The main symptoms of the STONE.
General variability of immune deficiency (barn) can be both for men and for women. In some patients, symptoms occur during the first years of life, but in many patients they can occur only at the age of 10-30 years or even later.
The main manifestation of STODOL in most patients is recurrent ears, paranasal sinuses, nose, bronchi and lungs. With significant severity and frequency of lung infections, irreversible damage to the bronchi (respiratory tract) may occur, with the development of chronic changes - enlargement and cicatricial changes - of these structures. Such a condition is called bronchiectasis.
These infections are usually caused by bacteria, commonly found in humans and often cause pneumonia (Haemophilus influenzae, pneumococci and staphylococci). Treatment of pulmonary infections is aimed at preventing their recurrence and concomitant chronic lung tissue injury. Regular cough in the morning with the release of yellow or green sputum can be a manifestation of chronic infection or bronchiectasis (enlargement, cicatricial changes and inflammation of the bronchi).
At STODOL can be enlarged cervical, chest or abdominal lymph nodes. The exact cause is unknown, but an increase in lymph nodes may be caused by infection, a violation of immune regulation, or a combination of these factors. Similarly, an enlargement of the spleen is often observed, as well as increased nodal accumulation of lymphocytes in the intestine walls, called plaque plaques.
Despite the fact that STODOL has reduced the formation of antibodies and low levels of immunoglobulins in the blood (hypogammaglobulinemia), some antibodies produced by the body of these patients can attack their own tissues (autoantibodies). These autoantibodies can attack and destroy blood cells (eg, red blood cells, leukocytes or platelets). Although in most patients the first manifestation of STODOL is a recurrent bacterial infection, about 20% of immune deficiency is first detected as a significant reduction in platelet count or even severe anemia due to the destruction of red blood cells.
Autoantibodies can also cause arthritis or endocrine disorders, such as thyroid disease. In some STUDOL patients who do not receive sufficient substitution therapy with immunoglobulins, a painful inflammation of one or more joints can also develop. This condition is called polyarthritis.
In most of these cases, the joint fluid does not contain bacteria. To make sure that there is no curable infection in the joints, you can use a needle to sample an intraarticulate fluid and examine it for the presence of bacteria.
In some cases that are difficult to diagnose, bacteria of the genus Mycoplasma may be the cause of this condition. Typically, arthritis associated with STOMOD may include large joints, such as knee, ankle, elbow and joints of the hands. Smaller joints (eg, joints of the fingers) are rarely affected.
The symptoms of inflammation of the joints usually disappear with sufficient immunoglobulin therapy and the correct administration of antibiotics. However, in some patients, arthritis can be observed even with sufficient substitution therapy with immunoglobulins.
In some patients, STODOL there are complaints of gastrointestinal disorders such as abdominal pain, increased gas formation, nausea, diarrhea and weight loss. A thorough examination of the digestive system may indicate a violation of absorption of fat and some sugars. If you take a small fragment (biopsy) of the intestinal mucosa, characteristic changes can be detected. These changes help diagnose and treat this disorder.
Some patients with impaired digestive tract function in a biopsy material and in the analysis of feces find a microscopic parasite - giardia giardia. Destroying this parasite with medicines can eliminate gastrointestinal disorders.
Finally, when STODOL can increase the risk of developing cancer, especially cancer of the lymphoid system, the skin and the gastrointestinal tract. In the absence of complications of the STONE, it is not accompanied by physical disturbances. Some STDOL patients may have enlarged spleen and lymph nodes.
With the development of chronic lung injury, the patient's physical capacity and lung volume (the maximum amount of air that can be collected arbitrarily in the lungs) may decrease. Defeat of the gastrointestinal tract may, in some cases, affect the normal growth of children or cause weight loss in adults.
The article is kindly provided by the world-wide organization IPOPI, which works to improve the lives of people with primary immunodeficiency. Copyright 2007 belongs to the Immune Deficiency Foundation, USA. The "Manual for Primary Immunodeficiency Diseases for Patients and Their Families", from which this material was taken under license, was developed by the Immune Deficiency Foundation with the support of Baxter Healthcare Corporation.