Pulmonary, Hematologic, and Rheumatic Disease Flashcards

1
Q

pulmonary function testing cutoffs for obstructive and restrictive pulmonary disease

A

obstructive: FEV1/FVC < 70%
restrictive: total lung capacity < 70% of predicted

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2
Q

ophthalmic disease processes associated with OSA?

A

floppy eyelid syndrome, keratoconus, NAION

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3
Q

ophthalmic side effects of CPAP?

A

increased IOP and dry eye

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4
Q

what immune modulating drugs are useful in acute asthma exacerbation?

A

systemic steroids. NOT inhaled steroids.

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5
Q

single most cost-effective and efficacious intervention to reducing risk and slowing progression of COPD?

A

smoking cessation

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6
Q

MOA of cromolyn sodium

A

mast cell stabilizer

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7
Q

COPD patients who use inhaled corticosteroids are at an increased risk for____

A

pneumonia

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8
Q

myeloid stem cells are precursors to____

A

erythrocytes, granulocytes, monocytes, and platelets

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9
Q

most common anemia worldwide?

A

iron deficiency

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10
Q

pathophys and best screening test for paroxysmal nocturnal hemoglobinuria?

A

RBCs are extra-sensative to complement-mediated cell lysis. flow cytometry

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11
Q

Treatment of choice for beta-thalassemia major?

A

allogeneic stem cell transplant

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12
Q

life span of circulating RBC?

A

120 days

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13
Q

cutoffs for microcytic, normocytic, and macrocytic anemia?

A

microcytic: MCV < 70
macrocytic: MCV > 100

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14
Q

what is hepcidin, and what is its relative value in iron-deficiency anemia?

A

peptide hormone that inhibits iron transport across intestinal epithelium and inhibits iron transport out of macrophages, thus decreasing serum iron. Hepcidin is compensatorily low in iron deficiency anemia

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15
Q

genetic mechanism and phenotype of alpha- and beta-thalassemia

A

alpha-thal: gene deletion leading to no synthesis of hemoglobin alpha chain

beta-thal: point mutation leading to impaired beta-chain synthesis

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16
Q

type of anemia described by defective incorporation of iron into hemoglobin

A

sideroblastic anemia

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17
Q

what type of anemia are alpha- and beta-thalassemia?

A

hypochromic, microcytic

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18
Q

source, mechanism and site of absorption, site and length of storage of vitamin B12

A

all animal products. binds with intrinsic factor produced by gastroepithelial cells and this complex is absorbed in terminal ileum. stored in liver, takes 3 years to deplete stores.

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19
Q

atrophic gastritis leading to decreased intrinsic factor production

A

pernicious anemia

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20
Q

type of anemia for B12 and folate deficiency?

A

megaloblastic (subset of macrocytic). Caused by impaired DNA synthesis

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21
Q

most sensitive and specific lab test for B12 deficiency (excluding blood smear)

A

serum methylmelonic acid (will be ELEVATED)

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22
Q

macrocytic anemia with peripheral neuropathy?

A

B12 deciciency

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23
Q

hypersegmented neutrophils in peripheral smear

A

folic acid deficiency

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24
Q

effect of folate supplementation for patient with B12 deficiency?

A

will correct anemia but will not affect neuropathy

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25
Q

lab features of hemolytic anemia

A

increased reticulocyte count, increased indirect bilirubin, increased LDH, decreased haptoglobin

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26
Q

what is haptoglobin and in what pathologic state is it reduced?

A

protein that binds free hemoglobin released by erythrocytes. this complex is then removed by the reticuloendothelial system. haptoglobin is low in hemolytic anemia

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27
Q

treatment indicated for all types of hemolytic anemia?

A

folate supplementation

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28
Q

inheritance, pathophys and definitive treatment for hereditary spherocytosis?

A

AD. RBC membrane is abnormal and cell is shaped like a sphere which ruptures more easily, especially in the spleen, leading to hemolytic anemia. splenectomy

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29
Q

inheritance and pathophys of G6PD deficiency?

A

XR. RBCs unable to deal with oxidative stress, leading to hemolytic anemia in setting of infection or precipitating drug. Heinz bodies = hemoglobin precipitates in RBCs

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30
Q

inheritance, pathophys, and definitive diagnosis of sickle cell anemia

A

AR causing amino acid substitution on beta-chain of hemoglobin which leads to erythrocyte cell membrane instability and sickling. hemoglobin electrophoresis

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31
Q

positive Coombs test?

A

autoimmune hemolytic anemia

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32
Q

platelet level where minor bleeding can occur? spontaneous bleeding?

A
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33
Q

what are the vitamin K-dependent coagulation factors?

A

factors, II, VII, IX, and X

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34
Q

ophthalmic side effects of CPAP?

A

increased IOP and dry eye

35
Q

what immune modulating drugs are useful in acute asthma exacerbation?

A

systemic steroids. NOT inhaled steroids.

36
Q

single most cost-effective and efficacious intervention to reducing risk and slowing progression of COPD?

A

smoking cessation

37
Q

MOA of cromolyn sodium

A

mast cell stabilizer

38
Q

COPD patients who use inhaled corticosteroids are at an increased risk for____

A

pneumonia

39
Q

myeloid stem cells are precursors to____

A

erythrocytes, granulocytes, monocytes, and platelets

40
Q

most common anemia worldwide?

A

iron deficiency

41
Q

pathophys and best screening test for paroxysmal nocturnal hemoglobinuria?

A

RBCs are extra-sensative to complement-mediated cell lysis. flow cytometry

42
Q

Treatment of choice for beta-thalassemia major?

A

allogeneic stem cell transplant

43
Q

life span of circulating RBC?

A

120 days

44
Q

cutoffs for microcytic, normocytic, and macrocytic anemia?

A

microcytic: MCV 100

45
Q

what is hepcidin, and what is its relative value in iron-deficiency anemia?

A

peptide hormone that inhibits iron transport across intestinal epithelium and inhibits iron transport out of macrophages, thus decreasing serum iron. Hepcidin is compensatorily low in iron deficiency anemia

46
Q

genetic mechanism and phenotype of alpha- and beta-thalassemia

A

alpha-thal: gene deletion leading to no synthesis of hemoglobin alpha chain

beta-thal: point mutation leading to impaired beta-chain synthesis

47
Q

type of anemia described by defective incorporation of iron into hemoglobin

A

sideroblastic anemia

48
Q

what type of anemia are alpha- and beta-thalassemia?

A

hypochromic, microcytic

49
Q

source, mechanism and site of absorption, site and length of storage of vitamin B12

A

all animal products. binds with intrinsic factor produces by gastroepithelial cells and this complex is absorbed in terminal ileum. stored in liver, takes 3 years to deplete stores.

50
Q

atrophic gastritis leading to decreased intrinsic factor production

A

pernicious anemia

51
Q

type of anemia for B12 and folate deficiency?

A

megaloblastic (subset of macrocytic). Caused by impaired DNA synthesis

52
Q

most sensitive and specific lab test for B12 deficiency (excluding blood smear)

A

serum methylmelonic acid (will be ELEVATED)

53
Q

macrocytic anemia with peripheral neuropathy?

A

B12 deciciency

54
Q

hypersegmented neutrophils in peripheral smear

A

folic acid deficiency

55
Q

effect of folate supplementation for patient with B12 deficiency?

A

will correct anemia but will not affect neuropathy

56
Q

lab features of hemolytic anemia

A

increased reticulocyte count, increased indirect bilirubin, increased LDH, decreased haptoglobin

57
Q

what is haptoglobin and in what pathologic state is it reduced?

A

protein that binds free hemoglobin released by erythrocytes. this complex is then removed by the reticuloendothelial system. haptoglobin is low in hemolytic anemia

58
Q

treatment indicated for all types of hemolytic anemia?

A

folate supplementation

59
Q

inheritance, pathophys and definitive treatment for hereditary spherocytosis?

A

AD. RBC membrane is abnormal and cell is shaped like a sphere which ruptures more easily, especially in the spleen, leading to hemolytic anemia. splenectomy

60
Q

inheritance and pathophys of G6PD deficiency?

A

XR. RBCs unable to deal with oxidative stress, leading to hemolytic anemia in setting of infection or precipitating drug. Heinz bodies = hemoglobin precipitates in RBCs

61
Q

inheritance, pathophys, and definitive diagnosis of sickle cell anemia

A

AR causing amino acid substitution on beta-chain of hemoglobin which leads to erythrocyte cell membrane instability and sickling. hemoglobin electrophoresis

62
Q

positive Coombs test?

A

autoimmune hemolytic anemia

63
Q

platelet level where minor bleeding can occur? spontaneous bleeding?

A
64
Q

PTT test useful for assessing response to what drug?

A

heparin

65
Q

what are the vitamin K-dependent coagulation factors?

A

factors, II, VII, IX, and X

66
Q

diagnosis and inheritance of localized dilation of capillaries and venules of skin and mucous membranes including conjunctiva

A

Osler-Weber-Rendu (hereditary hemorrhagic telangiectasia). AD. worsens with age, can lead to profuse bleeding

67
Q

diagnosis, inheritance, and ophthalmic findings of patient with hyperplastic skin, hyperextensible joints and easy bruising

A

Ehlers-Danlos. AD. angioid streaks, microcornea, myopia, RD, ectopia lentis

68
Q

diagnosis, inheritance, and ophthalmic findings of patient with bone fragility, deafness, and easy bruising

A

osteogenisis imperfecta (deafness from otosclerosis). AD. blue sclera

69
Q

more common cause of bleeding disorders: platelet abnormalities or coagulation abnormalities?

A

platelet disorders by far

70
Q

mechanism and first line treatment of ideopathic thrombocytopenic purpura

A

autoantibodies against platelets, often occurring after a viral illness. steroids.

71
Q

low platelet counts with thrombotic occlusions of microcirculation and hemolytic anemia

A

TTP (thrombotic thrombocytopenic purpura)

72
Q

mechanism and effective life span of aspirin?

A

irreversibly inhibits platelet aggregation, usually lasting 48-72 hours

73
Q

most common inherited coagulation factor deficiency? Inheritance? Coag study findings?

A

factor VIII (hemophilia A). XR. Prolonged PTT, normal PT/INR

74
Q

most common inherited bleeding disorder?

A

Von Willebrand disease

75
Q

cause of hemorrhagic disease of the newborn?

A

normal, mild-deficiency of vitamin-K dependent factors in the newborn. Now very rare with treatment of all newborns with vitamin K

76
Q

widespread activation of coagulation and fibrinolytic systems causing bleeding and clotting?

A

DIC

77
Q

most common inherited thrombophilia?

A

factor V Ledein

78
Q

Which of the following does NOT cause a hypercoagulable state?
Antithrombin III deficiency, Protein C deficiency, Protein S deficiency, factor V Leiden, Von Willebrand disease, prothrombin gene mutation, hyperhomocysteinemia, antiphospholipid antibody syndrome

A

Von Willebrand

79
Q

mechanism of acquired hyperhomocysteinemia?

A

nutritional deficiency of pyridoxine (B6), B12, or folate

80
Q

Which of the following is not appropriate method for vitamin K supplementation?

subcutaneous, intramuscular, or intravenous

A

IV vitamin K can lead to anaphylaxis and death

81
Q

mechanism of action of heparin

A

binds to antithrombin III and helps catalyze its antithrombin and anti-Xa activity

82
Q

Which of the following is NOT a benefit of low molecular weight heparin compared to unfractionated heparin?

(a) Aspirin can be safely coadministered
(b) Greater bioavailability when given subQ
(c) laboratory monitoring is not necessary
(d) lower risk of heparin-induced thrombocytopenia

A

(a). Aspirin should not be given to patients on unfractionated or LMWH heparin due to the risk of bleeding

83
Q

Which of the following is FALSE regarding direct thrombin inhibitors and factor Xa inhibitors?

(a) Neither requires direct laboratory monitoring
(b) Due to early onset of action (1-4 hours), neither requires bridging when switching to these from initial treatment such as heparin
(c) A major disadvantage is that there are no available antidotes for bleeding events
(d) Fondaparinux exclusively inhibits factor Xa and is eliminated by the liver

A

(d) Fondaparinux exclusively inhibits factor Xa and is eliminated by the KIDNEY and should be used cautiously in patients with renal disease