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
abnormal hemoglobin Hb S valine glutamic acid autosomal recessive deoxygenation dehydration sickle cell trait
sickle cell disease is classified
-disorder is initiated by _____ and _____
hypoxemia
acidosis
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.
sickling occasional intermittent oxygen tension blood hydrogen ion blood plasma osmolality plasma volume low
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.
vasoocclusive thrombotic microcirculation painful symmetric hands feet hand-foot syndrome vasoocclusive thrombotic microcirculation tangled masses rigid vasospasm log jam
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 _____
aplastic compensatory erythropoiesis erythrocytes sequestration liver spleen
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 _____
hyperhemolytic drugs infection red blood cell destruction accelerated acute chest endothelium underventilated inflamed lung reoxygenated
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.
bone marrow
stem cell transplants
well-matched stem cell donors
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
Hemophilia A VIII X recessive VIII X-linked men hemophilia B Christmas IX
