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Final FA review > 4/10&11 biochem/overall > Flashcards

4/10&11 biochem/overall Flashcards

1
Q

I—hyper-chylomicronemia

-whats the problem?

A

LPL deficiency or altered ApoC-II.

*ApoC-II = LPL cofactor.

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2
Q

I—hyper-chylomicronemia

-Sxs:

A

Causes pancreatitis, hepa tosplenomegaly, and eruptive/pruritic xanthomas.

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3
Q

I—hyper-chylomicronemia

  • what will blood lipid content be like?
  • is there inc. risk for atherosclerosis?
A

TG levels in chylos & VLDL wont drop.

  • TGs can get as high as 1600 - may cause acute pancreatitis.
  • excess TGs in blood can make it look milky white.
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4
Q

IIa—familial hyper-cholesterolemia

  • whats the problem?
  • what will inc. in blood content?
A
  • Absent or defectiveLDL receptors.
  • extremely high cholesterol (LDL has lots of cholesterol).
  • heterozygous ~ 300mg/dL
  • homozygous ~ 700mg/dL
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5
Q

IIa—familial hyper-cholesterolemia

-inheritance pattern?

A

-auto-dom.

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6
Q

IIa—familial hyper-cholesterolemia

-Sxs:

A

-Causes accelerated atherosclerosis (may have MI before

age 20), tendon (Achilles) xanthomas, and corneal arcus.

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7
Q

tendon (Achilles) xanthoma classically seen in which dyspilidemia?

A

IIa—familial hyper-cholesterolemia

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8
Q

IV—hyper-triglyceridemia

  • whats the problem?
  • inheritance pattern?
A

Hepatic overproduction of VLDL. Causes pancreatitis.

*Autosomal dominant.

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9
Q

Abetalipoproteinemia

-whats the problem?

A

Dec. synthesis of apolipoprotein B = inability to generate chylomicrons. Dec. secretion of cholesterol, VLDL into bloodstream =Ž fat accumulation in enterocytes.

  • ApoB-100 binds LDL receptor.
  • ApoB-48 req to send chylos out of intestines.
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10
Q

Abetalipoproteinemia

-blood lipid content?

A

-very low plasma TG & chol levels. Chylomicrons, VLDL, apoB = absent from blood.

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11
Q

Abetalipoproteinemia

  • Sxs:
  • presentation:
A

malabsorption, acanthocytes, neuro-problems.

-Presents in early childhood with failure to thrive, steatorrhea, acanthocytosis, ataxia, night blindness.

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12
Q

b-HCG level in ectopic pregnancy

-inc/dec/normal?

A

Lower than expected rise based on dates.

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13
Q

Connection btwn hypothyroidism & amenorrhea?

A
  • hypothyroidism leads to high TRH levels bc of low neg. feedback.
  • TRH stimulates PRL release.
  • PRL inhibits GnRH.
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14
Q

Relationship btwn TRH, PRL, GnRH?

A

TRH stimulates PRL release.

-PRL inhibts GnRH.

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15
Q

External hemorrhoid

-venous drainage below pectinate line?

A

inferior rectal vein => internal pudendal vein => internal iliac vein => IVC.

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16
Q

Loop diuretics

  • tox:
  • mnemonic:
A

OH DANG!

Ototoxicity, Hypokalemia, Dehydration, Allergy (sulfa), Nephritis (interstitial), Gout.

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17
Q

glipizide

  • what is it?
  • tox?
A

sulfonylurea

-hypoglycemia.

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18
Q

HELLP syndrome

  • whats HELLP stand for?
  • what type of cells will be seen in peripheral blood?
A

Hemolysis, Elevated Liver enzymes, Low Platelets.
-thrombotic microangiopathy in liver
-RBCs get sheared as they pass by these micro-clots
in the liver -> schistocytes.

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19
Q

Obsessive compulsive disorder

-Tx:

A

SSRIs, clomipramine.

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20
Q

How would b-HCG cause gynecomastia?

A

Acts like LH on leydig cells => makes testosterone => aromatized to estrogen.

(is this right?)

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21
Q

Which cells make testosterone?

A

Leydig cell, NOT sertoli cells!

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22
Q

3rd degree heart block

-Tx:

A

pacemaker

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23
Q

Leser-Trélat sign

  • sudden appearance of multiple what?
  • indicates what?
A
  • seborrheic keratoses.

- indicating an underlying malignancy (e.g., GI, lymphoid).

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24
Q

babesia vs malaria

-difference in appearance in RBC?

A

babesia has the maltese cross.

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25
Q

Aortic dissection

-can it cause unequal BP in UEs?

A

-yes, can cause markedly unequal BP in arms.

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26
Q

hemibalism

-ipsi or contralateral effects?

A

contra

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27
Q

Cerebellum

-does it get its info from ipsi or contra cortex?

A

Contra cerebrum via middle cerebellar peduncle.

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28
Q

Cerebellum

-does it get its proprioception from ipsi or contra spinal cord?

A

-ipsi spinal cord via inferior cerebellar peduncle.

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29
Q

Cerebellum: lateral lesion

-propensity to fall toward contra or ipsi side?

A

-you fall toward ipsi side (injured side).

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30
Q

rosacea

  • define:
  • is it painful?
A

Chronic skin condition characterized by facial redness, small and superficial dilated blood vessels on facial skin, papules, pustules, and swelling.
-can be painful.

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31
Q

strep pneumo & viridans

-is it hemolytic?

A

yes. alpha-hemolytic.

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32
Q

lichen sclerosus

  • what is it?
  • whos most at risk?
A
  • patchy, white skin that’s thinner than normal.
  • most often involves skin of the vulva, foreskin of the penis or skin around the anus.
  • post-menopausal women most at risk.
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33
Q

Struvite stones

-aka?

A

AMP

-Ammonium magnesium phosphate.

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34
Q

1st sign of puberty in girl?

A

-breast buds form (thelarche)

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35
Q

1st sign of puberty in boy?

A

-enlargement of the testicles and sprouting of pubic hair

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36
Q

Anti-social vs schizoid personality disorder

-whats the diff?

A

antisocial = psychopath.

  • no where near the same definition.
  • dont think anti-social as just not hanging out with people, it means disregard for and violation of rights of others, criminality, impulsivity.
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37
Q

Necrotizing enterocolitis

-happens in which pt pop?

A

neonates, usually preemies.

  • perforation can lead to sepsis & death.
  • can see air in bowel wall.
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38
Q

Which coag factor has shortest half life?

A

7

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39
Q

Which RNA viruses replicate in the nucleus?

A

orthomyxoviridae & retroviruses.

40
Q

Which DNA virus replicates in cytoplasm?

A

poxvirus.

41
Q

Clinical trials

  • whats phase 1?
  • what question are they answering?
  • who are the volunteers?
A

“is it safe”?

-Small number of healthy volunteers.

42
Q

Pleural Effusion:

  • breath sounds:
  • percussion:
  • fremitus:
A
  • breath sounds: dec.
  • percussion: dull
  • fremitus: dec
43
Q

Atelectasis (bronchial obstruction)

  • breath sounds:
  • percussion:
  • fremitus:
A
  • breath sounds: dec.
  • percussion: dull
  • fremitus: dec.

*-theres a vaccum there, not air. So its dull. Sound dont travel through a vacuum.

44
Q

Spont. pneumothorax

  • breath sounds:
  • percussion:
  • fremitus:
A
  • breath sounds: dec.
  • percussion: hyperresonant
  • fremitus: dec.
45
Q

Tension pneumothorax

  • breath sounds:
  • percussion:
  • fremitus:
A
  • breath sounds: dec
  • percussion: hyperresonant
  • fremitus: dec
46
Q

Consolidation (lobar pneumonia, pulm. edema)

  • breath sounds:
  • percussion:
  • fremitus:
A
  • breath sounds: bronchial breath sounds, late inspiratory crackles.
  • percussion: dull
  • fremitus: inc.
  • only one w/inc breath sounds or inc. fremitus!
  • also gets egophany (E to A sign).
47
Q

What shape are amyloid proteins in?

A

b-pleated sheet structures.

48
Q

If mean is 500 and SD = 100.

-67% of the people are in what range?

A

-67% are within 100 above and 100 below the mean, so 400-600.
So when it says that 68% of everyone is within 1 standard deviation - that means 1 sd above the mean and 1 sd below the mean.

49
Q

Mucopolysaccharidoses

  • name the 2 in this class
  • which one has corneal clouding?
  • mnemonic?
A
  • Hunter & Hurler syndromes.
  • Hurler has corneal clouding.
  • Hunters see clearly (no corneal clouding) and aggressively aim for the X (X-linked recessive).
50
Q

Fabry disease

  • enzyme deficiency?
  • what builds up?
A
  • α-galactosidase A

- Ceramide trihexoside

51
Q

Fabry disease

  • Sxs:
  • mnemonic:
  • do pts die from this?
A
  • Peripheral neuropathy of hands/feet, angiokeratomas, cardiovascular/renal disease.
  • angiokeratoma is a FABRic of vessels.
  • most pts will die even w/enzyme sup.
52
Q

Gaucher disease

  • which letter?
  • whats the menmonic?
A
  • U

- gaUcher: glUcocerebrosidase: glUcocerebroside: hUge organs: U survive: crUnched up paper.

53
Q

Gaucher disease

-presentation?

A
  • this pt will be older, maybe 30 years old.
  • can present w/chronic pain in extremities & back. Anemia, Hepatosplenomegaly, pancytopenia, aseptic necrosis of femur, bone crises.
54
Q

Lysosomal storage disease. They are all auto-recessive except which two? which are what inheritance?

A

Fabry & Hunter = X linked recessive.

55
Q

Niemann-Pick disease

  • which letter?
  • whats the menmonic?
A
  • I

- nIemann-pick: sphIngomyelinase: sphIngomyelin: bIg organs: lIpid-laden macro.

56
Q

Niemann-Pick disease

-presentation?

A
  • infants will have period of normal development, then lose previously acquired motor skills.
  • death before 3yo = profound neurological problems.
57
Q

Which lysosomal storage diseases have cherry-red macula?

-mnemonic?

A

Cherry-red macula in diseases w/hyphen in their name.

  • Tay-Sachs.
  • Niemann-Pick.
58
Q

Tay-Sachs disease

  • which letter?
  • whats the menmonic?
A
  • A

- tAy-sAchs: hex-A: gAngliosides: chArry-red mAcula: brAin.

59
Q

Tay-Sachs disease

-presentation?

A
  • infants will have period of normal development, then lose previously acquired motor skills.
  • death before 2yo = profound neurological problems.
60
Q

Niemann-Pick vs Tay-Sachs

-based just on presentation, whats the difference?

A

Niemann-Pick has hepatosplenomegaly, while Tay-Sachs has no organomegaly.

61
Q

Krabbe disease

  • Sxs:
  • characteristic cell?
A

Peripheral neuropathy, developmental delay, optic atrophy.

-globoid cells

62
Q

Krabbe disease

  • enzyme deficiency?
  • what builds up?
A
  • Galactocerebrosidase
  • Galactocerebroside, psychosine
  • buildup of these destroys the myelin sheath.
63
Q

Metachromatic leukodystrophy

  • Sxs:
  • do pts die from this?
A
  • Central and peripheral demyelination with ataxia, dementia.
  • death by early childhood.
64
Q

Metachromatic leukodystrophy

  • enzyme deficiency?
  • what builds up?
A
  • Arylsulfatase A

- Cerebroside sulfate

65
Q

Hurler syndrome

  • enzyme deficiency?
  • what builds up?
A
  • α-l-iduronidase.

- Heparan sulfate, dermatan sulfate.

66
Q

Hurler syndrome

-Sxs:

A

-Developmental delay, gargoylism, airway obstruction, corneal clouding, hepatosplenomegaly.

67
Q

Hunter syndrome

  • enzyme deficiency?
  • what builds up?
A
  • Iduronate sulfatase.

- Heparan sulfate, dermatan sulfate.

68
Q

Hunter syndrome

-Sxs:

A

Mild Hurler + aggressive behavior, no corneal clouding.

69
Q

Naive T-cell activation

-what are the 2 signals?

A
  • MHC I or II loaded w/Ag; TCR.
  • B7 (on APC); CD28 (on T cell).

*T cell now activated. Either CD8 or CD4 T cell, depending on if it was MHC I or II.

70
Q 
B-cell activation & class switching
-what are the 2 signals?
A
  • MHC II w/Ag; Th cell TCR.
  • CD40 receptor; CD40 ligand on Th cell.

*Th cell secretes cytokines that determine Ig class switching of B cell. B cell activates and undergoes class switching, affinity maturation, and antibody production.

71
Q

Why are Th2 cells needed in the germinal center when B-cells are undergonig affinity maturatino & class switching?

A
  • you need the IL-4 and IL-5 from Th2 cells to undergo class switching. So you need Th2 cells in that germinal center as well.
  • You need the Th2 cells to interact w/B cells in order to get that germinal center.
  • If you have Hyper IgM syndrome, no germinal centers are made.
72
Q

CD8 T cells

-what cytotoxic granules do they release?

A
  • perforin—helps to deliver the content of granules into target cell.
  • granzyme B—a serine protease, activates apoptosis inside target cell.
  • granulysin—antimicrobial, induces apoptosis.
73
Q

Cyclosporine

  • whats it bind?
  • whats it block?
A
  • Calcineurin inhibitor; binds cyclophilin.

- Blocks T cell activation by preventing IL-2 transcription.

74
Q

calcineurin inhibitors:

-what do u need to watch out for when using these?

A

Watch out for reactivation of TB!

75
Q

Cyclosporine

-notable toxicity?

A

Nephrotoxicity, hirsutism, gingival hyperplasia.

-hypertension, hyperlipidemia, hyperglycemia, tremor.

76
Q

gingival hyperplasia

  • which drugs can cause this?
  • mnemonic?
A

Phenytoin, verapamil, nifedipine, cyclosporine.

*Poor Vera Never Cums (bc of her nasty gingival hyperplasia).

77
Q

Name the 2 calcineurin inhibitors:

-what toxicity do they have in common?

A
  • cyclosporine, tacrolimus.

- both are highly nephrotoxic.

78
Q

Tacrolimus

  • whats it bind?
  • whats it block?
A
  • Calcineurin inhibitor; binds FK506 binding protein (FKBP).

- Blocks T cell activation by preventing IL-2 transcription.

79
Q

Tacrolimus

-notable tox:

A

-nephrotoxicity, inc. risk of diabetes, and neurotoxicity.

80
Q

Sirolimus (Rapamycin)

  • why use this instead of cyclosporine or tacrolimus?
  • mnemonic?
A
  • Kidney transplant rejection prophylaxis = so you obviously dont want to use an immunosuppressant that is nephrotoxic!
  • Kidney “sir-vives.”
81
Q

Sirolimus (Rapamycin)

-mechanism?

A
  • mTOR inhibitor; binds FKBP.
  • IL-2R receptor binds IL-2 and mTOR pathway gets activated, its a cellular proliferation pathway
  • Blocks T cell activation and B cell differentiation by preventing IL-2 signal transduction.
82
Q

Basiliximab

  • mech:
  • use?
A
  • Monoclonal antibody; blocks IL-2R.

- Kidney transplant rejection prophylaxis.

83
Q

mTOR pathway

  • what activates it?
  • whats it do/
A
  • IL-2R receptor binds IL-2 and mTOR pathway gets activated, its a cellular proliferation pathway.
  • causes proliferation of Helper T cells.
84
Q

Alemtuzumab

  • target?
  • use?
  • mnemonic?
A
  • CD52
  • CLL
  • “Alymtuzumab”—chronic lymphocytic leukemia.
85
Q

Bevacizumab

  • target?
  • use?
A
  • VEGF

- Colorectal cancer, renal cell carcinoma.

86
Q

Infliximab, adalimumab

  • target?
  • mnemonic?
A
  • TNF-α

- Rheumatoid arthritis “inflix” pain in “da limbs”.

87
Q

Denosumab

  • target?
  • use?
  • mnemonic?
A
  • RANKL
  • Osteoporosis; inhibits osteoclast maturation (mimics
    osteoprotegrin) .
  • Deno like “Dino”. Dinosaur “bones”. Dinosaurs are old, old people get bone problems, osteoporosis.
88
Q

Omalizumab

  • target?
  • use?
A
  • IgE

- Allergic asthma; prevents IgE binding to FceRI.

89
Q

Palivizumab

  • use:
  • mnemonic:
A
  • RSV prophylaxis for high-risk infants.

- PaliVIzumab—VIrus

90
Q

Cushing syndrome

-associated w/which cancer?

A

small cell carcinoma of lung

-can release ACTH.

91
Q

Dermatomyositis

-associated w/which cancer?

A

lung cancer

92
Q

Plummer-Vinson syndrome

-associated w/which cancer?

A

Squamous cell carcinoma of esophagus.

93
Q

BRAF

  • what does it code?
  • which cancer?
A
  • Serine/threonine kinase

- melanoma.

94
Q

L-myc

-which cancer?

A

Lung tumor

95
Q

ras

  • codes for what?
  • which cancer?
A
  • GTPase

- Colon cancer, lung cancer, pancreatic cancer.

96
Q

What happens to cells that dont express MHC-1?

A

Tumor cells are mutated and will express abnormal proteins. These abnormal proteins will be expressed on MHC-1, recognized by CD8 T cell, and killed. So tumor cells evade this by downregulating expression of MHC-1

-Cells that dont express MHC-1 get attacked by NK cells.

Final FA review (52 decks)

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