Practice quiz lecture 2.1 Flashcards
(31 cards)
True or false: There are absolutely no physiologic mechanisms to eliminate excess iron from the body
True
Which of the following are causes of iron overload?
a) Hereditary hemochromatosis (HH)
b) Ineffective erythropoiesis in beta thalassemia or anemias
c) Alcoholic liver disease
d) Chronic hepatitis
e) Blood transfusions
f) All of the above
g) None of the above
f) All of the above
With hereditary hemochromatosis (HH), the HFE gene on chromosome 6 leads to increased iron absorption from the ______________ and a decrease in hepcidin.
a) Large intestine
b) Duodenum
c) Jejunum
d) Ilium
e) Stomach
b) Duodenum
What 3 organs are most affected by hereditary hemochromatosis (HH)? Select 3
a) Liver
b) Pancreas
c) Heart
d) Pituitary gland
e) Kidneys
a) Liver
c) Heart
d) Pituitary gland
Which of the following is NOT a manifestation of iron overload?
a) Increased hepatic transaminases
b) Skin hyperpigmentation
c) Arrhythmias
d) Type 2 DM
e) Heart failure
f) Hypergonadism
f) Hypergonadism
Patients with iron overload (including HH) are at risk for infection with what?
a) Bacteria in general
b) Viruses in general
c) Siderophilic bacteria
d) HIV
c) Siderophilic bacteria
Iron studies: What 2 things may indicate iron overload?
a) High TSAT
b) High ferritin
c) High TIBC
d) Low TSAT
a) High TSAT
b) High ferritin
You perform iron studies tests and have identified iron overload greater than >1000 ng/mL. Which of the following tests do you need to do? Select all that apply
a) HFE genetic testing
b) Ultrasound
c) CT scan
d) MRI
e) Liver function tests
a) HFE genetic testing
d) MRI
e) Liver function tests
How long should you have a pt with symptomatic HH do weekly phlebotomy?
a) Until symptoms subside
b) For 6 months
c) For 1 year
d) For 2-3 years
e) Indefinitely
d) For 2-3 years
What is the most common inherited bleeding disorder?
a) Hemophilia A
b) Hemophilia B
c) Hemophilia C
d) Von Willebrand disease
d) Von Willebrand disease
Which of the following matches the correct factors to the correct pathways?
a) Extrinsic: 8, 9, 11, 12. Intrinsic: 1, 2, 5, 10. Common: 7.
b) Intrinsic: 1, 2, 7, 12. Extrinsic: 5, 8, 9, 11. Common: 10
c) Intrinsic: 8, 9, 11, 12. Common: 1, 2, 5, 10. Extrinsic: 7.
d) Intrinsic: 1, 2, 7, 12. Common: 5, 8, 9, 11. Extrinsic: 10.
c) Intrinsic: 8, 9, 11, 12. Common: 1, 2, 5, 10. Extrinsic: 7.
Which of the following contains the correct terminology?
a) Factor 1 = prothrombin; Factor 2 = fibrinogen.
b) Factor 1 = fibrinogen; Factor 2 = prothrombin.
c) Factor 1 = vWF; Factor 2 = prothrombin.
d) Factor 1 = prothrombin; Factor 2 = vWF.
b) Factor 1 = fibrinogen; Factor 2 = prothrombin.
Low factor ______ is hemophilia A; low factor ________ is hemophilia B; both have normal vWF.
a) VI; VIII
b) VIII; IX
c) IX; XII
d) X; VIII
a) VI; VIII
Factor activity testing should be measured in which of the following settings? Select all that apply
a) Male infants with a hemophilic maternal pedigree
b) Daughters of affected males or of carrier mothers
c) Anyone who experiences unexpected excessive bleeding with trauma/invasive procedures
d) Daughters of affected females or of carrier fathers
e) Male infants with a hemophilic paternal pedigree
a) Male infants with a hemophilic maternal pedigree
b) Daughters of affected males or of carrier mothers
c) Anyone who experiences unexpected excessive bleeding with trauma/invasive procedures
True or false: All male first-degree relatives of an individual with hemophilia should get genetic testing.
False (female first-degree relatives)
True or false: Desmopressin (DDAVP) (IV or intranasal) may be given as needed for mild hemophilia A, but not for hemophilia B.
True
True or false: Optimal care for severe hemophilia involves primary prophylactic factor replacement therapy.
True
Which of the following are correct? Select all that apply.
a) Desmopressin (DDAVP) brings factors 9 and 10 together to replace factor 8.
b) Desmopressin (DDAVP) promotes release of endogenous factor 8 and von Willebrand factor
c) Emicizumab brings factors 9 and 10 together to replace factor 8.
d) Emicizumab promotes release of endogenous factor 8 and von Willebrand factor
b) Desmopressin (DDAVP) promotes release of endogenous factor 8 and von Willebrand factor
c) Emicizumab brings factors 9 and 10 together to replace factor 8.
What may be useful as an adjunctive agent for mucosal bleeding or procedures to control local hemostasis in hemophilia?
a) Desmopressin
b) Emicizumab
c) Antifibrinolytics
d) Gene therapy
e) Aspirin
f) Opioids (PO)
c) Antifibrinolytics
True or false: Early data indicate that mortality from coronary artery disease is higher in hemophilia patients than the general male population.
False (it’s lower)
Which of the following contains correct information?
a) Hemophilia C = Autosomal recessive inheritance; Hemophilia A & B = X-linked dominant
b) Hemophilia C = Autosomal dominant inheritance; Hemophilia A & B = X-linked recessive
c) Hemophilia A & B = Autosomal recessive inheritance; Hemophilia C = X-linked dominant
d) Hemophilia A & B = Autosomal dominant inheritance; Hemophilia C= X-linked recessive
a) Hemophilia C = Autosomal recessive inheritance; Hemophilia A & B = X-linked dominant
Which of the following is the most common type of vWF disease?
a) Type 4
b) Type 3
c) Type 2
d) Type 1
d) Type 1
Which of the following types of vWF disease is a qualitative defect?
a) Type 4
b) Type 3
c) Type 2
d) Type 1
c) Type 2
True or false: aPTT and PT are most commonly normal in patients with vWD
True