What Is Primary Immunodeficiency?
General information on primary immunodeficiencies, in which the immune system is congenitally weak, their warning signs, diagnosis and the importance of specialist care.
Definition
Primary immunodeficiencies are a group of diseases in which one or more components of the immune system are congenitally missing or underactive. More than three hundred distinct types have been described. They differ from acquired (secondary) immune weakness; their basis is inherited. Symptoms usually begin in childhood, but some types first appear in adulthood.
- Type
- A group of congenital immune system diseases
- Main finding
- Frequent, severe or unusual infections
- Diagnosis
- Blood tests and specialist assessment
- Follow-up
- Regular care with an immunologist
Warning signs
The key clue is the frequency, severity and unusual course of infections. The main warning signs that call for medical assessment are: four or more ear infections in a year, two or more episodes of sinusitis or pneumonia in a year, two months or more of antibiotics with little effect, recurrent deep skin or organ abscesses, failure of an infant to gain weight and grow normally, persistent oral thrush, and a family history of primary immunodeficiency.
Diagnosis
Diagnosis usually begins with simple blood tests measuring the counts of immune cells and the levels of protective proteins. Where needed, advanced tests and genetic studies are performed at specialist centres. Early diagnosis matters: with proper care, infection frequency can be reduced and organ damage prevented.
Follow-up and daily life
Care is coordinated by an immunologist, who sets the treatment plan according to the type of disease. In daily life, hand hygiene, following the vaccination schedule recommended by the physician, balanced nutrition and early consultation at signs of infection are important. Early assessment can be planned for newborns in families with a diagnosis.
This page is intended as general health information; for diagnosis and treatment, please consult your physician.
Frequently asked questions
Does every child who is often ill have an immunodeficiency?
No. Several upper respiratory infections a year are normal at nursery and school age. What raises concern is severe course, poor response to treatment and unusual pathogens. Assessment belongs to the physician.
Can primary immunodeficiency be treated?
It varies by type, but treatment approaches that support the immune system exist, and with proper care patients' quality of life can be well preserved. The treatment plan belongs to the immunologist.
Does it occur in adults?
Yes. Some types first present in adulthood; recurrent sinusitis and pneumonia in adults also warrant assessment.
Can vaccines be given?
The decision depends on the type of disease; some vaccines are recommended while others may not be suitable. The schedule must be planned together with the treating specialist.
Aymed İlaç Sanayi manufactures authorised medicinal products. This page does not contain diagnostic or treatment advice; it is intended as general health information.