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97 Cards in this Set

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most common immunodeficiency syndromes: humoral / cell-mediated?
humoral/antibody deficiency syndrome
recurrent infections (encapsulated bugs) + no response to abx Tx

dx?
primary b-cell deficiency
immunodeficiency syndromes - increase risk of...(3)
1.infection
2.malignancy
3.autoimmune disorders
frequent URI after age > 6 mos. (OM, sinusitis, pna)
primary b-cell deficiency
XLA etiology
Bruton tyrosine kinase deficiency (no mature B cells)
XLA babies susceptible to...(2)
1.encapsulated bugs
2.life-threatening enteroviruses
CVID: low Ig?
IgA, IgG (hypogammaglobulinemia)
CVID: increased risk of...(2)
1.lymphoma
2.autoimmune disease
most common immunodeficiency
selective IgA deficiency
nl reaction to viruses, lots of bacterial infxns (gi, respiratory, urinary)
selective IgA deficiency
infants particularly susceptible to infections at age...
6-12 weeks
transient hypogammaglobulinemia of infancy
disorder when nl infant ig acquisition delayed
common infections seen in humoral immunodeficiency syndromes (3)
1.OM
2.PNA
3.sinusitis
T-cell abnlities predispose to...(3)
1.viruses
2.mycobacteria
3.fungi
no thymic shadow on CXR
DiGeorge's syndrome
how to measure T-cell fxn? (2)
1.in vitro mitogen testing
2.intradermal delayed hypersensitivity testing
DiGeorge syndrome Tx (initial and long-term)
1.initial: fix congenital heart defects, normocalcemia
2.long-term:thymic & BM transplant
congential heart disease + hypocalcemia
diGeorge's syndrome
diGeorge's syndrome: etiology
chromosome 22 deletion
SCID: inheritance pattern
1.autosomal recessive
2.X-linked
3.spontaneous
multiple illnesses in first few months of life. dx?
SCID
AT: inheritance pattern?
1.autosomal recessive
AT: increased incidence of...?
CA
1.non-Hodgkin lymphoma
2.gastric ca
cerebellar ataxia + oculocutaneous telangiectasia + malignancy + immmunodeficiency
AT
Wiskott-Aldrich's syndrome: inheritance pattern
X-linked recessive
atopic dermatitis + thrombocytopenia + B-cell defects + bleeding + infections
Wiskott-Aldrich's syndrome
abs do not respond to CHO ags properly. dz?
Wiskott-Aldrich's syndrome
neutropenia: causes?
1.infection
2.meds
3.abs
4.ca
5.aplastic anemia
most common inherited disorder of phagocytes
CGD (chronic granulomatous disease)
when neutrophils/monocytes cannot kill bugs after ingesting them. dz?
CGD
CGD: inheritance pattern
1.autosomal recessive
2.X-linked
"severe" neutropenia = ?
<500 neutrophils
"chronic" neutropenia = ?
> 2-3 months
dx test for CGD
nitroblue tetrazolium test (NBT)
acute neutropenia: Tx?
none.
CGD: daily prophylaxis
TMP-SMX + IFN-g
infection but no signs of infection. dx?
neutropenia
frequent skin infections/abscesses. dx?
CGD
allergic triad
1.allergic rhinitis
2.asthma
3.eczema (atopic dermatitis)
C' deficiencies: more susceptible to...?
1.bacterial infections
2.rheumatological diseases
most frequent cause of chronic/recurrent clear rhinorrhea
allergic rhinitis
2 characteristics of allergic rhinitis found on exam
1.allergic shiners
2.allergic salute
unilateral, thick, foul smelling nasal d/c. dx?
nasal foreign body
preferred method for allergy testing
1.RAST
2.direct skin testing
most effective Tx for allergies
allergen avoidance
hereditary angioedema - etiology
C1 esterase deficiency
signs & symptoms of food allergy in infants
1.irritability
2.diarrhea
3.fft
gold standard for food allergy dx
double-blind, placebo challenge-food challenge
JRA dx criteria
1.age<16
2.arthritis in at least 1 joint for 6 consecutive wks
3.arthritis = limited ROM, tenderness/pain on motion, increased warmth
4.exclusion of other causes
JRA: more common in boys/girls?
girls - JRA
JRA: risk factors
1.+FH
2.HLA-DR4,5
high spiking fevers + salmon-colored evanescent rash + lymphadenopathy + hepatosplenomegaly + (-)ANA + (-)RF
systemic onset JRA
JRA types w/o chronic uveitis
systemic onset JRA
most common JRA
pauciarticular JRA
large joint (knees/ankles) arthritis + (+)ANA + 70% remission
pauciarticular JRA
JRA w/(+)ANA indicates increased risk for...
aSx uveitis
polyarticular JRA: x joints?
5 or more joint (small/large) involvement
JRA affecting TMJ / cervical vertebrae
polyarticular JRA
inflammation of anterior chamber eventually leading to synechiae
uveitis / iritis / iridocyclitis
synechiae?
adhesion b/t iris and cornea/lens (irregular pupil)
T/F: uveitis can lead to visual loss?
true.
T/F: uveitis is aSx?
true
uveitis Tx
steroid eye drops + mydriatics
JRA: mainstay Tx
1.anti-inflammatory meds
2.physical therapy
pauciarticular JRA requires f/u for...
eye. opthalmologic surveillance q 3-4 mos.
widespread ct inflammation + arteriolar vasculitis
SLE
ag-ab complexes seen in...
SLE
SLE dx requires x of 11 criteria
4 of 11 for SLE dx
fever, malaise, wt loss - sx commonly seen in...
SLE
arthritis producing pain out of proportion to physical signs - dx?
SLE
most common clinical manifestation of SLE
lupus nephritis
lab findings for SLE
anemia, thrombocytopenia, leukopenia, decreased C', (+) ANA, (+)anti-dsDNA, (+)anti-Smith
anti-Ro and anti-La abs in SLE mom may cause...
congenital heart block in fetus
SLE tx
1.anti-inflammation meds
2.immunosuppresant meds
leading cause of death in SLE pts
renal failure
SLE: serious cardiac complication
Libman-Sacks endocarditis
T/F: SLE is a clinical dx.
true
SLE: age group target
late childhood
adolescence
females
AA/Hispanics
inflammatory dz of skin small vessels, striated muscle, GI tract
dermatomyositis
dermatomyositis w/o skin findings
polymyositis
dermatomyositis: target age group
age 2-10 (peak age 7)
girls
fatigue, malaise, wt loss, intermittent fevers - constitutional sx seen in...
dermatomyositis
dermatomyositis: weakness begins in proximal/distal muscle groups?
proximal muscle groups
dermatomyositis: elevated...?
CK levels
scaly erythematous papules on extensor surface of IP joints, elbows, knees
Gottron papules (dermatomyositis)
dermatomyositis: dz severity correlates with...
levels of serum acute-phase reactants (CRP, ESR)
dermatomyositis: tx
1.immunosuppresants
2.physical therapy
dermatomyositis: leading cause of death
GI perforation
painful, erythematous skin nodules, HTN, hematuria, encephalopathy, neuropathy
polyarteritis nodosa
IgA mediated vasculitis of GI + skin + joints + kidneys
Henoch-Schonlein purpura
dx criteria for Kawasaki
1.fever for 5 or more days
2.bilateral conjunctivitis
3.lips/oral cavity changes
4.erythema/edema of extremities
5.polymorphous rash (trunk)
6.cervical LN swelling >1.5cm
Kawasaki's disease: tx that reduces coronary artery aneurysms
IVIG
high fever + lymphadenopathy + mucocutaneous lesions
Kawasaki's disease
Kawasaki's disease: age group target
infants
young children
boys
Kawasaki's disease: 3 phases
acute, subacute, convalescent
Kawasaki's disease: most serious complications
cardiac
1.coronary vasculitis
2.coronary aneurysm
Kawasaki's disease: initial tx
aspirin