Primary Immunodeficiency Diseases (PIDD) Quiz

Primary immunodeficiency diseases are caused by inherited defects in a person’s immune function that lead to clinical disease. They can affect anyone at any age.

Question 1
True or false: Primary immunodeficiency diseases are rare conditions.
True
False

False: Selective IgA deficiency, a type of primary immunodeficiency disease, is more common than cystic fibrosis and sickle cell disease in African-American individuals, two conditions that many more people recognize. The true incidence and prevalence of most primary immunodeficiency diseases are not known because very often the diagnosis is not considered.

Question 2
People who have a primary immunodeficiency disease are most at risk for which of the following?
A. Heart disease
B. Cancer
C. Diabetes
D. Infections

D: One of the primary roles of the immune system is to defend the body against pathogens that cause infections. Immune deficiencies lead to weaknesses in that defense.

Question 3
Frequent or recurrent types of which of the following infections may strongly indicate the presence of a primary immunodeficiency disease?
A. Streptococcal pharyngitis ("strep throat")
B. Ear and sinus infections
C. Urinary tract infections
D. Viral upper respiratory tract infections

B: Recurrent ear and sinus infections are two warning signs of primary immunodeficiency. The other types of infections are not classic hallmarks of primary immunodeficiency diseases.

Question 4
Which of the following would be most worrisome for the presence of a primary immunodeficiency disease consisting of a problem with T lymphocyte function?
A. Recurrent ear, sinus and lung infections
B. Recurrent thrush (an infection of the mouth caused by yeast) or no thymus (a small organ in your upper chest, under your breastbone) on chest x-ray
C. Recurrent skin abscesses or poor wound healing
D. Recurrent warts

B: T lymphocytes are critical for protection, especially against a number of fungal and viral pathogens. These immune cells develop within the thymus gland, and absence of a thymus on a chest x-ray in a young infant can be a sign of missing T lymphocytes. Congenital absence of T lymphocytes is a medical emergency and must be treated immediately.

Question 5
Which of the following would be most worrisome for the presence of a primary immunodeficiency disease consisting of a problem with neutrophil function?
A. Recurrent ear, sinus and lung infections
B. Recurrent thrush, no thymus on chest x-ray
C. Recurrent skin abscesses or poor wound healing
D. Recurrent warts

C: Neutrophils engulf and digest bacteria and other types of organisms. They also play an important role in wound healing. Patients with chronic granulomatous disease, for example, develop recurrent abscesses of the skin if they are not adequately treated.

Question 6
My child has a very high serum IgE level. I should be concerned that he or she may have hyper-IgE syndrome if he or she also has which of the following?
A. Asthma
B. Eczema
C. Skin abscesses
D. Low platelet count

C: Very high serum IgE levels can be seen in patients with allergic diseases, such as asthma and eczema. Hyper-IgE syndrome is marked by susceptibility to infections, resulting in recurrent skin abscesses. Low platelet counts and elevated IgE levels can be found in another primary immunodeficiency disease, Wiskott-Aldrich syndrome.

Question 7
True or false: My doctor measured my child's immunoglobulin levels, and they are normal. That means that he or she does not have a primary immunodeficiency disease.
True
False

False: Patients with neutrophil defects typically have normal or sometimes even elevated levels of immunoglobulins. It is important to also note that having normal overall quantities of immunoglobulins does not necessarily mean that a person has normal antibody function.

Question 8
Which of the following tests might a doctor use to evaluate for defective B lymphocyte function in my child?
A. Measurement of antibody levels in the blood
B. Respiratory burst assay
C. Skin testing to Candida and/or mumps
D. Newborn screening

A: B lymphocytes make antibodies, which are especially important for defending against infections at mucosal surfaces, such as the ones along the respiratory and gastrointestinal tracts. Patients who cannot make antibodies have a primary immunodeficiency disease. While newborn screening tests have been developed and approved to look for T lymphocyte deficiency, no newborn screening tests are available at this time to evaluate for B lymphocyte defects.

Question 9
Which of the following tests might a doctor use to evaluate for defective T lymphocyte function?
A. Measurement of chloride levels in the sweat
B. Respiratory burst assay
C. Skin testing to Candida (yeast) and/or mumps
D. Ciliary biopsy

C: Skin tests to Candida and mumps can be used much in the same way as testing for tuberculosis is performed to assess for the presence of normal T lymphocyte function. The other tests do not evaluate T lymphocyte function. Elevated sweat chloride levels are found in cystic fibrosis, the respiratory burst assay is a test of neutrophil function, and ciliary biopsy is performed to assess for primary ciliary dyskinesia.

Question 10
True or false: All patients with primary immunodeficiency diseases require immunoglobulin replacement therapy to prevent infections.
True
False

False: Patients with neutrophil defects do not require immunoglobulin replacement therapy unless they have defective antibody function. Some patients with antibody deficiencies can be managed simply with long-term courses of antibiotics.

Question 11
True or false: My child has a primary immunodeficiency disease. That means that I need to keep him or her isolated to protect him or her from getting infections.
True
False

False: A patient who is properly managed and treated should not require isolation from public activities.

Question 12
True or false: Primary immunodeficiency diseases are incurable.
True
False

False: Many primary immunodeficiency diseases can now be cured by stem cell transplantation. New therapies, such as gene therapy and thymus transplantation, are actively being developed as techniques to permanently treat other specific primary immunodeficiency diseases.

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Learn more about primary immunodeficiency diseases symptoms, diagnosis, treatment and management.

 

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