Chapter 47: The Child with a Hematologic Alteration

Question 1

characterized by uncontrolled formation and deposition of fibrin thrombi

Answer 1

disseminated intravascular coagulation (DIC)

Question 2

manifestations may include excessive bleeding from injury, petechiae, and blood oozing from minor procedure sites

Answer 2

disseminated intravascular coagulation (DIC)

Question 3

treatment for iron deficiency anemia involves ___________

Answer 3

replacing iron

Question 4

acute sequestration crisis can result in these two complications

Answer 4

acute anemia
respiratory failure

Question 5

thalassemia major presents in children who inherit (1,2) copy/copies of the gene mutation

Answer 5

2

Question 6

patients with moderate to severe hemophilia are at risk for bleeding in the _________

Answer 6

joints

Question 7

treatment for painful episodes of a vasoocclusive crisis includes oral or IV __________ such as ___________ and/or _________, oral/IV ____________, aggressive ____________________ use, consistent pain ________________, and ______________ pain management

Answer 7

analgesics, morphine, NSAIDs, hydration, incentive spirometry, assessment, nonpharmacologic

Question 8

thalassemia minor presents in children who inherit (1,2) copy/copies of the gene mutation

Answer 8

1

Question 9

three causes of iron deficiency anemia in children

Answer 9

1. decreased iron intake
2. increased iron/blood loss
3. periods of increased growth

Question 10

complex process that results in simultaneous bleeding and clotting

Answer 10

disseminated intravascular coagulation (DIC)

Question 11

treatment for thalassemia focuses on three main aspects

Answer 11

RBC transfusions, chelation therapy, splenectomy

Question 12

most common form of anemia in children

Answer 12

iron deficiency anemia

Question 13

this condition is caused by a genetic abnormality in hemoglobin synthesis, where there is a reduction or absence of one of the chains of normal hemoglobin

Answer 13

thalassemia

Question 14

iron deficiency anemia has been linked to consumption of ___________, particularly before 12 months of age

Answer 14

cows’ milk

Question 15

characterized by underproduction of a carrier protein for factor VIII

Answer 15

von Willebrand disease

Question 16

another name for thalassemia major

Answer 16

Cooley anemia

Question 17

two age groups that have the most rapid growth and therefore increase risk of iron deficiency anemia

Answer 17

infants, adolescents

Question 18

diagnosis of thalassemia is made based on this lab work (4)

Answer 18

CBC, reticulocyte count, serum iron, TIBC

Question 19

dactylitis (hand-and-foot syndrome) in vasoocclusive crisis occurs in children aged _________ to _________ and is characterized by ________________________

Answer 19

6 months, 4 years
swelling of hands or feet, pain, warmth in affected area

Question 20

diagnosis of iron deficiency anemia involves these four lab tests

Answer 20

CBC, serum ferritin, serum iron, TIBC

Question 21

this bleeding disorder can cause septic shock and multiorgan failure

Answer 21

disseminated intravascular coagulation (DIC)

Question 22

hemophilia C lacks factor ____

Answer 22

XI

Question 23

the dietary supplement ____________ helps blood cells form appropriately in patients with sickle cell disease

Answer 23

folic acid

Question 24

treatment for acute chest syndrome with vasoocclusive crisis involves IV ___________ at ______ times maintenance, ___________, ___________, _____ transfusion, and ___________.

Answer 24

hydration, 1-1.5, antibiotics, oxygen, RBC, analgesics

Question 25

acute sequestration crisis is characterized by blood volume pooling in ________, causing enlargement, and life-threatening _____________

Answer 25

spleen, hypovolemic shock

Question 26

hemophilia B lacks factor ____

Answer 26

IX

Question 27

treatment of aplastic crisis involves these two things

Answer 27

1. RBC transfusions 2. treatment of symptoms

Question 28

painful episodes of a vasoocclusive crisis are typically characterized by pain in _______ or ________

Answer 28

bones, joints

Question 29

five sources of dietary iron

Answer 29

egg yolks, dark green leafy vegetables, dried fruits (apricots), beans, liver

Question 30

thalassemia is inherited in this pattern

Answer 30

autosomal recessive

Question 31

acquired bleeding disorder that results in low platelet counts with an unknown cause

Answer 31

immune thrombocytopenic purpura (ITP)

Question 32

priapism with vasoocclusive crisis is characterized by

Answer 32

persistent, painful erection

Question 33

hereditary blood disorder that results in an interruption in clotting cascade, where the patient lacks one of the clotting factors

Answer 33

hemophilia

Question 34

acute chest syndrome in a vasoocclusive crisis is typically characterized by these four manifestations

Answer 34

chest pain, fever, cough, abdominal pain

Question 35

sickle cell disease is typically found in people with these four ethnic backgrounds

Answer 35

African, Mediterranean, Middle Eastern, Indian

Question 36

manifestations include hepatosplenomegaly, pallor, bronze or greenish-yellow skin tone, anemia, delayed growth

Answer 36

beta-thalassemia

Question 37

five things that can precede a sickle cell crisis

Answer 37

illness, dehydration, hypoxia, increased altitude, trauma

Question 38

aggressive incentive spirometry use for painful episodes during a vasoocclusive crisis means ___ breaths every __ hours while awake

Answer 38

10, 2

Question 39

treatment of acute sequestration crisis includes emergency treatment to restore _____________________ with ____________ and __________ infusion, long-term ____________ therapy if recurrent, and eventual ______________ in cases of persistent recurrence

Answer 39

circulating blood volume, crystalloid, colloid (blood), transfusion, splenectomy

Question 40

lab tests show these six things when iron deficiency anemia is present

Answer 40

low RBCs decreased hemoglobin smaller RBCs decreased serum ferritin decreased serum iron increased TIBC

Question 41

these two medication classes help treat pain related to sickle cell disease

Answer 41

opioids (morphine) NSAIDs

Question 42

hemophilia A lacks factor ____

Answer 42

VIII

Question 43

iron deficiency anemia is most common in these three age groups

Answer 43

1. premature infants 2. infants between 9-24 months 3. adolescents

Question 44

to diagnose immune thrombocytopenic purpura, a ____________ is performed to rule out other causes of thrombocytopenia

Answer 44

bone marrow biopsy

Question 45

patients with sickle cell may have ___________ growth and puberty and may appear _______ or ______________ due to chronic hemolytic anemia

Answer 45

delayed, pale, jaundiced

Question 46

occurs when bone marrow ceases production of all cells

Answer 46

aplastic anemia

Question 47

three characteristic facial features of beta-thalassemia

Answer 47

frontal bossing (prominent and protruding forehead) maxillary prominence wide-set eyes with flattened nose

Question 48

aplastic crisis is characterized by

Answer 48

profound anemia caused by diminished erythropoiesis

Question 49

characterized by sudden onset of petechiae and bleeding from mucus membranes

Answer 49

immune thrombocytopenic purpura (ITP)

Question 50

treatment for cerebrovascular accident with vasoocclusive crisis includes long-term ________________ therapy or _________________, possible ___________ therapy, and sometimes requires extensive _____________.

Answer 50

RBC transfusion, erythrocytapheresis, chelation, rehabilitation

Question 51

this sickle-cell crisis includes these manifestations: decreased hemoglobin, acutely ill-looking child, pallor, irritability, tachycardia, impressively enlarged spleen, hypovolemic shock

Answer 51

acute sequestration crisis

Question 52

sickle cell disease refers to a group of congenital disorders, which include these three

Answer 52

sickled hemoglobin chronic hemolytic anemia ischemic tissue injury

Question 53

iron supplements are typically given ____ times a day and should be given ______________ to increase absorption

Answer 53

3, between meals

Question 54

treatment for hemophilia involves these three points

Answer 54

patient education to prevent injury/bleeding infusions of missing factor RICE therapy to treat bleeds

Question 55

treatment for priapism with vasoocclusive crisis includes ___________, ___________, avoiding ______________, and _____________ therapy if prolonged

Answer 55

analgesics, hydration, hot and cold packs, transfusion

Question 56

treatment for dactylitis is ______________ and involves these three things

Answer 56

self-limiting oral analgesics, IV or oral hydration, rest

Question 57

treatment of disseminated intravascular coagulation (DIC)

Answer 57

give platelets and anticoagulants at same time

Question 58

typical hemoglobin goal for patients with thalassemia

Answer 58

11

Question 59

Sources of __________ such as _______ impede absorption of iron

Answer 59

calcium, milk

Question 60

manifestations include general pallor, pale mucous membranes and conjunctiva, tachycardia, tachypnea, lethargy, increased fatigue, and irritability

Answer 60

iron deficiency anemia