Hematologic Conditions Flashcards

1
Q

______ is the most prevalent / preventable nutritional disorder

A

Iron deficiency anemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What populations of children have highest risk of developing iron deficiency anemia? (2)

A
  • Low socioeconomic status
  • 6 months - 2 years old
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What is the primary risk factor of iron deficiency anemia in young children?

A

Inadequate intake of dietary iron

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What is the primary risk factor of iron deficiency anemia in school-aged children?

A

Blood loss

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

______ decrease the incidence of iron deficiency anemia

A

WIC programs

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What is heme iron?

A

Iron found in animal sources (best form, easily absorbed)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What are some examples of foods rich in heme iron? (4)

A
  • Red meat
  • Seafood
  • Chicken
  • Eggs
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What is non-heme iron?

A

Iron found in plant sources

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What are some examples of foods rich in non-heme iron? (3)

A
  • Dark, leafy greens
  • Iron-fortified cereals
  • Iron-fortified formulas
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

When is iron normally transferred from mother to fetus?

A

During the last trimester of pregnancy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

A full-term infant has adequate iron stores for ______

A

5 - 6 months

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

A pre-term infant has adequate iron stores for ______

A

2 - 3 months

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Iron is needed for the formation of ______

A

Hemoglobin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Cow’s milk should not be given to a child until ______

A

12 months

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Describe the effects of early consumption of cow’s milk

A

Causes microscopic GI bleeding –> iron deficiency anemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Manifestations of iron deficiency anemia are related to …

A

Degree of tissue hypoxia (from lack of hemoglobin)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

______ bonds to stored iron

A

Ferritin

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

What type of iron supplement is most easily absorbed?

A

Ferrous iron

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

______ converts iron from ferritin into hemoglobin

A

Folic acid

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

What are the manifestations of iron deficiency anemia? (11)

A
  • SOB
  • Tachycardia
  • Muscle weakness
  • Fatigue
  • Poor feeding
  • Pale skin / mucous membranes
  • Hair loss
  • Headache
  • Decreased attention span
  • Depression
  • Dizziness
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

What laboratory findings are associated with the diagnosis of iron deficiency anemia? (4)

A
  • Decreased ferritin
  • Decreased RBC
  • Decreased H & H
  • Decreased MCV
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Describe the management of iron deficiency anemia (3)

A
  • Iron-fortified cereal
  • Oral iron supplements (ferrous iron)
  • Monitor for bleeding / malabsorption
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

Describe the management of iron deficiency anemia for infants (4)

A
  • Encourage breast milk
  • Iron-fortified formula
  • 2 servings of iron-rich food per day (after 6 months)
  • No cow’s milk until 12 months
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

Why is breast milk encouraged for infants?

A

Provides better iron absorption than formula

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Describe education associated with iron supplementation (4)

A
  • Give BID between meals (may give with / after meals if unable to tolerate)
  • Take with vitamin C to improve absorption
  • Increase protein / folic acid
  • Liquid form may stain teeth
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

What is the primary side effect associated with iron supplementation?

A

Dark stools

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

What are the possible results of severe / prolonged iron deficiency anemia?

A

Cognitive / behavioral impairment

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

What is sickle cell disease?

A

C-shaped RBCs due to abnormal hemoglobin gene (Hgb S)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

What type of disorder is sickle cell disease?

A

Autosomal recessive disorder

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

What population has highest risk of developing sickle cell disease?

A

African Americans

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

Describe the pathophysiology of sickle cell disease (2)

A
  • Sickled RBCs are unable to pass through circulation
  • Vaso-occlusion causes hypoxia / ischemia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

Most sickled cells …

A

Regain their shape, however irreversible sickling is possible

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

Manifestations of sickle cell disease are related to … (3)

A
  • Obstruction
  • RBC destruction
  • Vascular inflammation
34
Q

Describe the occurrence of manifestations of sickle cell disease (2)

A
  • Asymptomatic for first 4 - 6 months
  • Symptoms present during period illness (causes exacerbation)
35
Q

Why are children with sickle cell disease asymptomatic for the first 4 - 6 months of life?

A

Presence of fetal hemoglobin (HbF) - does not sickle

36
Q

Describe the pathophysiology of a vaso-occlusive crisis (2)

A
  • Oxygen cannot reach tissues, leading to ischemia
  • Causes extreme pain
37
Q

How long does vaso-occlusive crisis pain typically last?

A

4 - 6 days

38
Q

What is the first sign of a vaso-occlusive crisis in young children?

A

Hand-foot syndrome

39
Q

Describe hand-foot syndrome

A

Tender / warm / swollen hands and feet

40
Q

What are the manifestations of a vaso-occlusive crisis? (6)

A
  • CVA
  • SOB
  • Fatigue
  • Dizziness
  • Pneumonia symptoms
  • Priapism
41
Q

When is the peak incidence of CVAs associated with sickle cell disease?

A

Birth - 5 years

42
Q

Where are some common areas for pain associated with sickle cell disease? (5)

A
  • Lumbosacral spine
  • Shoulder
  • Elbow
  • Knee
  • Femur
43
Q

Describe the pathophysiology of a sequestration crisis (2)

A
  • Blood pooling in spleen / liver
  • Decreased circulation causes shock / cardiac arrest
44
Q

A sequestration crisis is typically seen in children of what age?

A

< 3 years old

45
Q

What is the mortality rate of a sequestration crisis?

46
Q

When is sickle cell disease usually diagnosed?

A

Newborn screening

47
Q

If sickle cell disease is not diagnosed in infancy, when does it usually manifest?

A

Toddler / pre-school years due to secondary illness causing excessive stress in the body leading to sickling

48
Q

What tests are used for the diagnosis of sickle cell disease? (3)

A
  • CBC
  • Sickledex
  • Hemoglobin electrophoresis
49
Q

Describe the prevention of sickle cell disease crisis (3)

A
  • Immunizations
  • Prophylactic antibiotics
  • Avoid stress on the body (fever, infection, acidosis, dehydration, hypothermia)
50
Q

Prophylactic antibiotics for sickle cell disease are especially important for …

A

Children who have had their spleen removed

51
Q

Describe the management of sickle cell disease (7)

A
  • Blood transfusions
  • Bedrest
  • Oxygen
  • NSAIDS
  • Hydroxyurea
  • Fluid / electrolyte replacement
  • Warm compress
52
Q

What are the functions of blood transfusions for sickle cell disease? (2)

A
  • Decrease blood viscosity
  • Replace lost RBCs
53
Q

What is the function of hydroxyurea for sickle cell disease?

A

Increases fetal hemoglobin (HbF) which does not sickle

54
Q

It is important to maintain an oxygen saturation of ______ in children with sickle cell disease

55
Q

Hypoxia related to sickle cell disease causes ______

A

Metabolic acidosis

56
Q

Why is a warm compress used for children with sickle cell disease?

A

Vasodilation to prevent vaso-occlusion

57
Q

DO NOT ______ to hot / swollen hands in sickle cell disease

58
Q

What pain medications are used for management of sickle cell disease? (3)

A
  • Codeine
  • Oxycodone
  • Morphine
59
Q

______ may be used for pain management of sickle cell disease

60
Q

Which pain medication is contraindicated for sickle cell disease?

A

meperidine (Demerol) - risk of seizures

61
Q

Most patients with sickle cell disease live into the ______ with optimal management

A

5th decade

62
Q

What age has the greatest risk for complications from sickle cell disease?

A

< 5 years old

63
Q

Most deaths from sickle cell disease in children < 5 years old are related to ______

64
Q

What is hemophilia A?

A

Deficiency of factor VIII

65
Q

What is the function of factor VIII?

A

Formation of thromboplastin (essential for blood clotting)

66
Q

What type of disorder is hemophilia A?

A

X-linked recessive disorder

67
Q

Describe the pathophysiology of hemophilia A (2)

A
  • Lack of factor VIII impairs coagulation
  • Causes fatal hemorrhage
68
Q

Where is the most common site of internal bleeding from hemophilia A?

A

Bleeding into the joint cavities / muscles

69
Q

Describe the risk of bleeding in various locations from hemophilia A (3)

A
  • GI tract bleeding = anemia
  • Neck / mouth / thorax bleeding = compromised airway
  • Intracranial bleeding = death
70
Q

______ occur after repeated bleeding episodes from hemophilia A

A

Bony changes / crippling deformities

71
Q

Describe the occurrence of bleeding associated with hemophilia A (2)

A
  • As child learns to walk, bruising may cause bleeding into joints (hemarthrosis)
  • By age 3 - 4, minor lacerations may cause persistent bleeding
72
Q

What are common forms of bleeding in hemophilia A? (2)

A
  • Hematuria
  • Epistaxis
73
Q

Describe the effects of hemarthrosis (2)

A
  • Limited movement in elbows, knees, and ankles
  • Risk of degenerative joint changes later in life
74
Q

______ is rare in hemophilia A during the first year of life

A

Spontaneous bleeding

75
Q

Injections / firm holding can result in ______ is hemophilia A

76
Q

What laboratory findings are associated with the diagnosis of hemophilia A? (2)

A
  • Decreased factor VIII
  • Prolonged PT / PTT
77
Q

Describe the management of hemophilia A (7)

A
  • Factor VIII concentrate
  • DDAVP - synthetic antidiuretic (increases factor VIII)
  • Exercise / physical therapy to maintain joint mobility
  • Rest
  • Ice
  • Compression
  • Elevation
78
Q

Why is ice used for children with hemophilia A?

A

Vasoconstriction to stop bleeding

79
Q

Children with hemophilia A must wear ______

A

A medic alert bracelet

80
Q

Describe the prognosis of hemophilia A

A

With proper monitoring / treatment children lead relatively normal lives

81
Q

Patients treated with plasma products for hemophilia A prior to 1985 …

A

Have likely HIV exposure