Chronic Granulomatous Disease (CGD) Quiz
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Questions and Answers

What is the consequence of abnormal oxidase activity in phagocytes?

  • Impaired bacterial killing (correct)
  • Enhanced chemotaxis
  • Increased production of reactive oxygen species
  • Normal respiratory burst

    • Which of the following microorganisms is associated with recurrent suppurative infections in individuals with impaired phagocytic activity?

  • Klebsiella pneumoniae
  • Streptococcus pneumoniae
  • Burkholderia cepacian (correct)
  • Escherichia coli

    • What is the result of increased respiratory burst activity in individuals with myeloperoxidase deficiency?

  • Impaired chemotaxis
  • Compensated bacterial killing (correct)
  • Reduced production of reactive oxygen species
  • Enhanced NADPH oxidase activity

    • What is the function of myeloperoxidase in phagocytes?

    <p>Bacterial killing</p> Signup and view all the answers

    What is the result of a negative Nitroblue Tetrazolium (NBT) screening test?

    <p>Impaired respiratory burst</p> Signup and view all the answers

    Which of the following is a characteristic of myeloperoxidase deficiency?

    <p>Absence of myeloperoxidase enzyme from neutrophils and monocytes</p> Signup and view all the answers

    What is the consequence of impaired NADPH oxidase activity in phagocytes?

    <p>Impaired bacterial killing</p> Signup and view all the answers

    What is the primary defect in Chronic Granulomatous Disease (CGD) that leads to impaired phagocytic activity?

    <p>Decreased ability of phagocytes to produce superoxide and reactive oxygen species</p> Signup and view all the answers

    Which of the following is a direct consequence of a mutation in the NADPH oxidase system in CGD?

    <p>Inability to form hydrogen peroxide</p> Signup and view all the answers

    What is the purpose of the NADPH oxidase enzyme in normal phagocytic cells?

    <p>To form hydrogen peroxide and reactive oxygen species</p> Signup and view all the answers

    Which of the following is a characteristic of Lazy Leukocyte Syndrome?

    <p>Defective release of cells from the bone marrow</p> Signup and view all the answers

    What is the result of a genetic defect in the NADPH oxidase system in CGD?

    <p>Inability of neutrophils to generate an oxidative burst</p> Signup and view all the answers

    What is the primary function of the NADPH oxidase enzyme in normal phagocytic cells?

    <p>To form hydrogen peroxide and reactive oxygen species</p> Signup and view all the answers

    Which test is used to diagnose Chronic Granulomatous Disease (CGD)?

    <p>Nitroblue Tetrazolium Reduction Test</p> Signup and view all the answers

    What is the primary defect in the neutrophils of individuals with Schwachman Syndrome?

    <p>Abnormal actin filament structure</p> Signup and view all the answers

    What is the primary function of NADPH oxidase in phagocytic cells?

    <p>To generate an oxidative burst to kill engulfed microorganisms</p> Signup and view all the answers

    What is the consequence of mutations in the genes responsible for NADPH oxidase proteins?

    <p>Inability of phagocytes to produce superoxide and reactive oxygen species</p> Signup and view all the answers

    What is the significance of the Nitroblue Tetrazolium (NBT) test in diagnosing phagocytic disorders?

    <p>It determines the ability of neutrophils to reduce NBT to formazan</p> Signup and view all the answers

    What is the primary consequence of defective chemotaxis in neutrophils?

    <p>Impaired mobility and directed migration</p> Signup and view all the answers

    Which of the following is a characteristic feature of catalase-positive microorganisms?

    <p>They are resistant to killing by reactive oxygen species</p> Signup and view all the answers

    What is the primary mechanism by which NADPH oxidase produces reactive oxygen species?

    <p>By producing hydrogen peroxide from molecular oxygen</p> Signup and view all the answers

    What is the primary problem in individuals with MPO deficiency?

    <p>Absence of oxidative destruction of microbes</p> Signup and view all the answers

    What is the effect of MPO deficiency on bacterial killing?

    <p>Bacterial killing is slowed but complete</p> Signup and view all the answers

    In which of the following conditions is MPO deficiency commonly seen?

    <p>Acute and Chronic Leukemia</p> Signup and view all the answers

    What is the genetic basis of CHS disease?

    <p>Mutation in the CHS1 LYST gene on chr 1q42.1-2</p> Signup and view all the answers

    What is the effect of CHS disease on lysosomes?

    <p>Abnormal lysosomes cannot fuse with phagosomes</p> Signup and view all the answers

    What is a common symptom of CHS disease?

    <p>Partial albinism (oculocutaneous)</p> Signup and view all the answers

    What is the relationship between NADPH oxidase and reactive oxygen species production?

    <p>NADPH oxidase produces reactive oxygen species</p> Signup and view all the answers

    What is the effect of NADPH oxidase mutations on phagocytic activity?

    <p>Phagocytic activity is impaired</p> Signup and view all the answers

    What is the effect of microfilament structure on chemotaxis?

    <p>Microfilament structure enhances chemotaxis</p> Signup and view all the answers

    What is the typical time frame for hemoglobin levels to return to normal after initiating adequate treatment?

    <p>2 months</p> Signup and view all the answers

    What can a failure to respond to iron treatment indicate?

    <p>Occult bleeding or inadequate absorption</p> Signup and view all the answers

    Why is it essential to continue iron therapy for another 3-4 months?

    <p>To prevent a relapse</p> Signup and view all the answers

    What is reflected in the peripheral blood film and indices for several months?

    <p>Biphasic population including microcytic cells</p> Signup and view all the answers

    What should be investigated if the patient has been adherent to the therapeutic regimen but fails to respond to iron treatment?

    <p>Hereditary causes of iron deficiency</p> Signup and view all the answers

    When does the anticipated rise in hemoglobin typically appear?

    <p>2-3 weeks</p> Signup and view all the answers

    What is the eventual outcome of the normocytic population?

    <p>It eventually predominates</p> Signup and view all the answers

    What is the goal of maintaining mild anemia in patients with hereditary hemochromatosis?

    <p>To prevent iron overload</p> Signup and view all the answers

    Why can't individuals who rely on transfusions to maintain Hgb levels be treated with phlebotomy?

    <p>Because transfusions are required to maintain Hgb levels</p> Signup and view all the answers

    What is the purpose of using iron-chelating drugs like deferoxamine?

    <p>To remove excess iron from the body</p> Signup and view all the answers

    How often should maintenance phlebotomy be performed in patients with hereditary hemochromatosis?

    <p>For life</p> Signup and view all the answers

    What is the typical volume of blood removed during each phlebotomy treatment session?

    <p>500 mL</p> Signup and view all the answers

    What is the duration of deferoxamine infusion pump treatment?

    <p>8-12 hours</p> Signup and view all the answers

    Why are oral iron chelators being developed?

    <p>All of the above</p> Signup and view all the answers

    What is the primary reason for taking iron supplements on an empty stomach?

    <p>To maximize absorption of the supplement</p> Signup and view all the answers

    In which cases is intravenous administration of iron dextrans typically used?

    <p>When intestinal absorption of iron is impaired</p> Signup and view all the answers

    What is the typical duration of iron replacement therapy?

    <p>6 months or longer</p> Signup and view all the answers

    When can a response to iron treatment be expected?

    <p>Within 2 days of treatment</p> Signup and view all the answers

    What is the significance of reticulocyte counts in assessing treatment response?

    <p>They increase within 5-10 days of treatment</p> Signup and view all the answers

    In which situations are RBC transfusions rarely warranted?

    <p>When patients have uncomplicated iron deficiency</p> Signup and view all the answers

    What is the advantage of using oral bovine lactoferrin for iron supplementation?

    <p>It has reduced intestinal side effects compared to ferrous sulfate</p> Signup and view all the answers

    What is the primary goal of treating any underlying contributing cause of iron deficiency?

    <p>To replenish the body's iron stores</p> Signup and view all the answers

    What is the effect of iron deficiency on red blood cell production?

    <p>Slowed production</p> Signup and view all the answers

    What is a characteristic of polychromatic normoblasts in iron-deficient bone marrow?

    <p>Nuclear-cytoplasmic asynchrony</p> Signup and view all the answers

    What is the standard prescription for iron supplementation?

    <p>Ferrous sulfate</p> Signup and view all the answers

    What is a characteristic of iron-deficient bone marrow initially?

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

    What is the reason for performing a bone marrow examination for iron deficiency?

    <p>When a bone marrow specimen is collected for other reasons</p> Signup and view all the answers

    What is the effect of iron supplementation on the body's iron stores?

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

    What is the significance of soluble transferrin receptor (sTfR) levels in iron deficiency?

    <p>They increase progressively with iron deficiency</p> Signup and view all the answers

    What is the effect of iron deficiency on the appearance of cell membranes?

    <p>They become irregular and shaggy</p> Signup and view all the answers

    What is the primary function of the Dense Tubular System in platelets?

    <p>To sequester calcium ions</p> Signup and view all the answers

    Which of the following events occurs during platelet activation?

    <p>Uptake of extracellular substances</p> Signup and view all the answers

    What is the purpose of platelet aggregation?

    <p>To form a temporary plug at the site of injury</p> Signup and view all the answers

    Which of the following coagulation factors interacts on the platelet surface to produce fibrin?

    <p>Factor X</p> Signup and view all the answers

    What is the role of the Open Canalicular System (SCCS) in platelets?

    <p>To uptake extracellular substances</p> Signup and view all the answers

    Which of the following is a consequence of impaired platelet adhesion?

    <p>Prolonged bleeding time</p> Signup and view all the answers

    What is the purpose of fibrin stabilization in the coagulation cascade?

    <p>To strengthen the fibrin clot</p> Signup and view all the answers

    Which of the following blood vessels plays a crucial role in repair after injury?

    <p>All of the above</p> Signup and view all the answers

    What is the primary function of platelets in the circulation?

    <p>To surveil blood vessel continuity</p> Signup and view all the answers

    What is the result of platelet activation and shape change?

    <p>Secretion of platelet granules</p> Signup and view all the answers

    What is the role of platelets in the coagulation cascade?

    <p>To provide a surface for coagulation factors</p> Signup and view all the answers

    What is the primary function of platelet aggregation?

    <p>To reinforce the primary hemostatic plug</p> Signup and view all the answers

    What is the ultimate outcome of the coagulation cascade?

    <p>Stable fibrin-platelet plug formation</p> Signup and view all the answers

    What is the primary mechanism by which blood clots are dissolved?

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

    What is the role of platelets in blood vessel repair?

    <p>To aid in the healing of injured tissues</p> Signup and view all the answers

    What is the response to collagen exposure and tissue factor exposure in the coagulation cascade?

    <p>Extrinsic pathway activation</p> Signup and view all the answers

    What is the purpose of platelet-fibrin clot formation?

    <p>To prevent blood loss</p> Signup and view all the answers

    What is the primary component of hemostasis?

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

    What is the result of primary hemostasis?

    <p>Formation of a primary hemostatic plug</p> Signup and view all the answers

    What is the function of smooth muscle cells in blood vessels?

    <p>To control blood vessel diameter</p> Signup and view all the answers

    What is the layer of the blood vessel wall that comes into contact with blood cells?

    <p>Tunica intima</p> Signup and view all the answers

    What is the term for the process by which platelets become activated and aggregate?

    <p>Platelet aggregation</p> Signup and view all the answers

    What is the purpose of TPA (Tissue Plasminogen Activator) in fibrinolysis?

    <p>To dissolve blood clots</p> Signup and view all the answers

    What is the layer of the blood vessel wall that contains smooth muscle cells and connective tissue?

    <p>Tunica media</p> Signup and view all the answers

    Which of the following platelet granules is involved in platelet aggregation and activation of the coagulation system?

    <p>α-granules</p> Signup and view all the answers

    What is the primary mechanism by which thrombin stimulates platelet aggregation?

    <p>Stimulation of phospholipases to form TxA2</p> Signup and view all the answers

    Which of the following platelet disorders is characterized by the absence or mutation of the GP IIb-IIIa receptor?

    <p>Glanzmann thrombasthenia</p> Signup and view all the answers

    What is the primary function of von Willebrand factor in platelet adhesion?

    <p>Linking platelets to the subendothelial matrix</p> Signup and view all the answers

    Which of the following platelet agonists is dependent on the presence of the GP IIb-IIIa receptor?

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

    What is the primary function of prostacyclin (PGI2) in the vascular endothelium?

    <p>Inhibiting platelet aggregation and inducing vasodilation</p> Signup and view all the answers

    Which of the following is a characteristic feature of Gray platelet syndrome?

    <p>Deficiency of α-granules</p> Signup and view all the answers

    What is the primary mechanism by which thromboxane A2 (TxA2) stimulates platelet aggregation?

    <p>Inducing platelet shape change</p> Signup and view all the answers

    Which platelet granule is responsible for releasing thrombospondin, a substance that promotes vascular repair?

    <p>a-granules</p> Signup and view all the answers

    What is the primary role of platelet-derived growth factor in the process of hemostasis?

    <p>Promoting smooth muscle growth for vessel repair</p> Signup and view all the answers

    Which platelet granule is involved in the release of serotonin, a substance that promotes vasoconstriction?

    <p>Dense bodies</p> Signup and view all the answers

    What is the primary mechanism by which platelets promote coagulation?

    <p>Providing a phospholipid surface for coagulation to occur</p> Signup and view all the answers

    Which of the following is NOT a function of platelets in the process of hemostasis?

    <p>Inducing apoptosis</p> Signup and view all the answers

    What is the primary role of thromboxane A2 in platelet function?

    <p>Promoting platelet aggregation</p> Signup and view all the answers

    Which platelet granule is involved in the release of fibrinogen, a cofactor in fibrin clot formation?

    <p>a-granules</p> Signup and view all the answers

    What is the primary function of HMWK in the coagulation cascade?

    <p>Enhancing coagulation cascade</p> Signup and view all the answers

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