set 2 Flashcards
b cell surface antigens
cd 19, 20, 21, 40. 21 for ebv. b7. mhc11
macrophages antigens
cd14 (receptor for pamps, eg lps) cd40 ccr5 mhc2 b7(cd80/86) fc and c3b receptors (both enhance pahgocytosis)
brain mets
primary lung > breast > melanoma, colon, kidney
liver mets
colon >> stomach > pancreas liver and lung are the most common mets after lymphadenopathy
bone mets
prostate, breast > lung, thyroid, kidney bone mets more common then primary breast (mixed), lung lytic, thyroid lytic, kidney lytic, prostate is blastic predilection for the axial skeleton
cri du chat synrome
congential microdeltion of short arm of 5 (46xx or xy 5p-). findings: microcephaly, moderate to sever intellectual diability, high pitched crying/meowing, epicanthal folds, cardiac defects (vsd) u world partial denove mutation
fetal erythropoesis
yolk sac (3 8 weeks) liver (6 weeks - birth) spleen (10 - 28 weeks) bone marrow (18 weeks)
heme synthesis
acute intermittent porphyria
pophobilinogen deaminase (formerly called uroporphyrinogen 1 synthase) autosomal dominant
accumulation of porphobilinogen and ala
5 ps
painful abdomen
port wine urine (red initially -> brown)
polyneuropathy
psycholigic (anxiety, confusion, paranoia)
precipated by drugs (cyp p450 induce), alcohol, starvation
treat with glucose, heme which inhibit ala synthase
porphyria cutanea tarda
uroporphyrinogen decarboxylase
ad
uroporphyrin (tea color urine) accumulates
blistering cutaneous photosensitivty and hyperpigmentation
most common porphyria
exacerbted with alcohol consumption
lead treatment
dimercaprol and edta 1st line
succimer in kids
type 1 hyper chylomicronemia
ar
lipoproteinlipase or apolipoportein c 11
increase in chylomicrons, tg, cholesterol
pnacreatitis
eruptive/pruitic xanthomas
no increased risk for atherosclerosis
creamy layer in the super natant
type 2 famiilial hypercholesterolemia
AD
abesent or defective ldl receptor
IIa: ldl and cholesterol increased
IIb: ldl, cholesterol, vldl increased
heterzygots (1:500): have cholesterol = 300 mg/dl
homozygotes (very rare): 700+ mg/dl
accelerated atherosclerosis (may have mi before age 20), tendon (achilles) xanthomas, corneal arcus
type III dysbetalipoproteinemia
ar
defective apoe
increased chylomicrons and vldl (remants)
increased triglyceride and cholesterol
premature atherosclerosis, tuberoeruptive xanthomas
xanthoma striatum palmare
type IV hypertriglyceridemia
AD
hepatic overproduction of vldl
increased vldl and triglycerides
hypertriglyceridemia (> 1000 mg/dl) can cause acute pancreatitis
erb palsy (waiters tip)
traction on upper c5 - c6
infants delivery and adults tramua
deltoid, supraspinatus -> abduction (arm hangs by side)
infraspinatus -> lateral roation (arm medially rotated)
biceps brachii -> flexion, supination (arm extended and pronated)
klumpe palsy
traction on lower trunk (c8-t1)
infants - upward force on arm during delivery
adults trauma (grabbing tree)
intrinsic hand muscles: lumbricals, interossi, thenar, hypothenar
total claw: all lumbricals damaged
thoracic outlet obstruction
compression of lower trunk and subclavian vessels
caused by cervical rib or pancoast tumor
intrinsic muscle of hand: lumbricals, interosseia, thenar, hypothenar
atrophy of instrinsic hand mucles, iscehmia, pain, and edema due to vascular compression
il-1
Secreted by macs
casues fever, acute inflammation. activates endotehlium to express adhesion molecules. Induces chemokine section to recruit wbcs.
neutrophil/mac migration
increased in acute phase reactants
shock
il 6
secreted by macs
causes fever and stimulates proeuction of acute phase proteins in the liver.
il 8
secreted by macs
major chemotactic factor for neutrophils
il 12
induces differentiation of t cells into th1 cells. activate nk cells + cd8.
tnf a
secreted by macs
activates endothelium, causes wbc recruitment, vascular leak.
septic shock, fever
causes cachexia in malignancy
maintains granulomas in TB
il 2
secreted by all t cells
(mostly helper cells)
stimulates growth of helper, cytotoxic, and regulatory t cells, and nk cells
il 3
secreted by t cells
supports growth and differentiation of bone marrow stem cells. functions like gm-csf
increases hematopoesis
inf g
secreted from th1
secreted by nk and t cells in response to antigen or il 12 from macs. stimultated macs to kill phagocytosed pathogens. inhibits differentiation of th2
also activates nk cells to kill cirus infected cells. increases mhc expression and antigen presentation on all cells.
il 4
from th2 cells
induces differentiation of t cells into th2 cells. promotes growth of b cells. enhances class switching to ige and igg.
il 5
promotes growth and differentiation of bcells. enhances class switching to iga. stimulates growth and differentiation of eosinophils.
il 10
attenuates inflammatory response. decreases expression of mhc II and th1 cytokines (il2 and inf g). inhibits activated macs and dendritic cells. also secreted by t regs. some enhancement of th2. decrease th1 differentiation and increase th2.
tgf b and il 10 both attenuated the immune response
dilated cardiomyopathy
most common cardiomyopathy
idiopathic or familial (usuallu AD)
ABCCCD
alcohol use, wet beri beri, coxsackie B viral myocarditis, chronic cocaine use, chagas disease, doxorubicin
hemochromatosis (k says late), sarcoidosis, peripartum.
findings: hf, s3, systolic regurgitant murmur, dilated heart on CXR
treatment:
na restriction, ace inhibitors, b blockers, diuretics, digoxin, ICD, heart transplant
Takotsubo cardiomyopathy
“broken heart syndrome”
ventricular apical balloning likely due to increased sympathetics (stressful situations)
eg death of love one, break up, chronic anxiety
temporary muscular weakening
can cause acute hf, arrythymia, ventricular rupture
those that survive prognosis is excellent
Hypertrophic (obstructive cardiomyopathy)
70 % familial
myosin binding protein c, b myosin heavy chain
findings: s4, systolic murmur, can see mitral regurgitation.
treatment:
cessation of high intesenity exercise, beta blockers or non dihydropyridine ca channel blockers (relieve obstruction)
icd if patient at high risk
crescendo decrescendo stysloic murmu like aortic stenosis
restrictive/infiltrative cardiomyopathy
Puppy LEASH
post radiation fibrosis, loffler syndrome, endocardial fibroelastosis (thick fibroelastic tissue in the endocardium of young children, amylodosis, sarcoidosis, hemochromatosis (although dilated cardiomyopathy is more common).
low voltage ekg depsite thick myocardium (especially in amyloid).
loffler syndrome: endomyocadrial fibrosis with prominent eosinophillic infiltrate.
presents as CHF
axillary roots
c5 -c6
musculocutaneous roots
c5-c7
radial roots
c5-t1
median roots
c5-t1
ulnar
c8-t1
reccurent branch of the median nerve roots
c5-t1
arm abduction
supraspinatus 0-15
deltoid 15-100
traps > 90
serratus anterior > 100
men 1
wermers
pituitary (prolactin or gh)
mutatin of men1 gene (codes for menin a tumor supressor gene on 11)
men 2a/2b
sipple syndrome
usually thyroid caner presents first
asssocaited with ret mutation (codes for receptor tyrosine kinase) in cells of neualcrest origin
men 2b also ret mutation
right atrial pressre
<5
right ventricular pressure
25/5
puomary artery pressure
25/10
PCWP pressure
4-12
left ventricular pressure
130/10
aortic pressure
130/90
mitral valve prolapse
late systolic crescendo.
most frequent valvular lesion
loudest just before s2
most of the time incidental
predispose to infective endocarditis
can be caused by rheumatic fever, chordae rupture
can cause arrythymia or mitral regurgitation
mxomatoud degeneration - primary or secodnary to marfan/ehlers
aoritc area
Systolic:
aortic stenosis
flow murmur (eq physiologic murmur)
aortic valve sclerosis
erb’s point
diastolic:
aoritc and pulmonic regurg
Systolic:
Hypertrophic cardiomyopathy
pulmonic area
systolic:
pulmonic stenosis
flow murmur
tricuspid area
holosystolic murmur:
tricuspid regurgitation
VSD
Diastolic:
tricuspid stenosis
ASD from increased flow across tricuspid valve
mitral area (apex)
holosystolic murmur
mitral regurgitation
systolic murmur
mitral valve prolapes
diastoliic:
mitral stenosis
mitral/tricuspid regurgitation
holosystolic high pitched blowing murmur
mitral - radiates to axilla
MR often due to iscehmic heart disease (post mi), MVP, LV dilation (CHF)
TR commonly due to RV dilation
Rheumatic fever or infecetive endocarditis can cause either MR or TR
