uWorld 21 Flashcards

1
Q

what is renal osteodystrophy

A

abnormal bone path seen in chronic kidney disease
PTH increase mobilizes calcium from bones by bone formation, causing OSTEOPENIA and pathologic bone changes similar to those seen in primary hyperthyroidism (OSTEOFIBROSA CYSTICA)

pts can also develop PTH resistance, resulting in low-turnover aplastic bone disease and OSTEOMALACIA

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2
Q

small intestinal bacterial overgrowth is characterized by what

A

overproduction of VITAMIN K and FOLATE, associated with nausea, bloating, abdominal discomfort, and malabsorption

B12, iron, zinc and fat soluble vitamins (except K) all decreased

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3
Q

what happens in the closed-ended gastroducodenal limb that occurs in a gastric bypass surgery

A

bacteria can proliferate (small intestinal bacterial overgrowth) and ferment any food that may be diverted into this segment

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4
Q

what enzyme plays a crucial role in imprinting

A

S-adenosyl-methionine (SAM)- a methyl donor

CYTOSINE methylation

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5
Q

what is an epigenetic process by which eukaryotic organisms modify genes expression without altering genetic code

A

DNA methylation (DNA methyltransferase)

methylation of cytosine-guanine dinucelotide repeats (CpGs) in the promoter region of genes effectively silences transcription of those genes

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6
Q

how does DNA methylation play a role in fragile X

A

methylation happens at CG repeats and fragile X is CGG trinucleotide repeats lead to hypermethylation of cytosine residues and FMR1 inactivation

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7
Q

what is epistasis

A

a phenomenon in which the allele of one gene affects the phenotypic expression of alleles in another gene

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8
Q

what is first-line treatment of major depressive disorder that DOES NOT cause sexual dysfunction

A

BUPROPION

norepi and dopamine reuptake inhibitor

also good for treating depression associated with hypersomnia and low energy

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9
Q

what is escitalopram

A

SSRI

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10
Q

what is a highly sedating antidepressant that can be helpful for patients with insomnia

A

TRAZODONE

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11
Q

the dorsal pancreatic bud forms what

A

pancreatic tail, body, most of the head, and the small ACCESSORY DUCT

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12
Q

the ventral pancreatic bud forms what

A

uncinate process, portion of the pancreatic head, and the proximal portion of the MAIN PANCREATIC DUCT

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13
Q

how is the main pancreatic duct (of Wirsung) created

A

fusion of the remainder of the accessory duct with the ventral duct

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14
Q

what is PANCREATIC DIVISUM

A

when ventral and dorsal pancreatic buds fail to fuse (usually occurs in week 8); the pancreatic secretions are instead drained via two separate duct systems

dominant dorsal duct (of SANTORINI) opens into the duodenum via the minor papilla, and is responsible for draining the majority of the pancreas
smaller ventral duct (of Wirsung) opens into he major papilla, and functions to drain the inferior/posterior portion of the head and unite process

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15
Q

how is pancreatic divisum diagnosed

A

can cause recurrent pancreatitis

normally incidental finding on imagining studies or at autopsy

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16
Q

what removes RNA primer as well as damaged DNA segments

A

5’ → 3’ exonuclease activity

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17
Q

what performs proofreading function that removes and replaces mismatched nucleotides on the newly formed daughter strands

A

3’ → 5’ exonuclease

defective in LYnch Syndrome (HNPCC)

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18
Q

what does an AP endonuclease do

A

cleaves 5’ end
used in base-excision repair
important repair of spontaneous/toxic deamination

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19
Q

how do nitrites cause poisoning

A

inducing conversion of heme iron into OXIDIZED ferric iron → methemoglobin formation

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20
Q

what does methemoglobinemia do to the skin and O2 in the blood

A

DUSKY discoloration (similar to cyanosis)

unable to carry oxygen, state of functional anemia is induced

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21
Q

pyruvate kinase deficiency causes what

A

chronic hemolytic anemia
splenomegaly
iron overload
result of impaired erythrocyte survival

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22
Q

what is acid maltase and what does a deficiency cause

A
ALPHA-1,4-GLUCOSIDASE deficiency
Pompe Disease (type II glycogen storage disease)

cardiomegaly, macroglossia, hepatomegaly, and profound muscular hypotonia
blood glucose levels normal

abnormal GLYCOGEN ACCUMULATION in LYSOSOMES vesicles is seen on muscle biopsy

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23
Q

what is von Gierke disease (Type I glycogen storage disease)

A
GLUCOSE-6-PHOSPHATASE deficiency
hepatomegaly and steatosis
fasting hypoglycemia
lactic acidosis
hyperuricemia and hyperlipidemia
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24
Q

what is Cori Disease (type III glycogen storage disease)

A

DEBRANCHING ENZYME (alpha-1,4-transferase and alpha-1,6-glucosidase) deficiency
hepatomegaly
ketotic hypoglycemia
hypotonia and weakness
abnormal glycogen with very short outer chains

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25
Q

what is McArdle disease (Type V glycogen storage disease)

A

GLYCOGEN PHOSPHORYLASE deficiency
muscle phosphorylase deficiency
weakness and fatigue with exercise
no rise in blood lactate levels after exercise

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26
Q

glycogen accumulation within lysosomal vacuoles is specific for what glycogen storage disease

A

Pompe

acid alpha-glucosidase deficicency

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27
Q

what is the GERMINAL MATRIX

A

highly cellular and vascularized layer in the subventricualr zone from which neurons and glial cells mirgrare out during brain development

contains numerous thin-walled vessels lacking the glial fibers that support other blood vessels throughout the brain

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28
Q

what are the common complications of prematurity

A
respiratory distress syndrome
patent ductus arteriosus
bronchopulmonary dysplasia
intraventricular hemorrhage (Germinal Matrix)
necrotizing enterocolitis
retinopathy of prematurity
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29
Q

intraventricular hemorrhage is a common complication of prematurity, where does it usually originate

A

GERMINAL MATRIX

vulnerable to hemodynamic instability as premature infants can have impaired autoregulation of cerebral blood flow

b/w 24-31 weeks gestation, germinal matrix becomes less prominent and its cellularity and vascularity decrease (reducing the risk of IVH)

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30
Q

berry aneurysms are associated with what disease states

A

Elhers-Danlos syndrome

AD polycystic kidney disease

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31
Q

how do infants get subdural hemotoma

A

rupture of cortical bridging veins

shaken baby syndrome (violent shaking)

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32
Q

what are the central effects of dopamine caused by L-dopa regardless of whether carbidopa is added to levodopa treatment

A

anxiety and agitation (hallucination, delusions, confusion, insomnia)

can actually be increased due to more dopamine in the brain

reducing dose can reverse these

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33
Q

how does carbidopa work

A

inhibits DOPA decarboxylase in the periphery

reduces arrhythmias, nausea, postural hypotension, hot flashes, and vomiting associated with levodopa
also increases compliance

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34
Q

where is concentration of PAH the lowest

A

Bowman’s Space (conc is similar to plasma)

majority is secreted in PROXIMAL TUBULE via carrier-mediated active transport
PAH is NOT REABSORBED by any portion of the nephron

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35
Q

what are the causes of erectile dysfunction

A
psychogenic stressors
performance anxiety or depression
mediaciones (SSRI, sympathetic blockers- methyldopa, clonidine, beta-blockers)
vascular or neurological impairment
genitourinary trauma (prostatectomy)
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36
Q

what is an important clue to psychogenic impotence

A

rapid onset
men who had no sexual difficulty until “one night when they could not have an erection” followed by persistent ED

NO LOSS OF MORNING WOOD

organic causes of impotence will complain of failure occurring intermittently and later became more persistence

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37
Q

in men what happens to sexual desire with age

A

does not decrease sexual desire

longer refractory period and take longer to get an erection

38
Q

vasectomy does not cause ED but what medical conditions can

A

prostate surgery
pelvic trauma
priapism

39
Q

1-week history of hemiplegia and expressive aphasia w/ CT showing hypo density in brain- what cell type would histological findings after staining for lipids lipids

A

MICROGLIA (come in 3-5 days after the onset of ischemia)

macrophages (microglia) phagocytize myelin and therefore stain for lipids

40
Q

when are astrocytes seen in brain repair after ischemia

A

within 2 weeks of injury

as necrotic tissue is resorbed, a cystic space forms that is then surrounded by astrocytes and newly formed capillaries
enlargement and proliferation of astrocytes peripherally around the area of necrosis is called GLIOSIS (glial scare formation)

41
Q

when are neutrophils seen following ischemia in the heart

A

24-48 hours after interruption of blood supply

do not phagocytize myelin remnants and therefore do not stain of lipids

42
Q

what drug used for treating mixed hyperlipidemia can precipitate GOUT

A

NIACIN

decreases renal excretion of URIC ACID, leading to elevated blood levels and increased risk for ACUTE GOUTY ARTHRITIS

43
Q

what is the most effective agent for RAISING HDL LEVELS

A

NIACIN

however, it has not been shown to reduce the risk of cardiac events, it is primarily used in patients who have failed other lipid-lowering drugs

44
Q

what lipid lowering drug can cause hypertriglyceridemia

A

BILE ACID-BINDING RESINS

GI upset and impaired absorption of nutrients also seen

45
Q

why lipid lowering drug can cause CHOLESTEROL GALLSTONES

A

FIBRATES

also severe myopathy w/ statins

46
Q

what testing should patients with Zollinger Ellison (gastrinoma) undergo and why

A

serum calcium, prolactin levels, many more

MULTIPLE ENDOCRINE NEOPLASIA (MEN) TYPE I

47
Q

what is a somatostatinoma

A

rare tumor of the pancreatic D cells that present with:
diabetes mellitus (diminished insulin secretion)
cholelithiasis (inhibition of CCK)
diarrhea/steatorrhea (inhibition of pancreatic enzyme/bicarbonate secretion)

48
Q

what are the contact precautions (infection control measures) of MDR organisms (MRSA, VRE), enteric organisms (C. diff), and scabies

A

hand hygiene (SOAP AND WATER for C Diff)
NONSTERILE gloves
GOWN
private room preferred

dedicated BP cuff and stethoscope should be left in the pts room

49
Q

what organisms require droplet precautions (wearing a simple facemark when within 6-10 feet of affected people)

A
Neisseria meningitides
influenza
Bordetella pertussis
mycoplasma pneumonias
RSV

gloves and gowns not required

50
Q

what are and when would airborne precautions be used

A

isolated room with negative pressure ventilation and a respiratory mask with filtering capacity (N95 mask)

TB and varicella (respiratory droplets that chill in air for a long time)

51
Q

the glossopharyngeal nerve proves general sensory to what

A
tympanic membrane (inner surface)
eustachian tube
posterior third of tongue
tonsillar region
upper pharynx (afferent portion of gag reflex)
carotid body
carotid sinus
52
Q

what is the IFN-gamma release assay (IGRA)

A

test for LATENT TB infection by measuring the response of T lymphocytes when exposed to antigens unique to TB

similar to TB skin tests, measure cell-mediated immunity, but lack of BCG cross-reactivity

53
Q

what findings suggest Toxo encephalitis in HIV patient

A

headaches, seizures, and multiple ring-enhancing CNS lesions

54
Q

5 year-old with difficulty breathing, dysphagia, drooling, fever (103)
white count of 23,000 with many bands (left shift)
swollen and CHERRY-RED EPIGLOTTIS on laryngoscopy
da fuq he got?

A

H. flu
either not vaccinated or missed a vaccination

inspiratory STRIDOR

older children would have sore throat proly not dysphagia and fever

55
Q

when prescribing opioids and suspecting abuse what should one do

A

validate the patients concern about pain control
engage the pt in a nonjudgmental, COLLABORATIVE DISCUSSION of how he is using the mediation; and EXPLORE REASONS for his ESCALATING USE

56
Q

what is Crigler-Najjar syndrome type 1

A

AR disorder of bilirubin metabolism caused by genetic lack of UGT enzyme needed to catalyze bile glucuronidaiton

build up of UNCONGUJATED bilirubin that is BOUND to ALBUMIN

can cross BBB and cause KERNICTERUS (bilirubin encephalopathy)- potentially fatal causing jaundice and neuralgic impairment (rigidity, lethargy, seizures)

57
Q

deficient bilirubin excretion into bile canaliculi is seen in what disease

A

DUBIN-JOHNSON SYNDROME (AR)
absence of biliary transport protein, MRP2 (multi drug resistance protein 2), used in hepatocellular excretion of bilirubin glucuronides into bile canaliculi

darkly pigmented liver and asymptomatic

conjugated hyperbilirubinemia

58
Q

impaired canalicular bile transit is seen in what disease

A

ROTOR SNYDROME (AR)
asymptomatic conjugated hyperbilirubinemia
often jaundiced but normal life expectancies

59
Q

what (and how) can anaerobes and Staph Aureus deconjugate bile acids

A

removal of glycine and taurine

results in lipid malabsorption b/c they less soluble and less able to form micelles

60
Q

what is stercobilin

A

urobilinogens that remain in the colon and are excreted

contribute dark color to stool

61
Q

exposure to aminoglycosides in utero can cause what

A

cranial nerve VIII problems

sensorineural hearing loss
vestibular instability and ataxia

62
Q

what odes exposure to ACEI do to fetus

A

impaired fetal renal function and cause fetal anuria and oligohydramnios

63
Q

what should be used to treat LYME in PREGNANT WOMEN

A

AMOXICILLIN

64
Q

acute painless PERMANENT monocular vision loss is characteristic of what

A

CENTRAL RETINAL ARTERY OCCLUSION (CRAO)

vision loss includes entire visual field and is often permanent

fundoscopic findings: PALE RETINA (due to ischemia and edema) and CHERRY-RED MACULA (fovea and fovea are thin and have a separate blood supply from the choroid artery; red choroid is easily visible underneath))

65
Q

what is the most common cause of central retinal artery occlusion (CRAO)

A

ATHERO- and THROMBOEMBOLISM

predisposing conditions are AFIB and CAROTID ARTERY STENOSIS or vasculitis (giant cell arteritis)

66
Q

what is amaurosis fugax

A

painless, transient, monocular vision loss caused by a small embolus to the ophthalmic artery
usually does not last more than a few seconds

67
Q

how does diabetic retinopathy manifest

A
blurry vision
black spots
floaters
decreased peripheral vision
acute visual loss may occur in the setting of a complication such a vitreous hemorrhage

NEOVASCUALRIZATION and HEMORRHAGE

68
Q

temporal lobe stroke can cause what visual defects

A
MEYERS LOOOP (GENICULOCALCARINE TRACT) fucked up
carries impulses from the lower retina
can cause contralateral upper quadrantanopsia ("pie in the sky" visual defect)
69
Q

left frontal lobe lesions have an association with what

A

APATHY

DEPRESSION

70
Q

right frontal lobe lesions have an association with what

A

DISINHIBITED BEHAVIOR

71
Q

the frontal lobe is primarily responsible for what

A

EXECUTIVE FUNCTION (ability to perform complex tasks and includes motivation, organization, planning an d purposeful action) and PERSONALITY

lesions: social and emotional behavior, sexual disinhibition and emotional lability to apathy and depression

72
Q

what happens when the corpus callous is injured

A

“split brain” syndrome
appear normal in general social situations but have inter hemispheric transfer of information
(pt unable to retrieve with one hand an object palpated with the other)

73
Q

parietal lobe lesions result in what

A

difficulties with spatial and visual perception

non-dominant lobe lesions (most commonly right-sided) can realist in HEMI-NEGLECT, constructional APRAXIA, and DENIAL OF PROBLEM

dominant lesions (commonly left-sided): GERSTMANN SYNDROME w/ right-left confusion and difficulty with writing and mathematics (AGRAPHIA and ACALCULIA, FINGER AGNOSIA, LEFT-RIGHT DISORIENTATION)

74
Q

what is Kluver-Bucy Syndrome

A

disinhibited behavior: hyperorality, hyperphagia, hypersexuality
bilateral amygdala lesion

associated with HSV-1 encephalitis

75
Q

what happens with non dominant (usually right-sided) lesions of the temporal lobe cause what

A

affect nonverbal memory, including musical ability

76
Q

what happens with dominant (usually left-sided) lesions of the temporal lobe cause what

A

verbal memory (word recognition)

77
Q

it is recommended that tratemtn of friends occur when

A

only in EMERGENCY SITUAwTIONs when NO OTHER PHYSICIAN is AVAILABLE

78
Q

the SMA and IMA are connected by what 2 anastomosis

A

MARGINAL ARTERY OF DRUMMOND (principal anastomosis) and inconsistency persistent ARC of RIOLAN (mesenteric meandering artery)

protect intestines from ischemia

79
Q

what two arteries branch off the external iliac artery before it becomes the femoral artery

A

inferior epigastric artery

deep circumflex iliac artery

80
Q

the IVC drains what part of the intestinwes

A

rectum (inferior and middle rectal veins drain into the internal iliac vein

81
Q

cyclosporine is metabolized how

A

liver by CYP3A ISOENZYMES

grapefruit juice can slow the breakdown of drugs metabolized by this pathway

82
Q

what is calcineurin inhibitor nephrotoxicity

A

new-onset hypertension and elevated levels of serum creatinine and cyclosporine

83
Q

what suggests fat embolism

A

acute-onset NEURALGIC abnormalities
HYPOXIA
PETECHIAL RASH
seen in pts 24-72 hours after (but can be as much as 2 weeks) LONG BONE AND/OR PELVIC FRACTURE

84
Q

when would one see a RED THROMBUS lodged in the pulmonary artery

A

thromboemoblim from a deep venous source

non neurologic impairment or petechial rash like w/ fat embolism

85
Q

what should be started in all HF patients with left ventricular (LV) systolic dysfunction (decompensated systolic HF) to improve survival

A

BETA-BLOCKERS (carvedilol, metoprolol)

decreases cardiac work and levels of circulating vasoconstriction hormones (norepi, renin, endothelin)

not used in unstable HF and should be introduced slowly to avoid worsening of the patient’s condition by further impairing cardiac output

86
Q

persistent back pain, fatiguability, constipation with atrophic tubules which contain large, obstructing, intensely eosinophilic, and elevated serum proteins casts suggests what

A

MULTIPLE MYELOMA

fatiguability (anemia)
constipation (hypercalcemia)
bone pain- back and ribs (bone lysis due to production of osteoclast-activating factor by myeloma cells)
elevated serum proteins (monoclonal proteins)
renal failure

AL AMYLOIDOSIS

87
Q

what si myeloma cast nephropathy (“myeloma kidney”)

A

excess excretion of free light chains (BENCE JONES PROTEINS) is the most common form of nephropathy
the light chains precipitate with Tamm Horsfall protein and for casts that cause TUBULAR OBSTRUCTION and EPITHELIAL INJURY

deposition of light chain fragments in the glomerular mesangium and capillary loops can also cause renal failure (AL AMYLOIDOSIS)

88
Q

hypersensitivity interstitial nephritis causes what

A

STERILE PYURIA
often ongoing exposure to an offending drug
eosinophilia and eosinophiluria may help confirm the diagnosis

89
Q

how does ischemic tubular necrosis classically present

A

muddy brown, granular, and epithelial cell casts and free tubular epithelial cells in the urine

90
Q

what does chronic lead intoxication due to the kidney

A

chronic tubulointerstitial nephritis (interstitial fibrosis and tubular atrophy seen on light microscopy) that leads to renal failure