The most common treatment for<unk>  is an intravenous infusion of immunoglobulin (IVIg, human IgG antibodies) every week, for life. IVIg is a human product extracted and pooled from thousands of blood donations. IVIg does not cure<unk>  but increases the patient's lifespan and quality of life, by generating passive immunity, and boosting the immune system. With treatment, the number and severity of infections is reduced. With IVIg,<unk>  patients may live a relatively healthy life. A patient should attempt reaching a state where his IgG blood count exceeds 800 mg/kg. The dose is based on the patient's weight and IgG blood-count. Muscle injections of immunoglobulin (IMIg) were common before IVIg was prevalent, but are less effective and much more painful; hence, IMIg is now uncommon. Subcutaneous treatment (SCIg) was recently approved by the U.S. Food and Drug Administration (FDA), which is recommended in cases of severe adverse reactions to the IVIg treatment. Antibiotics are another common supplementary treatment. Local antibiotic treatment (drops, lotions) are preferred over systemic treatment (pills) for long-term treatment, if possible. One of the future prospects of<unk>  treatment is gene therapy, which could potentially cure<unk> . Gene therapy technology is still in its infancy and may cause severe complications such as cancer and even death. Moreover, the long-term success and complications of this treatment are, as yet, unknown. Other considerations It is not recommended and dangerous for<unk>  patients to receive live attenuated vaccines such as live polio, or the measles, mumps, rubella (MMR vaccine). Special emphasis is given to avoiding the oral live attenuated SABIN-type polio vaccine that has been reported to cause polio to<unk>  patients. Furthermore, it is not known if active vaccines in general have any beneficial effect on<unk>  patients as they lack normal ability to maintain immune memory.<unk>  patients are specifically susceptible to viruses of the Enterovirus family, and mostly to: polio virus, coxsackie virus (hand, foot, and mouth disease) and Echoviruses. These may cause severe central nervous system conditions as chronic encephalitis, meningitis and death. An experimental anti-viral agent, pleconaril, is active against picornaviruses.<unk>  patients, however, are apparently immune to the Epstein-Barr virus (EBV), as they lack mature B cells (and so HLA co-receptors) needed for the viral infection. Patients with<unk>  are also more likely to have a history of septic arthritis. It is not known if<unk>  patients are able to generate an allergic reaction, as they lack functional IgE antibodies. There is no special hazard for<unk>  patients in dealing with pets or outdoor activities. Unlike in other primary immunodeficiencies<unk>  patients are at no greater risk for developing autoimmune illnesses.<unk>  <unk>  is similar to the primary immunodeficiency disorder Hypogammaglobulinemia (CVID), and their clinical conditions and treatment are almost identical. However, while<unk>  is a congenital disorder, with known genetic causes, CVID may occur in adulthood and its causes are not yet understood. In addition, to<unk>  a couple of autosomal recessive<unk>  gene mutations have been described including mutations in IGHM, IGLL1, CD79A/B, BLNK and deletion of the deletion of the terminal 14q32.33 chromosom.<unk>  was also historically mistaken as Severe Combined Immunodeficiency (SCID), a much more severe immune deficiency ("Bubble boys").A strain of laboratory mouse,<unk> , is used to study<unk> . These mice have a mutated version of the mouse Btk gene, and exhibit a similar, yet milder, immune deficiency as in<unk> .
X-linked agammaglobulinemia
Severe combined immunodeficiency
X-linked severe combined immunodeficiency
Common variable immunodeficiency
Combined immunodeficiencies
Primary immunodeficiency
Selective immunoglobulin A deficiency
Transient hypogammaglobulinemia of infancy