Exam 1 Flashcards

1
Q

DiGeorge Syndrome (complete or partial)

A

T cell disorder caused by chromosome deletion

  • hypocalcemia with tetany after birth
  • cardiac defects
  • low set ears
  • Complete DiGeorge –> need thymus transplant
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2
Q

Severe Combined Immunodeficiency Disease (SCID)

A

T cell/B cell immunity is deficient or absent

  • infection, pneumonia, oral ulcers, FTT, dermatitis
  • “Bubble Boy Disease”
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3
Q

Systemic Lupus Erythematosus (SLE)

A
  • AI disease of connective tissues and BVs
  • genetic component
  • fever, wt loss, fatigue, anemia, leukopenia, thrombocytopenia
  • Sx mild to life-threatening: arthritis, nephritis, vasculitis
  • RENAL DISEASE = most common cause of morbidity/mortality
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4
Q

SLE - Criteria for Diagnosis (must have four)

A
  1. renal d/o
  2. neurologic disorders
  3. hematologic disorders
  4. immunologic disorders
  5. antinuclear antibodies
  6. butterfly rash
  7. discoid rash
  8. photosensitivity
  9. oral ulcers
  10. arthritis
  11. serositis
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5
Q

Juvenile Idiopathic Arthritis (JIA)

A

early onset = better prognosis
inflammation > scar tissue > limited ROM of joints
early closure of epiphyseal plates > altered growth
90% of children have NEGATIVE rheumatic factor

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6
Q

s/s of JIA

A

(exacerbation/remission)

  • stiffness, loss of motion, swelling, pain
  • fever, rash
  • lymphadenopathy, splenomegaly, hepatomegaly
  • loss of mobility in affected joints
  • warmth to touch, usually without erythema
  • Sx worsen with stressors
  • delayed growth
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7
Q

JIA Diagnostics

A
  • ESR & CRP determine amount of inflammation
  • antinuclear antibodies are common, but not specific to JIA
  • leukocytosis and anemia during exacerbations
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8
Q

Pharmacologic Management of JIA

A
  • NSAIDS
  • DMARDs (disease modifying antirheumatic drugs)
  • SAARDs (slow-acting antiarthritic drugs)
  • Corticosteroids
  • cytotoxic agents
  • immunologic modulators
  • biologic response modifiers
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9
Q

Other Management of JIA

A

DON’T massage - risk of emboli
DON’T immobilize - risk of loss of function
DON’T elevate - does not help pain

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10
Q

Hypersensitivity Response: anaphylaxis

A

within seconds to minutes of exposure

  • IgE
  • most common type of allergy = BEE STING/POLLEN
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11
Q

Hypersensitivity Response: cytotoxic reaction

A

within 15-30 min of exposure

  • cell surface antigens (blood)
  • antigen/antibody binding activates complement
  • includes transfusions, Rh incompatibility, ITP, and AI hemolytic anemia
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12
Q

Hypersensitivity Response: immune complex disease

A

generally peaks at 6 hours

  • excess antigen/antibody complexes in circulation deposit into tissues
  • results in local inflammation
  • includes SLE, rheumatic fever, glomerulonephritis, rheumatoid arthritis
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13
Q

Hypersensitivity Response: delayed hypersensitivity

A

takes 24-27 hours to fully develop

  • antigen processed by macrophages and presented to T cells
  • sensitized T cells release lymphokines
  • includes contact dermatitis, poison ivy, tuberculin
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14
Q

foods to avoid with latex allergy

A

bananas, kiwis, avocados

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15
Q

IVIG

A

used to treat immune thrombocytopenic purpura (ITP), Kawasaki disease, primary immunodeficiency d/o’s, hemolytic anemia, AIDS, etc.

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16
Q

Cancer: most common malignancies in PEDS

A
  • leukemia
  • meduloblastoma
  • astrocytomas
  • ependymoma
  • gliomas
  • neuroblastoma
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17
Q

neuroblastoma

A

most commonly occurring tumor outside the cranium (IN THE NERVE TISSUE)
commonly a smooth, hard, non-tender mass that can occur anywhere along the SNS chain (commonly abdominal, adrenal, thoracic, and cervical)

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18
Q

Wilm’s tumor (nephroblastoma)

A

!!DO NOT PALPATE!!
intrarenal tumor (common abdominal tumor)
-associated with congenital anomalies: aniridia (no iris), hemohypertrophy (abnormal growth of half the body or a structure), GI anomalies, nevi, hemartomas
-possible genetic link

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19
Q

“bubble boy disease”

A

severe combined immunodeficiency disease (SCID)

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20
Q

Which cardiac defect has a machine-like murmur?

A

PDA

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21
Q

In which defect are peripheral pulses diminished in the LOWER extremities?

A

coarctation of the aorta

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22
Q

Treatment for Kawasaki’s Disease

A

IVIG

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23
Q

Kawasaki’s can cause what in relation to the heart?

A

vasculitis (inflammation of BV’s) – can l/t ANEURYSMS

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24
Q

Indomethacin: watch for _______

A

decreased platelets, decreased urinary output, and necrotizing enterocolitis

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25
Q

Prostaglandin E1 is used for ?

A

to keep ductus arteriosus OPEN

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26
Q

Respiratory SE of Prostaglandin E1

A

apnea –> infants must be on ventilators

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27
Q

Digoxin

A

Tx for CHF

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28
Q

Legs-Calves-Perthes Disease [avg duration]

A

can take up to 3-4 years to resolve

29
Q

Ex of when COMPARTMENT SYNDROME can occur?

A

with fractures/injuries to bones

30
Q

Fractures: NURSING ALERT!!

A
  • Pain unrelieved by meds 1 hr after administration
  • Swelling
  • Discoloration (pallor, cyanosis) of exposed parts
  • Decreased pulses
  • Decreased temp of extremities
  • Inability to move distal parts
31
Q

bone healing: neonates

A

2-3 weeks

32
Q

bone healing: early childhood

A

4 weeks

33
Q

bone healing: later childhood

A

6-8 weeks

34
Q

bone healing: adolescence

A

8-12 weeks

35
Q

keystone Sx of DDH in infant

A

unequal gluteal folds when infant is prone

36
Q

talipes varus

A

bending inward

37
Q

talipes valgus

A

bending out

38
Q

talipes equinus

A

plantar flexion, toes lower than heel

39
Q

talipes calcaneus

A

dorsiflexion, toes above the heel

40
Q

osteogenesis imperfecta (OI)

A

group of heterogeneous inherited CONNECTIVE TISSUE disorders

41
Q

Manifestations of OI

A
  • excessive fragility
  • bone defects
  • reduced cortical thickness of bones
  • hyperextensibility of ligaments
42
Q

Classifications of OI: Type I

A

mild fragility, blue sclera, some cases abnormal dentition

43
Q

Classifications of OI: Type II

A
  • *LETHAL [stillborn or die in early infancy]
  • severe bone fragility w/ multiple fractures at birth
  • autosomal recessive inheritance
44
Q

Classifications of OI: Type III

A
  • severe bone fragility l/t severe progressive deformities
  • blue sclera
  • marked growth failure
  • most die in childhood d/t cardio-respiratory failure
45
Q

Classifications of OI: Type IV

A
  • mild to moderate bone fragility
  • normal sclera
  • some cases abnormal dentition
  • short stature
  • variable deformity
  • autosomal dominant
46
Q

Therapeutic Management of OI

A
  • primarily supportive care
  • bone marrow transplant for severe OI (experimental)
  • Drugs are of limited benefit
47
Q

NSG Care Management of OI

A
  • caution w/ handling to prevent fractures
  • family edu.
  • occupational planning and genetic counseling
  • maximize independence and minimize fracture risks
  • OI Foundation as resource
48
Q

Muscular Dystrophies

A
  • group of inherited diseases characterized by muscle fiber degeneration and muscle wasting
  • starts from LE and moves up the body
  • *Most common form: Duchenne (x-linked)
49
Q

Manifestation of MD

A

(vary w/ type)

  • generalized weakness and hypotonia
  • difficulty sucking/swallowing
  • some ocular problems
  • in childhood, parents may notice child tripping, toe walking, enlarged calf muscles
50
Q

MD progression up the body

A

can potentially cause scoliosis, musculoskeletal conditions, cardiomyopathy, difficulty ingesting foods, respiratory distress

51
Q

Management of MD

A

steroids can help preserve walking and pulmonary function for longer
-HIGH fiber, HIGH protein, LOW calorie diet

52
Q

Legg-Calve-Perthes Disease

A
  • self limiting
  • idiopathic
  • more common in males age 4-8
  • avascular necrosis of femoral head –> disturbed circulation to the femoral head with ischemic aseptic necrosis
53
Q

Definitive Dx of Legg-Calve-Perthes Disease

A

MRI

54
Q

Slipped Capital Femoral Epiphyses

A
  • spontaneous displacement of the proximal femoral epiphyses in a posterior and inferior direction
  • EMERGENCY ; requires early Dx and Tx (surgery)
  • seen most often in males and obese children
  • bedrest initially
  • Sx: hip/thigh/knee pain
55
Q

osteomyelitis

A
  • inflammation and infection of bony tissue
  • marked leukocytosis
  • Dx: bone scans
  • Increased ESR and CRP due to inflammation
  • ANY organism can cause osteomyelitis
56
Q

types of osteomyelitis

A

exogenous: infectious agent invades bone following penetrating wound, open fracture, etc.
hematogenous: PREEXISTING INFECTION spreads to bone

57
Q

Tx for osteomyelitis

A

prompt, vigorous, IV ABX for 3-4 weeks or up to several months [may need central line for LT ABX]

58
Q

Tx for PDA

A

Indomethacin - CLOSES ductus arteriosus

59
Q

Four Parts of TET of Fallot

A
  1. Pulmonic Stenosis: narrowing of pulmonary valve which prevents BF from RV to pulmonary artery
  2. Right ventricular hypertrophy: heart muscle thickens bc RV is pumping against high pressure
  3. Ventricular septal defect: hole in the ventricular septum
  4. Overriding of the aorta: aortic valve enlarged and looks like it arises from both RV and LV
60
Q

TET spells

A

rapid drop in O2 that l/t cyanosis; can occur after feeding, crying, having a BM, or kicking legs upon walking
**knee-to-chest position is best for the child

61
Q

Therapeutic Management of Congestive HF

A
  • take apical pulse for 1 min. and listen to heart sounds
  • I&O
  • provide rest periods
  • daily weights
  • frequent, small meals
  • high calorie snacks
  • change positions frequently
  • low sodium diet
  • Meds: digoxin, ACE-I, BB, Lasix
62
Q

Complications of Kawasaki’s Disease

A
  • Vasculitis ; can l/t aneurysms
  • inflamed coronaries, heart muscle, heart lining/valves, and outer membrane of heart
  • arrhythmias and abnormal function of the heart valves can develop
63
Q

Sickle Cell Anemia

A
  • genetic mutation (hereditary)
  • Autosomal recessive
  • sickle cells obstruct the BV’s so normal RBC’s cannot get through > ischemic pain > chronic pain (round the clock pain meds for pts)
  • avascular necrosis of bone marrow = extreme pain
64
Q

Management of SCA

A

HYDRATION AND OXYGENATION OF SICKLED CELLS

65
Q

Thalassemias Pathophysiology

A

anemia results from defective synthesis of Hgb, structurally impaired RBCs, and shortened life of RBCs
-CHRONIC hypoxia

66
Q

Alpha Thalassemia

A
  • occurs in Chinese, Thai, African, and Mediterranean descent
  • usually results in “hydrops fetalis” - build up of bodily fluids in neonates
67
Q

Beta Thalassemias

A
  • occurs in Greeks, Italians, and Syrians
  • most common
  • Complications: RBCs fragile and easily destroyed (body tries to increase production of fetal Hgb)
  • As hemolysis increases, deposits in the skin l/t bronze skin color; LT may see liver failure, endocrine complications, HF
68
Q

Complications of Beta Thalassemias

A
  • frequent transfusions: hemosiderosis and alloimmunization
  • cardiac complications are LEADING CAUSE OF DEATH in these pts.
  • Chelating drugs (Desferal) allows Fe to be excreted in the urine
  • blood transfusions q2-4 weeks to maintain normal Hgb levels