Bocock Uworld Week 4.5

Front 1

In this type of anemia, RBCs go through an extra division to maintain Hb concentration?

Back 1

microcytic anemia

Front 2

What causes microcytic anemia?

Back 2

a decrease in the production of Hb

Front 3

What is hemoglobin made of?

Back 3

Hb= Heme + globin

note: Heme= Fe + protoporphyrin

Front 4

What are the 4 causes of microcytic anemia?

Back 4

(1) Iron deficiency
(2) Anemia of chronic disease
(3) sideroblastic anemia
(4) thalessemia

Front 5

Absorption of this substance occurs in the duodenum?

Back 5

iron

Front 6

Key transporter used by enterocytes to transport iron across the CM into the blood?

Back 6

ferroportin

Front 7

Delivers iron to BM MACS and the liver via the blood?

Back 7

transferrin

Front 8

Measure of transferrin molecules in the blood?

Back 8

TIBC

Front 9

% of tranferrin molecules bound by iron?

Back 9

% saturation

Front 10

Used to measure iron in BM MACS and the liver

Back 10

ferritin

Front 11

Common causes of iron deficeincy anemia in the developing world?

Back 11

ancolystoma and necator

note: hookworm infections

Front 12

Having this procedure may cause a decrease in iron absorption b/c acid maintains iron in the absorbable FE2+ state?

Back 12

gastrectomy

Front 13

What lab finding will always be inversely proportional to ferritin levels?

Back 13

TIBC

note: these values are always inverses of one another

Front 14

Key stages of iron deficiency anemia?

Back 14

(1) storage iron is depleted
(2) serum iron is depleted
(3) normocytic anemia
(4) microcytic, hypochromic anemia

Front 15

Clincal findings include anemia+ spoon shaped nails+ PICA

Back 15

iron deficiency anemia

Front 16

Iron deficiency anemia + dysphasia+ a beefy red tongue

Back 16

plummer vinson syndrome

Front 17

MC type of anemia in hospitilized patients?

Back 17

anemia of chronic disease


Note: this is the inability to use stored iron

Front 18

Locks iron into cells and suppresses EPO production

Back 18

hepcidin

Front 19

Due to a defect in protoporphyrin synthesis and causes a microcytic anemia?

Back 19

sideroblastic anemia

Front 20

The RL step in the production of protoporphyrin?

Back 20

succinyl COA---> aminolevulinic acid

Note: ALA synthetase requires vit B6

Front 21

Attaches protoporphyrin to iron to make heme in the mitochondria?

Back 21

ferrochetalase

note: this enzyme is inhibited by lead poisoning

Front 22

If protoporphyrin is deficient, what happens to iron that is transported into the cell to make heme?

Back 22

it gets trapped in the mitochondria

note: this forms ringed sideroblasts

Front 23

These are formed because of iron filled mito that form rings around the nucleus of RBCs?

Back 23

ringed sideroblasts

Front 24

MC congenital form of sideroblastic anemia is due to a defect in what enzyme?

Back 24

ALA synthetase

note: converts succinyl COA to ALA

Front 25

Common causes of sideroblastic anemia

Back 25

alcoholism, lead poisoning, and B6 deficiency

Front 26

MC cause of vitamin B6 deficiency?

Back 26

isoniazid for the rx of TB

Front 27

What are thalessemias?

Back 27

decreases in the SYNTHESIS of globin chains

Front 28

Thalessemia carriers are protected against what parasite?

Back 28

plasmodium falciparum malaria

Front 29

Tyoe of hemoglobin seen on electrophoresis in alpha thalassemia where 3 genes are deleted?

Back 29

HbH

note: HbH is a tetramer of Beta chains

Front 30

Type of Hb that is made of a tetramer of gamma chains and causes hydrops fetalis?

Back 30

Hb Barts

Front 31

Name the normal types of Hb?

Back 31

HbF--> 2 alpha, 2 gamma

HbA--> 2 alpha, 2 beta

HbA2--> 2 alpha, 2 delta

Front 32

What are the mechanisms of the alpha and beta thalessemias?

Back 32

alpha--> gene deletions

beta--> point mutations

Front 33

Type of thalessemia where you have tetramers of alpha chains that damage RBCs?

Back 33

Beta-thalassemia major

Front 34

Results in the expansion of erythropoesis into the skull w/ "crew cut" appearance on x-ray...and into the facial bones leading to "chipmunk" facies

Back 34

beta thalassemia major and sickle cell disease

Front 35

MCC of macrocytic anemia...these substances are needed to make DNA precursors?

Back 35

Folate and Vit B12

note: this causes megaloblastic anemia

Front 36

Reason you get big cells with folate and vit B12 deficiencies?

Back 36

cannot make DNA precursors and cells cannot divide

Front 37

3 characteristics of macrocytic anemias caused by folate or Vit B 12 deficiencies?

Back 37

(1) megaloblastic anemia
(2) hypersegmented neutrophils
(3) megaloblastic change in rapidly diving cells

Front 38

This vitamin is absorbed in the jejunum?

Back 38

Vit B9-folate

Front 39

Drug that inhibits DHF reductase and can lead to a folate deficiency?

Back 39

methotrexate

Front 40

A decrease in folate or b12 leads to a buildup of this substance?

Back 40

homocysteine

Front 41

Key finding that differentiates folate deficiency from a VitB12 deficiency?

Back 41

folate deficiency--> will have normal levels of methmalonic acid in the blood

note: B12 is needed to convert methylmalonic acid to succinyl Coa

Front 42

Molecule that binds free Hb in the serum during intravascular hemolysis?

Back 42

haptoglobin

Front 43

Where is the vit B12-intrinsic factor complex absorbed?

Back 43

ileum

Front 44

MC cause of Vit B12 deficiency where there is autoimmmune destruction of parietal cells?

Back 44

pernicious anemia

Front 45

Damage to the terminal ileum by chron's disease or dipyllobothrium latum-fish tapeworm leads to a deficiency in what vitamin?

Back 45

Vitamin B12

Front 46

In what type of pt would you suspect to see vit B12 deficiency?

Back 46

vegans

Front 47

Why does vit B12 deficiency ylead to spinal cord degeneration?

Back 47

buildup of methylmalonic acid impairs SC myelination

note: you need vit B12 to convert MMA to succ Coa

Front 48

2 main causes of normocytic anemia?

Back 48

(1) peripheral destruction rbcs
(2) underproduction of rbcs by the BM

Front 49

A corrected reticulocyte count >3% shows good marrow response and indicates what?

Back 49

peripheral destruction of rbcs

Front 50

Tethering proteins that are commonly damaged in hereditary spherocytosis?

Back 50

spectrin, ankyrin, band 3.1

Front 51

Lab findings include spherocytes w/ a loss of central pallor, increase in RDW, and an increase in MCHC?

Back 51

hereditary spherocytosis

Front 52

Virus that can invade erythrocytic precursors and can lead to anaplastic crisis?

Back 52

parvovirus B19

Front 53

How is hereditary spherocytosis diagnosed?

Back 53

increased fragility on the osmotic fragility test

Note: cells swell and burst when placed in a hypotonic solution

Front 54

What does a howell jolly body indicate?

Back 54

splenic dysfuntion

Note: job of spleen is to remove extra nuclear material

Front 55

Presents with swollen hands and feet due to vaso-inclusive infarcts of bone in an AA infant?

Back 55

sickle cell anemia

Front 56

MCC of death in adults with sickle cell disease?

Back 56

acute chest syndrome

note: due to vaso-inclusion in the pulm microcirculation

Front 57

Test used to cause cells to sickle in suspected pts with sickle cell trait?

Back 57

metabisulfite screen

Front 58

What is characetristic of HbC on blood smear?

Back 58

HbC crystals

Front 59

What is glutamic acid replaced with on the beta chain in HbC disease?

Back 59

lysine

Front 60

Aquired defect resulting in absent glycosylphophotidylinositol (GPI) which renders RBCs susceptible to complement damage?

Back 60

paroxsymal nocturnal hemoglobinuria (PNS)

note: results in no DAF on cell membrane

Front 61

Flow cytometrycan be used to detect lack of CD55 (DAF) on rbcs in this disease?

Back 61

paroxysmal nocturnal hemoglobinuria

Front 62

PNH involves a defect in myeloid stem cells and is associated with this leukemia?

Back 62

AML

Front 63

In G6PDH deficiency you will see what two features b/c of oxidative stress?

Back 63

heinz bodies and bite cells

Front 64

IgG mediated immune hemolytic anemia is associated with what syndrome?

Back 64

SLE

Front 65

IgM mediated immune hemolytic anemia is associated with what 2 conditions?

Back 65

(1) mycoplasma pneumoniae
(2) infectious mononucleosis

Front 66

Test that confirms the presence of ab coated rbcs and is used to diagnose immune hemolytic anemia?

Back 66

direct coombs test

Front 67

Test used to confirm the presence of ab in pts SERUM w/ IHA?

Back 67

indirect coombs test

Front 68

biopsy reveals empty fat marrow?

Back 68

aplastic anemia

Front 69

Equation that explains the velocity of blood flow?

Back 69

V=Q/A

note: Q=BF, A=CSA

Front 70

What is the equation for blood flow?

Back 70

Q=changeP/R
or CO=MAP-RAP/TPR

Front 71

What is the equation for resistance?

Back 71

R=8nL/r^4

Front 72

The inverse of elastance or stiffness

Back 72

compliance

Front 73

What is the equation for compliance?

Back 73

C=V/P

Front 74

How can you calculate the MAP?

Back 74

diastolic Pressure+1/3 pulse pressure

Front 75

Estimates the LA pressure?

Back 75

pulmonary wedge pressure

Front 76

What is the reason that conduction velocity oss slowest at the AV node?

Back 76

this allows the ventricles to fill before contraction

Front 77

These effects are changes in conductioon velocity...primarily in the AV node

Back 77

dromotropic effects

Front 78

This parameter can be measured by the width of the pressure-volume loop?

Back 78

stroke volume

Front 79

causes the fourth heart sound?

Back 79

filling of the ventricle by atrial systole

Front 80

Causes the first heart sound?

Back 80

closure of the AV valves

Front 81

Causes the 2nd heart sound?

Back 81

closure of the semilunar valves

note: the pulm and aortic

Front 82

What is the starling equation that describes fluid exchange across capillaries?

Back 82

J=(Pc-Pi)-(IIc-IIi)


note: when J is positive...movement is out of the capillary

Front 83

What is the formula for ejection fraction?

Back 83

EF=SV/EDV

note: SV=EDV-ESV

Front 84

What is the pneumonic for EEG waveforms for sleep?

Back 84

"BATS Drink Blood"

Stage I=theta
stage 2=ss and k complexes
stage 3=delta
stage 4=beta