Pediatrics Hematological / Oncological

Question 1

sickle cell is ___ ___ inheritance.

Answer 1

autosomal recessive

Question 2

With sickle cell there is severe ____ pain.

Answer 2

severe joint pain

Question 3

In sickle cell when the blood pools (often in the spleen) and causes splenomegaly & tenderness that is known as _____.

Answer 3

sequestration

Question 4

Acute exacerbation of sickle cell can be caused by ____, _____ , ____ ____, & _____.

Answer 4

hypoxia, exercise, high altitude, & fever.

Question 5

Name 5 common assessments you will see in a sickle cell patient.

Answer 5

1. pallor
2. pain (also chest pain)
3. fatigue
4. arthralgia (joint stiffness)
5. respiratory distress

Question 6

What are the top 4 treatments of sickle cell?

Answer 6

1. IV fluids (dilutes the blood)
2. Blood transfusion (better perfusion)
3. Oxygen (helps with hypoxia)
4. Medications

Question 7

_______ are the preferred pain managements for sickle cell.

Answer 7

analgesics

Question 8

_______ increases production of fetal hemoglobin to reduce crises.

Answer 8

Hydroxyurea

Question 9

_____ disease is acute systemic vasculitis.

Answer 9

Kawasaki Disease

Question 10

Kawasaki disease can result in coronary artery ____ & _____.

Answer 10

aneurysms & thrombosis

Question 11

A key assessment of Kawasakis disease is _____ tongue.

Answer 11

strawberry tongue (red & swollen)

Question 12

Since Kawasaki’s causes inflammation you see elevated _____.

Answer 12

elevated labs
1. CRP (C-reactive protein)
2. ESR (erythrocyte sedimentation rate)
3. WBC
4. Platelets

Question 13

____ is the treatment for Kawasaki disease.

Answer 13

Aspirin ( reduce fever & swelling)

Question 14

Aspirin prevents ____ ____.

Answer 14

blood clots

Question 15

What IV treatment do Kawasaki patients get?

Answer 15

Intravenous Immunoglobulin

Question 16

______ syndrome is acute encephalopathy following viral illness.

Answer 16

Reye Syndrome

Question 17

reyes syndrome is diagnosed by _____ biopsy.

Answer 17

liver biopsy

Question 18

Reye syndrome is characterized _____ edema & ____ changes in the liver.

Answer 18

Cerebral edema and fatty changes in the liver

Question 19

Reyes syndrome is associated with ____ use during a viral illness.

Answer 19

aspirin

Question 20

Patients with Reyes syndrome will have signs of _____ dysfunction.

Answer 20

liver dysfunction (jaundice , lethargy)

Question 21

Patients with Reyes syndrome have high _____ levels.

Answer 21

high ammonia levels

Question 22

Patients with Reyes syndrome have an altered _____ status.

Answer 22

altered mental status

Question 23

_____ & ______ are the medications of choice for fever in viral illness due to risk of Reye Syndrome.

Answer 23

Acetaminophen & Ibuprophen

Question 24

With Reyes you want to monitor ___ status, ____ function, and ___ & ___.

Answer 24

monitor neuro status (ICP) , liver function, & I&O’s.

Question 25

_______ a rare inherited disorder where the body is unable to breakdown phenylalanine, an amino acid.

Answer 25

Phenylketonuria (PKU)

Question 26

A normal level of phenylalanine is ____ - ____.

Answer 26

0-2 mg/dL

Question 27

PKU: > ____ mg/dL

Answer 27

> 20 mg/dL

Question 28

High levels of PKU can cause damage to the ______ ______ system, if toxic levels are not treated.

Answer 28

central nervous system

Question 29

What should the infant eat before the heel stick?

Answer 29

breastmilk or formula

Question 30

When is the heel stick done?

Answer 30

around 24 to 48 hours old

Question 31

Patients with PKU should avoid eating foods high in? (2)

Answer 31

Protein & phenylalanine

Question 32

What foods are high in protein & phenylalanine?

Answer 32

Meats
Diary products
Aspartame