Severe Combined Immunodeficiency (SCID): Symptoms and Treatment
40 Questions
0 Views

Choose a study mode

Play Quiz
Study Flashcards
Spaced Repetition
Chat to lesson

Podcast

Play an AI-generated podcast conversation about this lesson

Questions and Answers

What is the most common clinical feature associated with IgA deficiency?

  • Recurrent cellulitis
  • Recurrent otitis media (correct)
  • Chronic sinus congestion
  • Chronic diarrhea

    • Which of the following autoimmune diseases is more commonly seen in individuals with IgA deficiency?

  • Graves' disease
  • Rheumatoid arthritis (correct)
  • Multiple sclerosis
  • Type 1 diabetes

    • What potentially deadly complication can occur post-blood transfusion in individuals with IgA deficiency?

  • Sepsis
  • Acute kidney injury
  • Severe anemia
  • Life-threatening anaphylaxis (correct)

    • Which immunoglobulin is typically deficient in patients with Hyper-IgM syndrome?

    <p>IgG</p> Signup and view all the answers

    What genetic inheritance pattern is associated with Hyper-IgM syndrome due to a CD40L deficit or lack of function?

    <p>X-linked recessive</p> Signup and view all the answers

    What is the most common deficiency in complement defects?

    <p>C2 deficiency</p> Signup and view all the answers

    Which cytokine receptors are affected in X-linked SCID due to mutation in the gamma-chain?

    <p>IL-2 and IL-7</p> Signup and view all the answers

    What is the consequence of adenosine deaminase mutation in autosomal recessive SCID?

    <p>Accumulation of deoxyadenosine, toxic to rapidly dividing lymphocytes</p> Signup and view all the answers

    What pathology is commonly seen in SCID patients?

    <p>Depletion of T-cell areas in lymphatic tissues</p> Signup and view all the answers

    What condition may occur in SCID if the mother's T-cells are transferred across the placenta?

    <p>Graft-versus-host disease</p> Signup and view all the answers

    Which virulence factor produced by Staphylococcus aureus disrupts the membranes of a large number of cell types?

    <p>Leukocydin</p> Signup and view all the answers

    What is the primary function of Epidermal Cell Differentiation Inhibitors produced by Staphylococcus aureus?

    <p>Produces holes in blood vessel linings</p> Signup and view all the answers

    Which type of toxins from Staphylococcus aureus is also known as Toxic Shock Syndrome Toxin?

    <p>Exfoliative toxins</p> Signup and view all the answers

    What type of immunodeficiency is primarily genetic and often detected in infancy or childhood?

    <p>Primary Immunodeficiencies</p> Signup and view all the answers

    Immunodeficiencies that are acquired due to external factors like infection or chemotherapy are classified as:

    <p>Secondary Immunodeficiencies</p> Signup and view all the answers

    Which of the following statements about X-linked agammaglobulinemia (Bruton's agammaglobulinemia) is correct?

    <p>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.</p> Signup and view all the answers

    Which of the following statements about common variable immunodeficiency (CVID) is incorrect?

    <p>It is a single, well-defined disease with a known genetic cause.</p> Signup and view all the answers

    What is the approximate prevalence of isolated IgA deficiency in the population?

    <p>1 in 600</p> Signup and view all the answers

    Which of the following primary immunodeficiencies is the most common among B-cell deficiencies?

    <p>Isolated IgA deficiency</p> Signup and view all the answers

    Which of the following is the most appropriate treatment for X-linked agammaglobulinemia (Bruton's agammaglobulinemia)?

    <p>Intravenous immunoglobulin (IVIG) therapy</p> Signup and view all the answers

    What molecule is considered most important in the pathogenesis of hereditary angioedema?

    <p>Bradykinin</p> Signup and view all the answers

    Which of the following is NOT a clinical feature of hereditary angioedema?

    <p>Myocardial infarction</p> Signup and view all the answers

    What is the mechanism behind the increased risk of infections with prolonged systemic glucocorticoid use?

    <p>Suppression of the immune system</p> Signup and view all the answers

    Which of the following is the most accurate statement regarding the prevalence of hereditary angioedema?

    <p>It is a relatively uncommon disorder, affecting around 1 in 50,000 individuals.</p> Signup and view all the answers

    Which of the following statements about the treatment and prognosis of hereditary angioedema is correct?

    <p>Treatment with C1 inhibitor from blood products has greatly improved the prognosis, with mortality rates now less than 5%.</p> Signup and view all the answers

    What is the relative risk of bacterial infections in IBD patients on corticosteroids alone?

    <p>5-fold higher</p> Signup and view all the answers

    Which infection has a 4-fold higher relative risk in IBD patients on corticosteroids alone?

    <p>Tuberculosis</p> Signup and view all the answers

    What is the specific infection mentioned in relation to Pneumocystis jiroveci pneumonia?

    <p>Fungal infection of the lung</p> Signup and view all the answers

    Which infection can persist for several decades and reactivate with glucocorticoid exposure?

    <p>Strongyloidiasis</p> Signup and view all the answers

    What is the recommended treatment for Strongyloidiasis?

    <p>Antiparasitic medication</p> Signup and view all the answers

    Which virus causes Herpes Zoster (Shingles)?

    <p>Varicella zoster virus</p> Signup and view all the answers

    What infection should be tested for before initiating glucocorticoid treatment?

    <p>Tuberculosis</p> Signup and view all the answers

    Which type of infection can be acquired through direct contact with contaminated soil?

    <p>Parasitic infection</p> Signup and view all the answers

    Which opportunistic infections are mentioned in the text?

    <p>Aspergillosis, Tuberculosis, Candidiasis, Cryptococcosis</p> Signup and view all the answers

    What type of prophylactic or treatment approach is recommended for Pneumocystis jiroveci pneumonia?

    <p>Antibiotics</p> Signup and view all the answers

    In patients with X-linked hyper-IgM syndrome, which of the following is a characteristic finding?

    <p>Increased susceptibility to pyogenic infections and viral infections</p> Signup and view all the answers

    In 22q11 deletion (DiGeorge) syndrome, which of the following is the most likely underlying cause?

    <p>Deficiency in the T-box family of transcription factors important for branchial/pharyngeal arch development</p> Signup and view all the answers

    Which of the following clinical features is NOT associated with 22q11 deletion (DiGeorge) syndrome?

    <p>Elevated levels of parathyroid hormone and hypercalcemia</p> Signup and view all the answers

    What is the estimated prevalence of X-linked hyper-IgM syndrome?

    <p>1 in 1,000,000 live births</p> Signup and view all the answers

    Which of the following electrolyte abnormalities would be associated with hypoparathyroidism, a feature of 22q11 deletion (DiGeorge) syndrome?

    <p>Hypocalcemia</p> Signup and view all the answers

    More Like This

    Use Quizgecko on...
    Browser
    Open
    Browser
    Browser