CHAPTER 30

Question 1

sickle cell disease

• disorders are characterized by the presence of an _____ (_____)
• -mutation causes _____ to be replaced by _____
• is_____
• deoxygenation and dehydration: RBCs solidify and stretch into an elongated sickle cell shape
• sickle cell trait: child inherits Hb S from one parent and normal hemoglobin (Hb A) from the other; the heterozygous carrier rarely has symptoms

Answer 1

abnormal hemoglobin 
Hb S
valine
glutamic acid 
autosomal recessive 
deoxygenation 
dehydration
sickle cell trait

Question 2

sickle cell disease is classified

-disorder is initiated by _____ and _____

Answer 2

hypoxemia

acidosis

Question 3

inherited disorders of erythrocytes
sickle cell disease
-extent, severity, and clinical manifestations depend on the percentage of hemoglobin that is Hb S
-_____ is _____, _____ phenomenon
–decreased _____ (PO2) of the _____ (e.g. hypoxemia)
–increased _____ concentration in the _____ (decreased pH)
–increased _____, decreased _____, and _____ temperature

-polymerization: sickled erythrocyte stiffens, changing from a flexible, beneficial cell to an inflexible one that starves and damages tissues.

Answer 3

sickling 
occasional 
intermittent 
oxygen tension
blood
hydrogen ion
blood
plasma osmolality 
plasma volume
low

Question 4

inherited disorders of erythrocytes
sickle cell disease

_____ crisis (_____ crisis)

• sickling is in _____, extremely _____, and _____. _____ and _____ exhibit painful swelling (______)
• _____ crisis (_____ crisis) begins with sickling in the _____. As blood flow is obstructed by _____ of _____, _____ cells, _____ occurs and a _____ effect brings all blood flow through the vessel to a halt.

Answer 4

vasoocclusive 
thrombotic
microcirculation
painful
symmetric 
hands 
feet
hand-foot syndrome 
vasoocclusive 
thrombotic 
microcirculation 
tangled masses
rigid 
vasospasm 
log jam

Question 5

inherited disorders of erythrocytes

• _____ crisis
• _____ is compromised, thus limiting the number of _____ that are replaced. (aplastic crisis, a transient cessation in red blood cell production that results in acute anemia, occurs as a result of a viral infection. the virus causes a temporary shutdown of red blood cell production in the bone marrow, or reticulocytosis)
• _____ crisis
• -large amounts of blood pool in the _____ and _____

Answer 5

aplastic 
compensatory erythropoiesis 
erythrocytes 
sequestration 
liver 
spleen

Question 6

inherited disorders of erythrocytes
sickle cell disease
-_____ crisis
–crisis occurs in association with certain _____ or _____. rate of _____ is _____

• _____ syndrome
• sickled red blood cells attach to the _____ of the injured, _____, and _____ and fail to be _____

Answer 6

hyperhemolytic
drugs 
infection 
red blood cell destruction
accelerated 
acute chest 
endothelium 
underventilated
inflamed 
lung 
reoxygenated

Question 7

In sickle cell anemia- _____ and _____ are not an option

• children diagnosed with sickle cell anemia are not candidates for either bone marrow or stem cell transplants
• bone marrow or stem cell transplants can cure sickle cell anemia. however, the only reason these procedures are not currently an option for most children is because _____ are often difficult to find.

Answer 7

bone marrow
stem cell transplants
well-matched stem cell donors

Question 8

inherited hemorrhagic disorders
hemophilias
-serious bleeding disorders
-_____ (classic hemophilia): factor _____ deficiency, _____-linked _____ (hemophilia A (classic hemophilia) is caused by factor _____ deficiency and is inherited as an _____ disorder that affects _____

-_____ (_____ disease): factor _____ deficiency

Answer 8

Hemophilia A
VIII
X
recessive 
VIII
X-linked 
men
hemophilia B
Christmas 
IX