A healthy organism is protected by immune system cells from viral, fungal and bacterial attacks, allergens and other unfavorable factors. Primary immunodeficiency deprives a person of this barrier from the first years of life, but can manifest itself in adulthood. This disease requires constant monitoring by a specialist and a very long treatment.
Classification of primary congenital immunodeficiencies
The pathology under consideration is of 5 kinds, which are caused by insufficiency:
1. Insufficiency of cellular immunity:
- oroticide;
- the syndrome of Di Georgi;
- biotin-dependent fermentopathy;
- insufficiency of purinucleoside phosphorylase.
2. Phagocytic primary immunodeficiency:
- chronic granulomatous disease;
- cyclic and chronic acyclic neutropenia;
- Miller's syndrome ("lazy leukocytes".
3. Insufficiency of humoral cells:
- late immune start;
- Bruton's syndrome;
- selective deficiency of immunoglobulins.
4. Combined deficiency of cellular and humoral immunity:
- Wiskott-Aldrich syndrome;
- hypocalcemia ;
- reticular dysgenesis;
- the Glanzmann-Riniker syndrome.
5. Complementary failure:
- Quincke-Osler's edema;
- lupus erythematosus;
- rheumatoid arthritis.
Symptoms of primary immunodeficiency
There are no characteristic signs that allow to accurately reveal the described genetic pathology. Clinical manifestations are very diverse depending on the type, shape and severity of the disease.
To suspect primary immunodeficiency it is possible on such signs:
- susceptibility to frequent infectious and viral diseases;
- the minimal effect of taking antibiotics;
- candidiasis of skin with relapses;
- exacerbated abscesses;
- pneumonia 2 and more times a year;
- permanent otitis, sinusitis, bronchitis.
Treatment of primary immunodeficiency
Therapy is difficult, because you can not cure pathology. To improve the quality of life of patients, constant immunosubstitutional treatment with immunoglobulins is necessary, as well as careful selection of antibacterial, antiviral and antimycotic agents for infections.
Radical therapy of the described disease consists in bone marrow transplantation, which is better performed at a young age. But it is worth noting that this operation is very expensive, and sometimes it is difficult to find a donor with sufficient compatibility.