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BECOM 1 > BECOM Exam #4 > Flashcards

BECOM Exam #4 Flashcards

1
Q

Cystinuria

A

Poor resorption of acidic AAs, high Cys in urine, kidney stones; treated by urinary alkalinization with citrate or acetazolamide, dietary salt & Met restriction.

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2
Q

Cystinosis

A

(Pediatric)Lysosomal accumulation of cystine (oxidized cysteine); treated with cysteamine to form cysteamine-Cys for excretion. Can lead to Fanconi syndrome.

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3
Q

Homocysteinuria

A

Defects in CbS (usually). Displacement of lens (down and in)*, cognitive defects (variable), long limbs, increased thrombosis

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4
Q

Sideroblastic anemia

A 
Enough iron but it isn’t incorporated due to 
problems with red cell maturation.  
-Serum Iron: High
-Increased ferritin levels
-High transferrin saturation
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5
Q

microcytic hypochromic

A

-nucleated RBC

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6
Q

low stomach acid

A

low Fe absorption

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7
Q

pregnant women Fe levels

A

Brain mitochondrial Fe-S proteins

Fetal brain development

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8
Q

Causes of microcytic

anemias

A

thalassemia
Fe def
chronic disease
sideroblastic anemia

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9
Q

Fe def anemia

A

often associated with chronic blood loss

  • low iron and ferritin
  • high TIBC
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10
Q

Thalassemia anemia

A

normal Fe serum

-high iron and ferritin

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11
Q

A high UIBC, TIBC, or transferrin normally indicates

A

iron deficiency

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12
Q

hemochromatosis

A

Too much Fe

-High serum ferritin

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13
Q

non essential AA

A

alanine, aspartate, cysteine, glutamate

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14
Q

Ketogenic AA

A

leucine and lysine

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15
Q

ALT and AST cofactor

A

B6 (plp) (pyridoxal phosphate)

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16
Q

Non oxidative deamination enzymes

A

threonine, serine, cysteine, histidine

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17
Q

Ammonia as neurotoxin

A

high NH3+, high glutamate, low glutamate, low alpha ketoglutarate, low atp

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18
Q

NH3+ + HCO- = Carbonyl phosphate enzyme and cofactor

A

CPS-1 (NAG)

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19
Q

Urea amine donation

A

first: aspartate second: glutamate

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20
Q

Defects of urea cycle & treatment

A

phenylbutyrate (glutamate + NH3+ = glutamine ->

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21
Q

Maple syrup urine disease

A
  • branched chain amino acids

- defective Branched chain alpha ketoacid dehydrogenase

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22
Q

Maple syrup urine disease amino acids

A

-Leu, Ile & Val

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23
Q

Phenylketonuria (PKU)

A

Deficiency of phenylalanine hydroxylase

-PKU may be mimicked by deficiencies of enzymes that synthesize tetrahydrobiopterin (BH4), the PAH cofactor

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24
Q

Alkaptonuria

A

Mutation in homogentisic acid oxidase

-Homogentisic aciduria

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25
Q

Tyrosinemias

A

enzyme mutation in phenylalanine pathway

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26
Q

Folate and babies

A

High folate may mask peripheral indications of B12
deficiency, allow cryptic CNS effects

“Unmetabolized” folic acid may interfere with DHFR,
MTHFR

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27
Q

anti-folates (e.g. methotrexate) block

A

DHFR
-Note: Blocking DHFR  inhibit purine and thymidine synthesis, slow rapid cell division. Can cause megaloblastic anemia.

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28
Q

Fluorodeoxy-uracil

A

TS (dUMP->dTMP

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29
Q

main source of carbon for Folate cycle

A

serine

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30
Q

B12 used by 2 mammal enzymes

A

MS & methyl malonyl CoA-
Succinyl CoA mutase (uses
adenosyl cobalamin)
-Deficiency -> Methyl malonic acid accumulation, weakens myelin. Methyl malonic acidemia

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31
Q

Transsulfuration pathway enzymes

A

CBS (B6), CyL (B6)

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32
Q

RBC glycolysis specific pathway

A
  • NADPH -> Fe3+ -> Fe2+ (metHb)

- 1,3 BPG -> 2,3 BPG

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33
Q

1,3 BPG -> 2,3 BPG enzyme

A

bisphosphoglycerate mutase

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34
Q

microcytic, hypochromic

A
  • Fe def.
  • Thalassemia
  • Lead poisoning
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35
Q

macrocytic, normochromic

A
  • B12/Folate acid def

- erythroleukemia

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36
Q

Normocytic, normochromic

A

acute bleeding
sickle cell
metabolic defects

37
Q

All carbons and nitrogen in the porphyrin ring are derived from

A

glycine and succinyl CoA (all nitrogens from glycine).

38
Q

Heme synthesis enzymes and cofactors

A

ala syntheses (B6), ala dyhydratase (lead inhibits)

39
Q

acute porphyria

A

characterized by episodic acute neurovisceral attacks

40
Q

non acute porphyria

A

characterized by light-sensitive lesions

41
Q

van den Bergh’s test

A
  • Total bilirubin = conjugated (direct) + unconjugated (indirect)
  • Total bilirubin - unconjugated bilirubin (indirect bilirubin) must be released from albumin by addition of caffeine-benzoate
42
Q

If direct (i.e. conjugated) bilirubin is normal, then the problem is

A

an excess of unconjugated bilirubin, and the location of the problem is upstream of bilirubin excretion

43
Q

If direct bilirubin is elevated,

A

then the liver is conjugating bilirubin normally, but is not able to excrete it. Bile duct obstruction should be suspected.

44
Q

Beta-lactam antibiotics (penicillins, cephalosporins, carbapenams) target which offollowing bacterial components which ultimately results in destruction of the bacterium?

A

prevents peptidoglycan from cross-linking properly in the last stages of bacterial cell wall synthesis

45
Q

High transferrin means

A

low iron (deficiency)

46
Q

Hepcidin

A

more will degrade ferroportin

will take up less Fe

47
Q

Ferritin

A

binds Fe3+ in cell

48
Q

CD4

A

helper T cells

49
Q

CD8

A

cytotoxic T cells

50
Q

Adaptive cell

A

B T NK and antibodies

51
Q

lymph drainage

A

afferent, sub scapular sinus, cortex, paracortex, medullary sinus efferent

52
Q

primary lymph

A
  • made in bone marrow
  • B cells mature in bone marrow
  • thymus T cells mature
53
Q

secondary

A

where white blood cells meet APC

54
Q

Red pulp of spleen

A

sinuses filled with MΦ, erythrocytes, & dense network of reticular fibers (reticuloendothelial system is an old term that refers to a dense network of phagocytic cells, predominantly MΦ, in reticular connective tissues); this is where old, fragile RBCs are eliminated by splenic MΦ

55
Q

White pulp

A

dense lymphoid tissue; lymphocytes, DCs, MΦ surrounding small branches of splenic artery

56
Q

Marginal zone

A

dense lymphoid tissue; lymphocytes, DCs, MΦ surrounding small branches of splenic artery

57
Q

Marginal zone

A

surrounds outer perimeter of follicles & contains marginal zone B cells, which make T-independent responses (w/o T cell help) mainly to repetitive bacterial cell wall polysaccharide antigens—these MZ B cells underlie spleen’s effectiveness at opsonizing encapsulated bacteria & fungi; abundant splenic MΦ facilitate elimination of those opsonized pathogens.

58
Q

Periarteriolar lymphoid sheath (PALS)

A

Periarteriolar lymphoid sheath (PALS)—

59
Q

Hapten

A

molecule that can be recognized by immunoglobulins but incapable of eliciting an immune response by itself; can be coupled to protein carriers to induce immune response

60
Q

epitope and different types

A

the specific sequence on an antigen that binds to an antibody
-linear and discontinuous

61
Q

Characteristics of Pattern Recognition Receptors (PRRs):

A
  • Invariant.
  • Germ-line encoded.
  • Ever-present and expressed.
  • Recognize general structures that share certain characteristics, i.e. broad patterns, not very specific.
  • Protein subunits may oligomerize at varying levels & oligomerization can initiate downstream cell signaling.
62
Q

mast cells

A

contain histamine granules used during inflammation to cause vasodilation inc vascular permeability

63
Q

what cytokines does macrophages release?

A

IL-1 and TNF-a

64
Q

Th1

A

macrophages (intercellular pathogen)

65
Q

Th2

A

eosinophils (allergies and parasites)

66
Q

Th17

A

neutrophils (extracellular pathogen)

67
Q

G-CSF

A

neutrophils

68
Q

M-CSF

A

macrophages

69
Q

GM-CSF

A

dendritic cells

70
Q

IL-5

A

eosinophils

71
Q

where are macrophages and monocytes found

A
  • tissue

- circulation

72
Q

what cytokines do macrophage release

A

TNF-α, IL-1B, IL-12

73
Q

MCHI vs MCHII

A
  • all nucleated cells

- MCHII APC specific

74
Q

left shift

A

inc in immature neutrophils (band cells) due to

75
Q

Non-disease production fungi MAY cause disease in …. host

A

immunocompromised

76
Q

unicellular fungi

A

yeast

77
Q

multicellular fungi

A

(molds) which have long branching filaments called “hyphae”

78
Q

Masses of hyphae are referred to as

A

mycelium

79
Q

septate hyphae

A

crosswalls

80
Q

aseptate hyphae

A

no crosswalls

81
Q

Have cell walls primarily made of

A

chitin, glucan and mannan

82
Q

The sterols in the cell membrane are

A

egosterol

83
Q

in bacterial cell high glucose means

A

dec cAMP = low CAP

84
Q

in bacterial cell high low glucose means

A

inc cAMP = high CAP

85
Q

Conjugation

A

Donor cells transfer genetic information to a recipient cell

86
Q

Transformation

A

occurs when a recipient cell takes up a fragment of donor DNA from the surrounding growth medium

87
Q

Transduction

A

is the transfer of genetic material from a donor to a recipient cell by way of a bacteriophage

88
Q

Hfr

A

plasmid plus some chromosomal genes transferred

89
Q

F’

A

plasmid but cant donate

BECOM 1 (4 decks)

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