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Primary immunodeficiency (PID) is still defined as a rare disease, but it is estimated that it affects up to 1 in 400 people worldwide. The essence of primary immune deficiencies is the disturbance of the immune system. They cause frequent and severe viral and bacterial infections in children and adults. What are the causes and symptoms of primary immunodeficiencies? What is the treatment? These and other questions are answered by Dr. Hanna Suchanek, an immunologist at the University Clinical Center in Gdańsk.

Primary Immunodeficiency(PID) is a group of over 250 diseases, the essence of which is the malfunctioning of some elements of the immune system. As a result, patients with primary immunodeficiency disease are much more likely to develop severe infections and suffer from many chronic diseases, mainly of the respiratory tract. The biggest problem in Poland is still low awareness of the existence of PID, and thus - a large number of undiagnosed patients. Untreated immunodeficiencies can lead to serious chronic complications and even death.

Primary Immunodeficiencies - Causes

Primary immunodeficiencies are a group of genetic diseases.

Primary Immunodeficiencies - Symptoms

Some severe forms of primary immunodeficiency disease have symptoms that become apparent soon after birth. An example of such a condition is Di George syndrome, which causes facial distortions, heart disease and problems with the nervous system, which are seen at birth. - Primary immunodeficiencies can also manifest as autoimmune diseases, when the body is attacked by its own immune system. This is manifested, for example, by a decrease in the number of platelets, arthritis or vitiligo - explains Dr. Hanna Suchanek, an immunologist at the University Clinical Center in Gdańsk.

Serious infections are most often the first suspicions of PNO. These are usually severe, recurring infections that are difficult to treat despite the use of antibiotics. If we suffer from otitis, sinusitis, bronchitis or lung inflammation again during the year, we should not underestimate it, because it may bea signal that our immune system is not functioning properly.

Worth knowing

Although primary immunodeficiencies are congenital, they can be diagnosed in both children and adults, as symptoms may not always appear immediately. - In some, especially lighter cases, patients live with the disease many times for many years before appropriate tests allow to make a proper diagnosis - explains Adrian Górecki, president of the Association for people with immunodeficiency "Immunoprotect".

Primary Immunodeficiencies - Diagnosis

In a situation where PID is suspected, more detailed investigations of the immune system are performed. - They are usually done gradually so as to rule out the most severe forms of PID at the outset, such asSevere Combined Immunodeficiency(SCID). First, a complete blood count with a smear is performed, and then - if necessary - the levels of immunoglobulins are determined and other specialized tests are performed - says Dr. Suchanek.

A complete blood count is a major indicator of an abnormality related to the immune system, but it is not sufficient for an accurate diagnosis of primary immunodeficiency. Rather, blood count results are an indication for more accurate tests for specific types of cells. Such tests are usually performed by immunologists.

According to an expertHanna Suchanek, MD, PhD, immunologist from the University Clinical Center in Gdańsk

If a patient is diagnosed with an immunodeficiency, appropriate treatment can be started. Although PID cannot be completely cured, effective treatments improve the he alth of those who are sick, significantly reduce the number of infections, and allow them to lead an active life, develop a career, and have a family. The most commonly used is replacement immunoglobulin treatment, i.e. subcutaneous or intravenous administration of human immunoglobulin preparations, in most patients throughout their lives.

Primary Immunodeficiency - Treatment

For many primary immunodeficiencies, the body produces too little or no immunoglobulin. The administration of immunoglobulins is the most frequently used form of therapy because it allows to achieve the desired, safe level of antibodies. Thanks to this, it is possible to protect the body against a number of infections and reduce the severity of autoimmune symptoms.

Many people with PIDs also need antibiotics to helpprevent and treat infections. Antibiotics are effective against infections caused by bacteria. Sometimes it is also necessary to use other classes of drugs to fight infections caused by fungi (e.g. thrush) or viruses (e.g. chicken pox). For the most severe immunodeficiencies, such as SCID, bone marrow transplantation is used.

Usually, primary immunodeficiency patients have to take medication for the rest of their lives, but this does not interfere with their normal daily functioning. - A person with PID can and should lead a normal life. There are no contraindications for going to school, studying or working. Of course, you must remember to take certain precautions to minimize the risk of infection, but that's basically all - concludes Adrian Górecki.

Worth knowing

If you suspect you have a primary immunodeficiency, visit www.immunoprotect.pl and see where you can find the nearest specialist PNO diagnosis and treatment center.

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Immune system