USLME - Grab Bag Flashcards
Hypertrophy - types of cells that only do this (3)
Growth of cell through organelle production
- cardiac, skeletal, nerve
Hyperplasia
new cells from a stem cell
Dysplasia
disordered cell growth
also reversible
atrophy
decrease in stress -> decrease in organ size
Both:
apoptosis - lower cell #
smaller size - ubiquitin proteosome degradation and autophagy
Metaplasia
change in stress -> change in cell type
Reversible
chronic-> dysplasia
apocrine metaplasia of breast only one not increased risk for cancer
Aplasia
failure of cell production - embryogenisis
Hypoplasia
decreased cell production - embryogenesis
Ischemia
decreased blood flow
hypoxemia
low partial pressure of O2 in the blood
PaO2 <60mmHg
Coagulative necrosis - What is it and seen in?
necrotic tissue remains firm, cell structure is preserved
```
ischemic infarction (wedge shaped) - pale
except brain
~~~
liquefactive necrosis - What is it and seen in(3)?
necrotic tissue becomes liquified, enzymatic lysis
Brain infarct
abcess
pancreatitis
Gangrenous necrosis - what? and seen in (2)?
Resembles mummified tissue -
Ischemia of lower limp and GI
Caseous necrosis - what? and seen in (2)?
soft friable - cottage cheese
combination of coagulative and liquefactive
Seen in TB and fungal infections
Fat necrosis - what? and seen in(2)?
necrotic adipose w/ chalky white appearance w/ Ca deposit
Trauma to fat and pancreatitis mediated damage
Ca deposits on dead tissue - dystrophic calcification and normal serum [Ca] vs. metastatic which has high serum [Ca]
Fibrinoid necrosis - What? and seen in(2)?
necrotic damage to blood vessel - leaking proteins into vessel wall (pink)
Malignant HTN and vasculitis
PAS staining - periodic acid schiff
used to detect polysaccharides such as glycogen, and mucosubstances such as glycoproteins, glycolipids and mucins in tissues
Psommona Body tumors (4)
Papillary carcinoma of thyroid
Meningioma
papillary serous endometrial carcinoma
mesothelioma
Priaprism
Painful errection > 4 hrs
- tazadone, hydralazine, prazolin, chlorpromazine, PDE inhibitors, alprostadil SE
Neural crest cell derivatives
PNS Melanocytes chromaffin parafollicular cells schwann cells pia/arachnoid nines of skull odontoblasts aortiocpulmonary corner
Mutation in HOXD13 lead to
Caused by?
synpolydacyly
retinoic acid
desmoplasia
excessive formation of fibrous tissue, seen around some tumors
Type 1 collagen
Strong
-bONE, skin, tendons, scar tissue
defective in ostogenesis imperfecta
Type 2 Collagen
Slippery
-cartilage, CarTWOlage, vitreous body, nucleus puposis
Type 3 Collagen
Bloody
-Reticulin, skin, blood, vessels, granulation tissue(beefy red)
Type 4 collagen
Basement membrane
defective in alport syndrome
Vitamin C is necessary for what in collagen
hydroxylation of lysine and proline
AA necessary for collagen formation(3)
Glysine, lysine and proline
-latter 2 are hydroxylated
AA for elastin production(2)
Glysine and proline
Ehlers Danslos syndrome is characterized by and due to?
Classic -hyper extensible skin and hyper mobile joints
-AD mutation in alpha chain of collagen and cross linking
Vascular - Easy bruising and bleeding disorders
-Auto recessive mutation
Hereditary nephritis, cataracts and sensoorineural hearing loss
Alport syndrome
- due to Type IV collagen defect
hyperflexible, arachnodactyly, aortic dissection, lens dislocation
Marfans
- defect in fibrillin scaffolding
Connection between elastin and emphysemia
alpha 1 antitripsin deficiency does not protect from elastase breakdown of elastin
-defective alpha 1 antitrypsin also builds up in the liver -> hepatitis
Mutliple fractures at different stages seen on x ray think (2)
Osteogenisis imperfecta - look for blue sclera and hearing loss and dental imperfections (defect in Type 1)
Child abuse
HAVe 1 M&M
Gq receptors w/ increased Ca as a result
phospholipase C-> PIP2 split into DAG and IP3
DAG-> protein kinase C
IP3 -> increased Ca intracellularly and smooth muscle contraction
Renal cell carcinoma paraneoplastic
PTHrP -> hypercalcemia w/ excess PTH
Erythropoietin -> polycythemia
Aspiration of peanut goes where?
upright and supine
lower portion of R inferior lobe- upright
superior portion of R inferior lobe -supine
-exits superior off the bronchi
Metformin is contraindicated when?
Renal failure - due to lactic acidosis adverse effect
Glipizide instead (not if hepatic dysfunction)
Hemolytic anemia in a neonate w/ + coombs suggestive of?
ABO or Rh incompatibility
See jaundice
Coombs reagent is?
Anti human IgG
indirect -> testing serum, normal RBC-> see if all 3 react
direct-> testing for Ab on RBCs in serum and see agglutination
Winged scapula injury?
long thoracic nerve
Acetazolamide causes what metabolic dysfunction?
Acidosis w/ inhibition of carbonic anhydrase you have local diuretic loss of bicarb w/out replacement
- counter indicated in any acidosis
nerve exits from the spinal cord location?
C1-C7 above its vertebrae
C8, T1-12, L1-5 below its corresponding vertebra
Structure responsible for bone growth post fracture
periosteum containing osteoblasts
alpha fetoprotein tumor secretors(3)
heptaocellular carcinoma
yolk sac
nonseminomatous germ cell tumors
efficacy
Km and Vmax
maximal effect a drug can produce
-directly related to Vmax, NO effect on Km
potency
Km and Vmax
amount of drug to achieve a given effect
- high potence= high receptor affinity, inversely related to Km
kernicterus
neo natal high bilirubin levels -> brain dysfunction
ex- sulfonamides in pregnancy
Abx to avoid in pregnancy (8)
sulfonamides - kernicterus aminoglycosides - ototoxicity fluoroquinolones - cartilage damage Clarithromycin - embryotoxix tetracyclines - teeth ribavirin - teratogenic griseofulvin - teratogenic chloramphenical - grey baby
women’s oocytes are arrested in what stage for most of her life
prophase 1
Metaphase 2 right before conception
Poor prognosis cancers
Pancreatic adenocarcinoma
esophageal adenocarcinoma
MEN Type I tumors (3)
Pituitary - prolactin or GH
Parathyroid
Pancreatic endocrine - Zollinger Ellison, insulinomas, VIPoma,
Angiofibromas and lipomas common
“the diamond”
delerium Tremons
Seen in severe alcohol withdrawal w/ autonomic hyperactivity
presentation of failure to thrive, steatorrhea, acanthocytosis (Spiculated RBCs), ataxia and night blindness is?
Due to?
abetalipoproteinemia
Due to defect in microsomal tryglyceride transfer protein gene
inability to add B48 and B100 on VLD and chylomicrons to export out
Best way to test for chronic alcohol in the system
Serum gamma gutamyl transpeptidase (GGTCP)
Tox screen - acute alcohol levels
deficiency in arysulfitase A
metachromic leukodytrophy
accum of cererbroside sulfates
deficiency in beta-glucocerebrosidase
Gaucher disease
-> springolipidosis
Deficiency in beta-galactocerebrosidase
Krabbe disease
-> springolipidosis
Deficiency in sphingomyelinase
Niemann Pick disease
cherry red spots w/ Tay Sachs
Deficient in hexosaminididase A
Tay Sachs
Cherry red w/ Niemann Pick
t(11:22)
Ewing Sarcoma
Onion skin appearance on bone - w/ annaplastic small blue cell malignant tumors
Boys less than 15 yrs
yellow testicular tumor in young males?
Follow what marker
yolk sac tumor
follow AFP
Glycogen storage disease w/ cardiomegaly and other systemic findings (weakness w/ skeletal muscle)
implicated enzyme
Pompe
lysosomal alpha 1-4 glucosidase
Start a patient on anticoag (maybe post PE or acute coronary) and see a thrombocytopenia
Stop what drug and replace w/
Heparin due to HIT
lepirudin or bivalirudin
- does not use platelet factor 4 and have IgG activate against the complex
ixoide tick carried
borrelia burgidorferi
babesia microti
dermacentar tick carries
franciella tularnsis
rickettsia rickettsii
See a PE think of as a cause
DVT
like from the femoral vein
Bacteria associated w/ transformation
SHiN
Strep pneumo
H. influenza
Neisseria
Don’t forget if a kid presents w/ weight loss
may be diabetic and not bulimic
look at glucose levels
Presentation of seizures, renal, fever, thrombocytopenia, microangiopathic hemolytic anemia, w/ schistocytes and purport
Def in ADAM 13 cleavage
Thrombotic thrombocytopenic purpura
seen in younger women
lack of Fibrin split products and normal PT and PTT r/o DIC
very similar to HUS but here minus the mental ∆
Adenoyosis looks like endometriosis in presentation except
the uterus is enlarges,
endometrium w/in the myometrium
Muscle responsible for protrusion of the tounge
genioglossus
3 oncogenes associated w/ tyrosine kinase
HER2-neu
abl
ret
glalactose 1 phosphate uridyltransferase deficiency - presentation
similar to what other disease in theory
Auto recessive Galactosemia - STOP milk
FTT, jaundice, hepatomegaly, cataracts, retardation, Vomitting, diarrhea
Similar to aldose B deficiency and fructose - losing phosphates on sugars that can’t be utilized
2 virus families w/ reverse transcriptase capabilities
hepadnavirus - Hep B
Retrovirus - HIV
uses a RNA dependent DNA Polymerase
hemochromatosis presentation?
Bronze diabetes
- skin pigmentation
- DM
- Cirrhosis
prussian blue stains for Fe
infected RBCs are cleared by?
NK cells due to lack of MHC I
HSV encephalitis affects which lobe
temporal
soap bubble lesions in the brain?
cause and type?
Test w?
Monomorphic yeast
cryptococcous neoformans
lattex agglutination 1st
india ink not as sensitive (heavily encapsulated)
most common cause of death before reaching the hospital w/ Acute MI
arrhythmia
V fibrillation w/in 24-48 hrs
prosopagnosia
inability to recognize faces
bilateral lesion of visual cortex
Na stibogluconate
Rx for Leishmania donovani
Carried by the sandfly
Triad of mono?
histo feature
Fever
Pharyngitis
LAD
CMV or LAD
Downey or atypical cells (C8 in origination though look like B cells)
Products seen in fasting?(2)
actetoacetic acid
3 betahydroxybutric acid
FAP is associated w/ mutation on chromosome?
5q21
CEA levels are elevated in ?
Colorectal cancer - not a good screener, great for monitoring
Seen in pancreatitis also
Hemophilia A due to
Hemophelia “Aight”
VIII deficiency
B is 9
Initiating factors of the coat pathway
Intrinsic ->
Extrinisic
Sub endothelial collagen
- Factor 12, 11, 9, 8
Tissue factor release by endothelial
Factor 7
All meet at 10
Goal of Coag pathway ?
make thrombin
which catalyzes the transformation of Fibrinogen to fibrin
Plasin (from plasminogen) breaks it up
Low K effects which coag factors
2, 7, 9, 10
7 being key for extrinsic -> PT and INR in warfarin monitoring
D dimer vs fibrin degeneration products?
D dimer is specific to clots made w/ thrombin
- activity of both thrombin and plasmin must be present
FDPs come from fibrin (mainly and circulating fibrinogen precursor)
Importance of conjugate vaccines?
provides a a protein antigen to facilitate the conversion of IgM to IgG w/ T cells stimulation
polysaccharide only gets IgM
hyper pigmented mouth, lips, palms, soles and skin w/ harmatomas, crampy abdominal pain at increased risk for?
AD Peutz jeghers
Colorectal cancer and other visceral malignancies (pancreatic etc.)
weird barbiturate name?
thiopental
Wide QRS may indicate (3)
signal not from purkinje:
- premature ventricular contraction (PVC)
- ventricular tachy
- bundle branch block
narrow QRS indicates though the conduction system no matter what path or normal pays may be causing it
normal little box on ECG is
0.04sec
5 little boxes or 1 big box is?
Associated path if larger?
0.2 sec
If PR interval is bigger may have primary heart block
Normal PR interval
less than 200 msec (1 big box)
normal QRS interval
less than 120 msec (3 little boxes)
Common causes of L axis deviation (5)
inferior wall MI L anterior fasicular block L ventricular hypertrophy high diaphragm LBBB
Common causes of R axis deviation (5)
R ventricular hypertrophy Acute R heart strain (PE?) left posterior fasicular block RBBB dextrocarida
Positive deflection in ECG means?
Normally travels?
that the QRS is more above the line vs below (+) in the lead given
Normally travels down and to the left
Example positive in L I and LII normally
Lead where it is never normal to have positive deflection?
aVR
Target cells seen in?(4)
Thalassemia
hemoglobin C
Liver disease
asplenia - (sickle cell eventually)
Mutation of dystropin is found where
on the X gene-
need both for a girl(higher incidence in turner syndrome), one for a guy (recessive)
hernia travels through the inguinal canal through both deep and superficial rings and is lateral inferior epigastrics
Indirect hernia
hernia that only goes through the superficial ring by tearing a hill in the weak part of the abdominal wall in inguinal triangle medial to inferior epigastrics
direct hernia
stones, bones and groans presentation?
osteritis fibrosa cystica due to primary hyperparathyroidism
stones - renal stones
bones - increased alk phis and cystic spaces in the bones
groans- constipation/ weakness
Adductor pollicis inervated by?
Ulnar nerve
Lumbricals innervated by?
Lateral 2 median
Medial 2 ulnar
Interosseous muscle innervated by?
Ulnar nerve
- digital abduction and adduction
flexor pollicis brevis inervated by?
median nerve
Carpol tunnel and have them make an O w/ pinky and thumb
- thumb opposition
3 Paraneoplastic seen in small cell carcinoma
ACTH -> cushing
ADH -> SIADH
Abx against presynaptic Ca Channels -> lambert eaten syndrome
FeNa less than 1%
prerenal cause (hypovolumeia)
FeNa >2%
Intra renal cause like acute tubular necrosis
Normal BUN:creatine ratio
~15:1 usually
pathological >20:1 thinking prerenal causes
less than 15:1 interregnal issues like acute tubular necrosis
could also be causes w/ out flow like BPH leading to increased reabsorbtion of BUN into the blood
Increased aldosterone like in Conn syndrome leads to what metabolic disorder and why
metabolic alkalosis
increased aldosterone -> increase in K secreting channels in the cortical ducts in addition to eNaC Channels sequestering Na from the lumen
The K is lost but some of the K in the lumen also stimulates the loss of H as eel (alpha intercalated cells a little later on) -> H and K loss and alkalosis
Chronic renal failure can lead to this hormone imbalance?
Rx?
secondary hyperparathyroidism
due to impaired phosphate excretion and ultimately hypocalcemia
Calcimimetrics -> Cinacalate: increases sensitivity of PTH Ca sensing receptors decreasing PTH levels
Cinacalate?
increases sensitivity of PTH Ca sensing receptors of chief cells of parathyroid decreasing PTH levels
for secondary hyperparathyroidism
increasing the radius of a vessel drops resistance?
1/ (r^4)
Pure mucinous gland in the mouth?
Sublingual
- sub mandibular mix
parotid - serous
Supracondylar fracture damages what nerve?
seen w/ what deviation of the wrist?
median nerve
innervates flexor carpi radialis
flexor carpi ulnaris is unopposed (Ulnar nerve) leading to ulnar deviation
endocarditis in SLE
aseptic vegitations
libman-sacks endocarditis
LPL enzyme is found where?
endothelial cells
In addition to bleeding what other toxicity is associated w/ Warfarin (2)
skin/tissue necrosis w/ protein C deficiency(hypercoaguable and microvascular thrombosis)
-> sharp, demarcated erythematous, purpuritic
Drug- drug
lymph node biopsy shows large binucleated cells w/ prominent nucleoli and eosinophilic cytoplasm
Hodgkins
Key feature of PTSD
disturbance lasting > 1 month
-Regardless of symptoms
Acute stress disorder 2 days - 1 month
DVT predisposed by (3)
Stasis
hypercoagulability - defects in cascade (factor 5 - pregnant)
endothelial damage - exposed collagen
Vitamin K’s function?
Synthesized where?
gamma carboxylation of glutamic acid
synthesized by intestinal flora, risk w/ broad spectrum Abx long term
Lactulose given for?
MOA?
hepatic encephalopathy -> increased NH3 w/ urea cycle failure
reduces NH3 absorption from GI due to bacteria breaking it down and making it an acidic environment trapping NH3 and NH4+
Cardiac amyloidosis w/out other organs involved?
transthyretin (TTR) of primary amyloidosis
Fibrinoid necrosis can be seen in
- Fuck you pathoma (4)
Malignant hyper tension
preeclampsia
Vasculitis
Rheumatoid arthritis (w/ palisading epitheliod cells)
Hypertension and BPH think of using a?
Alpha 1 antgonist
Prazosin/Terazosin
metabolic alkalosis is a common problem w/ these drugs? (2)
Loop dieuretics - hyponatremia -> aldosterone and loss of K/H directly by alpha intercalated
thiazide diuretics - volume depletion -> aldosterone
axillary nerve runs w/ what artery?
Posterior Circumflex
See homovanillic acid, vanillylmandelic acid and metanephrine in the urine think of
Pheochromocytoma
COMT, MAOI -degradation products of
Dopamine
NE
Epi
CD14 found on
also has
Macrophages
CD40
CD 56 found on
also has?
NK cells
CD16
CD 28 found on
T cells
Most common cause of neonatal meningitis?
Streptococcus agalactia
- E coli 2nd
-listeria - 3rd
-
associated maladsorption complication w/ CF due to
deficiency in fat soluble vitamins ADEK w/ lack of lipase from the pancreatic duct
tender thyroid
cause?
Dequervians, sub acute throiditis,
- post viral
presents symptomatically like Hashimoto’s otherwise
+ combs test and hemolytic anemia >37 degrees C
which antibody?
means warm agglutinin - will see hyperbilirubinemia, unconjugated
Found in SLE, drugs and other auto immune
IgG
IgM against RBCs characteristic of
Cold agglutins w/ + combs at cold temps
Mycoplasma pneumonia or lymphoma cause
Immediate hypersensitivity
Type 1
Cytotoxic disease/hypersensitivity
Type 2
Delayed hypersensitivity
Type 4
Arthus Reaction
Type 3
eruptive xanthomas
yellow papules due to elevated tryglycerides
Rx for pregnant HTN (2)
how does it work?
Worry when diastolic > 100mmHg
Clonidine
-> direct alpha 2 agonist
alpha methydopa
-> converted to alpha methynorepiniphrine -> alpha 2 agonist
Known ethambutol complication
neurotoxin -> optic neuropathy
TB drug
non infectious cause of hand-foot erythema and desquamatizing rash
Concerned w?
Kawasaki on kids < 5 Also see -Cervical LAD -Fever* -conjunctival injection /bilateral -strawberry tongue/ pharyngeal erythema
concern of coronary aneurisms
hyperventilation leads to what changes in
pH?
vascular diameter
increases pH blowing off CO2 - respiratory alkalosis
Less CO2 -> less vasodilatation -> constriction
- lungs are an exception
Only DNA virus family that contains its own DNA dependent RNA polymerase(iron associated transcriptase)
Poxvirus
brick shaped complexes
Serum Marker for oxygen saturation
Lactic acid level
topical retinoid acid use and HA due to
pseudotumor cerebri
Vitamin A toxicity, tetracycline, corticosteriods have been implicated
Direct vs indirect Combs test
Direct Combs test is testing the blood for anti RBC antibodies attached - for example, the babies blood if they are undergoing hemolytic anemia
Indirect combs is testing the serum w/ test blood for the presence of anti RBC antibodies, if they cause clumping, example the mother who is Rho - and is concerned of IgG presence
painful sharply demarcated erythematous rah 3-10 days after atria fibrillation and Rx is started be concerned of
Warfarin skin necrosis and microthromboemboli with initial hypercoaguability and KO of protein C and S at first
may present as painful red rashes
associated immune compromised state w/ albinoism
Chediak Higashi Syndrome
Impaired microtubule function leading to NK cell disruption and other phagocytic cells
Hemagglutin
what our body makes antibodies to in the flu, necessary for viral attachment
the h in h1n1
n is neuaminidase
CD18
Implicated in leukocye adhesion deficiency
Lack of integrin -> increased infection and delayed wound healing
delayed separation of umbilical stump
stapedius is innervated by?
CN7
dampens sound w/ loud noises,
damage leads to hyperacusis
Cells that are CD30 and CD15 positive are
Reed Sternberg cells
seen in hodgkins lymphoma, usually no CD20
Tartate resistant acid phosphatase
TRAP
Used to stain for hairy cell leukemia
ADPCKD is associated with what major complication and treated w/ what
increased renin -> HTN
Rx w/ ACE or and ARB
Gastric hypertrophy w/ protein losing enteropathy is?
What is seen on histology?
Menetrier’s disease
Increased mucous cell(excess mucous production) and and decrease in parietal cells
Rugae of stomach hypertrophied
Renal Protective HTN medication in diabetes
Ace Inhibitors
- just don’t use in bilateral renal stenosis
Retinoblastoma caused by a mutation on what gene on what chromosome
Rb1
13
gene implicated in osterosarcoma as well
Perfusion limited gas example and look of the graph
CO2 and N20
The graph reaches alveolar pressure early along the length of the capillary. limited by the amount of blood flowing through
Diffusion limited gas example and look of the graph
CO and O2 in a diseased state (emphysema/fibrosis)
The graph of the partial pressure in the capillary does not reach alveolar pressure and is dependent. There is a difference
3 damn Pyruvate enzymes what are they- what are their products
Pyruvate kinase
Pyruvate dehydrogenase
Pyuvate Carboxylase
PEP -> Pyruvate
Pyruvate -> acetyl Co A
-needs thiamine
Pyruvate -> oxaloacetate (gluconeogenesis) -> PEP and eventally glucose via PEP carboxykinase (weird little circle)
Biotin needed
Medication for GAD w/ no sedation, addition or tolerance effects
Acts on what receptor
Buspirone
(not buproprion/welbutrin)
- takes 2 weeks to work
5HT1a agonist
Diuretic that retains Ca
Dieretic that loses Ca
Loops lose Ca
Thiazides retain (hypercalcemia w/ PTH hyperexpression)
Pyruvate kinase deficiency presents as?
hemolytic anemia due to increased rigidity of RBCs
May be seen in a newborn
Auto recessive
young patient comes in with mousy odor, mental retardation and eczema
PKU
Auto recessive
Polyhydraminos is due to?(2)
lack of fetal swallowing
- anencephaly
- intestinal blockage(atresias…)
- diaphragmatic herniation
increased urination
Most common dislocation of the humorous?
anterior
vitilago vs albinism
decreased # of melanocytes in vitilago vs decreased melanin production w/ albinosim (less tyrosinase activity)
secondary hyperparathyroidism is seen when?
Due to?
Seen in chronic renal failure due to:
Decreased renal 1 alpha hydroxylase making active D3
Decreased excretion of phosphate -> high PTH w/ low Ca levels
Vitamin A is used to treat (3)
Measles
acne
AML - M3
Ground glass appearance of HBV
fine cytoplasmic protein granules
most common lasting sequel of herpes zoster
post herpatic neuralgia
- can be upwards of a month
thiopental rapidly redistributes to?
adipose tissue and skeletal muscle
its a barbiturate used for induction of anesthesia
Pellagara deficiency presents as?
Can be prevented with?
Diarrhea, dermatitis and dementia
due to B3 (niacin deficiency). B3 is a constituent of NAD and NADP (derived from tryptophan if made endogenously, also can come from diet)
Which organ suffers the least risk of infarct due to a thrombi?
Liver - dual blood supply
infection that sickle cell patients are especially susceptible too
Salmonella and osteomyolitis(occlusion -> necrosis -> colonization)
- special capsule that prevents obsinization and vaccination
functional autosplenectomy in sickle cell
Vitamin overdose that leads to these symptoms-> loss of appetite, stupor and hypercalcemia
Similar to what pathology otherwise ?
Vitamin D
similar to Granulomatous diseases - sarcoidosis and TB w/ hypercalcemia secondary to high levels of calcitriol (active Vit D)
excitatory neurotransmitter involved in pain
substance P
lysosomal storage disease associated w/ renal failure
Fabrys disease
- X linked w/ peripheral neuropathy, angiokeratomas and CV disease as well
alpha glactodidase A is deficient
lysosomal storage disease associated w/ bone crisis and macrophages that look like tissue paper
Gaucher’s disease
Glucocerebrosase deficiency
Auto recessive
suprachondylar fracture going to effect what nerve?
median nerve
immune deficiency problem of endoderm development
Digeorge syndrome
Immune deficiency due to tyrosine kinase defect
Agammaglobulinemia
BTK gene (tyrosine kinase gene)
conversion disorder
neurologic affliction of voluntary motor or sensory (pain, optic, paralysis etc) in the presence of stressor and no medical findings can confirm it. it cannot be limited to pain or sexual dysfunction
There is no secondary gain with the loss of function as well.
Somatization form - multiple physical complaints
somatization disorder
numerous complaints starting prior to the age of 30
4 pain
2 GI
1 sexual
1 pseudoneurologic symptom
No medical findings
rose spots on the abdomen and abdominal distress w/ hepatosplenomegaly, hemhorragic enteritis
salmonella typhi
chamber of the heart laying next to the esophagus
Left atrium
Where is the descending aorta in relationship to the esophagus?
behind
herpes viruses are the exception to the rule in where they get their envelope for their membrane. Where is it from
nuclear membrane
CD surface marker that inhibits complement from binding
CD 55 and CD 59
CD surface markers found on Reed Sterberg Cells
CD15 and CD30
deficiency in vWF will lead to what ∆s in lab values?
increased bleeding time mainly b/c it facilitates platelet aggregation/adhesion to GP Ib–1X
wWf also carries factor VIII increasing its half life so ultimately prolonged PTT if deficient
Defect in delta aminolevulinic acid dehydratase (ALA dehydrastase) may be due to?
Accumulate?
What other enzyme is affected leading to basophilic stppiling
lead poisoning
protoporphyrin and delta ALA in the blood
Lead also blocks ferrochelatase -> blocking heme production
Acute intermittent porphyria enzyme defect and presentation (5)
defect in porphobilinogen (3rd step in heme synth)
painful ab port wine -colored urine polyneuropathy psychological disturbances precipitated by drugs
2 enzymes inactivated by lead
ferrochelatase -> final step in heme
delta-aminolevulinic acid dehydrates
- 3rd step in heme synthesis
(excess delta ALA in the serum)
ultimately microcytic anemia
Myoglobin construction and oxygen response
Made of only one monomer of glob in vs hemoglobin made of 2 usually (2 alpha and 2 beta)
the presence of only one glob in -> lack of positive cooper ability (either all on or all off) NOT sigmoidal hemoglobin sat oxygenation curve (relative left shift)
Patient w/ managed hyperthyroidism comes into a clinic with fever and throat pain. What extra step should be done and why?
WBC and differential due to agranulocytosis caused by propylthiouracil and methimazole
seronergic neurons are found where in the brain stem
ralphe nucleus
Patent foramen ovale occurs in what % of adults
20-30%
considered a variant of normal
-Functionally closed w/ high L atrial pressure vs R
-> paradoxical embolism w/ strokes and DVTs
History of RA w/ no alcohol Hx presents w. fatigue, R abdominal pain; labs show high ANA and ASMA, elevated IgG and no viral markers
autoimmune hepatitis
ASMA = antismooth muscle Ab- Type 1
Liver biopsy shows elevated LKM-1 antibodies, no alcohol Hx, no viral seromarkers. See infiltration of the portal and periprotal area w/ lymphocytes
autoimmune hepatitis
LKM-1 = liver/kidney microsomal Ab - Type II
Substance needed for relaxation of the LES
Nitric Oxide
Adenovirus causes (4)
DS linear virus w/o envelope
Pharygitis
Conjunctivitis
Pneumonia
hemorrhagic cystitis - most common viral cause in kids
Oseltamivir MOA
Acts to block neuroaminidase activity and viral release
2nd most common cause of osteomyolitis in kids
- Staph aureous
2nd Strep pyrogenies
Patient has a fever, abdominal pain and diarrhea w/ a little blood. A couple days later he has hematuria and oliguria. Dx?
Hemolytic Uremia Syndrome
Due to Shigella like toxin EHEC or from Shigella reentry
thrombocytopenia
No DIC
how does digoxin slow the HR
in addition to blocking Na/K ATPase -> increase in intracellular Ca
it also activates the vagus nerve
Drugs used as anti nausea and sea sickness
meclizine M1 and H1 blocker
Dihendrimate M1 and H1
Scopolamine - H1
acute pancreatitis is due to inappropriate activation of what enzyme most likely
trypsinogen -> trypsin which activates all pancreatic zymogens
lipase is secreted in active form and does not need further activation
Thiamine deficiency is bad in alcoholics b/c
it is a cofactor in enzymes like pyruvate dehydrogenase (links glycolysis to TCA) and thus impaired glucose breakdown. -> wernicke in the acute setting (mammary bodies) Korsakoff in chronic
give glucose w/out thiamine exacerbates
hemachromatosis is a defect in?
Iron absorption
Mutation on HLA - H on chromosome 6
essential cofactor for transamination reactions
B6 pyridoxine
ALT and AST
-alpha leto group -> amino acid
Decarboxylation reaction
glycogen phosphorylase
the onset of action of a inhaled anesthetic is dependent on what?
Example of a rapid onset drug
the lower the blood solubility the faster the onset. Spills into the brain that much faster
-can be expressed as blood/gas partition coefficient; lower the coefficient the faster the effect
NO2 is an example
Statisical power is
the capability of rejecting the null hypothesis when it is truly false
1 - beta
rare complication of a helical enveloped RNA virus that initially causes fever, cough, coryzea and conjunctivitis
subacute sclerosing panencephalitis
- due to virus missing an M antigen leading to no antibodies to this and failure of clearance
presents years later w/ataxia, myoclonus and visual disturbances
Live attenuated viruses(6)
Smallpox yellow fever chickenpox Sabins polio MMR Influenza - intranasal
Killed viruses(4)
RIP Always
Rabies
Influenza - injectes
Polio (sabins)
HAV
Responsible for the breakdown of very long FA
peroxisome
also oxidase and catalase to metabolize toxic substances
holosystolic blowing murmur
mitral regurgitation
esophageal varices is due to anastomose between
L gastric vein and esophageal veins
hemorrhoids is due to anastomose between
superior rectal veins and middle/inferior rectal veins
Superior laryngeal nerve innervates
external branch =motor - the cricothyroid
internal branch = sensory above the vocal cords
Rest of the larynx innervated by recurrent laryngeal nerve - > muscle and sensory (below vocal cords)
All Vagus
the part of the nephron w/ the highest osmolarity in absence of ADH
right after the descending loop of hence, only part really permeable to H2O if no ADH is available
enzyme in bacteria w/ 5-3’ exonucleus activity
DNA polymerase I, removes the RNA primers and replaces w/ gap with 5’-3’
Polymerase III does general replication
What drugs cause increased concern for Lithium toxicity if given together
Thiazide diuretics due to Li being treated like Na due to exclusive renal excretion. The loss of Na causes the proximal tubules to reabsorb more +1 cations (Na and Li)
also worry about NSAIDs and ACE inhib
beta blockers in hyperthyroid state help in 2 ways
mitigate sympathetic stimulation
block peripheral conversion of T3->T4 through some unknown way
18 month with fever, rhinitis and pharyngitis -> brassy cough and respiratory distress
Croup by parainfluenza
test for primary hypothyroidism
TSH
Drug induced SLE is often due to what sort of metabolism problem
slow acetylators in Phase II liver metabolism
vonjugation reactions -> very polar, inactive real excreted metabolites
HIV Viral proteins encoded
gag
env
pol
gag- p24; capsid
env - gp120 and gp 41; membrane
pol - reverse transcriptase
Drug of choice in ischemic ventricular tachycardia post MI?
lidocaine - Class 1B; Amiodarone also
tocainide
mexliletine
more specific RA marker
anti cyclic citrullinated peptide
anti CCP
red ragged muscle fibers on gomori Trichrome stain indicates
mitochondrial mutation
Lambert Eaton presentation (4)
auto Abs to presynaptic Ca channels
Have issues w/ proximal muscle weakness that IMPROVES w/ muscle use (vs myasthenia gravis)
also antimuscarinic and CN (like III) involvement common
50% associated w/ small cell lung CA
cafe au late spots associated w/ (2)
neurofibromatomas type 1
mckune albrite syndrome
Examples of JAK kinase activators(4)
Growth hormone, prolactin, IL2, receptors for cytokines
tyrsosine kinase associated
Examples of tyrosine kinase activators(3)
Growth factor receptors; EGF, PDGF, FGF
intrinsic kinase activity that phosphorylates RAS
polyribisylribitol phosphate Ab indicates protection from
Haemophilus influenza
- type b capsular polysaccharide conjugated to diphtheria toxoid
allosteric activator of gluconeogenesis is?
Leads to increased pyruvate carboxylase
acetyl CoA
Increased acetyl CoA( from beta oxidation of FA)
-> increased pyruvate carboxylase reaction which sets the stage to get pyruvate out of the mitochondria (trapped w/ pyruvate kinase action) by converting pyruvate into oxelacetate (w/ biotin and pyruvate carboxylase)
-> malate which leaves-> oxelacetate again which can be converted back to glucose (1st step PEP carboxykinase)
Rx for Conn’s Syndrome
Presentation?
Sprinolactone and epleronone
hypokalemic alkalosis, decreased renin activity, hypertension and fatigue, periodic HA
Primary abducter of the humerous
supraspinatus
- most commonly injured rotator cuff muscle, inserts superiorly on the head of the humorous and subject to impingement injury w/ acromion
air in the gull bladder w/ long standing Hx of choleithiathias
gallstone in the ileum
K1 antigen
E coli neonatal meningitis
what needs to be substituted when using amphotericin B
K and Mg
Renal toxicity is a big side effect, cause renal vasoconstriction and decreased GFR; increased distal tubule permeability w/ necrosis
parvo virus in adults vs kids
kids - 5ths disease
Adults is hydrous fettles if pregnant, bilateral arthritis ~ RA that is self resolving
Leptin is?
Acts where
a protein made my adipocytes that act on the arcuate nucleus of the hypothalamus blocking the production of neuropeptide Y -
says I’m full - produces alpha MSH instead
AA that feeds N directly into the urea cycle
Aspartate
2 causes of fat redistribution due to medications
- Glucocorticoids -> cushings syndrome
2. HAART -> lipodystrophy; protease inhibitors
Major contraindication for metformin
Renal failure
- Lactate acidosis is common side effect
Thyroid levels are monitored w/ what 2 drugs?
Lithium
Amiodarone
albumino cytologic dissociation
CSF finding in Guillain barre syndrome where the protein is increased w/ no change in cell count
Arteries w/ nerves to relook at
anterior interosseous posterior interosseous posterior circumflex suprascapular artery deep brachial artery dorsal scapular artey lateral thoracic artery Ulnar artery brachial artery
anterior interosseous - anterior interossous nerve (median)
posterior interosseous - deep radial
posterior circumflex - axillary
suprascapular artery - suprascapular nerve
deep brachial artery - radial
dorsal scapular artery - dorsal scapular nerve
lateral thoracic artery - long thoracic nerve
Ulnar artery - ulnar nerve
brachial artery - median nerve
general empiric treatment with a baby’s infection
ampicillin and gentimycin
SIDS
When does it occur
Prevention
2-4 months
sleep on back avoid co sleeping smoke exposure,
Preventative- breastfeeding, pacifier, fan
Risk factors for SIDS
Maternal
baby
smoker, low SES, <20
Low birth weight, male, premature, prone sleeping,
Low birth weight bevy defines as?
Risk of (5)
<2500 g
infections/sepsis neonatal RDS necrotizine entercolitis (feeding too fast) circulatory problems (insufficiency) SIDs
Neplasms in kids(12)
ALL Astrocytoma Neuroblastoma - adrenals hemangioma Wilms tumor Hepatoblastoma retinoblastoma rhabdomyosarcoma ewing sarcoma osteogenic sarcoma lymphoma teratoma
Tanner stages in
Boys genitalia
1 -prepubital
- enlargement - scotum /testes
- enlargement - penis length
- Penis grows in breadth/testes enlarge
- Adult
Tanner stage in girls
- prepubital
- bud elevation, aereola enlarges
- Further enlargement
- Areola and papilla form secondary mound
- mature
Female development timeline
Breasts/Growth/menarche
breasts around 11
Growth 12
menarche 13
Male development timeframe
tanner stage 2 - 12
growth 14-15
APGAR
means? - amount
A- appearance - 0 cyanotic, 1 hands/feet, 2 pink Pulse -0 none, 1: 100 Grimace -0 no response 2, pulls away from neg stimulus Activity -0 -limp 2- resisits extension R - respirations 0- none; 1- gasping; 2 crying vigorus
birth - 3 months highlights
rolls over at 3 months
social smile
orients and respons to voice
7-9 month highlights
Sits alone, crawls, transfers toys from hand to hand
Stanger anxiety - when someone approaches
babbles and plays peek a boo, name recognition
12-15 months highlights
walks, babinski disappears
separation anxiety - mom leaves and baby freaks
few words 1-3
12-24 month highlights
climbs stairs, stacks blocks (6 blocks at 2)
use of fork or spoon, 6 words at 15 months
2 word phrases
core gender identity at
2-3 yrs
when to begin potty training?
2-3
Parallel play vs cooperative play time line
2-3 yrs vs 4 yrs
Jumping kid and feeds w/ fork/spoon
2-3 yrs
copies lines and circle, stairs and complete sentences
3 yr old
grooms self, hops on one foot and has an imaginary friend
4 yr old
translocation w/ AML M3
characteristic smear/
(t 15:17)
Auer rods (many) -Some may be in M2 (t 8:21)
TdT - deoxynucleotidetransferase is positive when?
ALL
Precursor b
- CD 19 or CD10
Precursor T cells
- CD 1, CD2 or CD5
most common clinical manifestations of Vit E deficiency (2)
neuromuscular disease
-skeletal myopathy
-spinocerebellar ataxia
pigmented retinopathy
Hemolytic anemia
protects FA from beta oxidation
hydlrazine and minoxidil have what side effects?
reflex tachycardia and renin activation -> Na and water retention
Patients w/ essential fructosuria can still metabolize fructose partially through
hexokinase
fructose -> fructose 6 phosphate
low affinity
cricopharyngeal dysfunction leads to
zenker diverticulum
ecthyma gangreosum
necrotic black skin lesion associated w/ septic pseudomonas infections in immune compromised
true vocal cords are covered by?
other areas w/ same covering?
stratified squamous cells
includes upper half of posterior epiglottis, anterior epiglottis, oropharynx, laryngopharynx,
rest is pesudostratified ciliated columnar cells
short acting benzo - <6hrs
triazolam or alprazolam
loazepam is intermediate
the rest are long - >24hrs
- diazepam
- flurezapam
RPF vs RBF
Renal plasma flow vs renal blood flow
get renal blood flow from RPF/(1-Hct)
RPF = [U(urine PAH) x V]/ P(plasma PAH)
postcervical lymph nodes(3)
EBV - mono
cat scratch fever - bartonella henslae
acute otitis media
pigmented harmatoma in the iris
Lisch nodules in neurofibromatosis
50 yr old man w/ new unexplained skin yellowing and no other symptoms need to r/o
pancreatic CA - Head
painless jaundice
alpha keto acid dehydranse
deficiency implicated in maple syrup syndrome
- blocks degradation of leucine, isoleucine and valine
