG. There are 5 types of hypergammaglobulinemias associated with hyperIgM. MeSH considers hyperIgMsyndrome to be a form...
autosomal recessive. Many patients with autosomal dominant STAT3 hyper-IgE syndrome have characteristic facial and dental abnormalities, fail to lose...
the spleen are the main source of specific IgM production. In 1937, an antibody was observed in horses hyper-immunized with pneumococcus polysaccharide...
Infections with Pneumocystis pneumonia are also common in infants with hyperIgMsyndrome, an X-linked or autosomal recessive trait. The causative organism...
of an immune deficiency, such as X-linked agammaglobulinemia and hyperIgMsyndrome. Such injections are less common in modern medical practice than they...
To-Ku, an amphibious tank CGMP specific phosphodiesterase type 5 HyperIgMSyndrome Type 5 PDE5 inhibitor, a vasodilator drug Peugeot Type 5 Dietrich-Bugatti...
in the gene encoding mevalonate kinase cause hyper-IgD and periodic fever syndrome. International Hyper-IgD Study Group". Nat. Genet. 22 (2): 178–81. doi:10...
submarine Glutaric acidemia type 2 Glycogen storage disease type II Hyper-IgMsyndrome type 2 Hyperfinite type II factor Type 2 connector, used for charging...
IgM. IgG subclass deficiencies are also an integral component of other well-known primary immunodeficiency diseases, such as Wiskott–Aldrich syndrome...
deficiency. Normal numbers of B cells with decreased IgG and IgA and increased IgM: Hyper-IgMsyndromes Normal numbers of B cells with isotype or light chain...
infections and low antibody levels, specifically in immunoglobulin (Ig) types IgG, IgM, and IgA. Symptoms generally include high susceptibility to pathogens...
deficiency. Normal numbers of B cells with decreased IgG and IgA and increased IgM: Hyper-IgMsyndromes Normal numbers of B cells with isotype or light chain...
Global Information
