Bbc Segment 4

Front 1

Px presents with high arches, tremors, loss of proprioception

Back 1

Friedreich's ataxia

Front 2

describe guillain Barre syndrome
prognosis
findings?
associated with what bug?

Back 2

inflammation and demyelination of peripheral nerves and motor fibers of ventral roots. symmetric muscle weakness starting in distal lower extremites. facial paralysis in 50%

almost all survive
Increase CSF protein with normal cell count - albumin? and papilledema
Campy jejuni

Front 3

what is a neuroblastoma

Back 3

common tumor of children under 5.
from medulla of adrenals or sympathetic chain

Front 4

what thyroid dz has granulomas and comes after a viral illness?

what are the lab findings

Back 4

de quervains - elevated ESR, jaw pain, tender thyroid. may have low T3 but nothing clnically, and normal TSH

Front 5

name all the thyroid tumors

Back 5

papillary ca - young, after radiation thx, PSAMMOMA bodies
follicular - aggressive! and adenomatous
anaplastic - undifferentiated POOR prog
Medullary CA - Calcitonin!

Front 6

most common cause of hypoparathyroidism?
labs?

Back 6

accidental after thyroidectomy!
next is digeorge
Low PTH and low CA

Front 7

name reasons for hyperPTHism

Back 7

Primary - adenoma
2ndary - vit D def or chronic renal failure

Front 8

what is psuedohypoPTHism
lab values

Back 8

PTH receptor problem - short stature,
PTH elevated and Ca is LOW

Front 9

what is renal osteodystrophy?

Back 9

bone lesions due to 2ndary or tertiary hyperPTHism -
leads to excess phosphate uptake, binds to Ca, so you have hypocalcemia, high PTH and bone demineralization

Front 10

MEN2

Back 10

medullary Ca of thyroid(calcintonin) -100%
Pheo - 50 %
Primary hyperPTHism - 20%

Front 11

Old patient presents with flat greasy pigmented squamous lesions. looks pasted on.
pathology?

Back 11

Seborrheic keratosis - keratin filled cysts

Front 12

Px presents with honey colored crusts
what bug?

Back 12

impetigo
staph A or strep Pyogenes

Front 13

LEAD of lead poisoning

Back 13

Lead lines - gingiva(burtons) and long bones
Encephalitis and erythrocyte
Anemia and anemia(siderblastic)
Drop and Dimercaprol, EDTA and succimer

Front 14

what toxin can cause bladder cancer

Back 14

aniline and other aromatic amines

Front 15

what is the name of the acid fast stain

Back 15

ziehl nelson

Front 16

how does diptheria toxin work

Back 16

inhibits protein synthesis via ADP ribosylation of EF2

Front 17

what are the toxins of Staph A.

Back 17

alpha toxin - hemolysin, necrosis, cell death
TSST - cytokines to anaphylaxis

Front 18

how does cholera toxin work

Back 18

stimulates adenylate cyclase via activating Gs

Front 19

how does pertussis toxin work

Back 19

stimulates adenylate cyclase bia inhibiting Gi

Front 20

what bugs are microaerophilic?

Back 20

campylobacter jejuni
brucella abortus

Front 21

describe ITP
Px population?
findings?
tx?

Back 21

autoimmune - autoantibody vs platelets.
in children - follows URI
in adults - chronic
petechiae, purpura, epistaxis, NORMAL RED AND WHITE cells on smear
tx- stxs, then splenectomy

Front 22

what is the pentad of TTP?
what is happening?
smear?
tx?

Back 22

Intravascular hemolysis, renal failure (proteinuria, hematuria, increased Cr), thrombocytopenia, neuroprobs, fever
Acutely falling platelet counts - platelet fibrin thrombi
See shistiocytes on smear
Tx = IV Ig plasma exchange

Front 23

what is happening in HUS

Back 23

fulminant throbmocytopenia with hemolytic anemia, bloody diarrhea, abdom pain, seizures.
Glom sclerosis

Front 24

what 3 things cause warm agglutanin disease

Back 24

cancer, SLE, and drugs (pens, cephs, dopa, methyldopa)

Front 25

what 3 things can cause cold agglutatin disease

Back 25

mycoplasma pneumoniae
mononucleosis
lymphoma
remember 50% are idiopathic

Front 26

px presents with iron deficiency anemia, dysphagia, and weird looking nails

Back 26

plummer vinson syndrome -
also see atrophic glossitis

Front 27

what dz is marked by inability to remove oxygen radicals from bone marrow?
sx of this dz?

Back 27

faconi anemia

Front 28

what do howell jolly bodies indicate?

Back 28

RBCs are leaving the marrow prematurely - due to anemia?

Front 29

where do we see pappenheimer bodies?

Back 29

sideroblasts - in Fe overload

Front 30

Leukemia of children
what do we see

Back 30

acute lymphoblastic leukemia
see increased lymphoblasts

Front 31

px comes in with weight loss and red needle like things in a large looking celll on smear?
age?

Back 31

Acute myelogenous leukemia
auer rods
15-39

Front 32

what lab values assocated with Chronic myelogenous leukemia? genetics?

Back 32

CML - low Leukocyte Alkaline Phosphatase
9.22 bcr-abl

Front 33

What is mycosis fungoides

Back 33

NOT a fungal infection, it is a T cell lymphoma of the skin
an unusual variant of CLL

Front 34

CLL
what cell type?
sx?
age?

Back 34

Chronic lymphocytic leukemia
B cells
age>50

Front 35

Lab values and findings in multiple myeloma
Mnenomic?
Cytokines?

Back 35

clonal plasma cells >10%
light chains - monoclonal protein = Mspike
+ CRAB
Calcemia - hyper
Renal insufficiency
Anemia
Bone lesions (IL6 and 1-->osteoclast)

Front 36

px comes in with fatigue, weight loss, skin and mucous bleeding, headache and vision problems.
Lot of IgM

Back 36

Waldenstrom
Flame cells - esoinophili neoplastic plasma cells
Hyperviscosity from too much IgM leads to all the sx's

Front 37

what is troussea syndrome
px population?

Back 37

migratory thrombophlebitis
those with stasis - CHF and BF obstruction
and hypercoag states - pregnancy and cancer

Front 38

what are the 2 types of ateriolosclerosis and define

Back 38

hyperplastic - from malignant HTn
hyaline - from normal HTN and old age - leads to aneurysm via necrosis etc

Front 39

Henoch schonlein

Back 39

Joints, GI, skin (palpable purpura)

Front 40

what vasculitis is associated with HBV.
dz and sx? specific

Back 40

Polyarteritis nodosa (PAN) - medium vessels -
necrotizing inflammation of vessels due to WBC infiltration.
Fever, abdominal pain, melana, and renal dz (no glom nephritis) and HTN

Front 41

px takes a new drug and comes in with purpura, hemoptysis, hematuria, myalgia
dz, lab, and specify hematuria

Back 41

Microscopic polyangiitis
P anca +
Def has glom nephritis, unlike renal dz in PAN without nephritis

Front 42

what is buergers dz?
histology?
sx?

Back 42

HS reaction to TOBACCO
neutrophils surrounded by granuloma
Pain in distal limbs -> gangrene

Front 43

tx for kawasaki?

Back 43

aspirin and IV Ig

Front 44

p anca dz which is affects vessels like PAN + granulomas + eosinophil invasion in vessel wall and surrounding
affects what other tissues

Back 44

churge straus
skin, nerves, muscle, LUNG AND HEART

Front 45

Vascular problem in tertiary syphilis
looks like?

Back 45

leutic aneurysm - obliterative endarteritis of vasa vasorum of thoracic aorta
looks like tree barking

Front 46

role of protein C in clotting

Back 46

Binds protein s and they both work to cleave factor V and VIII to inhibit coagulation

Front 47

px comes in with vertigo, headache, vision problems and says they are itchy after showering

Back 47

polycythemia vera

Front 48

coagulation studies in won willebrand dz?

Back 48

increase BT
can see increased PTT...not always (diff sources diff answer)

Front 49

4 causes of vitamin K deficiency

Back 49

fat malabsorption (vit K is a fat soluble vitamin)
ABx (gut flora are primary PROVIDERS of vitamin K)
newborns (lack gut flora = hypercoag)
coumadin(warfarin) - prevents vit K dependent activation of II, VII, IX, X

Front 50

what is the problem in ITP

Back 50

a disorder of decreased platelet numbers (vs TTP w nl platelet numbers...they just aggregate and then appear low)
Ssx - petechiae purpursa, mucosal bleeding

Front 51

cause of ITP in adults

Back 51

anti structural platelelt Abs

Front 52

SSx in TTP

Back 52

severe thrombocytopenia
schistocytes (helmet cells, triagnular RBCs)
evidence of hemolysis (low Hg, high retic count, polychromasia on smear, high LDH)
fever
ischemic organ damage

Front 53

the most common enzymopathy

Back 53

g6pd deficiency

Front 54

explain g6pd deficiency in your own words

Back 54

XLR, blacks > whites
lack of g6pd means you can't reduced NAD to NADPH, so you can't regenerate reduced glutathione. lack of glutathione means you cant battle ROS, so you get hemolytic anemia

Front 55

classic findings on smear in g6pd deficiency?

Back 55

hemolytic anemia w HEINZ BODIES (oxidized Hg precipitated in RBCs) and BITE CELLS (result from macrophages taking a bite out of the RBCS to try and remove the Heinz bodies)

Front 56

what's the amino acid substitution in sickle cell anemia

Back 56

valine instead of glutamate at the 6th amino acid position on the beta globin chain

Front 57

Pt presents w jaundice, leg ulcers, and splenomegaly.....what should be on your DDx

Back 57

beta thalassemia

Front 58

what is the test of choice to detect isoimmunization in rh - females?

Back 58

indirect coombs test

Front 59

what causes megaloblastic anemia...in general

Back 59

anything that decreases DNA synth (think vitamin b12 or folate deficiency, anticancer drugs that target folate, or bugs that mess with folate/b12)

Front 60

classic peripheral smear finding in someone w megaloblastic anemia

Back 60

hypersegmented PMNs

Front 61

plummer vinson

Back 61

iDA, esophageal web, atrophic glossitis...usually an old lady

Front 62

fanconi anemia

Back 62

results from inability to remove ROS freom BM
AR
SSx - microcephaly, cafe au lait, SMALL OR ABSENT THUMB, DEFORMED OR ABSNENT RADIUS bone

Front 63

what is a howell jolly body

Back 63

visible nuclear fragments within RBCs...appear as basophilic granules
seeing these means RBCs are leaving the BM while still immature

Front 64

when do you see howell jolly bodies..in what dz process

Back 64

asplenia or functional hyposplenia

Front 65

heinz bodies are a smear finding in g6pd deficiency and what ellllse...

Back 65

alpha thal

Front 66

a patient comes in w iron overload secondary to deranged BM function...on smear he has siderocytes....RBCs w extra iron indicative of iron not bound to Hg....what is called when you see the iron sprinkled in the RBC?

Back 66

pappenheimer body

think...iron sprinkled on the RBC....pepperheimer...sprinkled on...

Front 67

felty's syndrome? an autoimmune dz....2 Sx and cause, -please

Back 67

felty's syndrome is a variat of RA that includes immune mediated destriction of PMNS via anti-PMN Abs

Front 68

most common malignancy of children

Back 68

ALL.....an overabundance of lympho BLASTS

Front 69

in AML, are the offending cells immature or mature?

Back 69

in AML, acute myelogenous leukemia, the cells are immature. all ACUTE leukemias are blastic. all chronic are mature

Front 70

you take a smear of a patient with AML and visualize red staining peroxidase positive needle like structures within the myeloblasts...what are these called

Back 70

AUER RODS....see that think AML....they are evidence of myeloid proliferation

Front 71

of all the myeoloproliferative disorders, CML has the highest chance of "getting worse" ...what can it potentially turn in to?

Back 71

AML

Front 72

what are the 4 subtypes of hodgkins lymphoma

Back 72

lymphocyte predominant
nodular sclerosing
mixed cellularity
lymphocyte depletion

Front 73

what do myeloma cells in multiple myeloma produce

Back 73

excess intact IgG or IgA
excess light chains
osteoclast activating factor (IL-6)
IL-1 (activates osteoclasts)

Front 74

you suspect multiple myeloma....what's the best way to Dx it. (do not say bence jones)

Back 74

eletrophoresis...look for the M spike

Front 75

waldenstrom's primary macroglobulinemia can lead to what syndrome and why

Back 75

hyperviscosity syndrome
due to overproduction of IgM (huge molecule that thickens blood)
SSx are nonspecific

Front 76

what will you see on a smear in waldenstrom's primary macroglobulinemia

Back 76

flame cells - eosinophilic neoplastic plasma cells

Front 77

what is trossea's syndrome

Back 77

migratory (diff places at different times) venous thrombosis

Front 78

Athersclerosis....what causes it...what cells associated...and what arteries hit

Back 78

atherosclerosis due to high LDL cholesterol
you see foam cells (cholesterol laden macrophage)
hits large and medium size aas

Front 79

monckeberg's arteriosclerosis....cause and what arteries?

Back 79

benign
calcium depoisits in BV media, producing lumps
radial and ulnar arteries usually

Front 80

hyperplastic arteriosclerosis....cause, arteries hit, and what on histo

Back 80

caused by malignant HTN (SBP >210 or DBP >150)
affects small arteries and arterioles
histo - ONION SKIN hyperplasia of BV media

Front 81

name 4 pANCA positive dzs

Back 81

hypersensitivity arteries
churg strauss
microscopic polyangiitis
primary sclerosing cholangitis

Front 82

microscopic polyangiitis and PAN look the same histologically...how can we DDx them?

Back 82

microscopic polyangiitis affects small BVS
PAN affects medium sized BVs
both show transmural necrotizing inflammation of arterial walls

Front 83

thromboangiitis obliterans (buerger's dz)

Back 83

hypersensitivity rxn to tobacco
granulomas fom in tibial and radial arteries and can progress to gangrene of distal limb

Front 84

how do you treat kawasaki dz

Back 84

IVIG and Aspirin

Front 85

+ pANCA
massive eosinophilic invasion of vessel wall and tissues
Pt presents w severe, recurrent asthma attacks and lung infiltrates

Back 85

churg strauss (allergic angiitis)

Front 86

behcet's syndrome is what kind of dz and what's the cause

Back 86

behcet's syndrome is a vasulitis of veins
vauses recurrent painful oral and genital ulcers
+ anti human oral mucosa Abs

Front 87

"tree barking of the intima of the thoracic aorta

Back 87

syphilitic leutic aneursym

Front 88

2 risk factors for aortic dissection

Back 88

HTN (major risk factor)
marfans

Front 89

what is the most common ASD

Back 89

ostium secundum......a fenestrated fossa ovale

Front 90

differentiate between infant and adult coarctation of the aorta

Back 90

infant is preductal stenosis
adult is post ductal
both with respect to the PDA (whether it is patent or not)

Front 91

ST elevation on ECG should make you think of what two ischemic heart disease causes

Back 91

ST elevation on ECG with a background of ischemic heart dz should make you think Q wave MI (STEMI), or prinzmetal's angina

Front 92

what two kinds of chest pain respond to Nitro (the others dont)

Back 92

prinzmetal's angina
stable angina

Front 93

acute rheumatic fever results in severe damage of what valve of the heart

Back 93

mitral

Front 94

what are the 3 kinds of pericarditis?

Back 94

fibrinous (#1 in world..transmural MI or dressler)
serous (#1 US, coxsackie B)
suppurative

Front 95

what kind of hypersensitivity is rheumatic fever

Back 95

type II hypersensitivity

Front 96

what is the pathonogmonic histological finding in rheumatic fever

Back 96

ASCHOFF body.....focal area of myocardial inflammation containing collagen, enlarged myocytes (anitschkow myocytes) and ASCHOFF cells (giant cells)