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AHN 554 > Sickle Cell > Flashcards

Sickle Cell Flashcards

1
Q

what is sickle cell dz

A
  • inherited autosomal recessive disorder

- Defect in hemoglobin molecule , specifically a variant in beta globin polypeptide

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2
Q

HbF

A

fetal hemoglobin, replaced by HbA (adult hemoglobin) during postnatal period

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3
Q

HbS

A

sickle hemoglobin, inherited variant of HbA

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4
Q

Hemoglobin S

A

variants of beta globin gene (HbS)

-dz expression occurs with inheritance of 2 copies of HbS, or HbS and another copy of beta globin variant

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5
Q

Sickle cell trait

A

when someone has sickle cell trait, they have HbS and HbA, no expression of disease. Under extreme circumstances pt can have mild expression of dz
May get renal dz, hematuria

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6
Q

Sickle cell dz (SS)

A
  • pt will have HbS and Hbs, moderate to severe hemolytic anemia in first months of life.
  • uncompensated hemolytic anemia, shortened RBC survival (16 days vs 120 days normally), increased RBC production (Erythropoiesis)
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7
Q

Hemoglobin sickle C dz (SC)

A

HbS and HbC, moderate to severe hemolytic anemia, increased risk for splenomegaly, retinal dz, and aseptic necrosis

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8
Q

Sickle/Beta Thalassemia (SA)

A

HbS and beta thalassemia, manifestations depend on degree of thalassemia

Similar to homozygous SS dz but have milder symptoms and crises

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9
Q

Hallmark of sickle cell

A

vaso-occlusive phenomena and hemolytic anemia

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10
Q

Sickle cell crisis

A

vaso occlusive leading to tissue ischemia and acute pain, acute chest syndrome, sequestration crisis, aplastic crisis, increased risk for stroke, organ damage, bacterial infection, and complications from blood transfusions

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11
Q

What 2 hematology dzs are protective against malaria

A

Thalassemia and sickle cell trait are protective against plasmodium falciparum malaria

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12
Q

neuro complications with sickle cell

A

TIA, stroke, unexplained coma, epilepsy, neurocognitive decline

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13
Q

Leading cause for morbidity and mortality for sickle cell

A
  • infection due to splenic dysfunction
  • bacterial infections: step pneumoniae and haemophilus influenzae
  • viral infections: H1N1, parovirus
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14
Q

Sickle cell symptoms related to vaso-occlusion

A

generalized pain in long bones and joints, fever, fatigue, malaise, abdomen pain, nausea, vomiting, decreased appetitie, swelling in hands and feet and joints, priapism

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15
Q

sickle cell symptoms unrelated to vaso-occlusion

A

depression, chronic hemolytic anemia, tachycardia, fever, tachypnea, splenomegaly (frequent in (SC), hepatomegaly, jaundice, cardiomegaly, retinopathy, physical immaturity, leg ulcers from trauma

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16
Q

test to confirm sickle cell

A
  • hemoglobin electrophoresis

- Will confirm hemoglobin genotype for sickle cell dz, and hemoglobin F (fetal) and A2 for presence of thalassemia

17
Q

Hematocrit in sickle cell

A

low, usually 20-30

18
Q

retic count in sickle cell

A

markedly elevated, 10-25%

19
Q

WBC count in sickle cell

A

elevated 12,000-15,000

20
Q

peripheral blood smear sickle cell

A

5-50% of cells are sickled

21
Q

Positive diagnosis of sickle cell, hemoglobin S: Hemoglobin S,A, and F

A

-Hemoglobin S: constitutes 85-95% of hemoglobin
-Hemoglobin A: not present in homozygous S dz
-Hemoglobin F are increased (associated with more benign dz)
)

22
Q

What lab levels are elevated with sickle cell (8)

A 
Serum indirect bilirubin (levels high)
Serum transaminase
Alkaline phosphatase
Factor VIII activity
von Willebrand factor antigen
Thrombin
Platelets (thrombocytosis)
Howell Jolly bodies and target cells with hyposplenism
23
Q

what labs are decreased in sickle cell (3)

A

Serum creatinine (b/c of increased GFR of kidneys, except in renal failure. Expect protein in urine
Plasma protein C
Plasma Protein S

24
Q

Imaging for sickle cell dz

A

Transcranial Doppler US to identify risk for stroke

CT and MRI to rule out aseptic necrosis

25
Q

Pain management in sickle cell

A
  • prompt tx
  • morphine, dilaudid
  • hospitalize if pain does not improve
  • oral opiods for breakthrough pain
  • NS boluses (0.5-1L)to reduce viscosity but avoid overload
  • Maintenance fluids: 1/4 or 1/2 NS and encourage oral intake
26
Q

O2 use in sickle cell crisis

A

give to hypoxic patients with partial pressure <60-70 mmHg, use humidified 02 at 40%

27
Q

Other meds for sickle cell crisis besides opiates

A

NSAIDS
Antihistamines
Tricyclic antidepressants
Phenothiazines for nausea and vomiting

28
Q

When to give transfusion

A 
Symptomatic anemia
acute events
preop for major surgery
acute sepsis or meningitis 
Prevention of recurrent stroke
Chronic hypoxia
recurrent and chronic anemia
29
Q

Prevention measures and chronic dz management

A
  • Folic acid daily
  • long term transfusion to prevent stroke if US positive
  • Pneumo, hepatitis, and flu vaccines
  • Foot care and protective shoes
  • annual PCP visit and 2-3 per year after age 30
  • annual eye exam until adult then every 2-3 years
  • chelation therapy with Deferoxamine if in iron overload
  • routine exchange transfusions
  • Ace inhibitors for microalbuminuria
  • Avoid temp extremes and physical exertion
30
Q

Birth control with sickle cell

A

NOT contraindicated

consider genetic counseling

31
Q

Sickle cell cure

A
  • in children

- with hematopoietic cell transplantaion

32
Q

Home meds that may help pain crisis

A

hydroxyurea and aliphatic butyrate salts decrease pain crisis by increasing level of fetal hemoglobin

33
Q

When is onset of sickle cell?

A

during 1st year of life, when hemoglobin F levels fall as a signal is sent to switch from production of gamma globin to beta globin

34
Q

Acute chest syndrome

A

acute chest pain , hypoxemia, pulmonary infiltrates on CXR

35
Q

Complications of sickle cell

A

-Osteomyelitis from ischemic necrosis of bone
-Infarction of renal medulla causing hematuria (happens more with sickle cell trait
-Retinopathy
-Pulmonary HTN
Delayed puberty
More prone to infection because of hyposplenism

36
Q

Presentation of sickle cell pt

A 
chronically ill
jaundiced
hepatomegaly
enlarged heart with systolic murmurs and hyperdynamic precordium
Nonhealing ulcers
retinopathy
37
Q

average life expectancy and cause of death for sickle cell

A

40-50 years old

death caused by multisystem organ failure

AHN 554 (13 decks)

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