40 Questions
What is the most common clinical feature associated with IgA deficiency?
Recurrent otitis media
Which of the following autoimmune diseases is more commonly seen in individuals with IgA deficiency?
Rheumatoid arthritis
What potentially deadly complication can occur post-blood transfusion in individuals with IgA deficiency?
Life-threatening anaphylaxis
Which immunoglobulin is typically deficient in patients with Hyper-IgM syndrome?
IgG
What genetic inheritance pattern is associated with Hyper-IgM syndrome due to a CD40L deficit or lack of function?
X-linked recessive
What is the most common deficiency in complement defects?
C2 deficiency
Which cytokine receptors are affected in X-linked SCID due to mutation in the gamma-chain?
IL-2 and IL-7
What is the consequence of adenosine deaminase mutation in autosomal recessive SCID?
Accumulation of deoxyadenosine, toxic to rapidly dividing lymphocytes
What pathology is commonly seen in SCID patients?
Depletion of T-cell areas in lymphatic tissues
What condition may occur in SCID if the mother's T-cells are transferred across the placenta?
Graft-versus-host disease
Which virulence factor produced by Staphylococcus aureus disrupts the membranes of a large number of cell types?
Leukocydin
What is the primary function of Epidermal Cell Differentiation Inhibitors produced by Staphylococcus aureus?
Produces holes in blood vessel linings
Which type of toxins from Staphylococcus aureus is also known as Toxic Shock Syndrome Toxin?
Exfoliative toxins
What type of immunodeficiency is primarily genetic and often detected in infancy or childhood?
Primary Immunodeficiencies
Immunodeficiencies that are acquired due to external factors like infection or chemotherapy are classified as:
Secondary Immunodeficiencies
Which of the following statements about X-linked agammaglobulinemia (Bruton's agammaglobulinemia) is correct?
It is caused by a defect in the ability of Pro-B cells to differentiate into Pre-B cells due to the lack of a tyrosine kinase that initiates recombination and antibody formation.
Which of the following statements about common variable immunodeficiency (CVID) is incorrect?
It is a single, well-defined disease with a known genetic cause.
What is the approximate prevalence of isolated IgA deficiency in the population?
1 in 600
Which of the following primary immunodeficiencies is the most common among B-cell deficiencies?
Isolated IgA deficiency
Which of the following is the most appropriate treatment for X-linked agammaglobulinemia (Bruton's agammaglobulinemia)?
Intravenous immunoglobulin (IVIG) therapy
What molecule is considered most important in the pathogenesis of hereditary angioedema?
Bradykinin
Which of the following is NOT a clinical feature of hereditary angioedema?
Myocardial infarction
What is the mechanism behind the increased risk of infections with prolonged systemic glucocorticoid use?
Suppression of the immune system
Which of the following is the most accurate statement regarding the prevalence of hereditary angioedema?
It is a relatively uncommon disorder, affecting around 1 in 50,000 individuals.
Which of the following statements about the treatment and prognosis of hereditary angioedema is correct?
Treatment with C1 inhibitor from blood products has greatly improved the prognosis, with mortality rates now less than 5%.
What is the relative risk of bacterial infections in IBD patients on corticosteroids alone?
5-fold higher
Which infection has a 4-fold higher relative risk in IBD patients on corticosteroids alone?
Tuberculosis
What is the specific infection mentioned in relation to Pneumocystis jiroveci pneumonia?
Fungal infection of the lung
Which infection can persist for several decades and reactivate with glucocorticoid exposure?
Strongyloidiasis
What is the recommended treatment for Strongyloidiasis?
Antiparasitic medication
Which virus causes Herpes Zoster (Shingles)?
Varicella zoster virus
What infection should be tested for before initiating glucocorticoid treatment?
Tuberculosis
Which type of infection can be acquired through direct contact with contaminated soil?
Parasitic infection
Which opportunistic infections are mentioned in the text?
Aspergillosis, Tuberculosis, Candidiasis, Cryptococcosis
What type of prophylactic or treatment approach is recommended for Pneumocystis jiroveci pneumonia?
Antibiotics
In patients with X-linked hyper-IgM syndrome, which of the following is a characteristic finding?
Increased susceptibility to pyogenic infections and viral infections
In 22q11 deletion (DiGeorge) syndrome, which of the following is the most likely underlying cause?
Deficiency in the T-box family of transcription factors important for branchial/pharyngeal arch development
Which of the following clinical features is NOT associated with 22q11 deletion (DiGeorge) syndrome?
Elevated levels of parathyroid hormone and hypercalcemia
What is the estimated prevalence of X-linked hyper-IgM syndrome?
1 in 1,000,000 live births
Which of the following electrolyte abnormalities would be associated with hypoparathyroidism, a feature of 22q11 deletion (DiGeorge) syndrome?
Hypocalcemia
Learn about the complex disorder known as Severe Combined Immunodeficiency (SCID), which is often diagnosed in childhood. Explore the symptoms, treatment options, and prognosis associated with this condition.
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