©THEBRIGHT EXAM STUDY SOLUTIONS 8/19/2024 2:07 PM
USMLE Step 1 - uWorld High Yield
Notes. Exam Questions With Correct
Answers
What are the two major microtubular motor proteins? Which does anterograde axonal transport,
which does retrograde axonal transport? - answer✔✔Kinesin, Dynein
Kinesin: Anterograde
Dynein: Retrograde`
What do you think, immunologically speaking, when you see someone with recurrent Neisseria
infections? - answer✔✔Inability to form the membrane attack (MAC) complex
this is a common complement deficiency
What is the defect in Chronic Granulomatous disease? What is the pathophysiology? -
answer✔✔NADPH Oxidase deficiency leads to the inability to kill intracellular organisms
Characteristic triad of ataxia telangiectasia? - answer✔✔cerebellar ataxia, telangiectasias,
increased risk of sinopulmonary infections
What is the major immune deficiency of ataxia telangiectasia? - answer✔✔IgA deficiency,
which predisposes to infections of the upper and lower airways (and other mucous membranes)
There's a useful mnemonic for Ataxia Telangiectasia and the gene that's mutated. What is it? -
answer✔✔ATM
Ataxia
Telangiectasia
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Mutated
ATM gene is responsible for DNA break repair
What is one reason that lead intoxication causes hypochromic anemia? -
answer✔✔Mitochondrial iron transport is important for Heme synthesis! It's inhibited by lead.
So you don't make heme in your mitochondria and you get hypochromic anemia.
What is the biochemical problem in Lesch-Nyhan syndrome? (And, only if you got that right,
what are the really cool symptoms of it?) - answer✔✔Defective purine catabolism, so buildup of
purines. It's an X-linked recessive disorder with mutated HGPRT gene (hypoxanthine-guanine
phosphoribosyltransferase, if you want to impress people).
Now, if you got HGPRT and purine catabolism right, you can say: self-mutilating behavior on
top of mental retardation, choreoathetoid (think Chorea) movements and spasticity.
What is the defect in Niemann-Pick disease?
What causes that?
What is the common macular finding? - answer✔✔Sphingolipid degradation is defective
Caused by an autosomal recessive defect in sphingomyelinase
Cherry Spot on the macula? Niemann Pick's or Tay Sach's.
If a baby gets exposed to an unclean knife (...) and subsequently develops rigid paralysis, what is
the diagnosis, where do we think this might happen, and how do we prevent it? -
answer✔✔Neonatal Tetanus
Developing Countries
Maternal immunization with tetanus toxoid is the best way to prevent it
an adeuately immunized mom will transfer IgG's across the placenta to the neonate
Which microtubule associated protein aids in anterograde transport of intracellular vesicles and
organelles? Which end of the microtubule do they go towards? - answer✔✔Kinesin.
Towards the (+) rapidly growing end.
What is an early BRAIN finding of Ataxia-Telangiectasia and how does it manifest itself? -
answer✔✔Cerebellar atrophy
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manifests as ataxia in the first years of life.
High yield path association for Alzheimer's disease? - answer✔✔Neurofibrillary tangles in the
neocortex
High yield path association for Parkinson's disease? - answer✔✔Loss of neurons in the
substantia nigra
High yield path association for Huntington disease? - answer✔✔Atrophy of caudate nucleus
What are the major manifestations of Ataxia-telangiectasia? - answer✔✔cerebellar ataxia,
oculocutaneous tenlangiectasias, repeated sinopulmonary infections, and an increased incidence
of malignancy
If you heard: amenorrhea, bitemporal hemianopsia, and enlargement of the pituitary gland on
brain imaging, what would you be thinking? - answer✔✔Prolactinoma, the most common
pituitary adenoma.
What embryological layer is the anterior pituitary derived from? - answer✔✔Surface ectoderm
What is the most common congenital abnormality associated with the use of Sodium Valproate
during pregnancy? - answer✔✔neural tube defects, e.g., meningocele
because valproate inhibits intestinal folic acid absorption resulting in teratogenicity
What is the characteristic histology of a craniopharyngioma? - answer✔✔On light microscopy,
cysts are lined by stratified squamous epithelium, possibly with Keratin perals. Cysts are usually
filled iwth a yellow, viscous fluid that's rich in cholesterol crystals.
Where do craniopharyngyomas arise from? - answer✔✔Craniopharyngiomas are calcified cystic
tumors that arise from remnants of Rathke's pouch (embryonic precuros of the anterior pituitary)
Failure of what process leads due neural tube defects? What can be seen in the amniotic fluid due
to leakage of fetal cebebrospinal fluid? - answer✔✔Failure of fusion of the neuropores.
Alpha-fetoprotein and Acetylcholinesterase may appear in the amniotic fluid
Can a competent patient refuse healthcare? Can a competent patient refuse knowledge of their
potential health situation? - answer✔✔yes and yes
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What is the consensus and professional standard regarding the receiving of gifts from patients? -
answer✔✔Don't do it. It's unethical to accept individual gifts from patients, especially of
signficant value.
AMA doesn't have clear cut answer, but they recommend careful consideration be used. some
general guidelines:
-cash gifts should never be accepted (this includes gift certificates)
-a gift should never influence treatment
-no specific dollar value is given; but as a rule of thumb, it shouldn't be above patient's or
physician's means
-psychiatrist must be super careful even with small gifts
-maintain consistency among all patients
-if physician would be embarrassed or uncomfortable if his colleagues found out about the gift,
don't accept it
Is displacement a mature or immature defense mechanism? What is displacement? -
answer✔✔Immature. A patient redirects emotions from the person or object that's causing the
emotions (e.g., wife), but who it woudl be completely unacceptable to direct those emotions
toward, to a more acceptable, but still inappropriate, person or object (e.g., a wall or a vase).
Is sublimation a mature or immature defense mechanism? How does it work? -
answer✔✔Mature. Unacceptable drives are redirected toward completely acceptable targets.
(Workout hard if you're angry)
Is projection a mature or immature defense mechanism? What is it? - answer✔✔Immature.
Unacceptable or personally disagreeable impulses are attributed to others; e.g., a student who
wants to cheat on a test accuses his classmates of cheating. Or the gayness thing
Is reaction formation immature or mature defense mechanism? what is it? - answer✔✔immature,
where unacceptable feeligns are IGNORED and the opposite sentiment is adopted (forced). man
who's mad at his wife instead compliments her dress
Is splitting a mature or immature defense mechanism? what is it? - answer✔✔immature.
frequently employed by patients with borderline personality disorder, everything is either "good
or bad."
What do you do if you suspect child abuse? Ask the parents or call child protective services? -
answer✔✔Call Child Protective Services immediately!
