Hematologic Disorders Flashcards

Question

prognosis of SCA

Answer 1

no cure (except possibly bone marrow transplant) frequent bacterial infections bacterial infection can lead to death in young children stokes are at an increased risk

Answer 2

erythropoietin or hydroxyuria | PCN prophylaxis

Answer 3

``` maintain hydration maintain oxygenation (avoid altitudes, hot weather, manage stress) pain management ( kids require more pain meds than normal child) ```

Answer 4

``` inherited blood disorder both parents must carry disorder HgB synthesis reduced or entirely absent chronic hypoxia detected in infancy or toddlerhood (pallor, FTT, severe anemia, hepatosplenomegaly) ```

Answer 5

severe anemia bronzed skin bone pain, skeletal deformities, fractures iron overload r/t transfusions, treat with chelating drugs

Answer 6

diagnosed with Hgb electrophoresis | blood transfusion to maintain normal levels (side effect hemosiderosis)

Answer 7

iron-chelating drugs- binds to excess iron for excretion by kidneys IV or subq over 8-10 hours multiple times/week may be given at home with IV pump per parents can be given orally also give oral vitamin c to facilitate iron binding

Answer 8

administer RBC monitor chelation therapy (n/v/d, decreased appetite, rash, increased liver enzymes, neutropenia, adequate hydration is VITAL

Answer 9

absence of both humeral and cell mediated immunity | unknown cause

Answer 10

``` early, frequent infections FTT chronic diarrhea persistent thrush adventitious lung sound r/t pneumonia low levels of immunoglobulins stem cell replacements BUBBLE KIDS- live in a sterile environment ```

Answer 11

``` IV immunoglobulin sterile environment bone marrow transplant prevent exposure to infections support and educate family ```

Answer 12

transmission from + mom prenatal, perinatal, breast milk blood transfusions unprotected sex

Answer 13

drugs and unprotected sex

Answer 14

antiviral therapy for first month of life

Answer 15

antivirals therapy for infants born to positive moms for first month of life aggressive antibiotic therapy for infections modified immunization schedule prophylaxis against opportunistic infections

Answer 16

acquired hemolytic disorder destruction of platelets in the spleen Bleed very easily

Answer 17

``` supportive anti-D antibody- plasma derived immunoglobulin that causes transient anemia by clearing the antibody coated RBC IVIG steroids for 1-2 weeks splenectomy and IVIG if chronic ```

Answer 18

educate parents assess for bleeding prevent trauma avoid unnecessary procedures, needle sticks, suppositories, rectal temps apply pressure for 5 minutes after venipuncture follow up care in home or clinic

Answer 19

deficiency of specific clotting factors | hereditary

Answer 20

classic hemophillia deficient of factor VIII males most affected degree of bleeding depends on amount of clotting factor and severity of a given injury

Answer 21

``` bleeding tendency symptoms may not occur until 6 months hemarthrosis- bleeding into the joint space (knee, ankle, elbow, leading to impaired mobility) ecchymosis (bruising) epistaxis bleeding after procedures ```

Answer 22

stiffness, tingling, or ache followed by decreased mobility, warmth, redness, welling and severe pain

Answer 23

``` prevent bleeding ( no contact sports, riding scooters, PAD child during activities) replacement of deficient factor ( VIII or DDAVP) regular exercise and PT ```

Answer 24

prevent bleeding safe environment, use of protective equipment avoid ASA sub q instead of IM venipuncture vs heel stick, no suppositories electric razors avoid contact sports soft toothbrush recognize and control bleeding (pressure x15min, RICE) be suspicious of headache, slurred speech, altered LOC, black tarry stools

Answer 25

unknown etiology occurs 6m - 16yrs generalized vasculitis of dermal capillaries extravasation of RBC's, produces petechial skin lesions inflammation and hemorrhage in GI tract may lead to ongoing nephrotic syndrome

Answer 26

``` most cases resolve without treatment corticosteroids and anticoags from severe or persistent cases symptomatic treatment maintain hydration monitor renal function ```