Use LEFT and RIGHT arrow keys to navigate between flashcards;
Use UP and DOWN arrow keys to flip the card;
H to show hint;
A reads text to speech;
97 Cards in this Set
- Front
- Back
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
|