Module 18 Exam 3 Flashcards
1
Q
what is sickle cell disease
A
- hereditary form of hemolytic anemia, resulting from a defective hemoglobin molecule
- autosomal recessive trait disorder with no symptoms
2
Q
what is the most common genetic disorder of the blood
A
sickle cell anemia
3
Q
who does sickle cell disease primarily occur in a
A
african american populations, white populations of mediterranean origin
4
Q
what is the blood test for sickle cell disease
A
hemoglobin electrophoresis
5
Q
when do signs and symptoms start appearing for sickle cell disease
A
within first 6 months with anemia and vasculopathy
6
Q
what may be impaired in sickle cell disease during the early years
A
growth and development, makes the susceptible to communicable diseases
7
Q
what causes thrombosis and infarction from sickle cell anemia
A
cause there is an increase in fluid viscosity
8
Q
what is severe hemolytic anemia
A
a hemolytic sickle cell disease where the hematocrit ranges from 18-30%
9
Q
what is the normal lifespan RBC
A
-90 to 120 days
10
Q
what is the lifespan of RBC in hemolytic anemias
A
10 to 15 days
11
Q
what is a sickle cell crisis
A
recurrences of clinical exacerbations of the disease with periods of remission characterize childhood and adolescence
12
Q
what are the precipitating factors of a sickle cell crisis
A
- can occur at any time without stimuli
- hypoxia, dehydration, sudden change in temp
- physical activity, extreme fatigue
- stress/anxiety, additional physical burden
13
Q
what are clinical symptoms of a sickle cell crisis
A
- reversible, pain episodes involving extremities head, back , chest
- swelling, fever, dehydration,
- infarctions
- symptoms of seizure, stroke, coma
14
Q
can a sickle cell crisis be fatal
A
yes
15
Q
what is the major organ that is effected in a sickle cell crisis
A
kidney
16
Q
what are some preventative measures that can be taken for sickle cell anemia
A
- stem cell transplant
- daily penicillin until 6
- promptly treat infections
- genetic counseling
- folate supplements
17
Q
what are tx for sickle cell anemia
A
- supportive and palliative tx
- pain releif
- antibiotics for infectious disease
- o2 therapy and blood transfusions
18
Q
what are the radiographic findings of sickle cell
A
- decreased radiodensity
- coarse trabecular pattern, step ladder
- sig. bone loss in children
- thinning of the border of the mandible
19
Q
what are oral manifestations of sickle cell disease
A
- 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
20
Q
what is the objective of appointment mangagement of sickle cell disease
A
do precipitate a sickle cell crisis
21
Q
Does sickle cell disease require antibiotic premed
A
some do
22
Q
what kind of local anesthetic for sickle cell disease
A
low doses of vasoconstrictors
23
Q
can you use n20 in sickle cells
A
yes oxygen must be above 50 %
24
Q
what is a polycythemia
A
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
25
what is relative polycythemia
-loss of plasma with a corresponding los of red blood cells, concentration of cells increases and relative polycythemia results
26
what can be causes of fluid loss in relative polycythemia
dehydration, diarrhea, repeated vomiting, sweating or loss of fluid from burns
27
is the risk of CVA and infarction increased in relative polycythemia
yes
28
what is polycythemia vera (primary polycythemia)
actual increase in RBC count and Hg value, white cell and platelet counts elevated, blood viscosity increases
29
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
30
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
31
what is the treatment for polycythemia vera
chemo
phlebotomy
avoid iron supplements and aspirin meds
32
what are the dental hygiene considerations for poly cythemia vera
- frequent maintenance appts
- review medical history
- eval pt blood test results
33
what is secondary polycythemia
erythrocytosis, and increase in the numbers of red blood cells
34
what can the increase in RBC production in secondary polycythemia result from
-hypoxia, neoplasms
35
what can bleeding tendencies in secondary polycythemia be partially controlled by
-control of gingival irritiants
36
why may disorders of the WBC occur
because of a decrease or an increase in cell numbers
37
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
38
what are specific infections in which leukopenia occurs
-HIV/AIDS, typhoid fever, infulenza, malaria, measles, German measles
39
what are diseases or intoxifications of the bone marrow in which leukopenia occurs
-chronic drug poisioning, ionizing radiation, autoimmune or drug induced immune reactions
40
what is agranulocytosis (malignant neutropenia)
-rare, serious disease involving the destruction of the bone marrow, a reduction in circulating neutrophils
41
what is the cause of the primary form of agranulocytosis? secondary
unknown
| by drugs and chemicals
42
what is the oral characteristic of agranulocytosis
presence of infection
43
what is leukocytosis
increase in the number of circulating WBC, caused by inflammatory and infectious states, trauma, exhertion, and other condititons
44
what is the most extreme abnormal cause of leukocytosis
leukemia
45
what is a leukemia
malignant neoplasm of immature WBC that muliply uncontrollably and become cancerous
46
what is leukemia characterized by
abnormally large numbers of immature WBC, located within circulating blood and bone marrow
47
what is lymphocytopenia
-abnormally low number of lymphocytes in the blood
48
what is the treatment of lymphocytopenia
-gamma globulin, antivirals if pt has AIDS
49
what does a hereditary deficency in lymphocytopenia require
bone marrow transplantation
50
what are the general types of bleeding disorders caused by
- pathology of the blood vessel wall
- platelet deficiency or dysfunction
- disorders of coagulation
51
what is the pathology of the vessel wall in bleeding disorders
-vascular fragility increased, petechial and purpuric hemorrhages
52
what are the 2 platelet deficiencys or dysfunctions in bleeding disorders
- throbocytopenia (lowered number of platelets)
| - Platelet dysfunction (interferece with clotting mechanism)
53
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)
```
54
what is Hemophilia A (classic)
cause by reduced amount of activity of factor VIII, 85% of people with hemophilia have this form
55
what is hemophilia B (christmas disease)
deficiency of a blood plasma protien called factor IX that effects the clotting properties of blood
56
both hemophilia A and B are _____ linked rescessive genetic diseases
x-linked, occurs primarily in males
57
what can the severity of hemophilia be related directly to
the level of the clotting factor in the circulating blood
58
what von willebrands disease
prolonged bleeding time in the presence of a normal platelet count, most common hereditary disorder of platelet function.
59
who does von willebrands occur in
males and females
60
what are extra oral signs of vonwillebrands
petechiae of skin
61
what are the effect of minor trauma in hemophilias
bleeding and bruising depends on the extent of the disease
62
what is hemarthroses in long term complications of hemophilia
bleeding into soft tissues of the joints, swelling, pain, incapacitation
63
what is the joing deformity in complicaitons of hemophilia
-permanent joint damage, can result and the patient may need splints, braces or orthopedic surgery
64
what is the intramuscular hemorrage in hemophilia
hemorrhage into muscles is accompanied by pain and limitation of motion
65
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
66
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
67
what needs to be included in the medical history of a bleeding disorder
-type, severity, tx, meds, and family history
68
does someone with a bleeding disorder require premed,
consult physician
69
do many dental procedures require factor replacement therapy before dental appointments
yes
70
what test are needed on the same day as dental tx
PT-within 24 hrs or INR
71
what is the most common side effect of warfin and coumadin
hemorrhage
72
what does a PT measure
status of extrinsic and common pathways of coagulation
73
what does an INR measure
standard method of measuring PT independent of thromboplastin reagent, more accurate thatn PT
74
what is an aPTT
mesures the status of the intrisic and common pathways of coagulation
75
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
