ch. 11 immuno, allergy, rheumatology
Terms
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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