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Pediatrics > Blood Dyscrasias > Flashcards

Blood Dyscrasias Flashcards

1
Q

Sickle Cell Anemia (SCA)

A

norman adult hemoglobin (HgbS) is partly or completely replaced by abnormal sickle hemoglobin S (HbS)

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2
Q

SCA Etiology

A

hereditary autosomal recessive disorder

primarily in africans/ eastern mediterrranean descent

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3
Q

SCA Dx

A 
Prenatal: Amniocentesis
Newborn screening: sickledex
hemoglobin electrophoresis
	-differentiates trait from disease
Transcranial Doppler (TCD) assess intracranial vascular flow ID CVA annually done ages 2-16
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4
Q

SCA Pathophysiology

A 
RBS sickling > blood viscosity
- blood flow obstruction
- tissue hypoxia
- tissue ischemia
- ischemia causes pain
> RBC destruction: chronic anemia
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5
Q

SCA s/s

A 
start around 6 mos
SOB/ fatigue
tachy
pallor
impaired healing/ freq infections
delayed physical growth
systolic heart murmur & heart failure
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6
Q

3 conditions causing RBCs to sickle

A

low oxygen concentrations
acidosis
dehydration

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7
Q

SCA: Vaso occlusive crisis

A 
Acute: 
-severe pain (bones, joints, abd)
-swollen joints, hands, feet
-anorexia, vomiting, fever
-hematuria
-obstructive jaundice
-visual dsiturbances
CVA
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8
Q

SCA: Vaso-occlusive chronic s/s

A 
resp inf
retinal detachment/ blindness
systolic murmurs
renal failure and enuresis
liver enlargement/ failure
spleen enlargement/ > inf
seizures
skeletal deformities
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9
Q

SCA Management

A 
bed rest
hydration
pain management
electrolyte replacement (corrective acidosis)
blood products
ABX (oral prophylactic penicillin)
heat & passive ROM
oxygen (short term)
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10
Q

SCA complication Prevention

A 
Transfusion Program 
-q 4-5- weeks
-maintaing Hgb 9-10
hydroxyureaw (PO med)
- > fetal hgb which doesn't sickle
BMT
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11
Q

SCA Pt & family education

A 
freq rest periods
no contact sports if spleen enlarged
avoid infections
avoid low O2 environments
adequate hydration
enuresis is a S/E of hydration
heat to affected area
maintain vaccination schedule
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12
Q

Hemophilia

A

A group of bleeding disorders from a congenital deficiency of coagulation proteins
most are X linked recessive factor VIII
mother is carrier and transmits to children

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13
Q

Hemophilia Dx

A

Females: DNA testing for trait
factor specific assays to ID deficiency
prolonged PTT

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14
Q

Hemophilia Pathophysiology

A 
Liver decreased clotting factor production 
	- factor assay <25%
injury response remains intact
	-vasoconstriction
	-platelet aggregation
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15
Q

Hemophilia s/s

A 
prolonged bleeding
hemorrhage
excessive bruising
spontaneous hematuria
hemathrosis (bleeding into joint cavity)
hematomas (swelling, pain, limited ROM)
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16
Q

Hemophilia Complication Prevention

A

Primary: regular schedule of receiving factor VIII
Secondary: factor infusion after joint bleed
Mild Cases: DDAVP (desmopression which > Factor VIII)
Antifibrinolytic agents (Amicar) inhibits breakdown of fibrin use for mouthwash or trauma surgery

17
Q

Hemophilia Management

A 
Blood transfusions for severe bleeding
RICE: rest, ice, compression, elevation
severe bleeding may need aspiration
pain management (No NSAIDS)
bleeding precautions
Active ROM (not passive)
18
Q

Idiopathic/ Immune Thrombocytopenia Purpura (ITP)

A

immune mediated platelet destruction cased by platelet antibodies
Autoimmune process

19
Q

ITP etiology

A

viral infections
drugs (heparin)
Collage vascular diseases (lupus)

20
Q

ITP types

A

Acute

  • majority of cases
  • self-limiting
  • resolves in days/weeks

Chronic
-duration > 6 mos

21
Q

ITP Dx

A

No difinitive test

Platelet count < 20,000

22
Q

ITP Management

A 
restrict activity
Anti-D antibody
platelet replacement
IVIG
Prednisone
Spenectomy for chronic severe form
23
Q

Aplastic Anemia

A

All formed elements of the blood are depressed

  • RBCs
  • WBCs
  • platelets
24
Q

Aplastic Anemia Etiology

A

Bone Marrow Depression
systemic diseases interfering w/ bone marrow activity
70% ideopathic

25
Q

Aplastic Anemia Dx

A

Bone Marrow Aspiration
Labs - < RBC
- < WBC
- < Platelets

26
Q

Aplastic Anemia Management

A

immunosupressive therapy
colony stimulating factors
BMT

27
Q

Iron Deficienct Anemia

A 
Physiologic Anemia
	-normal process
	-onset 2-3 mos
Iron Deficiency Anemia (9mos)
	-< iron intake
	-< iron stores
	-> iron loss
	-poor utilization of iron by body
28
Q

Iron Deficient Anemia Assessment

A

Overweight milk baby
pallor, pale mucous membranes
content, tired, fatigued
Ages Seen: 6-24mos, toddlers, female teens

29
Q

Iron Deficient Dx

A

< Hgb

TIBC totat iron binding capacity

30
Q

Aplastic Anemia s/s

A 
petechia
ecchymosis
pallor
epistaxis
fatigue/ weakness
tachycardia
anorexia
infection
31
Q

Iron Deficiency Tx

A

Infant: iron formula, iron supp, iron enriched cereal & food, limit milk intake to < 32 oz/day

Older child: > 12 mo. 24 oz milk max/day, may need to limit activities, need rest, iron supp

32
Q

Iron Supplement Education

A 
give on empty stomach
use dropper to avoid teeth stains
stools may be tarry/ > constipation
don't give w/ dairy
max effect: give in 3 divided doses/ day
O.D. can be fatal- child proof vitamins
over 6 months of age- give w/ citrus to > absorbtion

Pediatrics (8 decks)

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