Practice quiz lecture 2.1 Flashcards

(31 cards)

1
Q

True or false: There are absolutely no physiologic mechanisms to eliminate excess iron from the body

A

True

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2
Q

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

A

f) All of the above

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3
Q

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

A

b) Duodenum

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4
Q

What 3 organs are most affected by hereditary hemochromatosis (HH)? Select 3
a) Liver
b) Pancreas
c) Heart
d) Pituitary gland
e) Kidneys

A

a) Liver
c) Heart
d) Pituitary gland

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5
Q

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

A

f) Hypergonadism

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6
Q

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

A

c) Siderophilic bacteria

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7
Q

Iron studies: What 2 things may indicate iron overload?
a) High TSAT
b) High ferritin
c) High TIBC
d) Low TSAT

A

a) High TSAT
b) High ferritin

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8
Q

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

a) HFE genetic testing
d) MRI
e) Liver function tests

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9
Q

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

A

d) For 2-3 years

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10
Q

What is the most common inherited bleeding disorder?
a) Hemophilia A
b) Hemophilia B
c) Hemophilia C
d) Von Willebrand disease

A

d) Von Willebrand disease

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11
Q

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.

A

c) Intrinsic: 8, 9, 11, 12. Common: 1, 2, 5, 10. Extrinsic: 7.

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12
Q

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.

A

b) Factor 1 = fibrinogen; Factor 2 = prothrombin.

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13
Q

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

a) VI; VIII

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14
Q

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

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

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15
Q

True or false: All male first-degree relatives of an individual with hemophilia should get genetic testing.

A

False (female first-degree relatives)

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16
Q

True or false: Desmopressin (DDAVP) (IV or intranasal) may be given as needed for mild hemophilia A, but not for hemophilia B.

17
Q

True or false: Optimal care for severe hemophilia involves primary prophylactic factor replacement therapy.

18
Q

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

A

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.

19
Q

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)

A

c) Antifibrinolytics

20
Q

True or false: Early data indicate that mortality from coronary artery disease is higher in hemophilia patients than the general male population.

A

False (it’s lower)

21
Q

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

a) Hemophilia C = Autosomal recessive inheritance; Hemophilia A & B = X-linked dominant

22
Q

Which of the following is the most common type of vWF disease?
a) Type 4
b) Type 3
c) Type 2
d) Type 1

23
Q

Which of the following types of vWF disease is a qualitative defect?
a) Type 4
b) Type 3
c) Type 2
d) Type 1

24
Q

True or false: aPTT and PT are most commonly normal in patients with vWD

25
True or false: Desmopressin (DDAVP) is useful in the treatment of very severe bleeding in vWF disease
False
26
You have a hospitalized patient on broad-spectrum antibiotics who has poor or no oral intake. What are they at high risk for that could lead to prolonged PT (& later a prolonged PTT)? a) vWF deficiency b) Vitamin K deficiency c) Vitamin C deficiency d) Factor XI deficiency
b) Vitamin K deficiency
27
True or false: Immune Thrombocytopenic Purpura (ITP) is more often acute in kids than adults.
True
28
Which of the following are characteristic of ITP? a) Ecchymoses b) Petechiae c) Thrombocytopenia d) All of the above e) None of the above
d) All of the above
29
Mainstay of initial treatment of new-onset primary ITP is what? a) Corticosteroids +/- IV immunoglobulin (IVIG) b) Corticosteroids +/- antibiotics c) Desmopressin (DDAVP) +/- IV immunoglobulin (IVIG) d) Desmopressin (DDAVP) +/- antibiotics
a) Corticosteroids +/- IV immunoglobulin (IVIG)
30
Give 2 examples of Thrombotic Microangiopathies (TMAs). (Select 2) a) Immune Thrombocytopenic Purpura (ITP) b) Thrombotic thrombocytopenic purpura (TTP) c) Hemophilia A & B d) Hemolytic uremic syndrome (HUS) e) Hemophilia C
b) Thrombotic thrombocytopenic purpura (TTP) d) Hemolytic uremic syndrome (HUS)
31
What 2 findings are characteristic of Thrombotic Microangiopathies (TMAs)? a) Thrombocytosis + Macrocytic hemolytic anemia (MAHA) b) Thrombocytopenia + Liver dysfunction c) Thrombocytopenia + Microangiopathic hemolytic anemia (MAHA) d) Thrombocytosis + Microangiopathic hemolytic anemia (MAHA)
c) Thrombocytopenia + Microangiopathic hemolytic anemia (MAHA)