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Understanding Severe Primary Immunodeficiency, Its Causes, Management, and More

A primary immunodeficiency is a group of disorders that affect the immune system. The cause is a defect in one of more than 100 genes responsible for building resistant system components. In general, these diseases result in an inability to produce enough antibodies or white blood cells that can prevent and fight off germs. In addition, children with primary immunodeficiency diseases (PI) are at greater risk for frequent bacterial, fungal, parasitic, and viral infections.

Early signs and symptoms
The leading causes of primary immunodeficiencies are mutations in several genes that encode proteins involved in cell-mediated immunity (CMI). This genetic defect often leads to recurring bacterial, fungal, parasitic, or viral infections that can be fatal if left untreated.

Children with primary immunodeficiency diseases
Several types of primary immunodeficiencies affect different parts of a child’s immune system, including T-cells; B cells; macrophages; natural killer cells; dendritic cells; T-cell receptors; cytokine receptors; cell adhesion molecules. One of the most common signs is a history of repeated infection, especially in infancy and early childhood. The severity of immunodeficiency will determine how often one experiences infections.
The signs and symptoms include fever or chills, loss of appetite or poor growth, poor healing from injuries due to easy bruising or bleeding (especially from minor cuts), ear infections, sinus infections, pneumonia, bronchitis, meningitis, and skin infections. Among adults, the signs last longer and are hard to treat, and they do not respond to antibiotics or require IV antibiotics.

Diagnosis
In addition to clinical presentation and family history, laboratory tests are required to diagnose primary immunodeficiency disease. Blood tests can be used to determine the level of antibodies that your child has against common infections. The levels may vary depending on the type of infection and your child’s immune system status at the time they were tested.
A variety of other tests are available that check for specific genes associated with primary immunodeficiencies, such as X-linked agammaglobulinemia (XLA), Bruton tyrosine kinase deficiency (BTKD), Wiskott-Aldrich syndrome (WAS) and DiGeorge syndrome (DGS).
Primary immunodeficiencies are a general term that encompasses a set of more than 350 different genetic disorders that affect the immune system. The cause is a defect in one of more than 100 genes responsible for building resistant system components.
The immune system protects us from harmful substances that may cause diseases, such as bacteria, fungi, parasites, and viruses.

Prevention
It is noted that severe primary immunodeficiencies can be prevented by following the steps, such as washing hands often, maintaining dental hygiene, being physically active, eating healthy and balanced meals, getting enough sleep, maintaining a safe distance from people with colds, fevers and other infectious diseases, and consulting a doctor to make sure you get the suitable vaccine.

Treatment options
Treatment options depend on the type of primary immunodeficiency disease. The goal is to reduce additional complications caused by the infections and prevent them from recurring. Antibiotics, antivirals, and antifungals treat infections in patients with primary immunodeficiencies.
Remedies such as immunoglobulin replacement therapy and interferon-gamma therapy which help your immune system work better, are good for treating the condition.
Immunoglobulin replacement therapy is a medical treatment that replaces the antibodies in your blood, which is required for normal immune function and is self-administered. The first step in giving yourself immunoglobulin therapy is preparing the solution. You will need to dilute the reconstituted product with equal saline (salt water) or water. You can use a calibrated syringe, needle, or a 0-30 ml graduated cylinder with a 1 ml graduation line.

Other remedies
New methods such as stem cell transplant and gene therapy are becoming helpful in treating severe primary immunodeficiencies. Since the first stem cell transplantation in patients with severe immunodeficiency, many people have benefitted from treatment. As this requires a donor, there has also been an increase in donors.
In Gene therapy, the functional copy of the defective gene is restored in the patient’s stem cell. This therapy does not require a donor.

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