Pathoma - 2 (Inflammation and Wound Healing)

eliot2014's version from 2016-01-22 01:28

Section 1

Question Answer
What does CD14 (TLR) on macrophages recognize?LPS (PAMP) on outer membrane of gram negative bacteria
what does TLR activation upregulate?NFKB = nuclear transcription factor that activates the immune response genes
which prostaglandins mediate vasodilation and increased vascular permeability?PGD2, PGE2, PGI2
whats the major mechanism by which MAST CELLS allow of progression of inflammatory response?leukotrienes
what are 3 mediators of arteriolar smooth muscle relaxation for redness and warmth?histamine, prostaglandins and bradykinin
what molecule on neutrophils binds to endothelial P-selectin and E-selectin during rolling stage?Sialyl Lewis X binds to selectins
what 3 proteins are upregulated on endothelium by TNF and IL-1?E-selectin (rolling stage), VCAM + ICAM (adhesion stage)
what is leukocyte adhesion deficiency due to?deficiency in CD18 (integrin)
lack of pus and delayed separation of umbillical cord is seen in what disease?leukocyte adhesion deficiency
which catalase positive organisms infect CGD pts the most?pseudomonas cepacia, staph aureus, serratia, norcardia, aspergillus
MPO deficient pts are at increased risk from what infection?candida infections
pseudomonas cepacia is associated with what disease?CGD= a/w with bacteria that CATALASE
whats the most effective mechanism for destruction of phagocytic material?O2-dependent killing in neutrophils (vs O2-independent killing using lysozymes in macrophages)
what provides the second signal for CD8+ T-cells?IL-2 from CD4+ TH1
whats the role of IL-2?T-cell growth factor and second signal for CD8+ T-cell activation
what are the 2 signals to activate CD4+ T-cells?1) MHC-Ag to TCR
2) B7 on APC binds to CD28 on T-cells
whats the first signal required for B-cell activation?1) Ag binding to surface IgM on B-cells
whats the 2nd signal required for B-cell activation?2) CD40 receptor on B-cells binds to CD40 ligand on helper T-cells

autoimmune disorders

Question Answer
prevalence of autoimmune disorders in US1-2 %
Mutation associated with autoimmune polyendocrine syndromeAIRE
Autoimmune polyendocrine syndrome consists of (3 things)hypoparathyroidism, adrenal failure, and candida infections
What mutation is associated with autoimmune lymphoproliferative syndrome (ALPS)Fas apoptosis pathway mutations
Is Autoimmune polyendocrine syndrome a disorder of central or peripheral tolerance?central (in thymus specifically, not the bone marrow)
Is autoimmune lymphoproliferative syndrome (ALPS) a disorder of central or peripheral tolerance?peripheral
What two cytokines to Tregs create?IL-10 and TGF-beta
What is IPEX syndrome?Immune dysregulation, Polyendocrinopathy, Enteropathy, X-linked. Associated with FOXP3 mutation
FOXP3 mutation is associated withIPEX syndrome
CD25 polymorphisms are associated with which two autoimmune diseases?MS and Type 1 DM
What effect does estrogen have on immune cells?It reduces apoptosis of self-reactive B cells
What type of hypersensitivity reaction is SLE?Type III (nb hematologic problems are from type II hypersensitivity reaction)
Deficiency of what is associated with SLEearly complement proteins (C1q, C4, and C2)
What are common cause of death in SLErenal disease, infection (either due to immunosuppressant treatment or antibodies against WBCs), coronary atherosclerosis (occurs late)
What type of antibody is associated with SLE renal disease?anti-dsDNA
Classic findings in SLErash (malar or discoid), arthritis (involving 2 or more joints), serositis (pleuritis, pericarditis), hematologic disorders (e.g. cytopenias--these are a TYPE II HSR), oral/nasopharyngeal ulcers (usually painless), renal disease (diffuse proliferative glomerulonephritis=most common; membranous glomerulonephritis), Raynaud phenomenon, Photosensitivity, Postivit VDRL/RPR, antinuclear antibodies (sensitive but not specific), anti-dsDNA or anti-Sm antibodies (specific) neurologic disorders (seizures, psychosis), Liebman-Sacks endocarditis
which Abs cause false positive syphillis tests and falsely elevated PTT levels in SLE pts, respectively?anticardiolipin and lupus anticoagulant
what antibodies are associated with antiphospholipid antibody syndromeanticardiolipin, anti-beta2-glycoprotein I, and lupus anticoagulant
Women with cerebral stroke and recurrent pregnancy loss?antiphospholipid antibody syndrome
Antihistone antibody is associated withdrug-induced lupus
Which two organs are rarely involved in drug induced lupusCNS and kidney
What is Sjogrens?autoimmune destruction of lacrimal and salivary glands
Presentation of Sjogrendry eyes, dry mouth, and recurrent dental caries. May progress to ulceration of corneal epithelium and oral mucosa
What is sicca syndromeprimary sjogren syndrome
Which antibodies are associated with extraglandular manifestation of Sjogrens (e.g. neuropathy)anti-SSA and anti-SSB
what type of hypersensitivity rxn is sjorgens?Type IV hypersensitivity
anti-ribonucleoprotein antibodiesSjorgen
whats the target of anti-SS-A and anti-SS-B?ribonucleoproteins
what other autoimmune disease is associated with Sjorgens Syndrome?Rheumatoid Arthritis (HLA-DR4)
anti-SSA and anti-SSB are associated withSjogrens (maybe SLE)
Presentation of Sclerodermasclerosis of skin and visceral organs
Babies born to mothers with anti-SSAneonatal lupus and congenital heart block
pt with Sjorgens with BL enlargement of parotid glands, starts to get enlargment of one parotid gland?B-cell marginal zone lymphoma
What is CREST syndrometype of scleroderma (limited type)--skin involvement is limited to hands and face with late visceral involvement
What is CREST characterized byCalcinosis/anti-Centromere antibodies, Raynaud phenomenon, Esophageal dysmotility (due to fibrosis or lower wall of esophagus), Sclerodactyly, and Telangiectasias of skin
What is diffuse type Sclerodermaskin involvement is diffuse with early visceral invovlement
Organs commonly involved in diffuse sclerodermavessels (raynauds), GI (esophageal dysmotility and reflux--due to sclerosis-->delayed emptying of stomach-->GERD)--most common, Lungs (interstitial fibrosis and pulmonary hypertension)--most common cause of death, kidneys (scleroderma renal crisis)--2nd most common cause of death
anti-DNA topoisomerase I antibodiesdiffuse type scleroderma
antibodies against U1-ribonucleoprotein and ANA?Mixed Connective Tissue Disease
What is mixed connective tissue disease characterized bymixed features of SLE, systemic sclerosis, and polymyositis. They have Raynaud, severe arthritis, GERD, esophageal dysmotility, myalgia, and pulmonary hypertension. Lack severe renal and CNS problems.

Section 3

Question Answer
Name 2 stable tissues?liver hepatocytes and PCT of kidney (ATN)
What 3 things make up granulation tissue?capillaries
fibroblasts (Type III collagen)
myofibroblasts (contracts wound)
Whats the cofactor of collagenase (which replaces Type III collagen with Type 1?Zinc
Whats the growth factor for endothelium, smooth muscle, and fibroblasts?PDGF
What growth factor is important for angiogenesis? and skeletal muscle development?fibroblast growth factor
What 2 growth factors are crucial for angiogenesis?FGF and VGEF
whats the cofactor for lysyl oxidase, whcih crosslinks lysine and hydroxylysine to form stable collagen?copper
what collagen is predominant in hypertrophic scars? in keloids?hypertrophic scar = Type I collagen
Kelloid = Type III collagen