4/10&11 biochem/overall Flashcards Preview

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Flashcards in 4/10&11 biochem/overall Deck (96):
1

I—hyper-chylomicronemia
-whats the problem?

LPL deficiency or altered ApoC-II.
*ApoC-II = LPL cofactor.

2

I—hyper-chylomicronemia
-Sxs:

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

3

I—hyper-chylomicronemia
-what will blood lipid content be like?
-is there inc. risk for atherosclerosis?

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.

4

IIa—familial hyper-cholesterolemia
-whats the problem?
-what will inc. in blood content?

-Absent or defective LDL receptors.
-extremely high cholesterol (LDL has lots of cholesterol).
-heterozygous ~ 300mg/dL
-homozygous ~ 700mg/dL

5

IIa—familial hyper-cholesterolemia
-inheritance pattern?

-auto-dom.

6

IIa—familial hyper-cholesterolemia
-Sxs:

-Causes accelerated atherosclerosis (may have MI before
age 20), tendon (Achilles) xanthomas, and corneal arcus.

7

tendon (Achilles) xanthoma classically seen in which dyspilidemia?

IIa—familial hyper-cholesterolemia

8

IV—hyper-triglyceridemia
-whats the problem?
-inheritance pattern?

Hepatic overproduction of VLDL. Causes pancreatitis.
*Autosomal dominant.

9

Abetalipoproteinemia
-whats the problem?

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.

10

Abetalipoproteinemia
-blood lipid content?

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

11

Abetalipoproteinemia
-Sxs:
-presentation:

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

12

b-HCG level in ectopic pregnancy
-inc/dec/normal?

Lower than expected rise based on dates.

13

Connection btwn hypothyroidism & amenorrhea?

-hypothyroidism leads to high TRH levels bc of low neg. feedback.
-TRH stimulates PRL release.
-PRL inhibits GnRH.

14

Relationship btwn TRH, PRL, GnRH?

TRH stimulates PRL release.
-PRL inhibts GnRH.

15

External hemorrhoid
-venous drainage below pectinate line?

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

16

Loop diuretics
-tox:
-mnemonic:

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

17

glipizide
-what is it?
-tox?

sulfonylurea
-hypoglycemia.

18

HELLP syndrome
-whats HELLP stand for?
-what type of cells will be seen in peripheral blood?

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

19

Obsessive compulsive disorder
-Tx:

SSRIs, clomipramine.

20

How would b-HCG cause gynecomastia?

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

(is this right?)

21

Which cells make testosterone?

Leydig cell, NOT sertoli cells!

22

3rd degree heart block
-Tx:

pacemaker

23

Leser-Trélat sign
-sudden appearance of multiple what?
-indicates what?

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

24

babesia vs malaria
-difference in appearance in RBC?

babesia has the maltese cross.

25

Aortic dissection
-can it cause unequal BP in UEs?

-yes, can cause markedly unequal BP in arms.

26

hemibalism
-ipsi or contralateral effects?

contra

27

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

Contra cerebrum via middle cerebellar peduncle.

28

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

-ipsi spinal cord via inferior cerebellar peduncle.

29

Cerebellum: lateral lesion
-propensity to fall toward contra or ipsi side?

-you fall toward ipsi side (injured side).

30

rosacea
-define:
-is it painful?

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

31

strep pneumo & viridans
-is it hemolytic?

yes. alpha-hemolytic.

32

lichen sclerosus
-what is it?
-whos most at risk?

-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.

33

Struvite stones
-aka?

AMP
-Ammonium magnesium phosphate.

34

1st sign of puberty in girl?

-breast buds form (thelarche)

35

1st sign of puberty in boy?

-enlargement of the testicles and sprouting of pubic hair

36

Anti-social vs schizoid personality disorder
-whats the diff?

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.

37

Necrotizing enterocolitis
-happens in which pt pop?

neonates, usually preemies.
-perforation can lead to sepsis & death.
-can see air in bowel wall.

38

Which coag factor has shortest half life?

7

39

Which RNA viruses replicate in the nucleus?

orthomyxoviridae & retroviruses.

40

Which DNA virus replicates in cytoplasm?

poxvirus.

41

Clinical trials
-whats phase 1?
-what question are they answering?
-who are the volunteers?

"is it safe"?
-Small number of healthy volunteers.

42

Pleural Effusion:
-breath sounds:
-percussion:
-fremitus:

-breath sounds: dec.
-percussion: dull
-fremitus: dec

43

Atelectasis (bronchial obstruction)
-breath sounds:
-percussion:
-fremitus:

-breath sounds: dec.
-percussion: dull
-fremitus: dec.

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

44

Spont. pneumothorax
-breath sounds:
-percussion:
-fremitus:

-breath sounds: dec.
-percussion: hyperresonant
-fremitus: dec.

45

Tension pneumothorax
-breath sounds:
-percussion:
-fremitus:

-breath sounds: dec
-percussion: hyperresonant
-fremitus: dec

46

Consolidation (lobar pneumonia, pulm. edema)
-breath sounds:
-percussion:
-fremitus:

-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

What shape are amyloid proteins in?

b-pleated sheet structures.

48

If mean is 500 and SD = 100.
-67% of the people are in what range?

-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

Mucopolysaccharidoses
-name the 2 in this class
-which one has corneal clouding?
-mnemonic?

-Hunter & Hurler syndromes.
-Hurler has corneal clouding.
*Hunters see clearly (no corneal clouding) and aggressively aim for the X (X-linked recessive).

50

Fabry disease
-enzyme deficiency?
-what builds up?

-α-galactosidase A
-Ceramide trihexoside

51

Fabry disease
-Sxs:
-mnemonic:
-do pts die from this?

-Peripheral neuropathy of hands/feet, angiokeratomas, cardiovascular/renal disease.
-angiokeratoma is a FABRic of vessels.
*most pts will die even w/enzyme sup.

52

Gaucher disease
-which letter?
-whats the menmonic?

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

53

Gaucher disease
-presentation?

-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

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

Fabry & Hunter = X linked recessive.

55

Niemann-Pick disease
-which letter?
-whats the menmonic?

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

56

Niemann-Pick disease
-presentation?

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

57

Which lysosomal storage diseases have cherry-red macula?
-mnemonic?

Cherry-red macula in diseases w/hyphen in their name.
-Tay-Sachs.
-Niemann-Pick.

58

Tay-Sachs disease
-which letter?
-whats the menmonic?

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

59

Tay-Sachs disease
-presentation?

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

60

Niemann-Pick vs Tay-Sachs
-based just on presentation, whats the difference?

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

61

Krabbe disease
-Sxs:
-characteristic cell?

Peripheral neuropathy, developmental delay, optic atrophy.
-globoid cells

62

Krabbe disease
-enzyme deficiency?
-what builds up?

-Galactocerebrosidase
-Galactocerebroside, psychosine
*buildup of these destroys the myelin sheath.

63

Metachromatic leukodystrophy
-Sxs:
-do pts die from this?

-Central and peripheral demyelination with ataxia, dementia.
-death by early childhood.

64

Metachromatic leukodystrophy
-enzyme deficiency?
-what builds up?

-Arylsulfatase A
-Cerebroside sulfate

65

Hurler syndrome
-enzyme deficiency?
-what builds up?

-α-l-iduronidase.
-Heparan sulfate, dermatan sulfate.

66

Hurler syndrome
-Sxs:

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

67

Hunter syndrome
-enzyme deficiency?
-what builds up?

-Iduronate sulfatase.
-Heparan sulfate, dermatan sulfate.

68

Hunter syndrome
-Sxs:

Mild Hurler + aggressive behavior, no corneal clouding.

69

Naive T-cell activation
-what are the 2 signals?

-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

B-cell activation & class switching
-what are the 2 signals?

-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

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

-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

CD8 T cells
-what cytotoxic granules do they release?

-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

Cyclosporine
-whats it bind?
-whats it block?

-Calcineurin inhibitor; binds cyclophilin.
-Blocks T cell activation by preventing IL-2 transcription.

74

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

Watch out for reactivation of TB!

75

Cyclosporine
-notable toxicity?

Nephrotoxicity, hirsutism, gingival hyperplasia.

-hypertension, hyperlipidemia, hyperglycemia, tremor.

76

gingival hyperplasia
-which drugs can cause this?
-mnemonic?

Phenytoin, verapamil, nifedipine, cyclosporine.
*Poor Vera Never Cums (bc of her nasty gingival hyperplasia).

77

Name the 2 calcineurin inhibitors:
-what toxicity do they have in common?

-cyclosporine, tacrolimus.
-both are highly nephrotoxic.

78

Tacrolimus
-whats it bind?
-whats it block?

-Calcineurin inhibitor; binds FK506 binding protein (FKBP).
-Blocks T cell activation by preventing IL-2 transcription.

79

Tacrolimus
-notable tox:

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

80

Sirolimus (Rapamycin)
-why use this instead of cyclosporine or tacrolimus?
-mnemonic?

-Kidney transplant rejection prophylaxis = so you obviously dont want to use an immunosuppressant that is nephrotoxic!
*Kidney “sir-vives.”

81

Sirolimus (Rapamycin)
-mechanism?

-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

Basiliximab
-mech:
-use?

-Monoclonal antibody; blocks IL-2R.
-Kidney transplant rejection prophylaxis.

83

mTOR pathway
-what activates it?
-whats it do/

*IL-2R receptor binds IL-2 and mTOR pathway gets activated, its a cellular proliferation pathway.
-causes proliferation of Helper T cells.

84

Alemtuzumab
-target?
-use?
-mnemonic?

-CD52
-CLL
-“Alymtuzumab”—chronic lymphocytic leukemia.

85

Bevacizumab
-target?
-use?

-VEGF
-Colorectal cancer, renal cell carcinoma.

86

Infliximab, adalimumab
-target?
-mnemonic?

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

87

Denosumab
-target?
-use?
-mnemonic?

-RANKL
-Osteoporosis; inhibits osteoclast maturation (mimics
osteoprotegrin).
-Deno like "Dino". Dinosaur "bones". Dinosaurs are old, old people get bone problems, osteoporosis.

88

Omalizumab
-target?
-use?

-IgE
-Allergic asthma; prevents IgE binding to FceRI.

89

Palivizumab
-use:
-mnemonic:

-RSV prophylaxis for high-risk infants.
-PaliVIzumab—VIrus

90

Cushing syndrome
-associated w/which cancer?

small cell carcinoma of lung
-can release ACTH.

91

Dermatomyositis
-associated w/which cancer?

lung cancer

92

Plummer-Vinson syndrome
-associated w/which cancer?

Squamous cell carcinoma of esophagus.

93

BRAF
-what does it code?
-which cancer?

-Serine/threonine kinase
-melanoma.

94

L-myc
-which cancer?

Lung tumor

95

ras
-codes for what?
-which cancer?

-GTPase
-Colon cancer, lung cancer, pancreatic cancer.

96

What happens to cells that dont express MHC-1?

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.