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.