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class="infobox-above" style="background:#ccc">Hyper IgM syndrome</th></tr><tr style="background-color: #f8f9fa;"><td colspan="2" class="infobox-full-data"><span class="mw-default-size" typeof="mw:File/Frameless"><a href="/wiki/File:IgM_scheme.svg" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/7/77/IgM_scheme.svg/220px-IgM_scheme.svg.png" decoding="async" width="220" height="211" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/7/77/IgM_scheme.svg/330px-IgM_scheme.svg.png 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/7/77/IgM_scheme.svg/440px-IgM_scheme.svg.png 2x" data-file-width="400" data-file-height="384" /></a></span></td></tr><tr><td colspan="2" class="infobox-full-data"><a href="/wiki/Immunoglobulin_M" title="Immunoglobulin M">Immunoglobulin M</a></td></tr><tr><th scope="row" class="infobox-label"><a href="/wiki/Medical_specialty" title="Medical specialty">Specialty</a></th><td class="infobox-data"><a href="/wiki/Immunology" title="Immunology">Immunology</a> <span class="penicon autoconfirmed-show"><span class="mw-valign-text-top" typeof="mw:File/Frameless"><a href="https://www.wikidata.org/wiki/Q1617658?uselang=en#P1995" title="Edit this on Wikidata"><img alt="Edit this on Wikidata" src="//upload.wikimedia.org/wikipedia/en/thumb/8/8a/OOjs_UI_icon_edit-ltr-progressive.svg/10px-OOjs_UI_icon_edit-ltr-progressive.svg.png" decoding="async" width="10" height="10" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/en/thumb/8/8a/OOjs_UI_icon_edit-ltr-progressive.svg/15px-OOjs_UI_icon_edit-ltr-progressive.svg.png 1.5x, //upload.wikimedia.org/wikipedia/en/thumb/8/8a/OOjs_UI_icon_edit-ltr-progressive.svg/20px-OOjs_UI_icon_edit-ltr-progressive.svg.png 2x" data-file-width="20" data-file-height="20" /></a></span></span></td></tr><tr><th scope="row" class="infobox-label"><a href="/wiki/Signs_and_symptoms" title="Signs and symptoms">Symptoms</a></th><td class="infobox-data">Chronic diarrhea<sup id="cite_ref-emed_1-0" class="reference"><a href="#cite_note-emed-1"><span class="cite-bracket">[</span>1<span class="cite-bracket">]</span></a></sup></td></tr><tr><th scope="row" class="infobox-label">Types</th><td class="infobox-data">Hyper-IgM syndrome type 1, 2, 3, 4 and 5<sup id="cite_ref-om1_2-0" class="reference"><a href="#cite_note-om1-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-om2_3-0" class="reference"><a href="#cite_note-om2-3"><span class="cite-bracket">[</span>3<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-om3_4-0" class="reference"><a href="#cite_note-om3-4"><span class="cite-bracket">[</span>4<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-om5_5-0" class="reference"><a href="#cite_note-om5-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-6" class="reference"><a href="#cite_note-6"><span class="cite-bracket">[</span>6<span class="cite-bracket">]</span></a></sup></td></tr><tr><th scope="row" class="infobox-label"><a href="/wiki/Medical_diagnosis" title="Medical diagnosis">Diagnostic method</a></th><td class="infobox-data">MRI, Chest radiography<sup id="cite_ref-emed_1-1" class="reference"><a href="#cite_note-emed-1"><span class="cite-bracket">[</span>1<span class="cite-bracket">]</span></a></sup></td></tr><tr><th scope="row" class="infobox-label">Treatment</th><td class="infobox-data">Allogeneic hematopoietic cell transplantation<sup id="cite_ref-gen_7-0" class="reference"><a href="#cite_note-gen-7"><span class="cite-bracket">[</span>7<span class="cite-bracket">]</span></a></sup></td></tr></tbody></table> <p><b>Hyper IgM syndrome</b> is a rare <a href="/wiki/Primary_immunodeficiency" title="Primary immunodeficiency">primary immune deficiency disorders</a> characterized by low or absent levels of serum IgG, IgA, IgE and normal or increased levels of serum IgM.<sup id="cite_ref-:0_8-0" class="reference"><a href="#cite_note-:0-8"><span class="cite-bracket">[</span>8<span class="cite-bracket">]</span></a></sup> </p><p>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 <a href="/wiki/Immunosuppression" title="Immunosuppression">immunosuppression</a>. This syndrome is also known as immunoglobulin class switch recombination (Ig-CSR) deficiencies.<sup id="cite_ref-:1_9-0" class="reference"><a href="#cite_note-:1-9"><span class="cite-bracket">[</span>9<span class="cite-bracket">]</span></a></sup> The most common causes are mutations in the CD40 Ligand (<i>CD40LG</i>) gene located at Xq26.3-27 leading to X-linked HIGM (XHIGM) in males.<sup id="cite_ref-10" class="reference"><a href="#cite_note-10"><span class="cite-bracket">[</span>10<span class="cite-bracket">]</span></a></sup> </p> <meta property="mw:PageProp/toc" /> <div class="mw-heading mw-heading2"><h2 id="Types">Types</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Hyper_IgM_syndrome&action=edit&section=1" title="Edit section: Types"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>Five types of hyper IgM syndrome have been characterized: </p> <ul><li><i><a href="/wiki/Hyper-IgM_syndrome_type_1" title="Hyper-IgM syndrome type 1">Hyper-IgM syndrome type 1</a></i> (X-linked), characterized by mutations of the <i><a href="/wiki/CD40LG" class="mw-redirect" title="CD40LG">CD40LG</a></i> gene. In this type, lack of CD40L on the surfaces of <a href="/wiki/T_cell" title="T cell">T cells</a> results in defective signaling to B cells, which do not receive the needed signal to undergo <a href="/wiki/Immunoglobulin_class_switching" title="Immunoglobulin class switching">isotype switching</a>. Therefore, the only antibody secreted by the B cell is IgM, the least specific class of antibody.<sup id="cite_ref-om1_2-1" class="reference"><a href="#cite_note-om1-2"><span class="cite-bracket">[</span>2<span class="cite-bracket">]</span></a></sup></li> <li><i><a href="/wiki/Hyper-IgM_syndrome_type_2" title="Hyper-IgM syndrome type 2">Hyper-IgM syndrome type 2</a></i> (autosomal recessive), characterized by mutations of the <i><a href="/wiki/AICDA" class="mw-redirect" title="AICDA">AICDA</a></i> gene. In this type, B cells cannot recombine genetic material to change <a href="/wiki/Immunoglobulin_heavy_chain" title="Immunoglobulin heavy chain">heavy chain</a> production<sup id="cite_ref-om2_3-1" class="reference"><a href="#cite_note-om2-3"><span class="cite-bracket">[</span>3<span class="cite-bracket">]</span></a></sup></li> <li><i><a href="/wiki/Hyper-IgM_syndrome_type_3" title="Hyper-IgM syndrome type 3">Hyper-IgM syndrome type 3</a></i> characterized by mutations of the <i><a href="/wiki/CD40" class="mw-redirect" title="CD40">CD40</a></i> gene and it is inherited by autosomal recessive manner. It has similar phenotype of impaired <a href="/wiki/Immunoglobulin_class_switching" title="Immunoglobulin class switching">class switch recombination</a> and <a href="/wiki/Somatic_hypermutation" title="Somatic hypermutation">somatic hyper mutation</a> with CD40L deficiency but B cells from CD40 deficient patients are unable to undergo class switching <a href="/wiki/In_vitro" title="In vitro">in vitro</a> upon activation with agonists and cytokines as per their intrinsic defect .<sup id="cite_ref-:1_9-1" class="reference"><a href="#cite_note-:1-9"><span class="cite-bracket">[</span>9<span class="cite-bracket">]</span></a></sup></li> <li><i><a href="/wiki/Hyper-IgM_syndrome_type_4" title="Hyper-IgM syndrome type 4">Hyper-IgM syndrome type 4</a></i> caused by the mutation in the NEMO (nuclear factor κB essential modulator) complex, which, when mutated, is unable to phosphorylate IκB downstream of CD40 signaling.</li> <li><i><a href="/wiki/Hyper-IgM_syndrome_type_5" title="Hyper-IgM syndrome type 5">Hyper-IgM syndrome type 5</a></i> characterized by mutations of the <i><a href="/wiki/Uracil-DNA_glycosylase" title="Uracil-DNA glycosylase">UNG</a></i> gene.<sup id="cite_ref-om5_5-1" class="reference"><a href="#cite_note-om5-5"><span class="cite-bracket">[</span>5<span class="cite-bracket">]</span></a></sup> UNG is responsible for the cleavage of cytosines that have been deaminated by AID in single-stranded DNA.</li> <li><i><a href="/wiki/Hyper-IgM_syndrome_type_5" title="Hyper-IgM syndrome type 5">Hyper-IgM syndrome type 6</a></i> is the least-characterized of the HIGM types, as the gene is unknown. Resembles HIGM2, but AID is normal.</li></ul> <div class="mw-heading mw-heading2"><h2 id="Signs_and_symptoms">Signs and symptoms</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Hyper_IgM_syndrome&action=edit&section=2" title="Edit section: Signs and symptoms"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>The majority of patients with HIGM syndrome present with a broad spectrum of clinical symptoms even with a same genetic defects.<sup id="cite_ref-11" class="reference"><a href="#cite_note-11"><span class="cite-bracket">[</span>11<span class="cite-bracket">]</span></a></sup> They usually develop symptoms in infancy and second year of life, including increased susceptibility to infections by extracellular bacteria, sinus & ear infections, skin infections. Furthermore, these patients are prone to <a href="/wiki/Pulmonary_complications" class="mw-redirect" title="Pulmonary complications">pulmonary complications</a>, gastrointestinal manifestations, <a href="/wiki/Autoimmune_disease" title="Autoimmune disease">autoimmune disorders</a>, hematologic abnormalities, lymphoproloferation and <a href="/wiki/Malignancy" title="Malignancy">malignancies.</a> Heterozygous females in X-linked hyper IgM syndrome (HIGM1) are usually asymptomatic. However, immunologic testing has revealed that they exhibit reduced expression of CD40L when CD4+ T lymphocytes are activated. In some cases, females with significant reduction in circulating lymphocytes carrying the CD40L mutation due to skewed X-chromosome inactivation can present with symptoms resembling HIGM1 or <a href="/wiki/Common_variable_immunodeficiency" title="Common variable immunodeficiency">common variable immunodeficiency</a>.<sup id="cite_ref-:2_12-0" class="reference"><a href="#cite_note-:2-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup> Among the presentation consistent with hyper IgM syndrome are the following:<sup id="cite_ref-emed_1-2" class="reference"><a href="#cite_note-emed-1"><span class="cite-bracket">[</span>1<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-13" class="reference"><a href="#cite_note-13"><span class="cite-bracket">[</span>13<span class="cite-bracket">]</span></a></sup> </p> <figure class="mw-default-size" typeof="mw:File/Thumb"><a href="/wiki/File:Pneumocystis.jpg" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/6/6e/Pneumocystis.jpg/220px-Pneumocystis.jpg" decoding="async" width="220" height="133" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/6/6e/Pneumocystis.jpg/330px-Pneumocystis.jpg 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/6/6e/Pneumocystis.jpg/440px-Pneumocystis.jpg 2x" data-file-width="600" data-file-height="364" /></a><figcaption>Pneumocystis pneumonia</figcaption></figure> <ul><li><a href="/wiki/Infection" title="Infection">Infection</a>/<a href="/wiki/Pneumocystis_pneumonia" title="Pneumocystis pneumonia"><i>Pneumocystis</i> pneumonia</a> (PCP), which is common in infants with hyper IgM syndrome, is a serious illness.<sup id="cite_ref-nat_14-0" class="reference"><a href="#cite_note-nat-14"><span class="cite-bracket">[</span>14<span class="cite-bracket">]</span></a></sup> PCP is one of the most frequent and severe opportunistic infections in people with weakened immune systems. Many CD40 Ligand Deficiency are first diagnosed after having PCP in their first year of life. The fungus is common and is present in over 70% of healthy people's lungs, however, Hyper IgM patients are not able to fight it off without the administration of <a href="/wiki/Trimethoprim/sulfamethoxazole" title="Trimethoprim/sulfamethoxazole">Bactrim</a><sup class="noprint Inline-Template Template-Fact" style="white-space:nowrap;">[<i><a href="/wiki/Wikipedia:Identifying_reliable_sources_(medicine)" title="Wikipedia:Identifying reliable sources (medicine)"><span title="Material near this tag needs references to reliable medical sources. (November 2016)">medical citation needed</span></a></i>]</sup></li> <li><a href="/wiki/Hepatitis" title="Hepatitis">Hepatitis</a> (Hepatitis C)</li> <li>Chronic <a href="/wiki/Diarrhea" title="Diarrhea">diarrhea</a></li> <li><a href="/wiki/Hypothyroidism" title="Hypothyroidism">Hypothyroidism</a></li> <li><a href="/wiki/Neutropenia" title="Neutropenia">Neutropenia</a></li> <li><a href="/wiki/Arthritis" title="Arthritis">Arthritis</a></li> <li><a href="/wiki/Encephalopathy" title="Encephalopathy">Encephalopathy</a> (degenerative)</li></ul> <div class="mw-heading mw-heading2"><h2 id="Cause">Cause</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Hyper_IgM_syndrome&action=edit&section=3" title="Edit section: Cause"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <figure typeof="mw:File/Thumb"><a href="/wiki/File:Class_switch_recombination.png" class="mw-file-description"><img src="//upload.wikimedia.org/wikipedia/commons/thumb/b/b3/Class_switch_recombination.png/150px-Class_switch_recombination.png" decoding="async" width="150" height="150" class="mw-file-element" srcset="//upload.wikimedia.org/wikipedia/commons/thumb/b/b3/Class_switch_recombination.png/225px-Class_switch_recombination.png 1.5x, //upload.wikimedia.org/wikipedia/commons/thumb/b/b3/Class_switch_recombination.png/300px-Class_switch_recombination.png 2x" data-file-width="750" data-file-height="750" /></a><figcaption>Class switch recombination</figcaption></figure> <p>Different genetic defects cause HIgM syndrome, the vast majority are inherited as an <a href="/wiki/X-linked" class="mw-redirect" title="X-linked">X-linked</a> recessive genetic trait and most with the condition are male.<sup id="cite_ref-gen_7-1" class="reference"><a href="#cite_note-gen-7"><span class="cite-bracket">[</span>7<span class="cite-bracket">]</span></a></sup> </p><p>IgM is the form of <a href="/wiki/Antibody" title="Antibody">antibody</a> that all B cells produce initially before they undergo <a href="/wiki/Antibody#Class_switching" title="Antibody">class switching</a> due to exposure to a recognized antigen. Healthy <a href="/wiki/B_cells" class="mw-redirect" title="B cells">B cells</a> efficiently switch to other types of antibodies as needed to attack invading bacteria, viruses, and other pathogens. In people with hyper IgM syndromes, the B cells keep making IgM antibodies because they can't switch to a different antibody. This results in an overproduction of IgM antibodies and an underproduction of <a href="/wiki/IgA" class="mw-redirect" title="IgA">IgA</a>, <a href="/wiki/Immunoglobulin_G" title="Immunoglobulin G">IgG</a>, and <a href="/wiki/IgE" class="mw-redirect" title="IgE">IgE</a>.<sup id="cite_ref-15" class="reference"><a href="#cite_note-15"><span class="cite-bracket">[</span>15<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-gen_7-2" class="reference"><a href="#cite_note-gen-7"><span class="cite-bracket">[</span>7<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Pathophysiology">Pathophysiology</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Hyper_IgM_syndrome&action=edit&section=4" title="Edit section: Pathophysiology"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p><a href="/wiki/CD40" class="mw-redirect" title="CD40">CD40</a> is a co-stimulatory receptor on <a href="/wiki/B_cell" title="B cell">B cells</a> that, when bound to CD40 ligand (<a href="/wiki/CD40L" class="mw-redirect" title="CD40L">CD40L</a>), sends a signal to the <a href="/wiki/B-cell_receptor" title="B-cell receptor">B-cell receptor</a>.<sup id="cite_ref-16" class="reference"><a href="#cite_note-16"><span class="cite-bracket">[</span>16<span class="cite-bracket">]</span></a></sup> Defective interaction of CD40L-CD40 between <a href="/wiki/Cd4%2B_t_cells" class="mw-redirect" title="Cd4+ t cells">CD4+ T cells</a> and antigen presenting cells (APCs) is known as the underlying cause of HIGM syndromes. CD40L-CD40 interaction is the first step in B cell stimulation for class switch recombination (CSR) and <a href="/wiki/Somatic_hypermutation" title="Somatic hypermutation">somatic hyper mutation</a> (SHM) resulting in the generation of various Ig isotypes.<sup id="cite_ref-:0_8-1" class="reference"><a href="#cite_note-:0-8"><span class="cite-bracket">[</span>8<span class="cite-bracket">]</span></a></sup> Consequently, <a href="/wiki/Humoral_immune_response" class="mw-redirect" title="Humoral immune response">humoral immune response</a> is affected. Certain insults, usually from encapsulated bacteria and toxin, then have a greater opportunity to damage the body.<sup id="cite_ref-emed_1-3" class="reference"><a href="#cite_note-emed-1"><span class="cite-bracket">[</span>1<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Diagnosis">Diagnosis</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Hyper_IgM_syndrome&action=edit&section=5" title="Edit section: Diagnosis"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>The diagnosis of X-linked hyper IgM syndrome (HIGM1) is established in males with typical clinical and laboratory findings by identifying a hemizygous pathogenic variant in the CD40LG gene through molecular <a href="/wiki/Genetic_testing" title="Genetic testing">genetic testing</a>. In females, the diagnosis of HIGM1 is extremely rare. Heterozygous females are usually asymptomatic unless there is skewed X-chromosome inactivation.<sup id="cite_ref-:2_12-1" class="reference"><a href="#cite_note-:2-12"><span class="cite-bracket">[</span>12<span class="cite-bracket">]</span></a></sup> The diagnosis of hyper IgM syndrome can be done via the following methods and tests:<sup id="cite_ref-emed_1-4" class="reference"><a href="#cite_note-emed-1"><span class="cite-bracket">[</span>1<span class="cite-bracket">]</span></a></sup> </p> <ul><li><a href="/wiki/MRI" class="mw-redirect" title="MRI">MRI</a></li> <li>Chest <a href="/wiki/Radiography" title="Radiography">radiography</a></li> <li><a href="/wiki/Pulmonary_function_test" class="mw-redirect" title="Pulmonary function test">Pulmonary function test</a></li> <li><a href="/wiki/Lymph_node" title="Lymph node">Lymph node</a> test</li> <li>Flow Cytometry (evaluate the presence and function of certain immune cells, such as T cells and B cells)</li> <li>Genetic testing</li> <li>Blood test(Immunoglobulin levels, Antibody response, Complete blood count (CBC))</li></ul> <div class="mw-heading mw-heading2"><h2 id="Treatment">Treatment</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Hyper_IgM_syndrome&action=edit&section=6" title="Edit section: Treatment"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>The primary goal is to address the underlying defect in CD40L or other gene mutations causing HIGM. The potential for precise correction of the CD40LG gene in T cells and hematopoietic stem/progenitor cells (HSPC) to treat X-linked hyper-IgM Syndrome (HIGM1) is a promising avenue of research. However, the actual therapeutic efficacy of this approach is not yet fully understood and requires further investigation to determine its true potential. In addition to HSCT, supportive measures are crucial for managing infections and complications associated with HIGM. This may include <a href="/wiki/Antibiotic_prophylaxis" title="Antibiotic prophylaxis">antimicrobial prophylaxis</a>, immunoglobulin replacement therapy, and close monitoring of respiratory and gastrointestinal infections. Additionally, anti-microbial therapy, use of <a href="/wiki/Granulocyte_colony-stimulating_factor" title="Granulocyte colony-stimulating factor">granulocyte colony-stimulating factor</a>, <a href="/wiki/Immunosuppressants" class="mw-redirect" title="Immunosuppressants">immunosuppressants</a>, as well as other treatments, may be needed.<sup id="cite_ref-gen_7-3" class="reference"><a href="#cite_note-gen-7"><span class="cite-bracket">[</span>7<span class="cite-bracket">]</span></a></sup><sup id="cite_ref-17" class="reference"><a href="#cite_note-17"><span class="cite-bracket">[</span>17<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="Epidemiology">Epidemiology</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Hyper_IgM_syndrome&action=edit&section=7" title="Edit section: Epidemiology"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <p>All forms of hyper-IgM syndrome are rare. According to the US X-HIGM registry, the prevalence of X-linked hyper IgM syndrome (X-HIGM) during the period from 1984 to 1993 was approximately 1 in 1,000,000 live births. The estimated frequency of CD40L deficiency, a subtype of X-HIGM, is 2 in 1,000,000 in males. Limited data is available on the frequency of AICDA deficiency, another subtype of X-HIGM, but it is believed to affect less than 1 in 1,000,000 individuals. Globally, all forms of HIGM make up approximately 0.3% to 2.9% of all patients diagnosed with primary immunodeficiency disorders (PIDs).<sup id="cite_ref-:0_8-2" class="reference"><a href="#cite_note-:0-8"><span class="cite-bracket">[</span>8<span class="cite-bracket">]</span></a></sup> </p> <div class="mw-heading mw-heading2"><h2 id="See_also">See also</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Hyper_IgM_syndrome&action=edit&section=8" title="Edit section: See also"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <ul><li><a href="/wiki/X-linked_agammaglobulinemia" title="X-linked agammaglobulinemia">X-linked agammaglobulinemia</a></li> <li><a href="/wiki/Common_variable_immunodeficiency" title="Common variable immunodeficiency">Common variable immunodeficiency</a> (CVID)</li></ul> <div class="mw-heading mw-heading2"><h2 id="References">References</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Hyper_IgM_syndrome&action=edit&section=9" title="Edit section: References"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <style data-mw-deduplicate="TemplateStyles:r1239543626">.mw-parser-output .reflist{margin-bottom:0.5em;list-style-type:decimal}@media screen{.mw-parser-output .reflist{font-size:90%}}.mw-parser-output .reflist .references{font-size:100%;margin-bottom:0;list-style-type:inherit}.mw-parser-output .reflist-columns-2{column-width:30em}.mw-parser-output .reflist-columns-3{column-width:25em}.mw-parser-output .reflist-columns{margin-top:0.3em}.mw-parser-output .reflist-columns ol{margin-top:0}.mw-parser-output .reflist-columns li{page-break-inside:avoid;break-inside:avoid-column}.mw-parser-output .reflist-upper-alpha{list-style-type:upper-alpha}.mw-parser-output .reflist-upper-roman{list-style-type:upper-roman}.mw-parser-output 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a{background:url("//upload.wikimedia.org/wikipedia/commons/6/65/Lock-green.svg")right 0.1em center/9px no-repeat}.mw-parser-output .id-lock-limited.id-lock-limited a,.mw-parser-output .id-lock-registration.id-lock-registration a{background:url("//upload.wikimedia.org/wikipedia/commons/d/d6/Lock-gray-alt-2.svg")right 0.1em center/9px no-repeat}.mw-parser-output .id-lock-subscription.id-lock-subscription a{background:url("//upload.wikimedia.org/wikipedia/commons/a/aa/Lock-red-alt-2.svg")right 0.1em center/9px no-repeat}.mw-parser-output .cs1-ws-icon a{background:url("//upload.wikimedia.org/wikipedia/commons/4/4c/Wikisource-logo.svg")right 0.1em center/12px no-repeat}body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .id-lock-free a,body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .id-lock-limited a,body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .id-lock-registration a,body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .id-lock-subscription a,body:not(.skin-timeless):not(.skin-minerva) .mw-parser-output .cs1-ws-icon a{background-size:contain;padding:0 1em 0 0}.mw-parser-output .cs1-code{color:inherit;background:inherit;border:none;padding:inherit}.mw-parser-output .cs1-hidden-error{display:none;color:var(--color-error,#d33)}.mw-parser-output .cs1-visible-error{color:var(--color-error,#d33)}.mw-parser-output .cs1-maint{display:none;color:#085;margin-left:0.3em}.mw-parser-output .cs1-kern-left{padding-left:0.2em}.mw-parser-output .cs1-kern-right{padding-right:0.2em}.mw-parser-output .citation .mw-selflink{font-weight:inherit}@media screen{.mw-parser-output .cs1-format{font-size:95%}html.skin-theme-clientpref-night .mw-parser-output .cs1-maint{color:#18911f}}@media screen and (prefers-color-scheme:dark){html.skin-theme-clientpref-os .mw-parser-output .cs1-maint{color:#18911f}}</style><cite class="citation web cs1"><a rel="nofollow" class="external text" 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(eds.), <a rel="nofollow" class="external text" href="http://www.ncbi.nlm.nih.gov/books/NBK1402/">"X-Linked Hyper IgM Syndrome"</a>, <i>GeneReviews®</i>, Seattle (WA): University of Washington, Seattle, <a href="/wiki/PMID_(identifier)" class="mw-redirect" title="PMID (identifier)">PMID</a> <a rel="nofollow" class="external text" href="https://pubmed.ncbi.nlm.nih.gov/20301576">20301576</a><span class="reference-accessdate">, retrieved <span class="nowrap">22 June</span> 2023</span></cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.jtitle=GeneReviews%C2%AE&rft.atitle=X-Linked+Hyper+IgM+Syndrome&rft.date=1993&rft_id=info%3Apmid%2F20301576&rft.aulast=Dunn&rft.aufirst=Clinton+P.&rft.au=de+la+Morena%2C+M.+Teresa&rft_id=http%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fbooks%2FNBK1402%2F&rfr_id=info%3Asid%2Fen.wikipedia.org%3AHyper+IgM+syndrome" class="Z3988"></span></span> </li> <li id="cite_note-13"><span class="mw-cite-backlink"><b><a href="#cite_ref-13">^</a></b></span> <span class="reference-text"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFDaviesThrasher2016" class="citation journal cs1">Davies, E Graham; Thrasher, Adrian J (27 November 2016). <a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2855828">"Update on the hyper immunoglobulin M syndromes"</a>. <i>British Journal of Haematology</i>. <b>149</b> (2): 167–180. <a href="/wiki/Doi_(identifier)" class="mw-redirect" title="Doi (identifier)">doi</a>:<a rel="nofollow" class="external text" href="https://doi.org/10.1111%2Fj.1365-2141.2010.08077.x">10.1111/j.1365-2141.2010.08077.x</a>. <a href="/wiki/ISSN_(identifier)" class="mw-redirect" title="ISSN (identifier)">ISSN</a> <a rel="nofollow" class="external text" href="https://search.worldcat.org/issn/0007-1048">0007-1048</a>. <a href="/wiki/PMC_(identifier)" class="mw-redirect" title="PMC (identifier)">PMC</a> <span class="id-lock-free" title="Freely accessible"><a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2855828">2855828</a></span>. <a href="/wiki/PMID_(identifier)" class="mw-redirect" title="PMID (identifier)">PMID</a> <a rel="nofollow" class="external text" href="https://pubmed.ncbi.nlm.nih.gov/20180797">20180797</a>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.jtitle=British+Journal+of+Haematology&rft.atitle=Update+on+the+hyper+immunoglobulin+M+syndromes&rft.volume=149&rft.issue=2&rft.pages=167-180&rft.date=2016-11-27&rft_id=https%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fpmc%2Farticles%2FPMC2855828%23id-name%3DPMC&rft.issn=0007-1048&rft_id=info%3Apmid%2F20180797&rft_id=info%3Adoi%2F10.1111%2Fj.1365-2141.2010.08077.x&rft.aulast=Davies&rft.aufirst=E+Graham&rft.au=Thrasher%2C+Adrian+J&rft_id=https%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fpmc%2Farticles%2FPMC2855828&rfr_id=info%3Asid%2Fen.wikipedia.org%3AHyper+IgM+syndrome" class="Z3988"></span></span> </li> <li id="cite_note-nat-14"><span class="mw-cite-backlink"><b><a href="#cite_ref-nat_14-0">^</a></b></span> <span class="reference-text"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFEtzioniOchs2004" class="citation journal cs1">Etzioni, Amos; Ochs, Hans D. (1 October 2004). <a rel="nofollow" class="external text" href="https://doi.org/10.1203%2F01.PDR.0000139318.65842.4A">"The Hyper IgM Syndrome—An Evolving Story"</a>. <i>Pediatric Research</i>. <b>56</b> (4): 519–525. <a href="/wiki/Doi_(identifier)" class="mw-redirect" title="Doi (identifier)">doi</a>:<span class="id-lock-free" title="Freely accessible"><a rel="nofollow" class="external text" href="https://doi.org/10.1203%2F01.PDR.0000139318.65842.4A">10.1203/01.PDR.0000139318.65842.4A</a></span>. <a href="/wiki/ISSN_(identifier)" class="mw-redirect" title="ISSN (identifier)">ISSN</a> <a rel="nofollow" class="external text" href="https://search.worldcat.org/issn/0031-3998">0031-3998</a>. <a href="/wiki/PMID_(identifier)" class="mw-redirect" title="PMID (identifier)">PMID</a> <a rel="nofollow" class="external text" href="https://pubmed.ncbi.nlm.nih.gov/15319456">15319456</a>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.jtitle=Pediatric+Research&rft.atitle=The+Hyper+IgM+Syndrome%E2%80%94An+Evolving+Story&rft.volume=56&rft.issue=4&rft.pages=519-525&rft.date=2004-10-01&rft.issn=0031-3998&rft_id=info%3Apmid%2F15319456&rft_id=info%3Adoi%2F10.1203%2F01.PDR.0000139318.65842.4A&rft.aulast=Etzioni&rft.aufirst=Amos&rft.au=Ochs%2C+Hans+D.&rft_id=https%3A%2F%2Fdoi.org%2F10.1203%252F01.PDR.0000139318.65842.4A&rfr_id=info%3Asid%2Fen.wikipedia.org%3AHyper+IgM+syndrome" class="Z3988"></span></span> </li> <li id="cite_note-15"><span class="mw-cite-backlink"><b><a href="#cite_ref-15">^</a></b></span> <span class="reference-text"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFReference" class="citation web cs1">Reference, Genetics Home. <a rel="nofollow" class="external text" href="https://ghr.nlm.nih.gov/condition/x-linked-hyper-igm-syndrome">"X-linked hyper IgM syndrome"</a>. <i>Genetics Home Reference</i><span class="reference-accessdate">. Retrieved <span class="nowrap">27 November</span> 2016</span>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=unknown&rft.jtitle=Genetics+Home+Reference&rft.atitle=X-linked+hyper+IgM+syndrome&rft.aulast=Reference&rft.aufirst=Genetics+Home&rft_id=https%3A%2F%2Fghr.nlm.nih.gov%2Fcondition%2Fx-linked-hyper-igm-syndrome&rfr_id=info%3Asid%2Fen.wikipedia.org%3AHyper+IgM+syndrome" class="Z3988"></span></span> </li> <li id="cite_note-16"><span class="mw-cite-backlink"><b><a href="#cite_ref-16">^</a></b></span> <span class="reference-text"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFReference" class="citation web cs1">Reference, Genetics Home. <a rel="nofollow" class="external text" href="https://ghr.nlm.nih.gov/gene/CD40">"CD40 gene"</a>. <i>Genetics Home Reference</i><span class="reference-accessdate">. Retrieved <span class="nowrap">27 November</span> 2016</span>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=unknown&rft.jtitle=Genetics+Home+Reference&rft.atitle=CD40+gene&rft.aulast=Reference&rft.aufirst=Genetics+Home&rft_id=https%3A%2F%2Fghr.nlm.nih.gov%2Fgene%2FCD40&rfr_id=info%3Asid%2Fen.wikipedia.org%3AHyper+IgM+syndrome" class="Z3988"></span></span> </li> <li id="cite_note-17"><span class="mw-cite-backlink"><b><a href="#cite_ref-17">^</a></b></span> <span class="reference-text"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFVavassoriMercuriMarcovecchioCastiello2021" class="citation journal cs1">Vavassori, Valentina; Mercuri, Elisabetta; Marcovecchio, Genni E; Castiello, Maria C; Schiroli, Giulia; Albano, Luisa; Margulies, Carrie; Buquicchio, Frank; Fontana, Elena; Beretta, Stefano; Merelli, Ivan; Cappelleri, Andrea; Rancoita, Paola MV; Lougaris, Vassilios; Plebani, Alessandro (5 March 2021). <a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7933961">"Modeling, optimization, and comparable efficacy of T cell and hematopoietic stem cell gene editing for treating hyper‐IgM syndrome"</a>. <i>EMBO Molecular Medicine</i>. <b>13</b> (3): e13545. <a href="/wiki/Doi_(identifier)" class="mw-redirect" title="Doi (identifier)">doi</a>:<a rel="nofollow" class="external text" href="https://doi.org/10.15252%2Femmm.202013545">10.15252/emmm.202013545</a>. <a href="/wiki/ISSN_(identifier)" class="mw-redirect" title="ISSN (identifier)">ISSN</a> <a rel="nofollow" class="external text" href="https://search.worldcat.org/issn/1757-4676">1757-4676</a>. <a href="/wiki/PMC_(identifier)" class="mw-redirect" title="PMC (identifier)">PMC</a> <span class="id-lock-free" title="Freely accessible"><a rel="nofollow" class="external text" href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7933961">7933961</a></span>. <a href="/wiki/PMID_(identifier)" class="mw-redirect" title="PMID (identifier)">PMID</a> <a rel="nofollow" class="external text" href="https://pubmed.ncbi.nlm.nih.gov/33475257">33475257</a>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.jtitle=EMBO+Molecular+Medicine&rft.atitle=Modeling%2C+optimization%2C+and+comparable+efficacy+of+T+cell+and+hematopoietic+stem+cell+gene+editing+for+treating+hyper%E2%80%90IgM+syndrome&rft.volume=13&rft.issue=3&rft.pages=e13545&rft.date=2021-03-05&rft_id=https%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fpmc%2Farticles%2FPMC7933961%23id-name%3DPMC&rft.issn=1757-4676&rft_id=info%3Apmid%2F33475257&rft_id=info%3Adoi%2F10.15252%2Femmm.202013545&rft.aulast=Vavassori&rft.aufirst=Valentina&rft.au=Mercuri%2C+Elisabetta&rft.au=Marcovecchio%2C+Genni+E&rft.au=Castiello%2C+Maria+C&rft.au=Schiroli%2C+Giulia&rft.au=Albano%2C+Luisa&rft.au=Margulies%2C+Carrie&rft.au=Buquicchio%2C+Frank&rft.au=Fontana%2C+Elena&rft.au=Beretta%2C+Stefano&rft.au=Merelli%2C+Ivan&rft.au=Cappelleri%2C+Andrea&rft.au=Rancoita%2C+Paola+MV&rft.au=Lougaris%2C+Vassilios&rft.au=Plebani%2C+Alessandro&rft_id=https%3A%2F%2Fwww.ncbi.nlm.nih.gov%2Fpmc%2Farticles%2FPMC7933961&rfr_id=info%3Asid%2Fen.wikipedia.org%3AHyper+IgM+syndrome" class="Z3988"></span></span> </li> </ol></div></div> <div class="mw-heading mw-heading2"><h2 id="Further_reading">Further reading</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Hyper_IgM_syndrome&action=edit&section=10" title="Edit section: Further reading"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <ul><li><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFStroberGottesman2014" class="citation book cs1">Strober, Warren; Gottesman, Susan R. (2014). <a rel="nofollow" class="external text" href="https://books.google.com/books?id=mJltAwAAQBAJ&q=Hyper+IgM+syndrome&pg=PA65"><i>Immunology: Clinical Case Studies and Disease Pathophysiology</i></a>. John Wiley & Sons. <a href="/wiki/ISBN_(identifier)" class="mw-redirect" title="ISBN (identifier)">ISBN</a> <a href="/wiki/Special:BookSources/9781118966006" title="Special:BookSources/9781118966006"><bdi>9781118966006</bdi></a><span class="reference-accessdate">. Retrieved <span class="nowrap">27 November</span> 2016</span>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Abook&rft.genre=book&rft.btitle=Immunology%3A+Clinical+Case+Studies+and+Disease+Pathophysiology&rft.pub=John+Wiley+%26+Sons&rft.date=2014&rft.isbn=9781118966006&rft.aulast=Strober&rft.aufirst=Warren&rft.au=Gottesman%2C+Susan+R.&rft_id=https%3A%2F%2Fbooks.google.com%2Fbooks%3Fid%3DmJltAwAAQBAJ%26q%3DHyper%2BIgM%2Bsyndrome%26pg%3DPA65&rfr_id=info%3Asid%2Fen.wikipedia.org%3AHyper+IgM+syndrome" class="Z3988"></span></li> <li><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1238218222"><cite id="CITEREFKleinman2008" class="citation book cs1">Kleinman, Ronald (2008). <a rel="nofollow" class="external text" href="https://books.google.com/books?id=bG3nVUqIbegC&q=Hyper+IgM+syndrome&pg=PA877"><i>Walker's Pediatric Gastrointestinal Disease</i></a>. PMPH-USA. <a href="/wiki/ISBN_(identifier)" class="mw-redirect" title="ISBN (identifier)">ISBN</a> <a href="/wiki/Special:BookSources/9781550093643" title="Special:BookSources/9781550093643"><bdi>9781550093643</bdi></a><span class="reference-accessdate">. Retrieved <span class="nowrap">27 November</span> 2016</span>.</cite><span title="ctx_ver=Z39.88-2004&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Abook&rft.genre=book&rft.btitle=Walker%27s+Pediatric+Gastrointestinal+Disease&rft.pub=PMPH-USA&rft.date=2008&rft.isbn=9781550093643&rft.aulast=Kleinman&rft.aufirst=Ronald&rft_id=https%3A%2F%2Fbooks.google.com%2Fbooks%3Fid%3DbG3nVUqIbegC%26q%3DHyper%2BIgM%2Bsyndrome%26pg%3DPA877&rfr_id=info%3Asid%2Fen.wikipedia.org%3AHyper+IgM+syndrome" class="Z3988"></span></li></ul> <div class="mw-heading mw-heading2"><h2 id="External_links">External links</h2><span class="mw-editsection"><span class="mw-editsection-bracket">[</span><a href="/w/index.php?title=Hyper_IgM_syndrome&action=edit&section=11" title="Edit section: External links"><span>edit</span></a><span class="mw-editsection-bracket">]</span></span></div> <div class="navbox-styles"><style data-mw-deduplicate="TemplateStyles:r1236075235">.mw-parser-output .navbox{box-sizing:border-box;border:1px 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.navbox{display:none!important}}</style><style data-mw-deduplicate="TemplateStyles:r1129693374">.mw-parser-output .hlist dl,.mw-parser-output .hlist ol,.mw-parser-output .hlist ul{margin:0;padding:0}.mw-parser-output .hlist dd,.mw-parser-output .hlist dt,.mw-parser-output .hlist li{margin:0;display:inline}.mw-parser-output .hlist.inline,.mw-parser-output .hlist.inline dl,.mw-parser-output .hlist.inline ol,.mw-parser-output .hlist.inline ul,.mw-parser-output .hlist dl dl,.mw-parser-output .hlist dl ol,.mw-parser-output .hlist dl ul,.mw-parser-output .hlist ol dl,.mw-parser-output .hlist ol ol,.mw-parser-output .hlist ol ul,.mw-parser-output .hlist ul dl,.mw-parser-output .hlist ul ol,.mw-parser-output .hlist ul ul{display:inline}.mw-parser-output .hlist .mw-empty-li{display:none}.mw-parser-output .hlist dt::after{content:": "}.mw-parser-output .hlist dd::after,.mw-parser-output .hlist li::after{content:" · ";font-weight:bold}.mw-parser-output .hlist dd:last-child::after,.mw-parser-output .hlist dt:last-child::after,.mw-parser-output .hlist li:last-child::after{content:none}.mw-parser-output .hlist dd dd:first-child::before,.mw-parser-output .hlist dd dt:first-child::before,.mw-parser-output .hlist dd li:first-child::before,.mw-parser-output .hlist dt dd:first-child::before,.mw-parser-output .hlist dt dt:first-child::before,.mw-parser-output .hlist dt li:first-child::before,.mw-parser-output .hlist li dd:first-child::before,.mw-parser-output .hlist li dt:first-child::before,.mw-parser-output .hlist li li:first-child::before{content:" (";font-weight:normal}.mw-parser-output .hlist dd dd:last-child::after,.mw-parser-output .hlist dd dt:last-child::after,.mw-parser-output .hlist dd li:last-child::after,.mw-parser-output .hlist dt dd:last-child::after,.mw-parser-output .hlist dt dt:last-child::after,.mw-parser-output .hlist dt li:last-child::after,.mw-parser-output .hlist li dd:last-child::after,.mw-parser-output .hlist li dt:last-child::after,.mw-parser-output .hlist li li:last-child::after{content:")";font-weight:normal}.mw-parser-output .hlist ol{counter-reset:listitem}.mw-parser-output .hlist ol>li{counter-increment:listitem}.mw-parser-output .hlist ol>li::before{content:" "counter(listitem)"\a0 "}.mw-parser-output .hlist dd ol>li:first-child::before,.mw-parser-output .hlist dt ol>li:first-child::before,.mw-parser-output .hlist li ol>li:first-child::before{content:" ("counter(listitem)"\a0 "}</style><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1129693374"></div><div role="navigation" class="navbox" aria-label="Navbox" style="width:100%; margin:0.5em 0 0.5em 0;;padding:3px"><table class="nowraplinks navbox-inner" style="border-spacing:0;background:transparent;color:inherit"><tbody><tr><th scope="row" class="navbox-group" style="width:1%;background: #EAECF0;color:black;">Classification</th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"><div style="position:relative; float:right; font-size:0.8em;"><a href="https://www.wikidata.org/wiki/Q1617658" class="extiw" title="d:Q1617658">D</a></div><div class="hlist" style="text-align:left;"><ul><li><b><a href="/wiki/International_Statistical_Classification_of_Diseases_and_Related_Health_Problems" class="mw-redirect" title="International Statistical Classification of Diseases and Related Health Problems">ICD</a>-<a href="/wiki/ICD-10" title="ICD-10">10</a></b>: <a rel="nofollow" class="external text" href="https://icd.who.int/browse10/2019/en#/D80.5">D80.5</a></li><li><b><a href="/wiki/International_Statistical_Classification_of_Diseases_and_Related_Health_Problems" class="mw-redirect" title="International Statistical Classification of Diseases and Related Health Problems">ICD</a>-<a href="/wiki/List_of_ICD-9_codes" title="List of ICD-9 codes">9-CM</a></b>: <a rel="nofollow" class="external text" href="http://www.icd9data.com/getICD9Code.ashx?icd9=279.05">279.05</a></li><li><b><a href="/wiki/Medical_Subject_Headings" title="Medical Subject Headings">MeSH</a></b>: <a rel="nofollow" class="external text" href="https://meshb.nlm.nih.gov/record/ui?ui=D053306">D053306</a></li></ul></div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%;background: #EAECF0;color:black;">External resources</th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"><div class="hlist" style="text-align:left;"><ul><li><b><a href="/wiki/EMedicine" title="EMedicine">eMedicine</a></b>: <a rel="nofollow" class="external text" href="https://emedicine.medscape.com/ped/2457-overview">ped/2457</a></li></ul></div></div></td></tr></tbody></table></div> <style data-mw-deduplicate="TemplateStyles:r1235681985">.mw-parser-output .side-box{margin:4px 0;box-sizing:border-box;border:1px solid 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href="mw-data:TemplateStyles:r1129693374"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1236075235"></div><div role="navigation" class="navbox" aria-labelledby="Lymphoid_and_complement_disorders_causing_immunodeficiency" style="padding:3px"><table class="nowraplinks mw-collapsible autocollapse navbox-inner" style="border-spacing:0;background:transparent;color:inherit"><tbody><tr><th scope="col" class="navbox-title" colspan="2"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1129693374"><style data-mw-deduplicate="TemplateStyles:r1239400231">.mw-parser-output .navbar{display:inline;font-size:88%;font-weight:normal}.mw-parser-output .navbar-collapse{float:left;text-align:left}.mw-parser-output .navbar-boxtext{word-spacing:0}.mw-parser-output .navbar ul{display:inline-block;white-space:nowrap;line-height:inherit}.mw-parser-output .navbar-brackets::before{margin-right:-0.125em;content:"[ "}.mw-parser-output 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class="nv-talk"><a href="/wiki/Template_talk:Lymphoid_and_complement_immunodeficiency" title="Template talk:Lymphoid and complement immunodeficiency"><abbr title="Discuss this template">t</abbr></a></li><li class="nv-edit"><a href="/wiki/Special:EditPage/Template:Lymphoid_and_complement_immunodeficiency" title="Special:EditPage/Template:Lymphoid and complement immunodeficiency"><abbr title="Edit this template">e</abbr></a></li></ul></div><div id="Lymphoid_and_complement_disorders_causing_immunodeficiency" style="font-size:114%;margin:0 4em"><a href="/wiki/Lymphatic_system" title="Lymphatic system">Lymphoid</a> and <a href="/wiki/Complement_system" title="Complement system">complement</a> disorders causing <a href="/wiki/Immunodeficiency" title="Immunodeficiency">immunodeficiency</a></div></th></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Primary_immunodeficiency" title="Primary immunodeficiency">Primary</a></th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Antibody" title="Antibody">Antibody</a>/<a href="/wiki/Humoral_immune_deficiency" title="Humoral immune deficiency">humoral</a><br />(<a href="/wiki/B_cell" title="B cell">B</a>)</th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Hypogammaglobulinemia" title="Hypogammaglobulinemia">Hypogammaglobulinemia</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/X-linked_agammaglobulinemia" title="X-linked agammaglobulinemia">X-linked agammaglobulinemia</a></li> <li><a href="/wiki/Transient_hypogammaglobulinemia_of_infancy" title="Transient hypogammaglobulinemia of infancy">Transient hypogammaglobulinemia of infancy</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Dysgammaglobulinemia" title="Dysgammaglobulinemia">Dysgammaglobulinemia</a></th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Selective_immunoglobulin_A_deficiency" title="Selective immunoglobulin A deficiency">IgA deficiency</a></li> <li><a href="/wiki/IgG_deficiency" title="IgG deficiency">IgG deficiency</a></li> <li><a href="/wiki/Immunoglobulin_M_deficiency" class="mw-redirect" title="Immunoglobulin M deficiency">IgM deficiency</a></li> <li><a class="mw-selflink selflink">Hyper IgM syndrome</a> (<a href="/wiki/Hyper-IgM_syndrome_type_1" title="Hyper-IgM syndrome type 1">1</a></li> <li><a href="/wiki/Hyper-IgM_syndrome_type_2" title="Hyper-IgM syndrome type 2">2</a></li> <li><a href="/wiki/Hyper-IgM_syndrome_type_3" title="Hyper-IgM syndrome type 3">3</a></li> <li><a href="/wiki/Hyper-IgM_syndrome_type_4" title="Hyper-IgM syndrome type 4">4</a></li> <li><a href="/wiki/Hyper-IgM_syndrome_type_5" title="Hyper-IgM syndrome type 5">5</a>)</li> <li><a href="/wiki/Wiskott%E2%80%93Aldrich_syndrome" title="Wiskott–Aldrich syndrome">Wiskott–Aldrich syndrome</a></li> <li><a href="/wiki/Hyperimmunoglobulin_E_syndrome" title="Hyperimmunoglobulin E syndrome">Hyper-IgE syndrome</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Other</th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Common_variable_immunodeficiency" title="Common variable immunodeficiency">Common variable immunodeficiency</a></li> <li><a href="/wiki/ICF_syndrome" class="mw-redirect" title="ICF syndrome">ICF syndrome</a></li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/T_cell_deficiency" title="T cell deficiency">T cell deficiency</a><br />(<a href="/wiki/T_cell" title="T cell">T</a>)</th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><i><a href="/wiki/Thymic_hypoplasia" title="Thymic hypoplasia">thymic hypoplasia</a>:</i> <i>hypoparathyroid</i> (<a href="/wiki/DiGeorge_syndrome" title="DiGeorge syndrome">Di George's syndrome</a>)</li> <li><i>euparathyroid</i> (<a href="/wiki/Nezelof_syndrome" title="Nezelof syndrome">Nezelof syndrome</a></li> <li><a href="/wiki/Ataxia%E2%80%93telangiectasia" title="Ataxia–telangiectasia">Ataxia–telangiectasia</a>)</li></ul> <p><i>peripheral:</i> <a href="/wiki/Purine_nucleoside_phosphorylase_deficiency" title="Purine nucleoside phosphorylase deficiency">Purine nucleoside phosphorylase deficiency</a> </p> <ul><li><a class="mw-selflink selflink">Hyper IgM syndrome</a> (<a href="/wiki/Hyper-IgM_syndrome_type_1" title="Hyper-IgM syndrome type 1">1</a>)</li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Severe_combined_immunodeficiency" title="Severe combined immunodeficiency">Severe</a> <a href="/wiki/Combined_immunodeficiencies" title="Combined immunodeficiencies">combined</a><br />(B+T)</th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><i>x-linked:</i> <a href="/wiki/X-linked_severe_combined_immunodeficiency" title="X-linked severe combined immunodeficiency">X-SCID</a><br /><i>autosomal:</i> <a href="/wiki/Adenosine_deaminase_deficiency" title="Adenosine deaminase deficiency">Adenosine deaminase deficiency</a></li> <li><a href="/wiki/Omenn_syndrome" title="Omenn syndrome">Omenn syndrome</a></li> <li><a href="/wiki/ZAP70_deficiency" title="ZAP70 deficiency">ZAP70 deficiency</a></li> <li><a href="/wiki/Bare_lymphocyte_syndrome" title="Bare lymphocyte syndrome">Bare lymphocyte syndrome</a></li></ul> </div></td></tr></tbody></table><div></div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Immunodeficiency#acquired" title="Immunodeficiency">Acquired</a></th><td class="navbox-list-with-group navbox-list navbox-even hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/HIV/AIDS" title="HIV/AIDS">HIV/AIDS</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Leukopenia" title="Leukopenia">Leukopenia</a>:<br /><a href="/wiki/Lymphocytopenia" title="Lymphocytopenia">Lymphocytopenia</a></th><td class="navbox-list-with-group navbox-list navbox-odd hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Idiopathic_CD4%2B_lymphocytopenia" title="Idiopathic CD4+ lymphocytopenia">Idiopathic CD4+ lymphocytopenia</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Complement_deficiency" title="Complement deficiency">Complement<br />deficiency</a></th><td class="navbox-list-with-group navbox-list navbox-even hlist" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><i><a href="/wiki/C1-inhibitor" title="C1-inhibitor">C1-inhibitor</a></i> (<a href="/wiki/Angioedema" title="Angioedema">Angioedema</a>/<a href="/wiki/Hereditary_angioedema" title="Hereditary angioedema">Hereditary angioedema</a>)</li> <li><a href="/wiki/Complement_2_deficiency" title="Complement 2 deficiency">Complement 2 deficiency</a>/<a href="/wiki/Complement_4_deficiency" title="Complement 4 deficiency">Complement 4 deficiency</a></li> <li><a href="/wiki/MBL_deficiency" title="MBL deficiency">MBL deficiency</a></li> <li><a href="/wiki/Properdin_deficiency" title="Properdin deficiency">Properdin deficiency</a></li> <li><a href="/wiki/Complement_3_deficiency" title="Complement 3 deficiency">Complement 3 deficiency</a></li> <li><a href="/wiki/Terminal_complement_pathway_deficiency" title="Terminal complement pathway deficiency">Terminal complement pathway deficiency</a></li> <li><a href="/wiki/Paroxysmal_nocturnal_hemoglobinuria" title="Paroxysmal nocturnal hemoglobinuria">Paroxysmal nocturnal hemoglobinuria</a></li> <li><a href="/wiki/Complement_receptor_deficiency" class="mw-redirect" title="Complement receptor deficiency">Complement receptor deficiency</a></li></ul> </div></td></tr></tbody></table></div> <div class="navbox-styles"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1129693374"><link rel="mw-deduplicated-inline-style" href="mw-data:TemplateStyles:r1236075235"></div><div role="navigation" class="navbox" aria-labelledby="Medicine" style="padding:3px"><table class="nowraplinks hlist mw-collapsible autocollapse 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href="/wiki/Medical_specialty" title="Medical specialty">Specialties</a><br />and<br /><a href="/wiki/Subspecialty" title="Subspecialty">subspecialties</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"></div><table class="nowraplinks navbox-subgroup" style="border-spacing:0"><tbody><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Surgery" title="Surgery">Surgery</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Cardiac_surgery" title="Cardiac surgery">Cardiac surgery</a></li> <li><a href="/wiki/Cardiothoracic_surgery" title="Cardiothoracic surgery">Cardiothoracic surgery</a></li> <li><a href="/wiki/Endocrine_surgery" title="Endocrine surgery">Endocrine surgery</a></li> <li><a href="/wiki/Eye_surgery" title="Eye surgery">Eye surgery</a></li> <li><a href="/wiki/General_surgery" title="General surgery">General surgery</a> <ul><li><a href="/wiki/Colorectal_surgery" title="Colorectal surgery">Colorectal surgery</a></li> <li><a href="/wiki/Digestive_system_surgery" title="Digestive system surgery">Digestive system surgery</a></li></ul></li> <li><a href="/wiki/Neurosurgery" title="Neurosurgery">Neurosurgery</a></li> <li><a href="/wiki/Oral_and_maxillofacial_surgery" title="Oral and maxillofacial surgery">Oral and maxillofacial surgery</a></li> <li><a href="/wiki/Orthopedic_surgery" title="Orthopedic surgery">Orthopedic surgery</a></li> <li><a href="/wiki/Hand_surgery" title="Hand surgery">Hand surgery</a></li> <li><a href="/wiki/Otorhinolaryngology" title="Otorhinolaryngology">Otolaryngology</a> <ul><li><small>ENT</small></li></ul></li> <li><a href="/wiki/Pediatric_surgery" title="Pediatric surgery">Pediatric surgery</a></li> <li><a href="/wiki/Plastic_surgery" title="Plastic surgery">Plastic surgery</a></li> <li><a href="/wiki/Reproductive_surgery" title="Reproductive surgery">Reproductive surgery</a></li> <li><a href="/wiki/Surgical_oncology" title="Surgical oncology">Surgical oncology</a></li> <li><a href="/wiki/Organ_transplantation" title="Organ transplantation">Transplant surgery</a></li> <li><a href="/wiki/Trauma_surgery" title="Trauma surgery">Trauma surgery</a></li> <li><a href="/wiki/Urology" title="Urology">Urology</a> <ul><li><a href="/wiki/Andrology" title="Andrology">Andrology</a></li></ul></li> <li><a href="/wiki/Vascular_surgery" title="Vascular surgery">Vascular surgery</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Internal_medicine" title="Internal medicine">Internal<br />medicine</a></th><td class="navbox-list-with-group navbox-list navbox-even" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Allergy" title="Allergy">Allergy</a> / <a href="/wiki/Immunology" title="Immunology">Immunology</a></li> <li><a href="/wiki/Angiology" title="Angiology">Angiology</a></li> <li><a href="/wiki/Cardiology" title="Cardiology">Cardiology</a></li> <li><a href="/wiki/Endocrinology" title="Endocrinology">Endocrinology</a></li> <li><a href="/wiki/Gastroenterology" title="Gastroenterology">Gastroenterology</a> <ul><li><a href="/wiki/Hepatology" title="Hepatology">Hepatology</a></li></ul></li> <li><a href="/wiki/Geriatrics" title="Geriatrics">Geriatrics</a></li> <li><a href="/wiki/Hematology" title="Hematology">Hematology</a></li> <li><a href="/wiki/Hospital_medicine" title="Hospital medicine">Hospital medicine</a></li> <li><a href="/wiki/Infectious_diseases_(medical_specialty)" title="Infectious diseases (medical specialty)">Infectious diseases</a></li> <li><a href="/wiki/Nephrology" title="Nephrology">Nephrology</a></li> <li><a href="/wiki/Oncology" title="Oncology">Oncology</a></li> <li><a href="/wiki/Pulmonology" title="Pulmonology">Pulmonology</a></li> <li><a href="/wiki/Rheumatology" title="Rheumatology">Rheumatology</a></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%"><a href="/wiki/Obstetrics_and_gynaecology" title="Obstetrics and gynaecology">Obstetrics and<br />gynaecology</a></th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Gynaecology" title="Gynaecology">Gynaecology</a></li> <li><a href="/wiki/Gynecologic_oncology" title="Gynecologic oncology">Gynecologic oncology</a></li> <li><a href="/wiki/Maternal%E2%80%93fetal_medicine" title="Maternal–fetal medicine">Maternal–fetal medicine</a></li> <li><a href="/wiki/Obstetrics" title="Obstetrics">Obstetrics</a></li> <li><a href="/wiki/Reproductive_endocrinology_and_infertility" title="Reproductive endocrinology and infertility">Reproductive endocrinology and infertility</a></li> <li><a href="/wiki/Urogynecology" title="Urogynecology">Urogynecology</a></li></ul> </div></td></tr><tr><th 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title="Cytopathology">Cytopathology</a></li> <li><a href="/wiki/Medical_microbiology" title="Medical microbiology">Medical microbiology</a></li> <li><a href="/wiki/Transfusion_medicine" title="Transfusion medicine">Transfusion medicine</a></li></ul></li></ul> </div></td></tr><tr><th scope="row" class="navbox-group" style="width:1%">Other</th><td class="navbox-list-with-group navbox-list navbox-odd" style="width:100%;padding:0"><div style="padding:0 0.25em"> <ul><li><a href="/wiki/Addiction_medicine" title="Addiction medicine">Addiction medicine</a></li> <li><a href="/wiki/Adolescent_medicine" title="Adolescent medicine">Adolescent medicine</a></li> <li><a href="/wiki/Anesthesiology" title="Anesthesiology">Anesthesiology</a> <ul><li><a href="/wiki/Obstetric_anesthesiology" title="Obstetric anesthesiology">Obstetric anesthesiology</a></li> <li><a href="/wiki/Neurosurgical_anesthesia" title="Neurosurgical anesthesia">Neurosurgical anesthesiology</a></li></ul></li> <li><a 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