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

Lecture 47 + 48 + DLA Flashcards

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??

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)

A

transports cortisol in the blood

24
Q

RBP (retinal binding protein) (alpha-1)

A

transports retinal in the blood from the liver to the tissues

25
Q

Alpha-2 macroglobulin (alpha2)
function?
clinical?

A

inhibits many kinds of proteases

found in high amounts in those with Nephrotic syndrome due to damage the BM of the glomerulus

26
Q

Ceruloplasmin (alpha2)

function?

A

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
Q

Wilsons disease?

A

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
Q

Heptoglobin (alpha2)

function?

A

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
Q 
Transferrin (beta) 
function and clinical?
A

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
Q 
Hemopexin (beta) 
function and prevention?
A

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
Q

LDL (beta)

A

only have B-100

+ charge

32
Q

The immunoglobins? (gamma)

functions?

A

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
Q

multiple myeloma?

A

A tumor of the plasma cells and an example of monoclonal gammopathy

on the chart the gamma protein wave spikes!

34
Q

Hepatic acute phase?

+ and - reactions?

A

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
Q

C-reactive protein

A

synthesized and released during inflammation

36
Q

acute inflammation (Densitometry)

A

smaller albumin peak

higher alpha 1 and 2 peak

37
Q

Liver cirrhosis (densitometry)

A

smaller albumin peak
beta2-gamma bridge (both increase)

alpha2 peak is smaller

38
Q

multiple myeloma (densitometry)

A

high spike in gamma

39
Q

Nephrotic syndrome? (densitometry)

A

very low albumin

Very high alpha 2

40
Q

hypogamma globulinemia (densitometry)

A

no gamma peak

41
Q

Alpha 1 antitrypsin deficiency (densitometry)

A

very low alpha1 peak

42
Q

prolonged inflammation (densitometry)

A

Wide gamma peak

43
Q

Osmoregulation?

A

retainment or excretion of water
can be dilute or concentrated
depends on ADH

44
Q

ADH effect on V1 and V2

ADH effect on osmolarity?

A 
V1 = vasoconstriction 
V2= water retention

the higher the ADH concentration, the higher the plasma osmolarity

High ADH/ high osmolarity = thirst

45
Q

what happens during low protein states in the nephron?

A

limited ability to concentrate the urine

low urea / age below 1

46
Q

how can the urine concentrate to 1200 mOsm

A

By urea!

47
Q

vasa recta movement?

A

descending = salt in and water out

ascending = water in and salt out

matches descending LOH to prevent backtracking

48
Q

collecting duct during low and high ADH

A

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
Q

Diabetes insipidus?

types?

A

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
Q

How to test for the type of DI?

A

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
Q

clearance equation?

A

clearance = rate of excretion / plasma excretion

52
Q

osmolar clearance equation?

A

Cosm = urine osmolarity x flow rate / plasma osmolarity

53
Q

free water clearance equation

A

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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