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
what is the treatment for polycythemia vera
chemo
phlebotomy
avoid iron supplements and aspirin meds
what are the dental hygiene considerations for poly cythemia vera
- frequent maintenance appts
- review medical history
- eval pt blood test results
what is secondary polycythemia
erythrocytosis, and increase in the numbers of red blood cells
what can the increase in RBC production in secondary polycythemia result from
-hypoxia, neoplasms
what can bleeding tendencies in secondary polycythemia be partially controlled by
-control of gingival irritiants
why may disorders of the WBC occur
because of a decrease or an increase in cell numbers
what is leukopenia
-decrase in the total number of WBC results when production cant keep up with the turnover rate, or when accellerated rate of removal of cells occurs
what are specific infections in which leukopenia occurs
-HIV/AIDS, typhoid fever, infulenza, malaria, measles, German measles
what are diseases or intoxifications of the bone marrow in which leukopenia occurs
-chronic drug poisioning, ionizing radiation, autoimmune or drug induced immune reactions
what is agranulocytosis (malignant neutropenia)
-rare, serious disease involving the destruction of the bone marrow, a reduction in circulating neutrophils
what is the cause of the primary form of agranulocytosis? secondary
unknown
by drugs and chemicals
what is the oral characteristic of agranulocytosis
presence of infection
what is leukocytosis
increase in the number of circulating WBC, caused by inflammatory and infectious states, trauma, exhertion, and other condititons
what is the most extreme abnormal cause of leukocytosis
leukemia
what is a leukemia
malignant neoplasm of immature WBC that muliply uncontrollably and become cancerous
what is leukemia characterized by
abnormally large numbers of immature WBC, located within circulating blood and bone marrow
what is lymphocytopenia
-abnormally low number of lymphocytes in the blood
what is the treatment of lymphocytopenia
-gamma globulin, antivirals if pt has AIDS
what does a hereditary deficency in lymphocytopenia require
bone marrow transplantation
what are the general types of bleeding disorders caused by
- pathology of the blood vessel wall
- platelet deficiency or dysfunction
- disorders of coagulation
what is the pathology of the vessel wall in bleeding disorders
-vascular fragility increased, petechial and purpuric hemorrhages
what are the 2 platelet deficiencys or dysfunctions in bleeding disorders
- throbocytopenia (lowered number of platelets)
- Platelet dysfunction (interferece with clotting mechanism)
what are the two types of coagulation disorgders in bleeding disorders
aquired disorders- (vitamin K deficiency, essentail for factors VII, IX and X, liver disease, anticoagulation drugs) Hereditary disorders (deficiency or abnormality of a plasma protien, Hemophilia A and B, von willebrands factor)
what is Hemophilia A (classic)
cause by reduced amount of activity of factor VIII, 85% of people with hemophilia have this form
what is hemophilia B (christmas disease)
deficiency of a blood plasma protien called factor IX that effects the clotting properties of blood
both hemophilia A and B are _____ linked rescessive genetic diseases
x-linked, occurs primarily in males
what can the severity of hemophilia be related directly to
the level of the clotting factor in the circulating blood
what von willebrands disease
prolonged bleeding time in the presence of a normal platelet count, most common hereditary disorder of platelet function.
who does von willebrands occur in
males and females
what are extra oral signs of vonwillebrands
petechiae of skin
what are the effect of minor trauma in hemophilias
bleeding and bruising depends on the extent of the disease
what is hemarthroses in long term complications of hemophilia
bleeding into soft tissues of the joints, swelling, pain, incapacitation
what is the joing deformity in complicaitons of hemophilia
-permanent joint damage, can result and the patient may need splints, braces or orthopedic surgery
what is the intramuscular hemorrage in hemophilia
hemorrhage into muscles is accompanied by pain and limitation of motion
what is oral bleeding in the complications of hemophilia
- gingival bleeding common, even more when perio infeciton
- b/c of fear of bleeding patients may neglect brushing and flossing
how do you manage uncontrolled bleeding i
- stop tx
- if clotting doesnt occur with in a couple mins, apply gauze
- medical attention if bleeding doesnt stop
what needs to be included in the medical history of a bleeding disorder
-type, severity, tx, meds, and family history
does someone with a bleeding disorder require premed,
consult physician
do many dental procedures require factor replacement therapy before dental appointments
yes
what test are needed on the same day as dental tx
PT-within 24 hrs or INR
what is the most common side effect of warfin and coumadin
hemorrhage
what does a PT measure
status of extrinsic and common pathways of coagulation
what does an INR measure
standard method of measuring PT independent of thromboplastin reagent, more accurate thatn PT
what is an aPTT
mesures the status of the intrisic and common pathways of coagulation
What should we teach with OH in a blood disorder pt
- careful flossing, age appropriate preventative measures, nutritional counseling
- encourage to improve and maintain good oral heath
- meticulous biofilm removal, soft toothbrush
