Peds Hematologic/Immunologic Dysfunction Flashcards

1
Q

skin examination for children with hematologic dysfunction

A

pallor, petechiae, bruising

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2
Q

anemia

A

-Decrease in number of RBCs or hemoglobin (Hgb) which decreases the oxygen-carrying capacity of blood

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3
Q

Anemia with reduced Hgb concentration may be caused by

A

a dietary depletion of iron

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4
Q

s/s of anemia

A

lack of energy, easy fatigability, and pallor

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5
Q

Aplastic anemia

A

is a condition that occurs when your body stops producing enough new blood cells.

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6
Q

treatment for aplastic anemia

A

medications, blood transfusions or a stem cell transplant, also known as a bone marrow transplant.

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7
Q

red cell aplasia (PRCA) or erythroblastopenia

A

a type of anemia affecting the precursors to red blood cells but not to white blood cells. The bone marrow CEASES to produce red blood cells.

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8
Q

Immune thrombocytopenic purpura (ITP)

A

a bleeding disorder in which the immune system destroys platelets, which are necessary for normal blood clotting. People with the disease have too few platelets in the blood.

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9
Q

Disseminated intravascular coagulation (DIC)

A

a rare, life-threatening condition. DIC causes your blood to clot excessively. As a result, blood clots may reduce blood flow and block blood from reaching bodily organs.

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10
Q

treatment for anemia

A

-Transfusion after hemorrhage if needed
-Nutritional intervention for deficiency anemias

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11
Q

supportive care for anemia

A

-Intravenous (IV) fluids to replace intravascular volume
-Oxygen therapy
-Bed rest

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12
Q

iron deficiency anemia more common in

A

toddlers between 12 to 36 months and during the growth spurt in adolescence

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13
Q

Abnormal Hgb levels are common in what racial or ethnic groups

A

Southeast Asians, Africans , and Mediterranean

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14
Q

Hemoccult test

A

a lab test for hidden blood in the stools; tests for chronic intestinal bleeding

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15
Q

Decrease tissue oxygen needs:
Signs of exertions

A

tachycardia, palpitations, tachypnea, dyspnea, shortness of breath, hyperpnea (increased depth and rate of breathing), dizziness, lightheadedness, diaphoresis, and change in skin color. The child looks fatigued

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16
Q

Explaining Blood Components to Children: RBCs

A

Carry the oxygen you breathe from your lungs to all parts of your body.

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17
Q

Explaining Blood Components to Children: WBCs

A

Help keep germs from causing infection.

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18
Q

Explaining Blood Components to Children: platelets

A

Small parts of cells that help make bleeding stop by forming a clot (or scab) over the hurt area.

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19
Q

Explaining Blood Components to Children: Plasma

A

The liquid portion of blood, which has clotting factors that help make bleeding stop.

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20
Q

What is iron deficiency anemia caused by?

A

inadequate supply of dietary iron

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21
Q

Milk babies

A

overweight infant because of excessive milk ingestion
2 reasons become anemic: milk (poor source of iron)
increased fecal loss

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22
Q

liquid preparation of iron may

A

temporarily stain teeth; brush teeth after admin to lessen staining

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23
Q

iron-rich foods

A

iron-fortified cereals
lean meat, poultry, and fish
tofu
egg yolks
beans
raisins

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24
Q

what helps iron to get absorbed

A

Serve fruits and vegetables high in vitamin C or a glass of orange juice at mealtimes

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25
Q

Don’t give cow’s milk to babies under

A

1 year old

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26
Q

Sickle Cell Anemia

A

-Partial or complete replacement of normal Hgb with abnormal HbS
-Defect is inherited

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27
Q

sickle cell anemia onset

A

Newborns with SCA are generally asymptomatic because of the protective effect from the mother, Rapidly decreases during the first year, then child manifests symptoms

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28
Q

In areas of world where malaria is common, individuals with sickle cell trait tend to have

A

survival advantage over those without trait

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29
Q

Clinical features of sickle cell anemia

A

-Obstruction caused by sickled RBCs
-The vascular inflammation
-Increased RBC destruction
-Abnormal adhesion, entanglement, and meshing of rigid sickle-shaped cells
-Local hypoxia
-Cellular death

30
Q

Dactylitis

A

inflammation of a digit (either finger or toe) . The affected fingers and toes swell up into a sausage shape and can become painful.
-found in patients with sickle cell anemia

31
Q

Priapism

A

prolonged erection of the penis, persistent erection continues hours beyond or isn’t caused by sexual stimulation, usually painful
-found in patients with sickle cell anemia

32
Q

management for sickle cell anemia

A

-Prevent sickling
-Rest and minimize energy expenditure
-HYDRATION and OXYGENATION
-Electrolyte replacement
-Analgesia (no aspirin)
-Blood replacement
-Antibiotics
-PAIN MANAGEMENT

33
Q

what is the leading cause of death in young children with sickle cell

A

bacterial infection because these patients are immunocompromised

34
Q

what is the usual life span of a patient with sickle cell

A

into the fifth decade

35
Q

why is penicillin given prophylactically for patients with sickle cell

A

it significantly reduces the risk for pneumococcal infection in children with SCD; Started at 2 months of age

36
Q

what are things that can cause a sickle cell crisis

A

-Anything that increases body’s need for oxygen or alters transport of oxygen
-Trauma
-Infection, fever
-Physical and emotional stress
-Increased blood viscosity caused by dehydration
-Hypoxia

37
Q

Vaso-Occlusive Crisis (VOC)

A

“painful episode”; Characterized by ischemia causing mild to severe pain, Lasts from minutes to days or longer

38
Q

Vaso-Occlusive Crisis (VOC) treatment

A

-hydration
-oxygenation
-pain management

39
Q

Sequestration Crisis

A

-Pooling of a large amount of blood
-Usually in the spleen and infrequently in the liver

40
Q

what does Sequestration Crisis cause

A

decreased blood volume and ultimately shock

41
Q

Therapeutic Management of Sickle Cell Crisis

A

-Rest to minimize energy loss
-Hydration through oral or IV therapy
-Electrolyte replacement
-Analgesia for pain (children tend to be undermedicated)
-Blood replacement for anemia
-Antibiotics for infection
-Monitoring of reticulocyte count regularly to evaluate bone marrow function
-Blood transfusion: If given early in crisis, may reduce ischemia

42
Q

analgesia for pain used for children in a sickle cell crisis

A

-mild to moderate pain: NSAID (ibuprofen) acetaminophen
-severe pain - opioids (morphine, oxycodone admin either IV or po)
-given around the clock; PCA

43
Q

hemophilia

A

An X-linked recessive hereditary bleeding disorders that result from deficiencies of specific clotting factors

44
Q

Diagnostic Evaluation of Hemophilia

A

-History of bleeding episodes (overt prolonged bleeding, hemarthrosis, ecchymosis)
-X-linked inheritance

45
Q

lab findings for hemophilia

A

-Low levels of factor VIII or IX
-prolonged partial thromboplastin time (PTT)
-Normal: Platelet count, parathormone level, and fibrinogen level

46
Q

Hemarthrosis

A

bleeding into the joint cavities

47
Q

Ecchymosis

A

bruising

48
Q

Therapeutic Management of Hemophilia

A

-Replace missing clotting factors (Factor VIII)
-Desmopressin (DDAVP)

49
Q

Desmopressin (DDAVP) for hemophilia

A

a synthetic vasopressin that causes two to four times increase in factor VIII activity

50
Q

Prognosis of Hemophilia

A

Mild to moderate: patients live near-normal lives, no cure, control the symptoms and limit joint damage

51
Q

treatment options for hemophilia

A

uGene therapy
uA working copy of the factor VIII gene is introduced

52
Q

care management for hemophilia: prevent bleeding

A

-provide safe environment
-dental hygiene (soften toothbrush in warm water, sponge tipped toothbrush)

53
Q

appropriate sports for children with hemophilia

A

swimming, walking, jogging, tennis, golf, fishing, bowling

54
Q

Recognize and control bleeding for hemophilia by

A

using RICE

55
Q

Epistaxis

A

nosebleed

56
Q

Recurrent or severe episodes of nose bleeds may indicate what underlying disease

A

Vascular abnormalities, leukemia, thrombocytopenia, clotting factor deficiency diseases (von Willebrand disease and hemophilia)

57
Q

Care Management of Epistaxis

A

-Remain calm; keep child calm
-Bleeding usually stops within 10 minutes after nasal pressure
-Have child sit up and lean forward
-Apply pressure to the soft lower part of the nose

58
Q

HIV/AIDS

A

Virus that destroys the immune system that should protect the body from diseases.

59
Q

HIV/AIDS transmission

A

-sexual acts, blood transfusions, used hypodermic needles, or from mother to child during birth, breast milk

60
Q

what is given to HIV infected mothers to prevent transmission of HIV to the infant

A

HAART - Highly Active Anti Retroviral Therapy

61
Q

HIV takes over

A

CD4+T lymphocytes; CD4+T cell count drops, increasing risk of infections

62
Q

manifestations of HIV/AIDS

A

-Malnutrition, short stature, cardiomyopathy
-Enlarged lymph nodes and spleen, oral candidiasis

63
Q

diagnosis for infants with HIV

A

-Infants born to HIV + mothers will test positive
-Early testing using recombinase polymerase amplification (RPA)

64
Q

Care management for HIV/AIDS

A

-Education (transmission, control, standard precautions)
-Prevention counseling for adolescents
-Pain management
-Look at psychosocial aspects
-Confidentiality

65
Q

SCID (Severe Combined Immunodeficiency Disease)

A

Absence of both humoral and cell-mediated immunity.

66
Q

most common manifestation of SCID (Severe Combined Immunodeficiency Disease)

A

susceptibility to infection early in life, most often in the first months

67
Q

SCID (Severe Combined Immunodeficiency Disease) is characterized by

A

-chronic infections, failure to completely recover from infections, frequent reinfections, and infection with unusual agents.
-Failure to thrive is a consequence of the persistent illness.

68
Q

treatment for SCID (Severe Combined Immunodeficiency Disease)

A

if diagnosed with the first 3 months of life - bone marrow transplant
IVIG provides passive immunity until transplant performed

69
Q

Blood transfusion

A

-PRBCs, platelets
-Be alert to transfusion reactions
-Standard guidelines for all transfusion monitoring
-Use infusion pump

70
Q

Apheresis

A

-Removal of blood, separation of components, reinfuse portion
-Used to remove and save platelets from healthy donors