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
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2
Q

what is the most common genetic disorder of the blood

A

sickle cell anemia

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3
Q

who does sickle cell disease primarily occur in a

A

african american populations, white populations of mediterranean origin

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4
Q

what is the blood test for sickle cell disease

A

hemoglobin electrophoresis

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5
Q

when do signs and symptoms start appearing for sickle cell disease

A

within first 6 months with anemia and vasculopathy

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6
Q

what may be impaired in sickle cell disease during the early years

A

growth and development, makes the susceptible to communicable diseases

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7
Q

what causes thrombosis and infarction from sickle cell anemia

A

cause there is an increase in fluid viscosity

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8
Q

what is severe hemolytic anemia

A

a hemolytic sickle cell disease where the hematocrit ranges from 18-30%

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9
Q

what is the normal lifespan RBC

A

-90 to 120 days

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10
Q

what is the lifespan of RBC in hemolytic anemias

A

10 to 15 days

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11
Q

what is a sickle cell crisis

A

recurrences of clinical exacerbations of the disease with periods of remission characterize childhood and adolescence

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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
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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
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14
Q

can a sickle cell crisis be fatal

A

yes

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15
Q

what is the major organ that is effected in a sickle cell crisis

A

kidney

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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
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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
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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
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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
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20
Q

what is the objective of appointment mangagement of sickle cell disease

A

do precipitate a sickle cell crisis

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21
Q

Does sickle cell disease require antibiotic premed

A

some do

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22
Q

what kind of local anesthetic for sickle cell disease

A

low doses of vasoconstrictors

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23
Q

can you use n20 in sickle cells

A

yes oxygen must be above 50 %

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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

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25
Q

what is relative polycythemia

A

-loss of plasma with a corresponding los of red blood cells, concentration of cells increases and relative polycythemia results

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26
Q

what can be causes of fluid loss in relative polycythemia

A

dehydration, diarrhea, repeated vomiting, sweating or loss of fluid from burns

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27
Q

is the risk of CVA and infarction increased in relative polycythemia

A

yes

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28
Q

what is polycythemia vera (primary polycythemia)

A

actual increase in RBC count and Hg value, white cell and platelet counts elevated, blood viscosity increases

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29
Q

what is polycythemia vera caused by

A

its a neoplastic condition resulting from a bone disorder in which primitite red cells or stem cells proliferate cause is unknown

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30
Q

what are the oral signs and symptoms of polycythemia vera

A
  • tongue, mucous membranes are deep redish purple
  • gingiva are enlarged, bleed easily
  • submucosal petichiae, ecchymosis and hematoma formation
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31
Q

what is the treatment for polycythemia vera

A

chemo
phlebotomy
avoid iron supplements and aspirin meds

32
Q

what are the dental hygiene considerations for poly cythemia vera

A
  • frequent maintenance appts
  • review medical history
  • eval pt blood test results
33
Q

what is secondary polycythemia

A

erythrocytosis, and increase in the numbers of red blood cells

34
Q

what can the increase in RBC production in secondary polycythemia result from

A

-hypoxia, neoplasms

35
Q

what can bleeding tendencies in secondary polycythemia be partially controlled by

A

-control of gingival irritiants

36
Q

why may disorders of the WBC occur

A

because of a decrease or an increase in cell numbers

37
Q

what is leukopenia

A

-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
Q

what are specific infections in which leukopenia occurs

A

-HIV/AIDS, typhoid fever, infulenza, malaria, measles, German measles

39
Q

what are diseases or intoxifications of the bone marrow in which leukopenia occurs

A

-chronic drug poisioning, ionizing radiation, autoimmune or drug induced immune reactions

40
Q

what is agranulocytosis (malignant neutropenia)

A

-rare, serious disease involving the destruction of the bone marrow, a reduction in circulating neutrophils

41
Q

what is the cause of the primary form of agranulocytosis? secondary

A

unknown

by drugs and chemicals

42
Q

what is the oral characteristic of agranulocytosis

A

presence of infection

43
Q

what is leukocytosis

A

increase in the number of circulating WBC, caused by inflammatory and infectious states, trauma, exhertion, and other condititons

44
Q

what is the most extreme abnormal cause of leukocytosis

A

leukemia

45
Q

what is a leukemia

A

malignant neoplasm of immature WBC that muliply uncontrollably and become cancerous

46
Q

what is leukemia characterized by

A

abnormally large numbers of immature WBC, located within circulating blood and bone marrow

47
Q

what is lymphocytopenia

A

-abnormally low number of lymphocytes in the blood

48
Q

what is the treatment of lymphocytopenia

A

-gamma globulin, antivirals if pt has AIDS

49
Q

what does a hereditary deficency in lymphocytopenia require

A

bone marrow transplantation

50
Q

what are the general types of bleeding disorders caused by

A
  • pathology of the blood vessel wall
  • platelet deficiency or dysfunction
  • disorders of coagulation
51
Q

what is the pathology of the vessel wall in bleeding disorders

A

-vascular fragility increased, petechial and purpuric hemorrhages

52
Q

what are the 2 platelet deficiencys or dysfunctions in bleeding disorders

A
  • throbocytopenia (lowered number of platelets)

- Platelet dysfunction (interferece with clotting mechanism)

53
Q

what are the two types of coagulation disorgders in bleeding disorders

A
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
Q

what is Hemophilia A (classic)

A

cause by reduced amount of activity of factor VIII, 85% of people with hemophilia have this form

55
Q

what is hemophilia B (christmas disease)

A

deficiency of a blood plasma protien called factor IX that effects the clotting properties of blood

56
Q

both hemophilia A and B are _____ linked rescessive genetic diseases

A

x-linked, occurs primarily in males

57
Q

what can the severity of hemophilia be related directly to

A

the level of the clotting factor in the circulating blood

58
Q

what von willebrands disease

A

prolonged bleeding time in the presence of a normal platelet count, most common hereditary disorder of platelet function.

59
Q

who does von willebrands occur in

A

males and females

60
Q

what are extra oral signs of vonwillebrands

A

petechiae of skin

61
Q

what are the effect of minor trauma in hemophilias

A

bleeding and bruising depends on the extent of the disease

62
Q

what is hemarthroses in long term complications of hemophilia

A

bleeding into soft tissues of the joints, swelling, pain, incapacitation

63
Q

what is the joing deformity in complicaitons of hemophilia

A

-permanent joint damage, can result and the patient may need splints, braces or orthopedic surgery

64
Q

what is the intramuscular hemorrage in hemophilia

A

hemorrhage into muscles is accompanied by pain and limitation of motion

65
Q

what is oral bleeding in the complications of hemophilia

A
  • gingival bleeding common, even more when perio infeciton

- b/c of fear of bleeding patients may neglect brushing and flossing

66
Q

how do you manage uncontrolled bleeding i

A
  • stop tx
  • if clotting doesnt occur with in a couple mins, apply gauze
  • medical attention if bleeding doesnt stop
67
Q

what needs to be included in the medical history of a bleeding disorder

A

-type, severity, tx, meds, and family history

68
Q

does someone with a bleeding disorder require premed,

A

consult physician

69
Q

do many dental procedures require factor replacement therapy before dental appointments

A

yes

70
Q

what test are needed on the same day as dental tx

A

PT-within 24 hrs or INR

71
Q

what is the most common side effect of warfin and coumadin

A

hemorrhage

72
Q

what does a PT measure

A

status of extrinsic and common pathways of coagulation

73
Q

what does an INR measure

A

standard method of measuring PT independent of thromboplastin reagent, more accurate thatn PT

74
Q

what is an aPTT

A

mesures the status of the intrisic and common pathways of coagulation

75
Q

What should we teach with OH in a blood disorder pt

A
  • careful flossing, age appropriate preventative measures, nutritional counseling
  • encourage to improve and maintain good oral heath
  • meticulous biofilm removal, soft toothbrush