Module 18 Exam 3 Flashcards
what is sickle cell disease
- hereditary form of hemolytic anemia, resulting from a defective hemoglobin molecule
- autosomal recessive trait disorder with no symptoms
what is the most common genetic disorder of the blood
sickle cell anemia
who does sickle cell disease primarily occur in a
african american populations, white populations of mediterranean origin
what is the blood test for sickle cell disease
hemoglobin electrophoresis
when do signs and symptoms start appearing for sickle cell disease
within first 6 months with anemia and vasculopathy
what may be impaired in sickle cell disease during the early years
growth and development, makes the susceptible to communicable diseases
what causes thrombosis and infarction from sickle cell anemia
cause there is an increase in fluid viscosity
what is severe hemolytic anemia
a hemolytic sickle cell disease where the hematocrit ranges from 18-30%
what is the normal lifespan RBC
-90 to 120 days
what is the lifespan of RBC in hemolytic anemias
10 to 15 days
what is a sickle cell crisis
recurrences of clinical exacerbations of the disease with periods of remission characterize childhood and adolescence
what are the precipitating factors of a sickle cell crisis
- can occur at any time without stimuli
- hypoxia, dehydration, sudden change in temp
- physical activity, extreme fatigue
- stress/anxiety, additional physical burden
what are clinical symptoms of a sickle cell crisis
- reversible, pain episodes involving extremities head, back , chest
- swelling, fever, dehydration,
- infarctions
- symptoms of seizure, stroke, coma
can a sickle cell crisis be fatal
yes
what is the major organ that is effected in a sickle cell crisis
kidney
what are some preventative measures that can be taken for sickle cell anemia
- stem cell transplant
- daily penicillin until 6
- promptly treat infections
- genetic counseling
- folate supplements
what are tx for sickle cell anemia
- supportive and palliative tx
- pain releif
- antibiotics for infectious disease
- o2 therapy and blood transfusions
what are the radiographic findings of sickle cell
- decreased radiodensity
- coarse trabecular pattern, step ladder
- sig. bone loss in children
- thinning of the border of the mandible
what are oral manifestations of sickle cell disease
- pallor of tissues
- jaundice
- perio involvment can lead to sickle cell crisis
- pockets infection and bleeding
- delayed eruption, malocclusion, dentin hypomineralization
- facial and dental pain
what is the objective of appointment mangagement of sickle cell disease
do precipitate a sickle cell crisis
Does sickle cell disease require antibiotic premed
some do
what kind of local anesthetic for sickle cell disease
low doses of vasoconstrictors
can you use n20 in sickle cells
yes oxygen must be above 50 %
what is a polycythemia
increase in the number and concentration of blood cells above the normal level, hemoglobin and hematocrit levels are more than 55% in males and 50% infemales
what is relative polycythemia
-loss of plasma with a corresponding los of red blood cells, concentration of cells increases and relative polycythemia results
what can be causes of fluid loss in relative polycythemia
dehydration, diarrhea, repeated vomiting, sweating or loss of fluid from burns
is the risk of CVA and infarction increased in relative polycythemia
yes
what is polycythemia vera (primary polycythemia)
actual increase in RBC count and Hg value, white cell and platelet counts elevated, blood viscosity increases
what is polycythemia vera caused by
its a neoplastic condition resulting from a bone disorder in which primitite red cells or stem cells proliferate cause is unknown
what are the oral signs and symptoms of polycythemia vera
- tongue, mucous membranes are deep redish purple
- gingiva are enlarged, bleed easily
- submucosal petichiae, ecchymosis and hematoma formation