1.
Despite great advances in the treatment of childhood diseases, malignancy
remains an ominous threat. All of
the following statements regarding malignant diseases in children are true
except:
A.
Neoplastic disease is the leading cause of death in the 1-15 year old
population
B.
The leukemias are the most common malignancies of childhood
C.
Brain tumor is the most common solid tumor in childhood
D.
Ectodermal and endodermal carcinomas are rarely seen in children
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2. With regard to the association of infection related to long-term catheter
insertion in the pediatric population, which one of the following is the most
accurate:
A.
Infus-a-port carries a lower rate of infection than Broviac single lumen
catheters
B. Infus-a-port has a lower rate of
infection than both single lumen Broviac
and double lumen
Hickman catheters
C.
Double lumen catheters have a
higher rate of infection compared to
single lumen catheters
D.
There is no significant
difference in infection rate between the
externalized and the
subcutaneously
implanted catheters
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3.
A 4-year old boy with leukemia is being evaluated for fevers.
Physical examination does not reveal any focus of infection.
The white blood cell count is 1235 cells/mm3 with 12%
neutrophils, 2% band forms, and 86% lymphocytes.
The platelet count is 32,000/mm3.
Which of the following statements is most accurate regarding this
clinical scenario:
A.
Fifty to seventy percent of febrile episodes in oncology patients are
non-infectious
B.
The blood culture is positive in greater than 75% of disseminated fungal
infections
C. The degree of neutropenia does
not correlate with the morbidity and mortality due to bacterial infections
D. Bronchoscopy should be done at this point to evaluate for pneumocystis
E.
Candida and aspergillus species
are the two most common fungal agents causing fungal infections in pediatric
oncology patients
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4. All of the following
statements regarding the child with malignant disease are true except:
A.
P. carnii will be identified in
15% of patients despite previous
prophylaxis with trimethoprim-sulfamethoxazole.
B.
Granulocyte transfusions may be
beneficial in the setting of
neutropenia and culture proven
sepsis
C.
The minor complications resulting
from granulocyte transfusions can
usually be avoided with appropriate
pre-medication
D.
The half-life of transfused platelets is greater than 4 weeks
E.
Spontaneous bleeding may occur when the platelet count is less than
20,000/mm3
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5. Match the following
chemotherapeutic drug with its most likely hematologic effect:
A.
Actinomycin D
B.
Anthracycline
C.
L-Asparaginase
D.
Methotrexate
E.
Vincristine
F.
Glucocorticoids
____
1.
Antithrombin III deficiency
____
2.
Chronic hepatic dysfunction
____
3.
Hypofibrinoginemia
____
4.
Increases fibrinolysis
____
5.
Increases factors II, VII, VIII, X
____
6.
Decreases Vitamin K dependent factors
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6.
All of the following statements are true regarding pulmonary parenchymal
disease in children with malignancies except:
A.
The pulmonary parenchyma is commonly involved in leukemia
B.
The initial lesion from radiation
exposure occurs within 6 hours of
radiation therapy and
involves capillary
endothelial damage
C.
Bleomycin and Busulfan associated pulmonary fibrosis is dose
dependent
D.
Cyclophosphamide and methotrexate have been associated with
pulmonary
fibrosis
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7.
Select whether the following statements regarding malignancies and
chemotherapy in children are true or false:
____
Cyclophosphamide is associated with hemorrhagic cardiac necrosis
____ Anthracycline induced
cardiomyopathy manifests itself as myofibrillar loss
and cytoplasmic
vacuolization
____ Cardiomyopathy is unrelated to radiotherapy
____ Vincristine may induce SIADH
____
Intrathecal chemotherapy may cause seizures
____
When P. carinii infection
occurs after bone marrow transplant, it is most
commonly
seen at 2-4 weeks
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here for answer
8.
Match the following chemotherapeutic drug with its most closely
associated adverse neurologic effect:
A.
Methotrexate
B.
Cisplatin
C.
Vincristine
D.
5-FU
____
1.
Ototoxicity
____
2.
Acute cerebellar ataxia
____
3.
Aseptic meningitis
____
4.
SIADH
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9.
Match the following chemotherapeutic drug with its most commonly
associated complication:
A.
Methotrexate (high-dose)
B.
Cisplatin
C.
L-Aspariginase
D.
Cyclophosphamide
____
1.
Tubular necrosis
____
2.
Hemorrhagic cystitis
____
3.
Tubular precipitation
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10. All of the following statements
regarding bone marrow transplantation are true except:
A.
The most common indications are
acute myelogenous leukemia and acute lymphoblastic leukemia in remission
B.
In patients who receive
allogeneic bone marrow transplantation, the mortality rate is 20-35%
C.
The risk periods for pneumonitis
and pulmonary insufficiency are 2 weeks, 6-12 weeks, and 3-6 months after bone
marrow transplantation
D.
Cytomegalovirus infection is most common 6 months after a bone marrow
transplant
E. Cyclosporin may prevent acute rejection
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11. Match the chemotherapy drug with
its mechanism of action:
A.
Methotrexate
B.
Vincristine
C.
Doxorubicin
D.
Cyclophosphamide
E.
Glucocorticoids
____
1.
Antimetabolite
____
2.
Breaks DNA strands
____
3.
Inhibits microtubule function
____
4.
Antibiotic
____
5.
Lymphocytotoxic
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12. A
5-year old girl has prolonged bleeding after her dentist pulls a tooth. Additionally, her 16-year old sister has protracted monthly
menses of 7-9 days. You suspect von
Willebrand’s disease. Which of
the following statements regarding the coagulation system is/are true:
A.
The prothrombin time measures the extrinsic and common pathways
B.
The activated partial
thromboplastin time measures the intrinsic and common pathways
C.
The thrombin time measures the
coagulation cascade from prothrombin to stable fibrin
D.
Factors XII, XI, IX, and X comprise the intrinsic system
E.
All of the above
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13. Select
whether the following statements regarding the clotting system and blood
products are true or false:
____
The only source for factors V and XI is fresh frozen plasma
____
The preferred source for
factors II, VII, X, and antithrombin III is cryoprecipitate
____
Cryoprecipitate contains fibrinogen, factor VIII, and von Willebrand’s
factor
____
The main components of the
anti-thrombotic system include antithrombin III, protein C and protein S.
____
Protein C induces proteolysis
of factors V and VIII and neutralizes plasminogen activator inhibitor
____
Vitamin K dependent factors include II, VII, IX, and X
____
Vitamin K is a vitamin that is
stored in the body and therefore no exogenous supplementation is necessary
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14. All of the following are the main
components of the anti-thrombotic system except:
A.
Anti-thrombin III
B.
Protein C
C.
Protein S
D.
Protein B
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15. An
11-month old male with a history of biliary atresia has developed fulminant
hepatic failure with gastrointestinal bleeding and bleeding from his central
line site. You suspect a hepatic
coagulopathy. Which one of the
following statements regarding this situation is/are true:
A.
Dietary inadequacy and total parenteral nutrition are causes of Vitamin K
deficiency
B.
Fibrinogen, factors V, VIII, and
proteins C and S are depleted during disseminated intravascular coagulopathy
C.
In hepatic coagulopathy, a
prolonged PT indicates decreased synthesis of Vitamin K dependent factors
D.
Factor V synthesis is independent of Vitamin K availability
E.
All of the above
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16. Which
of the following is a true statement regarding coagulopathy associated with
cardiopulmonary bypass:
A.
Protamine is used to potentiate heparinization
B.
Hyperfibrinolysis causing
significant bleeding can be treated with epsilon-aminocaproic acid intravenously
C.
Vitamin K deficiency is a rarely noted event
D.
D-dimers are frequently elevated
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17. Which
of the following is/are true statements regarding the coagulopathy associated
massive transfusion:
A.
Hemolysis always occurs
B.
Platelet destruction occurs,
necessitating platelet transfusion to maintain a platelet count of 80,000 to
100, 000 /mm3
C.
Changing the transfusion circuit
may temporarily reduce the activity of the coagulation cascade
D.
A, B, and C
E.
B and C only
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18. Which
of the following biochemical changes is least likely to be associated with
massive transfusion:
A.
Disseminated intravascular coagulopathy
B.
Abnormal platelet function
C.
An increase in 2,3,-DPG
D.
Hyperkalemia
E.
Hypothermia and metabolic acidosis
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19. Which of the following regarding
Antithrombin III and heparin is/are true:
A.
Antithrombin III is synthesized in the liver and inhibits thrombin and
factor Xa
B.
Acquired Antithrombin III
deficiency is more common than the autosomal dominant inherited congenital type
C.
Antithrombin III concentrations < 70% are associated with thromboses
D.
Heparin-induced anti-platelet
antibodies occur in approximately 5% of patients receiving heparin
E.
All of the above
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20. Which
of the following statements is/are true regarding heparin-induced antiplatelet
antibodies:
A.
Typically occurs 4-15 days after first exposure to heparin
B.
Typically occurs 1-9 days following the second exposure to heparin
C.
Are associated with thromboses
D.
All of the above
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21. Which
of the following statements is/are true regarding Protein C and Protein S
anticoagulant systems:
A.
Protein C is a Vitamin K dependent protease which is produced by the
liver
B.
Protein C is deactivated by thrombin
C.
Protein C is catalyzed by thrombomodulin
D.
Protein C deficiency is most
commonly an acquired abnormality, rather than a congenital one
E.
A, C, and D only
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22. Which of the following statements
regarding fibrinolytic therapy is/are true:
A.
Streptokinase is produced by b-streptococci; it attaches to plasminogen and
is rapidly inactivated by naturally occurring antibodies with a half life of 30
minutes
B.
Urokinase can be produced from
human urine; it hydrolyzes plasminogen to plasmin with a half life of 30 minutes
C.
Tissue plasminogen activator
(t-PA) is produced in vascular endothelium; it converts plasminogen to plasmin
D.
Absolute contraindications to
fibrinolytic therapy included acute hemorrhage, recent cerebral vascular
accident, and aneurysm.
E.
All of the above
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23. A
1-week-old infant undergoes a cardiac catheterization for a diagnosis of a
cardiac defect. His right femoral
artery is cannulated. After the
procedure, his right leg is cold and his femoral pulse is barely palpable.
You suspect a thrombus. Which
of the following statements are true regarding thrombolytic therapy:
A.
Heparinzation is standard
management until recannulation or vascular integrity improves
B.
PTT should be 2.5-3.0 times normal during heparin therapy
C.
Streptokinase and t-PA are
contraindicated for femoral artery spasm after catheterization
D.
Indications for surgical
intervention of pulmonary emboli include pulmonary artery cross-sectional
obstruction > 50%, right ventricular failure, or shock
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24. Which of the following statements
is/are true regarding chronic anti-coagulation therapy:
A.
Warfarin inhibits synthesis of factors II, VII, IX, X, Protein C, and
Protein S
B.
Platelet aggregation is
decreased by blocking the synthesis of thromboxane A2 with
cyclo-oxygenase inhibitors
C.
Sulfinpyrazone or aspirin may be used
D.
Platelet aggregation is decreased by administration of a
phosphodiesterase inhibitor
E.
All of the above
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25. Match the following drug with its
site of action
A.
Aspirin
B.
Sulfinpyrazone
C.
Both
D.
Neither
____
1.
Cyclooxygenase inhibition
____
2.
Phosphodiesterase inhibition
____
3.
Irreversibly bound to thromboxane A2
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here for answer
26. All of the following regarding
thrombosis of prosthetic valves are true except:
A.
Biologic valves are less thromboembolic than mechanical valves
B.
Mitral valves are more thromboembolic than aortic valves
C.
Recommendations by the National
Heart, Lung and Blood Institute for mechanical valves include long term
anticoagulation
D.
Thromboprophylaxis for prosthetic
valves in pediatric patients does not decrease the occurrence of acute
thromboembolic effects
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Answers
1.
A
Trauma is the leading cause of
death in the 1 – 15 year old population.
Neoplastic disease is second. The
other responses are true. (Rogers
MC, et al. Textbook of Pediatric Intensive Care, 3rd Edition;
pp 1433-1434)
2.
D
Infection is higher in patients
with central lines than those without. Recent
retrospective data suggests that there are no differences in infection rates
between subcutaneously implanted, versus externalized catheters.
(Rogers, MC, et al. Textbook of Pediatric Intensive Care, 3rd
Edition; pp 1438)
3.
E
Fifty five to seventy percent of
febrile episodes in oncology patients are of infectious origin. Blood cultures
are positive in less than 50% of cases of serious disseminated fungal
infections. Candida albicans and
Aspergillus species are the most common fungal organisms.
Neutropenia is closely correlated with morbidity and mortality.
Pneumocystis is unlikely in this clinical scenario, however, Pneumocystis
is responsible for up to 50% of nonbacterial pneumonitis in oncology patients.
Chest radiographs demonstrate bilateral infiltrates radiating from the
hilum. (Rogers, MC, et al. Textbook
of Pediatric Intensive Care, 3rd Edition; pp 1438–1445; Table
42.6)
4.
D
The half life of transfused
platelets is 7 days; with significant alloimmunization, it can be hours.
All of the other statements are correct.
(Rogers, MC, et al. Textbook of Pediatric Intensive Care, 3rd
Edition; pp 1442–1443)
5.
D, E, C, B, F, A
Chemotherapy may
promote the development of a coagulopathy associated with an increased risk of
hemorrhage or thrombosis. Actinomycin
D and other antibiotics decrease the vitamin K dependent clotting factors.
Anthracycline increases fibrinolysis.
L-asparaginase may cause hypofibrinogenemia.
Methotrexate can cause an antithrombin III deficiency.
Vincristine may cause chronic hepatic dysfunction. Glucocorticoids
increase the levels of factors II, VII, VIII and X. (Rogers, MC, et al. Textbook
of Pediatric Intensive Care, 3rd Edition; pp 1442-1457)
6.
A
Primary pulmonary parenchymal
involvement with leukemia is very rare. Parenchymal
involvement is occasionally seen with histiocytosis X and metastatic disease (eg.
Osteogenic and Ewings sarcoma, and Wilms tumor). All other statements are
correct. (Rogers, MC, et al. Textbook
of Pediatric Intensive Care, 3rd Edition; pp 1445–1447, Table
42.8)
7.
T, T, F, T, T, F
A variety of
chemotherapeutics as well as radiotherapy can cause cardiomyopathy.
Effects appear to be dose related. Radiotherapy
may cause pericarditis with a chronic effusion.
Histologically, interstitial fibrosis with vascular narrowing is seen.
(Rogers, MC, et al. Textbook of Pediatric Intensive Care, 3rd
Edition; pp 1447–1448) The onset
of pneumocystis carinii infection typically occurs 3–6 months after bone
marrow transplantation. Bacterial or fungal infection can occur within the first
2 weeks. CMV infection occurs 6–12 weeks after bone marrow
transplantation. (Rogers, MC, et al. Textbook of Pediatric Intensive Care,
3rd Edition; pp 1454–1455)
8.
B, D, A, C
The child with neoplastic
disease may acquire a variety of neurologic deficits related to the neoplasm,
the therapy or a combination of both. Methotrexate
is associated with aseptic meningitis, arachnoiditis, demyelinization,
somnolence and chronic leukoencaphalopathy.
Cisplatin may cause ototoxicity, cerebral edema and seizures. Vincristine
is associated with SIADH. 5-FU may cause acute cerebellar ataxia. (Rogers, MC,
et al. Textbook of Pediatric Intensive Care, 3rd Edition; pp
1449–1452; Table 42.17)
9.
B, D, A Chemotherapy is commonly
associated with renal injury. High dose methotrexate is associated with renal
tubular injury. Cisplatin may cause
tubular necrosis; Cyclophosphamide and Ifosfamide are both associated with
hemorrhagic cystitis. L-Aparaginase is not associated with renal injury.
(Rogers, MC, et al. Textbook of Pediatric Intensive Care, 3rd
Edition; pp 1452–1453; Table 42.18)
10.
D
CMV infection occurs most commonly
6–12 weeks after bone marrow transplantation.
All of the other statements are correct. (Rogers, MC, et al. Textbook
of Pediatric Intensive Care, 3rd Edition; pp 1454–1455)
11.
A, D, B, C, E
Chemotherpeutic agents form the mainstay of treatment for
childhood neoplasms. All agents act by disrupting some aspect of normal cell
growth or division. Antimetabolites interact with various cell enzymes (eg.
Methotrexate inhibits the activity of dihydrofolate reductase). Vincristine, a
vinca alkaloid, inhibits microtubule function within the cell. This prevents the
formation of the spindle apparatus during metaphase, thus inhibiting cell
division. The anthracyclines (daunorubicin and doxorubicin) and Actinomycin D
inhibit the synthesis of DNA in tumor cells.
Alkylating agents like cyclophosphamide cause breaks in the DNA strands.
The glucocorticoids are directly lymphocytotoxic to lymphoid leukemia and
lymphoma cells. (Rogers, MC, et al. Textbook of Pediatric Intensive Care,
3rd Edition; pp 1456 –1458; Tables 42.23, 42.24, 42.26 and 42.27)
12.
E
Von Willebrand’s Disease is the
most common inherited bleeding disorder. One percent of the population have
detectable abnormalities in the VWD protein.
All of the statements are correct. (Rogers, MC, et al. Textbook of
Pediatric Intensive Care, 3rd Edition; pp 1414–1416; Figure
41.2)
13.
T, F, T, T, T, F
The preferred
source for factors II, VII, X and antithrombin III is fresh frozen plasma.
Vitamin K is not a stored vitamin. (Rogers, MC, et al. Textbook of Pediatric
Intensive Care, 3rd Edition; pp 1415–1416)
14.
D
Anti-thrombin III, Protein C and Protein S are the main components of the
anti-thrombotic system. Thrombomodulin
and heparin cofactor II, among others, are also included as endogenous
anticoagulants. Protein B is not
included among these components. (Rogers, MC, et al. Textbook of Pediatric
Intensive Care, 3rd Edition; pp 1421; Figure 41.3)
15.
E
When the liver itself is
diseased, abnormal coagulation results. The liver synthesizes fibrinogen,
pro-thrombin, protein C, protein S, antithrombin III, plasminogen, and factors
V, VII, IX, X, XI and XII. All of
the above statements are correct. (Rogers, MC, et al. Textbook of Pediatric
Intensive Care, 3rd Edition; pp 1408 – 1409 and 1415 – 1416)
16.
B
Epsilon-aminocaproic acid prevents
the breakdown of the fibrin clot by complexing with plasmin to prevent its
fibrinolytic activity. Protamine is
used to reverse the effects of heparin. Vitamin
K deficiency generally occurs within 2-3 days following cardiopulmonary bypass.
D-dimers are rarely elevated. (Rogers, MC, et al. Textbook of
Pediatric Intensive Care, 3rd Edition; pp 1405–1406; Table
41.11; Chang, AC, et al. Pediatric Cardiac Intensive Care; pp 397–399)
17.
D
Massive transfusion is defined as
the replacement of at least one blood volume; estimated as 75 mL/kg for children
less than 1 year of age and burn victims, and 70 mL/kg for all others. All of
the above statements are true. (Rogers,
MC, et al. Textbook of Pediatric Intensive Care, 3rd Edition;
pp 1403-1408)
18.
C
A variety of metabolic
abnormalities can be induced by massive transfusion. 2,3-diphosphoglycerate is
decreased in transfused red cells, which increases red cell affinity for oxygen.
Thus, oxygen unloading to tissues may be impaired.
All other statements are correct. (Rogers,
MC, et al. Textbook of Pediatric Intensive Care, 3rd Edtion;
pp 1421–1422; Gilman, AG, et al. Goodman and Gilman’s The Pharmacological
Basis of Therapeutics, 8th Edition; pp 1316)
19.
E
Antithrombin III primarily
inhibits the vitamin K dependent procoagulant factors (II, VII, IX, and X).
Deficiency will lead to recurrent thrombosis. Heparin induced anti-platelet
antibodies occur in approximately 5% of patients receiving heparin therapy. Most
cases are mild with platelet counts above 100,000/uL.
All of the above statements are correct. (Rogers, MC, et al. Textbook
of Pediatric Intensive Care, 3rd Edition; pp 1406–1407)
20.
D
Two forms of acute, heparin
induced thrombocytopenia occur. The
mild form occurs in approximately 5% of patients, 4 – 15 days after initiation
of full-dose heparin therapy (platelet counts above 100,000/uL). Severe
thrombocytopenia occurs less frequently. The
more severe form is associated with thrombotic complications.
All of the above statements are correct. (Gilman, AG, et al. Goodman
and Gilman’s The Pharmacological Basis of Therapeutics, 8th
Edition; pp 1316)
21.
E
Protein C activation is controlled
by several different mechanisms, including by thrombomodulin. Protein C activation and thrombin generation are tightly
coupled. Acquired and hereditary
deficiencies are the primary cause of thrombophila. (Nathan, DG, et al. Nathan
and Oski’s Hematology of Infancy and Childhood, 5th
Edition; pp 1545–1547)
22.
E
In contrast to heparin, which acts as a cofactor with antithrombin III to
prevent coagulation, the plasminogen activators, urokinase, streptokinase and
tPA increase fibrinolysis, thereby lysing the clot. Plasminogen is cleaved into plasmin by these activators.
Plasmin lyses clot directly. All
of the above statements are correct. (Rogers, MC, et al. Textbook of
Pediatric Intensive Care, 3rd Edtion; pp 1415–1416, 1423-1424)
23.
E
Both heparin and plasminogen activators (streptokinase, t-PA) may be used
to treat arterial thrombosis. The PTT should be kept 1.5–2.0 times normal
during heparin therapy. Note,
younger neonates may be resistant to thrombolytic therapy, possibly due to lower
levels of plasminogen. (Rogers, MC, et al. Textbook of Pediatric Intensive
Care, 3rd Edition; pp 1424)
24.
E
Disorders in children that are
treated with chronic anticoagulants include cardiac disorders (prosthetic
valves, Blalock-Taussig shunts, endovascular shunts), some cerebrovascular
events, Kawasaki’s disease. All
of the above statements are correct. (Nathan, DG, et al.
Nathan and Oski’s Hematology of Infancy and Childhood, 5th
Edition; pp 1704)
25.
C, D, A Aspirin is a potent and
irreversible inhibitor of cyclooxygenase and thromboxane A2. Sulfinpyrazone like
aspirin is also a nonsteroidal anti-inflammatory agent that reversibly inhibits
cyclooxygenase. (Gilman, AG, et al. Goodman and Gilman’s The
Pharmacological Basis of Therapeutics, 8th Edition; pp 652, 1524)
26.
D
Thromboprophylaxis for prosthetic
heart valves has reduced the occurrence of thromboemolic events from
approximately 6% to under 2%. The
other statements are true. (Michelson, AD, et al. Antithrombotic Therapy in
Children, Chest1998; 114 (5 Suppl):
748S–769S)