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Year 2 CV > Exam2, biochem, choudhury > Flashcards

Exam2, biochem, choudhury Flashcards

1
Q

Patient has muscle weakness with elevated amounts of TGL and primary long chain fatty acids many lipid vacuoles in muscle biopsy probable Dx?

A

carnitine deficiency.

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2
Q

What breaks down TGL and what are the products.

A

hormone sensitive lipase breaks TGL into glycerol and fatty acids.

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3
Q

what stimulates hormone sensitive lipase? inhibits?

A

epi and cortisol inhibited by insulin

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4
Q

where and how is glycerol converted to glucose.

A

in liver, via DHAP (gluconeogenesis)

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5
Q

.what stimulates gluconeogenesis

A

increased glucagon and cortisol

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6
Q

what converts FA to Acetyl CoA and where

A

Beta oxidation in the liver

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7
Q

what is Acetyl CoA used for

A

ketones and kreb cycle

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8
Q

Where are ketones primarily used for energy

A

cardiac muscle and brain tissue

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9
Q

what oxidation is used when Beta oxidation is defective

A

w oxidation, minor catabolic pathway for medium chain fatty acids

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10
Q

what is beta oxidation

A

process whihc FA are broken down in mitochondria to generate Acetylo CoA

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11
Q

how do short and medium chain FA enter mitochondria for beta oxidation

A

.diffuse freely into mitochondria

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12
Q

how do long chain FA eneter mitochondria

A

transported by carnitine shuttle to be oxidized

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13
Q

how do very long chain FA get oxidized

A

peroxisomes

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14
Q

How much energy is required to activate FA

A

2 high energy bonds

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15
Q

how does Long chain FA cross outer mitochondrial membrane

A

FA transporter

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16
Q

what is first enzyme required to activate FA in between mitochondrial layers

A

fatty acyl CoA synthetase that binds FA to CoA

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17
Q

how does FA CoA cross inner mitochondrial membrane

A

it doesnt first CoA is switched for carnitine via the carnitine acyltransferase 1 CPT-1 enzyme then Fa-carnitine gets shuttle across in carnitine transporter

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18
Q

What enzyme is inside mitochondria to convert carnitine back to CoA

A

Carnitine acyltransferase-2 CPT II

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19
Q

what does the converstion of Fa CoA to Acetylo CoA generate

A

FADH2 and NADH

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20
Q

what are signs of a myopathic CPT deficiency

A

muscle aches and weakness myoglobinuria prolonged exercise increased mm TGL

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21
Q

what are signs of MCAD deficiency medium chain acyl CoA dehydrogenase

A

fasting hypoglycemia no ketone bodies C8-C10 acyl carnitines in blood vomiting coma, death

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22
Q

how are CPT I and II deficiencys treated

A

avoiding fasting, dietary restrictions of long chain FA, carnitine supp

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23
Q

Which cPT deficiency is most common and assoc signs?

A

CPT II muscle weakness upon exercise, hyperammonemia,death

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24
Q

what can cause carnitine deficiency

A

inadequate intake inability to metabolize from enzyme deficiency decreaed endogenous synthesis from liver disorder excess loss (diarrhea, diuressis) hereditary disorder of leakage (primary carnitine deficiency) icnreased requirements for carnitine(sepsis) decreased muscle carnitine from mitochondrial impairment

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25
how are carnitine and or CPT deficiencies diagnosed
extreme reduction in plasma and muscle carnitine levels hypoglycemia muscle biopsy reveals significant lipid vacuoles
26
what does fasting ketogenesis tell you about carnitine disorders
if it is normal then carnitine transport is normal impaired when dietary carnitine intake interupted
27
brown urine, muscle pain in arms and legs during soccer (goes away at night) normal stature well fed sent home with recommendation to take dietary carnitine. most likely Dx?
CPT-II deficiency
28
what age group does MCAD usually manifest
first 3-5 yrs of life
29
what are xanthomas
lesions characterized by accumulation of lipid laden macrophages
30
what are the types of primary hyperlipoproteinemia
I- familial lipoprotein lipase deficiency II- hyperlipidemia III- familial dysbeta-lipoproteinemia IV-familial hyperTGLemia V
31
what are other causes of hyperlipoproteinemia
decreased synthesis of HDL, hepatic lipase deficiency
32
what will type I hyperlipidemia look like
severe elevation of chylomicrons in plasma increase plasma TGL levels no increase in plasma cholesterol early childhood with acute pancreatitis eruptive xanthomas
33
steatorhea is indicative of what underlying malfuncntion
something wrong with pancreas
34
What causes type IIa hyperlipidemia
accumulation LDL from familial LDL-R deficiency and familial defective apo B100`
35
what are signs of type IIa hyperlipidemia
increased plasma cholesterol and TGL manifest severe atherosclerosis can have tendinous xanthomas or tuberous and xanthelasmas
36
What causes type IIb hyperlipidemia
defective apoB100 protein
37
what are signs of type IIb hyperlipidemia
accumulation of both LDL and VLDL variable elevations in TGL and cholesterol may have tendinous xanthomas or tuberous and xanthelasmas
38
what causes type III hyperlipidemia
various mutations of opo-protein E impaires ability to bind IDL R
39
what are signs of type III hyperlipidemia
accumulation IDL increase in TGL and cholesterol premature atherosclerosis and xanthomas
40
what are signs of type IV hyperlipidemia
plasma cholesterol is normal eruptive xanthomas assoc with coronary heart disease, DM II, obesity and alcholism
41
what causes type IV hyperlipidemia
overpdocution VLDL
42
what causes type V hyperlipidemia
genetic defects of apo-lipoprotein C-II gene
43
what are signs of type V hyperlipidemia
accumulations of chylomicroms and VLDL severe elevations of TGL in plasma present in early childhood similar to type I with acute pancreatitis and eruptive xanthomas
44
what causes decreased synthesis of HDL
decreased formation of apo A-I and apo C-III decreased reversed cholesterol transport increased LDL levels
45
What does a hepatic lipase deficieny look likw
coronary heart disease, xanthomas accumulation of large TGL rich HDL and VLDL
46
what other diseases can lead to secondary hypercholesterolemia
pregnancy hypothyroidis, cholestasis acute intermittent porphyria
47
what other conditions/diseases can lead to secondary hyperTGLemia
DM pancreatitis gout type I glycogen storage disease alcoholism oral contraceptive use
48
what conditions can lead to hyper choelsterolemia and hyper TGLemia
nephrotic syndrome chronic renal failure steroid immunosuppressive therapy
49
what is xanthelasma palpebrarum
soft velvety flat yellow lesions associated with hyperlipidemia secondary to cholestasis
50
what are tuberous xanthomas
firm painless red yello nodules that develop in pressure areas like the extensor surface of knees and elbows associated with hypercholesterolemia and increased levels of LDL secondary to nephrotic syndrome and hypothyroidism
51
what is tendinous xanthomas
associated with secere hypercholesterolemia and elevated LDL levels lesions related to trauma nodules related to tendons or ligaments secondary to cholestasis
52
what are eruptive xanthomas
crops of small red-yellow papules that may spontaneously resolve over weeks associated with hyperTGLemia seconary to DM
53
what are plane xanthomas
associated with dysbetalipoproteinemia cover large areas of face neck thorax seconary to cholestasis
54
how do you evaluate if patient has hyperlipoproteinemia
measure plasma lipid and lipoprotein levels after overnight fast 12-16 hours abnormal lipoprotein patterns need to be identified electrophoresis and ultracentrifugation of whole plasma for Dx
55
What are Tx for hyperlipoproteinemia
dietary lipid lowering agents: statins, fibrates, bile acid binding resins, probucol or nicotinic acid
56
when to eruptive, tuberous, and tendinous xanthomas clear up
eruptive resolve in weeks of systemic Tx tubuers after months of Tx tendinous take years to resolve or may persist indefinitely
57
majority of cholesterol is made or taken in diet? where in the body is it made?
majority is made in body all nucleated cells can synthesize cholesterol in the ER and cytosol
58
What is HDL-C
scavenger to lwoer serum cholesterol
59
what is LDL-C
transporter for cholesterol from liver to peripheral tissues
60
what is HDL
transporter of cholesterol from peripheral tissues to liver for degradation
61
What can prolonged elevated levels of cholesterol in plasma lead to
artherosclerosis DM
62
What are statins
act as competitive inhibitors of HMG-CoA reductase so that it cannot be converted to mevalonate so inhibiting cholesterol biosynthesis
63
what is niacin
vit B3 inhibits release of FFA from adipose decreaseing VLDL synthesis and inhibting secretion so decreased LDL production and increased HDL by unknown mechanism
64
what are fibrates
lower plasma TGL by decreased secretion of TGL and VLDL by liver
65
what are ezetimibes
reduce blood cholesterol levels by inhibiting absorption of cholesterol by intestine names: setia or ezetrol
66
What are resins
bile acid sequestrants promote excretion of bile acids in stool diverts cholesterol to bil acid synthesis up regulates LDL R and enhanced LDL clearance from plasma names: cholestyramine, colestipol and colesevelam

Year 2 CV (31 decks)

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