Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

STEP Flash Cards > PT5 > Flashcards

PT5 Flashcards

1
Q

Ornithine transcarbamoylase deficiency

A

Most common urea cycle disorder; X-linked recessive; cannot eliminate ammonia - excess carbamoyl phosphate converted to orotic acid (so you’ll see inc. levels in blood and urine) - decrease BUN and increased ammonia levels in blood

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

If you cannot transport ornithine from cytosol to mitochondria, which substance should you restrict from diet?

A

Protein - you generate ammonia from AA (from protein degradation) and inability to metabolize ammonia into urea means you should restrict protein intake

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Deficiencies of complement factors that form the MAC attack often experience recurrent infections by…

A

Neisseria species

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

DiGeorge syndrome

A

Congenital absence of thymus and parathyroid glands caused by maldevelopment of 3rd and 4th pharyngeal pouches - results in pure T-cell dysfunction (T-cell lymphopenia) and thymic hypoplasia. You see recurrent viral and fungal infections (lack of T-cells) and hypocalcemia (no PTH) - tetany

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Chronic granulomatous disease

A

Example of deficient intracellular killing; X-linked immunodeficiency that results from inability of phagocytes to synthesize NADPH oxidase - an enzyme essential for lysosomal oxidative burst.
Sx: recurrent infections with catalase+ organisms (ex: Staph)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Formula to calculate confidence interval limits

A

Mean +/- 1.96*SD/sqrt(n)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Clinical use for TCAs

A

Major depression, OCD, fibromyalgia, painful diabetic neuropathy

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Side fx of TCA’s

A

Sedation, alpha-1 blocking effects (hypotension), anti-cholinergic (tachycardia, urinary retention, dry mouth)
Tri-C’s = convulsions, coma, cardiotoxicity

Treatment: NaHCO3 for cardio toxicity

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

PCP MOA

A

hallucinogen; inhibits NMDA receptor activation; can cause feelings of detachment and distance; slurred speech, ataxia, nystagmus, paranoia, hallucinations, hostile, aggressive

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What type of necrosis is seen with hypoxic injury to the CNS

A

Liquefactive - infarct releases lysosomal enzymes and phagocytic cells migrate to remove necrotic tissue (leaving cystic cavity) which then becomes surrounded by astrocytes to form scar (gliosis)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

HIV+ female brought to ER with new onset tonic-clonic seizures and MRI shows spherical lesions with surrounding edema in right frontal lobe - diagnosis & treatment?

A

Toxoplasmosis (T. gondii) -cat is the host for this - HIV individuals can develop ecephalitis with necrotizing brain lesions - results in fever, headache, altered mental status and seizures; you see RING-ENHANCING LESIONS in both hemispheres

Rx: pyrimethamine & sulfadiazine

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Presentation fo P. jirovecii

A

Pneumocystis pneumonia (PCP) - diffuse interstitial pneumo; bilateral CXR appearance; disc-shaped yeast on methenamine silver stain

Rx: TMP-SMX, dapsone, can use Amphotericin to treat cryptococcal meningitis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Two most common cause of focal brain lesions (ring-enhancing lesions) in HIV+ patients

A
  1. Toxoplasmosis

2. Primary CNS lymphoma (B-lymphocytes)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What is used to treat emergent acute coronary syndrome

A

Beta blockers

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Reserpine MOA

A

inhibits dopamine entry into presynaptic vesicles - this reduces BP and HR - is not used to treat acute coronary syndrome

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Pinealoma

A

Typically seen in children, most often they are germinomas, cause obstructive hydrocephalus, Perinaud syndrome, precocious puberty (stim. beta-hCG)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Vitamin A toxicity

A

Acute: nausea, vomiting, blurred vision
Chronic: alopecia, dry skin, hepatotoxicity, hepatosplenomegaly, visual problems, papilledema

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Niacin deficiency

A

Pellagra: dementia, dermatitis, diarrhea, death

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

B12 deficiency

A

pernicious anemia, anemic, icteric, smooth shiny tongue (atrophic glossitis), shuffling, broad-based gate, mentally slow

20
Q

Excess vitamin C

A

can give false negative stool guaiac results associated with diarrhea and abdominal bloating

21
Q

Propionic acidemia

A

poor feeding, vomiting, lethargy, anion gap acidosis - breakdown of valine, threonine, methionine, cholesterol, leads to formation of propionic acid which then is converted to methylmalonyl CoA

22
Q

Primary cause of lacunar infarcts

A

Hypertensive arteriolar sclerosis forming micro clots (microatheromas)

23
Q

First line therapy for generalized anxiety and panic disorder

A

SSRI (ex: paroxetine) - but this takes a while to work (~4 wks) - so you can sometimes add a benzo in that waiting period to further reduce anxiety/panic

24
Q

Short-acting benzos

A

Triazolam, Alprazolam

25
Q

Fragile X syndrome

A

most common cause of inherited mental retardation and 2nd most common cause of congenital mental retardation (Downs is #1)

Males: mental retardation, LONG face, prominent jaw, macroorchidism, mitral valve prolapse, short, joint laxity, scoliosis, double jointed thumbs

26
Q

Klinefelter

A

47, XXY - mild mental retardation, tall, gyenocomastia, small tetes, infertility

27
Q

Turner syndrome

A

45 XO - primary amenorrhea and mild mental retardation, short, webbed neck, shielded chest, ovarian dysgenesis

28
Q

Marfans

A

tall, arachnodactyly, ectopia lentis, dilatation of proximal aorta

29
Q

Radial head subluxation

A

aka nursemaid’s elbow- commonly injured in children - can damage deep branch of radial nerve (wrist drop) - sensation still intact because nerve has branched by that point and superficial radial n. not affected

30
Q

Musculocutaneous nerve pierces…

A

heads of the coracobrachialis

31
Q

Homocystinuria

A

most common inborn error of methionine metabolism - cystathionine synthetase deficiency - you get thromboembolic episodes (esp in brain)

50% respond dramatically to pyridoxine (B6) supplementation

32
Q

B1, B2, B3, B5, B6, B7, B9, B12

A

thiamine, riboflavin, niacin, pantothenic acid, pyridoxine, biotin, folic acid, cobalamins

33
Q

Which NT can help modulate opiod tolerance

A

glutamate can interact with opioid pathways to modulate morphine tolerance - binds to NMDA receptors can can increase phosphorylation of opioid receptors which will increase morphine tolerance.

NMDA antagonists (ketamine) can block morphine tolerance
Dextromethorphan can reverse tolerance via NMDA antagonism
34
Q

Carbamazepine MOA and side fx

A

blocks voltage-gated Na channels in cortical neurons - stabilizes them in an inactivated state
Side fx: bone marrow suppression (agranulocytosis and thrombocytopenia), hepatotoxic, SIADH

35
Q

Side effects of first generation antihistamines

A

blockade of other pathways:
musucarinic pathways - you get blurry vision, dry mouth, urinary retention

serotenergic pathways, you see appetite stimulation

alpha adrenergic pathways - you see postural dizziness

Sedation

36
Q

Second-generation antihistamines (example)

A

Fexofenadine- does not have antimuscarinic, antiserotonergic, or anti-alpha adrenergic properties
DO NOT CROSS BBB (non-sedating)

37
Q

Effective against absence AND tonic-clonic seizures

A

Valproate

38
Q

C. botulinum poisoning

A

Diplopia, dysphagia, dysphonia, nausea, dry mouth, descending paralysis
Prevents binding and fusion of ACh vesicles with plasma membrane (blocking release into synapse)

39
Q

Treatment for narcolepsy

A

agents that promote wakefulness (psychostimulant) - MODAFINIL (non-amphetamine stimulant)

40
Q

Narcolepsy pathophysiology

A

low levels of orexin (hypocretin)

41
Q

Arginase

A

enzyme that converts arginine to ornithine and subsequently releases urea
Rx: low-protein diet that is devoid of arginine
Sx: spastic paresis, choreoathetoid movements

42
Q

Valproate toxicities

A

associated with neural tube defects - inhibits intestinal folic acid absorption

43
Q

Rabies clinica presentation and what receptor does it act on

A

agitation, disorientation, pharyngospasm, photophobia, coma, death - rabies encephalitis - avoidance of food and water - mouth foaming - RHABDOVIRIDAE binds to nicotinic ACh receptors on peripheral nerves and retrograde movement to CNS

44
Q

Endogenous opioids: beta endorphin comes from?

A

POMC polypeptide - also gives off ACTH and MSH (- so they are all related!

45
Q

Somatomedin C is structurally similar to?

A

Insulin - also called insulin like growth factor

STEP Flash Cards (14 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions