Brainscape's Knowledge GenomeTM


Top Flashcards (Certified)
All Flashcards

Oncology > Sickle cell disease > Flashcards

Sickle cell disease Flashcards

1
Q

How is sickle cell disease inherited?

A

autosomal recessive; two abnormal Hgb genes

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

What is the mutated version of the Hgb gene?

A

Hemoglobin S

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

What is the normal version of the Hgb gene?

A

Hemoglobin A

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

What is the cause of the mutated HbS gene?

A

glutamic acid is replaced with valine

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

How do sickle cells affect blood flow?

A
  1. vaso- occlusion
  2. decreased oxygen delivery to tissues
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What type of hemoglobin do newborns have?

A

fetal hemoglobin (HcF)

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

When do symptoms of sickle cell disease present?

A

5 months of age after HbF decreases and HbS levels increase

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What are the symptoms of sickle cell disease?

A
  1. pain
  2. anemia
  3. growth delays
  4. stroke
  5. infections due to blocked spleen
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What are acute complications of sickle cell disease?

A
  1. vaso- occlusive crisis
  2. fever
  3. acute chest syndrome
  4. anemia
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What are the chronic complications of sickle cell disease?

A

infection prophylaxis
hydroxyurea

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

What is the most common complication of vaso-occlusive crisis?

A

impeded blood flow –> pain

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

What can a vaso-occlusive crisis be caused by?

A

infection
emotional stress
exposure to cold, wind, or high-altitude

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

Where and how does pain usually present?

A

lower back, chest, legs/arms ina symmetric pattern

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

What are treatments for vaso-occlusive crisis?

A
  1. IV fluids
  2. NSAIDs
  3. opioid analgesics
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

How soon should treatment begin for a vaso-occlusive crisis?

A

30 min of triage- 60 min of registration

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

What is used for mild-moderate pain?

A

anti-inflammatory meds (NSAIDs or acetaminophen)

17
Q

What is used for severe pain?

A

Oral opioid analgesics

18
Q

What routes of opioid analgesic should NOT be used?

A

IM
transdermal

19
Q

What kind of infections are patients with SCD at higher risk for?

A

encapsulated organisms caused by functional asplenia

20
Q

When is a fever treated as a medical emergency?

A

any fever (orla >/= 38.3) in a child with SCD

21
Q

What bugs need to be covered for fever in SCD?

A

s. pneumonia
GM - enteric organisms
possibly mycoplasma pneumonia

22
Q

What agents are used to treat fever is SCD?

A

3rd gen cephalosporins (ceftriaxone)
+/- macrolide (mycoplasma pneumoniae)
+/- vancomycin (acutely ill)

23
Q

What is the cause of acute chest syndrome?

A

vaso-occlusion of pulmonary vasculature

24
Q

How is acute chest syndrome diagnosed?

A

new pulmonary infiltrate on chest x ray
AND ONE OF THE FOLLOWING
Fever/ tachypnea/ chest pain/ dyspnea/ hypoxia

25
What is the treatment for acute chest syndrome?
1. supplemental oxygen 2. bronchodilators 3. empiric ABX (cephalosporin and macrolide)
26
Why should opioids and IV fluids be used with caution in acute chest syndrome?
concern for analgesia-induced hypoventilation and pulmonary edema
27
When are blood transfusions recommended?
life-threatening complications: symptomatic severe acute chest syndrome multisystem organ failure medium or high-risk surgical procedures
28
When should blood transfusions NOT be used?
asymptomatic anemia uncomplicated pain crisis
29
What agent is used for infection prophylaxis to decrease the incidence of pneumococcal disease?
Penicillin Erythromycin (allergy)
30
What vaccines should children get to prevent infection?
1. PCV 13 (newborns) 2. meningococcal conjugate vaccine (2 months and older) 3. annual influenza vaccine
31
Why is Hydroxyurea used in SCD?
increases FHb, reduces hospitalization end-organ damage, and stroke
32
What role does Hydroxyurea have in SCD?
prevent pharmacologic agent for pediatric patients with recurrent vaso-occlusive episodes
33
How long of a trial of the maximum tolerated dose is required before discontinuing treatment?
6 months
34
How long can hydroxyurea be taken if they have clinical response?
indefinitely
35
What are common adverse effects of Hydroxyurea?
1. myelosuppression (mild) 2. skin/ nail changes 3. GI disturbances 4. hair loss/ thinning 5. leg ulcers
36
What are the serious adverse effects of Hydroxyurea?
1. growth delays 2. infertility 3. possible secondary leukemias or other cancers
37
At what age should Hydroxyurea begin OFFERED if asymptomatic?
9 y/o
38
What does the therapy of Hydroxyurea effectiveness depend on
adherence to the dosage regimen

Oncology (22 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2025 Bold Learning Solutions. Terms and Conditions