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USMLE Step 1 – Uworld High Yield Notes Complete Test 2022/2023
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USMLE Step 1 – Uworld High Yield NotesComplete Test 2022/2023
What are the two major microtubular motor proteins? Which does anterograde axonal transport,
which does retrograde axonal transport? Correct Answer: Kinesin, Dynein
Kinesin: Anterograde
Dynein: Retrograde`
What do you think, immunologically speaking, when you see someone with recurrent Neisseria
infections? Correct 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? Correct
Answer: NADPH Oxidase deficiency leads to the inability to kill intracellular organisms
Characteristic triad of ataxia telangiectasia? Correct Answer: cerebellar ataxia, telangiectasias,
increased risk of sinopulmonary infections
What is the major immune deficiency of ataxia telangiectasia? Correct 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? Correct
Answer: ATM
Ataxia
Telangiectasia
Mutated
ATM gene is responsible for DNA break repair
What is one reason that lead intoxication causes hypochromic anemia? Correct 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?) Correct 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? Correct 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? Correct 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? Correct Answer: Kinesin.
Towards the (+) rapidly growing end.
What is an early BRAIN finding of Ataxia-Telangiectasia and how does it manifest itself? Correct
Answer: Cerebellar atrophy
manifests as ataxia in the first years of life.
High yield path association for Alzheimer's disease? Correct Answer: Neurofibrillary tangles in the
neocortex
High yield path association for Parkinson's disease? Correct Answer: Loss of neurons in the
substantia nigra
High yield path association for Huntington disease? Correct Answer: Atrophy of caudate nucleus
What are the major manifestations of Ataxia-telangiectasia? Correct 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? Correct Answer: Prolactinoma, the most common pituitary
adenoma.
What embryological layer is the anterior pituitary derived from? Correct Answer: Surface ectoderm
What is the most common congenital abnormality associated with the use of Sodium Valproate during
pregnancy? Correct 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? Correct 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? Correct 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? Correct 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? Correct Answer: yes and yes
What is the consensus and professional standard regarding the receiving of gifts from patients?
Correct 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? Correct 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? Correct 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? Correct 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? Correct 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? Correct 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? Correct
Answer: Call Child Protective Services immediately!
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