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CPR II (last of T1) > Lecture 47 + 48 + DLA > Flashcards

Lecture 47 + 48 + DLA Flashcards

(53 cards)

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1
Q

what gene encodes for AAT?

alpha 1 anti-trypsin

A

SERPINA1 gene

serine protease inhibitor

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2
Q

what is the target for AAT?

A

elastase

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3
Q

what is the role of AAT?

A

protect lung tissue from the elastase released from neutrophils during infection

also protects from non-specific damage

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4
Q

different alleles of the AAT gene?

A

PiMM
PiMZ
PiSZ
PiZZ

M = functional 
Z = most common deficiency 
S = deficiency, but not as bad as Z
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5
Q

COPD and ATTD

A

autosomal recessive disorder

caused by a loss of function

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6
Q

what is common in those with the PiZZ deficiency?

A

liver disease

protein product will accumulate in the cell and will not be secreted

forms aggregates

‘attainment of new function’

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7
Q

how to treat AATD?

A

IV administration of A1AT protein

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8
Q

what are the four regulators of the effective circulating volume (ECV)?

A

RAAS
sympathetic NS
starling forces
ANP (atrial natriuretic peptide)

all these increase total body sodium

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9
Q

movement of the proteins in serum protein electrophoresis?

A 
albumin moves the furthest (most negative) 
Alpha-1 
Alpha -2 
Beta 
Gamma moves the least (most +)
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10
Q

what are the proteins in the Alpha-1 globulin bands?

A

Alpha 1 antitrypsin
alpha fetoprotein
transcortin
Retinal binding protein

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11
Q

What are the proteins in the alpha-2 globulin bands?

A

Alpha2 macroglobulin
Ceruloplasmin
haptoglobin

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12
Q

what are the proteins in the beta band?

A

transferrin
hemopexin
LDL

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13
Q

what are the proteins in the gamma band

A

immunoglobins

IgG, IgM, IgA, IgD, and IgE

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14
Q

functions of albumin?

A

maintenance of osmotic pressure

transport Ca and whatnot

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15
Q

general function of alpha and beta globulins

A

enzymes, transport, and inhibitor proteins

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16
Q

general functions of immunoglobins

A

immune function

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17
Q

Congenital analbuminemia?

A

appear normal and do not get edema

other proteins regulate osmotic pressure early on

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18
Q

what happens during decreased synthesis of albumin?

A

Kwashiorkor - deficiency of protein, thus less albumin

liver cirrhosis - liver damage impairs protein synthesis

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19
Q

increased loss of albumin??

A

Kidney disease - loss in urine due to basement membrane damage in the glomerulus

severe burns - loss of serum

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20
Q

Alpha 1 antitrypsin deficiency (alpha-1)

A

alpha 1 antitrypsin is a protein released by the liver to inhibit neutrophil elastase in the lung alveoli

this deficiency leads to degradation of elastin in the lung tissue, thus emphysema

hereditary?
due to a defective N-glycosylation, thus reduced release from the liver
can lead to pulmonary and liver disease

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21
Q

alpha Fetoprotein (alpha-1)

A

is found in a large amount during fetal life
low levels of albumin

fetoprotein levels are low in adults, normally, but can increase if one has liver cancer

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22
Q

during pregnancy AFP levels can mean what??

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A

high level of maternal AFP can lead to a neural tube defect

lows levels of maternal AFP can point at down syndrome

23
Q

Transcortin (alpha-1)

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A

transports cortisol in the blood

24
Q

RBP (retinal binding protein) (alpha-1)

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A

transports retinal in the blood from the liver to the tissues

25
Alpha-2 macroglobulin (alpha2) function? clinical?
inhibits many kinds of proteases found in high amounts in those with Nephrotic syndrome due to damage the BM of the glomerulus
26
Ceruloplasmin (alpha2) | function?
used for copper transport in the blood also ferroxidase activity = prevents radical damage done by ferrous ion by forming ferric iron that can bind to transferrin for transport
27
Wilsons disease?
have very low levels of ceruloplasmin as hepatic copper binding is decreased thus, the liver releases aceruloplasmin, without copper, and is degraded in the blood have a copper transporting ATPase deficiency damage due to copper accumulation occurs hepatic dysfunction, neurological and psychiatric symptoms Kayser-Fleischer rings can be seen!
28
Heptoglobin (alpha2) | function?
binds to free hemoglobin dimers in the blood and prevents loss of Hb in the urine heptoglobin-hemoglobin complex is taken up by macrophages; this explains why acute hemolysis leads to low levels of free heptoglobin
29
``` Transferrin (beta) function and clinical? ```
transports ferric iron in the blood transferrin can bind two atoms for transport clinical: low saturation = seen in those with iron deficiency high saturation = iron overload
30
``` Hemopexin (beta) function and prevention? ```
binds to free heme in the blood and prevents the loss of heme-iron heme-hemopexin can be taken up into heptacytes and the iron is bound to ferritin (storage protein) prevents: heme-induced damage to PM heme usage of microbes
31
LDL (beta)
only have B-100 | + charge
32
The immunoglobins? (gamma) | functions?
IgM = first antibody to be produced IgG = produced by multiple exposures to same antigen; passive immunity to fetus IgE = allergic reaction IgA = main antibody found in human milk IgD = role is not known
33
multiple myeloma?
A tumor of the plasma cells and an example of monoclonal gammopathy on the chart the gamma protein wave spikes!
34
Hepatic acute phase? | + and - reactions?
overall positive response that prevents damage positive: expressed in higher amounts since they reduce inflammation and deprive microbes 1. alpha-1 antitrypsin 2. ceruloplasmin 3. heptoglobin 4. hemopexin negative: reduced expression 1. albumin 2. transcortin 3. RBP 4. transferrin
35
C-reactive protein
synthesized and released during inflammation
36
acute inflammation (Densitometry)
smaller albumin peak | higher alpha 1 and 2 peak
37
Liver cirrhosis (densitometry)
smaller albumin peak beta2-gamma bridge (both increase) alpha2 peak is smaller
38
multiple myeloma (densitometry)
high spike in gamma
39
Nephrotic syndrome? (densitometry)
very low albumin | Very high alpha 2
40
hypogamma globulinemia (densitometry)
no gamma peak
41
Alpha 1 antitrypsin deficiency (densitometry)
very low alpha1 peak
42
prolonged inflammation (densitometry)
Wide gamma peak
43
Osmoregulation?
retainment or excretion of water can be dilute or concentrated depends on ADH
44
ADH effect on V1 and V2 ADH effect on osmolarity?
``` V1 = vasoconstriction V2= water retention ``` the higher the ADH concentration, the higher the plasma osmolarity High ADH/ high osmolarity = thirst
45
what happens during low protein states in the nephron?
limited ability to concentrate the urine low urea / age below 1
46
how can the urine concentrate to 1200 mOsm
By urea!
47
vasa recta movement?
descending = salt in and water out ascending = water in and salt out matches descending LOH to prevent backtracking
48
collecting duct during low and high ADH
Low: collecting duct is impermeable to water no urea reabsorption slightly hyperosmotic high: dehydrated permeable to water by AQP-2 urea reabsorption highly hyperosmotic
49
Diabetes insipidus? | types?
This is due to a ADH deficiency nephrogenic DI: V2 receptor mutation; AQP-2 mutation acquired by lithium therapy central DI: congenital lack of ADH acquired by head trauma
50
How to test for the type of DI?
No water to drink for 4 to 6 hours. central and nephrogenic will have no increase in urine osmolarity give ADH central DI: large increase in osmolarity nephrogenic: still no change
51
clearance equation?
clearance = rate of excretion / plasma excretion
52
osmolar clearance equation?
Cosm = urine osmolarity x flow rate / plasma osmolarity
53
free water clearance equation
water clearance = V - (Uosm x V / Posm) water clearance = V - Cosm ``` 0 = iso-osmotic + = hypo-osmotic - = hyper-osmotic ```

CPR II (last of T1) (14 decks)

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