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X-linked Agammaglobulinemia and Common Variable Immunodeficiency

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X-linked Agammaglobulinemia and Common Variable Immunodeficiency

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Questions and Answers

What is the main reason behind the lack of B-cells in X-linked agammaglobulinemia (Bruton’s)?

Deficiency in the production of antibodies

Excessive production of light chains inhibiting B-cell development

Overproduction of T-cells leading to suppression of B-cells

Inability of pro-B cells to differentiate into pre-B cells due to tyrosine kinase deficiency (correct)

Which condition is characterized by hypogammaglobulinemia, reduced lymphocytes, and decreased T-cell activity?

Common variable immunodeficiency (correct)

Isolated IgA deficiency

X-linked agammaglobulinemia (Bruton’s)

Hypogammaglobulinemia

What is a common symptom associated with Isolated IgA deficiency?

Otitis media (correct)

Arthritis

Kidney failure

Heart disease

Why can a celiac test result show a false negative in a patient with IgA deficiency?

Low IgA levels in blood serum affect the test accuracy Signup and view all the answers

What is the primary biological defect in Hyper IgM syndrome?

Deficiency or lack of function of CD40L Signup and view all the answers

Which immune deficiency can result in life-threatening anaphylaxis post blood transfusion due to recognition of transfused IgA as foreign?

Isolated IgA deficiency Signup and view all the answers

Which of the following is true about hyper IgM syndrome?

It is an autosomal recessive disorder that primarily affects males Signup and view all the answers

What is the prognosis for hyper IgM syndrome?

Poor prognosis Signup and view all the answers

DiGeorge syndrome is also known as:

22q11 deletion syndrome Signup and view all the answers

What is the primary biological cause of DiGeorge syndrome?

Abnormal development of the 3rd and 4th pharyngeal pouches, leading to a small thymus and T-cell deficiency Signup and view all the answers

What is the primary immunodeficiency associated with severe combined immunodeficiency (SCID)?

Mutation in cytokine receptors or the RAG gene, leading to a lack of T lymphocytes and NK cells Signup and view all the answers

Which of the following is an example of an innate immunodeficiency?

Hereditary angioedema Signup and view all the answers