STEP Misc. Flashcards
Pathology of hemolytic Uremic Syndrome
Infection with enterohemorrhagic Escherichia coli and Shigella dysenteriae from a production of shiga like toxin and shiga toxin respectively
symptoms: microangiopathic hemolytic anemia (fatigue, jaundice, dark urine), thrombocytopenia, renal dysfunction
language development at 12months
says first words, points to objects, uses several gestures with vocalizing, recognizes names of two objects, looks at them when named
language development at 15 months
uses three to five words
points to one body part
mature jargoning
language development at 18 months
uses 10-25 words
points to self
imitates environmental sounds
language development at 24 months
50+ words, 50% intelligibility, uses 2 word sentences, follows two step commands
language development at 36 months
uses 200+ words, 3 word sentences, 75% intelligibility, uses pronouns correctly
language disorders
deficiency in understand and producing language
can result in frustration
4 year old child milestones
walks down stairs with alternating feet, catches a ball, balances on one foot
draws square, ties a knot, cuts with scissors
able to tell sories
imaginative play/group play
language disorders
deficiency in understand and producing language
can result in frustration
**obtain a audiology evaluation for any child with a deficient language development
etiology of Paget disease of breast
ductal carcinoma insitu has malignant cells that migrate into the lactiferous sinuses and nipple skin
accumulation of malignant cells disrupts the normal epithelial barrier
(resembles eczema- erythema and scaling crust around nipple)
- large cells with clear cytoplasm confined to the epidermis
MOA of local anesthetics
inhibit sodium channels and is most effective in rapidly firing neurons
classes of local anesthetics
esters (one i)
amides (two i’s)
side effect of bupivicaine
cardio toxicity
order or sensory loss from a local anesthetics
pain, temperature, touch, pressure
side effect of bupivicaine
cardio toxicity
order or sensory loss from a local anesthetics
pain, temperature, touch, pressure
classes of local anesthetics
esters (one i)
amides (two i’s)
side effect of benzocaine
methemoglobinemia
special considerations for local anesthetics
if pt is allergic to ester compound use amide
if there is infected tissue there will need to be a higher dose (infection causes an acidic environment)
signs of riboflavin (vitamin B2) deficiency
angular chelitis, stomatitis, glossitis, rash, normocytic anemia
signs of niacin deficiency
diarrhea, dermatitis, dementia, neurological disturbances
-rash on sun exposed areas
classes of local anesthetics
esters (one i)
amides (two i’s)
special considerations for local anesthetics
if pt is allergic to ester compound use amide
if there is infected tissue there will need to be a higher dose (infection causes an acidic environment)
signs of niacin deficiency
diarrhea, dermatitis, dementia, neurological disturbances
-rash on sun exposed areas
signs of folate deficiency
microcytic megalobalastic anemia with increase homocysteine levels with hyperhsegmented neutrophils
signs of vitamin B6 deficiency
peripheral neuropathy, sideroblastic anemia (microcytic), chili tis, stomatitis, glossitis
signs of vitamin C deficiency
easy bruising petechiae, bleeding gums, anemia, coiled hair
vitamin E deficiency
biliary disease, pancreatitis, hemolytic anemia , muscle weakness, ataxia
diffuse esophageal spasms and treatment
uncontrolled/uncoordiated contractions of the esophagus with a normal cardiac workup (can present with chest pain and dysphagia or solids and liquids)
calcium channel blockers ( reduce smooth muscle contractility by blocking voltage dependent L-type calcium channels)
pericarditis on ECG
diffuse ST segment elevation with reciprocal PR segment depression
special considerations for local anesthetics
if pt is allergic to ester compound use amide
if there is infected tissue there will need to be a higher dose (infection causes an acidic environment)
vitamin E deficiency
biliary disease, pancreatitis, hemolytic anemia , muscle weakness, ataxia
pericarditis on ECG
diffuse ST segment elevation with reciprocal PR segment depression
signs of vitamin B6 deficiency
peripheral neuropathy, sideroblastic anemia (microcytic), chili tis, stomatitis, glossitis
classes of local anesthetics
esters (one i)
amides (two i’s)
signs of benzodiazepine overdose and treatment
ataxia, AMS, lethargy, slight respiratory depression
treatment: flumazenil ( inhibits alcohol dehydrogenase)
cocaine overdose signs and treatment
tachycardia, hypertension, pupillary dilation, agitation, hallucinations
treatment: bentos, Mixed a/b receptors blocker
opioid overdose signs and treatment
AMS, pupillary constriction, lethargy, respiratory depression and respiratory acidosis, constipation
treatment: naloxone
features of fragile X syndrome
prominent forehead, intellectual disability, large everted ears, long narrow face, MVP
pathogenesis of fragile X syndrome
X-linked dominant trinucleotide repeats of the CGG in the FMR1 gene that causes HYPERMETHYLATION and decreased gene expression
how does MVP sound
late systolic crescendo murmur with a mid systolic click
Down syndrome cardiac complication
endocardial cushion defect (ASD)
Duchenne muscular dystrophy defect
dilated cardiomyopathy
Friedrich ataxia cardiac complication
hypertrophic cardiomyopathy
homocystineura cardiac complication
increased risk of myocardial infarction and stroke
marfan syndrome cardiac complication
aortic dissection, aortic aneurysm, aortic regurgitation, MVP
pomp disease cardiac complications
cardiomegaly, hypertrophic cardiomyopathy
digeorge syndrome cardiac complication
conotruncal abnormalities (ToF, truncus arteriosis)
runer syndrome cardiac complications
bicuspid aortic valve, coarctation of the aorta
williams syndrome cardiac complications
supravalvular aortic stenosis
ASD
wide flexed splitting of A2
cardiac complications in alcohol exposure in utero
ASD, PDA, ToF, VSD
congenital rubella cardiac complications
PDA, pulmonary artery stenosis
cardiac complications from poorly controlled maternal diabetes
transposition of the great vessels, VSD
lithium exposure cardiac complications
ebstein anomaly ( enlarged atria)
hypertrophic cardiomyopathy murmur intensity
increases with reduced preload and decreases with increased preload
- increased preload will precent the approximation of the IV septum and the mortal calve during systole
squatting
increases preload
special considerations for local anesthetics
if pt is allergic to ester compound use amide
if there is infected tissue there will need to be a higher dose (infection causes an acidic environment)
vitamin E deficiency
biliary disease, pancreatitis, hemolytic anemia , muscle weakness, ataxia
classes of local anesthetics
esters (one i)
amides (two i’s)
hand grip
increases afterload
signs of vitamin B6 deficiency
peripheral neuropathy, sideroblastic anemia (microcytic), chili tis, stomatitis, glossitis
familial adenomatous polyposis
AD loss of function mutation in APC suppressor gene on chromosome 5
polyps after puberty and 100% of ppl will develop colorectal cancer
can also have Desmond tumors, brain tumors, and osteomas
Lynch syndrome
AD mutation that leads to defective DNA mismatch repair with microsatilliate instability
-colorectal cancer, endometrial cancer, ovarian cancer
MSH2,MLH1, MSH6
KRAS mutations are implicated in
spontaneous colorectal adenomas that give unregulated intracellular signaling
explain the spontaneous colorectal progression for colorectal adenomas
loss of APC gene leads to decreased interceulalr adhesion and increased proliferation
KRAS mutation leads to unregulated intracellular signaling
loss of TP53 leads to tumor formation
juvenilie polyposis syndrome is caused by
autosomal dominant mutations in SMAD
have hermartosous polyps before the age of 5
special considerations for local anesthetics
if pt is allergic to ester compound use amide
if there is infected tissue there will need to be a higher dose (infection causes an acidic environment)
signs of vitamin B6 deficiency
peripheral neuropathy, sideroblastic anemia (microcytic), chili tis, stomatitis, glossitis
Lynch syndrome
AD mutation that leads to defective DNA mismatch repair with microsatilliate instability
-colorectal cancer, endometrial cancer, ovarian cancer
MSH2,MLH1, MSH6
vitamin E deficiency
biliary disease, pancreatitis, hemolytic anemia , muscle weakness, ataxia
classes of local anesthetics
esters (one i)
amides (two i’s)
juvenilie polyposis syndrome is caused by
autosomal dominant mutations in SMAD
have hermartosous polyps before the age of 5
explain the spontaneous colorectal progression for colorectal adenomas
loss of APC gene leads to decreased interceulalr adhesion and increased proliferation
KRAS mutation leads to unregulated intracellular signaling
loss of TP53 leads to tumor formation
peutz-jeghers syndrome
AD mutation in STK11
-hyperpigmentaed macules on the mouth, lips, hands, and genitalia with an increased risk of breast and gi cancers.
vitamin E deficiency
biliary disease, pancreatitis, hemolytic anemia , muscle weakness, ataxia
classes of local anesthetics
esters (one i)
amides (two i’s)
signs of vitamin B6 deficiency
peripheral neuropathy, sideroblastic anemia (microcytic), chili tis, stomatitis, glossitis
special considerations for local anesthetics
if pt is allergic to ester compound use amide
if there is infected tissue there will need to be a higher dose (infection causes an acidic environment)
Lynch syndrome
AD mutation that leads to defective DNA mismatch repair with microsatilliate instability
-colorectal cancer, endometrial cancer, ovarian cancer
MSH2,MLH1, MSH6
class II antiarrythmics MOA
amiodarone, dofetilide, and sotalol
they block potassium channels which inhibits outward depolarizing currents during phase 3 prolonging ventricular repolarization
more common in dofetilide and sotalol
patients with atrial fibrillation can be treated with chemical cardio version to receive rhythm control what are the options
amioderone, defetilide, flecainide, and sotalol
prolonged ventricular depolarization on ECG
wide QRS –> torasdes
signs of vitamin B6 deficiency
peripheral neuropathy, sideroblastic anemia (microcytic), chili tis, stomatitis, glossitis
special considerations for local anesthetics
if pt is allergic to ester compound use amide
if there is infected tissue there will need to be a higher dose (infection causes an acidic environment)
vitamin E deficiency
biliary disease, pancreatitis, hemolytic anemia , muscle weakness, ataxia
Lynch syndrome
AD mutation that leads to defective DNA mismatch repair with microsatilliate instability
-colorectal cancer, endometrial cancer, ovarian cancer
MSH2,MLH1, MSH6
patients with atrial fibrillation can be treated with chemical cardio version to receive rhythm control what are the options
amioderone, defetilide, flecainide, and sotalol
classes of local anesthetics
esters (one i)
amides (two i’s)
pathogenesis of squamous cell carcinoma
loss of TP53 tumor suppressor gene
biopsy of actinic keratosis shows
hyperkeratosis (thickening of the stratum corneum) and parakeratosis (nuclei in the stratum corneum)
pathogenesis of melanoma
activating mutation in BRAF
medications used to treat BRAF positive melanomas
dabrafenib and vemurafenib which are BRAF inhibitors
classes of local anesthetics
esters (one i)
amides (two i’s)
biopsy of actinic keratosis shows
hyperkeratosis (thickening of the stratum corneum) and parakeratosis (nuclei in the stratum corneum)
patients with atrial fibrillation can be treated with chemical cardio version to receive rhythm control what are the options
amioderone, defetilide, flecainide, and sotalol
special considerations for local anesthetics
if pt is allergic to ester compound use amide
if there is infected tissue there will need to be a higher dose (infection causes an acidic environment)
precursor ot melanoma
dysplastic nevus or lentigo maligna
Lynch syndrome
AD mutation that leads to defective DNA mismatch repair with microsatilliate instability
-colorectal cancer, endometrial cancer, ovarian cancer
MSH2,MLH1, MSH6
signs of vitamin B6 deficiency
peripheral neuropathy, sideroblastic anemia (microcytic), chili tis, stomatitis, glossitis
vitamin E deficiency
biliary disease, pancreatitis, hemolytic anemia , muscle weakness, ataxia
nevus histology
rete rige bridging, shouldering, and lamellar fibrosis
lentigo maligna histology
blue black stained skin with malignant melanocytes that grow along the stratum basalt without invasion below the epidermis
pathogenesis of basal cell carcinoma
loss of function in the PTCH1 tumor suppresor gene which activated sonic hedgehog signaling pathway
classes of local anesthetics
esters (one i)
amides (two i’s)
pathogenesis of basal cell carcinoma
loss of function in the PTCH1 tumor suppresor gene which activated sonic hedgehog signaling pathway
vitamin E deficiency
biliary disease, pancreatitis, hemolytic anemia , muscle weakness, ataxia
special considerations for local anesthetics
if pt is allergic to ester compound use amide
if there is infected tissue there will need to be a higher dose (infection causes an acidic environment)
pathogenesis of psoriasis
mutation of the major histocompatibility complex receptor subtype
patients with atrial fibrillation can be treated with chemical cardio version to receive rhythm control what are the options
amioderone, defetilide, flecainide, and sotalol
biopsy of actinic keratosis shows
hyperkeratosis (thickening of the stratum corneum) and parakeratosis (nuclei in the stratum corneum)
cocaine overdose signs and treatment
tachycardia, hypertension, pupillary dilation, agitation, hallucinations
treatment: bentos, Mixed a/b receptors blocker
medications used to treat BRAF positive melanomas
dabrafenib and vemurafenib which are BRAF inhibitors
signs of benzodiazepine overdose and treatment
ataxia, AMS, lethargy, slight respiratory depression
treatment: flumazenil ( inhibits alcohol dehydrogenase)
signs of vitamin B6 deficiency
peripheral neuropathy, sideroblastic anemia (microcytic), chili tis, stomatitis, glossitis
Lynch syndrome
AD mutation that leads to defective DNA mismatch repair with microsatilliate instability
-colorectal cancer, endometrial cancer, ovarian cancer
MSH2,MLH1, MSH6
many round cells with multiple mitoses with clear cytoplasmic vacuoles scalloping the nucleus
liposarcomas
(most common soft tissue sarcoma)