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Things I Suck At Day 1
KYCOM COMLEX
235
Medical
Graduate
07/20/2014

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Term
13 autosomal recessive disorders
Definition
cystic fibrosis
phenylketonuria
albinism
a1 AT deficiency
thallassemias
glycogen storage diseases
mucopolysaccharidoses (- hunter)
sphingolipidoses (- fabray)
PCKD (infant)
hemochromatosis
chediak higashi
bernard soulier
glanzmanns
Term
13 autosomal dominant disorders
Definition
familial hypercholesterolemia
familial polyposis coli
spherocytosis
von willebrand disease
ehlers danlos syndrome
marfan syndrome
achondroplasia
NF1 - von recklinghausen
NF2 - acoustic neuroma
tuberous sclerosis
von hippel lindau
huntingtons disease
PCKD (adult)
Term
12 X-linked recessive disorders
Definition
hemophilia A (VIII)
hemophilia B (IX)
G6PDH decifiency
fragile X syndrome
fabray
lesch nyhan
duchenne
becker
burtons ammaglobulinemia
wiskott aldrich
chronic granulomatous
hunters
Term
4 deletion disorders
Definition
cri di chat (5p)
retinoblastoma (13q)
Prader Willi (15q11-13 paternal)
Angelman (15q11-13 maternal)
Term
2 x-linked dominant disorders
Definition
hypophosphatemic rickets
incontinentia pigmenti
Term
1 mitochondrial inheritance disorder
Definition
leber's optic neuropathy
Term
cystic fibrosis: defect, signs, inheritance
Definition
defect in Cl transmembrane movement in epithelial cells

meconium ileus, viscous mucous, recurrent respiratory infections (<6mo S. aureus, >6mo pseudomonas), NaCl in sweat and tears, pancreatitis, cholelithiasis, malnutrition

AR
Term
phenylketonuria: defect, signs, inheritance
Definition
- phenylalanine hydroxylase
unable to metabolize phenylalanine, thus cannot make melanin or NE/Epi/DOPA
phenylketones build up

neurotoxicity, lighter complexion, mental retardation

AR
Term
albinism: defect, signs, inheritance
Definition
- tyrosinase, unable to make melanin
hypopigmentation
AR
Term
a1-AT deficiency: defect, signs, inheritance
Definition
unable to inhibit elastase causing emphysema

builds up in liver causing cirrhosis

AR
Term
glycogen storage diseases and their inheritance
Definition
AR
von girke, pompe, mcardle
Term
mucopolysaccharidoses and their inheritance
Definition
hurler, scheie - AR
hunter - XR
Term
sphingolipidoses and their inheritance
Definition
neimann pick, gauchers, keabbes, tay sacs, metachromatic dystrophy - AR
fabray - XR
Term
polycystic kidney disease (infant): signs, inheritance
Definition
non functional kidney and liver cysts lead to failure
fatal
AR
Term
hematochromatosis: defect, signs, inheritance
Definition
SI takes up excess Fe
deposits in liver, pancreas, heart, skin

cirrhosis, DM 1 like, cardiomyopathy, bronze skin

AR
Term
chediak higashi: defect, signs, inheritance
Definition
WBC have abnormal microtubules (falty movement, lysosomes, granules)

recirrent infection, lymphoid cancers, albinism, neuropathy

AR
Term
bernard soulier: defect, signs, inheritance
Definition
AR

lack of Gp1b for platelet adhesion to vWF

bleeding
Term
glanzmann's thrombasthenia
Definition
excessive bleeding

AR

lack of Gp2b3a for platelet to platelet adhesion
Term
familial hypercholesterolemia: defect, signs, inheritance
Definition
abnormal LDL receptor so unable to process LDL in the liver

AD

athlerosclerosis, CAD, stroke, PVD, mesenteric ischemia
Term
familial polyposis coli: defect, signs, inheritance
Definition
AD

thousands of colon polyps and cancer
Term
spherocytosis: defect, signs, inheritance
Definition
- spectrin causes round RBC that are eaten in spleen causing anemia

AD
Term
von willebrand disease: defect, signs, inheritance
Definition
bleeding

-vWF so cannot bind Gp1b and factor VIII

AD
Term
ehlers danlos: defect, signs, inheritance
Definition
abnormal collagen or elastin

hyperextensible skin, joints, valve disorders
Term
marfan syndrome: defect, signs, inheritance
Definition
- fibrillin causes defective microfibrils in CT

tall, thin, ectopic lentis, aortic dilation and aneurysm, archnodactyly, mitralvalve prolapse

AD
Term
achondroplasia: defect, signs, inheritance
Definition
decreased chondrocyte proliferation in growth plate of long bones

short limbs and digits, early epiphyseal ossification, torso and head normal size

AD
Term
NF1: defect, signs, inheritance
Definition
AD loss of NF1 tumor supressior gene

neurofibromas
optic nerve gliomas
pigmented nodules in iris (lisch nodules)
cafe au lait spots
axillary freckling - pathopneumonic
Term
NF2: defect, signs, inheritance
Definition
AD loss of NF2 tumor supressor

BL schwannomas
meningiomas
hearing deficits
Term
tuberous sclerosis: signs
Definition
harmatomas: benign brain tumor, potato
cysts: liver, kidney, pancreas
MR
facial angiofibromas
shagreens patches: leathery yellow, sacrum
ash leaf pathches: leaf like hypopigmented
Term
von hippel lindau: signs
Definition
high risk renal cell CA (polycythemia, high AP)
caipllary hemangiomas in cerebellum and retina
cysts: liver, kidney, pancreas
Term
huntingtons disease: defect, signs, inheritance
Definition
CAG repeat on CH 4
degeneration of caudate, putamen, frontal cortex

30-40yo, chorea, psych, cognitive decline
Term
PCKD adult: signs
Definition
renal cysts
berry aneurysms -> subarachnoid hemorrhages
Term
hemophilia A: cause, inheritance
Definition
- factor VIII
XR
Term
hemophilia B: cause, inheritance
Definition
- factor IX
XR
Term
G6PDH deficiency: cause, inheritance
Definition
cant get rid of ROS in RBC
hemolysis when stressed
XR
Term
fragile X syndrome: defect, signs, inheritance
Definition
structural defect in CH X, CGG repeat with anticipation, XR

MR, large ears and jaw, macroorchidism, 50% female carriers have MR
Term
fabray disease: defect, signs, inheritance
Definition
- a galactosidase A
sphingolipidosis
XR

angiokeratomas (wart with telangestica), renal failure, NO CNS DISEASE
Term
lesch nyhan: defect, signs, inheritance
Definition
- HGPRT causes increase in uric acid because it cannot be recycled
XR
leads to self mutilation
Term
duchenne: defect, signs, inheritance
Definition
- dystrophin leads to muscle atrophy, high CK, proximal muscle weakness first
XR
Term
becker: defect, signs, inheritance
Definition
duchenne but less severe, longer before muscle atrophies
decreased dystrophin
XR
Term
burtons ammaglobulinemia: defect, signs, inheritance
Definition
low or absent MATURE B cells
recurrent bacterial infections
XR
Term
wiskott aldrich: defect, signs, inheritance
Definition
otitis media, eczema, thrombocytopenia, IgM dysfunction
XR
Term
chronic granulomatous disease: defect, signs, inheritance
Definition
granulomas form because neutrophils and macrophages cant kill substance
HSN IV
Term
hunters: defect, signs, inheritance
Definition
mucopolysaccharidoses
XR
MR, gargoyle faces
Term
cri di chat: defect, signs, inheritance
Definition
5p deletion

high pitched cat like cry
microcephaly
MR
Term
retinoblastoma: defect, signs, inheritance
Definition
malignant retinal tumor of childhood

13q deletion
Term
prader willi: defect, signs, inheritance
Definition
15q11-13 paternal deletion

hypotonicity, obesity, MR
Term
angelman syndrome: defect, signs, inheritance
Definition
15q11-13 maternal deletion
smiles, wide gait, ataxia, MR, laughter
Term
hypophosphatemic rickets: defect, signs, inheritance
Definition
inherited vitamin D resistance
bowed legs
XD
Term
incontinentia pigmenti: defect, signs, inheritance
Definition
XD
miniteeth
patchy aloplecia
Term
lebers optic neuropathy: defect, signs, inheritance
Definition
BL blindness sometime after 15yo
mitochondrial inheritance
retina is ATP dependent so mitochondrial defect decreases ATP causing blindness
Term
auto-antibody for...
1. autoimmune hemolytic anemia
2. bullous phenigoid
3. DM1
4. phemigus
5. microscopic polyangiitis
Definition
1. anti-RBC
2. anti-epidermal BM
3. anti-B islet cell
4. anti-keratinocyte junction
5. p-ANCA anti-neutrophil cytoplasm
Term
auto-antibody for...
1. polymyositis
2. progressive systemic sclerosis
3. sjogerns
4. ITP
5. vitiligo
Definition
1. ANA, anti-Jo1
2. anti-SCL70
3. anti-SSA/Ro, anti-SSB/La
4. anti-structural platelet
5. anti-melanocyte
Term
antoantibody for...
1. RA
2. SLE
3. drug SLE
4. CREST
5. myasthenia gravis
Definition
1. anti-IgG (RF)
2. ANA, anti-dsDNA
3. anti-histone
4. anti-centromere
5. anti-ACh receptor
Term
autoantibody for...
1. graves disease
2. hashimotos thyroiditis
3. wegeners granulomatosis
4. celiac sprue
5. goodpastures
6. primary biliary cirrhosis
Definition
1. anti-TSH receptor
2. anti-microsomal
3. anti-neutrophil cytoplasm (ANCA)
4. anti-glidin
5. anti-glomerular BM
6. anti-mitochondrial
Term
4 types of hypersensitivity
Definition
hyperacyte: HSN II, preformed Ab
acute: memory T cells made, HSN, IV
chronic: Ab-made, HSN II
graft-vs-host: T cells in transplant attack, HSN IV
Term
most common cause of osteomyelitis
Definition
S. aureus - normal
salmonella - sickke cell
serratia/pseudomonas - IV drug users
Term
leukocyte adhesion deficiency 1
Definition
defect in LAF-1 integrin (CD18) on phagocytes which inpairs migration and chemotaxis

bacterial skin and mucosa infection
NO PUSS
impaired wound healing
DELAYED UMBILICAL SEPARATION
Term
chediak higashi
Definition
defect in lysosome trafficking gene and microtubules

STAPH AND STREP INFECTION
ALBINISM
PERIPHERAL NEUROPATHY
Term
chronic granulomatous disease
Definition
defect in NADPH oxidase, ROS cannot be made for respiratory burst

infection with CATALASE POSITIVE organisms PLACESS (PSEUDOMONAS, LISTERIA, ASPERGILLUS, CANDIDIA, E COLI, S AUREUS, SERRATIA)
Term
burtons ammaglobulinemia
Definition
defect in BTK TK, no mature B cells
XR

NORMAL B CELL COUNT, DECREASED Ig
ABSENT LYMPH NODES AND TONSILS

PYOGENIC BACTERIA infection
Term
IgA deficiency
Definition
no class switching to IgA

bacterial infectious of mucous membranes
Term
CVID
Definition
defect in B cell maturation

NORMAL AMOUNT OF B CELLS
DECREASED Ig

pyogenic bacterial infections
Term
DiGeorge syndrome
Definition
defect in 3rd-4th pharyngeal pouches causing absent thymus and parathyroids

PROTOZOA, VIRAL AND FUNGAL INFECTIONS
TETANY, HYPOCALCEMIA

OFTEN HAVE TERTOLOGY OF FALLOT OF TRUNCUS ARTERIOSUS

decreased T cells, PTH, Ca
detection with FISH
Term
chronic mucocutaneous candidiasis
Definition
T cell dysfunction
chronic candidia
NORMAL T CELL NUMBERS
Term
hyper IgM syndrome
Definition
inability of B cells to class switch away from IgM because T cells won't tell them to (CD40 defect)

pyogenic bacterial infections (pneumocystis, CMV, cryptosporidium)
Term
Job syndrome
Definition
AD
defect in STAT3 in T17 cells so they cant recruit neutrophils
hypger IgE
drecreased INFy

sinopulmonary infections, candidia, cold abscesses, retained primary teeth, eczema
Term
SCID
Definition
failure to thrive, chronic diarrhea, thrush, bacteria, viral, fungal, protozoal infection

decreased T cell receptor activation
decreased germinal T cells
decreased T cells
NO LYMPH NODES
PNEUMOCYSTITS PNEUMONIA
Term
ataxia telengectasia
Definition
defect in DNA repair of strand breaks

ataxia, spider angiomas, IgA deficiency
low T cells
sinopulmonary infections
Term
wiskott aldrich
Definition
mutation in WAS gene
T cells cannot reorganize actin cytoskeleton
B cells not stimulated to make IgM

thrombocytopenic purpura
eczema
recurrent infections
autoimmune disease and malignancy risk
otitis media
Term
explain clotting cascade
Definition
intrinsic: hageman (XII) > XI > IX + VIII > X

extrinsic: tissue factor > VII > X

common: X + V + thrombin (II) > fibrinogen (I) > clot > XIII stabilizes
Term
testing for bleeding disorders
Definition
bleeding time: platelets
PT: extrinsic and common
PTT: intrinsic and common
TT: common
Term
von willebrand disease: inheritance, signs, cause
Definition
-vWF, cannot bind VII and Gp1b on platelets
AD
increased menstural bleeding, increased PTT and bleeding time
Term
hemophilia A: inheritance, signs, cause
Definition
lack of VII, increased PTT, XR, bleeding
Term
hemophilia B: inheritance, signs, cause
Definition
lack of factor IX
XR
increased PTT
Term
vitamin K deficiency: inheritance, signs, cause
Definition
non functional II, VII, IX, X due to lack of y-carboxylation

increased PT, PTT, TT

causes: fat malabsrption, antibiotics, newborns, warfarin
Term
why is warfarin prescribed with heparin
Definition
at first warfarin stops protein C and S which inhibitors factors V and VIII so clotting increases
Term
ITP: signs, cause
Definition
children: post viral, self limited
adult: anti-structural platelet (Gp2b/3a)

decreased platelets, petechiae, purpura, mucosal bleeding
Term
TTP: signs, cause
Definition
spontaneous platelet aggregation
thrombocytopenia
schistocytes: helmet cells due to spleen destruction
hemolysis: low Hb, reticulocytes, polychromasia (break on staining), high LDH (organ damage), fever
Term
spherocytosis: diagnosis, inheritance, signs, cause
Definition
-spectrin makes RBC spheres
hemolysis of RBC, splenomeagly

positive osmotic fragility test, increased MCHC and RDW

AD

removal of spleen increases encapsulated infection risk
Term
encapsulated bacteria
Definition
even some killers have pretty nice capsules

E. coli
S. pneumo, salmonella, GBS
Klebsiella
H. influenza
Pseudomonas
Neisseria
Cryptococcus neoformins
Term
G6PDH deficiency: inheritance, signs, cause, diagnosis
Definition
XR, more in AA
RBC sensitive to oxidative damage

back pain, hemoglobinuria
HEINZ BODIES (DENATURED HB)

stress makes me eat bites of fava beans with heinz ketchup
Term
sickle cell anemia: inheritance, signs, cause
Definition
HbS point mutation of B chain glutamic acid with valine, AR

crystalization when hypoxia, acidic, dehydrated

provides resistance to malaria

autosplenectomy/HOWELL JOLLY BODIES
salmonella osteomyelitis
vasoocclusive crisis
renal papillary ncrosis
Term
a-thallasemia: inheritance, signs, cause
Definition
decreased z globin chain synthesis
4 deletion: excess y-globin form y4 (HbBart)
2-3 deletion: microcytic anemia
Term
b-thallasemia: inheritance, signs, cause
Definition
decreases B globin chain synthesis

minor: increased HbA2, microcytic anemia

major (cooleys): severe microcytic anemia, no B globin, CHIPMUNK FACIES, EXTRAMEDULLARY HEMATOPOESIS
Term
warm agglutinin disease: signs, cause
Definition
cause: methyldopa, dopa, penicillin, cephalopsorin, lymphoma, leukemia, SLE

WEG: warm, extravascular hemolysis, IgG

anti-RBC antibodies cause autoimmune hemolysis
Term
cold agglutinin disease: signs, cause
Definition
cause: mycoplasma pneumonia, mononucleosis, lymphoma, idiopathic, CLL

CIM: cold, intravascular hemolysis, IgM

anti-RBC antibodies cause autoimmune hemolysis
Term
coombs tests
Definition
direct: detect immune hemolysis, find Ab directly bound to RBC

indirect: find free floating Ab, test for isoimmunization in Rh- females
Term
megaloblastic anemias: causes, signs
Definition
B12 deficiency: diphyllobothrium latum, diet, type A gastritis, pernicious anemia. shows increase in homocysteine and decrease in methionine, glossitis, peripheral neuropath, loss of proprioception and vibration, dementia, spasticity

folate deficiency: alcoholics, methotrexate, TMP, pnehytoin, pregnancy

other signs: hypersegmented nutrophils
Term
plummen vinson: cause, signs
Definition
anemia due to low grade long term Fe deficiency

esophageal webs, dysphagia of solids only, atrophic glossitis
Term
fanconi anemia: inheritance, signs, cause
Definition
inability to remove ROS form marrow
AR

microcephaly, cafe au lait, no thumbs or radius, aplastic anemia, leukemia, lymphoma
Term
what disease do these cells go with...
1. acanthocyte
2. basophilic stippling
3. bite cell
4. ringed siderblast
5. schistocyte / helmet
6. spherocyte
7. teardrop cell
8. target cell
9. heinz bodies
10. howell jolly bodies
Definition
1. liver disease
2. lead poisoning, alcohol abuse, thallasemias
3. G6PDH deficiency
4. sideroblastic anemia, excess Fe
5. DIC, TTP, HUS, traumatic hemolysis
6. spherocytosis
7. marrow infiltration
8. asplenia, liver disease, HbC disease, thallasemia
9. G6PDH deficiency (Hb percipitate)
10. basophilic nuclear remnant, seen with no spleen
Term
Fe deficiency anemia: signs, cause
Definition
due to chronic bleed (GI, menagorrhea), malnutrition, malabsorption, pregnancy

increased TBIC, decreased ferritin, microcytosis, hypochromia

fatigue, conjunctival pallor
Term
lead poisoning: signs, cause
Definition
inhibits ALA dehydratase and ferrochetalase stopping heme synthesis and increases protophorphyin

inhibits RNA degradation causing RBC to retain aggregates or rRNA (BASOPHILLIC STIPPLING)

LLEEAADD S

L ead lines on gingive (burtons)
L ead lines on long bones
E ncephalopathy
E rythrocyte basophillic stippling
A bdominal colic
A anemia sideoblastic
D rop of wrist and foot
D imercaprol and EDTA 1st line tx
S uccimer for chelation in kids
Term
anemia of chronic disease: signs, cause
Definition
inflammation increases HEPCIDIN which finds ferroportin on macrophages and inhibits Fe release

decreased Fe and TBIC
increased ferritin

normocytic then microcytic hypochromatic
Term
sideroblastic anemia: inheritance, signs, cause
Definition
XR defect in ALA synthase

ringed sideroblasts with Fe in mitochondria in marrow

increased Fe and TBIC, increased ferritin

causes siderocytes: Fe in RBC not bound to Hb (pappenheimer bodies)
Term
paroxysmal nocturnal hemoglobinuria: cause
Definition
defect in GPI anchor for factors that protect RBC from complement
Term
reticulocyte appearance
Definition
blue lace patterns due to rRNA remnants
Term
felty's syndrome: signs
Definition
RA with anti-neutrophil antibodies that cause neutropenia
Term
causes of increased neutrophils, eosinophils, lymphocytes, monocytes
Definition
neutrophils: inflammation, bacteria, cancer, leukemia

eosinophils: HSN 1, helminths

lymphocytes: granulomatous diseases, virus, pertussis

monocytes: granulomatous diseases, infectious mononucleosis
Term
factor V leiden
Definition
mutant factor V resistant to degration by protein C
hypercoaguability
Term
ALL signs
Definition
most common malignancy in kids

lymphadenopathy
lymphoblastic
+TdT
impaired marrow
Term
AML signs
Definition
myelobasts
+auer rods: red staining peroxidase positive needle like
impaired marrow
Term
CML signs
Definition
myelocytes
philadelphia chromososm (9;22) cbr-abl fusion makes oncogene
LOW LEUKOCYTE ALKALIKE PHOSPHATASE (LAP) (no mature granules)

blast crisis: can change to AML
Term
CLL signs
Definition
lymphocytes
impaired marrow
SMUDGE CELLS
SLL = varient caused by sezary syndrome (leukemic component of mycosis fungoides from the skin)
Term
hairy cell leukemia signs
Definition
middle age white dude
SPLENOMEAGLY
B cells with hair like projections
impaired marrow
+TRAP
+CD25
marrow fibrosis, dry tap on aspiration
Term
burkitt lymphoma: cause, signs
Definition
t(8;14) c-myc (8) Ig heavy chain (14)
STARRY SKY
lymphocytes and macrophages
EBV
jaw lesion (africa)
pelvis/abdomen lesion (other places)
Term
diffuse large B cell lymphoma cause
Definition
t(14;18)
most common non-hodgkins in adults
Term
mantle cell lymphoma cause
Definition
t(11;14) cyclin D1 (11), Ig heavy chain (14)

CD5+
Term
follicular lymphoma cause
Definition
t(14;18) Ig heavy chain (14), bcl-2 (18)

waxing and waining lymphadonaopathy
Term
adult T cell lymphoma
Definition
HTLV-1 virus associated with IV drug abuse

cutaneous lesions, lytic bone lesions, hypercalcemia
Term
hodgkins lymphoma: types, signs
Definition
reed sternberg cells
fever, night sweats, weight loss
50% associated with EBV
giant cells with bilobed nuclei (owl eyes)

2 eyes x CD15 = CD30

nodular sclerosing: M=F, has sclerosis to keep cancer in cage
mixed cellularity: has sclerosis to keep cancer in cage
lymphocyte rich: best prognosis
lymphocyte depleted: poor prognosis
Term
monoclonal gammopathy: cause
Definition
excess functional Ig
benign, could progress to multiple myeloma
Term
multiple myeloma: cause, signs, dx
Definition
myeloma cells: plasma cell tumor in marrow
secrete IgG, IgA, light chains (bence jones proteins), IL-6 and 1 (activates osteoclasts making punched out lesions)

hypercalcemia, leukocytopenia, amyloidosis (AL light chain), renal insufficiency

M spike on electrophoresis SPEP
bence jones proteins on UPEP, plasma cells with CLOCK FACE CHROMATIN (intracytoplasmic Ig)
Term
waldenstorms primary macroglobulinemia: cause, signs
Definition
M spike due to IgM increase which causes hyperviscosity
FLAME CELLS: eosinophilic neoplastic plasma cells

fatigue, weakness, bleeding, headache, visual problems, neuro problems
Term
phlebothrombosis risk factors
Definition
risk factors for clots in veins...
blood stasis, hypercoaguability, endothelial cell damage
Term
trosseau's syndrome: cause, sign
Definition
migratory venous thrombosis
common with CHG, pregnancy, adenocarcinoma
Term
acute intermittent prophyria: cause, signs
Definition
- prophobilinogen deaminase so
porphilinogen, delta-ALA, and coporphobilinogen (urine) accumulate

5P's
painful abdomen
port wine coloredurine
polyneuropathy
psych disturbance
percipitated by drugs, etoh, starvation
Term
prophyria cutanea tarda: cause, signs
Definition
- uroporphyrinogen decarboxylase
uroporphyrin accumulates causing tea colored urine
photosensitivity leads to blistering
Term
5 chromosome translocations and their disease
Definition
9;22 CML philaldelphia
8;14 burkitt (c-myc)
11;14 mantle cell (cyclin D)
14;18 follicular lymphome (bcl-2)
15;17 M3 type AML
Term
langerhans cell histocytosis
Definition
proliferation of langers (monocyte lineage) in child

lytic bone lesions
otitis media involing mastoid

+S100 +CD1a
+birbeck granules (tennis rackets)
Term
polycythemia vera
Definition
CML type
HCT >55%
JAK2 mutation
itching after shower
erythromelalgia: burning with red blue discoloration
NO EPO INCREASE (THAT WOULD BE 2 POLYCYTHEMIA)
Term
essential thrombocytosis
Definition
CML type
overproduction of platelets
bleeding, thrombosis
enlarged megakaryocytes in marrow
Term
myelofibrosis
Definition
CML type
fibrosis of marrow
TEARDROP RBC
Term
mockenbergs arterlosclerosis
Definition
benign
Ca deposits in vessel media product lumps
radial and ulnar arteries
often palpable
Term
hyperplastic arteriolosclerosis
Definition
due to malignant HTN >210/150
small arteries and aeterioles
onion skin hyperplasia of media causes progressive lumen narrowing
Term
hayline arteriolosclerosis
Definition
inflammation and use: HTN, age, DM
thickened basement membrane
nephrosclerosis of kidney
media can atrophy causing aneurysm
Term
hypersensitivity arterities
Definition
HSN III to HSV, EBV, HIB
+P-ANCA
small vessels: skin, muscle, brain, kidney
PAINFUL PURITIC PURPURA ON BUTT
Term
henoch schonlein purpura
Definition
IgA complex deposition
small vessels
children
PALPABLE PURPURA ON BUTT
arthralgias, BLOODY DIARRHEA
glomerulonephritis

cause: URI virus, drug penicillin, vaccination
Term
polyarteritis nodosa
Definition
transmural necrotizing inflammation of artery with WBC invading
due to HBC, HCV, hairy cell leukemia

fever, abdominal pain, renal disease NOT GN, HTN, neuritis, myalgia
Term
microscopic polyangiitis
Definition
small vessels
+P-ANCA
reaction to drugs penicillin
GN - RPGN
pulmonary capillaritis
palpable purpura (due to skin capillaries)
hemoptysis
hematria
myalgias (due to M capillaries)
Term
thromboangiitis obliterans (burgers)
Definition
HSN to tobacco
TIBIAL AND RADIAL ARTERIES
granulomas
gangrene
Term
giant cell arteritis
Definition
granuloma
aortic, carotid, vertebral, temporal arteries

fever, fatigue, temporal headache, high ESR, jaw claudication, SUDDEN MONOCULAR BLINDNESS

female >50
Term
polymyalgia rheumatica
Definition
systemic giant cell arterities
arthralgia, myalgia, fever, VERY high ESR, LIMB CLAUDICATION, CVA, MI, organ infarcts
Term
wegners granulomatosis
Definition
+C-ANCA
granulomas of small vessels
sinusitis, GN-RPGN, cavitary lung lesions, sinus polyps
Term
takaysu arteritis
Definition
granuloma
thickened aortic arch
asian 15-40
night sweats, CP, absent or weak peripherial pulse (decreased aortic recoil)
Term
kawasaki disease
Definition
fever, conjunctivitis, red palms and soles, HIGH RISK CORONARY ARTERY ANEURYSM, oral mucosa edema, cervical lymphadenopathy

1ST LINE TX ASPIRIN and IVIG
Term
churg strauss
Definition
granulomas
PAN like vessel wall
eosinophil invasion
+P-ANCA
LUNG AND HEART
asthma like attack, lung infiltrates
myocarditis, coronary destruction
Term
bechets syndrome
Definition
phlebitis
oral and genital ulcers like herpes
uveitis, iritis, arthritis of KNEE
silk route
+anti-human oral mucosa Ab
-tzank test
Term
syphilitic aneurysm
Definition
leutic aneurysm
obliterative endarteritis of vasa vasorum of thoracic aorta
ischemia of media
shrinkage of media causes buckle and tree barking
aortic regurgitation
Term
aortic dissection
Definition
intima tears
can rupture and hemorrhage
double barrel sign
risk in HTN and marfans

A or 2: ascending aorta
B or 3: descending aorta
1: both

dyspnea, cough (laryngeal compresion), aortic regurgitation, tearing CP
Term
murmurs...
1. blowing
2. harsh
3. click
4. machine
Definition
1. regurgitation
2. stenosis
3. prolapse
4. PDA
Term
cause of aortic stenosis, aortic regurgitation, mitral stenosis, mitral regurgitation, mitral prolapse
Definition
aortic stenosis: calcification, congenital bicuspid

aortic regurgitation: HTN, leutic aneurysm, dissection, ankylosing spondylitis

mitral stenosis: rheumatic heart disease

mitral regurgitation: MVP, rheumatic heart disease, endocarditis, papillary muscle ruprute after MI

mitral prolapse: marfans, elhers danlos, fragile X
Term
random word association...
1. boot heart
2. aschoff body
3. charcot laiden crystals
4. curshman spirals
5. panda sign
6. red and gray hepatization
Definition
1. tetralogy of fallot
2. multinucleated giant cells and enlarged monocytes in rheumatic fever
3. eosiophils in asthmatic spit
4. live swirly eosinophils in asthmatic spit
5. sarcoidosis of parotid glands
6. red first when lobe fills with RBC gray when it fills with fibrin in pneumococcus
Term
causes of ST depression and their blood markers
Definition
stable angina: none
unstable angina: CK-MB
non-Q wave MI: CK-MB, troponin
Term
causes of ST elevation and their blood markers
Definition
Q wave (transmual) MI: CK-MB, troponin
prinzmetal angina: none
Term
acute vs subacute infective endocarditis
Definition
acute: S. aureus, strep, drug ucers, diabetic

subacute: Strep viridans, E. coli, prior valve pathology
Term
marantic endocarditis
Definition
thrombi on endocardium
seen in chronic illness esp adenocardinoma (mucin clots)
Term
causes of the three types of pericarditis
Definition
fibrinous; MI, dresslers (pericarditis post MI)

serous: coxackie B, uremia, rheumatic, scleroderma, SLE

suppurative: s. pneumo, s. aureus
Term
obstructive vs restrictive
Definition
obstructive: decreased FEV1, FVC, FEV/FVC ratio, increased TLS

restrictive: normal FEV1 and ratio, decreased TLC
Term
bronchiectasis
Definition
irreversible focal bronchial dilation

cause: infection usually with kartageners syndrome or cystic fibrosis

purulent hemoptysis
Term
location and buzz word
1. asbestosis
2. anthracosis
3. berylliosis
4. silicosis
Definition
1. lower, pleural plaque, ferruginous bodies
2. upper, right heart failure, black lung
3. lower, technology, flourescence
4. upper, quarts, sand/stone cutting, TB!!
Term
diffuse idiopathic fibrosis
Definition
aka UIP
honeycomb lung
fatal in years
finger clubbing
VELCROW SOUND
Term
cause of
1. bronchopneumonia
2. lobar pneumonia
3. atypical pneumonia
Definition
1. hemophilus, pseudomonas, staph
2. pneumococcus, klebsiella, legionares
3. viruses, mycoplasma, legionaires
Term
cause of ronchi, rales, wheezing
Definition
ronchi: chronic bronchitis, bronchopneumonia
rales: bronchopneumonia, atypical pneumonia, legionares
wheeze: asthma
Term
1. adenocarcinoma
2. squamous cell CA
3. large cell CA
4. small cell CA
Definition
1. peripherial
2. central
3. peripherial
4. central
Term
lung CA paraneoplastics
Definition
SCC: PTH (hypercalcemia)
SmCC: ADH (HTN), ACTH (Cushings), eaton lambert (Ab to NMJ Ca channels)
Term
3 nephritic GN and their buzz words
Definition
RPGN: crescents (proliferation of bowmans), 1 Ab, 2 complex, 3 ANCA

acute proliferative: post strep, lumpy bumpy, IgG and C3 subEPIthelial, mesangial proliferation

goodpastures: anti-glomerular GM, linear IgG and C3, hemoptysis
Term
3 nephrotic GN and their buzz words
Definition
minimal change: loss of foot processes, URI HX, #1 in kids

membranous GN: spike and dome IgG and C3 subEPIthelial

focal segmental: sclerosis, chronic ureteral reflux, heroin, <35yo
Term
3 nephritic and nephroti GN and their buzz words
Definition
membranoproliferative: URI, HepC, heroin, SLE, mesangial proliferation, TRAM TRACK C3 subENDOthelial, decreased serum complement

IgA nephropathy (bergers): URI or GI viral infection, IgA complex deposits, increased serum IgA

diabetic/kimmelstiel wilson: 20y with DM, microalbuminuria, small kidneys, sclerosis of BM in nodules
Term
random buzz word...
1. muddy brown casts
2. triple stone
3. Ca oxylate
4. cystinuria
5. reinke crystals
6. beeding of bile ducts
Definition
1. acute tubular necrosis
2. staghorn calculus/struvite caused by urea splitting proteus or saphrophyticus
3. uric acid stone
4. cystine stone, black
5. leydig cell tumor
6. narrowing and dilation primary sclerosis cholangitis
Term
timeline of troponin, CK-MB, Mb
Definition
troponin: released 2-4h, peak 12h, lasts 7d

CK-MB: peak 10-24h, lasts 2-3d

Mb: peaks 2h, lasts less than CK-MB
Term
candidiasis vulvovaginitis
Definition
pH <4.5 bready odor
germ tubes and pseudohyphae
DOC miconazole, second fluconazole
puritis, erythema, satelite lesions, curd like discharge
Term
trichomoniasis
Definition
DOC metronidazole
puritis, frothy green discharge, putrid smell
darting trich on wet prep
often carried asymptomatically in rectum
Term
bacterial vaginosis
Definition
gardnerella and mobilnucus
DOC metronidazole
gray white discharge
+KOH whiff test
fishy odor
clue cells
Term
gonorrhea
Definition
DOC ceftriaxone + doxy for chalmydia
purulent discharge
ascends to cause PID and salpingitis
Term
chalmydia
Definition
non-gonoccal urethritis
DOC doxycyclinie + ceftriaxone for gonorrhea
Term
lymphogranuloma venerum
Definition
eruption of skin surface
chalmydia trachomatis L1-3
DOC docycycline
Term
granuloma inguinale
Definition
klebsiella granolomatis / donovania granulomatis

blocks lymph drainage of genitals in inguinal area

DOC tetracycline
Term
chancroid
Definition
H. ducrey
school of fish on microscope
painful canchre
DOC ceftriaxone
Term
syphillis
Definition
treponema pallidum
DOC pen G
1. painless canchre
2. rash palms and soles, condylomata lata on genitals (flat, light brown/pink)
3. gumma, tabes dorsalis (destruction of posterior columns), psychosis, dementia, luetic aneurysm, aortitis, aortic regurgitation
Term
condyloma acuminatum
Definition
genital warts
painless
koliocytes: perinuclear clearing
HPV 6, 11, 16, 18, 30s

cause cancer via E6/p53 and E7/Rb
Term
genital herpes
Definition
HSV1/2
painful fluid filled vesicles in group on red base
trigeminal ganglion if oral, lumbar/sacral ganglion if genital
+tzank test for multinucleated giant cells
DOC acyclovir
Term
chalmydial cervicitis
Definition
asymptomatic
ascends to cause PID
DOC doxycycline + ceftriaxone for gonorrhea
Term
testicle tumor serum markers and basic facts...
1. seminoma
2. choriocarcinoma
3. yolk sac tumor
4. teratoma
5. embrynonal
Definition
1. most common, like ovarina dysgenesis, hCG
2. teens, like ovarian choriocarcinoma, hCG
3. like endodermal sinus tumor, aFP
4. 2+ germ layers, immature cells
5. painful, hCG, 2nd most common
Term
ovarian tumor cell types...
1. serous
2. mucinous
3. endometroud
4. clear cell
5. brenner
Definition
1. cystadenoma/CA, fallopian epithelium serous filling
2. cystadenoma/Ca, columnar epithelium, mucus filling, pseudomyxoma peritonei
3. glandular tissue, solid
4. lots of cytoplasm, often with endometroisis or endometrial CA
5. celioblastoma, like bladder cells, coffee bean nuclei
Term
ovarian tumor general info...
1. teratoma
2. dysgegeminoma
3. endodermal sinus
4. choriocarcinoma
Definition
1. 2+ germ layers, dermoid cyst (teeth, skin), struma ovarii (hyperthyroid part)
2. like seminoma
3. like yolk sac tumor, aFP
4. agressive, hCG, trophoblast
Term
ovarian tumor general info...
1. granulosa theca
2. sertoli leydig
3. fibroma
4. krukenberg
Definition
1. secretes estrogen, call exner bodies, causes endometrial CA
2. adenoblastoma, makes testosterone, virulization, deep voice
3. meigs syndrome (ascites, pleural effusion), solid tumor, spindle fibroblasts
4. metastasis of signet ring cells from stomach to ovary, mucous pushes nuclei to side
Term
buzz word association
1. whorled smooth muscle
2. brown bloody foul smell discharge
3. large for gestational age
4. bloody nipple discharge
5. log shaped tumor
6. low hGC
Definition
1. uterine leiomyoma/fibroid
2. choriocarcinoma
3. hydatidiform mole
4. intraductal papilloma
5. phylloides breast tumor / cystosarcoma phylloides
6. ectopic pregnancy
Term
buzz word association
1. BRACA1
2. gelatenous (breast cancer)
3. microcalcifications (breast cancer)
4. peau d orange, nipple retraction
5. signet ring cells (breast cancer)
6. cells in single file (breast CA)
7. pagets disease
Definition
1. medullary breast CA
2. colloid/mucinous CA
3. DCIS comedocarcinoma
4. DCIS
5. LCIS
6. LCIS
7. DCIS
Term
klinefelter syndrome
Definition
XXY
testicle atrophy
enuchoid body
tall, long
gynecomastia
female hair
Term
turner syndrome
Definition
XO
short
ovarian dysgenesis/streak ovary
shield chest
bicuspid aorta
preductal coarctation
webbed neck
horseshoe kidney
primary amenorrhea
Term
preeclampsia
Definition
>140/90
>300 protein/24h
>20 wks
abnormal placenta spiral arteries causes vasoconstriction

headache, scotoma, oliguria, thrombocytopenia
Term
eclampsia
Definition
preeclampsia + seizure
can cause intrancranial hemorrhage and ARDS
TX: anti-HTN, IV MgS, delivery
Term
HELP syndrome
Definition
hemolysis
elevated liver enzymes
low
platelets

severe preeclampsia
TX: delivery
Term
adenomyosis
Definition
extension of endometrial glands into myometrium due to basalis hyperplasia
large, soft, globular uterus
Term
adenomyoma
Definition
endometrial polyp, may contain myometrium
Term
3 vaginal tumors
Definition
1. SCC, usually secondary to cervical
2. clear cell, mom took DES
3. sarcoma botryoides, rhabdomyosarcoma, <4yo, spindle shaped tumor cells desmin +
Term
inflammatory breast tumor
Definition
dermal lymphatic invasion by carconoma
peau d'orange
blocks lymphatic drainage
Term
acute mastitis: cause, treatment
Definition
breast abscess during breast feeding via S. aureus
DX dicloxacillin
Term
BPH vs prostic adenocarcinoma
Definition
BPH: middle and lateral lobes, frequency, nocturia, difficulty starting and stopping pee, PSA

adenocarcinoma: posterior lobe, PSA, osteoblastic metastasis, ALP
Term
buzz word association..
1. tet spells
2. wide fixed S2 split with loud S1
3. turner syndrome heart
4. radiofemoral delay
5. corneal arcus
Definition
1. tertalogy of fallot
2. ASD
3. infantile coarctation of aorta
4. adult coarctation of aorta
5. lipid deposit on cornea with hypercholesterolemia
Term
buzz word association...
1. diabetic mother infant
2. down syndrome heart
3. rubella heart
4. string of beads
5. mediastinal widening
Definition
1. transposition of the great vessels
2. ASD, VSD, endocardial cushion defect
3. PDA, pulmonary artery stenosis
4. fibromuscular dysplasia
5. aortic dissection
Term
MI complications
Definition
arrhythmia: #1 COD before hospital
LV failure/pulmonary edema
cardiogenic shock: #1 most fatal
ventricular wall rupture
papillary muscle rupture
septal rupture
ventricular pseudoaneurysm formation
pericarditis fibrinous
dressler syndrome: autoimmune fibrinous pericarditis
Term
3 cardiomyopathies
Definition
dilated: #1, alcohol, beriberi, coxackie B, cocaine, chagas, doxorubicin. S3, BALLOON HEART

hypertrophic: familial B-myosin mutation, dredreich ataxia, SUDDEN DEATH IN YOUNG ATHLETES, S4

restrictive: sarcoidosis, amyloidosis, fibrosis, loffler syndrome (eosinophils), hemochromatosis). LOW VOLTAGE ECG THICK MYOCARDIUM
Term
buzz word association...
1. nutmeg liver
2. oslers nodes, janeway lesions, splinter hemorrhages
3. #1 heart tumor
4. kussmauls sign
Definition
1. right heart failure
2. acute endocarditis
3. myxoma (ball valve in LA, syncope)
4. constrictive pericarditis, restrictive cardiomyopathy, RA or RV tumor
Term
general info of vascular tumors...
1. strawberry hemangioma
2. cherry hemangioma
3. pyogenic granuloma
4. cystic hygroma
5. glomus tumor
Definition
1. benign, capillary, infant, regress 5yo
2. benign, capillary, elderly, no regreess
3. capillary, ulcerate and bleed, trauma, pregnancy
4. cavernous, neck, turner syndrome
5. painful, red-blue, fingernail, smooth muscle cells of glomus body
Term
vascular tumor gener info...
1. bacillary angiomatosis
2. angiosarcoma
3. lymphangiosarcoma
4. kaposki sarcoma
Definition
1. benign, capillary, AIDS, bartonella henselae
2. head, neck, boob, sun
3. lymphedema, post-radical mastectom
4. skin, mouth, GI, respiratory, HHV-8, HIV
Term
buzz word association...
1. fatty casts
2. waxy casts
3. stones in chrons
4. coffin lid stone
5. envelope/dumbbell stone
6. rhomboid or rosette stone
7. hexagon stone
8. WAGR
9. squamous bladder metaplasia
10. thyroidation of kidney
Definition
1. nephrotic syndrome
2. renal failure
3. Ca oxylate kidney stones
4. ammonium MgP renal stones (struvite)
5. calcium stone
6. uric acid stone
7. cysteine
8. wilms tumor, anridia, GU malformation, retardation
9. schistosoma haematobium, smoking
10. chronic pyelonephritis
Term
buzz word association...
1. homan sign
2. ivory pleural plaques
3. ferruginous bodies
4. egg shell calcification of hilar nodes
5. kulchitsky cells
6. chromogramin A
7. keratin pearls, intracellular bridges
Definition
1. dorsi flexion of foot DVT
2. asbestosis
3. asbestosis, dumbells, gold-brown
4. silicosis
5. dark blue cells in SmCC
6. bronchial carcnoid lung tumor
7. lung SCC
Term
3 areas of lymph node
Definition
follicle: B cell proliferation, germinal centers

medulla: cords (lymphocytes and plasma cells), medullary sinuses (communicate with lymphatics and have macrophages)

paracortex: T cells, middle region, has venules so T and B cells can enter blood
Term
T cell development and function
Definition
marrow: T cell precursor

thymus: CD4+CD8+ > positive selection in cortex, negative selection in medulla > become CD4+ OR CD8+

activation: APC (B cell, macro, dendritic) activates by presenting antigen on MHCII to TCR on Th cell, then B7/CD28 signaling

class switching: Th cell binds B cell on CD40 and tells it to class switch
Term
antibody structure and diversity
Definition
FAB: antigen binding, determines idiotype
FC: carboxy, complement binding, determines isotyoe (IgM, G, etc)

diversity is caused by: VJ (light chain) VDJ (heavy chain), heavy chain recombination with light chain, SOMATIC HYPERMUTATION after antigen stimulation
Term
Ig types and main facts
Definition
IgG: #1 in delayed response, most abundent, crosses placenta, opsonizes

IgA: mucous membranes, tears, saliva, breast milk (colostrum)

IgM: #1 immediate responder, on surface of B cells, large

IgD: on B cells

IgE: on mase cells and basophils, allergen, HSNI, histamine, eosinophils, helminths
Term
main problem in each deficiency...
1. C1 esterase inhibitor deficiency
2. C3 deficiency
3. C5-9 deficiency
4. DAF (GPI anchor) deficiency
Definition
1. hereditary angioedema
2. pyogenic URI infections, HSN III
3. neisseria infections
4. complement lysis of RBC (paroxysmal noctournal hemoglobinuria)
Term
live attenuated vaccines
Definition
MMR
polio sabin
influenza intranasal
varicella
yellow fever
Term
ARDS: criteria, most common cause, mechanism
Definition
damage of alveolar capillaries and leaking protein into lung causes hayline membranes leads to rapid fibrosis
gram negative sepsis
temp >100.4 or <98.6
HR >90
RR >20 or low CO2 (resp alkalosis)
WBC <4000 or >12000 or >10% bands
Term
DOC and MOA actinomyces israelii
Definition
penicillin (gram positive rod anaerobe)
inactivating PBP which stop crosslinking in cell wall
Term
DOC bordatella pertussis
Definition
erythromycin
Term
DOC coxiella burnetti
Definition
doxycycline (causes Q ever, pneumonia, hepatitis, myocarditis)
Term
DOC nocardia asteroides
Definition
TMP/SMX (aerobe causes lung abscess)
Term
obligate anaerobes lack ____ and ____
Definition
catalase
superoxide dismutase
Term
dopamine antagonists
Definition
cabergoline
bromocryptine
Term
what drugs lower LES pressure causing GERD
Definition
anything that decreases excitatory cholinergic input that induces NO

nitrates, CCB, a-blockers, B-agonists
Term
1. cross sectional study
2. randomized controlled trial
3. chohort study
4. meta analysis
Definition
1. collect data from 1 group of people to assess prevalence of disease and risk factors, fast inexpensibe, no follow up

2. studies tx effecacy, control and tx groups, gold standard for mature research

3. observational, two groups chosen based on exposure status, no follow up

4. gather info from already completed studies and form overall conclusion
Term
rubeola signs
Definition
cough, coryza (cold like), conjunctivitis, koplik spots, rash starting on face spreads down (gone 2d before measles)
Term
signs of excess GH
Definition
aceomeagly, increased insulin, colon polyps, micrognathia (enlargement of face, hands, feet), microglossia, hyperhydrosis, prognathism, arthritis
Term
signs of GH deficiency
Definition
altered lipid metabolism, increased SC fat, decreased muscle mass, decreased bone density, decreased exercise performance, low energy, poor memory, impaired sleep, emotional instability, hypoglycemia, impaired glyconeogenesis
Term
choices ok for HTN in pregnancy
Definition
labetalol and hydralazine are DOC
nifedipine and methyldopa are ALT
Term
what drug causes cyanide poisoning, what is its use
Definition
nitroprusside, HTN
Term
risks of preeclampsia
Definition
placenta abruption
acute renal failure
pulemonary edema
DIC
eclampsia
Term
signs of Mg sulfate toxicity and treatment
Definition
loss of DTR, respiratory paralysis, come
TX calcium gluconate
Term
what are the 3 spaces of the shoulder, their borders, and contents
Definition
quadrangular: teres minor, humerus, teres major, long head of biceps. AXILLARY NERVE, POSTERIOR CIRCUMFLEX ARTERY

triangular interval: teres major, long and short head of the biceps. PROFUNDA BRACHII A AND RADIAL NERVE

triangular space: teres major, teres minor, long head of biceps, CIRCUMFLUX SCAPULAR A
Term
DOC latent TB, MOA
Definition
isoniazid, blocks mycolic acid synthesis
Term
types of shock
Definition
obstructive: tension pneumothorax, large PE

adrenal shock: nausea, fever, vomiting, confusion, hyperkalemic hyponatremia metabolic acidosis

distributive: AV shunt decreases TPR, high CO, hypotension, high PP, low diastolic pressyre, often septic shock

hypovolemic shock: fluid loss, dehydration, vomiting, low CO, high TPR

neurogenic shock: spinal cord or CNS injury, trauma, bradycardic, hypotensive

cardiogenic: LV failure
Term
associations with type A and O blood
Definition
A: gastric tumors
O: peptic ulcer disease
Term
subarachnoid hemorrhage
Definition
worse headache of life
ruptured berry aneurysm (HTN
Term
DOC, growth media legionella pneumophila
Definition
levofloxacin/flouroquinolones, buffered charcoal yeast extract agar, silver stain for sputum
Term
most common cause of bacterial parotitis
Definition
S. aureus
Term
cervical ligaments
Definition
alar: odontoid to foramen mangnum
cruciform
-transverse: C1 to dense posterior
-superior band: transverse to occiput
-inferior band: transverse to C2
tectoral membrane: superior continuation of posterior longitudinal ligament
anterior longitudinal ligament
Term
cervical cause of headache
Definition
cervical dysfunction irritates greater occipital nerve
suboccipital muscles tight in tension headahce
Term
changes in cervical spine after wiplash, best treatment
Definition
C2-7 sidebent and rotated opposite
indirect fascial or counterstrain to prevent further spasm
Term
cervical cause of sinusitis
Definition
suboccipital muscles interfere with venous drainage
Term
most common cause of nerve root pressure in the cervical spine
Definition
uncinate processes on joints of luschka / uncovertebral joints degenerate and hypertrophy with arthritis and are close to intervertebral foramen
Term
diagnosis of cervical stenosis vs disc herniation
Definition
spurlings: if backward bending makes symptoms worse it is stenosis

forward bending makes herniation worse
Term
location of cervical sympathetic ganglia
Definition
inferior: ventral 1st rib
middle: C6
superior: C2-4
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