Exam 1 Flashcards
DiGeorge Syndrome (complete or partial)
T cell disorder caused by chromosome deletion
- hypocalcemia with tetany after birth
- cardiac defects
- low set ears
- Complete DiGeorge –> need thymus transplant
Severe Combined Immunodeficiency Disease (SCID)
T cell/B cell immunity is deficient or absent
- infection, pneumonia, oral ulcers, FTT, dermatitis
- “Bubble Boy Disease”
Systemic Lupus Erythematosus (SLE)
- 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
SLE - Criteria for Diagnosis (must have four)
- renal d/o
- neurologic disorders
- hematologic disorders
- immunologic disorders
- antinuclear antibodies
- butterfly rash
- discoid rash
- photosensitivity
- oral ulcers
- arthritis
- serositis
Juvenile Idiopathic Arthritis (JIA)
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
s/s of JIA
(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
JIA Diagnostics
- ESR & CRP determine amount of inflammation
- antinuclear antibodies are common, but not specific to JIA
- leukocytosis and anemia during exacerbations
Pharmacologic Management of JIA
- NSAIDS
- DMARDs (disease modifying antirheumatic drugs)
- SAARDs (slow-acting antiarthritic drugs)
- Corticosteroids
- cytotoxic agents
- immunologic modulators
- biologic response modifiers
Other Management of JIA
DON’T massage - risk of emboli
DON’T immobilize - risk of loss of function
DON’T elevate - does not help pain
Hypersensitivity Response: anaphylaxis
within seconds to minutes of exposure
- IgE
- most common type of allergy = BEE STING/POLLEN
Hypersensitivity Response: cytotoxic reaction
within 15-30 min of exposure
- cell surface antigens (blood)
- antigen/antibody binding activates complement
- includes transfusions, Rh incompatibility, ITP, and AI hemolytic anemia
Hypersensitivity Response: immune complex disease
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
Hypersensitivity Response: delayed hypersensitivity
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
foods to avoid with latex allergy
bananas, kiwis, avocados
IVIG
used to treat immune thrombocytopenic purpura (ITP), Kawasaki disease, primary immunodeficiency d/o’s, hemolytic anemia, AIDS, etc.
Cancer: most common malignancies in PEDS
- leukemia
- meduloblastoma
- astrocytomas
- ependymoma
- gliomas
- neuroblastoma
neuroblastoma
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)
Wilm’s tumor (nephroblastoma)
!!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
“bubble boy disease”
severe combined immunodeficiency disease (SCID)
Which cardiac defect has a machine-like murmur?
PDA
In which defect are peripheral pulses diminished in the LOWER extremities?
coarctation of the aorta
Treatment for Kawasaki’s Disease
IVIG
Kawasaki’s can cause what in relation to the heart?
vasculitis (inflammation of BV’s) – can l/t ANEURYSMS
Indomethacin: watch for _______
decreased platelets, decreased urinary output, and necrotizing enterocolitis
Prostaglandin E1 is used for ?
to keep ductus arteriosus OPEN
Respiratory SE of Prostaglandin E1
apnea –> infants must be on ventilators
Digoxin
Tx for CHF